The journals I kept through our journey

Jeff's Journey

(A compilation of emails from the beginning to the end of his valiant struggle with Wilson's disease)
To be used for the book "Wilson's disease - A Father and Son's Journey into Hell and back again!"

{Note: because of the litigation and subsequent settlement against this demonic HMO, I am not at liberty to reveal its name or the names of the doctors involved. I have changed the name of this 2 word HMO to just "HMO." I hope the readers will be able to put together the name of this 2 word HMO by the clues left in this summary. Anyone who has had experience with this HMO will know which one I am talking about. I believe it is the largest HMO in the country.}

In The Beginning... How it all began. 1999

My son Jeff was diagnosed about 2 1/2 years ago with Wilson's disease, a genetically inherited degenerative disease that affects the liver and the brain. The disease is so rare that only 1 in 40,000 people get it. Both parents have to carry the regressive gene as well as a normal gene, and then a regressive gene from each parent has to be passed on before a child can be affected. The possibilities are as follows: Husband-2 normal, Wife-2 normal = normal child; husband-1 normal, 1 regressive and wife-2 normal = normal child, but a carrier; husband-2-normal and wife-1 normal, 1 regressive=normal child, but a carrier; husband-1 The parents are simply carriers with no symptoms, passing the gene on to one, several or all of their children. The liver does not process copper like it should thereby causing a toxic accumulation of copper in
the body. If it is not diagnosed and treated in time, the patient will suffer irreparable liver and brain damage and eventually die.

There is no cure for this disease but it is treatable, and if a person gets diagnosed before any major symptoms appear, they can live a totally normal life by taking a zinc supplement 3 times a day for the rest of their lives. Unfortunately, Wilson's Disease is very rarely diagnosed until all other more common diagnoses are exhausted, and even then it is still overlooked. The list of improper diagnoses can include hepatitis, multiple sclerosis, Parkinson's disease, palsy, insanity and so on and so on. The sad thing is that if the neurological symptoms continue to worsen to the point of irreparable brain damage, there's not a lot anyone can do. The initial test is so simple it is scary to think that the disease is usually not detected until well into doing its damage.

Jeff had gone to a local optometrist (fortunately well-trained) to get contact lenses. During the exam the doctor recognized the telltale Kayser-Fleischer copper-colored rings around his irises. He advised Jeff to see a doctor immediately which Jeff did. After a special blood and urine test he was positively diagnosed and put on penicillamine, a de-coppering agent with many adverse side effects for most people, Jeff included. For some reason, the doctor wanted a second opinion after the problems with side effects. Jeff was sent to a specialist who ordered a CTScan. The doctor decided Jeff had a brain tumor! After several agonizing weeks of waiting, the doctor informed Jeff that he didn't have a brain tumor. The entire family was relieved. Even though Wilson's Disease is incurable and we were all really upset about that, it is treatable if it is detected early enough, but a brain tumor was just plain scary!

The doctor then decided that he had a degenerative sinus bone disease and would need a special operation to repair it before his entire sinus structure dissolved. Once again the family was in turmoil. The main point to make here is that the original Wilson's disease diagnosis just sort of got lost and forgotten in the madness.

Jeff decided against doing anything about the sinus problem. After all the doctors weren't very encouraging with multiple incorrect diagnoses. Then around June of 2001 his speech started to slur. This was right after a wisdom tooth had been pulled so I thought maybe a nerve was damaged or maybe he had had a small stroke. He went to many doctors and they all seemed to agree on the same diagnosis - he had a speech problem! When I asked him why he said they didn't know. At the same time he was having problems at work. His co-workers were making fun of and mimicking the way he talked. This brought his emotional level down dramatically. At this point one doctor decided that Jeff needed a psychiatric evaluation because of his speech problem and his emotional state.

Jeff was missing a lot of work because his speech was getting worse and other symptoms such as bloody noses, chronic headaches and insomnia had him going back to the doctor. He was going to emergency because his work/insurance doctor was pushing for a psychiatric exam which he didn't think he needed. At this time he lost the lease on the condo he was sharing with his half-brother Danny. He moved in with his older friend Betty and continued to work the best he could with all his problems. He continued to get sicker and missed more work. On a weekend in August he spent in the hospital for blood tests and observation because he knew something was wrong, his work fired him for excessive absences. Jeff was devastated. It was bad enough that he was sick and didn't know what was wrong, but to be ridiculed and then fired by job he had held for 5 years and worked his way up to a good-paying position put him over the edge! His employer fought his unemployment benefits and Jeff had to appeal. He won and had until the middle of February to find a job.


Jeff had no money and nowhere to go so I welcomed him with open arms to my little house on 2.5 acres on the central coast of California which I shared with his step-mother. On his last day in Fresno on his way out of town, his Camaro was broadsided in the front by an old man in a big truck. The man said it was his fault, gave Jeff his information and left. As Jeff was trying to figure out how to get his car off the road since he had no money for a tow truck, a police officer came by to offer assistance. Jeff told him what happened and the officer asked for his ID. The next thing Jeff knew, his car was being towed away and the officer confiscated his license. It turns out Jeff had a speeding ticket about a year earlier and although he paid it, he paid late and the court doesn't send out late notices, just warrants! At this point Jeff so demoralized I am surprised he could even go on with these new problems added to his health problems.

He was supposed to move in with a friend but just before he got here, his friend was evicted from the property his mobile home was on. It seems that the owner of the property put it there illegally. Add another obstacle to his already confusing life. Jeff is a good young man so why was this happening to him? I told him he was at the bottom and now he needed to start looking up and things would get better. Were we ever in for a reality check!

Jeff did not want to impose on me and his step mother since we didn't even have a bedroom for him, but I told him he could stay until he found another place and a job. It was early September when he moved in. I watched him over the next few months try without any luck to get a job. A great position in a nuclear power plant came up which was in his line of expertise. He told me that they did a psychological exam as well as verbal interviews. He was not chosen for the job even though his qualifications and experience were more than enough to land it. Jeff sank deeper and again I reassured him.

It was about December when I noticed Jeff's walking seemed a bit labored. I asked him if he had injured himself and he said his legs just felt a little bit stiff. He had a new HMO plan he got through his friend Betty so I suggested that he go and get a complete checkup. Betty took him to the doctor, or I should say a physician's assistant since doctors were scarce in that HMO hospital. They ran some tests on him but found nothing wrong other than his speech and sent him to a speech therapist to help with his slurring. I should also mention at this point that he told the assistant during his first visit that he had been previously diagnosed with Wilson's disease.

Over the next two months Jeff's speech and walking got worse. The HMO hospital ran different tests for
him with no luck. Finally at the beginning of February Jeff went back to Clovis to stay with his friend Betty who worked at his hospital. I told him to go back to the hospital and take Betty with him, and if he didn't get some answers I would kick his butt! I think Jeff is typical of a lot of young men at that age. They just assume that if a doctor says they are ok then they must be ok. I told Betty to make sure the PA (physician's assistant) was aware of Jeff's disabilities and how they seemed to be getting worse. This time the PA took notice and ordered an MRI to see if Jeff's brain was ok. Visions of brain tumor raced through my head again. The results of the MRI were not good. The PA said there were what appeared to be plaques in the brain tissue and there was a good possibility that Jeff had Multiple Sclerosis since he had all the other symptoms as well. Jeff was referred to a neurologist who looked at the MRI and said it wasn't MS. He was pretty sure Jeff had Wilson's disease. Jeff only told them that several months earlier! By this time his hands were shaking with the traditional tremors of Wilson's. The neurologist scheduled an immediate 24 hour urine test, an ophthalmologist appointment for the next week to verify the Kayser-Fleischer rings and a liver biopsy for sometime in a couple of weeks to determine if there was any liver damage. I saw Jeff deteriorate rapidly in less than a week and these guys were talking tests that could take up to a month?

Once the Wilson's word resurfaced, my wife and I immediately got on the internet to find out as much about the disease as we could. I was amazed by the amount of information available and the constant references to misdiagnosis and ignorance of treatment options by physicians. The neurologist finally called and confirmed that Jeff indeed had Wilson's disease. The fact that Jeff told them that 3 months prior was not mentioned. The physician's assistant said the case was way over her head and referred Jeff to a real doctor finally. The neurologist put Jeff on 500mg of Penicillamine 3 times a day, which is the drug of choice of physicians living in the dark ages. The problem with Penicillamine is that it has extremely detrimental neurological side effects and devastating brain damage if the patient is already experiencing neurological symptoms. It basically back-flushes the toxic copper out of the liver, which is a good thing, however, in doing so it sends it to the brain which just adds to the damage already there. The additional damage is generally irreversible!

When I discovered this widely available fact, even to the lay person, I was understandably upset. I found a support group at Wilsonsdisease.org, joined and immediately told our story to the group. Dr. Ascher Selner sent me an email to call him. I thought it was an 'autobot' from the group so ignored it at first. Several people emailed me and told me Dr. Selner was serious so I emailed him my number. He called later that day and when he heard that Jeff was on Penicillamine and how much the dosage was, he emphatically told me (and I do not quote) "if you don't want a vegetable for a son in a couple of weeks, get him off Penicillamine immediately!" I called Betty and told her to stop Jeff's medication and why. She didn't argue. He told me of the best research center in the country at the University of Michigan Medical Center run by Dr. George Brewer. Dr. Brewer, along with a few other doctors, is probably the foremost genetics research doctor on the planet for Wilson's disease. He gave me Dr.Brewer's number so I called Dr. Brewer's office immediately. His receptionist got some pertinent information and asked if I could fax the test results. I contacted Betty who works at the hospital where Jeff was seen, and she faxed me the results. I then faxed those to Dr. Brewer's office. He contacted me and told me to tell Jeff's neurologist to take Jeff off the Penicillamine immediately and why, and gave me his number if there was a problem. There was a problem! The neurologist didn't understand why I went through an internet doctor so I gave him Dr. Brewer's number. Dr. Brewer told me later that Jeff's neurologist called him and that they had "a nice chat!" He told me they were conducting a research study on a new medication for Wilson's disease to get FDA approval. It is an 8 week program where a standard double-blind study is done with one of two medications to test the efficacy of the experimental medication compared to an approved FDA medication. The FDA approved medication is Trientine which is as effective as Penicillamine but without the adverse side effects and brain damage. The new medication is Tetrathiomolybdate or TM. It appears to work as fast as or faster than Trientine, but seems to have less side effects. Dr. Brewer told me I should get Jeff here as soon as possible because in his case, every day we wait, he will deteriorate. I could see that happening before my eyes, and it was emotionally devastating to me. I called American Airlines and they gave us both a "compassion" fare which enabled multiple changes in return dates for both of us. I called my employer and told him I had an emergency with my son and had to get him to Michigan ASAP. He understood and told me to do what I had to do. We w re to leave Wednesday, February 13th. Since Jeff was in Clovis now with Betty, I would have to drive from my home 165 miles away on Tuesday and then from there to San Francisco. When my wife Linda got home that night I told her the news. She was very upset but at the same time worried about taking care of the house and property by herself. I told her I was only going to stay for about 1 1\2 weeks, then Betty was going to fly out for a week, then his mother would drive up from Kentucky and finally, Linda and I would fly out the last week to pick him up. Little did we know how all of these plans and our lives were about to change! This is where our journey begins...


January, 2002

Sent: Saturday, January 26, 2002 11:46 PM
>Subject: Jeff
>
>
>Well we got the results from Jeff's MRI and the Neurologist. The MRI as I said showed positive signs of MS. However, the Neurologist took a close look and said it was NOT MS. This seemed like a good thing until he told us what it was. He was Wilson's disease which is a defective gene that BOTH parents have to give to him. Only 1 in 30,000 people get this disease. It is NOT curable and fatal by age 30 if not treated in time. This is what he was diagnosed with several years ago and started treatment on it, but his new doctor (several years ago) said he didn't have it and took him off the medication. The Neurologist said that if you stop taking the medication, you can die in 3 months! What it is is that the liver does not eliminate copper in the system like it should, then it builds up in excessive doses in the organs such as the liver, prostate, eyes and brain. He is going in for a liver biopsy in a couple of weeks to determine the extent of the damage it has already done. He could have hepatitis or cirrhosis of the liver. He also has a bladder infection that won't go away so he is going to a Urologist to see if the prostate and kidneys have been damaged. He is going to an Ophthalmologist next week to confirm the disease. The speech slurring and the drooling and the muscular problems are all caused by the excessive copper in the brain. The Neurologist did a simple memory test and he couldn't remember the 3rd thing correctly so that is also a problem. The medication is supposed to reverse some or most of the speech/mental/muscular problems but not the organ problems. We will know more after the tests in the next couple of weeks. I hope this helps on the update of his condition. This is all we know at this point. The doctor says this started at birth but doesn't start manifesting until age 4. Then it really starts affecting the person at around age 20-25. Then if it is not diagnosed and treated by age 30, the person dies ALWAYS!!! At least we hope that this is the right diagnosis and he will start treatment ASAP. Sorry I'm rambling but that is just a Dirlam Kaden trait isn't it??? :-)
>
>I hope you are all doing well and Mike and Terri I hope you are enjoying your new house. Post some pictures on myfamily or something so we can see it.
>
>Pat

Sent: Sunday, January 27, 2002 9:05 PM
>Subject: (no subject)
>
>
>Hi. Sorry it took so long to send you some information on Jeff.
>
>Jeff saw the neurologist on Friday. He tested him in various ways, what is today's date, who is the president of the USA, who was the president before him, etc. He tested his memory by giving him three words to remember and then asked him to repeat them again after three minutes. He had Jeff walk for him in the hallway. He also checked his reflexes. He asked Jeff a number of questions regarding how he felt and what problems he was having.
>
>When he was finished he told Jeff he did not think he had MS or any similar condition. He thinks that everything is related to a surplus of copper in Jeff's system. He said that could account for the symptoms that Jeff was having including the problem with talking. He is referring Jeff to an ophthalmologist to check his eyes for the copper rings that Jeff was told he had about two years ago. He is also referring Jeff to a GI specialist for a confirmation of the surplus copper in Jeff's liver. The GI doctor may do a liver biopsy to confirm the diagnosis. Once Jeff has seen these two doctors and if they confirm the diagnosis of excess copper in Jeff's system, the doctor said there was some medication he could give to Jeff that should substantially improve his speech and other symptoms. Jeff already has an appointment with the ophthalmologist for 2/6 and we are waiting for the appointment with the GI doctor.
>
>Jeff went back home yesterday. I will be going back to pick him up this coming weekend so he will be back here for that appointment. Hopefully he will have an appointment with the GI doctor that same week. They are also doing more blood and urine tests to confirm the copper in his system.
>
>I think Jeff feels better to think they may figure out what is the problem and do something to make it better. At least someone is doing something to try to help.
>
>Hope all went well with your trip back to Bishop. I am sorry that I did not get to see you while you were here. I hope to be able to see you next time you are back in town. Please be sure to let me know when you are coming back so we can get together. I will keep you informed on what is going on with Jeff and the doctors.
>
>Say hello to Tom for me as well as the rest of the family. Write back when you can.
>Betty

Dear Pat:
> Thank you for letting me know about Jeff right away. I'm very concerned about this. I'm appalled that Jeff was misdiagnosed (again) several years back. You said he will be seeing an Ophthalmologist to confirm the disease. I don't understand the connection. How do they do a liver biopsy? Is it actually a biopsy or do they do blood work to check the liver condition? That's what they've done with Jenny a couple of times. Has the doctor started him on medication again, or is he waiting for the other things to get checked out first? If I remember correctly, he was told that his copper level was high some time back. When will he be seeing the Urologist? Please keep me up-to-date on all these things, Pat. I'm extremely worried about Jeff.
> I know you're not religious, but it sure wouldn't hurt for you to start saying some prayers. I believe God will hear you regardless.
> My heart goes out to you as well as Jeff. You and Jeff have always had a good and close relationship and I can only imagine what this is doing to you. Be strong, honey. Jeff needs that from you. I'm including you in my prayers, too.
> I love you,
> Mom

Sent: Monday, January 28, 2002 3:37 PM
>Subject: Re: Jeff
>
>
>He is seeing the Ophthalmologist to confirm the copper rings that should be present in his eyes for Wilson's disease. They cannot prescribe any specific medication for Wilson's disease until this test. The liver biopsy can be done in the doctor's office. They just draw out some fluid from it and test it. The urologist is only if his kidney problem doesn't clear up in the next week. He goes to the eye doctor next Wednesday and hopefully shortly after that he will start some type of medication. Linda told me that last night while sitting on the bed with her watching TV his hands were shaking badly from time to time and he constantly made some sort of noise and his breathing didn't sound real good. I cried after I went to bed just thinking of all of our old times when he was such a cute little bug and we did so much together. Now he can't even communicate with anyone. His knees are real bad too. It is hard for him to walk without it hurting his legs. I hope the doctor is right and the medication will help him regain most of his speech and muscle coordination. Thanks for all your prayers. I know your life is hard for you as well because of all your kid's problems but I just think it is so unfair for Jeff when every time he turns around and tells me life is so hard and I say it will only get better, it seems to take another turn for the worse!
>
>Love, Pat

February, 2002

Sent: Sunday, February 03, 2002 9:04 PM
>Subject: Jeff
>
>
>Just a quick update on Jeff. He is hopefully going to a consultation tomorrow to start his medication. The doctors have not decided that yet, but after observing Jeff this last week, I told Betty to try and get him in tomorrow. He is moving slower and slower and yesterday when we all went to lunch, he could not cut a burrito with a knife. I had to help him. Also, the plate almost fell off the table because he could not keep it in place as he tried to "saw" the burrito. It is very difficult for me to watch all of this happening. His Ophthalmologist appointment is still Thursday and that hopefully (not really a great thing) will confirm the disease. He should get the medication then for sure. He still can't get the GI for the liver biopsy any sooner than the 26th. The main concern right now is that he get on some medication so more damage won't be done. I really fear that it is becoming irreversible and the longer he has to wait, the worse it gets. I tried to talk to him the other night and he just kept putting his hand up to his head in response. I've been hugging him a lot lately!! Betty is going to call and let me know tomorrow night if Jeff is going to get the medication early. He will be in Fresno until next weekend when he returns. Then he will have to go back for the GI. I will keep you all posted and please put Jeff in your prayers. I really want him back the way he was 1 year ago. He will have to stay on the medication the rest of his life, but I want him to be able to walk and talk again, and at LEAST be able to laugh which he also can't do anymore.
>
>Love, Pat

Dear Pat:
> Be sure you or Betty let me know what happened at the ophthalmologist...whether it was confirmed or not. Also, about whether they are starting him on medication right away. I pray that the medicine will help him a great deal.
> This is breaking my heart, and I know it's much worse for you than me. I love Jeff very much, especially after taking care of him for so many years...he has a very special place in my heart (as do you). I pray for him (and you) every day, every time I think about him. At this point, I guess that's about all we can do. I know it's really tearing you up, honey, but just keep giving him lots of hugs and stay strong for him...he really needs that, I think.
> I'll be going back to Fresno on the 14th or 15th, I believe. Mercedes has called Joel again, saying she just can't deal with James. He's a good little boy...just a normal, active, talkative, noisy 3 year old (4 next month) and that's what gets to her. Anyway, we'll be going over there to pick him up again. I told Joel (and Mercedes) that I wasn't going to bounce back and forth over there every couple of weeks, and Joel has said if she sent him back again soon (which it is) that he was going to tell her he was keeping him for several months at least this time. We do need to get him, though, because I believe she's probably pretty rotten to him when he gets on her nerves, which is almost all the time. James says he loves her and he misses her after he's here for 4 or 5 weeks, but he also says "Mom is mean to me." I'm going to tell her that if she wants him back sooner than several months, she'll have to figure out how to come and get him or start paying for my trip.
> Guess that's about all for now, honey. I love you very much and pray that you remain strong through this very bad time. We must be optimistic and believe that Jeff will get much better with medication. I just wish I was around so we could all give each other a lot of hugs.
> Love, Mom

Sent: Wednesday, February 06, 2002 10:36 PM
>Subject: Re: Jeff
>
>
>Jeff is probably going to still be in Fresno that week (14th or 15th). He is finally going to see a real doctor, not a nurse practitioner and the soonest Betty could get him in is next Wednesday the 13th. She has been calling twice a day to try to get him into the GI for the liver biopsy before the 26th but so far no luck. She will call me tomorrow to let me know what the Ophthalmologist says and hopefully have some answer on his medication. Martha called last night and was pretty upset. She and Kelley are coming out in late March or early April to visit Jeff. She is auctioning off her parent's farm and will get between $30,000 and $100,000. Hopefully she will give some of that to Jeff (through me of course so he can't spend it all or get it taken away by creditors). I will email you tomorrow night with the results if I hear of any.
>
>Thanks for all your prayers and love. I know Jeff is special to you and you are very special to him!
>
>Love, Pat

Sent: Thursday, February 07, 2002 10:05 PM
>Subject: latest of Jeff
>
>
>Well today Jeff saw the Ophthalmologist and it was confirmed. He didn't even have to dilate Jeff's eyes to see the copper rings in them. He definitely has Wilson's disease. He is seeing the GI tomorrow and hopefully will get a liver biopsy to find out what damage has been done. He is going to a urologist for that problem and now has an ear infection and his skin has become scaly so he is on ear antibiotics, hydrocortisone for his skin, very heavy sleep medication for his sleepless nights, and starting tomorrow, Penicillamine which can cause more neurological worsening. This is needed to eliminate the copper from the brain first and then he may be able to go onto Zinc Sulfate which has less side effects. The bottom line is: they finally diagnosed him with Wilson's disease (which we knew he had anyway for the last 2 years); he is going to start medication which should arrest its damage, hopefully in the brain, but with similar side effects; his liver may be damaged, but we won't know until the biopsy; he may be disabled for quite a while as long as the disease has not permanently damaged his body; hopefully he will get better with treatment.
>
>Thank you all for your prayers and concern. I just hope that someday (and hopefully soon) Jeff will be back to his old outgoing happy self which I miss dearly!!!!
>
>Mike, I was advised that you should have Mike tested since the disease is hereditary and he may also be carrying the gene. If he is and his ex also is, then Cherokee has it. The doctor said all of Jeff's cousins should be tested. It is basically a test for excessive copper in the system and copper rings in the eyes. It may not be a bad idea for you to have your kids checked also Gayla. And Mom, you should have Joel's and Jenny's kids checked. Any of my brothers and sisters can have the gene and it just takes the other half to have it and the offspring will definitely have it!!
>
>I will keep you all posted as things progress.....
>
>
>Love, Pat

Sent: Saturday, February 09, 2002 12:32 AM
>Subject: update
>
>
>Well, Jeff's appointment for the GI got cancelled today. He is now rescheduled for next Wednesday for a regular doctor and the head of the GI department since they have determined that his condition is worse than they thought. I was contacted by email today by the founder of the Wilson's Disease National Organization (he is a doctor and specialist). He gave me 2 numbers and 2 fax numbers, his email and 2 home phone numbers to call him ASAP. He wants to discuss the medication that the doctors put Jeff on. It seems that what I read is true...he can get much worse using this specific medication and a lot of non-specialist doctors normally prescribe it. He recommended a specialist in California who will gladly (and from what I understand) consult with and recommend treatment with the doctors in charge of Jeff. I will try to contact all of them next week. He will probably be in Fresno for another couple of weeks while the doctors monitor the medication. I will of course keep you all posted.....
>
>Love, Pat

Sent: Sunday, February 10, 2002 1:22 AM
>Subject: latest on Jeff
>
>
>I got a call today from the Founder of the Wilson's Disease Foundation of the US and he told me to tell Jeff to stop his medication immediately. He said that 500mg 3 times a day was waaaaaay too much to start and it could have serious irreversible effects. He told me that I am supposed to call a Dr. Brewer in Michigan first thing Monday morning. Dr. Brewer is the foremost authority in the world on Wilson's disease and also a geneticist. He is the head of the Genetics research foundation at the University hospital there. They want Jeff to go to Michigan within the next week to start treatment ASAP. Dr. Sellner told me Jeff is too far gone in his symptoms to mess around with amateur doctors who know nothing of the disease other than what they read in their medical school manuals. Betty said that only 1 doctor had ever seen a Wilson's disease patient in his entire career. I talked to a lady tonight with the disease and she said her doctor started her on a high dosage of the Penicillamine and within weeks she could no longer feed herself and could barely move. She went to Michigan and was there for 3 years! I hope they don't mean Jeff would be there even close to that, but they give him the latest treatment, monitor him EVERY DAY (the HMO doctors monitor only once every couple of weeks to a month if that much), and even do speech therapy and whatever else is needed all for free!!! They don't let him go until he has made a pretty good recovery from the neurological problems he has. Every one I have talked to in the group who has had experience with the program says it was the best thing that could have happened to them or their children. I have talked to many people in the support group who have kids the same age as Jeff with the disease. One lady has 2 boys who were diagnosed at 25 and 27 with it. They were pretty messed up but went through the program and now lead fairly normal lives. One still has tremors in his hands and arms so he can no longer do his architectural drafting. He helps out in the family business designing web pages. The other one is married with a 4 year old daughter. Many of them are on some form of disability. If Jeff does go to Michigan, I will probably not be able to go with him unless they pay for it but Jeff's mom said she could drive up there and be with him for the first couple of days. Whatever it takes to make him well again..........I'll keep you all posted as I find out more Monday......
>
>Thanks for all your prayers and love......
>
>Patrick

Monday, Feb. 11, 2002

I called Dr. Brewer in Michigan today and he asked me about Jeff's condition. I described what was going on and he asked me when I could get Jeff to Ann Arbor. I asked him "how soon should he get there?" and Dr. Brewer said "tomorrow!" That really scared me. He told me Jeff's condition will deteriorate rapidly and he needs to get on the de-coppering program as soon as possible. As soon as I hung up the phone I called American Airlines and made 2 reservations for us. They cost $550 each because they were made so quickly but the lady gave us what is called a compassion fare. That way either of us can come back whenever we want and the return date can be changed as many times as is necessary. Jeff's return date is set to April 14th and mine is set for February 21st. I had to call Barry at Circuit City and tell him I would be gone for 2 weeks. He understood and said that would be ok. I guess I will be packing all of our stuff up and calling Betty to let Jeff know we will be leaving tomorrow. Our plane leaves Wednesday so I have to pick Jeff up from Betty's in Clovis tomorrow and head up to San Francisco. Uncle Bud said we could stay at his house overnight and I could leave my car there until I get back in a couple of weeks. Betty said she could go with us which will be nice. Jeff and I are both scared about this whole thing!

Tuesday, Feb. 12, 2002

I left today for Fresno to pick up Jeff. Betty took her car and followed us up to Pacifica. We got in to Uncle Bud's around 7pm and decided to go to dinner. Well, it is Pacifica so nothing except Denny's was open. During dinner Jeff freaked Betty and I out because he was chewing and all of a sudden just stopped. We asked him what was wrong but he couldn't answer. He just sat there frozen. I really got upset and kept asking him what was wrong. Finally I asked him if something was stuck in his mouth and he shook his head "yes." I told him to open his mouth and spit it out. It turns out there was a piece of bread stuck in his mouth and since it was soft he couldn't feel it with his tongue and just locked up. It was very scary. If this is what is going to happen from now on, Jeff and I are both going to freak out during this ordeal. We went to bed around 10 pm because tomorrow is going to be a really sad day for Betty and a very long day for me and Jeff. Betty has to say goodbye to Jeff for 2 months although she is going to visit in early March.

Wednesday, February 13, 2002

We got up early this morning, took showers and left for the airport in Betty's car. Mine will stay at Uncle Bud's for a couple of weeks. We ate breakfast at the airport. Jeff and I had omelets. Jeff had a little problem again eating his food. I am really getting worried about this. At least we will be checked in to the University of Michigan medical center tomorrow and Jeff will be in the best hands in the world for this disease. The plane left around noon. Jeff got searched and I got mad. He had to take off his boots and he could barely do it. I told them he had a rare genetic disease and he couldn't move very well. They said it was just a random check and he got chosen. We gave Betty a big hug and she cried a lot as Jeff and I disappeared out of site. We left for St. Louis on a very long 5 hour flight. We saw the snow-covered Sierra Nevada mountains and I told Jeff he would not see them again and home for 2 months. I could tell he was scared as was I. I took several pictures of Jeff looking out the window as California slowly faded away. We got into St. Louis at dusk so we couldn't see much. Our plane to Detroit was delayed so we got into Detroit late. As we walked up to the terminal from the gate Jeff just suddenly stopped. He froze standing up and tears started coming down from his eyes. I panicked. I kept asking him what was wrong and he couldn't say anything. He finally started moving slowly and I got him into a chair. I couldn't find the cab driver who was supposed to meet us anywhere. Some guy came up to help us and I could tell he wanted money. I called the cab company and they said the driver was there and left. He was on his way back though. The guy helped us with our luggage and I gave him five dollars. We were in a strange place thousands of miles from our home in California and Jeff was having serious problems. I was paranoid. Finally the cab driver showed up and got us and our suitcases in the cab. As soon as we walked outside the freezing Detroit wind hit us and our pant legs froze instantly to our legs. There was snow on the ground. Jeff seemed to enjoy this but I didn't. I was very scared for him but tried not to show it. We got to our motel room around 11 pm and checked in. We got a handicapped room. I guess they thought Jeff was in a wheelchair. We ordered pizza and watched TV until around 1 am. We didn't have to be at the hospital before 2 pm tomorrow so we weren't worried about the time. I took some pictures of Jeff and we went to bed.

Thursday, February 14, 2002

Today is the big day. Not only is it Valentine's day and here we are thousands of miles from home, it is also the first day of Jeff's two month stay at the hospital. We got up around 11am and had the continental breakfast. We called a taxi to take us to the hospital. I had reservations for 1 ½ weeks at the hotel and would be visiting Jeff every day at the hospital. When we arrived at the hospital we could not believe how huge it was. I have never seen such a sight. It looked like a small city! The taxi driver told us it had 35,000 employees and just under 1000 rooms. Wow!! It sits on the University of Michigan Campus which covers 10 square miles and has over 100,000 students. Each school has its own campus so students don't have to zip all over the place to fill their general requirements for a major. Ann Arbor is really beautiful with snow everywhere. The driver said it is very beautiful in the spring when all the trees blossom. The hospital sits behind the Huron river and I'm told generates its own electricity. It is like a small city!

We went up to the seventh floor B and walked up to the nurse's station. We met a very nice lady named Denise. She stuttered pretty badly and walked really fast. I wondered if she had Wilson's disease. She showed us to Jeff's room and we started unpacking Jeff's suitcases. The view from Jeff's room was stunning. He had the corner suit! You could see much of the University campus from his window and also the Huron river. Everything was covered with snow. A while later we met Daisy. She is a physician's assistant and personal assistant to Dr. Brewer. She told us we would meet Dr. Brewer tomorrow. He comes in every Monday and Friday to see the patients. We found out there were three other Wilson's patients there as well. One was next door. His name is Jerry. He is 27 and from Connecticut I think. Another one is Alysia who is 14 I think and from Washington. The third is a little girl named Marita. She is 8 or 9 and from Washington also. I noticed that the parents of all these kids are staying with them in their rooms. Daisy said I could stay with Jeff if I wanted to but I said I had reservations for the next 2 weeks at a hotel. The nurse came in and weighed Jeff and started his first 24 hour urine collection. He would have to do this twice a week. He had to collect every bit of urine for a 24 hour period and put it in an acid-washed container. Then it went to the lab to determine how much copper was in it. This would tell Dr. Brewer Jeff's progress week by week. We had to fill out tons of paperwork for the FDA study Jeff would be on. Basically there were two medications in the study. One is Trientine which is FDA approved for Wilson's. The other is Tetrathiomolybdate which is pending FDA approval after this study is completed. It is called a "double-blind" study because nobody knows (except Dr. Brewer) which medication Jeff will be on. The capsules are double encased so the nurses don't know which one is which. It is set up so each patient gets a certain number of doses each day and some are placebos so nobody knows who gets what. Jeff will find out which medication he was on during his first year follow up. He has to go back to Ann Arbor for 3 years for around 4 days each time for a follow up study to see how he is doing. I stayed with Jeff until around 9pm when I had to leave before they locked the doors. I kissed him goodnight and said I would see him early in the morning and then I left. I was not real comfortable leaving him there alone on his first night but the nurses assured me they would take good care of him. Jeff told me he would be ok and started watching television. They had lots of channels, especially the cartoon network which Jeff loves so he felt right at home. I got back to the hotel and watched television for a couple of hours and then went to bed.

Friday, February 15, 2002

I got to the hospital around 10am this morning. Jeff seemed to be doing all right. When he had to pee, he got a urinal out of his little refrigerator built into the wall and took it into the bathroom. When he finished, he put it back into the refrigerator. He told me that is how they do it. The nurses woke him up at 7am to give him his medications and drew blood. He said his breakfast was pretty bad. What do you expect from hospital food? We met Dr. Brewer today and he seemed pretty nice but kind of aloof. He went over the next 8 weeks strategy and explained how Jeff would get a speech pathology and neurology examination twice a week to check on his progress. Jeff seemed ok with all of this. Jeff's mother Martha showed up this afternoon with her friend Valerie. They are also staying at the Microtel where I am staying. I am not totally thrilled that she is here. She hasn't been that involved in Jeff's life until now when he is sick. Still, I guess it is nice for him to see her. The plan is that I stay until February 21st which is next Thursday, then Betty will be there the next Monday and stay for a week. Then Martha will come back and stay with Jeff for at least a month. Then I will come back one week before he leaves and stay with him for the trip home. I hope Martha will be back when she says. I don't want Jeff to be left alone for even a day much less a couple of weeks. I can never count on her to do what she says. She is selling her parent's farm and she said when everything is finalized, she will be back. If something goes wrong, however, she may NOT be back and Jeff may be alone for several weeks. This really is tearing me up because I do not want him left alone even though these people really will take good care of him. He just needs the support of a family member and I can't take off the entire 2 months from work. We can't handle that financially. Martha left around 7pm and I said "goodnight" to Jeff around 9pm and went back to the hotel in a taxi.

Sent: Saturday, February 16, 2002 4:13 PM
>Subject: Re: Jeff
>
>
> > he is here now in Ann Arbor with me. It is really tough. Last night he
>went
> > to the bathroom and just stood there for over an hour paralyzed. The nurse
> > finally found him. I can't imagine the terror going through his mind as he
> > stood there not being able to move or speak or even push the emergency
> > button. they are all here today monitoring him again so hopefully all will
> > be ok. will keep you posted. Dr. Hedera came by with Daisy and discussed the possibility of putting a feeding tube in Jeff's stomach if he has any more freezing episodes. Daisy said it would be extremely dangerous for Jeff to have a freezing episode with food in his mouth because of the danger of aspiration (inhaling food) and subsequent pneumonia. I was NOT pleased on hearing this. That is the last thing that I would want for Jeff. He is already going through enough. A tube surgically put into his stomach just sounds really bad. Daisy said the speech pathologist would determine if Jeff needed the feeding tube. If he does, that would eliminate normal eating. He would have to have some type of liquid formula like the little girl down the hall. Today Martha and her friend Valerie came to visit. We decided to go to the local Meijer (similar to Wal-Mart) to get some clothes and a shaver for Jeff. It was very cold outside and there was still some snow on the ground. Jeff had a rough time at the store. He couldn't walk very fast so I just took my time with him. We bought him some shoes, a shaver, lots of clothes and some food and goodies for us. When we got back to the hospital room I noticed that my brand new digital camera was gone. I asked at the desk and they checked to see if anyone had removed it for safety. Nobody knew anything about it. It was gone! I wasn't so upset that it cost me almost $2,000. I was very upset because it had a lot of pictures of Jeff on the plane and in the hotel room and the first pictures of his room. The research center administrator, Dr. Wiley, came by to talk to me. He told me the hospital would replace the camera if I filed a theft report. I told him my insurance should cover it minus a $500 deductible. Dr. Wiley said the hospital would then cover the deductible. They figured it was an inside job and probably one of the cleaning people. I told them how I had hidden it behind a chair with a foot stool in front of that so the only way someone would be able to see it is if they were cleaning the room and moved everything. The security officer came in and took down the information. I can't believe someone could be that cold and uncaring to steal a camera out of a sick patient's room! I am really upset however because of the pictures that were lost. These were going to be a part of this journal for Jeff's journey. I only have memories now of the way he looked on the plane looking out the window over the great plains. He looked scared and uncertain of his future especially since he would be in a hospital for the next 2 months. I feel very sad about the loss of these pictures and I just hope that Jeff doesn't feel in any way responsible for the loss of the camera since we are here because of his illness. I told him absolutely NOT to worry about that. The camera is replaceable but the pictures are not. After a quiet evening, Martha and Valerie left and I left around 9pm again after giving Jeff a big hug and kiss goodnight. I told him not to stay up until 2am watching television this time.
>
> > Love, Pat

Sunday, February 17, 2002

Sunday is an easy day for the hospital patients. All of the doctors are gone so everything is laid back. Jeff and I decided to go to the movies. We saw Super Troopers. What a DUMB movie! Of course, Jeff liked it though. Since we don't have a car, everything is done by taxi. I am learning fast about taxi's in this area. They are expensive! It cost us $12 to get to the theater and $12 to get back to the hospital. I had to take Jeff's pills with us so he would stay on schedule. It took him a while to get to the room with the movie and he seemed to have problems swallowing his pills. I am getting worried about this because if he can't swallow anymore he is going to have to have the stomach tube inserted and I just don't want that to happen, especially since I am leaving next week. Martha decided to spend the night with Jeff tonight since she is going back to Kentucky tomorrow. At dinner Jeff had a really rough time swallowing all of his pills. He has so many I can see why, but they just sit there in his mouth like he doesn't know what to do with them. He does eventually swallow them though and he has no problems with eating solid food. It is strange that he has a problem with just the pills. I said my goodbyes around 9 and Valerie took me back to the hotel. I am really getting worried about Jeff's swallowing. I know that Daisy is really going to push for that stomach tube tomorrow if Jeff doesn't do too well with his speech therapy.


Monday, February 18, 2002

Martha left this morning. She came by and said her goodbyes and told me she paid for the rest of my stay in the hotel. That was very nice of her. She said she would be back as soon as the family farm sold in about a month. Jeff saw Karen, the speech pathologist today. He froze while drinking water. It just sat there in his mouth and he couldn't swallow. I think that she overloaded his brain with all the things she tried to get him to do. Karen recommended that Jeff have a peg tube placed in his stomach and that it was not a bad thing. If Jeff has any more bad freezing episodes and ends up inhaling food or liquids, he could get pneumonia which is very BAD for a Wilson's patient. She said she was going to recommend this to the doctors and Daisy. I do NOT want Jeff to have a tube placed in his stomach. That would be the ultimate bummer for him since he wouldn't be able to eat by mouth anymore and he loves food too much for that. If he does get the feeding tube put in I would have to come back a week early to learn how to make his formula and give it to him. The little girl down the hall named Marita just had a feeding tube put in her stomach and for the last couple of nights we have heard her screaming in pain. The nurses said it takes a while for the stomach to get accustomed to food going directly into it and that is probably why she is in so much pain. I can see the worry in Jeff's eyes. It seems that things are starting to progress in the wrong direction. Maybe this disease is much worse than we thought. Jeff is not talking very much lately. He is stating to shake his head a lot for yes and no questions. Daisy told him that he must answer her by speaking and she wouldn't accept head movements. Jeff and I both looked at each other like "Man, what a pain she is!" I like her though and know that she knows what she is talking about. My problem is that if I feel Jeff is having a hard time doing something I will let him have the easy way out because I don't want him to hurt. Daisy says that I cannot fall into that trap. If Jeff is ever to recover, I must make sure that he keeps walking and talking all the time. I know she is right but it is so hard for me to make Jeff do something that hurts him. This is going to be a challenge. I hugged Jeff and cried as I said goodbye. I apologized for being so weak when he was so strong. It is going to be very hard leaving him here for 6 weeks this Thursday. I went back to the hotel around 10pm and cried for hours.

Sent: Tuesday, February 19, 2002 1:08 AM
>Subject: Jeff
>
>
> >Jeff had a fairly mellow day today. He had a catheter run into his bladder
> > (luckily it was empty so he may not have to have it again for a
>while...this
> > is to insure there is no residual after he goes and possible
> > infection...they are very thorough). His night nurse tonight said that she
> > couldn't understand why all these Wilson's patients needed so much
>attention
> > (she is an older biddy) and when it was time to take his pill, she just
> > stared at him and didn't offer any suggestions in a sweet tone as the
>other
> > ones do). I will probably mention her to the doctors tomorrow. I haven't
> > seen her in 2 hours and the other night shift nurses check on Jeff every
> > hour. That is just another thing to worry about. Today he cried which
>broke
> > my heart. He just stands facing the mirror and stares at himself. I can
>only
> > faintly imagine what he is going through. I cannot add him to my insurance
> > and his HMO hospital is 165 miles away which is NOT an option for Jeff. I will
> > probably have to get him on Medical if possible. The medication costs
>$3/day
> > without insurance! He may have to have a feeding tube inserted in his
> > stomach for a year if he cannot chew food within the next week. He has
>been
> > on soft food for the last couple of days because of the freezing episodes.
> > He is taking a medication that helps for that so I hope it will help him
> > since I won't be here after Thursday. I hope I can make it back home and
> > even function after that. We are going to try and catch a movie tomorrow
>if
> > he doesn't have his liver biopsy. Hopefully that will be Wednesday. That
> > will determine how much liver damage he has. Again, hopefully it will not
>be
> > horribly severe but the doctors warned me that it could. Let's all keep
> > praying for him and me cause I sure do need the support!!!!! :-(
> >
> > Love, Pat

Subject: Jeff
>Wednesday, February 20, 2002
>
> > Well I'm going home tomorrow. It is going to be one of the toughest things
>I
> > will ever have to do!! Jeff had a really bad day today. He had his liver
> > biopsy done at 8:30 this morning before I got here and the lab techs neglected to give him any pain killers after the
> > procedure. I got here at 10am and was told I would have to wait to see him until he was released from downstairs. He had to wait almost 3 hours to get any pain medication because they could not
> > give it to him until he was released back to his doctor! When he got here
>he
> > was shaking and looked horrible. It took almost 1 hour for the medication
>to
> > really take effect and he was grimacing the whole time. I couldn't help it
> > and no matter how hard I tried, I just started crying. He grabbed my hand
> > and squeezed it hard and it made me feel even worse. I was trying to be
> > strong and here he was in pain trying to help me. He just stares into my
> > eyes and tries to let me know it is ok as if I am the one in pain. Well, I
> > guess I am and he knows it and that is what keeps tearing me apart.
> > He got better as the day progressed. We are just watching TV
>tonight
> > and taking it easy. I am leaving in the morning at 11am but it won't be easy after what happened today. How can I leave my child alone in a strange place with an incurable disease? I don't know what else to do. I will be back in San
> > Francisco a little after 5pm. I will stay at Uncle Bud's and then leave
>for
> > home Friday morning. I am not going back to work until Monday. Linda and I
> > had a scheduled train trip to Santa Barbara (2 for 1 Feb. special) for
> > Saturday and I think I should still go on it. I need a break from all this
> > sadness.
> >
> > Daisy said she is probably going to have to put a feeding tube
>into
> > Jeff's stomach which he will have to have for a year to keep him from
> > choking when he is eating and his body freezes. He could get food or
>liquid
> > in his lungs and that could be very dangerous so they don't want to take a
> > chance. She will let me know in the next week if he will require it.
> >
> > Linda and I are going to be back on Thursday, April 11th because if he
>does
> > get the food tube, we will have to learn how to prepare his food and
>inject
> > it into him. He can still eat regularly but if he needs it, he has the
>food
> > tube. We will be coming home with Jeff on April 14th, Sunday and leave
>Uncle
> > Bud's on Monday the 15th and be back home. I will probably take that next
> > week off to adjust to Jeff's new routine of medication and feeding.
> >
> > It is going to be really tough financially, but his doctor said he will
>need
> > constant care for at least a year. At the end of the year, he has to come
> > back for 3 to 5 days for an evaluation. I hope he will be feeling much
> > better then. He told me yesterday he feels worse than when he got here.
>The
> > doctor said he will probably still get worse before he starts to get
>better.
> > One of the mother's here had to come back because her 27 year-old son got
> > worse. He can no longer feed himself and he freezes really bad and coughs
> > horribly sometimes for 5-10 minutes. An 8 year-old girl here can no longer
> > get out of bed and has to be fed through a tube. So I guess Jeff is lucky
>so
> > far as long as he doesn't get any worse. Let's all pray for that!
> >
> > It would be great if you all could send Jeff some cards or small gifts.
>The
> > other kids have stuff all over there rooms so they don't look so much like
>a
> > hospital room. He has gotten gifts so far from me, his mother, Danny and
> > Betty. I got him some flowers and a plant and a card. It would be nice to
> > fill up his large window sill with a lot of cards!!! If you haven't
>already
> > got it, his address is:
> >
> > Jeff Dirlam
> > University Hospital
> > Clinical Research Center
> > 7A Room 7402
> > Ann Arbor, MI 48109-0108
> >
> > He doesn't do well on phone calls so I won't give that out at this time.
>I
> > hope you are all doing well. It is hard to focus on anyone else right now
> > but Jeff and I do care how all of you are doing and love you all very
>much.
> > Thanks for all of your wonderful support!!!!
> >
> > Love, Pat and Jeff
I called Linda tonight and told her I just couldn't leave Jeff right now. He was in so much pain this morning and scared and there is no way in hell I can leave him in this condition. She was understandably upset but I started crying and told her he was in bad shape and he would have nobody for support for the next couple of weeks and I just couldn't do that to him. I went back to Jeff's room and told him of my decision that I was going to stay another couple of weeks. He immediately smiled and looked like a new person. I am so glad that I changed my mind. I gave him the biggest hug ever and told him that he knew I couldn't leave him like this. He smiled again and gave me a hug. We spent the rest of the night before I left watching television and just being happy. I don't know how I am supposed to pay for another week at the hotel but I'll just have to deal with it. Some of the nurses said that I could possibly stay in Jeff's room like some of the other patient's mothers do. I will ask Daisy tomorrow. I left around 10 pm again but feeling much happier than when I got there this morning.

Sent: Thursday, February 21, 2002 2:03 PM
>Subject: Jeff
>
>
>I think everyone knows already of my decision to stay with Jeff until
>March 7th. If not, you do now. I talked to the nurses and doctors
>yesterday and they all said Jeff would be ok. I talked to the mothers
>and they said if there was any way possible to stay, then do so for
>the sake of your kid's progress. Jeff was doing really bad yesterday
>and this will happen extensively throughout the 1st 2 months of
>treatment. Not quite as bad after that in the next 6 months, then he
>will start healing and feeling better within the first year. One
>mother told me her son told her it was ok for her to go and now he
>cannot speak, hardly walk and coughs constantly. She flew back
>immediately to be with him and hasn't left his side since. This is
>common I've been told in Wilson's disease if no one is there for
>support. I asked Jeff last night if he felt better or worse than when
>he got here and he motioned "worse." I asked him if he was scared and
>he nodded "yes." I told him not to worry and answer me honestly if he
>wanted me to stay or not and he said "yes" so here I am. How could I
>leave him alone in that condition!!! After I made my decision the
>nurses and doctors all said I made a very wise decision as it would
>be very bad for him to be here in the initial stages alone. Why
>couldn't they just tell me that in the beginning and avoid all the
>anguish between us both???
>
>I asked Jeff today (he is doing much better knowing that I am not
>leaving) exactly how this whole thing worked in his brain. He told me
>that he knows exactly what he wants to say but his brain just doesn't
>send the signal to his mouth so he can't talk. When he freezes,
>usually drinking water, he knows how to swallow and tries to think to
>swallow but his mouth and throat just don't respond. when any
>neurological symptom happens, the copper in his brain prevents the
>nerves from sending the signal to the appropriate part of his body.
>Very interesting but scary indeed if it were your body. It gets much
>worse at night and when he is exhausted from tests or a long day. His
>brain basically shuts down at that point and he can only just lie in
>his bed and his face muscles twist all up and his body goes into
>tremors where he shakes violently. This is the copper in the muscles,
>totally separate from the copper in the brain symptoms.
>
>This is also very hard on Linda because she is alone in the country
>and has to take care of all the house things and still work and not
>be here for Jeff. She is really stressed about it all and I don't
>blame her. She feels bad about not supporting Jeff by being with him
>but I told her she is supporting us by taking care of the house. If
>you guys would like to drop her an email now and then to help and
>console her, please do so. For those of you who don't know it, it is:
>lindadirlam@hotmail.com

I moved into the front part of Jeff's room today. Daisy said it would be fine. I checked out of the hotel and brought everything here. Jeff is very happy that I am here and SO AM I!
>
>Well, anyway, thanks again to all of you for your support and prayers
>and I am so glad that I will be able to stay with Jeff another couple
>of weeks. I hope his mother shows up on March 12th or he will be
>alone when Betty leaves from then until we get there in April. That I
>don't want to happen. I will keep you all posted. Also, if you can
>give me Gayla's new email, I forgot it. If you would like to forward
>this message to her (one of you) I would appreciate it.
>
>Love, Pat and Jeff

Sent: Sunday, February 24, 2002 5:23 PM
>Subject: Jeff
>
>
>Well Jeff has gotten worse again. He hasn't been able to talk in 2
>days and now he freezes all the time. If he stands up and tries to
>drink anything, his whole body freezes and I have to forcefully pull
>his arms down so he can get the straw out of his mouth. I have to
>push his legs to make him go. If he keeps walking, he is ok but he
>walks very slowly. It took him 2 pills and about 20 minutes to
>swallow his medicine tonight. If this continues, they will put a food
>tube in him. I hope it doesn't come to that. I still don't understand
>why this happens the way it does. Through simple yes and no nods I
>have gotten out of him that he tells his brain what to do and expects
>it to happen just like we do, but the body part doesn't respond. He
>tries so hard sometimes his whole body shakes and almost convulses,
>yet nothing happens. It is very frustrating for him and you can see
>it in his face. I think he is a lot braver than I! He will definitely
>need full time care at home and Linda will not be able to do it so
>that leaves me to stay home. I am going to have to really work on our
>finances but there is NO choice in the matter. I love him dearly and
>want to make sure he gets the best care. When he freezes, he would
>stand there for hours if no one was there to help him. I have seen a
>few of the nurses here do nothing except tell him to move when he
>can't. I'm sure a caretaker at home would be the same some of the
>time. I can't imagine being frozen and unable to move or tell anyone
>for more than a few minutes much less an hour or more. It is really
>scary to see it!
>
>Well for the good news, we did go see a movie today however I think
>that is part of the problem. I don't want to keep him cooped up but
>it always seems to be detrimental to him. The WD patient next door
>freezes all the time but refuses to stay put so he walks for hours.
>He got Jeff to go with him yesterday and they walked for hours. I
>think this is what started Jeff downhill. The doctors want him to
>walk and talk but when he strains his body like that (everyday tasks
>for us) it seems that the copper gets loosened up or something and
>effects some other part of his body. His legs and arms were very
>stiff today, almost locked in position. He does keep a good outlook
>on things considering his handicaps. He is always smiling when he can
>and pats me on the back and hugs me a lot. I wish it were me instead
>of him!!! Uncle Bud and Aunt Mar, if you feel like it, maybe you can
>put a little more time on the phone card for me. It is getting down
>to around 300 minutes. I try not to use it much but I talk to Linda a
>long time because she is so down right now from being there all alone
>and her job is really getting bad. I didn't know until today that she
>didn't have the number to Jeff's room! Gayla, happy birthday coming
>up. Mike and Terri I hope your new house is going great and no more
>ice for a couple of years. It is supposed to snow again by the middle
>of next week and down to low 20's or less...COLLLLLLLLD!! Mom, as
>always I know your thoughts are always with Jeff and I and I
>appreciate that. Betty, your support and generosity has always been
>appreciated more than you may have imagined.
>
>Thank you all for your continued support in our most trying time!!!
>
>Love, Pat and Jeff

Sent: Monday, February 25, 2002 6:07 PM
>Subject: Jeff
>
>
>Some of you already got an update earlier today but I might have
>forgot something so here is the update for all...
>
>Well Jeff's night was indeed a nightmare. He just got worse and worse
>and finally I called the nurse. It appears that he has something
>wrong in his privates and it is NOT an infection. He was asked how
>bad it was from 1 to 10 and he said 10. He cannot talk anymore or
>move his head so he blinked his eyes to let us know. It was very hard
>for me to deal with. He has been in immense pain but couldn't tell
>anyone. He had x-rays today and will see the urologist and neurologist
>tomorrow. Hopefully that will help. He is on pain medication right
>now. He can no longer feed himself so I am doing that for him. It is
>really sad to see him deteriorate but the doctors warned me this
>would happen before he gets any better. The doctor today said a good
>1 1/2 to 2 years before he can be quasi-normal again. Hopefully with
>a lot of rest and fixing the pain problem, he will be able to talk
>again and move and feed himself. I think that the pain is totally
>short circuiting his already overloaded brain. The results of the
>liver biopsy showed that he does have Cirrhosis but it is not as bad
>as it could be and it will partially repair itself in the next year.
>Please keep any cards and letters and gifts coming in as it does
>cheer Jeff up a tad! Thanks for the wonderful card, book and gift
>Danny. Jeff has started reading the book and hangs the ornament?
>right over his head on the bed!
>
>Well, we are going to get up to 10" of snow tonight and down to about
>8 degrees I think they said. we will have to bundle up!!!
>
>Love, Pat and Jeff

Sent: Tuesday, February 26, 2002 5:40 PM
>Subject: update
>
>
>Now I don't know if you got my last email or not. I hate my email. it
>was long and not to uplifting as usual lately but it was important so
>I'll try to paraphrase it here. If you guys got it, just ignore this
>one. I just wrote it a few minutes ago so if it is dated yesterday,
>that is not it!
>
>Jeff saw the urologist and the neurologist today and they found
>nothing physical wrong with his privates, i.e. x-rays, cultures, etc.
>The neurologist thinks it is a rogue nerve or muscle contraction
>caused by the copper leaving his body. His doctor ordered the nurses
>NOT to give him any more codeine because she doesn't want him to get
>addicted and for it to effect the de-coppering process. In the
>meantime, Jeff faces extremely bad pain with no way to alleviate it.
>It is quite painful for me to look at his face which is twisted into
>sort of a smile knowing that he is in tremendous pain and can do
>nothing about it. He can no longer feed himself, talk or even move
>his head much. He blinks his eyes for simple yes and no questions.
>He froze many times today eating solid food and I'm afraid they'll put
>a food tube in him if he can't eat. The little girl down the hall
>screams every time they feed her that way. I guess it is a problem
>with air or something entering in the stomach. We managed to get
>outside today for a brief few minutes and threw snowballs at each
>other (well he sort of tossed one at me). It took a long time to get
>him back to his room as he moves very slowly. I think I forgot to
>mention in yesterdays letter that he does have cirrhosis of the liver
>but it is not life threatening at this point. the de-coppering drugs
>are getting it out of the liver quickly (which could account for some
>of the problems he's having) and it should heal in the next 2 years
>somewhat.
>
>Now the problem I face is simple. I am supposed to go back next
>Thursday and I don't think I can do it. The mothers all tell me I
>shouldn't. I know Betty will be here on the 6th of march and then his
>mother will be here on the 12th. Betty will take very good care of
>Jeff but I don't think his mother is capable of it. Also, the nurses
>no longer have time to take complete care of the patients. Things
>such as bathing, brushing the teeth, etc., all have to be done by a
>parent. I know Jeff would not want Betty or his mother to bathe him
>because of his shyness. The nurses only have 2-4 patients each but
>some of them are requiring more care all the time. One nurse has to
>draw blood every 10 minutes from a patient. The guy next to Jeff can
>no longer shower and sucks on a pacifier all day. It is pretty scary.
>Jeff is in so much pain I think that in itself is shorting out some
>of the other brain functions like eating and walking. The doctors
>don't really agree but I know if I am in excruciating pain, I don't
>function very well either. I really need to get at least one more
>month of work in and have to get Jeff's disability payments set up
>along with his medi-cal and some sort of compensation for me for
>taking care of him through the state. I will definitely not be
>working when he comes home. He will definitely need round the clock
>care. When I look into his eyes and that twisted "smiling" face I am
>pretty sure he is terrified at the thought that I am going to leave
>him. I don't think I could bear doing that to him or myself. This
>whole thing is tearing Linda up inside as well because her job is
>going very badly and she has no friends there for support. It is not
>easy taking care of a house out in the country either. I need some
>advice. I am going broke on hospital food but we in theory could
>survive if I stayed here the full time. Linda sends me the bills and
>I pay them so we are ok there for about another month(and thanks so
>much to all who have contributed, we greatly appreciate that). I was
>just hoping I could work another month for some extra cash before I
>quit but the mothers here say if you leave your son will get worse
>and you'll have to come back early anyway. I can see that in the boy
>next door who is now on the pacifier. They are not just saying this.
>The doctors agree that a parent should be there with him at all
>times. His mother has really never been his parent and he is not
>close to her. I have faith in Betty doing everything right for Jeff
>and comforting him but she can only be here for a week.
>
>Give me some options, family. I know what I need to do but I know
>what my heart tells me I must do. I just feel worried about Linda as
>well being there that long by herself without any emotional support.
>
>Love, Pat and Jeff AND Linda

Sent: Thursday, February 28, 2002 1:46 PM
>Subject: questions
>
>
>I went to wilsonsdisease.org I think and found a lot of information
>and joined a support group. The doctor hasn't said what the copper
>levels are yet. It is kept secret from the other doctors because they
>would know what study drug he is on. I should know by Friday. They
>put him back on codeine and are going to run a whole series of nerve
>tests on him. They also have a nerve center here just for mysterious
>things like that. They want him to feel good while he is getting
>de-coppered. The liver does have damage but it can be reversed to a
>point. The whole body can heal as the copper leaves it. The brain
>will still have some damage but not to the extent it does now. A guy
>came in last night who was in here 4 years ago with similar but not
>as bad as Jeff's symptoms. He can now walk and talk fine. He said it
>took 3 years to recover to the point he is today. They were going to
>put him into an asylum because they thought he was mentally deranged
>from the tremors. Pretty scary stuff. He talked to Jeff and gave him
>(and me) hope!!!!
>
>Thanks for all your support. I know Jeff would love to be able to see
>you as he loves you dearly and so do I!!!! :-)
>
>Pat and Jeff

Sent: Thursday, February 28, 2002 6:25 PM
>Subject: Jeff
>
>
>Well today was a better day for Jeff. He is still in excruciating
>pain in his privates which sounds awful and you can see it on his
>face, but he swallowed his pills better, only froze twice at
>breakfast (food in his mouth and not being able to chew), and was
>moving around a bit. The pain center is hopefully going to check him
>out tomorrow and try to determine what the problem is and how to deal
>with it so he can get on with his treatment without painful
>distractions. Imagine trying to win over this awful debilitating
>disease and then have such an incredible pain that it negates all
>benefits of treatment! They don't want this to happen. It is all
>covered under the research grant so no money out of pocket. This
>hospital is great!!! The nurses all love Jeff because he is so nice
>and cooperative, but we already know that about him. The other guy
>his age pings the nurses all night long and they are really getting
>tired of it. His mother is no longer staying with him at night so he
>gets really upset, cries and gets angry. I feel sorry for him because
>his wife really doesn't want to deal with his disease yet wants him
>to come home to her. His mother knows his wife will not give him the
>"loving" care that he needs. She observed her pulling his hair and
>"gently" hitting his head when she couldn't understand him. He
>doesn't know what to do. I think it is simple; he should live with
>his mother. If he expects a good recovery, he has to be with someone
>who will not only care for him in the good times, but also totally
>"care" for him in the bad which are quite a few. Luckily the human
>resources people here have sent a counselor to help him with this
>decision. I guess Jeff is lucky that I am and always will be here for
>him. If he had a wife that treated him like Jerry's wife, I would
>think he would want to stay with me. If he didn't, he would stay with
>me anyway! I want him back, or at least as close to, the way he was!!!
>
>Also, Mom, don't get the wrong idea about Linda. She is really
>holding up well under the circumstances. She has done a lot to help
>me with the bills, the paperwork, and holding down the fort while I
>am gone. It is really hard for her right now because her boss is NOT
>supportive of this whole thing and he keeps saying he needs someone
>who can work under pressure, etc. really aiming it at her. It is
>really hard for her to cope and then go home to an empty house and
>not being here to support me. We have had lots of talks about this
>and have actually gotten closer because of it.
>
>Anyway, Jeff just came out and hugged me and actually whispered
>"goodnight" to me so there really is light at the end of the tunnel.
>
>Love, Pat and Jeff


March, 2002

Sent: Saturday, March 02, 2002 4:18 PM
>Subject: Jeff...worse
>
>
>Well it finally happened. Jeff was in so much pain last night that he
>stood up from 1am to 4am and then they gave him a pain medication
>that knocked him out. Then they got him up at 5am, 7:30am, 8:30am,
>9:30am and finally at noon to give him all the pills he has to take
>at specific intervals. I took him down to the cafeteria in a
>wheelchair at lunch and he seemed to do ok. It took a while to get
>his pills down but he ate ok. Then about 2 hours later he went to the
>bathroom and came out and just stood there with his face all twisted.
>I noticed that his spine seemed to really curve in toward the bottom
>and called a nurse. She said it was part of the Wilson's disease
>contracting his muscles. We tried to move him over to the bed and he
>let out a really scary sound of pain. His face really twisted in
>agony. He later indicated that it felt like his whole back was having
>a charley horse. We finally got him into bed after about 10 minutes
>of very small movements and sounds of pain from Jeff. The nurse put
>some hot packs under his back and we let him rest. When dinner time
>came he could not swallow his pills. In fact his mouth froze open so
>he could not eat or drink anything. All he did is drool. He could
>not get out of bed by himself and could barely make it to the
>bathroom. The head nurse was consulted and they called the
>neurologist and they decided to put a tube down his nose into his
>stomach. They said the most important thing is for him to get those
>pills and probably all of his symptoms are coming from the copper
>breaking up and being eliminated from his body. He did not seem to
>mind when I told him, but his face has been expressionless for so
>long now I don't know what he feels. He says (blinks only again...once
>for yes, twice for no)he is ok with it but I just don't know. If he
>is ok with it he is the strongest kid I know because I would be
>terrified! I of course am not holding up well with this new
>development. It just seems to get worse and worse. The nurses try to
>comfort me telling me I knew this could happen and he will only start
>getting better but it is hard to see your child like this! Well on
>the up side, this morning when he was sleeping I went outside and dug
>a big "I love you Jeff" in the snow so when we were going to lunch
>and he always stops at the big picture windows to look outside, he
>looked 7 stories down and saw a big message for him. That seemed to
>put a very tiny smile on his face!!
>
>Love, Pat and Jeff

Sent: Sunday, March 03, 2002 7:20 PM
>Subject: update on Jeff
>
>
>Well, even though he didn't agree, today was a better day for Jeff.
>It started out with him not being able to walk very well and not
>being able to get up or go back to bed by himself. Now he is slowly
>walking to the bathroom and getting up and sitting down by himself,
>albeit very slowly. All of the doctors are going to be here tomorrow
>to discuss his case and their action. The pain center director, the
>neurologist and Jeff's doctor will discuss with me the options for
>leaving in the nasal tube or putting in a stomach tube. The surgery
>for the stomach tube is very painful, especially because they have to
>fill his stomach with gas to expand it and that is very painful for a
>couple of days, not to mention the hole they put in his side and
>stomach. I will tell them to handle his groin pain which is still
>very intense first before they can put the food tube in, if needed,
>because I don't want him more miserable than he already is. All of
>his medicine goes through the nasal tube now and he seems to be ok
>with that but they don't like to leave it in very long because it can
>cause nasal ulcers, throat infections, etc. The food tube would
>probably have to remain in his stomach for at least a year. If he can
>make it through the next 6 weeks with the nasal tube or several
>replacements, then when he gets home he will only have to take 3
>pills a day compared to the 10-12 he gets now. That would be a lot
>easier on him. His doctor warns however that if he gets worse in the
>next year and can no longer eat, then he will have to have the
>stomach tube put in so it would be better to do it now. It is a big
>decision. He only woke up about 4 times during the night and I along
>with him so I got slightly better sleep. Going to bed at 11 and
>getting up 4 times and then being woke up at 7:30 is just too hard
>for me. Hopefully tonight after his nerve-deadening medication (3X
>the dose) and the pain medication, he will sleep soundly. It seems no
>matter what they give him he still lies awake and shakes all night.
>Tomorrow will be the 3rd day of the Neurontin and the neurologist
>said it would take 3 days for it to alleviate the pain. We will see
>then. Betty will be here for a week on Wednesday the 6th. I am
>trying to figure out a way to get Linda out here for the return trip.
>She would get here on Thursday, April 11th and we would all leave
>together on Sunday, April 14th. We would all end up back in San
>Francisco at Uncle Buds. Betty is going to meet us there and then
>Monday morning, after profusely thanking Uncle Bud and Aunt Mar and
>the family for all of there (as usual) unselfish and loving help), we
>will head home. We will have too many bags to fit in the Camaro so
>Betty offered to take the bags back in her Isuzu. I feel I need to
>get Linda back here because she has had to be in the house alone for
>2 months without me to support her in this very trying time. She
>wanted to be here for Jeff but someone had to stay behind. She has
>been very supportive and helpful in getting things done for Jeff's
>disability insurance, etc. I think if there is any way, I need to get
>her out here just so she can feel that she helped more in some way.
>She doesn't really want me to because it would cut into our finances
>to take care of Jeff when we get home. Since I am not going back to
>work for a year or more to take full time care of him, things will be
>tight. Betty has graciously said she would come over every couple of
>weekends to relieve us so Linda and I can go somewhere and know that
>Jeff is in excellent, loving care. This would probably be the only
>trip Linda and I can take in the next 2 years, so I'm going to try to
>figure out if it is possible.
>
>Well, enough of that. It is snowing off and on here and tonight we
>are supposed to get up to 4 inches of snow. That should be fun! All
>in all, it was not a really bad day for us. Tomorrow may be
>different, but I just told Jeff to always find something good each
>day and he'll get better. I actually got a slight smile out of
>him!!!!! :-)
>
>Love, Pat and Jeff

Sent: Monday, March 04, 2002 2:16 PM
>Subject: Jeff
>
>
>Well Jeff was seen by the neurologist today and it was decided that
>his condition has deteriorated and he has lost too much weight so
>they are going to do the operation tomorrow to put the food tube in
>his stomach. This way he will get all the nutrition and medication he
>needs without struggling to eat it and no chance of aspiration
>(choking on the food and possibly getting pneumonia). Jeff is more
>willing to have it done than I am. He will have to have it in there
>either for up to a year or possibly the rest of his life. They now
>don't know how much of the damage will be reversible. Most patients
>get a little worse but then they get better within a few weeks. All
>are concerned here. He is on a double-blind study with a standard
>drug used to de-copper his body and an experimental one that is
>supposed to work much faster. Nobody knows except his big doctor
>which one he is on. The neurologist and speech pathologist are going
>to send today's results to him and he will make the decision tonight
>to either keep Jeff on the current study drug or give us the option
>to switch to the opposite drug. At that time, we will find out which
>drug he is on. If he is on the standard one, well it is still ok but
>I would definitely get him on to the experimental one. They can't
>just give him that one because it is not completely FDA approved yet.
>They have to test around 3,000 patients before it can be approved.
>Dr. Brewer told me though that it is the most promising drug yet in
>the treatment of WD. So, if he is not on TM as it is called, I will
>definitely get him on it if I have the choice.
>
>We are going to the cafeteria tonight for dinner. His doctor told us
>he could have "one last" real meal with solid foods tonight.
>Unfortunately, all they are having is tuna noodle casserole, catfish
>(yeah Danny would go for that!) and chicken breast with mushrooms all
>over it, all of which he hates! Well, it may be Wendy's.... :-(
>
>I'll let you all know how the surgery goes tomorrow.....
>
>Love, Pat and Jeff

Sent: Tuesday, March 05, 2002 3:39 PM
>Subject: Jeff on March 5
>
>
>First of all, in case some of you don't know who some of you are,
>bornhald2001 is Danny, Gayla is my sister in Oklahoma City,
>laresebboop is Betty Larese, Jeff's very good friend in Clovis,
>CA,tomjo is Jeff's grandmother in Bishop, CA, mdirlam is my brother
>Mike, cwyldy is Tari Dirlam, Mike's wife, tracyfulton is Linda's
>sister Tracy and tricka is my Uncle Bud. I may add people in the
>future to this list. One is the other Wilson's patient who is going
>home Friday. His name is Jerry and he wants to keep in touch with
>Jeff and how he is doing. Jerry is married so that is especially hard
>since he won't be able to work for at least 2 years and can't even
>communicate right now without spelling out each letter on a pad!
>
>Which brings me to Jeff. This morning was his best yet! They finally
>got him on the medication he should have been on from the beginning
>(the medication for his pain, not the WD). After the neurologist
>talked to us last night he figured out (we hope) what the problem was
>and changed Jeff's pain medication to an anti-spasm medication along
>with the nerve medication along with the study drug along with the
>galvin maintenance drug along with the anti-freezing medication along
>with the general pain medication. I think that is all he is getting.
>No wonder he has trouble swallowing!!! Anyway, he slept completely
>through the night only waking once to go to the bathroom at 5am. We
>woke up at 8am, showered and then he had to go to surgery. So much
>for feeling better! He did say good morning to me though and he
>looked much better. Once he heals from the stomach tube (the doctor
>described the pain as getting shot in the stomach with a
>shotgun...nice!) I hope he will continue on the path he was on this
>morning. He insisted on getting up to go to the bathroom a couple
>hours after he woke up and immediately tore open the wound and they
>had to get the GI people up fast to fix it. He is just a real champ
>in that arena. He is pretty doped up right now for pain but still
>managed to eat some Jell-O and a vanilla milk shake the dieticians
>made just for him. He even talked a little. Betty will be here
>tomorrow so that should cheer him up as well. I hope with all the
>medication he will be better tomorrow. It takes about 3-5 days for
>the surgery to heal. He will eat breakfast tomorrow then it is on the
>stomach tube for feedings from then on. I can still let him eat from
>the mouth from time to time but he has to be watched in case he
>freezes and chokes on the food. I am going to start working with him
>on increasing the volume of his speech as long as he can talk. His
>speech comes and goes as is to be expected. The main thing to be
>careful of is when he can talk to strengthen his voice so he can keep
>talking. The same thing goes with his muscles. He walks very slow now
>but he can still walk. His neighbor Jerry walks very fast and
>sometimes takes really fast little tiny steps like he's running and
>then sort of falls against something to get his balance and just
>waits until he can move again. Jerry's biggest handicaps are that he
>can't be understood when he talks and he coughs constantly. The
>doctors cannot find anything wrong with him and have tried many
>(including neurological) medications. He can cough for 20 minutes at
>a time and not stop. Sometimes you will see him gently slapping both
>sides of his face and pressing his lips together with his hands and
>sometimes a simple glass of water will do the trick. Each patient
>displays their own unique symptoms!
>
>Well, that is about all for now. I feel that with this new
>medication, Jeff appears to be doing much better and he looks as
>though he feels that way as well. Thanks again for all of your moral
>support!!!! :-)
>
>Pat and Jeff

Sent: Wednesday, March 06, 2002 6:22 PM
>Subject: Jeff, day 21
>
>
>Well today was one of those not so good days for Jeff again. There
>are a lot of good and bad days with this disease. He had a really
>good nights sleep only waking up at 7:30 for breakfast. However, they
>had him on 2 doses of codeine so that was probably it. His doctor
>said no more codeine unless he absolutely has to have it. He is now
>back on his milder pain killer and that is ok but the stomach
>feedings have started and I think that is the problem. Even though he
>doesn't mind eating through his stomach, it has made the incision
>sore. This is where his pain is. Also, he insists on getting up and
>going to the bathroom himself and getting up and getting back into
>bed puts strain on his stomach muscles.
>
>His mom Martha and Betty both showed up today. Betty was expected.
>Martha was a surprise. I think all this commotion also added to his
>condition. No one really wants a lot of visitors when they are in the
>hospital and Jeff is no different. Even though he is in for a long
>time, he still doesn't feel good. Martha bought Jeff a lot of clothes
>and a few for me. Linda really loved that last part!! Well, its the
>thought that counts and they were all on sale. Martha came in a car
>she just bought so she might take us all out this weekend to the
>movies or shopping or sightseeing. Lots of beautiful old buildings
>and dorms around here.
>
>Well, hopefully tomorrow will be a better day. Martha is planning to
>stay for about a week as is Betty. Then she is coming back April 1st
>with Jeff's half-sister Kelley. Then Kelley is flying back home on
>the 7th and I guess Martha is staying until we leave. I really think
>that is too many people here for Jeff and especially since they are
>females and he really needs privacy for some things. He hasn't said
>that yet but I get the feeling he thinks that.
>
>Pat and Jeff

Date: Sat, 9 Mar 2002 06:41:44 -0800
>
>Well we had a fun day yesterday but as usual, Jeff paid the price!
>Me, Betty and Jeff went for a long walk yesterday morning and Jeff
>did really good. When he got tired we had the wheelchair for him. He
>cannot walk more than about 10 minutes without getting really slow.
>His legs just start to freeze up then he can only put one foot out
>slowly at a time in little steps. We still had fun though. It was
>almost 60 degrees with no snow. Later on, Martha showed up and we all
>went to Meijer to get food and stuff since
>all of that is so expensive here. Jeff was sitting in one of those
>handicapped carts and we pushed him around. Betty and Martha didn't
>notice but I did. Jeff's face started twisting in that usual "pained"
>look. I asked him if he was ok and of course he said "yes." I
>persisted and finally he said he had to go to the bathroom. I knew
>this was the start, it is how it always starts. He starts getting
>that pain and just goes downhill from there. I told everyone that we
>had to go back. Jeff was quiet for the rest of the day. I had Martha
>and Betty leave around 9pm and Jeff got his pain medication. He woke
>up several times during the night and finally at 5am, he was in
>immense pain. His heart rate was real high and his breathing was very
>heavy with a groan in each breath. The nurse gave him a pain pill and
>checked the "residual" in his stomach. With the feeding tube, his
>stomach should be empty about 20 minutes after he is fed. He still
>had almost 100cc's in there almost 9 hours after he ate! He said his
>only pain was his stomach and it was bad. The only thing I observed
>earlier in the day was that his lunch/dinner nurse really pushed the
>food through the tube because she was so busy. The entire floor now
>is filled with other patients. Last night, Jerry's room (the 27 year
>old WD patient that Jeff befriended left yesterday...his program
>finished) was filled with a man who had his eyes bandaged shut and a
>hazard sign put on his door that says something like patient has
>radioactive material in his body...yuck). The nurses who previously
>had 2 patients each now have about 10 patients each and are really
>stressing. Paula is usually really nice and caring toward Jeff but
>last night she just seemed hurried and unfriendly. It usually takes a
>couple of minutes to get the 600cc in his feeding tube but she got it
>all in less than 20 seconds! I noticed his face when she did that
>and I could tell it didn't feel good, especially if it is ice cold.
>They are supposed to heat it slightly first. I asked him later if it
>hurt and he said "yes." I told him he is going to have to start
>telling the nurses when something is wrong or uncomfortable. They all
>know he is the nicest, sweetest, most undemanding patient they have,
>but he also never complains when he should!!! Anyway, right now he
>is sleeping, hopefully comfortably, and probably won't wake up for at
>least 4 hours because of the pain medication. They put a feeding bag
>on his tube for breakfast. This takes about 30 minutes to feed him
>rather than 30 seconds! Hopefully we will have a better day today!!!!
>
>Love, Pat and Jeff

March 10
Thanks to all who sent me the missing emails. I now have them all and
continue writing my journal. Jeff is still not doing too well. He
looks better but the pain is back. Tomorrow he'll see his doctor
again for a new course of action. Tonight as he was getting into bed
on his side he let out this awful sound. I was on the phone with
Linda and had to go. I guess his stomach tube broke loose inside
because it was pushed in and the retaining tube that holds it on the
outside was really loose, about 1 inch out from his skin. I could
easily push the tube in and out from the skin. It was gross! I asked
the nurse and she said this is common. The only thing that holds the
tube in is a button on the inside of the stomach and the moveable
retainer on the outside. I guess once it completely heals it won't be
as bad or feel so uncomfortable. Betty is leaving tomorrow and I
know she is sad. They hugged a long time tonight. She is such a
wonderful person, I just don't know what we would have done without
her there for Jeff! She is going to be there in San Francisco when we
get back and help us take all the suitcases back to Arroyo Grande. I
didn't think about it but the Camaro will not hold that many!!! I
hope it is ok with you Uncle Bud and Aunt Mar if she comes on Sunday
and stays the night so she can help us go back Monday. One nice thing
happened today (I always try to find a positive thing each day now).
It snowed quite a lot this morning and we had gale force winds and
hail last night, so there was some snow on the ground this afternoon.
We all went outside and immediately froze. It was 20 degrees out
there with a wind chill of around 8 I think! Anyway, I made a little
snowball and gave it to Jeff and he threw it overhand over his head
and it landed right on Betty's glasses!!! then everyone threw one at
everyone else. It was fun but it only lasted for about 5 minutes and
we were racing back(Jeff in his wheelchair) to the warmth of the
hospital!!!!!

We met a new kid named Andrew from Ontario tonight. He is 24 years
old and is on his 1 year follow-up. He had symptoms 1 1/2 years
before his diagnosis. The doctors thought he had cancer and gave him
a bone marrow transplant. Then they thought he was crazy and tried to
commit him. When he was finally diagnosed, he was put on
Penicillamine (the nasty drug) and went downhill fast. He made it to
Ann Arbor and got the treatment and was doing better. Unfortunately,
Canadian doctors don't believe that the Galzin (zinc maintenance
medication) works so they prescribed Penicillamine again. He
protested and caused such a scene that at one point he was handcuffed
and brought before a psychiatrist and they tried to commit him again.
His mother stepped in and luckily saved his life. Because he was not
on any treatment for several months, he deteriorated badly. Now he
speaks but with a retarded sort of tone. He may never recover his
normal speech again because once you are on the maintenance
medication, you can never get off of it for more than a couple of
days or your brain will suffer irreparable damage. It is really sad.
He said he has tried to commit suicide 3 times, the last time in
September by standing in front of a train. He lost his fiancÚ because
she wanted him to get a job as soon as he got back from the treatment
which was completely impossible. He lost most of his friends because
they couldn't or wouldn't understand his mood swings. He said it is
really a lonely and scary disease, but with the support of family and
new friends, he is making a slow but steady recovery. He is such a
nice kid (and he stands 6'6") I sure hope he makes it. We are getting
his email address to keep in contact with him when he leaves in 4
days.

That about raps up another day. This journey just gets more
interesting each day!

Love, Pat and Jeff

March 12
Martha is leaving tomorrow afternoon. Betty left yesterday. Jeff has
been staying up until 10-12 every night and I think this has added to
his tiredness and pain problem. He should be able to get a little
more rest and not have someone in his room at all times talking, etc.
The speech pathologist says this can really wear a WD patient out
real fast! A lot of the day I just sit next to Jeff's bed in a
comfortable chair and read a book or watch TV. I try to be as quiet
as possible so as to relax his brain. This helps in the healing
process. We do get up and walk a bit each day but he is down to
little tiny baby steps right now and has lost his voice again. I hope
with a couple of weeks of quiet rest he will be on the upside again.
He really enjoyed Betty's visit. They just couldn't do a whole lot
together because Martha was always present. She is a kind woman but
can be very overbearing and the arguments we got into and she go into
with the nurses were just not good for Jeff. The doctors have
basically come to accept that his pain is Wilson-caused rogue neurons
in the brain. I think I mentioned this in my last email. There is no
pain medication other than total vegetarian state that can alleviate
this pain. It will just have to work itself out as the brain heals.
This could take weeks, months or years. They are still trying
different combinations of medications to see if that helps. So far it
hasn't! The hospital mascot, a Labrador came to see Jeff today. They
had a good time!!

Love, Pat and Jeff

March 14
Well I hate to bring bad news since I try to accentuate the positive
but the neurologist saw Jeff today and his evaluation is that Jeff is
deteriorating rapidly from where he was 2 weeks ago. He said that
this only happens once in a great while. Most patients level off
after about a month of treatment. Tomorrow the head doctor along with
the others including the neurologist are going to meet with me to
decide the course of action. It will either be nothing or change to a
different study drug. The one I wish Jeff was on is TM which is the
one pending FDA approval after much testing. It works very fast and
usually stops any further neurological symptoms. Unfortunately since
it is not yet approved, they can't just give it to Jeff. He has to
have either that drug or the approved drug which works slower with
some side effects. If, however, he is digressing, then they can
switch to the other study drug. He may already be getting TM and it
just doesn't work for him, or he may be one of the rare ones who
continues to slide even though the drugs are working to get the
copper out. I told the neurologist that if it could happen to anybody
it would most definitely happen to Jeff from his past luck! He can
only shuffle his feet now and move very small amounts at a time. It
takes him about 3 minutes to get to the bathroom from his chair which
is about 7 feet away. At this rate, he could be wheelchair bound in a
week. If it gets to that, he will not be able to just walk again as
he gets better. It could take quite some time. The little girl down
the hall has the same problem. She could walk, talk and eat on her
own when she got here. She is now wheelchair bound, cannot eat (she
also has the feeding tube) and can only make strange sounds. Jeff has
not been able to talk at an audible level for about 2 weeks now. He
can still make sounds but only if you put your ear up to his mouth.
He also has patekei (don't know how to spell but pronounced
pat-ee-key-eye I think) which is bursting of blood vessels in the
feet. His feet are icy cold and purple with red blood dots all over
and some have formed into big blotches. Also, his gums bleed very
badly. This is all caused by the cirrhosis of the liver from what I'm
told.

On the good side, his free toxic copper level when he got here was 39
which is very high. The normal is 10-15. He is now at 15! His urine
copper level when he got here was 700. Normal is supposed to be
25-50. The doctor can't tell me what it is at now since that would
let us know what study drug he is on but he told me it was 20% less
(duh) so it is around 575. That is still high after 1 month but the
girls father told me hers did about the same after 1 month and she IS
on TM!

Now they have increased his anti-depressant/nerve drug to double and
tonight are starting him on another anti-depressant used for
Parkinson's patients which helps with lower extremity pain. He
already looks so doped up it is depressing and he is still in pain. I
am going to talk to them tomorrow and if these medications still
don't help by Monday, I will have them take him off all the pain
medications since they don't work anyway. They did a blood flow test
tonight to test his clotting ability. They put a pressure sleeve at
40 psi on his arm then used this razor blade type thing to make a
3/8" slash in his arm. Then they let it bleed until it coagulated. It
took 10 minutes to stop bleeding. It really seems cruel but they had
to make sure his blood wasn't so thin that he could bleed to death if
he cut himself. They also had to give him an enema because he hadn't
gone for 6 days! Unfortunately, all of the pain medications they are
giving him cause constipation so it just adds insult to injury...

WHEN WILL IT EVER END!!!!!! I can just imagine what you must have
gone through Tracy just watching Jeff go through all of this!!!

We did make it outside today. We went up to the roof of the parking
structure and Jeff spent about 10 minutes just gazing out on the
Huron River winding through the University of Michigan campus. He
really enjoyed that. We will send everyone pictures when we get back
so you can all see the enormity of it all. The hospital has been
rated number 1 in the entire country many times. This year it is
number 7. The campus is 10 square miles and each school (business,
engineering, medicine, etc.) has its own campus complete with dorms.
I think I already told everyone this but just in case... It has
130,000 students and 30,000 employees. The medical center, located on
the campus, has 505 clinics, 32 research centers and institutes and 3
hospitals all in one complex!!! It is HUGE!!!

One more "fun" thing. Even though all of this is going on and Jeff
has to be some amazing starship trooper to be able to handle all of
this, the morning nurse asked me why there is always a medicine cup
or a roll of silk tape on the floor in my room every morning. I told
her it is because Jeff can get up to go to the bathroom but he can't
get back into bed so he picks up anything "light" he can find and
throws it on my head to wake me up. It works every time!!! We had a
good laugh about that!

Well, I'll let you all know what the outcome of tomorrow's meeting is
tomorrow night. Thanks again for all of your prayers and support...

Love, Pat and Jeff

March 15
Well despite all the bad information I got today about Jeff's
progress and the study medications and the long talk with the
research doctor, Jeff had a great day today! Unfortunately, Danny
didn't!!!

Linda just emailed me and told me that Danny's car was stolen out of
the parking stall in his apartments last night. He finally managed to
get his license and then a car and had to drive it all the way back
from Napa by himself (his first long journey and alone to boot) and
then it gets stolen. Is there something wrong with our family? Is it
too much to ask to just have normal lives without all this grief???
Danny really needed that car and it was very gracious of Victor's
parents (Victor is Tracy's husband...Tracy is Linda's sister)to
arrange for Danny to have it and it was in excellent shape. He was
riding his bike in bad weather to school every day and it was a LONG
way. Even his bike, the one Jeff gave him, was stolen and his
employer and friend Reed bought him a new one. Then he had to ride it
to work, so he really got a workout and had to plan hours ahead of
time to be places. The car was a real blessing for him. I can imagine
the anguish and fear that went through him when he discovered it
missing. I think it is just sick that people do that not realizing
the impact they have on the victim. Danny, I am really sorry this
happened and I hope the police find it in one piece. I doubt you had
full coverage insurance on it since it is expensive and it was older,
so hopefully they will find it and it will be ok. If you get it back
ok, definitely buy a club for it (or we will buy one for you)!!!!!

Back to Jeff although it seems almost unimportant after Danny's
news...

I talked at length to the genetics doctor today about Jeff's case.
The neurologist increased the dosage of one anti-depressant and
prescribed another on top of that and his pain medication. I told Dr.
Brewer that Jeff was starting to look like a zombie and was STILL in
pain. He told the neurologist to cancel the oldest anti-depressant
since it didn't work and just try the new one. I was concerned about
the information I read that stated that one of the medications he
could be getting could cause some irreversible neurological damage
just like the bad stuff he was originally on. I have noticed in the
last two weeks that he can only move a few inches at a time now and
his voice is pretty much gone. The idea is for the patient to
stabilize in about 2 weeks to a month and then they start getting
better over the next 2 years. Only a very few get worse during the 2
month stay. Well, Jeff is one of those few! The problem is that the
article I read (written by Dr. Brewer) states that if they get worse
during their stay, they will only recover to the point of when they
got here which in Jeff's case means slurred speech and very slow
walking and the possibility of a food tube in his stomach for the
rest of his life! I was not very happy about what I read and asked
what the chances were of him getting off the study and on to a better
medication. It seems that he is NOT on the medication that is being
studied by the FDA since that seems to be a wonder drug. If he is on
the FDA approved drug (remember, it is a double-blind study and
nobody except Dr. Brewer knows which medication he is on), there are
more side effects and it doesn't work as fast as the experimental one
so his condition could deteriorate during the 2 month stay. Dr.
Brewer had to remind me that it was out of his control since it was a
government-approved study and he has to follow the guidelines or
else. This, of course, could be at Jeff's expense. He did agree to
the study though and he is still getting the best FDA approved
medication on the market to date. I just hope the other drug wouldn't
have done a much better job. Dr. Brewer also reminded me that the
papers I was reading were several years old and the drug then (the
one I suspect he is on...Trientine) was also experimental and they
knew little about it. Since then, they have discovered it works very
good. It is just that TM works much better!!! I won't know which drug
he is actually on until his 1 year checkup. They will tell me then.
Anyway, after all of that, today he was in a good mood and his pain
level was only at a 4 all day. We took a LONG walk around the outside
of the hospital and it has to be over a square mile of walking,
mostly uphill. It was not easy pushing that wheelchair uphill and I
paid for it!!! Jeff sure enjoyed getting out though. I hope tomorrow
is another good day. If it is and Sunday is also, we may go to the
movies Sunday afternoon. The way he walks though will really be a
handicap getting him in and out but he would love to go.

Again, I really feel bad about Danny's car and at the same time, very
angry at who did it and (I'm sorry but that's the way I feel after
all the crap that has happened to this family) hope something bad
happens to them!!

Love, Pat and Jeff

March 16
Jeff had another good day today. He actually talked to me and talked
to Betty on the phone for 10 minutes. He didn't say much to her, but
did talk a little. He even played a few tricks on me and smiled. This
is really encouraging for me probably more than it is for him. He is
always so tough and determined it makes me pale in comparison. He
wanted to go to the movies today which really bothered me because of
his laborious walking. I was afraid he would get really tired out as
in the past. Something as simple as walking for a few minutes can
have devastating effects on him for up to a week. Well we got to the
movies by taxi and when inside, I asked if they had a wheelchair or
something to help him get to his seat. They unfortunately said no. I
spent the next 15 minutes supporting Jeff on my shoulder and an arm
around his waist getting him from the lobby to the movie room. People
were staring but no one offered any assistance. About 30 minutes into
the movie he had to pee so we spent about 10 minutes getting to the
bathroom and another 10 getting back. Finally when it ended, we
waited for everyone to leave the theater so I could help him out. The
aisles are only big enough for 2 people and with him moving VERY slow
and me supporting him, I didn't want people to get antsy. One father
and his kids coming in just sort of pushed us aside! Anyway, the taxi
came and we got back and I asked him how he was doing. He said he was
ok (well, he was too tired to talk anymore, so he nodded his head). I
am hoping that with Sunday ahead of us as an easy, no tests day,
he'll be fine. His pain is a little higher tonight but not at a 10 as
it has been in the past after he exerts himself. I could not believe
the amount of energy it took for him just to do something we all just
instinctively do. I could tell his brain was really focusing on
putting one foot in front of the other and then his legs would try to
pick up his feet slowly. He was exhausted by the time we reached our
seats. I was exhausted just watching, helping and encouraging him to
get there.

All in all, it was a great day!!!

Love, Pat and Jeff

Date: Sun, 17 Mar 2002 19:30:29 EST

Dear Patrick and Jeff,

WOW!!! I had to reread the first sentence of your e-mail... Arroyo
Grande
huh? Amazing!!
I do have Wilson's and would like to meet you when you arrive back
in
California. Do know that you are in great hands with Dr. Brewer and
his
staff in Ann
Arbor
. I was one of his first group of patients way back in 1983. I
am on
zinc acetate. Now, I could go on and on, but I think I'll wait to
see you
guys in Arroyo Grande. Until then, I wish you both the best in Ann
Arbor
.

Sincerely,
Delia Ruiz

P.S. Please tell Dr. Brewer that I say "Hello"!

Well people I hate to do this to you but Jeff appears to be going
downhill again. It seems every time we do something together that
requires mental strain, he body sinks even further down. I guess it
does something to the neurons, maybe releases more copper and causes
more damage, I just don't know. He can now just barely walk and only
about 1/2" at a time. He has to hold onto something to keep his
balance. I talked to the doctors last week and they said to expect
some worsening but that is what they told me when he first got here.
Some worsening was walking slower but I fear that in a week he will
be confined to a wheelchair. I am going to talk to his personal
assistant tomorrow. She comes back after 6 days off. He also has a
rash where his feeding tube comes out of his stomach and it looks
"icky." I know the nurses take care of him but he just doesn't look
right. Daisy will be able to tell and maybe convince the doctors that
the medication he is on is taking too long to stabilize him. Maybe
they don't see the difference but I don't want a son who walked in
here not to be able to walk out of here. It looks like it is going in
that direction. It is heartbreaking to see him struggling just to get
to the bathroom, not wanting to ask anyone for help. I fear the day
is coming soon when he will not be able to use his legs and I know he
is getting scared also. This disease is just the cruelest thing I've
ever experienced. It lets you feel ok then when you want to enjoy
your good feelings it slam dunks you hard onto the concrete! Also,
they give him 600cc of liquid diet 4 times a day and at 10pm when he
gets the last of it he just hurts from so much liquid. I will talk to
Daisy about that also. She doesn't want him to lose any more weight
but he is so uncomfortable I can't stand to see it. These people are
the best in the world but I just see him slipping farther down and
wonder just how good they are. I know he cannot get better help
anywhere else on the planet but it still hurts!

Ok, I said I would find at least one good thing each day... This
morning I woke up and noticed he had put on a new sweater his mother
bought him. I asked him why he did it because it is normally too hot
for him to wear a sweater. He kept pointing to the stripes on it in
several different places. I didn't get it. He finally motioned for me
to get close to his mouth and he quietly breathed the word "green."
Then he smiled. I realized it was St. Paddy's day and smiled back and
let him pinch me for not wearing green! We have 27 days left!!!!!!

Love, Pat and Jeff

March 18
Ok I'll try to be more positive. Yesterday was just a BAD day. Today
is not any better and Jeff is back to his "10" pain, but Daisy is
also back and took charge once more. He has a slight infection AND
rash where the stomach tube was put in. She took care of that for him
and told the nurses off. They are supposed to change the dressings
twice a day and weren't doing it at all. The drainage from the wound
was running down his stomach and irritated the skin, hence the rash.
She is giving him an oral antibiotic. Daisy said the last thing Jeff
needs is a high fever or other complications when he is already going
through so much. She also said "no more outdoor trips" because they
just really wipe him out and his body suffers the entire week
following. She said he needs rest, rest, rest!!! The bright side is
that Daisy noticed that Jeff's walking is much worse than a week ago
but she again reassured me that it will get better. She said she has
still seen people in much worse shape than Jeff and on their yearly
checkup, they were walking fine. She told me that I have to be strong
for Jeff and I have been and will be. It is just tough when you see
them slipping each day. I know many of you have been through this
with loved ones that didn't return. Jeff isn't going anywhere as long
as he stays within his copper tolerance the rest of his life (the
newsletter refers to people every month who succumb from the disease,
usually of liver failure), but it doesn't make it any easier watching
him slowly lose his ability to walk!

Anyway, Daisy is really great. She knows what I'm going through
because she has seen it month after month for 6 years now, and she
reassures me that Jeff will be better next year. That is music to
both our ears!!! It may snow tonight. It is very cloudy and we went
outside for a while and it was real cold. That usually means snow.

Bye for now....

Pat and Jeff

March 19
Well today Daisy and I decided that Jeff will get some physical
therapy. It is not on the agenda since no one gets paid when it is a
research study, but Jeff can no longer walk on his own without losing
his balance and only moves about an inch at a time. Daisy made
arrangements for the physical therapist to come in tomorrow and she
will work with Jeff and draw some exercises for me to help him with.
She is also going to see if he can handle a walker. Daisy (AND I)
don't want Jeff to lose the ability to walk at all so we hope this
will work. The physical therapist said that if he needed a walker for
more than a short period, the hospital would buy him one. They are
all just so nice!

His pain is back with a vengeance. I can't help but think it is my
fault. I told the doctors to take him off some of the anti-depressant
and opiate pain medications because he was still in pain and only
opened his eyes about half way during the day. This looked like he
was drugged out of his mind and still in pain. The main research
doctor agreed with me and ordered several of them stopped. I
basically told you all this yesterday or the day before I think. He
then had a great Friday and Saturday, but after the show, he went
downhill fast. Daisy told me that one medication was really working
for him and why did I stop it. I told her it was because she deserted
us and I didn't know (smile). She got him back on one and his pain
level went back down to a 5. The problem is it won't officially start
until tomorrow at 7am so the pain is going back up again. When that
happens, we both wake up every hour. Since he can no longer walk to
the bathroom by himself, I wake up and help him. We are both really
getting tired of doing that!! Then we get awakened at 7am by the
nurses for his first feeding and I usually can't get back to sleep.
Jeff sleeps until about 10am before I wake him up. It is really
exhausting.

We now have 25 days left and counting EVERY day!!!

Love, Pat and Jeff

March 20
All the doctors saw Jeff today and the main doctor agreed that Jeff
is getting worse in his walking and recommended changing his
medication. However, the neurologist said that even though his
walking was worse, his tremors were not as bad thereby canceling out
his walking numbers so the medication can not be changed. I really
wonder if they realize the impact this can have on a person's life???
We all know that Jeff will get better and walk again but the turmoil
and anxiety going on in his brain must be incredible. He wants to get
better and is supposed to but he just keeps getting worse in the
place where he is supposed to get better! He keeps his chin up
though and I am proud of him for that. I don't think I could do it.
He got a walker today but the physical therapist made him walk over
200 feet and I knew the consequences. Sure enough, now he can't walk
even with the walker. It is now Dad's shoulder he hangs on to get to
and from the bathroom. With his pain the way it is, he gets up every
15 minutes to go so it is very tiring. Hopefully the new medication
he is on will relieve that in the next few days.

His friend Jerry (the one who coughed all the time) is back in the
hospital. He stopped sending us emails then his wife sent one saying
he was in. She didn't elaborate but we all know why. She expected him
to cook for her and get a job when he got back and he can't even go
to the bathroom by himself. The one thing all of us parents agree on
is that Wilson's disease patients need a LOT of unfaltering love and
support 24 hours a day by a loved one or they are NOT going to make
it. It will be tough for me and Linda and when Betty relieves us
occasionally it will be tough on her, but that is what is needed to
get Jeff back to some semblance of a life he had 2 years ago. Without
that dedication, he will not make it and the doctors and parents of
Wilson's patients all echo that repeatedly. I hope Jerry's mom
intervenes and just takes him back!!!!

Well, Jeff is getting his last feeding of the night and his nurse
brought him some chocolate ice cream and I smuggled in a frosty from
Wendy's so we have to enjoy it before the night is over. Only 24
days left!!!!!

Love, Pat and Jeff

March 21
Well I'm not going to say much tonight. The main doctor thinks Jeff's
pain is psychosomatic. I say he's full of s#^&!! Jeff's pain is real
and his whole body shakes from it and it makes him go to the bathroom
every 15 minutes and no pain medication seems to work. The other
patient with the violent cough was the same way. Nothing worked. It
is a wayward neuron in the brain telling the nerve to react to
something that isn't there. Even I can see that. All Wilson's
patients have some horrible thing they can't control. The little girl
down the hall has intense migraine headaches that make her scream
sometimes for hours. It is chilling to hear her through the night. It
doesn't make Jeff feel any better. He has been in absolute pain for 3
straight days now and his walking is almost non-existent but they
insist he still walk with a walker. I understand the principle but
his walking gets a little worse each time. I now have to help him in
and out of bed all night long.

Ok, enough of the down...It snowed today, violently at some times.
Jeff's afternoon nurse forbade us to go out since it was only 20
degrees outside and with the wind chill probably 5 degrees. We
watched the snow violently throwing itself against our 7th story
window and the trees blowing wildly in the wind and made our plans.
When Paula was busy with another patient, I put Jeff's hooded
sweater, jacket and gloves on and I put my sweater (I later wished it
was hooded), jacket and gloves on and we snuck down the elevator.
Just as we made it outside the sun came out and it stopped snowing.
DAMN!!! I decided to head over to the children's hospital and wait it
out. I told Jeff it was only a matter of minutes. Sure enough, it
started coming down with a vengeance again. We rushed outside and I
instantly froze my ears off. Jeff was quite comfortable as he always
is in the cold. I made a little snowball and threw it at him. I gave
him one and he threw it at me and it went directly to my neckline and
down my shirt. Boy was that unpleasant!!! Anyway, I could only stand
a few minutes of it but I did get a picture of Jeff with all his
garments and he looks like ET! I will post a picture (many actually
on my family) when we get back, and send some to whomever asks.

We are going to sleep now. Tomorrow's high is supposed to be 30 and
the low 20 with snow flurries again all day. We can't wait!!!!!!

Love, Pat and Jeff

March 22
Well today was interesting. It started off really bad since Jeff woke
up every hour from 11pm to 7am. By the time he stayed up at 10 and I
stayed up at 7 we were totally exhausted. It was really trying for
me. I actually got mad at Jeff for having to go to the bathroom so
much. I told him he needed to handle the pain better since no
medication was going to help him.....and then I broke down of course!
Taking care of a sick loved one can really be taxing on the body and
mind! I guess it boils down to total frustration. I told the morning
nurse that when the main doctor came in, Jeff was going back on
Neurontin (anti-depressant/pain medication) and I wanted the bladder
spasm med increased to 3 times a day. Also I wanted the
constipation-causing opiate Ultram cut down to only at bedtime. There
are just so many combinations it makes you sick. I was so upset I
told the morning nurse if they didn't do as I requested I would pull
Jeff out of the program. After all, they said that all patients get a
little worse, then level off during there stay here. Every single
patient I've seen come through these doors leaves in much WORSE
shape!!! She told me that is one thing she would like to change but
doesn't dare say anything. She has worked with WD patients for over
10 years and most leave in worse shape. She thinks the doctors should
tell the patients and parents that when they get here instead of "oh,
they will get a little worse, then stabilize, then get better." She
thinks the doctors don't want to scare the patients or their parents.
I believe not knowing the full story is much worse than just telling
the truth, especially when they act surprised that the patient is
deteriorating. When Jeff had the liver biopsy they told him he would
feel a slight discomfort. I reassured him it wouldn't really hurt.
THEN, they forgot to give him any pain medication and after the
biopsy, he had to wait for a gurney for 3 hours so he had no
medication for 5 hours!! That is one of the reasons I decided to stay
that first week.

Jerry has the bad cough that no one could get rid of and Marita has
migraine headaches and screams in the night. None of the parents were
aware this would happen. I had a talk with the head nurse and told
her the doctors need to tell the parents honestly what could happen.
A good example is when Jeff had the GI tube put in his stomach, again
the doctors said mild discomfort. I asked the male nurse in charge of
Jeff in surgery what it would feel like and that the doctors said
mild discomfort. He told Jeff in the worst case, it would feel like
someone just shot a hole in your stomach with a shotgun! At least
Jeff was prepared and when it didn't hurt that bad, he AND I were
happy!

Anyway, the doctor put him back on the anti-depressant, increased the
bladder spasm med and cut back on the constipating med to nighttime
only. Hopefully this will allow him AND I to sleep once again at
night. The big contention is always between parents and doctors. I
sit there all day long with Jeff and they evaluate him for 5 minutes.
I think I'm right and they think they are right.

Mary the morning nurse explained to me though why he is getting
worse. The medications are working very well because they are pulling
the copper out of the liver and brain quickly. In doing so, the
copper plaques in the brain are eliminated and a sort of 'blister' is
left in their place. This is what causes the neurological worsening.
Why can't they just say that "our treatment is going to make your
loved one worse at first but after the first 6 months they should
gradually start to improve." I would love honesty when it comes to a
loved one.

Today was a wild day for snow. It was fierce most of the day but
never stayed on the ground. Jeff and I went out in it again but the
wind was bad and the wind chill was 0 degrees. My ears froze within 5
minutes but we did get in one small snowball each!

Love, Pat and Jeff

March 23
Well last night was really interesting. It is the first night that I
did not wake up when Jeff had to go. For one thing, he didn't need to
go every hour so the reinstated medication started working again.
However, as with our luck, he woke up at 3am and threw several things
at me. I was so exhausted from the night before, I didn't wake up. He
called for the nurse and two nurses came in, right past me to help
him to the bathroom. I STILL didn't wake up. The next hour was a
nightmare!!! They both pulled him into the bathroom and stood there,
one under each arm and waited for him to pee. I don't know about you
other people out there, but I wouldn't have been able to go. Either
could Jeff. He stood there for over 30 minutes with them trying to
encourage him and he just froze. They couldn't even budge him!
Finally, one brought a wheelchair into the room and made so much
noise it woke me up. I rushed in to see what the problem was. He
looked awful. His face was so twisted you wouldn't even recognize
him. I told them to get him to bed and we got him there in about 5
minutes. I told them I would take care of it. Jeff and I stood there
until 4am with him trying to go. Finally I had him lie down in bed
and put a plastic sheet under him and the urinal up to him and said
just let 'er rip! Still nothing! I was getting pretty antsy because
the only solution then was the dreaded catheter and I knew he didn't
want that. While I was trying to talk him into going (remember the
times we had swordfights in the stalls in movie theaters when you
were little?...sorry ladies, the guys really do this!) I noticed he
had fallen asleep. I kissed his forehead and told him I WOULD wake up
the next time he had to go. That was exactly 1 hour later at 5am. I
felt something hit my head and woke up. He was sitting up looking
pretty bad and I immediately got him to the restroom. After about 5
minutes and a gallon, he felt relief and so did I. His nurse came in
with some medications and I got him back to bed and he immediately
fell asleep again. This time he stayed asleep up until 9am. He even
slept through breakfast! His nurse, Chris told me to go back to sleep
to since I needed it and she was right. We both woke up at 11am. I
felt much better. Jeff was still in pain but now his frequency is
about 1-2 hours compared to every 15 minutes. The medication is
starting to work again. It doesn't really help the pain, it just
delays the urge to go to a reasonable time. After lunch, he slept
until almost 5pm. I told the nurses to just let him sleep since he
needed it.

He can't really walk with the walker any more. That lasted only 2
days. His legs looks like they are made of spaghetti or Jell-O or else
he is really bowlegged. He moves in very tiny steps and fast as long
as I hold on to him. If he uses the walker, he just stops and
freezes. I asked him if he hated it when I made him walk and he
blinked twice..."no." I asked him if he understood that he had to
walk to keep everything lubricated so he could walk normally in the
future and he blinked once..."yes." I told him if I were in his place
I would be protesting and fighting against walking because it hurt
and I was so proud of him for being so tough and wanting to get well
again. I also told him I would do anything I could to help him
achieve that goal no matter how painful it was to me. It seems I feel
more pain making him walk than he does walking. It is just
interesting to see after a 10 foot walk his whole body clammy and his
face sweating. Something we could do in 5 seconds takes him 5 minutes!

The graveyard nurse made an excellent comment. The biggest problem we
are having is the fact that he cannot get into the bathroom in his
room because of the layout. She suggested that we move next door to
Jerry's old room where the bathroom door was twice as big and it was
very spacious inside. More importantly, Jeff could use the walker to
get all the way inside by himself. I spent this afternoon moving his
stuff into the new room. He is much happier now and so am I!!!

Love, Pat and Jeff

New phone number is 734-936-7403
Room number is 7403
New address:
just change the 7A Room 7402 to 7A Room 7403

Pat

March 24
Well I don't know whether or not I should continue with the updates.
It has been really taxing on me the last two weeks watching Jeff
slip. He can no longer walk (his legs are like cooked spaghetti) and
he can't swallow water anymore. His mouth is twisted so out of
proportion that I have to try to squeeze his lips together around a
straw (and his jaw is locked tight in the open position so it isn't
easy) to get him to be able to suck a little water and then he
usually gets too much and chokes. We have to be very careful that he
doesn't get too much down his lungs or he will get pneumonia on top
of everything else. If I pour a little water in his mouth from a
glass, it just sits there until it runs down his throat and chokes
him. He hasn't completely lost the use of his arms yet and I hope he
doesn't. I think that would be the final blow to him. He can still go
to the bathroom if I get him to it in a wheelchair and stand him up.
He can lock his legs so he doesn't fall down and use his arms for
balance. His pain is so immense right now, even after they started
all the pain medication back up, that it is hard to be with him or
look at him because of his expression. I don't want to keep burdening
all of you with the details of his deteriorating condition. I am
going to talk again VERY SERIOUSLY with his doctors tomorrow and let
them know they did NOT say this was going to happen. They told us he
would get a little worse before he stabilized and then start getting
better. He has just under 3 weeks left and I am going to take home a
totally handicapped son. If they knew he would get this bad they
should have told us. Jeff and I would have been scared but not as
scared as we are now. All I can think, and I'm sure Jeff does too, is
that something is going wrong. His doctors better have a good answer
for me tomorrow. They keep saying they go by a week to week point
system and he only gets a little bit worse each week so the points
aren't high enough to switch to another medication. A person can only
take so much pain before they break and I'm surprised Jeff has not
broken yet. I would have probably 2 weeks ago.

I made up an alphabet for him today so he can punch letters to let me
know what he wants or thinks. He can't talk at all any more. When I
gave it to him I told him I wanted to know what he was thinking or
feeling and he typed "I love you." That just tore me up!! We just
want to come home!!!

I'm sorry to keep at this. It does help me vent my agony and
frustration but if any of you don't want to hear the negative side of
this I will totally understand and take you off the list. There are
occasionally good things that happen but in the last 2 weeks, they
are very insignificant to what is really happening to him. Thank you
for all of your support out there. Your personal emails to me really
help to keep me going and the going is getting harder every day!!

Love, Pat and Jeff

March 25
Well I guess all of you want to support us and I thank you immensely.
sorry about my spelling lately but the stress has taken its toll on
my brain.! Well I have some really great news but first the icky
stuff.

Last night was another nightmare. Jeff was so frozen his face looked
entirely like another person. His mouth was so frozen, when I tried
to put a little water in it, it just drooled back out. The nurse tried
to give him some chocolate pudding which he ate about 3 bites then
the rest just sort of got "stuck" and I had to suction it out. He
tried to go to the bathroom and his back spasmed again so bad you
could put a large softball in the well it made in the small of his
back. I tried to straighten him and he groaned with pain although a
groan for him is silent...just some air being released from his
mouth...very frightening. I tried to sit him on the pot so he could
go that way but his body was so stiff I couldn't bend him. I called
the nurse and she decided rather than to put a straight catheter in
him to try a condom catheter which a lot of paralyzed people use. I
had him lie down on the bed and then talked to him the next hour how
it was ok to 'pee his bed' because it would go in the bag. He just
couldn't do it. He was in immense pain from the spasms so I started
brushing his hair with my hand and talking softly to him. He finally
went to sleep. At 3am he woke up as usual and since 2 rolls of tape
didn't wake me up he turned the light on which did wake me up. He was
really looking bad and I tried to convince him to just relax and go.
I got up on the bed with him and held him but still no release. I
finally did what I did earlier and lulled him back to sleep. I
decided that the doctors would hear me and hear me good. I knew he
had to be on Trientine because this was the only one of the two study
drugs which could cause neurological worsening in Dr. Brewer's own
words. This had gone far enough!!!!!

He woke up this morning around 10am. He looked really good. I asked
him how he was doing and he motioned "fine." I asked him how his pain
level was and he held up 4 fingers. I asked him if he had to 'pee'
and he motioned no. That was very unusual if you consider he normally
has to go every 15-30 minutes. This was really great news but I knew
from past experience it would be short-lived. Dr. Brewer and his clan
came in shortly after. A different neurologist saw him and Dr. Brewer
actually sat down in a chair for about 30 minutes...the longest visit
ever! She went through all the routines and he did them fine, even
walking with the walker (something he couldn't even begin to do over
the weekend). When she tried to get him to talk all she got is a
breathy sound. He couldn't write anymore. He got to the 'e' in Jeff.
When he stood still, his legs were like spaghetti at least. Dr.
Brewer just said thank you very much, we will go and discuss the
evaluation and let you know. I told him if he came back in 30 minutes
he would see Jeff as he usually is. It only takes about 30 minutes
upon waking to deteriorate. Sure enough, as soon as they left, he
couldn't go pee, he couldn't drink through a straw and his face
froze. His walking stopped also. I said in a snickering tone "Jeff,
why couldn't you be like this when Dr. Brewer was here? Now he'll
think you are 'progressing nicely' again." He gave me a smile but his
face had twisted up again. I thought "good, at least Brewer will see
him like he is all day long!"

Dr. Brewer came in about 30 minutes later and to my shock told us
that he felt that Jeff had deteriorated enough to end the double
blind study. He said Jeff was on Trientine (just as I had expected)
and that sometimes it does make a person neurologically worse
although it is reversible, unlike Penicillamine which is
catastrophic. He explained that Trientine is the drug of choice now
for liver symptomatic Wilson's patients but is being clinically tried
for neurologic patients. It has more side effects and can cause more
neurologic worsening in some patients. I told him I suspected this
was the case with Jeff. He said that every Wilson's patient is
different though and Jeff could have neurologic worsening with any
medication no matter how good it is. It is just the way of Wilson's.
He gave us the options of just using Zinc which he is already on and
is the maintenance drug for the rest of his life or he could start on
Ammonium Tetrathiomolybdate which has the least side effects and no
know neurological symptom worsening to date. This is the drug he is
testing for FDA approval and the drug I was hoping Jeff was on.
Jeff's eyes lit up when he heard that! I said of course we would
switch to TM. It takes 4 weeks to really work though and we will be
gone in less than 3 so Dr. Brewer will give us an ample supply to
finish out the treatment.

After that, Jeff and I just grinned at each other big time. I got a
picture of him with his biggest smile yet. Later on he had his first
non-aided bowel movement since he's been here. Things are starting to
look up! Even though Jeff is still in pain and has the same problems,
we both feel now he has a better chance for a better recovery with
this new medication. The neurologist also put him on a new
Parkinson's medication to reduce freezing with the least amount of
side effects. However, Daisy was concerned about his lack of
urinating so she took him off Tramadol which helped for bladder
spasms. I have observed that even though he feels like he has to go
constantly and the pain is immense, he can only go now about every 3
hours which seems pretty normal. She decided it wasn't. If she is
wrong, he will be up every hour again tonight. We put a leg condom
catheter on him though so hopefully he can go whenever he wants now
without worrying about getting up.

It is really snowing here today. We went outside and made snowballs
and threw them at each other. While I was making one, he hit me in
the back with one...cheater!!! I let him walk in the snow to hear his
feet crunching it. We got about 2 inches. I wrote another message in
the snow "Patdaddy loves (heart with 's) Pooty." These are our
private names for each other since he was a baby so DON'T tell him I
told you that! Anyway, when we went back up to the seventh floor, I
took a picture of it below. It looks really cool and he really
grinned on that one!!!!

All in all, this is my longest entry to date but it is the most
promising and the little point of light is now visible!

Love, Pat and Jeff

Addendum to March 25
Just wanted you all to know that Jeff's pain level has dropped to a 3
tonight. That is the lowest he has been since he's been here! He is
even moving around in his wheelchair and smiling a lot more! I hope
this is a sign of things to come sooner. I told him when he has bad
days to remember today. Tomorrow is supposed to bring more snow so we
should have more fun! Let's all cross our fingers and all that pray,
pray more and just turn this thing around!!!!! :-)

Pat and JEFF

March 26
Jeff woke up this morning with a "0" for pain. That is a first since
he has been here. I do believe the new medication is working! He has
been smiling a lot and even played chucky cheese basketball with me
(from his wheelchair of course). His pain level has varied from 0 to
3 but the new condom catheter bag has really helped also although
that brings up another really bad but humorous problem. I felt all
along that his pain, caused by involuntary bladder spasms (another
treacherous condition Wilson's can cause...not specifically bladder
spasms but rogue neuron firing that causes all types of pains
(Jerry's incurable cough for instance)). Anyway, these spasms were
excrutiating and made him feel like he had to go constantly and
every 15 minutes (every hour at night) he couldn't wait any longer
and had to go or he'd tremor himself to death. The problem is that he
would have to make it to a bathroom which meant getting to one from
his wheelchair or a walker or just me struggling to shoulder support
him to one. This could take a while then he would just stand there
for 20 minutes trying to go when there was nothing there. This
aggravated his back muscles and caused them to spasm and, well, you
probably get the picture. He was constantly in pain from this for
the last 4 weeks. I would have personally shot myself if I felt the
way he must have felt. This is when his face would twist into an
unrecognizable form and his whole body would convulse. This ON TOP of
Wilson's. That is why we say it is so evil!!! Anyway, back to the
bag. I felt if he had a bag tied to his leg and could go whenever he
wanted to, it would not only relieve a lot of the pain, but also the
anxiety of getting to the bathroom on time even if he didn't go. It
seems to be working. HOWEVERRRRRRRRRRRRRRRRRRRR.......we are having a
real problem keeping the prophylactic on! It keeps coming off and so
far he has gone 6 times and each time it has gone all over him, the
bed, his wheelchair or the floor! It is sort of humorous to him
because his pain is way down now but the mess is up! The urology
nurse, Bruce, came in today and thought he had it figured out. Obviously he
was wrong. The last two times Jeff filled the bag and the
prophylactic just sort of popped off! There is a new craze for guys
to wear these at sports events and bars or wherever drinking is
concerned because they don't have to leave and miss anything. If
these are the same size as the one Jeff has it definitely would NOT
work for a beer drinker!!!

Well, I wrote an earlier email but the hospital LAN connection
crashed as it was being sent so this is what I remember. I think in
the earlier one I explained in detail the study drugs and how they
affected patients, etc. Or, that one could have been last nights
email. The big news is simply Jeff is doing much better today after
switching to the experimental FDA pending medication and a new
medication for Parkinson's patients and it hopefully is working. Time
will tell. WD patients roller coaster so this could be just the top
and he'll slide back down. Right now we are enjoying the view. I want
to thank Mike for his wise words on how to look at a good day and
when you have a bad one, don't wish you were totally well, just try
to get back to the "good" day. It is easier to achieve this on a
gradient than trying to do it all at once. If the next day starts out
better, then just try to make it better yet and go from there. I also
appreciate Tracy's kind insights. She has been through an awful lot
herself with her own medical problems and her mother's. Actually, you
all send wonderful support our way and we really do appreciate and
NEED it!

Love, Pat and Jeff

March 27

Hi, it's Denise. I'll e-mail you my personal email soon. That way we can stay supportive and discuss what's going on. I'll keep our family e-mail for Jerry.

Here's the story. Jerry got upset and locked himself down in the basement. He was screaming FU at the top of his lungs and pounding on the door so hard it shook the upstairs floor. I called my mom and dad. They came over for 2 hours, and Jerry was calmed down. He still appeared a little anxious though. We talked again and later went to bed. He woke me up @ abut midnight and wanted to look at porn on the internet. I don't really believe in that, but all I wanted to do was sleep. I said do whatever you think you need to. He then woke me up shortly after 1AM saying he needed a credit card to put into the internet. I told him that wasn't in his best interests and he went berserk. He started throwing things, punching the doors, and running around the house. I had called my mom, she came over. He was still crazy. He headed for the kitchen where the garage door is. I thought he was going to run outside. He turned to the right, grabbed the knives out of the butcher !
block!! I was behind him grabbed his arms and the knives fell to the floor. Next I ran to call 9-1-1. The cops came and talked to him. He said he knew he needed help and they brought him to the psych ward. He also kept asking for his pills all night. I had to hide them, cuz he searched the entire house looking for them. I've got to go, cuz I'm at work.

Please hang in there. We'll all been dealt a bad set of cards. I'm sure you won't be pleased to know his parents blew up at me yesterday during our family session before Jerry could be released. His dad said I locked him in the basement. Give me a break!!! Besides how do you lock someone in a room when it locks from the inside. That's not all that was said, but I'm so sick of remembering everything. They insist the psychotic behavior would not have happened if he had been at their house. Whatever!!

I have been by Jerry's side the last 4 1/2 years. The last 2 he has been sick. I intend to stick with him through this, as I know he will once be back to the Jerry I know. It is very tough, but I believe we can make it through this. Denise

Remember they will have access to this e-mail. I'll get you my other address soon.
wiengsima
e-mail: wiengsima@genevaonline.com

Today was another great day for Jeff. He woke up this morning and
actually whispered "hello" to me. Dr. Brewer came in early and Jeff
said "hello" to him as well. They were very pleased with his
improvements. They reminded us both that this was a "high" and there
would still be "lows" but they didn't think he would get any lower on
the scale neurologically. Could he get much worse??? I found out
some more interesting information about the study. Jeff was more of a
guinea pig than we thought. According to the nurses, TM has been
proven successful in treating the hepatitic and neurological symptoms
of Wilson's. However, in order for the FDA to approve it, it has to
be compared in a double-blind study with another medication. As I
said in a previous email, the FDA wanted Dr. Brewer to use
Penicillamine which is known to cause irreversible brain damage in at
least half of the cases with neurological symptoms. Dr. Brewer
refused on ethical grounds. They told him he could use Trientine
which works best with liver-related Wilson's. Trientine's side
effects were not yet known and initial studies indicated it caused
some 'reversible' brain damage in neurological patients. Since the
damage was not permanent, Dr. Brewer went with Trientine. This is the
only way he could get TM approved by the FDA. The study was NOT to
validate TM as an effective medication for Wilson's but to determine
which medication was more effective with the least side effects and
'reversible' brain damage in neurological patients! Jeff was in this
study. The nurses told me after the study that they knew without a
doubt that Jeff would get much worse but couldn't say anything to
jeopardize the study. This is the way FDA testing goes unfortunately.
At least he didn't get a placebo which would have done nothing for
his disease and would have killed him eventually. Since both TM and
Trientine work to reduce the risk of death, either way Jeff would be
saved and back to some semblance of a normal life in a few years.
Unfortunately, he was chosen to test Trientine. He told me it was
better him than some other poor sap but of course I still feel the
pain. Knowing that everyone but us knew he would probably get much
worse was really upsetting to me. Of course, his case is just one
more case against Trientine and one more case for TM, which gets it
one more vote closer to FDA approval. I am so glad the nurses are
also patient advocates on this floor.

His pain level has been a zero to two most of the day. He still can't
walk (only baby steps with a walker) and he can't talk (but he can
whisper now) and he still has occasional freezing spells but that is
to be expected for the next year or two. Jerry's wife emailed me this
morning and said he went berserk one night and grabbed a big knife
from the butcher block and ran down into the basement. His wife
called 911 and they committed him to a hospital for a week (I think I
already said he had been committed). He tried to take all of his
anti-depressant pills and pounded on all the walls and screamed. He
was released to his parents yesterday and will live with them now. We
tried to tell him his wife couldn't handle taking care of him but
they both insisted. She came down for 1 day to see how to take care
of him and had no idea how serious and random Wilson's can be. One
day you can be fine and the next day trying to commit suicide or
murder if you are psychologically affected which many patients are.
Thank God Jeff isn't so far and hopefully won't be. She said she was
so drained after 1 week of his care that she could not function at
all. Welcome to the real world of Wilson's wife! She had no idea how
to care for Jerry. She was planning to take care of him for a week
then go back to work and leave him home alone for 8 hours every day.
We warned her over and over that was not possible. Now his parents
hate her and blame Wilson's on her for telling them for 2 years that
he was just imagining sickness. Funny, that is what a lot of Jeff's
friends and co-workers thought. I reminder her that Jerry's PARENTS
gave him Wilson's, not her! I told her that Jerry told me he loved
her very much and wanted to be with her and I told him it was a bad
idea because even though she loved him, she could not devote her
entire life to him for the next 2 years unless she gave up her job.
Jerry didn't want that and wanted it to work but we here all knew it
would fail badly and it did. I told her not to blame herself, just be
there for Jerry when he needed her and be there at the end of the
journey because he would really need her then. I also told Jerry that
Jeff said on their annual checkup next year that Jerry better be in
good shape or Jeff would kick his butt...that is if Jeff could lift
his leg up high enough!!!! Hopefully that will give Jerry and his
wife a boost!

Sorry these are getting long, but I figured a long happy one is
better than a long sad one!

Love, Pat and Jeff

March 29
Jeff has been pretty much stable the last two days. He can not talk
again and his walking is really shaky (basically bad) but his pain
level is down around 2 to 5 most of the time which is livable to him.
Daisy gets him to walk every day to a window in the hall which is
about 50 feet from his room. This seems short but it takes him around
15 minutes and when he gets there, he just collapses in the chair and
falls asleep for a couple of hours. She says this is good for him
because we don't want to get his legs stiff. I disagree with the
method. I believe he should do short walks with the walker several
times a day to keep his legs loose and not to tire him out
excessively. I understand that physical therapy rebuilds muscles or
rehabilitates muscles after an accident or illness, but the one thing
I always hear is that it is physically painful for the patient. That
is why most patients hate their physical therapists because they
cause them so much pain even though they are helping to rehabilitate
them. In a Wilson's patient, there is no pain associated with the
muscles being exercised. It just mentally exhausts the patient and
they "shut down" their entire body. Jeff just can't go any farther
once he has "maxed" out and you have to pull the walker to even get
him to move. I asked him if it was painful to walk and he said "no",
just tiring. Next week the physical therapist will be back and she is
going to make him walk around the corridor which is probably 200-300
feet. It will take him 30-45 minutes to do it if he makes it and his
brain will pay for it for probably a good 3 days to a week. This is
the big contention that us parents have with the staff here. They all
seem to think that WD patients will stiffen up if they don't use
their muscles (I agree) and they must over exert themselves to keep
the muscles working. There is a fine line here and some of you may
agree with the doctors. The problem I have with it is that Jeff does
not protest, does not complain of pain. He simply can go so far and
then his brain shuts off and he will stand there gazing out into
nowhere and you can talk all you want but he won't respond. Marita's
(the 8 year old girl who ended up in a wheelchair 2 days after she
got here) is now walking WITHOUT a walker because her dad would NOT
let them walk her until she passed out (which happened!). He believe
like me that a little bit each day and NOT tiring them out is better
than a lot in a short time and wiping them out for the rest of the
day and in some cases for days on end.

Anyway, we have 13 days left and we are getting very BORED!! We put a
puzzle together today and did laundry. That is about as exciting as
it gets! The main thing however is that Jeff has stabilized for now
on his pain and we can both live with no voice and stiff legs for now
as long as the pain stays away!

Love, Pat and Jeff

March 30
Jeff did not have as good a day today as in the past week. His pain
is up a little but the effects of Wilson's are really taking over. It
could be that the new medication is really pulling the copper out
fast and it is causing the problems. His face is really distorted
again, he cannot swallow and his body shakes or tremors badly. He
motioned he was not in pain, just felt very strange. He always looks
like he is in immense pain even though he indicates he isn't.

There is an interesting thread on the Wilsonsdisease.org list server
right now. One of the members, George, brought up the topic on
children. He said he thought it was unfair for a Wilson's patient to
bring children into the world and put them through the hell he went
through. He married a woman with children already and can't believe
other people would do that to a child. Several pregnant women
responded that they would take the chance to have a baby. A huge can
of worms was opened. I said that while I could understand George's
opinion, he has touched so many lives with his humor and his tragic
story that he should imagine a world without himself. Many people
agreed with me. Then a lady wrote saying that her 14 year old brother
died from liver failure years ago from Wilson's and she and her 3
other siblings were then diagnosed as well. Her liver failed at 18
and since then she has had a heart problem, never married and her
life has been just plain hell. She said if she had the choice, she
would never have been made. I really sympathize with her but at the
same time I told her that her brother sacrificed his life so his
other siblings could live on. She has had a bad life but she has made
friends and made the awareness of the disease greater just by telling
her story.

If you were to join this list server, you would be shocked by the
stories. It is just awful what these people have to endure, either as
a patient or as a parent. I can vouch for that! Some of these people
have been to hell and back and right back into hell again! I don't
know what any of your thoughts on this but it is an interesting
subject. I can't imagine a world without Jeff and if I knew that I
would give him this disease before he was born I'm not sure what I
would have done. To not allow him to be here on this Earth and
contribute to its history in any small way he might is just
unthinkable!

We now have 13 days left and counting. We are so bored, we hit every
floor we were allowed on in the hospital today just to see what was
on it. We finally got pictures of the elusive chipmunk in the
courtyard by the children's hospital. We saw it there several weeks
ago just sitting on a chair. By the time I got my camera out it was
gone. We had been sitting in the sun for about 30 minutes when Jeff
motioned over to the bushes. There he was just sitting there gawking
at us! I got out the camera and moved in. I set it to telephoto and
got him. Then he ran. I was happy that I got him but wished I could
have captured him on the seat again since it was so cute. Then as I
was getting Jeff back in his wheelchair he motioned to me again.
There it was. The little dude was just sitting on the chair waiting
for his picture to be taken. I carefully walked up close, zoomed in
and "snap!" Jeff and I gave each other the high 5 and went back
inside. Oh well, so much for an exciting day!

Love, Pat and Jeff

March 31
Patrick,

Thank you for your support. I do have a lot of things in my life
I am happy for. My Husband, my family, my two cats, I have a
few friends I would consider Good Friends.
I have just really had a rough road to get to where I am now. I
am sure that is what make my soul so strong and able to survive
and strive in my world today.

Sometimes life gets a little too much to handle, but all in all
I make it through.

I think the hardest part is to not let myself get so down about
things. I have a few happy thoughts I go to when life gets too
rough.

I pray your son will be alright and not have to endure the
battle of needing a new liver. I admire you for taking the time
to be there for him when he needs you the most. That is one
thing that will make him stronger. When I was ill right before
I had my transplant, I could barely get off the couch without
help I was so weak and helpless. My parents were there for me
every step of the way. My little brother who is 16 now was only
9 at the point of my sickest days, and his innocent heart and
love gave me the strength to make sure I kept in the right frame
of mind. I remember my parents would bring him to the hospital
to see me and he'd sit in my room he'd have such an optimistic
look on his face and he'd say "Sis, your gonna be alright, I'll
take care of you."

If I wasn't here today, I would have never known "love". I
think sometimes that is why my life was spared, If you die never
knowing the true meaning of *LOVE* have you truly lived?

Anyway, Just more of my thoughts,
Thank you for letting me just ramble on, I don't think I've
really been so open about my life before, but wow, it feels
wonderful.

Thank you for understanding,
Luella

Today was a fun day. Today was a boring day. We are bored! Darlene,
one of the nurses gave us a kite for Easter. We went up on the
parking garage roof and let it go. The wind, which is always wild,
wasn't. The kite went up, then down, then up, then down. Well, it did
get us out of the room. Most of the day was spent in Jeff's room.
Robert, Debbie and Marita left today. We had a tearful embrace and
farewell. They are staying in a motel by the airport then flying back
to Washington tomorrow. Marita was talking and actually asked me for
a hug. A few weeks ago she could only scream through the night with
pain.

Debbie the nurse brought us some ham, deep-fried turkey, smoked
polish sausage and scalloped potatoes for Easter dinner. We had that
and then some Easter cake for desert. Jeff isn't supposed to eat any
solids but he has never had a problem with that, just light liquids.
Daisy just equates choking on water equals choking on anything.
Tomorrow will be back to the old grind with blood draws, 24 hour
urine collections and DAISY!!! She really is nice but sometimes she
pushes Jeff too much and then he is worn out for the rest of the day
and then she thinks there is something wrong because he is too tired.
I can't seem to get through to her on that issue. He will also see
the speech pathologist tomorrow. Hopefully he will have a better day
than last Monday. We got some e-cards from Wilsons.org members today.
Also, a few said they would stop by before we leave. The lady I told
you about last night sent a very touching letter back today
acknowledging that her life was not that bad even though she lost her
brother and her liver. She said when she was sick and needed a liver,
her little brother stayed by her side and said he would always take
care of her. She told me he was a very kind person because of that
and HAS always been there for her. He is the only sibling who didn't
have WD. She thanked me for opening her eyes back up to the world
around her and how she has touched it. I got tears in my eyes
thinking that through our hard times, I could help someone who made
our journey look like Disneyland.

We are retiring early tonight since next week will be a hard one for
Jeff. I will try to get Daisy to ease off but I know she won't and
either will the physical therapist who is back from vacation. Jeff
took his biggest steps today, some as big as 1 1/2 feet for each
foot. When Daisy and the physical therapist get through with him,
he'll be back to baby steps. They get mad if I interfere and warn me
that he'll never be able to walk again unless I push him. I don't
know...I think that 1 1/2 foot steps are better than little 2" skids
across the floor, but who am I to argue with a professional? :-)

Pat and Jeff

April, 2002

April 1

If our offspring don't have wd, then they'll definitely be carriers
of the disease, according to the formula I was taught
by Dr. I.H. Scheinberg many years ago. If a wd mates with a wd
carrier, the chances of offspring having wd are
50/50. If a wd carrier mates with another wd carrier, they'll have
25% chance of having wd offspring, 25% chance
of totally healthy offspring (not even a carrier); 50% chance of
having wd carriers. If a carrier mates with a non-wd,
non-carrier, then there's a 50% chance that offspring will be
carriers, 50% chance that they'll be non-diseased and
non-carriers.

Jeff did pretty good today. He had a peaceful nights sleep and woke
up around 10am. Daisy was gone today so he got to sleep in.
Unfortunately, he started snoring SO LOUDLY at 2am it kept me awake
until 5am. I tried everything but could not quiet him down. I read
about snoring and interestingly enough Wilson's Disease can actually
aggravate the problem. It causes the tongue to loosen and the throat
muscles to tighten which further restricts the air flow. That is what
causes snoring in the first place. He has always snored to a point
but this is the decibel rating of a locomotive! I was going nuts
last night. I had such a bad night's sleep I looked like I had aged
10 years when I woke up. Even the nurses commented on it. I put him
on his side and he still snored. I hope tonight is better.

Jeff's mom called tonight and said she would delay her arrival for a
couple of days because her daughter broke up with her boyfriend and
they were weighing their options on what to do with their lives. They
are thinking of moving to California by us to be close with Jeff.
This would not only be a nightmare for me (and Jeff and Linda) it
would be catastrophic financially for her. I tried to explain that to
her but she thinks I just don't want her near (duh!). I hope I can
talk her out of it because I don't want her around Jeff that much
during his recovery. She did not help him at all while she was here.
In fact, the nurses all disliked her and she insulted Daisy and
Suzanne by saying they were only Physician's Assistants and Nurse
Practitioners and NOT doctors so they shouldn't be telling her what
was best for Jeff! I was furious. She also helped in Jerry's
regression. The last night he was here he wanted to spend it with
Jeff and me. I told Martha at 9pm she needed to go because Jeff
needed his rest. Her problem is that she never stops talking and none
of it usually makes sense so it strains your brain just trying to
figure out what she is talking about. In Jeff's case, it really tired
him out. Betty and Jerry were still in the room. Jerry was being a
little wild but it just amused Jeff. Well, Martha left but on her way
out she told the nurse that Jerry was really bothering Jeff. I didn't
know this and when Paula came in to get Jerry out, she told him it
was time for bed. I thought that it was time for him to go to bed
since I didn't know everything about his routine. He did not want to
leave and I had to help him out of the room. Paula was really mad
because he wouldn't leave and it just got worse. The rest of the
night, I found out later, Jerry was going crazy. They finally had to
call security on him and lock him in his room. He just went downhill
from there. I found out, after Paula wasn't very nice to Jeff the
next couple of days that Jerry's mom complained to Daisy and Paula
got in trouble for treating Jerry that way. If I had known that it
was all Martha's doing, I would have told Paula that Jerry was more
than welcome in our room. Martha always means well but sometimes
it can really be annoying!
Enough of that. Jeff did pretty well in Speech Pathology today. He
rated 4 points higher than last week. He still cannot talk but his
facial movements were better and he could write a little more
discernable. The rest of the day he slept. Just one hour of any kind
of therapy requires about 3-4 hours of sleep to recover.

There are only 13 days left till D-day and we are counting them down!

Love, Pat and Jeff

April 2
Not much to report today. Just winding things down. Trying to get all
the medical supplies and prescriptions taken care of through Betty.
The days are getting longer and more boring. If anyone is interested
in figuring out what the chances are of their kids having WD here are
the stats according to a dr. on the internet. You would still be have
to be gene tested to find out for sure and it would be interesting to
find out which side of our family (mom's or Mike's and my father's)
WD came from. If Gayla and/or Mike has the gene it came from Mom's
side. If just Mike has the gene then it probably came from our
father's side. Anyway, here they are:

If our offspring don't have wd, then they'll definitely be carriers
of the disease, according to the formula I was taught
by Dr. I.H. Scheinberg many years ago. If a wd mates with a wd
carrier, the chances of offspring having wd are
50/50. If a wd carrier mates with another wd carrier, they'll have
25% chance of having wd offspring, 25% chance
of totally healthy offspring (not even a carrier); 50% chance of
having wd carriers. If a carrier mates with a non-wd,
non-carrier, then there's a 50% chance that offspring will be
carriers, 50% chance that they'll be non-diseased and
non-carriers.

Jeff did ok today. Still freezes a lot after exerting himself.
Physical therapist showed up today and had him walk so he slept again
for about 3 hours. I got better sleep last night because when Jeff
woke me up at 2am snoring I lasted until 2:30 and turned him on his
side and he actually was quiet. The nurse tonight is going to see if
the doctors opt for a sleep apnea device where a mask is put on his
face and air is pumped in to prevent his airway from collapsing.
Also, interesting as it is, I was talking to a nurse about liver
transplants and we thought that once the defective liver is removed,
the new liver can process copper ok and you should be fine for the
rest of your life. However, all WD patients I have talked to who have
had a liver transplant STILL have to be on the maintenance
medication. I think this is because the body still has the defective
gene and it starts all over? I am going to ask Dr. Brewer tomorrow
and find out!!

Love Pat and Jeff.

April 3
Last night was another bad night for me. Jeff snored so bad and
sounded like he was gasping for air that I couldn't sleep. I rolled
him over on his side and around 4am he woke me up indicating that his
side was really sore. I had to move him on his back and he
immediately began to reverberate throughout the hallways again. I
finally put the head of his bed up to a sitting position. This
morning when Dr. Brewer and clan came in I asked them if there was
anything they could do. They are going to do a sleep apnea study next
Monday and Tuesday. There are no funds for this study in the research
center so Daisy asked one of her Neurology friends if he would do it
and they are getting someone from Canada to come down to actually do
the 2 day study. They are SO NICE!!! If they determine Jeff has sleep
apnea, they can prescribe some kind of device that fits over the face
and pumps fresh air into their breathing airway to hopefully prevent
this condition. Surgery is also an option down the road. Dr. Brewer
thinks it might be all the pain and anti-depressant medications Jeff
gets right before bedtime. It may be sedating him too much and
relaxing his throat muscles. Also, Wilson's plays a part in
controlling the throat muscles and tongue which all are involved in
the airway passage.

Dr. Brewer suggested that Jeff stay another week. We all were in
shock. He said Jeff's copper levels are not where they should be yet
and wanted us to stay if possible. I said we had already made our
plane reservations and Linda's was also set and betty had time off
and Dale (Jeff's friend) was coming over to help the next day, etc.,
etc. He talked it over with Daisy and decided to let Jeff take the TM
medication home with him and finish out the rest of the study. Since
it is not yet FDA approved they are not really supposed to do that
but Jeff needs to get the full treatment. I told him if we had to for
Jeff's health of course we would stay. He said that was ok and we
would only need to have a blood test done the week after Jeff
finished the treatment. What a relief!!!

It was sunny outside today and yet snow was flying past our window.
The last little fling before spring as the weatherman put it! Jeff's
pain was low again today but he still freezes up a lot lately,
especially after a physical workout like today on the exercise bike.
Daisy is going to instruct me on how to get all of his meds and
feedings coordinated when we get home. The nutritionist is going to
show me how to make his formula. We will need ultra slim fast as part
of the formula since it has no copper and has everything else. If any
of you see coupons in the paper for it, please cut them out and send
them to us (unless you use it yourself..:-)). Everything is starting
to wind down and we can't wait!!!!!!!

Love, Pat and Jeff

April 4
Well I said there would be good days and there would be bad days and
today was definitely a BAD day! Jeff was in pain from 4am this
morning. The nurse didn't give him his pain medication last night so
at 4am he woke up. The night nurse gave him some and he finally went
to sleep. He woke up around 10am but was in pain again. We did not
give him any more pain med because he can only have that particular
one every 12 hours and I wanted to be sure he could get it again
tonight at bedtime. Because of the 24 hour urine collection the
doctors don't want him using the leg catheter bag. I put a new one on
him anyway and didn't get it just right and it leaked out all over
the bed. It is very important that they get ALL of his urine
specimens over a 24 hour period to adjust the medication. If any is
missing or contaminated it could really screw up the medication. I
had to decide to leave the bag off today. This causes so much anxiety
for Jeff that his pain level doubles or triples. He has been grabbing
his pants all day long and just pulling on them. It tears me up to
see it but I told him we only have 2 more to go. Next Monday (which
is also the sleep study that night) and next Thursday (the last full
night he will be here).

Betty told me that the HMO won't cover the Galzin maintenance
medication he will need for the rest of his life since it is not in
their formulary. I will get Dr. Brewer to contact Jeff's new doctor
and see if he can convince him it is the only medication Jeff can be
on. Maybe that might help something. It is $100/month if the HMO won't
cover it. I will talk to Dr. Brewer tomorrow and see what our options
are on that since Jeff HAS to have it. We can't just look around and
think about it for a couple of months. He HAS to have 3 pills a day
every day for the rest of his life or a cure is found or he gets a
new liver, bottom line!!

Jeff's mother Martha is back. She called from the hotel tonight and
will be here tomorrow. I guess she is serious about moving. She had a
duplex in escrow and opted out of it. It only cost her $1,000 to get
out!!!! With that kind of stupidity, which she is famous for, she
will be out of money in no time. I was hoping that after all these
years of neglect, she might find it in her heart to give Jeff some of
her inheritance money to help offset his medical bills in the near
and distant future. But the way she is spending it (I think she has
already y blown at least $25,000 in less than a month) there will be
nothing left for Jeff. I definitely am going to try to convince her
that moving to California would be one of the dumbest things she has
ever done and she won't last more than a year with its much higher
cost of living. If she even thinks about moving close to Jeff there
is going to be trouble. He has indicated to me that he definitely
does NOT want her to take care of him ever and knowing her mental
problems, I know that to be true! I will keep you all posted on the
ensuing battle...

Love, Pat and Jeff

April 5
Jeff's pain was much better today. I guess the relief of not having
to hold it and then go every 15 minutes really helps. The catheter
bag is a life-saver, or at least a pain-saver. I found out that
Jeff's HMO will not cover the Galzin Zinc treatment but Dr. Brewer's
office said if we fill out a form, Gate pharmaceuticals (still can't
spell it) might give us a year's supply for free or at a reduced
price. That is a $100/month savings for us if it is free. Jeff's mom
showed up today and I guess that made my pain level go up! I heard
her telling Jeff that she definitely planned to move to California in
the next couple of months. OUCH!!! She plans to take Jeff maybe 2
days a week to care for him. She just does NOT have a grip on reality
or this situation. I would not let her even take Jeff anywhere in his
condition for the first year. She plans on taking us all to Canada
next Saturday (even Linda). Daisy said absolutely not. Martha just
doesn't get it. She thinks Jeff is just not able to walk and talk and
he can do all these other things. She does not GET the seriousness of
his illness and the fact that it is NOT curable. She wants everything
to be like it was when he was a baby and she even acts and treats him
that way. I have to really hold back on her, otherwise, I'd scream!!
I told her to get a wheelchair for Jeff today so we could go out this
weekend. She didn't and thinks he can get around with the walker. He
can walk approximately 15 feet in a walker then just stops dead in
his tracks. His brain won't let him go any farther. It can take up to
10 minutes just to get him that 15 feet. This last week could turn
out to be the longest yet!!! I have so much preparations to do next
week with the doctors, nutritionists, therapists, etc. I really don't
need another pain in my @$% added to all that!

Ok, enough of that. It snowed again today surprisingly and Jeff and I
went out into it and got some pictures of him with large snowflakes
falling all around him. It was the last fling (again!) before spring
as the weatherman keeps saying. It was sunny, blue sky and snowing!

We are both really anxious to get back home. We hope Mike and family
will be able to see us in St. Louis. We can't wait to get to San
Francisco
and see Uncle Bud, Aunt Mar and family. We can't wait to
see Betty again. We definitely can't wait to see Linda again but
we'll see her before we get back. We'll see her Friday when she gets
here. We are looking forward to seeing all of you, even you
eventually Gayla.

Love Pat and Jeff

April 6
Jeff had another good day today. Martha got here around 1pm and we
went to the local Wal-Mart clone called Meijer. We didn't get much but
Martha rented a wheelchair for Jeff so it was very un-stressful for
him. We then went to Fantastic Sams and got Jeff a haircut. They made
me mad because it was 4pm, they had 2 customers waiting and said they
wouldn't take any more people even though they closed at 5pm. They
finally agreed to take Jeff since he was in a wheelchair but not me.
When Jeff was done it was 4:20 and only 1 other guy was in the place.
Martha, the typical non-frugal and generous person that she is (just like Jeff)
gave the lady who refused me an $11tip! The haircut came to $14 and she gave
her $25!! I was so mad it would have been a miracle if I gave her a buck.
After all, a buck can get you 20 minutes on 10-10-220 or whatever that is called! :
Anyway, we got back to the room and packed some stuff in suitcases and Martha
left. Tomorrow we are going to see ET the 20th anniversary. Jeff and
I saw it when he was 5 so I hope we can enjoy it. Martha tends to
talk through an entire movie, especially when the exciting scenes are
about to take place. She'll say "Oh, this is the best part, you know,
when Cruella DeVille falls in the cake batter and ends up getting
baked...it is so funny, you'll love it!" I kid you not!!!

Jeff starts sleep therapy Monday night and physical therapy for the
rest of the week. Linda gets here at midnight Friday am so she'll get
to see Jeff's room and doctors before he checks out. Saturday,
contrary to Martha's ideas will be a quiet one. She insists that we
are going to drive to Canada and I KNOW we are going to take it easy.
It will be the first full day Jeff is out of the hospital and the
next two days will be spent traveling. After our simple romp today
he spent about 3 hours totally passed out in his bed. It doesn't take
much to tire him out...

Love, Pat and Jeff

April 7
The earplugs worked! Laura gave me some last night and I was able to
sleep through Jeff's snoring. I could still hear him occasionally (I
think that is just part of my paternal instinct) but it didn't really
keep me awake. Tomorrow starts his 2 day sleep study to see what they
can do about it. Tomorrow also starts the wind-down to leaving. It is
great in one way but kind of sad in another. I will miss everybody
here and all the fantastic help and support they have given us.

Martha was late today so we didn't get to the first movie. We ended
up going at 2:50 and saw the 20th anniversary edition of ET. Jeff and
I both enjoyed it. It was like a DejaVu for us. There were a few new
scenes like when ET falls in the bathtub and loves it! Martha fell
asleep during the movie and started snoring really loud. How
embarrassing. Jeff had his leg catheter on and we also had the
wheelchair for him so it was a very pleasant experience without the
tension and anxiety of last time. The last movie we went to burned so
much energy out of him he had his worst 2 weeks ever. I think you all
will remember that. That was just before he was switched over to TM.
The Trientine he was on would free up the copper in his body and send
it to his brain. Any physical or mental exertion seemed to trigger
the copper into all sorts of problems. I am glad we are over that for
now and hopefully for good. I overheard Martha telling Jeff again
that she expected to see him in California in about 2 months and by
then he should be walking and talking. Like I said last night, she
does not have any reality on anything. Daisy said we are looking at a
good six months at least for Jeff to even begin to walk and talk a
little. I know Martha wants only the best for Jeff but she also expects
him to fully recover in a very short time. I don't want Jeff to start thinking
that as well because it just isn't going to happen that quickly.
The next couple of days are going to be so busy for us I hope I can
still email everyone at night. I should be able to as most of the
stuff goes on before 5pm. Jeff and I are both getting antsy to return
home!

Love, Pat and Jeff

April 8
Well we are a little nervous about going home. After all, they do
take care of everything here. Jeff is about to go into sleep therapy.
A technician is already set up. He will put sensors on Jeff and then
when Jeff goes to sleep he will videotape him and watch him through a
window all night! Then, depending on what the neurologist determines,
he will go through it again tomorrow night but with some type of
apparatus to help him sleep. We may need that when we get back because
Betty is going to stay until Wednesday to help out and Dale (Jeff's
friend from Fresno) is going to come Monday afternoon and stay until
Wednesday to help set the house up for Jeff (handicap bars, shower,
bed, etc.). If Jeff snores like he does now, nobody will get any
sleep except Jeff!

Martha NEVER showed up today. I finally called her around 6pm and she
said she was sleeping all day. I guess that should answer any of your
concerns about whether or not I would let her have him 2 days per
week! That is one of the reasons I went for custody in the first
place. I found him one morning sleeping on the kitchen floor with
only his diapers on, with a bad cold, with paint cans all around him,
some of them open (and enamel), the gas oven on with the oven door
open (her heater was broken so she used the oven to heat the
apartment), and her sound asleep on the couch! Another time I found
him on the easement between the 1st and 2nd floor apartments
crawling around close to the edge with her again sound asleep inside
the apartment. Her daughter, whom she said would be raised perfectly,
got pregnant when she was either 12 or 14 and Martha let the guy live
with them!!! She just had another baby and gave it up for adoption.

NEED I SAY MORE???

{Note: I have since gone back over these emails and realized I was pretty bitter against Jeff's mother Martha. She is a very loving and caring person and has just had a lot of bad luck over the years. She has always had Jeff's best interests at heart and mine as well. You all must understand that this was a very emotionally draining time in my life and I didn't want anything or anyone else to make it worse. I felt at the time that Martha was trying to invalidate and complicate everything I did. In reality, she really thought she was helping and was very concerned about Jeff and me!}

Jeff verbalized the entire alphabet today and actually said a few
words to me. When Daisy came in he said "good morning Daisy." she was
really happy. He even surprised Lydia, the nutritionist tonight by
saying Hello to her. The doctors and nutritionists are going over
things with me and giving me supplies for our trip. The airlines
called today and will have a wheelchair ready for Jeff through the
whole trip. Hopefully Linda will be able to get out of the fog on
time from San Luis Obispo. It has been foggy there a lot lately and
the planes get delayed all the time.

tomorrow Jeff has his last speech therapy and Wednesday his last
visit from Dr. Brewer. It is really winding down!!! Remember, I will
not be able to send any emails from Friday night until we get back
Monday. I may be able to then just to let everyone know we made it
ok. I can send pager emails to Betty up until Saturday afternoon and
she could forward them to all of you. Linda will join us on Friday.
Hopefully I'll see Mike and family on Sunday. I'll see Uncle Bud and
family and Betty on Sunday also. I guess Uncle Bud could email the
rest of you on Sunday.

Love, Pat and Jeff


April 9
Jeff had another great day. He said his alphabet this morning and a
few words. It isn't very much, very monotone and mostly whispers with
a couple of throaty sounds, but it is at least understandable. He did
his final video also. He did an entry video where an assistant of Dr.
Brewer asks him to do all sorts of things like talking, walking,
dexterity, etc. He passed that one with flying colors. Today the
assistant only had him do a few things in the wheelchair. No walking,
talking, etc. Hopefully the video next year will look more like the
entry video this year.

A guy from the Wilson's list server came in today to meet me and Jeff.
His name is Gerry Fortuna and he has had Wilson's since 1986. He was the first
patient under Dr. Brewer's care to try TM as a medication in 1987. He
was misdiagnosed by 9 doctors. His workplace finally told him he had
to go to a psychiatrist to treat his manias since they believed all
of his neurological symptoms were mental. He told them he was sick
and not crazy and they said if he didn't go to a psychiatrist they
would no longer pay him! It sounds very similar to Jeff's case. He
finally went and the psychiatrist couldn't understand him so his
responses had to be basic head shakes. Luckily the psychiatrist
figured out he must have something physical wrong with him and there
just happened to be a convention in the city he was in that week and
lots of specialists. I don't remember why but the psychiatrist had an
ophthalmologist look at his eyes and the doctor saw the telltale rings.
Dr. Brewer was also at the convention extolling on this great new
promising medication for Wilson's disease called Ammonium
Tetrathiomolybdate (or something like that) better known as TM. They
all got together and it was confirmed that Jerry had Wilson's Disease
and he was in Ann Arbor 2 days later. He was on the treatment for 2
months just like Jeff and went back for 5 yearly checkups. He walks
and talks fine now. His speech every so often hangs up but other than
that you wouldn't know it. He started walking every day as much as he
could and sat down when he got tired. He eventually built up his
walking to 12 miles a day. He went back to work as an accountant
after 1 1/2 years. He said that is early for most WD patients but he
wanted to break himself in early. It took almost 1 year for his
handwriting to come back. He said he had to re-learn just about every
motor skill most people take for granted. His biggest problem was his
tremors. Since most accountants back then still used 10-key, his
middle finger had the most problems and he tended to add a lot of
5555555555555555555555's in his figures and had to redo them. He
laughed at that one. He told me of another WD patient I have
mentioned before. His name is George Feldman. George took Gerry to a Detroit
Tigers game during Gerry's recovery. Gerry still couldn't walk very
fast. He basically shuffled like Jeff does. George took Penicillamine
like Jeff started on (the one that destroys the brain) for about 15
years and he lost the ability to speak clearly. He sounds like he is
drunk! Well they were walking down the stairs to their seats and
since Gerry could barely move some cops came down and started
harassing them to speed up or they'd throw them out. George (a great
defender of the handicapped) blew up at the cops and told them about
Gerry's condition. The problem is that George sounded like he was
drunk! George has never had a drink in his life but try to convince
the cops of that. Gerry really laughed when he told that story. He
said they came very close to getting thrown in jail. Anyway, he got a
smile out of Jeff's face. He called the man in charge of the whole
Wilson's group and he will be coming in tomorrow at 7pm to visit with
me and Jeff. When we get back home we'll meet the publisher of the WD
newsletter who lives about 7 miles from our house.

Jeff is doing his last sleep study tonight. The results from last
night definitely show he has sleep apnea. The technician said Jeff
stopped breathing quite a few times during the night. He put the
C-Pap device on him tonight and all the electrodes again. The nose
mask he put on freaked Jeff out. You are supposed to keep your mouth
open and breathe through your nose. Jeff always keeps his mouth open
so a rush of air kept coming out of his mouth and every so often he
would just lock up and his airway would shut off. The technician
finally tried a face mask which worked quite nicely. As Jeff was
drifting off the technician showed me the readings on the computer.
Jeff's breathing was like waves. Very gentle and undulating. Last
night it was like a wave crashing to shore then low tide for a while
then a huge tidal wave suddenly out of nowhere. Corry will adjust the
machine during the night to find the optimum air setting for Jeff and
then a doctor tomorrow will write a prescription for it. They can set
the machine to start off with a gentle flow of air and then increase
it as the patient drifts off to sleep. Hopefully Jeff will get his
first really good nights sleep in a long time!

Love, Pat and Jeff

April 10
Some of you already know this but the rest of you need to know. First
of all, Jeff slept very well last night with the breathing machine.
The technician said he was like a slowly flowing tide. He stayed
awake the entire day without dozing off which proves he did get a
good night sleep. Hopefully now Jeff's HMO will cover the C-Pap machine
he will need. Dr. Brewer said people with severe sleep apnea can have
heart attacks because they stop breathing so many times during the
night. It is really scary to observe, especially when it is you son!

The big news follows: As you all know, Jeff was put on a medication
which is already FDA approved to determine if it had bad side effects
in neurologic patients. I think the first five weeks will attest to
that! Then he was switched to the wonder drug TM. When he first
arrived here, his urine copper level was at 670. The normal reading
should be around 25-50. After the first 5 weeks, his urine copper
level dropped only 20% to about 540. Dr. Brewer said this is normal
and most patients leave with their copper levels still in the low
hundreds. The zinc therapy usually finishes the drop within the first
six months. Marita, the little girl who left last week had a level I
believe at 200 when she left. I asked Daisy if she could at least get
me the last count because Dr. Brewer was going to keep Jeff on TM for
another week to week and one half to get the copper level a little
lower. He would have to take it four times a day without fail in
addition to his zinc three times a day. Well she came back glowing
and said his urine copper level had dropped to
65!!!!!!!!!!!!!!!!!!!!!!!
That is such fantastic news!!! That means that most of the copper is
now out of his body and he is on the road to recovery. He has lots of
problems with his throat and mouth freezing and his hands shaking and
all kinds of weird little neurological things, but that is because
there is a lot of scar tissue now in the brain where the copper was
and it has to start healing.

Linda is flying out tomorrow morning at 11:30 and won't get here
until tomorrow night at midnight. Now that is a LONG flight! Jeff's
mom is supposed to pick her up. I cannot get back in the hospital
that late at night and I DON'T want Martha spending the last night
with Jeff! I gave her a taxi number just in case Martha doesn't show
up but I won't know if she got here ok until the next morning. Kind
of scary for both of us. We are very anxious to get back home even
though we know it will be a rough road, but it will be at home! I
will probably send one more email tomorrow night and that will be all
until we get back home. I guess all of your prayers did do some
good!!! :-)

Love, Pat and Jeff

April 11
Today is our last full day. Thank God!!!! We are really wired up
about leaving. Today Jeff walked to and from the bathroom (a 10 foot
walk) in less than a minute with his walker! It usually takes him
5-10 minutes. I showed Daisy and she was so excited. This is the
first time Jeff has been able to continuously push the walker and
move without stopping for several minutes between movements. We were
all happy. I am getting pictures of all the nurses with Jeff and
tomorrow I'll get one of Dr. Brewer hopefully and especially Daisy
with Jeff. We are giving her a really nice planter with a neat plant
in it that says "I love you" on it. Actually I bought it for Jeff but
we can't really take it back so we thought Daisy might like it to
remember Jeff by. Jeff still has a bad facial freezing problem but
Daisy now says it is caused by the higher dose of the new
anti-Parkinson medication he is on. She ordered it cut in half so he
can still reap the benefits but possibly not suffer from the face
freezing. Martha was with Jeff for about 2 hours as I got the hotel
room readied for Linda. When I got back she left about 15 minutes
later and he just threw up his hands. He was obviously frustrated. He
typed out on his little sheet that she never stopped talking and
fidgeting with him the whole time I was gone. He needs a lot of quiet
and she definitely cannot give him that!

Linda is on the plane now as I type but should be in Detroit in about
1 hour. Hopefully all will go well and I will see her tomorrow at
1pm. This will be the last email for a few days. We are all happy
here that Jeff is improving so much in the last week. He will still
have a lot of "bad" days but for now we are really enjoying the good
ones.

Love, Pat and Jeff

April 12
This is it! Today is it!!! We are out of here!!! Yeah!!!! Actually it is a little sad and a little scary. Jeff and I have gotten to know the doctors and nurses so well here, it is hard to leave. But, we must move on with our lives and back to the house on the coast and back to civilization! We got our picture with Dr. Brewer and Daisy today and Jeff gave Daisy the plant. She loved it and told Jeff it would be big and beautiful when he returned next year. It was an emotional goodbye for us. Linda got here with Martha around noon and I showed Linda around the complex. She was quite impressed. We went to the cafeteria one last time for lunch and I used up the meal tickets. Jeff and Martha stayed in his room. When we got back, we loaded all the suitcases onto a couple of wheelchairs and left. We went out to Martha's car and got in and drove away! We stayed in the Microtel in the same room we stayed in the night we arrived. It was rather eerie. Jeff started having a problem with his feet almost immediately. I was worried because we were no longer in the hospital. We just sort of hung around in the motel room the rest of the day. I had enough formula for tomorrow (Saturday) and then some Ultra Slim fast for the plane trip to Uncle Bud's on Sunday. I know that the plane trip is going to be really hard on Jeff. It will be hard on all of us.

Love, Pat and Jeff and Linda

April 13
Martha and Linda went shopping today and did some sightseeing. Jeff and I went outside. It was a fairly nice day. We went over to a park-like area by the Holiday Inn and just sat there on a bench for a couple of hours. We were just glad to be out of the hospital and free again. We finally headed back to the Microtel around 4pm when Martha and Linda came back. Martha rented "Harry Potter" for us and we watched that. Martha started snoring so loud we couldn't hear the movie. We all laughed at that. She ordered pizza from the same place we got it from our first day. Jeff had diarrhea tonight. I think he is really freaking out about the plane trip home. I am very concerned because we no longer have the security of the hospital and a professional Wilson-trained staff. Martha will take us to the airport tomorrow morning.

April 14
What a nightmare! Jeff was searched in his wheelchair at the airport. We thought we lost one of our bags and it was behind us the whole time. Martha cried and left. Linda started getting really sick. It took forever to get Jeff on the plane in those little aisle wheelchairs but we managed. As soon as the plane took off, Jeff started acting really strange and threw up all over himself. I panicked because his head went straight back and he could have aspirated. He threw up twice and looked really bad. The stewardess gave us some towels and that helped. I got really scared thinking about what could be wrong. Then Linda started getting really sick with coughing, headache, etc. I was in the middle and feeling really bad. Jeff finally mellowed out but the flight only lasted about an hour. As we approached St. Luis, Jeff threw up again. I felt so sorry for him. When the plane landed, some paramedics came aboard and started asking a lot of questions like "do you have permission to take him out of the hospital?" and "Can he continue to fly?" and "is he infectious?" We finally were let off the plane and we went to a bathroom to clean up. Then I bought him a souvenir T-shirt from St. Luis and put it on. The plane had already boarded so we had to get on last. Jeff did not look very good but I told him we would be home soon and all would be ok. As soon as the plane took off, Jeff threw up again! This time it wasn't very much but he tried to hide it from me so his mouth was straight up in the air and he started choking. I was so scared it was horrible. This flight lasted 4 hours and I thought it would never end. He finally went to sleep off and on and I encouraged it. When he woke up, he just looked awful. I prayed that the flight would end soon. Those four hours were the longest in my life! When we finally saw California I told Jeff "we are home, son!" and that seemed to cheer him up a little. I was worried about him throwing up again when we landed but he didn't. We got off the plane last and walked out on the good old Earth again but this time it was California Earth and it felt good. Uncle Bud, April and Betty were there waiting for us at the gate. What an emotional reunion that was. Uncle Bud was a little shocked at the condition Jeff was in since the last time he saw Jeff about 4 months earlier he was walking and talking. Betty had Jeff's new wheelchair waiting and he tried it out. It was a good fit. We got our bags and left the terminal. Jeff went in Uncle Bud's car with me and Linda went with Betty in her Isuzu. We all met at Uncle Buds. Aunt Mar was there to greet us. Jeff was in a lot of pain with his feet but I know he was glad to be back in California. We stayed downstairs that night since Jeff couldn't walk up stairs anymore. The whole family came to see us that night. Carol, Douglas, April and Andy. It was a nice reunion. Jeff had a rough night sleeping but finally managed to get a few hours in.

April 22
Today we left for home. I took Jeff in my Camaro which hadn't been driven for 2 months. Linda went with Betty in Betty's car and followed us. Jeff seemed to be pretty happy about going home finally even though he still hurt pretty badly. I know that the next couple of months are going to be really rough because we have to go through his HMO and the doctors there are just not as caring or even concerned about this disease. His neurologist does not even know anything about Wilson's Disease but I can at least call Dr. Brewer and Daisy any time if I have any doubts about Jeff's treatment. We need to get him on MediCal ASAP when we get home so he can at least get some local treatment. Jeff's HMO won't give him any services in our area because it is "out" of the local coverage area. If Jeff gets sick, my only option is to drive him to Fresno on a 3 hour one-way trip. I let the windows down and we drove all the way home with the wind blowing on Jeff's face. He seemed to like that a lot. He didn't move much. I could tell he was in deep thought about what he had been through in the last 2 months. We arrived home around 6pm. I had a hell of a time getting Jeff into the house since it was not equipped for wheelchairs or walkers. I set up the couch for him to sleep on and realized right away that we would have to get him a roll-away or hospital bed. I made his formula and we just sort of spent the rest of the day unpacking and organizing things. Jeff turned on the cartoon channel of course and watched it for hours. Betty slept on the sofa bed with Jeff so he wouldn't be alone. I slept in my own bed for the first time in 2 months. It was nice. I think Jeff and I had both forgotten how nice it is where we live. I left the front door open and we just looked out at the view. It was beautiful. We are glad to be home!!!

The rest of April was not recorded in a journal nor did I email people with any information. We were all just acclimating to our old surroundings. I bought a roll-away bed for Jeff and realized it was not going to work. He really liked it but even though it was longer than his body, he still tended to slide off the bottom! His showers were fun. I put up a lot of bars in the bathroom for him to grab on to when he went in. I installed a special shower head like the one in the hospital and bought a shower seat so he could sit down. Going number 2 was just as much fun. Jeff could get to the seat but he could no longer wipe himself so I had to help him with that. I realized that I was going to have to retrain him in everything just like when he was a little boy. This seemed like a really great thing to me. We could be together again and start over. Jeff and I have really become even closer now since this whole ordeal started if that is even possible. I still did not have the time to fill out the MediCal forms nor did I even know how. Jeff seemed to be doing better during the month in his walking and even a little talking but his feet still hurt pretty bad. One of the favorite things we did was sitting out in the yard on our old beat up lawn chairs and just looking at nature. We would do that for hours sometimes. I decided that I was going to redo the yard and plant some nice scented flowers in the garden so when I opened the door in the morning, the fragrance would fill the room. When we went outside, Jeff would have a nice lawn and beautiful flowers surrounding him. I just didn't have any idea WHEN I would have enough time to do all of that!

April 29

Jeff has not been doing too good lately. Last week we went to Fresno
for his doctor visits and he was doing really great during it all.
The only thing I noticed was that he seemed to be really "hyper." He
was fidgeting all over and had a lot of nervous energy. He could
however get in and out of the car by himself and really impressed the
physical therapist with his walker walking. He has a new pain in his
rump and thigh muscles in his left leg but the doctor prescribed a
muscle relaxant and that caused his pain to go down to a "3" which
was very low. He got more restless as we got home and that night as
he slept he had several outbursts where he actually talked very loud
but made no sense. That was Friday night. He has not been able to get
out of bed since that time. We tried Saturday and Sunday but he was
in agony and eventually didn't respond to much of anything. I called
his doctors back in Michigan and they told me to take him off the
muscle relaxant and to remember that after all of that exertion it is
expected for him to regress for at least a week. He looks really bad
just laying there and just touching his left leg causes lots of pain.
That is the leg he uses to get in and out of the car though so that
would make sense. He has not been able to drink water for 3 days as
well because of the facial dystonia(freezing) caused by the pain. I
had to swab his mouth down a lot today just to get the dried gook out
of it. It is really hard for me to see him this way but I just keep
reminding myself and him that it will pass. Please keep all your
prayers coming in as we still need them!

Love, Pat, Linda and JEFF

May, 2002

May 4

Jeff was doing really great last week but got real "hyper" for some
reason. He was getting in and out of the car by himself (out of the
wheelchair into the car that is) and walked for the physical
therapist with the walker the best he has ever done since going to
Michigan. However, last Friday night when we got home he was
extremely hyper and although he can't talk, when he went to sleep he
was almost shouting in his sleep. He has since become almost a
vegetable! He cannot hold himself up even with a walker (his body
looks like a zigzag) and his mouth is constantly frozen open. His
eyes just stare into nothingness and he has partially lost the
ability to control his bowels and his bladder. This has been
devastating to me! To see him do so well and then to totally reverse
to a complete invalid is just awful. I called the doctors in Michigan
and they said when he exerted himself that much it could cause him to
go the other way for about a week but he has not changed. Every time
I get him up to take a shower or exercise a little it completely
wears him out and he pants for about an hour and freezes really bad.

He has been out of Ann Arbor now for about 3 weeks. The doctors said
the first 6 months would be the worst but it is awful that he got
better then really bad. Jeff takes maybe a couple of sips of water a
day when his mouth unfreezes but sometimes he gets no water for 2
days. I have to wet his mouth with a sponge. He is also on a C-Pap
machine to aid in his breathing at night because he has sleep apnea
really bad. I have been having a hard time dealing with it all and
really let him have it last night. I told him he could stand up all
night as far as I cared (I was trying to get him off the exercise
bike and since he is 6'4" and 220 pounds and I couldn't do it by
myself so I got frustrated and mad at him for not trying even though
he was trying so hard) and then I just broke down and cried for about
an hour. Linda consoled me and talked to Jeff for a long time. I
finally went to the store to get my mind off of things for an hour. I
called Betty and she came over today. We are going back to her house
tomorrow and checking him into the hospital for physical therapy and
a neurological checkup. I am so close to him it hurts real bad just
to look at him. I cannot do this by myself so Betty offered to take
some of the burden off of me for a couple of weeks. I just can't
believe I said those things to him when a WD patient's emotional
state is the most fragile in the first 6 months.

I hope this will help since Betty is so gracious and said she would
take care of him for me after she gets off work each day and on the
weekend so I can go do something to take my mind off of the whole
ordeal (although I could never do that completely). I know Jeff will
get better but it is just so agonizing to see him stare off into
nothingness knowing that he is fully cognizant and his mind is
constantly thinking but he can't even show that he is feeling
anything. Anyway, I will still be checking my email at Betty's but I
will be gone from home for 1 to 2 weeks. I didn't email this to mom
because I did so already last night in reply to her awful email about
Joel, Jenny and Julian (what's new for her??).

Happy birthday Uncle Bud!!!!


May 17
Love, Pat, Linda and JEFF :-)

This is NOT a good update. We just got back from Fresno. We have been
there 2 weeks because I could not care for Jeff by myself as he got
worse. He has now got worse than then. His whole left side is fading.
Even his eye doesn't open fully. I am very upset and concerned about
it. I got Betty to get 2 MRI's ordered for him next Wednesday. They
are going to do his whole spine and see if a nerve is messed up. If
that isn't the case the only thing left is his brain and that is a
scary thought. His brain should be healing yet it is causing more
problems. I hope it is just a pinched nerve. The therapists are now
talking about a skilled nursing facility until he improves. The
problem is his muscles are SO weak now he can't even lift his arms
much less his legs. If he goes in a facility like that they will only
care for him. He needs the pain eliminated NOW so he can heal. He
could get into a physical therapy program in a home but they won't
really care for him as well. It is frustrating! I cried for about 2
hours this morning. His mouth has been open for 2 weeks and the
inside is just awful. It is so dried out with dried phlegm and mucous
it is really horrible. I have to clean it several times a day with
special swabs and keep it moist with a spray bottle. I hope by
decreasing the anti-Parkinson drug he is on it will help. It was
increased in Michigan and his mouth froze open so they decreased it.
The neurologist here increased it and his mouth froze again.

It is so unfair that Jeff has to go through this it is unbearable. He
is still determined to get well and told me today (by squeezing
hands) that he wanted to go home. We have to go back next Tuesday for
3 days but he insisted. He indicated he would feel better being at
home. I couldn't help but oblige. I need it also. I couldn't sleep
the last couple of nights and my chest really hurts bad now. The
strain is taking its toll on me but I will be there for him no matter
what. It was so good to see Linda today when she got home. We hugged
and I gave her her Mother's day present. She really liked it. I
missed Mother's day And Valentines day AND Easter!

I will keep you all posted on what happens next week. The MRI results
should be read by Thursday and we are also doing a 24 hour urine test
which will be analyzed by the end of the month to make sure his
copper level is still ok. If it is not, he may have to go back to
Michigan. Thanks to all of you for your encouragement and prayers!!!

Love, Pat, Jeff and Linda

May 18
Jeff has been having a LOT of problems since he got back from Ann
Arbor
. For some reason his entire left side appears to be in pain or
going numb. It started about 1 month ago when he was getting in and
out of the car from his wheelchair on his own and actually walking
quite well with the walker. The next day, his hamstring and glut
muscles were excruciating. It has been getting progressively worse.
In fact, it is so bad that he hasn't been able to do much of anything
and now his muscle tone is going fast. He can no longer lift a Pepsi
can over his head. We had a home therapist come out and she said it
appeared that his sciatic nerve which runs from the hamstring down
through his leg might be the problem. It could be from stiff muscles
or a strain or what? He is getting 2 MRI's done next Wednesday on his
spine to see if something is wrong. We have tried every type of pain
medication including ones for muscle spasm and NOTHING works. I
finally tried a TENS unit on his leg and it relieved the pain
somewhat as long as it was on. His left eye doesn't open as far as
his right eye and his left hand has water blisters on the tips of
every finger! This is really freaking me out and the doctors at his
hospital never seem to take anything seriously. They say he may have
to be admitted to a skilled nursing facility if I can't take care of
him but they won't solve the muscle problem and give him the
attention he needs. My question is this: Has any of you experienced
this type of symptom when you were healing/recovering from the
de-coppering? Jeff is still fighting so hard to keep his sanity
through all the pain and it just isn't fair. I don't know if I'm
doing anything wrong. I work with him every day and try to keep his
muscle tone up but it is getting harder and harder for me to get him
from his bed to the bathroom/chair, etc. He is 6'3" and 225. I am old
and tired! If we could just solve his pain problem we could get on
with his recovery. I am hoping it is NOT some of his brain cells that
are damaged so badly that the pain will never go away. I would have
given up long ago and it makes me love him even greater that he keeps
trying to hold on. Please, if any of you have experienced similar
things while recovering, let me know to reassure both of us that
things WILL get better. I am almost at the end of my rope and sinking
fast!!!

Patrick

May 23
I hate to send you all this message this way. I wish I could call you
all but I don't have time.
Well Jeff ended up in the hospital today. He started breathing really
fast and labored last night and this morning was worse. I thought
maybe when I had lifted him up I broke a rib or something so I took
him to emergency at 10am this morning. It turns out he has some body
infection and they transferred him to ICU on special antibiotics for
3 days. I told them I would not let him be admitted unless they let
me stay in the ICU room with him. They said that was against their
policy and I said "see ya" so they got a bed for me. The doctor in
emergency told me that Wilson's was so serious that there was NO hope
for Jeff and had I discussed life sustaining conditions and organ
transplant with him. I was furious!!! This was AFTER he read the
complete charts from UM. I hate these (I'm sorry for my language)
ASSHOLES who have never even seen a Wilson's patient and then think
they no everything about it. He told me he would arrange for Jeff to
be placed in a nursing home. I explained to him that he knew nothing
and that almost all Wilson's patients who have been in Jeff's shoes
are now walking and talking and he just shook his head and said "I'm
sorry, that is just not true!" If I could shoot (I know it should be
educate but it is my son they are talking about) every doctor who
claimed to know everything about Wilson's, maybe we could get down to
some serious diagnoses and save a LOT of pain for patients and their
loved ones. I am at Betty's right now to pick up his feeding tube
formula because they can't get him the copper-free ingredients until
Saturday and he hasn't eaten OR had any of his meds since last night.
Also, the pharmacist took his Galzin and said "we can't allow any
'experimental' drugs during his stay!" I am going back to the
hospital now so I can be there to "protect" my son from these
hackers! I will not be able to respond for a couple of days but will
let you all know what happens.
I have not eaten since last night myself and we did not get a room
until 7pm even though we got to emergency at 10am. We were in
emergency until 6:30!!

I AM SO SICK AND TIRED OF THIS $%#%&^#^#^ IGNORANCE!!!!!!!!!!!!!!

Pat and Jeff

May 26
Sorry this is so long but you all know that is my style and I really
needed to get this off my chest...you will see why!!!!

Well they say it can't get any worse but....Jeff was admitted and put
in ICU. I spent 2 days there with him and then transferred to a
regular room. I gave them the EXACT formula for his tube feeding and
they told me they wouldn't have the ingredients until Saturday (this
was Thursday). I guess it is ok for him not to eat for 2 days??? I
told them I would bring the ingredients. The first day they made it
he got 700cc one feeding and 900cc's the other. They also gave him
his zinc with EACH feeding until I finally demanded to see what he
got each time. Because he was in ICU I couldn't do anything but I
just demanded to be involved in everything. The nutritionist came up
several times to make sure the formula was right and it still never
was. I also told them he needed Lactaid with each feeding and he
NEVER got that! Friday night he needed a catheter tip for his Foley
catheter. They said they couldn't find any (remember, this IS ICU!!).
The next morning we woke up at 7am and Jeff's bed was soaked and he
was freezing. The catheter tip had come off as expected (they hadn't
changed it since we checked in Thursday morning)and they only last
about 12 hours). I told the duty nurse and he said he would get Jeff
changed and new bed sheets. It finally got done at 10am...3 hours
later!!!! I was furious. His meals were never on time and they
insisted on continuing to give him his Galzin with his meals. Finally
I got him transferred to a standard room where MORE nurses and MORE
aides worked. I thought finally I would get better care for him.
Saturday afternoon the nurse was very nice and said she would have
the formula mess straightened out and let me give the meds. Well,
that night the nurse tried to give Jeff the Galzin with his feeding
again! Jeff's doctor (the idiot who thinks he is going to die) came
and had me sign a form that stated what I wanted to do WHEN Jeff
became a vegetable. He had checked a box that stated the patient was
mentally incapable of making any decisions. I told him to uncheck
that box. He told me that Jeff could not talk. I told him Jeff did
just fine by squeezing your hand and "did you ever try to communicate
with him?" Jeff's neurologist came in at the same time and said hello
to Jeff and Jeff slightly waived to him. The neurologist then asked
Jeff some questions and took Jeff's hand in his and got squeezes for
responses. I told the "idiot" doctor "It looks like he can
communicate just fine to me!" Jeff seemed to get worse and look worse
as the days progressed and they never could get his medication or
feeding formula correct. The last straw came this morning when at 7am
he needed a bowel movement. I paged the nurses station and they said
a nurse would be right in. I waited 10 minutes and called again. She
told me all the nurses were busy but she would let one know ASAP. I
waited another 20 minutes and Jeff was really squirming around in bed
so I went outside the room and found his nurse walking by. I asked
her if she was coming to help get him up for his bowel movement and
she said "what?" Nobody ever told her. It took another 20 minutes for
her to get an aide and then for them to decide Jeff was too big to
get out of bed and placed a tiny bedpan under him. Of course he could
not go like that and they would not let me get him out of bed to go
to the bathroom. The final straw was when the nurse looked at his arm
and gasped. When he went into emergency, the nurse there had to draw
blood and give him an IV. I would say that this nurse must have been
a total novice because he could not get a vein and Jeff has never had
a problem donating blood for doctors and nurses. I watched as the
nurse kept probing and pushing the IV in and out of Jeff's arm. It
was making me cringe just watching his ineptness. It turns out that
the IV was not in exactly right and over a period of a couple of days
the saline solution leaked out of his vein and into his arm. His arm
was twice its normal size and very hard. The tape they used also
caused horrible blisters around the IV site. Needless to say, we both
had had it!!! A doctor making his rounds came in to check on Jeff and
saw what was going on and told me that Jeff was over his infection
and if I was smart I would get him out of there ASAP! He said Jeff
had a greater chance of getting a bad infectious disease while there
and from what he saw of his treatment, he would be better off back
home with me. We didn't have to ask twice. I told him to sign the
release papers and we would be gone. One nice thing however...I live
165 miles away from the hospital he has to go to and they told us
they would pay for an ambulance ride for Jeff all the way back to our
house! I think they were probably glad to get rid of me and believe
me, the feelings were mutual! The last thing was when the ambulance
lady asked me why I would refuse any kind of resuscitation for Jeff
while he was en route to our house if something happened to him. I
did not understand so she showed me the form his "idiot" doctor
signed and it said I had wished that Jeff not be given any type of
CPR, etc. in the event that his heart stopped! I don't know where
that came from but I just rolled my eyes in my head and she gave me
the paper to tear up!!!!

We are now back at home and quite happy!

Pat and Jeff

June, 2002

June 3
You would NOT believe the nightmare we went through and are STILL
going through with that hospital. He went for his neurology appt. on
the 22nd of May and Dr. Hadera and Jeff's neurologist discussed the course
of action (thank you VERY much!) and he was put on Artane. The next
day he was complaining of shortness of breath and really bad pain in
his lower right rib cage. I thought it was his liver or a broken rib.
I called the ambulance and they took him to emergency. He was in
there for 9 hours and they decided to admit him to intensive care. He
did not have any damage after they ran lots of blood tests and x-rays
BUT he did have an infection and a fever and dehydration from a tiny
dime-sized bed sore on his foot I didn't even know about. He has been
bedridden for over a month because of the intense pain in his left
leg and it pulling up (he could not stand up because he couldn't
straighten the leg). I think they called it Cellulitis or something
like that. They monitored him for 2 days and
moved him to the regular hospital for another 2 days. When he was
admitted Thursday evening they told me they could not feed him for 2
days because they could not get the ingredients for his formula. I
told them they could buy them at any supermarket and they said they
couldn't do that. I finally told them I would bring what I had so
they could make it. I worked with them for 3 days and they STILL
could NOT get it right. It was lumpy, too much one feeding and not
enough the next. They also kept giving him his Galzin WITH his meals
and I finally exploded and told them I would give him all of his meds
myself. The first day in ICU Jeff's catheter tip came off because
they couldn't seem to find a new one the night before even though I
repeatedly asked them to find one. At 7am I noticed his bed was
soaked and I asked the nurse to please take care of it. He finally
got around to it 3 hours later!!! The next day it took 45 minutes for
them to find a bedpan so he could have a bowel movement. By then he
could no longer go. The first day in emergency a nurse kept trying to
put an IV in Jeff's arm and seemed to be having a lot of trouble.
Jeff has never had a problem with that before and the guy looked like
an amateur. Four days later on Sunday morning Jeff's arm was the size
of Popeye's and hard as a rock! The nurse finally noticed it and
freaked out. She immediately had a doctor come in and he told us that
the fluid had leaked out of the IV into Jeff's arm and it would
dissipate within a couple of days and I quote: "If I were you, I
would get him out of here today...there are a lot of viruses floating
around the hospital right now and it is not safe for him! His
infection is under control and his fever and dehydration are in
check. We will send him home on 7 days of antibiotics, but get him
out of here!" The ambulance company came and took Jeff back to my
house 165 miles away which I thought was nice. The ambulance driver
noticed that I did not want Jeff revived if his heart stopped and I
told her I did not say that. It turns out his idiot doctor (we have
now changed him) had me sign a form stating Jeff was not to be
revived in any situation but explained it to me that it would be only
if he was in an irreversible coma or extensive brain damage (he seems
to think Jeff has irreversible brain damage!). Also, he had marked
that Jeff was not mentally capable of responding to anything and that
really pissed me off.

Anyway, to make a long story longer (you know me) he came home last
Sunday and developed a really bad case of diarrheaa and has had it now
for 8 days!!!!!!! I called the hospital last Tuesday and they told me
it was probably c-diff (I think) or some other virus and to get them
a stool sample. They got it on Wednesday and told me the results
would be in by Friday or Saturday at the latest and a doctor would
call by Friday afternoon to prescribe a stronger prescription-based
anti-diarrhea medication. In the meantime I could give him Kaopectate
as often as he needed it. I called Cewin and she and Dr. Wiley told
me to cut his formula down to 1200ml and give him 1200ml of Gatorade
to put electrolytes back in his body. I got NO calls from a doctor on
Friday and no calls about the stool samples on Saturday. I called
this morning and a nurse told me that a doctor would call me by 3pm
at the latest. At 3pm I called and they said the doctor was TOO BUSY
and would probably get back to me tomorrow. I told them my son has
had chronic diarrhea (up to 4 times a day) for 8 days now and I don't
think it is a minor problem. She told me to bring him in and I
refused. I said I am not going to try to get a bed-ridden kid out of
bed just to have the doctor give me a stronger medication when he can
do that over the phone. The specimen cultures all came back negative
so they don't know why he has diarrhea but don't seem too concerned.
Daisy, he has gone through 4 LARGE bottles of Kaopectate and about
$200 worth of Depends and those blue bed undercovers, baby wipes,
paper towels, etc, not to mention major discomfort. He had an attack
last night at 1am and we were up until 5am when his problem finally
settled down. I am so furious at this HMO (I told you it was bad!)
that I don't know what to do. I am trying to get Medi-Cal for him but
I have to go to them, they won't come to the house in this small
area. His neurologist finally ordered a portable suction machine
because he is also generating a lot of phlegm in his throat now and
because of the pain (and dysphasia) his mouth is wide open and his
neck is getting stiff so his head goes straight back and the phlegm
gets stuck in the back of his throat and his swallowing doesn't work
so he gags on it and it almost chokes him to death. This happens
chronically every night even though I prop his body in a sitting
position. Tonight he coughed up at least 1/2 cup of saliva that had
accumulated in the back of his throat. I won't get the machine until
Friday however when Betty comes from Fresno because they won't
authorize Apria over here to deliver it to us since I am NOT in
Jeff's HMO's local area! This is such a nightmare it is really straining
my nerves to the breaking point. Jeff is doing better on his leg pain
with the Artane but has been set back drastically with this diarrhea
problem. The doctor just called me and prescribed some antibiotic
(starts with an O I think) for C-diff (again I think it is called
this). He said give Jeff Kaopectate up to 6 times a day and call him
in 3 days if Jeff is not better. That would make his diarrhea problem
11 days and running at that point. Why are some doctors and
organizations this bad Daisy? Don't they remember the Hippocratic oath
or don't they teach that anymore just like California doesn't teach
the Constitution to a lot of kids any more? Betty just told me the
virus going around in the hospital lasts about 3 weeks and has put
several employees there in wheel chairs. It attacks the joints and
makes people so weak they can't walk at all. Jeff has a real problem
with his left arm now but I don't think it is that type of virus. He
does not throw up because of it, he throws up because of the saliva
accumulation.

You asked me how Jeff was doing? I hope this answers that question.
It is so unfair that we have to go through all this crap just because
he has such a BAD HMO and we can't move on to his recovery. It just
pushes it further and further away. As soon as his diarrhea is over I
am going to really work with him to get him back in shape so he can
at least stand up in a walker again. Now he just collapses or hangs
on to me and is dead weight! Once he is fairly strong again I can
enroll him in an intensive physical rehabilitation center. My mother
in law works there and Jeff's HMO covers it but only if they can
withstand 3 hours of intense physical and speech and occupational
therapy every day of the week. It lasts about 2-4 weeks and most
people do really well with it. One good thing...Jeff's loud voice has
returned. You can not understand anything he says but his voice is
good ol' Jeff's volume!! I wish we were back in Michigan under your
wonderful care!!!!!! Do you still have the plant and the planter Jeff
gave you? He is still going to walk in and see it next year!!!

Pat

June 6
They say that no news is good news. In Jeff's case that just isn't
that way it works. Since his hospital fiasco he has had chronic
diarrhea for 11 days straight now. He has lost 20 pounds even though
I am giving him electrolytes, etc. I took him to the doctor yesterday
(a 6 hour round trip) to make sure nothing else was wrong. We now
have a new doctor and he seems to be very nice and concerned. He
checked for pneumonia, etc. and found Jeff to be ok. The bad pain in
his side (right where his liver is) is probably due to scar tissue
from the copper coming out. It only hurts when I turn him on his side
to clean him. Daisy agreed that it is probably from that. His liver
enzymes were fine so that would indicate there is nothing seriously
wrong with the liver. His windpipe and esophagus are real sore which
was another concern. The doctor again agreed with Daisy and said it
was probably because of the fact that Jeff has had his mouth frozen
wide open for the last month and dry unfiltered air is constantly
going down that way. As for the throwing up mucousy stuff, that is
probably because he cannot swallow correctly (try to swallow with you
mouth wide open and your tongue all the way back in your mouth and
you'll see what I mean). He just makes a clicking sound with his
throat and water sort of goes down. Saliva eventually accumulates a
lot in the very back of his throat where the bulk of his tongue is
and that eventually gags him and his head goes straight up in the air
(mouth facing ceiling) and he cannot breath because if he did he
would inhale all that mucous! I have to be there and force his head
down so it will all come out. He cannot bend his neck very far
because of the Dystonia that is creeping through his body (stone-like
stiffness). I now have a suction machine to get the mucous out of his
mouth. It is very tiring but I have to be there most of the time.
Once the diarrhea is over we can increase the anti-dystonia
medication which is working pretty good for his leg now. He is also
extremely iron deficient but the doctor said to wait for the diarrhea
to clear before we worry about that. Daisy was shocked and asked for
his latest blood results so she could give me a course of action. We
will still be in Fresno on the 13th and 14th Mom so please make sure
you can still make it. We really need to get that big hug from
you!!!!!! Now, if all this isn't enough, he has a really bad diaper
rash because I have had to use depends on him and mostly he passes
stomach acid and it just eats up the skin. I have been using Desitin
which is helping but yesterday in the doctors office he couldn't sit
still because his bottom hurt so bad. What is really sad is that it
is not "one thing after another" with Jeff's condition. It seems more
like "one thing ON TOP of another!!!" Danny and Betty will be here
this weekend so that will give Linda and I a break and we will have
company!!

Well that is about it for now. I am going to try to get a MediCal
person out to the house or at betty's when we are there so they can
explain how it works. I have to make sure I can get some kind of
supplemental money for taking care of Jeff and we can get local
doctors and ALL of the supplies he needs. If they can at least give
me a local doctor for his regular medical needs and possibly a
hospital bed and supplemental income for me taking care of him, etc.,
we could still use the HMO for his dietary needs and durable medical
supplies. They have been very good on all the small stuff. They just
won't supply anything big or any nursing or home health care as long
as he is with me. We would have to move to Fresno for that and that
AIN'T gonna happen!!!!! The big disadvantage of MediCal is that it
would cost him $700/month co pay because he gets over the maximum
allowed income from disability to qualify. In other words, if you are
disabled and make over $600/month, you have to pay Medical the
difference in order to get health care. IN OTHER WORDS...you are
expected to live on $600/month in California to qualify for medical
benefits!!!!!

I will keep you all posted if any good things happen but so far it is
just spiraling down into the abyss and we haven't seen any light yet.
We know it is supposed to be there and Jeff is STILL hanging in
there. He has lost all of his dignity (Betty and Linda have to help
me sometimes change him with his diarrhea which is REALLY BAD AND
MESSY), but he still keeps hope for his recovery as do I. I would
have given up so long ago from all the pain and I don't know how he
does it but I am still trying as hard as I can to stay positive for
him. I know they keep saying he will get better and it would really
be nice for some little sign just so Jeff and I can believe what
"they" say!!!!!

Love, Pat and Jeff and Linda

June 7
Jeff has lost a lot of weight in the last 2 weeks. I would say at least
15-20 pounds. He swallows from the very back of his throat now and
makes a clicking sound. His tongue is very far back in his mouth and
I cannot get him to push it forward any more. We are seeing the
speech pathologist next week and I think I may have to decide whether
or not to put him in a home. I am constantly rushing to his aid when
he chokes on his own phlegm and since he throws his head straight
back, he cannot take a breath or he would choke to death. Several
times he has turned blue and I have to bend him over quickly (not an
easy job in bed) and pound on his back. I am not qualified to keep
doing this and it really scares me. I think I am doing him more harm
than good. He is just NOT getting better. This is NOT how you
described most people get and NOT how most of the other patients
are/were doing when they got out of treatment. I think it has to be
simply the care I am giving him is incorrect. I do not push his
therapy when he hurts. I have not even tried to do anything while he
has had chronic diarrhea and now he has no strength left in his
limbs. I really feel I am doing more damage to him than he deserves.
Maybe in some type of home he will do better. The problem is that his
insurance will only cover skilled nursing and he doesn't qualify for
that. I will have to put him in a convalescent hospital (an old folks
home). That is the only place that would take him from my
understanding. To do that I will have to have MediCal insurance and
they will take $700/month out of his disability check to even begin
to cover it. That means I will have to go back to work and only see
him occasionally. This is NOT how it is supposed to be Daisy. If I
sound depressed I am VERY depressed. I thought we would make it
through this with a few bad turns but it has been downhill for the
last 2 months and not even a slight flicker of improvement. It has to
be the way I am taking care of him. I love him too much to really
push him and now he has lost everything. I really worry about a home
and whether they would even do anything with him other than keep him
clean and fed. They may not even get his zinc correct since the
hospital couldn't even get it right. I am just at my wits end and
emotionally shattered over this. I want him back the way he was and
it keeps looking like he will never be that way again. I can't lift
him by myself and I am injuring my muscles trying to do so. I can't
get anyone to come out to the house because we don't live in the HMO
area to get benefits. Would a home really be that bad for him? I'm so
afraid that they would only take care of him and do absolutely
nothing else. He is not ready for a skilled nursing facility yet so
that is my only choice. I just don't want him to end up there the
rest of his life. We want so much to come back next year with Jeff
walking in beside me but at this point in time he may not even be
able to come back next year.

HELP HELP HELP!!!!!!

June 9
This is a nightmare that just doesn't end. Jeff was getting a little better with the diarrhea, then it just went crazy again. Betty was here this weekend and he seemed to be doing a little better until she left tonight around 7. After that he started having diarrhea again and started throwing up. His neck is so stiff I know he had to inhale some of it which is extremely dangerous. Danny was still here with us. I was going to go to the store but Linda and Danny were both afraid to be left alone with Jeff. Finally, around 11pm Jeff started looking really bad. I asked him if he thought he needed to go to the hospital and he motioned "yes." I told him how he hated hospitals and that he probably really didn't need to go. I asked him again and he motioned "yes." I knew something had to be wrong. He was breathing really fast and hard and his heart rate was just pounding. I called the HMO help line and told the nurse of my concern about getting him to a hospital when they may decide it was not life-threatening. When I described his condition to her she told me to hang up and dial 911. I did and told Jeff the ambulance would be on its way. We were all scared but no one was as scared as Jeff. I knew he must have known something was really wrong with his body. When the paramedics got there, they had to move him from his new adjustable bed which he got to sleep in one night to the gurney. It was not really easy but they did it. They took him to Arroyo Grande Community Hospital where he was admitted to Emergency. The doctors and nurses there immediately went into action. Jeff was coughing up so much mucous and then blood it was horrible. He started vomiting and his heart rate soared to 150 and his respiration to 40 breaths per minute. It was so scary to see him like this. The doctors did some x rays and told us they thought he had double pneumonia. I could not believe this. Daisy warned us to make sure Jeff never got pneumonia but this was double pneumonia, the worst type! Oh my God, my baby is so sick. The doctors aren't sure if he is going to make it. Oh my God, what can I do? This is not happening. We stayed with him until 5am when they moved him to ICU and the nurse there was wonderful. She had to suction him almost every 5 minutes. She told us to go home and get some sleep. She would let us know if anything major happened. Linda, Danny and I were so tired yet so scared. I called Betty and told her what happened and she freaked. It was bad for all!

June 10
We went back to ICU this morning and Jeff was still having a lot of problems. The nurse on duty was really nice. She said they had a rough night but he made it through. I told her Jeff was tough and she agreed. Linda stayed out of work today to be with me and Jeff. Danny was here as well. He is going back on Wednesday so at least he can be with us a couple more days. Betty is coming on Wednesday to be here with us also. I noticed the suction canister by Jeff's bed and it was full of gunky, bloody stuff. The nurse said it was all coming from Jeff's lungs. The doctor came in and talked to me. His name was Dr. Weinstein. He was an extremely nice man and said he would be Jeff's doctor until Wednesday when another doctor would take his place. He confirmed the double pneumonia and said they almost lost Jeff last night. I talked to the case manager, Roxanne and we set up an appointment with the nutritionist and she helped me get in touch with a lady for MediCal. Roxanne told me that once Jeff was stable, the HMO wanted him moved back to Fresno. I said that could not happen. Roxanne told me I needed to get Jeff on MediCal ASAP then and maybe the lady could help. Dr. Feinstein assured me that he would NOT release Jeff until he was very stable and it was HIS call as long as Jeff was in ICU, NOT the HMO's! I felt some relief in that but knew I had to get MediCal in the next week. You normally have to get Social Security Disability first and that can take many tries but Roxanne said if I got a letter from the doctor, MediCal might be approved within a couple of days. I will go Tuesday to meet with a lady in San Luis Obispo and see what she can do. I spent the rest of the day filling out the forms the best of my ability and the hospital ordered a copy of all of Jeff's records so far. I went home and made copies of all the University of Michigan stuff I had and any of the HMO information I also had. I didn't stay in ICU with Jeff because the nurses were taking such good care of him I didn't want to interfere. I left with Linda around 9pm tonight.

June 14
Jeff is still in ICU and will be there throughout the weekend and
possibly next week. He really has double pneumonia BAD! The doctors
at this hospital are addressing lots of things however, like his leg
spasms, dysphasia, burning stomach and physical therapy. This is NOT
like his HMO that does absolutely nothing except supply medications
and durable medical goods. Jeff also was approved for Medi-Cal
yesterday which means he can now have doctors where we live and not
only at his HMO 165 miles away! They are already setting up in-home
physical therapy when he gets out of the hospital and a case worker
is going to come out to my house and evaluate how many hours/day I
take care of Jeff (basically 24/7) and pay me minimum wage to do so.
This should offset the $700/month share of cost for Medi-Cal he has
to pay because of his outrageously "high" disability payment per
month. That will leave him with $600/month to survive. What the hell
would he do if I wasn't around??? You can't live hardly anywhere for
$600/month and especially NOT in California!!! Anyway, the best news
is that when his HMO demands him back when he gets out of ICU I can
tell them where to go!!! I can't wait and either can Jeff!!!!!! :-)

June 25th

Dr. Brewer sent Jeff a letter today stating that the study between it
and Trientine has been a major success. Also, and I quote, "the
University of Michigan has recently licensed Tetrathiomolybdate to a
company (Attenuon LLC in San Diego) for commercial development.
Attenuon plans on getting the drug licensed for Wilson's disease as
soon as possible. They need to "audit" our patient records to help
get the records organized for an FDA audit, which will occur when we
put in a New Drug Application to the FDA for approval of the drug."

It looks as if TM is FINALLY going to be approved for use in
neurologically presenting WD patients! Many of you and Jeff were the
guinea pigs and hopefully this will enable doctors to get with it
and HAVE an option to treat this disease properly, of course only if
they can figure it out!. Just thought you all might like to know
this. some of you probably got the same letter. It is at least a step
in the right direction for the medical journals and formularies to be
updated concerning this more or less complication-free treatment of
WD.

Patrick

June 26th

Jeff has finally been released from the hospital after 2 1/2 weeks of hell! It was a better experience than the last one but still a lot of problems. Bottom line, hospitals suck! I guess we got spoiled in Michigan with 2 patients/nurse. This one had 6-7 patients/nurse and it was a critical care unit! He got home and now has a NEW problem. They didn't work very hard keeping his bottom very clean so now he has horrendous diaper rash type sores and some are even bloody. I thought I took good care of him but after seeing what the hospitals do, and I'm sure a lot of nursing homes, I feel pretty good about it. He is so sore he can't lay in bed on his butt. He had an air bed at the hospital so we never realized he had the pain. I went to the store to get one of those hemorrhoid pillows but it was not inflatable and $20. Instead I bought one of those kiddie inflatable donuts for swimming for $1.29!!! It works great! His right hand looks like he was in a serious fire from the blisters he got from the wrong tape they used on him. I finally had them put up a sign explaining the tape to use. His double pneumonia is basically in check but he has some "fluid on the lung" which is between the lung and some tissue by the muscle. If it gets bad the lung could collapse so we have to make sure he breathes heavily and coughs and takes his medication. Breathing heavily isn't hard since he gets so worked up over the pain his pulse rate shoots up to 145 and his respiration up to about 35 breaths/minute. That gets him close to hyperventilating. I am still glad he is home and the home health people are coming out tomorrow along with physical, speech and occupational therapy to evaluate him. He should be getting all 3 therapies 3 times/week each! Thanks for all your prayers so far...we still have a LONG way to go!!!

Pat and Jeff and Linda

July, 2002

July 1st

Jeff is supposed to start physical, occupational and speech therapy tomorrow. Unfortunately, he is really taking a turn for the worse. His pulse rate is constantly up to 125 and over and his respiration is around 25 to 35 breathes/minute. If he continues at this rate the therapists will NOT work with him. They would worry about hyperventilation and the possibility of brain damage due to lack of oxygen to the brain. I don't understand why now, after waiting all this time to start progressing, Jeff is going downhill which will prevent him from getting better. His mouth and throat are so dry now that his tongue gets stuck in the back of his throat and cuts off his airway which causes him to breath faster which further aggravates the problem. I am at my wits end. At this point I don't know whether to put Jeff in a home and just give up or what. If he can't get therapy they will tell Medi-Cal and Medi-Cal will tell me he needs to be put in a convalescent home since he cannot get any better. I have already been told this by the social worker. If I refuse, they will cut off his Medi-Cal benefits since I am not doing what they will cover. We are so close and now it seems to be so far away. I am about to give up! When Jeff is in pain now I just have to ignore him. There is nothing I can give him for pain. We have the strongest medication just below illegal narcotics now to give him and it doesn't really do anything. He sleeps for 2 hours then wakes up in pain for 4 hours. It is so draining on me psychologically and physically I just have to shut it out now. I am about to lose it all! I'm sorry this is so depressing to you all and I may not write too many more of these because I don't want to depress or anger any of you. We are all just living day to day now with not much hope for improvement at this point in time. I hope the therapists will still be willing to work with Jeff with his increased heart rate and breathing. They are our last hope but they won't endanger his life or brain and he is at the cutoff point right now before they even start to work with him........

Pat, Linda and Jeff


July 10th

Dear All,
I had just talked to SP Arora father of Rajesh.
HIS FATHER BROKE THE NEWS THAT RAJESH HAS EXPIRED AT 10 AM
HIS FATHER BITTERLY SPOKE "HE HAS NOT GIVEN HIS LIFE TO WILSONS DISEASE BUT TO FAKE MEDICINES AND THE UNSCRUPLOUS ELEMENTS MAKING LIFE SAVING MEDICINES AS FAKE."

HE PRAYED LET NO PATIENT DIES OF FAKE MEDICINES AND REQUESTS OTHERS TO PRAY.
It is really a bad news for the Wilson family. I saw him curved like a round ball, reduced to a thin body weighing 31 kg. His father brought him to my house three months ago. He was put on the back seat. I talked to his father for some time and we wanted to look at Rajesh we found that he was not on the backseat. We then heard a cry and we found him beneath the seat. He became so thin that he slipped from a gap of 5 inches. But he was talked to me with slurred and stammering speach. When I asked him How are you, he replied I am fine uncle and he replied my many questions.

He asked me
UNCLE WILL YOU GET ME CURED FROM GOOD DOCTORS and with good medicines? I HAD NO REPLY. I COULD NOT SPEAK FOR A MOMMENT THEN I TOLD HIM YES, RAJESH YES, I WILL.I COULD SEE LITTLE SMILE ON HIS FACE.
I would request all to pray for the family. His tel 91 1762 440722.Let us pray for the family. I am thankful to Dr.Sellner for his offered help/advice.
thanking you,
with kind regards.
July 11th 2002

(Devinder Singh)

July 18th

It looks like Jeff is finally improving! He has not needed suctioning as much anymore and his right foot is not so sensitive that he screams when you touch it. When I get him up he actually puts some weight on his legs so it is not so hard on my back. He also steps when I support him and move him toward a chair. His mouth is frozen open again and it doesn't close as good as it did last week. I have an appointment on July 30th in Fresno to maybe give him a Botox (botulism toxin) injection in his jaw to relieve the frozen mouth. However, it could backfire and make it worse and the 3 hour trip would be really hard on him so I don't know if we will attempt that or not. I added some brackets to my Camaro's passenger seat so now I can slide the seat all the way back to the back seat and Jeff can not only get in easier, he can also stretch his legs out fully when we go somewhere! His behind is still sore with a few bedsores from the long hospital stay and that is still worrisome. If any of those get infected he may end up back in the hospital so I am monitoring those closely and we have a home health nurse coming out several times a week to monitor them as well. The doctor showed up again last Sunday for 1 1/2 hours. It is so wonderful to have a doctor come to your house and then actually care about the patient. We go over a lot of details, the medications and how they are working, any things we need for Jeff, etc. He is wonderful!

One drawback, Jeff's mother and half-sister are going to be here tonight for up to a month. Martha and Kellie have had a rough time on their vacation and absolutely hate the heat so maybe they will go back to Kentucky once they have their fill of California. She should only be here for about 2 weeks max and then try to find a place to live. I will keep you all posted on that issue. The most important thing though is that Jeff is finally starting to turn around and feel better for a change. He even sleeps through the whole night again occasionally!!!!

July 19th

Here is a letter from Davinder Singh and a compilation of WD patients from the List Serve that responded to Davinder's request to show that Wilson's patients don't always die from the disease as the lawyers for the hospitals there are maintaining. I thought it was interesting.

Dear Members,
I have taken up the case of Rajesh one more youngman Hardamjit Singh who died in 98 with substandard medicine and the case of Bullet my son in the High Court. High Court has taken up the case suo motto on the basis of news items and is being heard. The lawyer who is not a doc has told the court that Wilson's Disease is a Congenital Disease and is Degenerative. The patient has to die within a few years even if treated since it is a degenerative i.e. he meant that Rajesh has died at 31 due to the nature of disease and has nothing to do with the treatment. I have told my lawyer that the

Hon'able Court
is being misinformed and the plea of the defending lawyer is not based on facts. I told my lawyer that it is a treatable disease and if right treatment is given then all symptoms of the disease goes and the patient lives a normal long life and most of the people has no difficulty in their profession due to Wilson's disease. l would request the members to give the information as per details given.
1.Name
2.At what age Wilson's Disease Diagnosed
3.Treatment years:
a) on Penicillamine
b) Trientine
c) Zinc
4.Profession
5.Present age
6.Sports if any
7.Any other information on the subject
I have decided to fight the case for all the sufferers and would request the members to kindly supply articles, book references. I am requesting Dr.Henry Sellner to send information/data needed in the matter to help me to fight. I shall be thankful to you.
thanking you,
with kind regards.
July 19th 2002

( Devinder Singh)

hi here is my information
name BELINDA DIGGLES
2-JUST BEFORE MY 41st BIRTHDAY
3-FOR THE FIRST 3 WEEKS PENN,THENTRIENTINE FOR 10 MONTHS,STARTED TAKING ZINC ACETATE WITH THIS FOR 7 MONTHS FOR THE LAST 4 WEEKS ONLY TAKING ZINC
4-I WORK IN A SUPPERMARKET DOING SOME HEAVY LIFTING.I WORK 24 HOURS A WEEK. I ALSO RUN A HOUSE AND HAVE 2 BOYS TO LOOK AFTER AND A HUSBAND.
5- AGE 41 BUT VERY SOON TO BE 42[2nd AUGUST]
6- POWER WALKING WHEN I GET TIME
7-FROM FIRST SING OF SOME THING BEING WRONG WITH ME, TO GOING BACK TO WORK WAS 6 MONTHS.I HAVE NEVER BEEN TOLD THAT I WOULD NOT BE ABLE TO WORK JUST THAT IT MIGHT TAKE TIME,AND WITH THE RIGHT DRUGS AND REST I WOULD BE ABLE TO HAVE A GOOD LIFE.
I HOPE THIS HELPS YOU
BELINDA


1.Name: Luella Gookins-Bechtold
2.At what age Wilson's Disease Diagnosed: Age 11
3.Treatment years: 8 years before transplant
a) on Penicillamine: 8 years
b) Trientine
c) Zinc
4.Profession: Home maker
5.Present age: 26
6.Sports if any
7.Any other information on the subject: I had a transplant at the age of 19. Since then I have been doing pretty well. ;o)

Hi Devinder,

Here is my info.

Name: George Feldmann(Canada)
Diagnosed: on my 19th birthday
Present age: 55
Treatment: Penicillamine for 20 years Galzin for past 16 years
Occupation: retired (I worked for Canada Packers---meat packing & slaughter house for 20 years as a labourer)---worked for the Federal Government as a clerk for 7 years---among a few other jobs
Sports: golfing, lots of walking-lots of volunteer work --

Devinder, Here is my info hope it helps

Name: Tammy Finocchiaro

Age diagnosed: 21

Treatment yrs:penn and triente (3 months on each)since 1999 been taking galzin

Profession: Homemaker and mom

Present Age: 33

Devinder,

My info:

Name: Rob Adlers(Canada)
Diagnosed: 23
Present age: 32
Treatment: Penicillamine for 7 years, Syprine and Zinc for almost 3 years.
Occupation: Laser Software Quality Assurance for Virtek Vision Intl., part-time musician
Sports: biking, skating, hiking, skiing
Other: I had to quit using Penicillamine because it had induced an auto-immune disorder which was harming me. Changing to Syprine also revealed that the Penicillamine was failing to remove all the stray copper in my body.

Name: Fran Corda-Whitehead
2.At what age Wilson's Disease Diagnosed: 8 y/o (year- 1968)
Projected age of demise without treatment based on rate of progression: 15 y/o
3
.Treatment years: 34
a) on Penicillamine (by MSD): 33 years
b) Trientine (by MSD): ~2months (from 9/02)
c) Zinc: ~8 months (from 11/02)
4.Profession: Professional Nurse
5.Present age: 42 y/o
6.Sports if any: adolescence - cheerleader, distance walking, calisthenics;
current: distance walking
7.Any other information on the subject:
* Initial clinical presentation -- hepato-lenticular.
* Timely + Proper treatment reversed
all symptoms, therefore
* Enabled to meet all life milestones (education, career, marriage, childbirth)
* Lifelong surveillance + treatment of disease process and collateral issues utilizing the advances of current research and medical resources are imperative to maintain optimum:
- cost-effectiveness
- health
- productivity
- quality of life
- fear / frustration reduction in patient / population secondary to impending
or current catastrophic illness.


1.Name: Ed Pei
2.At what age Wilson's Disease Diagnosed: approximately 9 years old
3.Treatment years: 26
a) on Penicillamine: 26
4.Profession: International Import/Exporter
5.Present age: 35
6.Sports if any: Softball/Baseball (since before I was 9 and continue to this day), Golf, American Football, Volleyball, Running and general exercises.

Good Luck, hope this information helps.

Hi I hope this helps

1. Valerie Rogers (USA)
2. onset of Wilson's Disease age 15
3. age now 35
yrs on Penicillamine 20
4. I work in retail. I am a Specialty Team Leader for Bread and Circus ( whole foods)
I work a 40+ hour work week on my feet.
5. present age 35

1. Name: Ellen Freedman
2. WD diagnosed at age 48.
3. Treatment years:
a) Penicillamine - 2 months
b) Trientine (Syprine) - 4 months
c) Zinc gluconate (health foods store) - 9 months
d) Galzin (zinc acetate) - 11 years to present
4. Profession: educator
5. Present age: I will be 60 Sept. 24th
6. Sports: walking
7. I had liver failure after gall bladder surgery in 1990. Luckily I was diagnosed while hospitalized in about ten days. The ophthalmologist saw KF rings. Then the liver biopsy and other tests confirmed WD. I was on leave from work for six months until I got my strength back. I have been working full time since then. Currently I teach 9th grade algebra.

Name: Pam Simington
Diagnosed: Age 32 (2001)
Present age: Age 33
Treatment: Originally, Galzin. Currently: Nature's Plus Zinc (over the counter preparation)
Occupation: Home schooling mother/teacher, Nursing Student, Certified Medical Assistant
Sports: Karate, Rappelling, Hiking, Water sports, Target Practice (Firearms) Volleyball ---all as time allows!

1.Name
Bethli
2.At what age Wilson's Disease Diagnosed
Aged 8

3.Treatment years:
a) on Penicillamine
From November 1974 to March 1985.
From December 1985 to August 2000.

b) Trientine
Never

c) Zinc
From March 1985 to December 1985.

4.Profession
B.A. in Art History and German Language and Literature
Post-Graduate Diploma of Librarianship
Graduate Diploma of Business (Information Systems)
Currently working in Knowledge Management

5.Present age
35

6.Sports if any
Never been any good at sports, but cycled to school for about 8 years. Wear glasses and found that handicapped me when I attempted to play tennis. Currently learning to sail, as live in sailing capital of the world and home of the America's Cup.

7.Any other information on the subject
Spleen removed in January 1987.
Liver Transplant in August 2000.

Am going to GERMANY in SEVEN WEEKS!!! First holiday since the liver transplant and very excited. Currently getting letters about the drugs, and leaving all contact addresses and phone numbers with the Liver Unit

1. Richard Higashi
2. 21 at years old pre-symptomatic diagnosed by KF ring and blood test only, no
liver Bx.
3. 1965 at 23 years neurological symptom.
a) Penicillamine Merck Sharp & Dohme NDC 602 (250 mg 4TD) for 26 years
1965-1991.
b) Never on Trientine.
c) Zinc Sulfate Upsher-Smith NDC 080 (220 mg 3TD) for 7 years 1991-1998.
d) Zinc Acetate Gate NDC 208 (50 mg 3TD, now 2TD) 1998-Present
4. Consulting electrical engineer for acute care hospitals & diagnostic facilities.
5. 60.
6. No time for sports. Take a short walk when I am not tire from stressful work.
7. I am Wilson's survivor also a Cancer survivor.

Devinder,
Here is my info...
1.Name Michelle Cohen
2.At what age Wilson's Disease Diagnosed 21
3.Treatment years: 11
a) on Penicillamine no
b) Trientine previously
c) Zinc am on now.
4.Profession Substitute teacher
5.Present age 33
6.Sports if any walking daily
7.Any other information on the subject zinc works well for me but keeps me tired. I take Tylenol ONLY for any pain.
+++++++++++++++++++++++++++++++++++++++++++++++++
Me=^.".^=oW
"A House Is not a HOME without a CAT."

Here you go. I hope this helps

1. James Esler
2. Diagnosed at 19
3.a) On Penicillamine 16 years
b) On Trientine 7 months
c) Never on Zinc
4. Not at liberty to say.
5. 36
6. Running and working out.

Devinder,
I am very sorry to hear about your loss. I hope it helps to know how many of us care.
This is the information you requested.

1.Name : Laura Lokken
2.At what age Wilson's Disease Diagnosed : 19
3.Treatment years: 21
a) on Penicillamine : 7
b) Trientine : none
c) Zinc : 15 (I was taking zinc and Penicillamine together for awhile.)
4.Profession : lab technician
5.Present age : 40
6.Sports if any : Scuba diving
7.Any other information on the subject : My doctor was misinformed. He told me it was all right to take zinc with food. After doing this for about 13 years, I was found to have major liver damage from a build up of copper in my system. I am currently back on track, taking Galzin 3 times a day on an empty stomach.

Name: Judy Bowen (North Carolina) Diagnosed: at age 29,neurological &
psychiatric symptons,KF rings present at time of diagnoses, blood work & 24
hour copper output to confirm, no liver biopsy--treatment 1,500
mg.Penicillamine for 29 years, body could no longer tolerate
Penicillamine---Galzin Zinc Acetate (4) months, Zinc Gluconate (10)
months--no medication at present because all my test are very low, Copper
very low--keeping close watch monthly, doing great, as soon as readings come
up will start back on Zinc. Retired--Certified Dental Assistant, fill in
occasionally when needed--volunteer work in schools etc.( volunteer of the
year 1987 high school) one son age 28,my age
59.Sports---golfing,bowling,Duke Basketball,tennis,walking & excersing, I try
walking at least 20-40 minutes a day, love sports but my favorite thing is
dancing. It has been a hard road to travel dealing with WD but I am indeed a
Wilson's Disease survivor.

Patricia Hanson
Diagnosed at age 33
I've been on treatment for 44 years
On Penicillamine for 30 years
Trientine for 14 years (still on it)
Zinc for one month (2-3 years ago) It did absolutely nothing for me
Credit Manager for a lumber company
I am 77 years YOUNG
Watching baseball games

Hope this helps. It DOES prove that with
right medication (not that fake stuff) we
Wilsonians' do survive


Here is my info:
1.Samantha Bentley
2.Aged 37 at time of diagnosis
3.Penicillamine for 2 years
4.Desktop theme and web designer
5.Current age:39
6.Do not play sports like to watch soccer. Enjoy reading, working on my PC, movies
7.Severe neurological symptoms eg.tremors,extremely slurred speech, inability to focus my eyes on writing, lack of balance and coordination, extreme tiredness. Most of the symptoms gone after 1 year on Penicillamine.

Hope this helps
Sam

July 29th

I haven't written for a while. Jeff is getting lots of in-home physical, occupational and speech therapy. It isn't helping a whole lot. He is still very stiff and bedridden. I notice when I get him up to a chair he can at least push with his legs a little bit. I think he is depressed about the whole thing. He stayed so up and cheerful for so many months with so many obstacles thrown at him. I think he just finally sort of gave up. The therapists work with him and say he does pretty good, except for speech. His mouth is frozen wide open again (it looks just like this little guy- -> ) and he can close it just a little once in a while. We are leaving today for Fresno where his HMO is for a neurology appointment tomorrow. The doctor is going to evaluate Jeff for Botox injections. I hope he qualifies and the doctor gives him the injections and, most importantly, they work!!! His mouth and throat are SO raw from being open for the last 3 months that it is just awful. Also, because of the cirrhosis, his gums bleed a lot so he has a lot of dried blood behind his teeth constantly and it is very hard to clean when you can't really get in there with a toothbrush and water. The toothbrush make his gums bleed horribly and the water chokes him! We are still aiming at getting him into intensive rehab by the end of August. Typical for his HMO though, the doctor claims he can't see Jeff until the end of September. This is what happened before when he left Ann Arbor. The doctor told us in April that he couldn't see Jeff until June and look what happened to Jeff in those 2 months! The only other solution is to get him into rehab here locally but I will have to pay $810/month share of cost to get him in. Since I am living on Jeff's disability check (which runs out in January) and my wife's meager salary, it would be impossible. I am getting some supplemental money from the state for taking care of Jeff but the catch is when he goes into rehab, I WON'T be taking care of him so I lose the money but still have to pay the $810/month!!!! Does this nightmare ever end??? In addition to all of this, his mother (some of you read the posts about her when we were in Michigan) is HERE and planning to move within 3 miles of our house!! My wife REALLY likes that idea!

The really good news is that Dr. Brewer's office called and told us that Jeff's 24 hour urine copper level is down to 50!!! His zinc level is over 350 so that is good I guess. I think we are going to do another MRI on his brain to see if the damage went outside the basal ganglia area because of some of the ways he acts and reacts. He has trouble opening his eyes now and looks like he is drugged when he does. They dart around a lot and when he stares he doesn't seem to be focusing on any one particular thing. Just a thought!

I'll let all of you know how the Botox went. I have heard that it takes a couple of days to a week to work so we'll see......

Patrick and Jeff

July 30th

We traveled to Fresno yesterday for the Botox injection this morning. It turns out that the head of Neurology is quite a nice guy. He just evaluated Jeff and told me to come back in a week or so and I said "no way!" I told him of our long journey here so he told us to come back this afternoon and he would give Jeff an injection for the mouth and even one for the neck. It went very well. He stuck a needle probe in the muscle and then put an injection in. That really had to hurt but Jeff took it as usual like he does. I just don't know how he can withstand all of that pain all the time and then get more thrown at him without just giving up or freaking out. He is such a strong person. I never knew my son was that strong until the ordeal with this disease. I hurt so badly for him and yet I admire him so much because he gives me strength in addition to his own. We went back home without too many problems. He was quiet and did fine. We have a rehab appointment for August 8th to see if he can get in. I hope the doctor will agree to that. Jeff really wants to get into rehab so he can walk again. He is still afraid of the Fresno rehab because it uses doctors from the HMO for the HMO's patients. Betty told me that Jeff told her that HMO would kill him someday. I wish I could put him the rehab hospital over here but the lady there told me MediCal would only pay for about 2 weeks and NOT if he was getting in home therapy so that is out. I told Jeff that this rehab hospital is supposed to be one of the best in California and he could stay up to 100 days if he needed to and he would probably walk out of there. He seemed to like that idea.

August, 2002

August 9th
I was hoping to say that this would be the final solution to Jeff's problems. He is now in rehab but I had to fight the HMO doctor to get him there. The doctor said Jeff was not even close to being eligible. I told him Jeff was and to have a person from the rehab hospital come and evaluate him. She did and said he would do fine there. The doctor reluctantly admitted him. Everyone there is really great, but the major problem is that instead of turning Jeff over to the doctors at the rehab hospital, the HMO doctor is still in charge of Jeff's future at the hospital. The nurses there said that is how the HMO does it. Jeff is their youngest patient and nobody there has ever seen a Wilson's patient, much less heard of it. I explained in detail (as is customary) what Wilson's is and how it affects the patient to every person in that hospital. I wrote a 2 page letter to all with Jeff's history and prognosis so all could read it. I also explained that I must be notified if anything different were to occur like changing his meds, etc.

The first day was great. I stayed with Jeff that night to acclimate him to his surroundings but told him I would not stay every night because I wanted him to get used to relying on other people as well, and I also trusted the staff completely. The next day he had diarrhea 5 times! The older CNA there told me this is common with new patients because of their nervousness. I did not think that was it so I observed everything they did. When dinnertime came the nurse did a bolus feeding where she "dumped" 800ml of his ice-cold formula into an open syringe in less than 5 minutes. I asked her how long they had been doing that and she said "since yesterday." I immediately went to the head nurse manager who oversees the entire operation and told her Jeff has always had a gravity bag feeding of 'warmed' formula over a 30 minute period and he was supposed to be fed that way. She jumped out of her chair and stated "that's it!" She immediately ordered warmed gravity bag feedings from then on. The next thing I observed was the nurse mixing Jeff's zinc with tap water. I also observed the hospital had copper pipes. I told the nurse that Jeff should ONLY get bottled water for everything, especially the zinc. He readily agreed and put a note up for all the other nurses. They are really very efficient.

The next morning when I arrived they told me Jeff had a bad night. He had vomited several times after his last feeding and that later that night he had the worst diarrhea she had ever seen twice. When I said good morning to Jeff I noticed his eyes. All I could see is the whites! He could not focus on anything. A while later the nurse came in with some medication which was NOT on his regular schedule so I asked him what it was. He told me it was some powerful medication to prevent nausea. When I looked it up on my PDA (I have the same programs the doctors use and I HAVE to have it for obvious reasons) it said it's most common side effects were chronic diarrhea and drowsiness. I immediately went to the nurse manager and told her what I had observed and why was he given a new medication without my authorization. She said the doctor thought it would help with the nausea. I asked her specifically when Jeff vomited and she said the nurse said it was while she was suctioning him. I told the nurse manager to think about it. If you had a large wand stuck down your throat just after you ate wouldn't you throw up? She agreed. I told her not only was Jeff's nausea caused by a wand stuck down his throat, but now he had really chronic diarrhea caused by the new medication. If you want to really depress an already extremely scared and depress 25 year old young man with constant visions of a very gloomy future, let's just keep this up! She was very upset and ordered the medication stopped immediately.

Yesterday morning when I got there, his eyes were open and he had NOT had diarrhea or nausea the night before and actually got some sleep. He had now had a total of 15 hours sleep over a 4 day period. I left yesterday afternoon for a couple of days. My wife just started a new job and is stressing over it so I told Jeff he was in good hands and I'd be back in a couple of days. Our family friend Betty who has been through all of this with us will be there several times a day for him while I'm gone. It was hard to leave and a tearful event but I wanted him to understand that he has to get used to trusting other people as well. Today Betty went by the hospital before work to check on him. His eyes were rolled up in the sockets again. She asked what had changed and they told her since his legs were hurting really bad from the therapy, they gave him some OxyIR which is the strongest medication you can get below morphine or illegal drugs. I have given him Roxycodone occasionally for pain so I thought it to be ok since I had authorized it. Betty told me they have been giving it to him every 6 hours. I asked if he was in pain the whole time and she said "no, they just give it to him every 6 hours!" Then his HMO doctor came in and saw his eyes and said "I think he should be taken back to that "other" hospital where his father lives." This is NOT even his regular hospital, it was just the one he was taken to for his double pneumonia emergency stay. Worse yet, he said it right in FRONT of Jeff!!

I know some of you have experienced this kind of horror in the past but this is pretty much the LAST chance Jeff has and his crappy cheap HMO is already trying to boot him out. Without this acute rehab there will not be much hope for Jeff. Medical has already indicated that his in-home therapy is coming to an end and they WON'T cover him in rehab since he is getting in-home care. THIS NIGHTMARE NEVER ENDS!!!!!!!!! Jeff is only a few months away from regaining his strength and walking again. His mouth is starting to close up a little again. He has hope. Then it all gets shattered by a cheap HMO. I have never been sue crazy but I'll tell you now, if they hurt my boy any more I will sue until hell freezes over. They just don't seem to care that he has a good 50 or more years left of his life and to spend it in a vegetative state is NOT what he or I ever intended! IT IS NOT FAIR IT IS NOT FAIR IT IS NOT FAIR!!!!!!! I am about to crack people! I can't watch my baby wither away like this with no hope when it is totally NOT necessary. Please, I need your support more than ever now!!!!!

Love, Patrick and Jeff


August 16th
New development...The therapists and nurses all stuck by me earlier this week and outvoted the doctor to have Jeff stay another week. The nurse manager said none of them had ever seen a Wilson's patient in their careers, they had only read about it in medical school. The therapists are taking Jeff on as a personal challenge and the nurses are welcoming my input and expertise! This place is rated number 16 in the entire country for an acute rehab hospital and they consider Jeff and Wilson's disease another notch on their belts of experience. They are all taking wonderful care of him. Yesterday the main physical therapist was amazed that Jeff stood on his own (holding on to a table) without anyone's help. The first of the week he was telling me his hopes for Jeff were to make it easier for me to transfer him to and from bed. They thanked me immensely for the letter I wrote explaining Wilson's disease, Jeff's history and the experience with doctors. The only problem I have is still with the HMO doctor. Jeff now has a new problem. He can't open his eyelids by himself and when you help him, his eyeballs are sort of "frozen" looking straight up as if he was drugged out of his gourd! Nobody is really sure what it is. I explained that mysterious things can happen during the recovery period. The doctor told me he talked to Jeff's neurologist and told me that all the family can do is be supportive of him in the future with this grave problem. I told him it would pass and more than likely next week Jeff's ears would start to uncontrollably wiggle!!! He didn't think that was funny and left but everyone else laughed! Oh, Jeff's mouth is finally starting to close again. I rubbed the roof of his mouth with a sponge swab and he clamped down on it with his lips. It looked like he was sucking on a sucker! I just hope he can stay there for his full 2 months. I think they will do wonders with him!!!!

Patrick and Jeff

August 21st

He's still there and hopefully will be for another couple of weeks. They seem to like trying to help him. I pissed the HMO doctor off really bad today though. His idiot neurologist told the doctor that Jeff's frozen eye problem (his eyeballs are stuck in the top of the socket syndrome) are just a natural progression of Wilson's disease and the family must be supportive. He did NOT even come to look at Jeff. I called Dr. Brewer and he told me it sounded like Jeff was drugged out of his gourd. When I told him what medications Jeff was on he told me to tell the doctor to stop them NOW!!! That made the doctor mad thinking I was bypassing Jeff's "idiot" neurologist. Well I was!!!! I smoothed things over though and all should be ok by tomorrow. Hopefully the med reduction will start tomorrow. Betty will let me know. I stay for a couple of days then come back here to visit Linda then go again. This Friday Linda and I are going back for the weekend. Then I will bring her back Sunday and leave again that night to be there Monday until probably Thursday. Betty stays with Jeff through the weekend. It is tough but it is his last chance for recovery. He was approved today for Social Security disability and it was the first time! That is almost unheard of. They usually reject it 3 times before it is approved. His monthly payments now will be $500/month then increase to $950/month when his state disability runs out. However, MediCal will eat most of that anyway because he is only allowed $700/month to be on MediCal and they will take the rest away from him to get any benefits. We are all still holding up here. Hopefully you are too. Once Jeff is out of rehab they are going to schedule him for outpatient care 3 times/week in 3 week blocks so we may meet up in Fresno in September even if he is out.


Love, Pat

August 22nd

Email to Lisa and Reed Hughes after seeing the video of Reed one year after going to Michigan and getting the therapy records: Wow! This was such an inspiration! To see Reed at his one-year follow-up talking and walking! Jeff will really appreciate seeing this. I am going to show it to the therapists Saturday and show them all the records. They are both really interested in this disease. A little note of contention as always...I talked to Dr. Brewer and told him how Jeff looked and that he had lost 60 pounds in 2 months and his eyes were rolled in the back of his head. He looks very drugged. I told him the neurologist would not come to see him in the rehab center. Instead, he told us that the eye problem was just a natural progression of Wilson's disease and the family would just have to be supportive! Dr. Brewer really chuckled over that one. He asked me what medications Jeff was on and when I told him, he said "that is why Jeff's eyes are rolled back in his head!" He is getting the strongest dose of codeine legally available, a huge amount of Neurontin and klonopin. These combined create a heavily sedated effect. I told the doctor that Daisy (Jeff's physician's assistant in Ann Arbor) was "appalled" about the medication Jeff was on. This infuriated the doctor at the rehab center so I told him what an idiot I thought the neurologist was since he didn't even look at Jeff to determine if he was getting too much medication. Since they both work for the HMO, I guess it really offended the physical doctor. I tried to smooth things over but the bottom line is that they are now reducing the Neurontin and klonopin and stopped the codeine. Hopefully in a week or so Jeff will start coming back and be able to participate a little more in his therapy. I am sure this video and the records will encourage the therapists and hopefully (but doubtfully) even the doctors! Do you need these records or the videotape back????


Thanks, and we hope to meet both of you someday....Patrick and Jeff

August 27th

He will hopefully get to stay another couple of weeks. He has had a very bad bout with diarrhea the last couple of days and his congestion seems worse. But!!! He can stand on his legs with help and today I saw him walk along the parallel bars with his body totally straight. He was standing almost all of his 6'4"!!! He also is getting looser in the arms and his mouth formed an "o" yesterday and he said "oh." I just hope the problem with his eyes freezing in the top of the sockets will fix itself soon so he can see again.... Thanks for your concern... I'm glad to hear that Tom is about to go for his experimental treatment. Interesting enough, Jeff's experimental drug which has finally been FDA approved was called TM for short!

Pat

August 28th

Jeff has been in rehab now for about 3 weeks. Tomorrow he goes to the hospital to get a new peg tube put in his stomach. The old one is 6 months old and getting a little loose. Yesterday he got up on the parallel bars from his wheelchair by himself and with help from the physical therapist, walked from one end to the other! This morning he got to the edge of the bed by himself and was helped very little getting into his wheelchair. He also put his sandals on. His mouth is almost closed now, however it still opens wide up quite a lot, but he can get it pretty close to closing by himself. With help he can close it all the way! He made several "O" sounds the last couple of days. We still don't know what type of coverage he has. Betty says he has unlimited rehab according to the plan specialists. However another plan specialist called her today and said he only has 60 days total of in and out patient rehab. We will hopefully find out Friday. If he can get at least 2 months of intensive rehab in the hospital and then get lots of out-patient visits he should be able to walk by the end of the year. The therapists are all amazed at Jeff's progression, especially since they initially thought he would never get much farther than helping me slightly getting him into his wheelchair from bed. They are getting really scientific and using all of their training to get him to balance in bed while sitting and standing without falling over. It is really interesting to watch, especially when Jeff does it and they get all wide-eyed! He is off all of his medications now that made him druggy. Dr. Brewer in Michigan told me that Jeff was getting way too much and should be taken off all of them now. I had a fight with the doctor but I won of course and now Jeff is really starting to "wake up." He has a new problem now with his eyeballs being stuck in the top of the sockets and you have to open his eyelids for him. Hopefully this is just a passing thing and he will be showing his baby blues again soon. We were going to try to schedule his 1 year follow-up to coincide with the convention next year but now I'm not sure. I read through a post that it has been moved to Washington DC. We have never been to New York or Washington DC so I guess it wouldn't matter were we go but it can't be too rough on Jeff. Any comments on this? I heard DC is NOT the safest place to go anymore but New York is also so HUGE!! Los Angeles is big, smoggy and very overcrowded but at least I know it and we could drive there. New York or DC are very far away and we would all be pretty nervous going that far, but we'd love to go to the convention.

Pat and Jeff
August 30th

My beautiful blue-eyed baby Jeff has died!
Jeff died this morning at 5am. He was doing really great but in the
> last couple of days he was sweating and breathing fast and his hands
> and feet were ice cold. I got in late last night and saw that he was
> NOT in good shape. An ambulance came to take him to emergency. The
> nurses in the rehab hospital said he had a 101.7 fever and cold
> hands. The paramedics looked at him and said he was in severe shock.
> They got him into the ambulance and I followed them down in the
> elevator holding his hand the whole time. He looked up at me and
> squeezed my hand. I told him everything would be ok, it was just to
> find out why he was so cold and out of breath and I loved him very
> much. I left for the emergency room and as soon as the ambulance
> driver closed his door, Jeff stopped breathing. They did CPR on him
> and took him to a different "closer" hospital. I found out 20 minutes
> later. When I got there, they would not let me in his room. The
> doctor finally came in and told me that as soon as he got there, his
> pulse was 180 and then it just dropped. They hit him 5 times with the
> electrodes and brought him back. They told me his prognosis was very
> grim. I though, yeah, right, this is Jeff they are talking about. I
> went to see him and told him he has always been a trooper and this
> was no exception. They said they were going to move him to CCU and to
> get ready. I hugged him and told him I loved him more than anything
> else in this world including life itself and he would pull through as
> always. Then his pulse went up to 200 and suddenly dropped. They
> tried their best but just couldn't bring him back. I screamed at him
> to come back and pounded on his chest and blew in his mouth but it
> was to no avail. He was looking up at me with his baby blues and his
> mouth open, dead! I am devastated beyond words. I don't think I can
> go on in life without my little "Pooty." He was the world to me and
> without him my life is nothing. We had so many good times planned
> ahead for the future. I even bought him a brand new PT dream cruiser
> for when he started driving again. The doctors said he had a clot
> probably in his leg that moved up to his lungs and caused a massive
> coronary or something like that. He was at his HMO hospital earlier
> that morning to get his peg-tube replaced and had an ULTRASOUND for
> blood clots but they say they found nothing. He was like this for 2
> days and when I got there last night it was too late. Now I found out
> that his HMO is refusing for an autopsy to be done on Jeff. They say
> he died of complications from Wilson's Disease! They are covering
> their own butts. I am trying to get Dr. Brewer to make them do one
> because of the FDA study. I don't even now the official cause of
> death. We are going to have him cremated and scatter his ashes in
> Mosquito Creek where he loved to camp and at the beach where we lived. We
> will NOT get to see you guys this spring. I am sorry, Jeff really was
> getting better and happy about it and wanted to walk across that
> stage so badly he busted his butt to get there. This is what probably
> killed him. The long months of laying in bed with pneumonia and other
> problems probably caused blood clots. Then his eagerness to
> participate in his therapy broke some clots loose and they entered
> his lungs. That is what one doctor said.
>
> Jeff was going to write a book about his experiences and when he
> could talk, was going to dictate to me his thoughts for the last
> year. We were going to name the book "Wilson's Disease: A father and
> son's journey through hell and back again." What do I name it now????
> Love, Pat and Jeff always in our prayers and dreams......

September, 2002

September 1st

Jeff's funeral will be on Saturday, September 7th at 10:30 am. For those of you who would like to send flowers, the address is:

Chapel of the Light

1620 W. Belmont Avenue
Fresno, CA 93728


thanks to all of you who sent such beautiful condolences. We got the card from you and Russ today Lenore. It was very funny. Jeff would have loved it! I came home for the first time today and had to see all of Jeff's things. It was torturous! I will try to write the book that Jeff and I were going to do when he was capable of talking again. I will keep his legacy of perseverance and hope alive. He NEVER gave up. Even to the very end the doctors said they'd never seen anyone fight so hard to stay alive. It was just too great a strain on his already love-filled heart. I have already seen him in the sunset smiling at me!!

Patrick

September 2nd

Letter from Gayla
Pat,

I was relieved to hear that you were with Jeff. Until you wrote, I didn't know if you were there at that time or not. You were there to comfort him even though you were going through such pain. That is the worst thing that can happen to any parent and it is even harder because you and Jeff were so close. I do think that it would be a good idea to write the book when you can. I think Jeff would have wanted that and it will help so many people to understand what an awful disease it is. It also could quite possibly save many lives. I cried again when I read your e-mail. It hurts to think of all that you are going through and will continue to go through. I am so sorry.

I talked to Mike Jr. about Jeff when we were at their house and he had said what a great guy his cousin was. You could hear in his voice how much he loved Jeff. I got to know Jeff through your e-mails, our ICQ chats and visiting with Mike Jr. I loved him and was looking forward to meeting him.

You have been through so much Pat and will be going through so much more in the days to come. I know it is probably very difficult to sleep but please try to get some rest. I wish that I could attend the service to be there for you. Know that my thoughts and prayers are with you and will continue to be with you always. You were a wonderful, loving, and caring father, mother and best friend to Jeff and you were part of the reason he was such a courageous young man. He had an excellent role model all of his life. You learned many things from Jeff also by being there for him always, sharing things together, etc. I want to think that he is watching over you now.

I know that words do not help much at a time like this, but again I am so sorry. We love you.

Love,

Gayla


September 3rd


A letter from Robert, Deb and Marrita Jenkins (Marita who was in Ann Arbor with Jeff)
Pat:
I just read my e-mails this morning. May god bless you and guide you through this most difficult time.:
You loss is our loss, my heart mind and soul grieves. I went to school and gave Marrita a long strong
hug and told her it was from Jeff. When we got home I let her know Jeff had passed away. Her reply was He's
with Grandpa now, I will miss him. Tears rolled down her cheeks as she drifted off to sleep.

I then read your e-mail sent on the 28th. What you described about Jeff is almost the same process Marrita is going through, the eye locking started Aug 17th and we just got it under control using Benadryl.

Pat Jeff death should be investigated. When we took Marrita off her meds it was a gradual process that took weeks.

Pat I am angry about Jeff's death I am also deeply sadden by is departure. Please e-mail your phone # to me.

God bless you and may he bring peace to your soul.

Love...Robert, Deb, Jennol and Marrita
Mary Draper suggested that instead of Wilson's list members sending flowers, they make a donation to the Wilson's Disease Association. I think it is a wonderful and necessary idea Mary. I just thought people on the list would do that anyway so I didn't suggest it. I will print and hand out a lot of those Saturday. So far, there are going to be over 200 people attending his funeral at last count. I am so happy he had so many friends and many have not seen each other for years. Yesterday, his best friend over here finally came over. He and Jeff witnessed their best friend drown many years ago and it devastated them both. Jeff told Dan that Skye was at peace and happy now but Dan couldn't deal with it. When Dan found out Jeff was sick, he wouldn't visit for fear of what happened to his other friend. Jeff told me that it was ok and he understood why Dan was that way. I had no way of contacting Jeff's old friends here as they all had moved away. I told Dan I had to see him urgently. His dad told him the night before that Jeff had passed away. Dan was coming over and stopped at a local 7/11 the gang used to hang out at back in the 90's. Amazingly, every one of Jeff's old friends were there. They were seeing several off on an airplane and decided to meet there. Some had come 800 miles for the send off. Yesterday afternoon, a whole caravan of cars came to my house and all of Jeff's friends were there! I know Jeff had a hand in getting them all back together for me. They are all coming to his funeral Saturday. It was a very tearful reunion but very joyful as well because they all talked of many happy times when Jeff helped them out of a jam or was just being his beautiful fun self. I was told many wonderful stories about Jeff. When I am ready to spread his ashes on the beach they are all going to be there one last time for Jeff and we will all do it together. He would love that!

The autopsy is today and I am going to find out exactly what happened. I do believe Jeff was short-changed because of ignorance. The doctors involved all continued to maintain that every little thing that happened to Jeff was because of Wilson's Disease and it is NOT TRUE!!! I am extremely bitter and if Jeff's death could have been prevented because of this ignorance then I owe it to him to pursue it like a tiger and I will! In his last hours as he was slowly dying, I trusted the nurses and they kept calling the doctor who kept saying to just monitor him. When the doctor did finally call the paramedics 3 1/2 hours later, it was too late. I watched him die and had no idea even though all the signs of extreme shock were present. I am not a nurse or doctor however and did not know the signs. I did know that he should not have blue hands and feet and under his eyes and blood as thick as syrup however, but still I trusted their opinions. I truly believe that if I had pushed my gut instinct, Jeff may still be alive today. All I am saying is that if it comes out that the medical profession attributes every little ache and pain to Wilson's because of their ignorance, they are putting another life at risk and my son's suffering will hopefully bring some happiness to another family who won't have to go through the unbearable loss I am feeling right now. All of you new people to the list: always watch your loved ones, especially when in the presence of a doctor or nurse. If they don't look or feel right to you, demand attention immediately. Also, and most importantly, never ASSUME the doctor or nurses are doing what you ask them to do. ASK EVERY SINGLE DAY IF ANY MEDICATIONS HAVE CHANGED!!!!! I told Jeff's doctor I wanted back on the medication for his stomach burning Tuesday morning before I left for a couple of days. He agreed and as soon as I left, he cancelled the order. For the next 3 days until I found out Thursday night, Jeff was suffering horribly from a burning stomach. When I last saw him, he let me know that it hurt very badly. If he could cry, he would have. I was furious that the doctor ignored my demand. When he had his peg tube replaced earlier that morning the GI doctor ordered Zantac for Jeff's burning stomach. The doctor (who is only a physiatrist -physical doctor) cancelled that order as well. If you consider that Jeff had virtually no copper in his diet from his formula and was taking 3 Galzin's a day which can cause a lot of stomach distress, and then for this doctor to not give him any medication to relieve the horrible burning pain he had because it was, and I quote, just the "natural course of Wilson's Disease and the family will have to be supportive" is unimaginable to me. I am saying this to the group because you have all followed our journey through this nightmare health system he had and the first doctor he ever saw if any of you remember told him he was going to die and would never get any better. Because of their ignorance, his prophecy came true at the expense of my beautiful son. Jeff knew he would beat it and prove them all wrong and he did! His last 2 days proved that when he stood tall and proud for the first time. I am just so thankful to God that I witnessed that and told him how much I loved him and now we were over the hump and on the road home. Jeff's strength and courage must live on in the hearts of all the people out there with this disease and the loved ones who take care of them. He never refused to do anything that wouldn't make him better and he NEVER EVER stopped thinking he was not going to recover. Jeff KNEW he would get better and he proved it to the world!

God bless you all for your support in this most unbearable time of my life.

Love, Patrick

September 4th

Message to Anna Fiasca from the Wilson's List: Anna, treasure every moment with your daughter and make sure you never assume the doctors are doing what you think they are doing. I just lost my son Jeff Friday and your daughter sounds so much like him. He was a fighter to the end and proved that he could beat it. Unfortunately, the doctors he had had a different opinion and in the end he died from lack of care. I was with him when he died. It was the saddest day of my life. At least Jeff got to experience standing tall to all of his 6'4" again and was able to say the word "no" when the nurses asked him to say something (he had not been able to talk or eat or close his mouth for 4 months). It sounds like your daughter's doctor is really fantastic and really cares for her. Just always keep on top of her medications and treatments, especially if she has to go into a hospital. The general consensus amongst doctors in most hospitals it seems is that Wilson's will kill you and all they can do is make the person comfortable. Jeff never believed that and that will be his legacy to the group and the world. If your daughter is treated and the copper can be pulled out in time without complete failure, there is a chance for a fairly normal life. If she needs a transplant, at least she will no longer have Wilson's disease but she would have to deal with the anti-rejection medications the rest of her life. There are quite a few liver transplant survivors on the list who can help you and her through it. I know that Jeff will be watching over everyone with this disease now because he always cared so much for everyone. Jeff was 25 when he died with his whole future in front of him. He was ready to get back onto the walk of life and he knew he was doing it that last day. Now he will walk that walk with other Wilson's patients wherever they are and whenever they need him. That is just the way he was and still is. Tell your daughter she is NOT alone in this fight. She has a guardian angel looking over her shoulder!!!

Patrick Dirlam

September 5th



I don't know if anyone is interested but Jeff's obituary posted online today in the Fresno Bee. It will be in the San Luis Obispo Tribune tomorrow and probably online as well. The Bee has a nice picture of him. I don't know if the direct link will work...this is it: http://www.legacy.com/fresnobee/LegacySubPage2.asp?Page=LifeStory&PersonId=475894 if this doesn't work, just go to fresnobee.com then select obituaries and then todays if it is Sept. 5, otherwise, just choose Sept. 5th as the day to view. Thanks to all of you for your gracious support during this most unbearable time in our lives. We will never forget your love and prayers...

Patrick and Linda Dirlam

Answer to question about an autopsy: I got one but not without a fight. The HMO said one wasn't necessary because the cause of death was due to complications from Wilson's disease. I told them the FDA required it and they had better do a complete one even though it made me sick they were going to destroy my son's beautiful body. My mother wanted to say farewell to him one last time but I told her after the autopsy, it was NOT an option. The HMO then agreed that they would do the autopsy but they refused to transport the body 2 miles from the hospital he died in to their hospital!!! The funeral home finally stepped in and said they would do it for me. We won't know the results for 1 to 2 months and when the truth comes out, heads will roll! Unfortunately, the HMO has a "no-sue" policy that all members have to sign. You have to go into arbitration and the neutral arbitrator is HIRED by the HMO!! Nevertheless, I am still going to find out exactly what happened and I believe he died of shock, possibly septic shock but the fact of the matter is he was in shock and gradually getting worse over a period of 4 hours and the RN on duty, who was present with him the whole time, did not even recognize the basic symptoms of shock! The paramedic who finally arrived at 2:30 am immediately saw it and freaked and rushed Jeff downstairs in less than 5 minutes and went full sirens and lights. I just wish I had known the symptoms of shock. Jeff may still be here today and walking in a walker right now. I entrusted my son's life with a trained professional and lost! It is especially hard right now because last Thursday is the day he started slipping. When I got there at 11pm he was doing pretty bad but they did nothing until Friday morning at 2:30am and he died at 5:01am Friday morning. It is the one week anniversary and although it may seem ridiculous to some, I am getting sicker by the minute just counting down the minutes and re-living Jeff's last hours.

One nice thing though is that my wife and I tonight went outside and noticed the sky was a fire red. The mountains and the trees were this eerie orangish color. I took a picture of it. We rushed to the beach and walked out to the water. I took a few spectacular shots of the sunset which Jeff just loved to watch at the beach. We let the water run over our feet and cried our eyes out. It was so sad yet so beautiful. I am posting the picture of the sunset over the mountains where the ocean is. This is where Jeff always liked to be to view the spectacular sunsets we have here, but tonight's sunset was very sad but very special for us.

Patrick

September 8th

We had Jeff's funeral yesterday. It was the hardest thing I will ever have to do. When I am thirsty, I think of Jeff's mouth being frozen open with no way of drinking and I ignore the thirst. When I hit my head or leg on something, I think of the pain Jeff was in for months and I don't care. Pain and suffering seem so trite now when I think of all the suffering that Jeff endured over the last 6 months. I am in despair.

Jeff's funeral ended up with about 150 people. It was beautiful. People he had touched from all over came to pay their last respects. It was standing room only. It made me feel very warm inside knowing how loved Jeff was. Many people told stories of how Jeff cheered them up with his smile and antics. I played 2 songs that meant so much to Jeff and I. One was "Here with me" by Dido. It was Jeff's favorite song. I never really listened to the words until after he passed away. It was uncanny. She was singing about me. No matter how tough I appear to be, I am nothing without my son! The second song was "Time" by Alan Parson's Project. It is a song I told Jeff he would have to play at my funeral because it reflected how I felt about him and me. He told me I would have to play it at his funeral if he went first. I told him a long time ago that would NEVER happen. Fate is so fickle! The whole congregation cried over that song. IT was hard for me but I know Jeff would not want everyone to leave crying and sad so I had a special last request of Jeff. We handed out Kazoos to everyone and the organist (a famous local pizza parlor and church organist) played "you are my sunshine." It was so beautiful to hear over 100 kazoos singing that song to honor Jeff. After that we did "it's a small world after all." Everyone left happy. I got Jeff's ashes today and returned home to the coast. It is ironic that the box of ashes weighs almost to the ounce the exact weight Jeff weighed when he was born. I found his keepsake chest last week and discovered a treasure. I had saved all of his baby teeth. I am going to put them in with his ashes and his best friends and our family are going to scatter them in the Sierra Nevada mountains by Yosemite where he loved to camp and reflect on life. Then sometime in the near future we are going to scatter the rest on the Pacific Coast in a place only he and a few friends knew about. It was a very private spot for Jeff and his friends and I think it fitting his ashes come to rest there. That way he will be in the places he loved at the same time. The mountains and the coast!

I don't know how much I can contribute to this group in the coming weeks. My grief is just too great. I will inform you when Jeff's autopsy results are in and the course I will pursue. I know he died wrongly in the hands of incompetents as I stood by and watched unknowingly and helplessly, and that is extremely hard to live with. But it must not happen again to anyone else. Jeff would want at least that! For now, I lay out under the stars gazing at the mountains in Jeff's hammock with a bottle of beer in my hand toasting my beautiful son!!

Patrick

September 10th

For those interested, our home town paper ran a much nicer, longer obituary for Jeff telling a little more about his life. If any are interested, I would appreciate it if you could read about what he had done in his short life and maybe sign the guestbook so we'll have something to remember how many people he touched in his life with his struggle and just being Jeff.

thanks, Patrick and Linda

The link is: http://www.legacy.com/sanluisobispo/LegacySubPage2.asp?Page=LifeStory&PersonId=478747

or if you go to http://www.sanluisobispo.com/mld/sanluisobispotribune/ and click on for Sept. 6th and click on Jeff Dirlam.

September 16th

I got Jeff's death certificate today. It says he died of Cardiac Arrest DUE to Wilson's Disease. That is a bunch of crap!!! Thank God I had the autopsy done. Dr. Brewer said the heart isn't affected due to Wilson's disease so something else went wrong. The doctors tried to claim that copper gets into all the organs including the heart and destroys it. All idiots!!! Jeff's heart, from the last ekg and blood tests the day before was totally normal. Since I saw him in shock for 4 hours and didn't know it and EITHER DID THE RN I guess that is probably why his heart gave out. He was so full of love and happiness for everyone as you all know that his heart just couldn't take any more abuse. I am getting ALL of the medical records including the medication charts from the rehab hospital. The nurse manager is going to send them to me no charge. That should show that he was originally put on Heparin for blood clots then taken off it for some strange reason. It should also show that he was supposed to be getting medication for his horribly burning stomach for 3 days but the doctor cancelled the order as soon as I left KNOWING FULL WELL that Jeff was in immense pain. If they do, then I 'm heading directly for the best legal attorney for wrongful death, negligence, malpractice, etc. in the country. Jeff will NOT die in vain! HE fought too hard for too long to be shuffled under the carpet by those ignorant, beurocratic, uncaring doctors. They all just kept telling me "Wilson's disease..., oh.....well, we'll try to make him comfortable, that's about all we can do." I am so sick of hearing this. This is why my baby died. Because of ignorant medical "supposed" professionals who only read their medical books (outdated) and had never seen a Wilson's patient before. The only cases they ever heard about were the liver failure ones who died, therefore ALL Wilson's patients die! I cannot accept this ignorance any longer. This ignorance caused my valiant, determined beyond superhuman, son to lose his battle with this disease. They will be punished for their callousness. Jeff was an inspiration for the entire Wilson's community and to have him snuffed out like that is unspeakable and inhumane. I will have the autopsy report within a month and I will let you know what the results were. I think it will show he had a massive blood clot in the lung which caused his heart to over-exert itself which caused his blood to thicken from lack of oxygen which caused his extremities to turn blue which put him into shock which caused his heart to overload and finally after racing to 200 beats per minute, it just said "enough is enough." The original emergency doctor told me that Jeff fought to his last heartbeat trying to survive. He couldn't believe that Jeff survived as long as he did in this condition for the last several hours with his heartbeat so high. This is what hurts me the most. He never gave up, he just didn't have a chance with the ignorance all around him.

I am sorry if I get so agitated and upset about all of this but it is really all that is keeping me going right now. If not for my anger I don't think I could go on. I miss my little "Pooty" so very much, it is like a black hole and I'm being sucked into it's infinite darkness!!! The light at the end of the tunnel that so many of you told Jeff in your emails has burned out for Jeff now, but for me it is righting a wrong which I will do for my son until my last fighting breath is taken!!! I owe that to Jeff and to all of the Wilson's patients in the world who are subjected to ignorance and beurocracy!!!!

Patrick

September 16th

Since this is NOT the way Jeff would want things I thought I would share a happy moment. Jeff always wanted a better telescope. We had a real cheesy one for years but he always found enjoyment in it looking at the fuzzy stars. In honor of his memory I bought a really nice one last week. It has been foggy almost every night here for the last 2 weeks since we live a short distance from the ocean. My planetarium software said that Jupiter and Saturn were below the horizon until around 3 am every morning. The problem with that is that I am asleep at 3 am and it is too foggy to even see across the street. I talk to Jeff a lot lately even though I haven't heard any replies yet. Last night it got foggy even before it got dark. I went to sleep around 10am and was very restless all night. Finally I could not sleep any longer. Something was telling me to just get up. I looked at the clock and it was 3am on the dot. For some reason I decided to look outside and it was clear as a bell! I rushed the telescope outside and set it up. For the first time I saw the beautiful rings of Saturn and 4 moons of Jupiter! I took a picture of the moon (a small picture of it is attached) with my digital camera connected to the scope (I made the adapter earlier in the day) and finally quit at 5am! I felt really good and just thanked Jeff. As always, I felt like it was just a coincidence but I still thanked Jeff for waking me up. After all, he can see it all from his viewpoint!

Patrick
September 22nd

I put the San Luis Obispo California Tribune guest book online permanently so people can post new messages from now on for me or Jeff either in body or in spirit. It will hopefully NEVER expire! Pass it on to other family members or friends so that everyone can post nice thoughts, remembrances, stories, poems, birthday greetings (Jeff was born on November 10th, 1976), etc.

The link is: http://www.legacy.com/Link.asp?ID=GB00478747

Love, Pat and Linda

September 28th

Last night I went to the Wilson's List archives to read all the emails I sent and replies I got since February when I first contacted the group. It was sort of enlightening but also very depressing. I read so many emails of support and was so worried about Jeff's condition while he was in Ann Arbor it is disturbing to think how upset I was then when I look back on it all compared to what happened AFTER he got back! I found an Easter email saying how a nurse gave Jeff a kite and we went up on the parking garage roof and flew it. The wing was deserted so I asked for someone to come and visit Jeff before we went home to give him support. Gerry Fortuna showed up a couple days later and really made Jeff's day! He saw that a person CAN get better with this disease and that inspired him to keep fighting. I only wish he didn't have to fight ignorant and uncaring doctors. I read the emails when he was in the HMO hospital and then in Arroyo Grande with double pneumonia. Again, I voiced how I couldn't imagine how things could get any worse. I always told Jeff that he could only go so low and then he could look nowhere else but up. Then I read the emails about the rehab hospital and how Jeff had finally stood his 6'5" and was so proud. Then, 2 days later the "my beautiful blue-eyed baby has died!" email. I started crying again thinking about what I had said to Jeff over the last year. It can only get a little worse then it WILL get better. That is the whole reason Jeff kept pushing onward with so much pain. He knew he could only go so low and then he would see the light. He finally saw the light on that Tuesday but somebody cruelly extinguished it! I never considered death as the ultimate "low" point for Jeff. HE was just too good a human being to deserve that. I probably shouldn't have read the archives but it helped to read all of the support letters and to actually meet a few of you. It seems so senseless now since Jeff didn't make it. It seems so senseless that he struggled for a year and was in so much pain just to lose the battle he wasn't even supposed to lose! It is so unfair. I still can't come to grips with all that Jeff went through just to be snuffed out when he was winning. I will never understand that. He wanted so bad to meet all of you and especially WALK across that stage next year to prove that he COULD beat this evil disease. I wanted to be the proud father with tears of joy in my eyes watching him do it! I wanted to hug the lot of you for all of your support. I wanted Jeff to LIVE!!!!! I will never get over this....I'm sorry.

Patrick.....................................................................................................................and Jeff

Letter from Gayla
Dear Pat,

Blake and I are currently raising three monarchs. Jeff's name or initials will be on each one we release.

Love,

Gayla


October, 2002

October 1st

Letter from Gayla
Dear Pat,

I just wanted to let you know that we caught a male Monarch today, tagged it and released it. The tag has Jeff's initials on it. I will send you the number of the tag tomorrow. I made a special note on my notes I keep so that I will remember that if it is found. We have another male Monarch to tag tomorrow and release and I will do the same with it.

I will write more tomorrow.

Love,

Gayla

October 2nd

I sold Jeff's hospital bed today. I have slept in it since I got back from that fateful night so it was quite depressing but I figured I might as well do it. I could have kept it for another month or two I suppose but I just decided to get rid of it. The lady said it was for her mother who had just had a stroke. She said she was old but aren't people living longer these days? I had to excuse myself when she said that and went to cry for a few minutes in another room. She asked me if my son was ok now and I just told her "yes, he is no longer in pain" and left it at that. I gave her the inflatable shampoo sink that Jeff loved so much. I had been using no-rinse shampoo on him and it made his head real itchy until the occupational therapist told me about the inflatable sink. When I tried that the first time and I could pour warm water on Jeff's head and really shampoo it he absolutely loved it. I massaged his scalp for a long time and he really enjoyed that. I told the lady that her mother would love it also. I also gave her some bed pads and misc. stuff. She was very happy and I just said my son would want her mother to have them.

I then went to his friends house where Jeff's storage shed is and started moving his stuff back over to our house. I found his artists box (whatever you call it...it is really big and holds all of his paintings) and it had a lot of the paintings he had done over the years and I had no idea what happened to them. Some of them were very good if not unusual. I am going to hang some of them. I also found a photo envelope with a lot of pictures taken in the place we are going to spread his ashes this weekend. He loved it up there very much and I didn't know any pictures of the place existed. A couple of them had Jeff in them camping. What really did me in however was the little stuffed tiger I found. I gave that to him when he was probably 4 years old. It was a souvenir from either a wild animal park or somewhere like that. Over the years he had many stuffed animals but eventually they were all discarded. I had no idea he had saved that one which was always his favorite. I just completely lost it and cried my eyes out. I didn't realize how much that little stuffed tiger had meant to him and had never seen it in all these years. I put it on my dashboard along with his favorite hat (Winchester Mystery House) so it will always be with me now! I still can't believe he is gone. It has been a month now but it seems like years and yet I still feel like he will just show up soon and things will be back the way they were. It is just so hard to accept!

Thanks to all of you again for your continued support. I am trying to finish up the journal of Jeff's journey and maybe I will offer it as a word document or PDF file or something for anyone who wants to read it when it is finished. I still may try to turn it into a book so you guys would all have the first copies in a way.

Patrick...........and Jeff

October 3rd

Today I got the last of the Jeff's stuff out of storage. I found his high school diploma and an old ticket stub from the only concert we went to together. It was for his 21st birthday and the group was Emerson, Lake and Palmer for those of you who know them. He grew up on them when he was a child and always wanted to see them but they broke up. I told him how amazing it was that only 3 people could sound like 5! I told him that what you hear on the albums is what you get in concert-no multiple takes. He couldn't believe this until he saw them. He was floored! I was up there at the stage dancing (just like old times) and going crazy and Jeff just cracked up and joined in. We were so hyped up all the way home from the concert it was great. I also found a LOT of short introspections and stories he wrote. Some of them were when he was younger but a few were written, according to his best friend, after he started getting really sick from Wilson's but didn't know what was wrong. A lot of people started making fun of the way he talked and he started doing things that didn't make a lot of sense to his friends and lost several because of that. His best friend tried to stick with him but even he fell prey to Jeff's emotional frustrations and told me Jeff was psychologically messed up. That is when I realized something physical was really wrong with Jeff. A parent generally knows their kids pretty well and I KNEW Jeff was not mentally ill. This was just before he got fired from his job for being excessively absent from his illness. All of this really took its toll on Jeff's spirit. This is when he started going to the place in the Sierra's below Yosemite where we are going to scatter his ashes. It helped him "detach" himself from all the pain and suffering he was going through with apparently no one except his father believing there was something wrong with him. I did not know he was going through all of this until one day when he called me and told me he was going to kill himself because nobody believed him any more and they all made fun of the way he talked. I told him to go outside and listen to the wind in the trees and smell the pines (he worked at a casino in the mountains). I told him to think of the first time he caught a fish and I thought he had just snagged his line on a rock again. He laughed and remembered that he told me he was determined to reel it in to prove to me that he could catch one. It was the biggest fish we caught that day! That cheered him up and I told him to come to my house that weekend and we would talk. That is when I realized he was really sick. Once he got away from his job and moved in with me he started really feeling good about himself again even though he was getting worse. It took six months for the doctors to figure out (for the second time) that he had Wilson's disease. At home and even when he was in Michigan, he would sometimes stand in front of a mirror and just stare. Jeff loved mirrors. I recall that once in the hospital in Michigan when we first got there he did that in silence and tears started falling from his eyes. I asked him if he was ok but he couldn't answer. He just kept staring into the mirror. I hugged him and told him everything would be ok. Anyway, back to the stories. His best friend told me that Jeff wrote a lot of introspectives at that time and had put them away somewhere. He read a couple of them and thought Jeff was crazy. Jeff always had this fascination with death but he still loved life. I read some of those handwritten "thoughts" today and decided to type them all out since his handwriting was getting pretty bad and they are very hard to decipher. I thought the group might want to read one of these. Just look at it as a very troubled young man with seemingly no where to turn and no one to understand him about the changes he is going through and the pain that is just starting to raise its ugly head! This one is especially troubling to me. If I had only known how much mental anguish Jeff was in because of the people around him (since he loved everyone it was hard to accept their ridicule) I would have acted sooner. The story follows...

Patrick

Mirror Image
(summer of 2001)
by Jeff Dirlam

I sit and stare into a mirror, an evil mirror full of hate. Hate from a world full of pain, my pain! The colors I see around me are dull. The flowers I sit before me all wilt. Life has no beauty for me. I feel I am an eagle born with no wings. I feel as powerful as that eagle. I yell and people listen. As I pass by, people turn to look, but the faces I see before me are full of pity. The stars shine down calling me. My brother and sister want me but all I can do is look up and see a tear drip from my eye and where it falls, death follows. Evil hearts light at my heels. I stare into the mirror, an evil mirror hollow throughout. My portrait stares back. A disgusting, disfigured man. A broken soul - myself! I try and smile but the reflection contorts into a scornful look. The mirror shatters from a move I never felt. Sweet, simple red blood drips from the wounds in the mirror. The reflection screams for revenge. Pain and suffering with no cause. Smooth waves of depression rush in. Why are people laughing? So many faces laughing. Stop the laughter before it turns to pain pounding into my ear. And suddenly, the laughter stops! Sweet red blood falls like rain. Warmth settles through my dying body and the only reflection I see is myself - my own face. For once truly something looking at me!

October 6th
We drove up to the Sierra Nevadas yesterday to the place Jeff loved to go when he was troubled. He also loved going to this place when he was happy but it really had a consoling effect on his being when he was depressed and I can see why. I had forgotten how serene and beautiful it is! The picture of the creek is where we spread his ashes. The creek runs into the small lake. There is hardly anyone ever there. On a Saturday there were 2 couples besides us! I videotaped the entire thing and made it into a beautiful movie complete with music which I will put on tape for those family members and friends that want a copy. It was really hard to part with some of Jeff's remains but as the lone duck drifted down the creek, so did Jeff! I think he is definitely more at peace now that he is back where he loved to be. I just wish him and I could have been there once again and I could hold his body and tell him how much I love him instead of holding his ashes......

Patrick........................................and Jeff

October 9th

Email to Dr. Brewer
I am supposed to get the autopsy report within the next two weeks and will send you a copy. I got the full report from the emergency hospital where Jeff died. The reason for his death as they noted it was:

Differential diagnosis:

1. Acute pulmonary embolus
2. Atypical myocardial infarction
3. Electrolyte abnormality causing the dysrhythmia
4. Pneumonia
5. Congestive heart failure
6. (additional diagnosis): Acute respiratory acidosis


Emergency Department diagnosis:

1. Acute respiratory arrest
2. Acute cardiopulmonary arrest
3. Wilson's disease

I am not sure what all of this means but the fact is they all said that upon examination his extremities and skin was cyanotic (blue??) and that the rehab hospital stated "Pt @ SJ rehab, PT unresponsive ALL DAY, cool extremities, labored breathing." Why didn't the rehab hospital do something earlier if he was cool (blue) all day and had labored breathing???

I would like a straight answer from you DR. Brewer. I know you say that every patient is different and not all recover as fully as others but you never mentioned that Jeff could die from this disease other than non-compliance with zinc or complications like the pneumonia he got. I would like to know if at ANY time you felt that Jeff would NOT make it through this disease BECAUSE of this disease itself. I mean specifically that before he left Ann Arbor did you feel in any way that Jeff would probably die from this disease? The reason I ask is that if there is any malpractice or negligence on the part of the rehab hospital and it sounds to me like there was, then the attorneys are going to rip me and Jeff to shreds claiming that Wilson's disease was the underlying reason that Jeff died. There is no way in HELL that Jeff had a bad heart. That was his STRONGEST point! He had lost 70 pounds since the pneumonia and the rehab hospital didn't seem too concerned about it. I freaked out and that is when I called Cewin (Debbie) and had her discuss it with the nutritionist at the rehab. Nothing was done. Also, I have not got the records from the rehab hospital yet but it will show that he was taken OFF his stomach medication (Carafate) for no apparent reason for the last 3 days (right after I left) and when his stomach tube was replaced August 29th in the morning by the head of the GI department she put him on Zantac for the pain but the rehab doctor took him off of that immediately also. Jeff suffered needlessly for 3 days in immense pain with his stomach. Also, I do not believe they were giving him Heparin any more and that morning they did an Ultrasound on his leg for concern of blood clots. But, they still did not put him on Heparin as far as I know and that may have saved his life since the emergency report said something about a pulmonary embolism which I think is a blood clot in the lung. I just need to know if you really thought Jeff had a fine chance of survival and the only question would be his neurological recovery. You may be asked to testify in this matter and I hope that you would to prevent further deaths from the ignorance of doctors. They all at his HMO told me that there really never was much hope for him since he had Wilson's disease and they did everything they could. I don't believe that bullshit! I just need to know from you, Dr. Brewer if you thought from your experience that Jeff had a normal chance of survival and a pretty good chance at a good recovery from his symptoms.

Thank you so much for all of your help and originally saving Jeff's life. I am indebted to you for that. I have quite a few pictures of Jeff with the nurses and you and Daisy. I would like to send copies of them to you or the hospital so each one of them and you can have a picture of them with Jeff in his happier days. I hope this is all right with you.

Thank you,


Patrick Dirlam

October 11th

I got the autopsy report today in the mail. It was an absolute joke! It was 2 pages with a bunch of irrelevant stuff and a short summary with "the most likely cause of death is due to a cardiac event." What kind of an autopsy is that??? They can't be specific as to the EXACT cause of death? It also states that the final autopsy diagnosis was:

I. Cirrhosis, liver, severe.
A. S/P PEZG tube placement.
B. History of Wilson's Disease.
C. Peritoneal fluid, 200 cc, milky/greenish.

II. Pulmonary congestion, bilateral, lower lobes,
right lung = 500 gm, left lung = 650 gm.
A. Pleural adhesions to rib cage.

The medical summary states:

The patient is a 356 year old male, who had a diagnosis of Wilson's Disease for two years with associated dysphasia and neuropsychological problems. The patient had participated in a research study with the University of Michigan Medical Center for a few months in the beginning of this year with subsequent placement of a percutaneous endoscopic gastrostomy tube. The patient also had recent admission on 5/23/02 for fever, dehydration with right foot Cellulitis and later with pneumonia. The patient had a PEZG tube replaced in the last week of August and subsequently developed shortness of breath. The patient was admitted to Saint Agnes and expired on 8/30/02.

At autopsy, there is severe cirrhosis of the liver consistent with the history of Wilson's Disease. No pulmonary thromboembolism or GI bleeding identified. In this case, the most likely cause of death in due to a cardiac event.

Can you believe this??? First of all, the emergency hospital did an x-ray and found a massive pulmonary embolism (blood clot) in his lung which caused his heart to pump harder which eventually caused the cardiac arrest. Secondly, the emergency hospital mentioned something in the charts about the stomach tube being several inches higher than it should be. Not the original but how it was inserted that day???
The HMO hospital replaced the tube that morning and noted bleeding at that time. Jeff was also very agitated and the nurse taking care of him said his clothes were drenched from head to toe and told the doctor when he came in that morning that Jeff's leg and stomach hurt really bad. Instead of canceling the appointment, he simply said "that's good" and left! She asked if she could go to the hospital for the replacement with Jeff since he was so agitated and they said "no!" However, when he arrived, the GI doctor noted his agitation and found his stomach to be in a mess so she ordered Zantac which the rehab doctor promptly cancelled! Also, they did an ultrasound on his leg because they thought he might have blood clots. It is just interesting that everything that the HMO didn't do is "covered" by the HMO pathologist's weak autopsy report! I called and demanded an explanation and the receptionist said they don't give out the full report to family members. I demanded it and she is sending it today. I asked why, if he had severe cirrhosis there was no indication of how bad or how much copper damage there was. She said that would be a lab thing and it probably wasn't done. I asked her how you could NOT do a complete lab workup when it was supposed to be a COMPLETE autopsy. She said she didn't know the answer to that. I questioned her on the "most likely cause of death" line and she said that is what the pathologist wrote. I said "I thought a complete autopsy was to find out EXACTLY the cause of death, not just the "most likely cause?" She didn't have an answer for that.

I am totally devastated by this. It appears that once again, the HMO is covering it's ass! I can't imagine how a pathologist could not determine the exact cause of death. He mentions pneumonia which is what he was treated for in ANOTHER hospital and Wilson's disease which he was treated for in Michigan. No mention of anything since then as the cause. No mention of simply "bad medicine practices" as the underlying cause of his heart attack. No mention of WHY he had the heart attack in the first place. The only mention of a cause is the fact that he DIDN'T have a blood clot in the lung which the emergency hospital SAW on the X-ray and that the stomach tube area had no problem.

I talked to an attorney and it seems that California does NOT favor malpractice cases and most attorneys won't even take one on because of the expense. The most Jeff would be awarded for the immense pain and suffering he was in in the last 3 days of his life is $250,000. He left no heirs or wife so California considers him "worthless" just like New York does. If an attorney can not assume they will easily get $1,000,000 out of the case they won't even take it. I can't deal with any of this much longer. Not only did they kill my son, now they are trying to brush him under the carpet. Please help!!!


Patrick...............................................................and Jeff mad as hell!!!

Letter to the University of Michigan staff
Daisy, Dr. Brewer, nurses and staff: Oct. 11, 2002

I just wanted to thank all of you so much for what you did for my son Jeffrey. He really did enjoy being there in Ann Arbor because he knew that in your care he would get better. He was always afraid of his HMO, in fact he said they would kill him someday and I guess his prophecy came true unfortunately. Even though he had a rough time at UMMC he knew it was to save his life and you guys were all the best in the world to do just that. At no time whatsoever did Jeff ever worry about dying while at UMMC. He wanted so badly to come back next year and show you all how he could walk and talk again. He was just starting to realize that dream when he died. He finally stood up his 6'5" and walked from one end of the parallel bars to the other and the day before he died he actually closed his mouth and when the nurse asked him to say something he said "no!" Since that is no longer possible I wanted to give something back to all of you. I took pictures of a good many nurses and staff during our last days there and thought that each of you might like to have a picture of you and Jeff. I think this way he will always be with each of you. I didn't get pictures of all of you and I'm sorry for that. Daisy, please get these pictures to everyone. I made three of you, Suzanne and Dr. Brewer so each one of you could have one. Maybe you could put your copy by the plant Jeff gave you. I included a couple of extra ones of him smiling and one on Easter morning with his Easter Bunny. I sleep with that bunny every day now! I am also sending you one of Jeff outside when it was snowing. He loved your Michigan weather and you can tell by his loose sweater and socks! I was completely dressed with sweater, coat, etc. and still freezing but Jeff loved it! I am also enclosing the picture I took of the message in the snow I wrote early one morning for Jeff. As most of you know he would always go to that 7th floor window every morning while waiting for the elevator and just look out at the scenery and the courtyard below so one morning I went out before he woke up and carved "I love Jeff" in the snow and almost got frostbite from it! When he went to the window he saw the message and gave me a big hug and smile. I miss him so much!!! The next picture is of Jeff and me together. We will be together again soon!!! The last picture is a place called Manzanita Lake and Jeff named the creek running into it appropriately as Mosquito creek. This is where we scattered his ashes. It is just below Yosemite national Park and it is so serene and beautiful you can't imagine. Jeff went there a lot in his final days before he got too sick to drive when he was depressed. He would sit out on a rock and just listen to the wind in the trees, smell the scent of pine and watch the water flow down towards the ocean where I lived. His friend told me Jeff went there all the time. Jeff and I used to go there a lot when he was a little kid but I didn't know how much he loved this place until after he died.

There is one person you may not recognize. Her name is Barbara and she worked on the first floor in patient relations or something like that. She was in charge of lots of things but one was giving out meal tickets. She was the manager I think and it was right next to the ATM machine and bank. She was very special to me and Jeff and I would appreciate her getting the picture if she still works at the hospital. I know she said she might be moving to LA or DC.

I am going to miss all of you next year. I was really hoping that Jeff and I could see you all again. It would have been very special to both of us. Now, all we have are memories and pain. A special thanks goes out to Mary the graveyard nurse for offering us her car on the weekends to go places although Jeff was not in good shape to do that. Another thanks goes to Paula for offering us tickets to her performance. Again, Jeff was not well enough to make it but he really did want to go Paula. Mary for being so funny, Chris and Kris for being so good, Amanda for being so quiet in the early AM, Debbie for being so nice, Denise for always being so thoughtful, Bruce I think, for finally figuring out Jeff's anxiety problem with the condom catheter! Irene…what can I say! A certain person (I won't mention her name but Irene knows who she is :) for sneaking Jeff ice cream and pudding at night occasionally…he really LOVED that!!! Darlene (I hope you got him this) for the kite on Easter (I didn't get a picture of you so I am sending a picture of Jeff flying the kite…he loved kites!) Dr. Wiley for being so friendly and helpful, Barbara for all of her fantastic help and smiles for Jeff, and I forgot which one of you brought the ham dinner on Easter but it was Jeff's last complete meal, I think it was Debbie. She didn't get it for him because he wasn't supposed to eat solid food anymore but I gave him some in little tiny pieces and he loved it! For any of you I forgot, I'm sorry. All of you were wonderful and Jeff always felt safe with you.

So many people attended his funeral that it was standing room only. Over 150 people showed up. I didn't realize just how many people Jeff had touched in his short 25 years but they all came and paid there respects to a great human being. One who never complained even when he should (the nurses can all vouch for that!), one who would spend his last dime on you if you needed it, one who always found a way to make people smile! As a final tribute to my son, I handed out Kazoos and 150 people sang "you are my sunshine" in honor of Jeff. It was really beautiful and a testimony to the loss this world sustained by his death.

I love you all for all of your help, understanding, support and fantastic care of my son during his two month stay at your hospital. If not for the terrible treatment he received after he left Michigan, he would be able to tell you that himself to your faces next year!

Love,


Patrick Dirlam
October 14th

Letter to Lenore Sillery
I have not heard from you in a while. Did you sign off the list? How are your boys. How is the one with the brain problems coming along. I forgot their names...I'm sorry. I have been in a real turmoil with the autopsy report, the rehab hospital charts and the emergency hospital charts. They seem to contradict each other but the HMO doctor reports AND the HMO autopsy report seem to fit perfectly together! Since the HMO must know that I plan to sue them they have covered their butts perfectly. The emergency hospital x-ray shows a large pulmonary embolism and cardiac arrest was the cause of death. The HMO put Wilson's disease as the cause of death and the autopsy performed by the HMO showed NO SIGNS of pulmonary embolism and MOST LIKELY cause of death was a heart problem and SEVERE LIVER CIRRHOSIS. What kind of autopsy was that? I have never heard of a complete autopsy coming up with a most likely cause of death. I thought that a complete autopsy gave a DEFINITIVE reason for death! I am at the end of my rope Lenore. It appears that Jeff suffered all those months for absolutely nothing! Who cares if he gave lots of people hope? Who cares if he tried his best? If he knew that he was going to die and the HMO would simply sweep him under the carpet I don't think he would have tried so hard for so long. It breaks my heart to now know that my baby suffered so terribly much for nothing! We were already close. This did not bring us any closer. It actually put a strain on our relationship because I could not deal with all of his pain and suffering and that hurt me to the core and I know it hurt him as well. I loved Jeff more than life itself and knowing that he had to suffer so much in the last few months and then just die MOST LIKELY from a heart problem when his heart was in perfect condition is causing me more pain than you can imagine. SOMETHING caused the heart attack. The emergency hospital said it was a blood clot and the autopsy showed he had no blood clots. They took almost 5 days to perform the autopsy and I don't know if that could be a factor. Maybe those things can dissipate over a couple of days. All I know is that my baby died a wrongful death and nobody cares any more. Even attorneys won't touch the case because in the eyes of the state of California, Jeff wasn't worth anything because he did not leave any heirs. I am heartbroken Lenore. I am supposed to go on with wonderful memories of 25 years of my beautiful son knowing that he died a wrongful death. I am crying now more than when he died. Take care of you children Lenore because God knows the doctors certainly won't! They are just like cars to doctors. Some can be saved and some have problems which they can't figure out so the attitude is "just let them keep running until they give out and then send them to the scrap yard!" They all say they are sorry but the next day they have completely forgotten about it! Within a week after Jeff died each of his doctors except for one told us they needed his HMO number because they didn't know who we were talking about and THAT is NOT an exaggeration!

I'm sorry but I have missed your emails. You have always been the most personable and helpful to me because you CAN relate. If you have not been on the list lately, I have included pictures of where we spread Jeff's ashes and I could email them to you if you are interested. It is just below Yosemite at a very small lake and creek that Jeff loved to go when things got bad. I am going to privately spread some of his ashes by the place we used to go when he first discovered the ocean. I'll never forget the first video of him running up and down the sand dunes when he was 5. He was at the top at sunset and the sky was beautiful. It overwhelmed him even at that early age. He came down and told me "you know why I like the ocean Dad? It is because you can see it all!!" I sent the video of his younger years and his graduation and finally the spreading of his ashes to all of our family members so they could see what a happy person Jeff was. He loved life and that is the only reason he held on even though he was sure his HMO would kill him. He did NOT want to go into rehab in Fresno because the HMO doctors would still be in charge of his care. He was right in the end. His HMO did kill him and now I have to live with the fact that I sent him to his death. No matter what anyone says about "oh you did everything you could to help him" and he wouldn't want you to think that way" the bottom line is that Jeff knew that this HMO would kill him because of their fatalistic attitude toward his disease and he was right and I made it easy for them to realize his fear! How can you live with that?????


Love, Patrick

October 15th

I went to Jeff and my favorite spot today where we used to camp when he was a child at North Beach Campground. I videotaped the tree he used to climb on and then walked up the trail he loved. I walked down the dunes and looked up and found the one in his early videos. I walked up that one and let his ashes blow into the wind. It was very sad but I felt good that he was now in both places he loved. I videotaped the whole thing for future family viewing.

October 16th

Got the full autopsy report today. It still says Jeff died of a heart problem but not why! I know his heart was strong and he did NOT die of liver failure as the HMO doctors seem to think. I know they blew it on him but how can I prove it. It is so unfair to him to end this way. Talked to my mom for the last time. I wish I could have seen her one more time. I talked to Mom Dee and told her to tell Pop that I loved him very much. I thought that today was Thursday and tomorrow was d-day but I was wrong. Oh well, one more day won't kill me…hehe….

October 17th

Talked to Dr. Brewer today. Faxed the autopsy report to him. Went over it on the phone with him and he said it sounds like Jeff died of Septic shock! That is what Linda and I thought. If I had not left Jeff on Tuesday I would have seen his stomach condition get worse and the carafate not working and done something about it. Also I would not have allowed him to get his stomach tube replaced. I wish I had of been there for him his last 3 days of life. I will be soon however! I sent all of the charts from the rehab hospital and St. Agnes along with the autopsy report today to Betty. Hopefully she and Linda will pursue the lawsuit if there is one. I got Jeff's shed all ready and told Linda I was going to the beach on the motorcycle. I made a spot to hide the cycle and then fixed the doors so they can be locked on the inside. I don't want anything to go wrong with this. It is NOT my intention to be saved! I feel bad that Linda won't know until she realized I'm not coming home early and then will have to go into the night worrying and probably call the police. They will look and then come out Saturday I hope. I should be good and gone by then. Hopefully she won't discover the body. I don't think she would think of looking in the shed but the police will, especially if they find the motorcycle before she does which they should as well. I am really sorry it has to end this way but I just can't go on happily in this life without my Pooty! I went to Pismo and walked out on the pier. I really cried a lot thinking of when Jeff and I used to do this 20 years ago. Nothing had changed except Jeff would never walk it again with me. I cannot walk it again myself. Too many memories to handle it. I then went out on the beach and found a feather and a sand dollar and remembered when Jeff and I used to make sand castles together. It was very hard to confront but I did it. I told him I would see him very soon. I am sorry that I didn't get to say goodbye to everyone and see Danny again for his birthday. I really wanted to see my mother once more as well but I will be watching over her as well as Linda if she comes to the funeral. Hopefully Jeff will be there with me!!!

October 18, 2002

I am going into the shed. Going to play Jeff's favorite CD over and over (Dido). Going to have his ashes close to my body. Going to hold on to the Easter Bunny I got him in Michigan. Going to look at some of his pictures. Going to reminisce my life and Jeff's. Going to drift off……

Goodbye all! I love you! Hello son!!!!!!!!!

October 22, 2002

I am logging this entry 2 weeks after this date because I am still here! I remember all the details of what transpired in the shed up until I lost conciousness. I did not wake up until Tuesday, October 26th. Linda came home and knew immediately something was wrong. She found the new toaster I bought for her and went upstairs and found the present I bought for Danny with the note “give to Danny on his birthday” on top. Then she found some pictures I had taken of myself with the note “please give these to family members if they want one” and that did it. She called her father in a panic and he told her to call 911. The police finally arrived and discovered my motorcycle covered up out on the property. They followed an extension cord out to the shed and noticed it was locked from the inside. They asked her if she knew why it was locked from the inside or why the cord was running to it and she said she didn’t know. They broke the door down and found me inside. There was a plastic bag over my head and when they took it off, my face was swollen up the size of a watermelon. I was barely breathing (the paramedic later said about once every 3 minutes) and I looked really bad. The officer told Linda he didn’t think I was going to make it. The paramedics got there and rushed me to the hospital. The police found the note and the empty bottles of the approximately 300 pills I took. Each bottle had at least 50 pills and each medication was lethal in a high dose. There were around 6 different medications as I recall ranging from oxycontin to neurontin and all were either narcotic pain killers or anti-depressants. I did quite a lot of research on the internet to ensure that this “cocktail” would be enough to put even the strongest person away. An overdose of each individual medication was fatal, so a combination of 6 of the strongest should ensure success. To be sure however, just as I was going under, I tied two plastic bags over my head tightly so I would run out of oxygen to finish the job.

So how did I end up writing this entry??? It is an amazing story and one that needs to be told for those comtemplating suicide. I did not go into some form of state of bliss. All I fell into was total darkness and total unawareness. There was no bright light and no Jeff to greet me. Just silence, darkness and no sense of awareness at all! On the living end of this experience, it was a nightmare! They rushed me to emergency and the doctor who first saw me told Linda I was in a coma and had oxygen cut off from my brain for over 7 hours so even if I did survive, which was doubtful, I would be a vegetable for the rest of my life. I had severe damage to my brain and organs and was put on life support. I had double pneumonia and wasn’t expected to survive the night. The doctor told Linda it was the worst attempt at suicide he had ever seen in his entire career. I was in the coma for 3 days. Danny came that night and my mother did as well. The doctor told Linda that she should pull the plug on me because if I did survive, I would be in such bad shape that I would have to be in a care facility unable to function by myself. She told me that “someone” or some voice in her head kept telling her NOT to pull the plug. She was sure it was Jeff speaking to her. The doctor warned Linda of the consequences if I survived but she insisted that he do everything he could to save me. On Monday night, the 25th, I started coming out of the coma according to Linda. I don’t remember any of this however because I was totally delirious. What ensued in the hospital room and in my unconciousness was an amazing story of physical distress and spiritual enlightenment.

As my body started to come out of the coma, it started kicking and sitting up and tossing all over the place. This freaked Linda, Danny and my mother out completely and the doctor warned Linda that this would happen and this is probably how I would be for the rest of my life. I was mumbling total nonsense and fighting everyone. I was put in restraints but broke every restraint they could find. The doctor told Linda I could not keep this up for long because my heart would give out. She freaked out and cried but the doctor could not give me any medications because my body was so full of drugs already. This went on for several hours and my strength to break free was incredible. The male nurses had to come in to help restrain me. The doctor went home telling Linda she would have to make arrangement to have me moved to a care facility when I was released. Finally, just as quickly as my body started going nuts, I just fell back into the coma. What was going on with me spiritually was totally a different story and changed my life forever. I woke up in a very white, sterile looking room. It was kind of “fuzzy” and I remember I was lying on a bed. There was a bed next to me and even though it wasn’t occupied, I knew that Jeff was in that bed. I tried to talk to him but heard nothing. Then one of the walls opened up to these huge doors with blinding white light shining through. They looked almost 50 feet tall! A group of beings (I don’t know if they were angels or just people) all dressed in blinding white came in and surrounded Jeff’s and my bed. I thought “this is it…the pearly gates, the white light..I’m going to be with Jeff at last!”  Several of the beings gathered around Jeff’s bed and began wheeling it into the light. My bed didn’t move. I remember sitting up in the bed and asking, then shouting “hey, where are you going? Don’t leave me here! I am going to! Jeff, where are they taking you?” I was in a panic and started fighting to get free. I had many beings now surrounding me and holding me down. I tried in vain to break loose. I grabbed onto Jeff’s bed and held on for dear life. I was not going to let them take him without me. This is why I did what I did…to be with my son…and I wasn’t going to lose him now. I fought with them for what seems like an eternity but he just kept getting farther away. Suddenly, I heard Jeff’s voice. It was very calm with no pain and full of love. He said “Dad, please let me go. It is my time and not yours. I am not in pain anymore and I am happy. I will always be here for you and will be waiting when it is your time. You still have much to do here and you can’t come with me. I will be ok. Please let me go Dad. I love you!” With that said, I let go of the bed and watched my son disappear into the bright light and then I lost all awareness once again.

I awoke Tuesday morning, the 26th. My mother was at my side. I looked at her and said “mom?” She asked me if I knew who I was and I said “of course I do!” She called the doctor who came in immediately and asked me several questions. He asked me my name, who was the president and some other standard questions. The only question I got wrong was “do you know what day it is?” I answered “Friday.” I was obviously several days off! He told my mother that there was nothing he could come up with to explain my recovery without any apparent brain damage other than a “miracle!” He ran many tests on me and it appeared that my pneumonia was in total remission, my brain functions were normal and my organs had miraculously healed! I then proceeded to tell my mother the story of my experience the night before and she told me what had transpired with my body. We had a good cry and both of us had a different outlook on death and the hereafter!