HI,
My husband was diagnosed with PSC in 2009. Since then he has lost most of his facial hair. Doctors say this has no relation to PSC, but is another auto-immune disorder. Has anyone else suffered from hair loss or any other auto-immune disease along with PSC. I would be interested in your comments.
I can't say a definate yes, but there is some research that suggests that a lack of vitamin D (like many PSCers have) is a factor in hair loss.
Hmmm...that is interesting...I will have to look into it.
in the past 5-7 years i can say my hair has fallen out a lot and is very weak ;o(
I lost hair on my legs and arms. My hair got thinner. My facial hair remaind the same. I attributed it to the Stage IV liver disease and all the medications I took. I never asked a do for about it. I got a transplant on 2/8/12, and hope I start growing hair again. I had a live donor transplant at Northwestern Memorial Hosiptal in Chicago. I feel great. You don’t take any pre-transplant meds any more. You take new ones that help with the growth of the liver, infection control and rejection med. they take you off the meds, except the rejection drug, Prograf over a one year period. I go for a blood test twice a week. They adjust the meds as necessary. I hope your husband gets a liver soon. I lived with PSC for 12 years. I was in bad shape when I got transplanted. Look into live dknot transplantation, especially of your MELD score has stayed low and you have been getting sicker. PSC is a disease that does not score as bad as you are on MELD, and MELD is the only criteria used to allocate cadaveric livers. You can die from PSC long before you will qualify for a UNOS liver. A transplant surgeon at a university told me the only way I will get a transplant is through live donation. It took 9 months and a miracle to find a donor and get transplanted. Praise God it happened.
Thanks for sharing Paul. I'm so glad you found a donor. I did not know about the MELD score. We are from Canada and the doctors have never mentioned it. Whenever we ask if we are on the transplant list yet they say that my husbands liver is not in that condition yet. I don't know what to think. I will have to ask them about a MELD score. My husband's blood type is O negative...a rare blood type...he is only 25 at the moment and was diagnosed in 2009. Would you be able to share with us your PSC journey along with the syptoms that you had. We don't really know anyone with PSC and its always encouraging and interesting to hear from others. We are going to the PSC conference in Rochester the first weekend of May. That will be a first for us. It should be interesting as well. Thank-you for your response!
Sabine
Model for End Stage Liver Disease (MELD) is used in the United States to determine eligibility for a cadaver liver. I don’t know if Canada uses the same protocol. Before January 2001, there was no MELD. U.S. doctors subjectively decided when their patient was sick enough to warrant cadaveric liver transplantation. As you might guess, politics, reputation, pushiness and who-knows-who made the system unfair. So they came up with a liver enzyme formula that became known as MELD. it was theorized that all people with liver disease can be accurately compared this way, and the sickest patients would get a liver. The trouble is, most PSC patients do not score high enough on the MELD scale to ever get a liver. Defenders of MELD will tell you that nobody needs a liver if their MELD number is below the cutoff. Not so. I was very sick, being hospitalized with liver failure every 4 to 6 weeks. I would bounce back and my MELD score would be 10 or 11. MELD is a scale from 0 (really healthy) to 40 (death is eminent). The country is divided into regions. Each one has it’s own threshold number. My region is 21. The Chicago region is currently 29. It is all based on the availability of cadaver organs and the regional demand for organs. I have been told that Jacksonville, Florida, is doing transplants with a score of 11. When you have a transplant, you are making a long-term commitment for continuing medical care. You cannot just go there, get a transplant and jet home (unless you are Steve Jobs).
Some PSC patients qualify under MELD. Most do not. Some hepatologists believe they can request an exception based on how bad your health has become or if you develop bile duct cancer. Mayo Clinic (authors of MELD) told me no exceptions unless you have been diagnosed with cancer, treated with radiation and chemotherapy and been cancer free for six months. Then you get half the points needed to qualify for a liver and you need the majority vote of the regional MELD board to get an additional 3 points each three months. If cancer redevelops, you have to submit to treatment again and start over again. (see posts on this forum by “Greatdane61” She is a survivor of this inhumane, midevil procedure)
MELD, whether it is used in Canada or not, is unfair to PSC patients. Most will die before qualifying or will be in such poor health when they do qualify that they won’t survive surgery. As a transplant recipient, I can assure you that you want to be healthy for that surgery.
PSC’ers that I have become acquainted with are positive thinkers, daring, have faith in God, have a good sense of humor and obstacles don’t stop them from soldiering on. That is my endorsement for how to deal with this disease: it works.
Hi Sabine,
I am pleased to share my PSC journey with you. Hepatologists are the most knowledgeable physicians regarding PSC. The facts are that they may have had or currently have other PSC patients, but everyone of us is different in symptomology and general health. There are so few PSC patients that the medical field has not gathered a great deal of knowledge about what it is, how it is acquired and treatments besides a liver transplant.
I had End-stage IV liver cirrhosis when we attended the PSCPartners Conference in 2010. I was just starting to show symptoms of deterioration then, and working as a public school business manager which is a very physically and mentally demanding job. Dee, my wife and I met other PSC'ers for the very first time. Some had advanced symptoms of jaundice and itching. One 25 year old man had Stage II cirrhosis and he was in terrible shape. He was jaundiced and had grown bone spurs on his forehead, jaw and hands that made him look like an old man. He was married just 2 years and they had postponed having children. He and his wife were very demoralized. I did all I could to cheer them up. The quirky PSC was worse for him than it was for me! Every patient is different, thus making it hard for your hematologist to inform you about what is going to happen or when you will need a transplant.
I know now that many of the PSC'ers at the conference were in worse shape than me. Were their MELD scores too low for a transplant? How many, if any, died before getting a transplant? I will never know. I suspect the hematologists don't know either. They hope for the best for their PSC patient(s), but ultimately the question is, will you live long enough to get a transplant? After waiting 4 years and then being told I would need a live donor in order to get a transplant, I turned my complete attention to finding a donor and dealing with my deteriorating condition (though my MELD score stayed the same). My MELD score hit a high point of 16 and steadily dropped to 11 or 12. Remember, you need higher the numberto qualify for a cadaver liver.
Now, back to your question regarding symptoms and my journey. In 2000 I had gall bladder surgery and a liver biopsy that showed Stage III liver cirrhosis. I also had elevated enzymes on annual tests from 1995 through 2000. The surgery was done at a major hospital, yet no doctor correctly connected the three as pointing to PSC as the cause, and it was. In 2007 I learned I had had PSC since 2000. I was very healthy and active. I was told PSC is treatable but not curable without a transplant. My hepatologist said some PSC patients need a transplant quickly and others liver their whole life without needing a transplant. I learned later that the average life-span was 10 years after diagnosis, so that "whole life without needing a transplant," was misleading if your whole life is just 10 years.
In 2009 I started having problems with fatigue and ammonia retention. A healthy liver cleans ammonia, a toxin that makes you forgetful and affects your thinking, out of your system. They had me take Cholestyramine, a powder three times day to absorb ammonia. It helped a little.
By 2010 I was working every other day as a school business manager. It took the off day to recover. Ammonia was interfering with the thought processing I needed for my occupation, so I retired in September 2010. After that, I began losing body muscle and tissue at an alarming rate. It shocked my hematologist, who had seen me just 3 months earlier. My liver had decreased its protein production and my cells were not being replaced. My MELD was 11.
My hepatologist suggested I get listed for transplant in another region to improve my chances. Getting listed is an involved series of tests. Each region has its own availability of organs usually expressed as the MELD number they are doing transplants. My region does transplants at 22. It was at the second hospital that the surgeon told me I would not live long enough to get a transplant. He encouraged me to seek a live donor.
We did not know where to start. Our daughter was the only family member with the same blood type.
She was tested, but was not anatomically compatible. She started a Facebook page called, Help Paul Find a Liver Donor. Two friends took inquiry calls and referrals to the hospital. I was in such bad shape that I had no energy to help.
A high school friend of our daughter's saw the Facebook page. They had not seen each other in 17 years. She was the same blood type and in her heart, wanted to be a living donor. She passed the testing as a perfect match. Our families got together for dinner a week before the surgery. It was a joyous occasion. Her family was very supportive. She never wavered in wanting to go ahead with the surgery. Right up to the last minute her doctors tried to talk her out of doing it. The basis: you are a healthy person going to submit to major surgery. There are risks, even death. Kathy said, let's do it.
When I came to in the recovery room, my first question was, "how is Kathy." She was fine. A few hours later, her husband wheeled her into my room and we held hands. She had a big smile on her face. When we see each other now we call it a "liver reunion." In two months, her liver completely regrew. I am about 50% regrown at present.
Right after the surgery my skin color became normal and I had energy again. My normal voice returned and the memory loss due to ammonia buildup was gone. Every day I get stronger. I drive to a diagnostic facility twice a week for blood tests that are monitored by the transplant center. They only call if something needs adjusting or if they need me to come in for a visit. Otherwise, I just go once a month. It seems miraculous to me. I finally have good health back. It would not have been possible without faith. All things are possible to them that believe. I feel like I am on the other side looking back. I never want to forget what it was like with PSC so that I can help others on their journey back to health.
If you have any questions, please ask. CaringBridge is a web service that let Kathy and me tell our day to day transplant story and it has many comments from "guests" that visited the site. I encourage you to go there yourself and read some of the events and comments. http://www.caringbridge.org/visit/paulandkathy It links back to my daughter's Facebook page. None of this could have taken place without her dedication and the love of so many people. Our local transplant support group helped me for two years before I had my transplant. They are a bunch of survivor warriors with so much positive energy that it is contagious just to be around them. Here is the website: https://www.facebook.com/pages/Transplant-Support-Group-of-Northern...
I met my friend Rich, when my wife was updating someone at work about how I was doing with PSC. Another person heard her say "PSC" and said she thought her brother-in-law had the same disease. Dee said it's not likely because it is so rare. But, the woman said she would check. Guess what? He had PSC. We lived 30 minutes from each other. In addition, we grew up in the same town and lived a mile from each other, went to the same high school, but did not know each other then. The two of us have both had live donor transplants and are tight buddies now. He is a year younger than me. And, our wives have so much in common as caregivers. They have been a great support to us.
Well, that's the story. Ask.
Peace,
Paul
Sabine said:
Thanks for sharing Paul. I'm so glad you found a donor. I did not know about the MELD score. We are from Canada and the doctors have never mentioned it. Whenever we ask if we are on the transplant list yet they say that my husbands liver is not in that condition yet. I don't know what to think. I will have to ask them about a MELD score. My husband's blood type is O negative...a rare blood type...he is only 25 at the moment and was diagnosed in 2009. Would you be able to share with us your PSC journey along with the syptoms that you had. We don't really know anyone with PSC and its always encouraging and interesting to hear from others. We are going to the PSC conference in Rochester the first weekend of May. That will be a first for us. It should be interesting as well. Thank-you for your response!
Sabine
Paul - you got a new liver!!! Congrats! Amazing huh?! cheers Deb