I thought this was going to be a long drawn out process

My son is 19. He is a college student and played on his colleges soccer team this year. He was diagnosed with UC and possible PSC last year. Our Dr. at Mayo Clinic Said “as best as he can tell Phillip may have Small bile duct PSC”. He doesn’t have any strong indicators as of yet. His liver biopsy did not indicate clearly If he has PSC. They are basing his diagnosis on elevated liver enzymes and the fact that he has UC. He feels fine. His liver enzymes AST and ALT have returned to normal after being elevated for a year and a half. His ALK Phosphotase and GGT and Bilirubin have never been an issue. Phillip is currently taking Ursodiol and Azathiaprine…they have brought his ALT and AST to normal, BUT during Christmas Phillip started complaining of itching. His Total Bilirubin is 2.7. They repeated his blood work and I am waiting for results. Is this the beginning?..

His AST and ALT were normal…his Bilirubin came down to 1.8 but we are waiting for a Heptglobulin test. Does anyone know what this test is looking for?

HI Seuss, I am in the same situation. My son got very sick in May 2009. He is now 24 years old. If you read the PSC chat rooms, you will find that many sufferers have itching, and it seems to be a normal sympton if your son is diagnosed with PSC. The biggest problem with young men seems to be keeping them on the medicaiton. I am constantly looking over my sons shoulder to make sure that he is taking it. We will be having another "chat" about this soon. My son is not a drinker, and I think that was a really huge plus. You may find if your son turns out to have PSC that his sleep patterns are very disrupted. this is probably the biggest issue that we deal with on a daily basis. It makes my son constantly tired. He had to reduce his study load. He is working though as well. He has told me that it is his job to have the disease and my job to manage it. At this point, that is how we deal with it on a day to day basis. Hope everything gets sorted out for you and your son. All the best.

I do not know what the Heptglobulin is however I am familiar with the itching. The Ursodiol should help with that. Also there is Cholestyramine which can be prescribed which can also help with itching. The Ursodiol keeps the liver enzymes in check however is not treating the underlying disease. In my case, Celiac is the underlying issue, then UC, then my gallbladder necrotized, then PSC and a bout of bacterial cholangitis as well. However for awhile I have had no trouble with it. It is a highly vairable course.

Seuss said:

His AST and ALT were normal...his Bilirubin came down to 1.8 but we are waiting for a Heptglobulin test. Does anyone know what this test is looking for?

I was officially diagnosed with PSC about a year ago. I have had Crohn's for the past 48 years, although I was unmedicated and in remission for many years. The PSC diagnosis coincided with a Crohn's flare. I have not taken any medications for the PSC, but I have taken Prednisone/Immuran for the Crohns for the last 6 months. Also 7 months ago I began the SCD Diet to treat the Crohn's. Over the last 4 months my ALK Phosphotase, ALT, AST, Bilirubin have all either returned to normal or moved in the right direction and are very close to normal. I do not know whether to attribute this to the Pred/Immuran, the SCD diet, or both. And I am aware that this is anecdotal evidence, but my gastroenterologist who specializes in PSC was surprised and commented that he "hadn't seen that before." In any case, the SCD diet is an extremely useful support to my treatment and you and your son might want to look into it. People have had excellent results with it for a variety of inflammatory gut issues. And because he is so young he might very well have a more rapid response than some of us who have had the condition for many years. It would help with the UC and may very well help with the PSC as well.

PSC is a long drawn out process for many. The first signs are indeed elevated ALT and AST levels and life may continue without any other symptoms for some for up to a decade or even longer. UC may show up as one of the first real discomforts in 80% of the patients.

Adrian Mak

I was diagnosed with PSC in 2007 at age 59. I was healthy, fit and very active. They discovered it from an abdominal x-ray that showed my spleen was six times larger than it should be. I was told I had End stage IV liver cirrhosis caused by PSC which had also caused Ulcerative Colitis (in remission) and gall bladder disease. My gall bladder was removed in 2000, but none of the doctors recognized the underlying disease was PSC In 2007 they put me on Urso, Cholastyramine and Lactolose. I only experienced mild itching that came and went. I received a live donor liver on February 8, 2012, and am in perfect health today. Here is a picture of my donor's healthy liver and my diseased liver (red).



Phillips Haptoglobin test was normal...my doctor said we may have Gilberts syndrome, which can cause an elevated bilirubin. We are repeating liver panel next week.

Hi there, my son has PSC and autoimmune hepititis (most PSC people have a secondary disease as well as PSC) and he also has Gilberts syndrome. When he is taking all the medication, all his levels drop down to normal figures.

Seuss said:

Phillips Haptoglobin test was normal...my doctor said we may have Gilberts syndrome, which can cause an elevated bilirubin. We are repeating liver panel next week.

Gilberts syndrome will benefit him on a transplant list. I know it is not a good thing, but it is an exception to the MELD system. PSC does not enjoy that same exemption, but it should.

Hi Paul, I did not know this about Gilberts syndrome. Why does that make an exception for the MELD system?

Paul Hain said:

Gilberts syndrome will benefit him on a transplant list. I know it is not a good thing, but it is an exception to the MELD system. PSC does not enjoy that same exemption, but it should.

I spoke to Nay (the fiance. I got tired of writing 'fiance" I felt like THAT annoying girl. LOL.) about Phillip.

Nay's start to PSC was very similar to Phillip. A soccer player as well, he's energy levels dropped and that was a red flag for a needed visit to the doc. There were also some pains I believe. PSC was diagnosed. His condition was at a stand still, at one point for 2 years dormant with his blood testing well. He parents and doc thought they had beat the disease. A flare up proved otherwise. From 16 to 18 his parents maintained and controlled his meds. When he went away for college, Nay did not want to recognize the seriousness of PSC so he pretty much ignored the whole issue until about 23 when he was forced to take it serious as he was having problems with stones and flareups. Nay's advice, on living with PSC as a young guy, stay positive and active. A healthy diet is most important, that was Nay's number one point. Since he changed his diet, he's felt a world of difference in his level of energy and mood.

Keep us updated on Phillip's news. Prayers and positive thoughts! Xxx.

-A

I tried to research the reason Gilbert's Syndrome is exempted from MELD and got this answer. The MELD method was developed by Mayo in Rochester, MN, as the fairest way to allocate cadaver livers. It is based on how liver disease develops in alcoholics and thought to be predictive for all other liver diseases. The United Network for Organ Sharing (UNOS) along with the Federal Department of Health and Human Services administer the MELD decision-making. I was not able to find who was on their governing board so I could write a letter of appeal to them. They keep their identity very well hidden.They adopted MELD as the rule in 2001. However, they made an exception for Gilbert's Syndrome. My hepatologist thought that, although they claim to be apolitical and impartial, someone influenced that board of decision-makers.

I am a type "O" universal donor. When I was at Mayo for evaluation, they said my low MELD of 12 would persist until I had a liver failure I would not recover from (I had been hospitalized with liver failure many times, but always bounced back). Then, my MELD would shoot up within a day or two to 34-38. At that point, they would look for an available liver. "O" livers can be used by all other blood types, which further reduced my chance of getting one. If I survived those remaining few days of life, I would get a liver. I got sicker and sicker, exactly as people with Gilbert's Syndrome, but they get the liver, while I get to die. They said no exception would be made for PSC except cancer (see below).

You can look Gilbert's syndrome up on the internet. Bottom line: People with Gilbert's get sick and their MELD is low. They Get a liver, while people with PSC do not. The end-stage symptoms are very similar to PSC. My hepatologist's opinion was that there is no scientific reason why Gilbert's Syndrome is exempted and PSC is not.

About PSC and cancer. Mayo told me that bile duct cancer affects 15-20% of people with PSC (I don't mean to scare you), but is treatable. Here is what happens. AN ERCP biopsy indicates you have cancer. You are removed from the transplant eligibility list. You undergo Chemotherapy and Radiation for three months. This is very hard on you physically. Another ERCP is performed to insure all cancer cells are gone. After 6 months more, another ERCP is performed. If no cancer is found, you are placed back on the transplant list. You are awarded half the MELD points you need to be eligible for a transplant. Here is the math: your MELD is 11 and they are transplanting at 29. The difference is 18 points, so you are awarded 9. Your new MELD is 20. Every two months, they do another ERCP. If you are cancer free, you get 2 more points. If everything goes well, your wait will be eight months before you are eligible for a transplant. The treatment, wait, and point growth process will take about two years. If you develop bile duct cancer again, you go off the list and the process starts all over.

I had no faith that I would ever get a cadaver liver. I was steadily getting weaker and sicker. My transplant surgeon told me I would not live long enough to get a cadaver liver, and that I needed to go to a hospital that did live donor transplants and pursue finding a donor. Fear, tears, rage, reason, refusing to give up, faith, and trust in good lead me to my donor. I am a well man today, cured of PSC.
AussieMum said:

Hi Paul, I did not know this about Gilberts syndrome. Why does that make an exception for the MELD system?

Paul Hain said:

Gilberts syndrome will benefit him on a transplant list. I know it is not a good thing, but it is an exception to the MELD system. PSC does not enjoy that same exemption, but it should.

Hi Paul

Big thanks for explaining that for me. It is very interesting to see it how it all plays out. I am so glad you are free of PSC now.

Hi,

My son is 16. He has just been diagnosed with UC & PSC. He had all of the symptoms. The itching is a sign. Has he had a colonoscopy yet? This has all happened to us in the course of one week. We still don't have all of the results back yet. I don't know what stage he is in yet. We find out Monday. I would suggest calling or e-mailing his Dr. My sons Dr. has been great about replying to e-mails. I started journaling what triggers attacks in my son, his sleep pattern, skin, everything and anything I can think of to help aid in his treatment. I would have your son write down any symptoms even non related and take them with you to the next appointment. My thoughts and prayers are with you & your family.

Thanks…yes Phillip had a colonoscopy over a year ago. He is supposed to have another one this summer. His UC is under control, but he is having labs drawn weekly for his liver issues.

I have kept a detailed diary for a year and a half. We can’t see any pattern other than stress that triggers UC.

Thank you. I'm free of PSC, but I never want to forget what you are all going through. Perhaps, my experience can help others.

AussieMum said:

Hi Paul

Big thanks for explaining that for me. It is very interesting to see it how it all plays out. I am so glad you are free of PSC now.

Do write down all the details. You think you will remember, but they end up being just a blur. The more informative with detail you can be, the better your doctor can help.



■■■■■■■■■■■■■■■■■■■■■■■■■■ said:

Hi,

My son is 16. He has just been diagnosed with UC & PSC. He had all of the symptoms. The itching is a sign. Has he had a colonoscopy yet? This has all happened to us in the course of one week. We still don't have all of the results back yet. I don't know what stage he is in yet. We find out Monday. I would suggest calling or e-mailing his Dr. My sons Dr. has been great about replying to e-mails. I started journaling what triggers attacks in my son, his sleep pattern, skin, everything and anything I can think of to help aid in his treatment. I would have your son write down any symptoms even non related and take them with you to the next appointment. My thoughts and prayers are with you & your family.

I actually went through what Paul described with cholangiocarcinoma and being on/off the list. I am going to say a few things differently than what he said. For the most part accurate enough to give you an understanding of the process.

I thought I was healing from PSC when wild horses had to drag me to get an US and CT scan of liver. Huge denial of the disease at that point in my life - 2004. Diagnosed PSC - a tumor the size of a racquetball, infected inside, sitting completely in left lobe of liver. Was put in contact w/ transplant unit at U of Colo.

The chief of unit said I would be allowed a transplant if I could be free of cancer 6 months. But first they had to decide whether they would even work on me, and the infection had to be treated. So I had infusions every morning for 2 months to treat infection. It took the 2 months for U of C to agree to surgery.

They removed left lobe of liver and lymph nodes to biopsy. Very difficult recovery as they had to cut into heart and lung chamber to remove adhesed tumor.

Six months later I was still free of cancer (PET scans every 3 months). I met with them, was allowed to attend informational transplant classes, and told I would be allowed to be listed at the higher MELD. Exception was my case had to be reviewed by regional board Q 3 months. If there was a majority vote 'no' there would be no transplant.

My vote was always 3 yes, 2 no and 1 abstain. Basically called a split decision. National UNOS rule are that the patient gets the benefit of the doubt and so I was allowed to be listed. Every 3 months PET, reapplication to be listed, vote, and then I'd get the call from coordinator I was still on.

Once I requested to read comments. They were 'I don't think this person deserves a transplant' and 'I think this person should be free of cancer 2 years before they get transplant.'

I was called approximately 15 months after tumor removeable - they had a liver for me (still makes me cry).

May 25, 2006 I was granted life once again.

If you have questions email me @ tall ■■■■■■■■■■■■.

All the best Cheers Deb

Another story will begin again because PSC returned in transplant and another 1 cm tumor of cholangiocarcinoma was found in the orginal bed of the first tumor but was in the body wall not the liver. Patiently waiting to see how this will play out.

And Paul you lucky dog you! I know you deserve a transplant and doesn't it feel wonderful to feel alive and healthy again. Mine was only to short. I really hope I get the chance someday to feel that way again. Cheers Deb



Paul Hain said:

I tried to research the reason Gilbert's Syndrome is exempted from MELD and got this answer. The MELD method was developed by Mayo in Rochester, MN, as the fairest way to allocate cadaver livers. It is based on how liver disease develops in alcoholics and thought to be predictive for all other liver diseases. The United Network for Organ Sharing (UNOS) along with the Federal Department of Health and Human Services administer the MELD decision-making. I was not able to find who was on their governing board so I could write a letter of appeal to them. They keep their identity very well hidden.They adopted MELD as the rule in 2001. However, they made an exception for Gilbert's Syndrome. My hepatologist thought that, although they claim to be apolitical and impartial, someone influenced that board of decision-makers.

I am a type "O" universal donor. When I was at Mayo for evaluation, they said my low MELD of 12 would persist until I had a liver failure I would not recover from (I had been hospitalized with liver failure many times, but always bounced back). Then, my MELD would shoot up within a day or two to 34-38. At that point, they would look for an available liver. "O" livers can be used by all other blood types, which further reduced my chance of getting one. If I survived those remaining few days of life, I would get a liver. I got sicker and sicker, exactly as people with Gilbert's Syndrome, but they get the liver, while I get to die. They said no exception would be made for PSC except cancer (see below).

You can look Gilbert's syndrome up on the internet. Bottom line: People with Gilbert's get sick and their MELD is low. They Get a liver, while people with PSC do not. The end-stage symptoms are very similar to PSC. My hepatologist's opinion was that there is no scientific reason why Gilbert's Syndrome is exempted and PSC is not.

About PSC and cancer. Mayo told me that bile duct cancer affects 15-20% of people with PSC (I don't mean to scare you), but is treatable. Here is what happens. AN ERCP biopsy indicates you have cancer. You are removed from the transplant eligibility list. You undergo Chemotherapy and Radiation for three months. This is very hard on you physically. Another ERCP is performed to insure all cancer cells are gone. After 6 months more, another ERCP is performed. If no cancer is found, you are placed back on the transplant list. You are awarded half the MELD points you need to be eligible for a transplant. Here is the math: your MELD is 11 and they are transplanting at 29. The difference is 18 points, so you are awarded 9. Your new MELD is 20. Every two months, they do another ERCP. If you are cancer free, you get 2 more points. If everything goes well, your wait will be eight months before you are eligible for a transplant. The treatment, wait, and point growth process will take about two years. If you develop bile duct cancer again, you go off the list and the process starts all over.

I had no faith that I would ever get a cadaver liver. I was steadily getting weaker and sicker. My transplant surgeon told me I would not live long enough to get a cadaver liver, and that I needed to go to a hospital that did live donor transplants and pursue finding a donor. Fear, tears, rage, reason, refusing to give up, faith, and trust in good lead me to my donor. I am a well man today, cured of PSC.
AussieMum said:

Hi Paul, I did not know this about Gilberts syndrome. Why does that make an exception for the MELD system?

Paul Hain said:

Gilberts syndrome will benefit him on a transplant list. I know it is not a good thing, but it is an exception to the MELD system. PSC does not enjoy that same exemption, but it should. To answer your question more directly, I think Gilbert's Syndrome was made an exception to the MELD rule for personal, not medical reasons. I think PSC should be exempt as well, because it, too, does not register on the MELD criteria just like Gilbert's. It's not good that your son has Gilbert's, but it will probably make him eligible for a transplant so he can be healthy again. I wish you and him the best.