to imkindly and anyone else that has knowledge of cryptogenic liver disease

[ctc]

Hi, I am new to this forum, but referred to a website that you had made in the past. But I can't get on it anymore, something different comes up when I click on my "favorites" saved site. It was a support group for patients with cirhosis (sp?) Has the address changed? - I have so many questions that I don't know where to begin. 3 1/2 years ago we discovered that my son had an cryptogenic liver disease. at first they thought it was cancer, then they were sure that it was cystic fibrosis now they are saying that it is a strange variation of psc or that it will probably develop into psc in time. he also has overlapping symptoms of pbc. He has cirhosis,(the damage is in the area around his bile ducts) his pancreas is damaged (fibrosis) his spleen is damaged somewhat & he has chrohn's or colitis. three weeks ago he was in the hospital with a hemoglobin count of 6.9, he has had one iron shot and three iron infusions since. His doctor has said in the past that nothing about his disease makes sense or follows a normal pattern. he takes 35 pills a day. his imuran is now up tp 250 mg. They say his body metabolizes it so fast that it needs to be that high. He has portal hypertension, varices in his esophagus and colon. How do we know if his disease is being treated correctly? ctc

[imkindly]

Hi
We still have the support group. It's a Delphi board. Delphi modified the URL (address) on us a couple of months ago.
Here's our new address:

http://forums.delphiforums.com/liverfailure/messages

If you go there, scroll down the column on the LEFT side of your screen, and click on any (purple) topic that interests you.

(We have topics like "Primary Sclerosing Cholangitis" (PSC)...."AIH - Autoimmune Hepatitis"....etc.)

Clicking on a topic, will make a whole list of messages on that topic appear.
Click on any that interest you.


~~~~~In order to post any messages of your own on the board, you'll need to create a nickname and password. (It's free.)


PSC and PBC resemble each other in alot of ways. They both belong  to a group of liver diseases called the "cholangiopathies". (diseases in which the cells that line the bile ducts, cholangiocytes, are affected.)

Here's some general information about PSC that may help:
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
Below is part of an article taken from
http://www.pathoplus.com/cholangi.htm
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
PRIMARY SCLEROSING CHOLANGITIS (PSC)

Both Primary Biliary Cirrhosis (PBC) and Primary Sclerosing Cholangitis (PSC) are similar in that they have an autoimmune origin, but they are different diseases, involving different etiologies and inflammatory pathways.

Interestingly enough a major difference lies in the populations most at risk for each of these two autoimmune cholangitis’s.

PBC typically occurs in middle aged females, whereas PSC primarily affects young men with inflammatory bowel disease.

With PSC 70% of those afflicted are men and 70% have co-existing inflammatory bowel disease, usually ulcerative colitis.
The average age at diagnosis for PBC is 40.

In PSC the inflammatory process of the bile ducts is chronic in nature, it results in the destruction of the bile ducts, fibrosis of liver tissue with subsequent development of cirrhosis.
PSC is often complicated by recurrent episodes of bacterial cholangitis.

Patients with PBC have an increased risk of cholangiocarcinoma (bile duct cancer).

The symptoms include pruritis, jaundice, fatigue, fever, weight loss .
Often the disease is only discovered when a blood test reveals elevated levels of serum alkaline phosphatase and gamma-glutamyltranspeptidase on testing for other reasons.

The definitive diagnosis is made by endoscopic retrograde cholangiopancreatography (ERCP) which reveal characteristic findings that can distinguish PSC from other cholangiopathies (for example PBC).
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

Both can lead to cirrhosis.
Some of the things you mention (portal hypertension, varices, low hemoglobin) are caused by cirrhosis.
You also mentioned spleen problems (if you mean an 'enlarged' spleen---that would be caused by the portal hypertension).

If I can be of any help to you, let me know.
I'm not a doctor, but we can try to locate information for you, from medical websites.

Feel free to visit the support group at
http://forums.delphiforums.com/liverfailure/messages

[Wools]

First let me thank imkindly for the websites for more info on PSC.  Our son also had PSC diagnosised at age 22.  We watched his health fail daily, weight loss, jaundice, pancreatitis, portalhypertension, cholangitis. We went to one transplant center that was not very aggressive.  We were advised to take him elsewhere where they would be aggressive in getting him a new liver. The wait is so long on the list that we wondered if
he would make it. The average wait is 9 month to 2 years. That is for a status 2B on UNOS list.  Go to the UNOS website to learn about the centers and the wait. We transfered our son to a center in Florida and he had a new liver in weeks. It has been 8 months and he is doing wonderful. He is back to college and work.
We were advised to be aggressive for a liver transplant because of the chance of colon cancer and repeat bouts of cholangitis.  Looking back it was a good decision and hopefully the worst is behind us.  If you have any questions about PSC or the transplant please feel free to ask.