If you’ve never heard of it, that’s ok; a lot of medical professionals haven’t either. Primary sclerosing cholangitis is a rare disease that attacks and scars the biliary tree (the tubes that bile passes through in and around the liver and bile ducts). Eventually, the scarring causes cirrhosis of the liver, necessitating transplant. In some patients, it also causes cholangiocarcinoma, or bile duct cancer. There is no effective treatment yet except transplant, although there are some new and exciting drugs in trials. Most people with PSC also suffer from Crohn’s disease or ulcerative colitis, although no one really knows what the link is yet.
The most common symptoms of PSC are severe itching, fatigue, right upper quadrant pain, night sweats, and fevers, but as the disease progresses, more symptoms like malnutrition, ascites, jaundice, insomnia, edema, hepatic encephalopathy, and other fun stuff can occur. Some patients never progress that far and others experience liver failure soon after the onset of symptoms.
I’m pretty lucky in that I have made it almost twenty years since diagnosis and only had cirrhosis for a few years. Right now I am trying to get listed for transplant, which involves lots and lots of testing, and regular screening for cholangiocarcinoma. It’s both terrifying and exciting because it’s been so many years since I actually felt good and I have heard that life after transplant is amazing, but actually getting the transplant is not guaranteed and can take a long time.
A lot has changed in how this disease is treated over the last twenty years, though. When I was first diagnosed, I was told people with PSC weren’t good transplant candidates, and now we know that people with PSC have a great success rate with transplants. My first ob/gyn told me I had to abort my daughter or risk killing both of us, but I changed to a high risk specialist and it turned out that my symptoms actually improved during pregnancy. There’s no way to know what will come next but I can keep hoping.