What is primary sclerosing cholangitis anyway?

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.

incurable

Old friends used to make jokes about me being incurable.  At the time, I was young, I had just been diagnosed, and although I had dropped out of school to have lots of surgery and try to recover, I still had the optimism required to see the humor in it.  I had been told I wouldn’t live much past thirty if I made it that long, so I took up smoking and reading full-time, since I loved both and didn’t expect lung cancer to be an issue.

Going back to school seemed ludicrous, since it was unlikely I’d ever be well enough to work outside of the house and even graduating didn’t seem like that realistic of a goal – my last semester I watched my grades fall as I stopped to puke in the bushes nearer my apartment each time I tried to walk to school.  There was no amount of accommodations that made my GPA seem salvageable, and the situation seemed so absurd that I didn’t even protest when my first husband enlisted in the army and asked for Germany as a first duty station.  If I was going to devote the rest of my life to trying to raise my daughter, puking in bushes, and chain-smoking, I couldn’t think of a better place to do it than Europe.  Primary sclerosing cholangitis was relatively unheard of, so when we were asked during in-processing if it would require me to be in the exceptional family member program, I crossed my fingers and said no.  There was no treatment anyway, so I could be incurable and untreatable at least as happily while seeing the world as I could in Houston.

A decade and a half has passed since then, and while I have developed many strategies for avoiding random acts of yard sabotage, there is still no cure for primary sclerosing cholangitis.  There is still no treatment, although there are some in trials that look promising, and to borrow a line from Monty Python, I’m not dead yet,

so I have gone back to school (and given up smoking).

Hopefully, at some point I can trade in being a professional student for some sort of writing or editing work that can also be toted around in a backpack and worked on from any bed I happen to be making a desk, whether it is mine or in a hospital, or on the other side of the world.  Alternately, I’d settle for a cure, a liver transplant, or a miracle.

Friends look nervous these days when I refer to myself as incurable, although I usually mean my sense of humor, or optimism, or some other impractical habit.  But the optimism seems to be as accurate as any prognosis I have ever been given, so I’m going to ignore the ending of his story to close this introduction with one of my favorite heroes of impossible situations: