Scott’s Story

Update 7/25/11: I was transplanted on the fourth of July, and despite a few bumps, am recovering nicely.

Update 4/3/11: I've passed all the tests and survived the treatments thus far. I'll probably be listed for transplant very soon.

A couple years ago, I was diagnosed with Primary Sclerosing Cholangitis (PSC), a semi-rare condition which has no understood cause and no known cure outside of liver transplant. PSC causes a stricturing of the bile ducts which, in turn, causes bile to scar the liver. The rate of progression is variable, but liver failure is the ultimate result without transplant. Although there are some people who are long term survivors without transplant (15+ yrs), I am not going to be so fortunate.

As you can imagine, PSC does present some unique challenges. For instance, the skin is irritated by bilirubin--consequently pruritis (itching--often extreme and "under the skin") is common. Also, the liver stores energy, so fatique is a challenge as well. Pain, digestion problems, and brain fog are also common. The big fear, however, of almost all PSCers is the 15-30% chance of getting cholangiocarcinoma--a cancer that is otherwise almost unheard of (except in Asia where liver flukes also cause it). I have just been diagnosed with CCA.

Last September I was in the hospital for pancreatitis, a common side-effect of an ERCP (which is a procedure I need every six weeks now to change my stent) and they ran some tests which caused concern. Many tests later, and it's confirmed.

CCA is a "bad" cancer. Survival really depends on surgical excision. Excision of the cancer includes much of the surrounding area, including half of the liver. The rub: PSCers don't have strong enough livers to survive losing half. Consequently, a full liver transplant is about our only hope for long term survival, but often it is not possible. Most hospitals will not do transplants on a patient with cancer because the anti-rejection medications (immunosuppressents) would allow the cancer to flourish unabated.

Fortunately, I'm going to the University of Michigan, one of only a few places I know of that will consider transplantation with CCA. (The other places are the Mayo centers.)

The good news:
My scans have shown no other definite cancers.

The gray area:
I have cirrhosis and varices (from portal hypertension). These factors will help in getting me listed for transplant. That's good, as long as the liver doesn't get too bad and my arteries don't explode.

The scary:
I have some suspiciously enlarged lymph nodes they still need to biopsy. If they are cancerous, I'm out of the protocol.

After radiation and chemo, I have to go through a few more tests and scans, including some laporoscopy and biopsies, to check for any cancers that can't be seen. Again, any spreading of cancer, and I'm out.

And then, it's just a waiting game, hoping for that rare liver to become available. (Thank you if you are listed as a donor!) The average wait is six months at U of M. If we can keep the cancer contained, it could work out.

(Then it's just a matter of surviving the transplant, which is more difficult and dangerous than a heart transplant--but really, I'm not so concerned about that anymore.)

As you can see, it's precarious, but I'm in good spirits and have a lot of loving support. But hey, who can't use more love! Lay it on me!