Living with PSC

My oldest son, Wil, has an interesting journey with his assorted health problems. It first came up when he was about 3 1/2 complaining of abdominal pain. So we went to the hospital ER (why do those things always happen on a Friday evening?) and he was diagnosed with AIH (Autoimmune Hepatitis). Doses of prednisone (a form of steroids) pretty much sustained him throughout his childhood, monthly visits to a gastroenterologist, getting punctured at least 6 times a year for blood work; his health pretty much was stable.
This is Wil eating lunch during our recent visit in Seattle
Until he was around 8 years old, he started to gain too much weight, basically his body was retaining too much water, probably due to the side effect of taking steriods for far too long. Our gastronologist pretty much gave up and referred him to a children liver specialist at Children's Hospital in Aurora.

This specialist has taken one look at him and said, "This is a textbook case of PSC." Now we as a family are relieved to know what exactly what the condition is.

Now that we got "PSC" on our minds, we can do more research on the causes and effect of PSC.
Here is what we know -- PSC stands for a mouthful and "spellful" Primary Sclerosing Cholangitis. In a nutshell, PSC is a condition that damages your liver over time due to improperly shaped bile ducts that causes cirrhosis of the liver. This is the same disease that took the Chicago Bears running back great Walter Payton's life too soon.

PSC patients usually has another acronym, in other words, afflation with some other disease such as Ulcerative Colitis, Autoimmune Hepatitis (AIH), Crohn’s Disease, diabetes, kidney & colon problems and so on. In his case, it is the dreaded Ulcerative Colitis (UC).

Anyway, thanks to that diagnosis, we were able to find a PSC Conference which happens to be in Denver this year! What timing! I attended the conference and wow, it was emotionally hard on me since I see other families going through it and seeing how they deal with it. PSC affects ANYONE at all ages, you may have PSC, but not symptomatic until later in life. Pretty scary, but it's similar to what I have read about with tuberculosis/consumption -- doesn't discriminate against socioeconomic backgrounds, nor a specific ethnic group.

The takeaway from the conference is basically you need to go “with the flow” as folks with PSC has a tendency to not remain focused/concentrated for long period of time, go through emotional and trying times due to the side effect of the medicine cocktail. Kinda ironic I said "cocktail" since alcohol damages liver. By my count, he takes 3 different combinations of 7 different drugs 3 times a day. He is definitely overmedicated, but it’s a relatively new diagnosed disease and all doctors are at a loss as to what to do.

How to cure PSC at the moment? Right now there is no cure, but of course people are working on it, however, I don’t think it’d be something that can be cured because once the bile ducts are damaged beyond repair, how can you fix it? However, we can find ways to do early diagnosis and quicker fixes so ducts don’t get further damaged. The best “cure” is liver transplant.

I’ve heard stories of liver transplants and with it pretty impressive 30-40 year survival rates. Technically, once you have a liver transplant and the liver is not rejected in the recipient, the quality of life would be improved remarkably. Still have to take meds to minimize rejection, but its only one med for PSC compared to the plethora he takes now. For UC, that’s more meds.

On a side note, thanks goodness we had decided to homeschool our kids otherwise he will be in and out of school and having interruptions through his day due to taking medicine and going for frequent bathroom breaks.

Right now at this time, I’m spending the weekend at the hospital because he seems to have high blood cell count, in other words, a possible Unitary Tract Infection, they’re not too sure yet... it is likely he has kidney infection which is common with Ulcerative Colitis.

I’ll try to keep you posted from time to time as my son progresses along in life fighting PSC. I wanted to add that I wouldn’t know what I would do without my wife in support of his condition. She is very diligent about making sure everything is in order, his meds, and watching his health like a hawk. Again, she is just great while at the same time homeschooling and caring for 2 kids during the day. Its nuts, but the days goes by so fast that it’s bearable. We’re just taking it day by day.