What can I expect to go through?

[Guest guest]

I am a 25 year old male who was diagnosed with PSC in Feb 2005. I

was diagnosed with UC in 2000.

In Jan 2005, I was on a cruise and had flu like symptoms, but they

weren't totally normal symptoms. I was very tired, had night sweats,

and had really dark colored urine. When I saw the doctor, he gave me

a blood test, and all of my liver enzymes were about 2-5 times

normal. My GI doc did an ERCP and ultrasound. The ultrasound showed

nothing, but he was unable to tell from the ERCP. He recommended

that I go to Mayo to be checked out. I went up there and saw Dr.

Talwalker who specializes in PSC. When they did an ERCP, they were

certain that I had signs of PSC. They said that there wasn't much

scaring but that there was some. Dr. Talwalker wanted me to enter a

study that he was conducting. However, when they gave me a blood

test at a later date for the study, all my liver enzymes were

normal. He said that I was not a candidate for the study since they

would not be able to tell if the drugs were helping since my enzymes

were normal. My treatment plan is to get a liver ultrasound and a

colonoscopy every year, to get blood tests every six months, and to

get an ERCP every 5 years. Since all of my liver enzymes are normal,

my doctors are not sure when the disease is going to start to

progress. From what I have read, it is several years from when

symptoms first show up to needing a new liver. What can I expect

during those years? Will I have those flu like symptoms most days or

will I feel fairly normal? I am also curious how long people live

with this. I have read that the average time is 10 years. However,

I have also read that with a liver transplant that people can live

completely normal lives from then on. I am hopeful that since I have

a contact at Mayo that when I need a transplant that the doctors

there will do it. I also hear that survival rates of transplants are

getting much higher. Thanks for any information.

[Guest guest]

All good questions - let me see if I can get a start on them.

Elevated LFTs followed by " normal " LFTs is not particularly uncommon, and is best characterized by calling it periodic sludge flow/blockage. When the bile is thinner (whether by Urso, hydration or some other means), there are no symptoms. When it thickens, it is difficult to pass through our already narrowed bile ducts, resulting in the symptoms you observed. This happened to me in the spring of 2000, and after an unsuccessful balloon dilatation (and subsequent pancreatitis), I was asymptomatic for a year. The 2nd cholangitis episode got me a trip to Mayo, where they successfully dilated and stented my CBD. Eventually, it may get worse where further intervention is needed.

The protocols for medical studies are frequently arcane to us, but in this case it wouldn't have helped them at all, since you're asymptomatic.

Your treatment plan is consistent with mine in all respects.

Longevity numbers (with PSC) are all over the place. The average time you quote is the number most frequently given for time to transplantation from diagnosis, not death. We have people in the group that have had PSC for 30+ years and are still doing well.

I'm sure our transplantees will field your transplant questions, but most do live a normal life. The troubling part is the rate of recurrence of PSC after transplantation. Again, numerous rates are in the literature, from 5 to 25% (if I remember correctly).

One thing to keep in mind is that there is pretty strong evidence that PSC is much more prevalent than observed, with the idea that many are totally asymptomatic, and may stay that way.

Please check out the FAQs at http://www.psc-literature.org/FAQ.htm for a good background. Sounds like you have a lot of the facts already - keep asking the good questions!

Arne

56 - UC 1977, PSC 2000

Alive and well in Minnesota