Re: New to PSC (but learning)

December 11, 2004

Welcome to the group and I'm sorry for your diagnosis.

I know that this is a hard time for you but this group will help you

anyway it can.

You are joining at a time when I feel very optimistic as we are

currently working on starting a foundation to promote research for

treatment and a cure. I know how devastated you must feel to be joining

us as I just joined in June when my college senior was diagnosed with

UC/PSC . I have been very emotional and feeling hopeless until I

started to work on this. We are only just beginning but I want you to

know that you should keep thinking positively and know that this group

is not only here for support but also to be proactive in helping each

other. Feel free to ask questions,

Lee mother of Bill 22 UC/PSC 06/04

>

> As a new member, please allow me to introduce myself. I am, at the

> time of this writing, a 45-year old male, married 20 years, father

> of four, who is about to be diagnosed with PSC.

December 11, 2004

Dear ;

Welcome to the group. Sorry that your pending(?) PSC diagnosis brings

you here, but glad that you found this group. The ERCP will probably

be the most definitive test .... and might allow for exclusion of

other causes of bile duct obstruction.

I don't think that anyone can say for sure how long anyone has with

this disease. It seems so variable in its rate of progression between

individual patients, and seems to be characterized by intermittent

remissions and flares for which there is no obvious explanation.

There is a variant of PSC called small-duct PSC that seems to have

much better prognosis than large-duct PSC. Cases of long-standing

asymptomatic PSC have been well documented:

__________________________________

Dig Dis Sci. 1981 Sep;26(9):778-82.

Long-standing asymptomatic primary sclerosing cholangitis: report of

three cases.

Chapman RW, Burroughs AK, Bass NM, Sherlock S.

Three patients, two males and one female, with asymptomatic primary

sclerosing cholangitis (PSC) are described. The diagnosis was made in

each case by endoscopic retrograde cholangiography after

investigation of persistent elevation of the serum alkaline

phosphatase. All three have remained completely well without any

medical or surgical treatment for 3, 7, and 15 years, respectively,

despite extensive involvement of the biliary tree. Follow-up liver

biopsies in two have shown no histological evidence of progression to

secondary biliary cirrhosis. PSC may occur more frequently and may

follow a less severe clinical course than previously recognized.

Publication Types:

Case Reports

PMID: 7285744

__________________________________

Our son was diagnosed with PSC as a result of routine blood work that

showed elevated ALT, AST, alkaline phosphatase and GGT. Ultrasound

and a liver biospy were not immediately helpful in diagnosis. Only an

ERCP was able to confirm the diagnosis. He developed itching some 6

months after diagnosis, but has found relief from itching with a

prescription of rifampin. He is currently taking ursodiol, rifampin

and asacol for ulcerative colitis (UC). [uC was found only after PSC

diagnosis, which then prompted a colonoscopy, because it is well

known that 70 - 75% of patients with PSC also have inflammatory bowel

disease].

My wife am I are scientists/biologists by training and have spent a

lot of time gathering information on this orphan disease and its many

complications. We've started a web site that represents a continually

growing collection of articles on PSC and related disease. This might

save you some time in hunting for information:

http://www.psc-literature.org/

Let us know if you have any questions.

Best regards,

Dave

(father of (19); PSC 07/03; UC 08/03)

December 11, 2004

Dear D.

Welcome to the group! I was diagnosed in October and have recently

done the tests you referenced and some others since July. Having a young

family (a 2 year old) and being a old parents (I'm 43) does lend itself to

many questions. This site also has an excellent library & I'm sure you will

want to visit it if you aren't already. In terms of elivated liver tests,

this isn't really such a big deal. Mine are all 10X normal limits. The

predictive factors are those that go into your MELD score. If there is a

piece of good news about this disease, it is that for the most part it moves

slowly, giving folks 5, 10, even 20 or more years before a transplant is

necessary. Lots can happen in 20 years. Liver transplants for example were

almost non existant 20 years ago, and for those few who got them, terrible

long term survival rates was the norm.

Take information in as you can, but try not to overwhelm yourself

with information. There are other engineers in this group, and load of

helpful, kind souls. Let us know how we can be of help.

Best Holiday Wishes

jd

UC 1973, Jpouch 2000, Pouchitis 2001, PSC 10-04

ston City IL

krmpotich@...

New to PSC (but learning)

As a new member, please allow me to introduce myself. I am, at the

time of this writing, a 45-year old male, married 20 years, father

of four, who is about to be diagnosed with PSC. For 14 years, the

only indication that something might be amiss was the presence of

elevated liver enzymes on routine blood work. However, for almost

two months now, I have been itching and have experienced some other

symptoms. An ERCP (next week) is the next step in getting a

definitive diagnosis, but this is what has gone on so far:

In 1990 I had my first blood work as part of an employment

interview. It showed GGT at almost twice the normal high. Four

years later, upon switching jobs, I again had my blood tested. This

time, GGT was three times normal, while ALT and AST were mildly

elevated. Follow-up tests by my GP over the next year ruled out

hepatitis, hemochromatosis, and 's disease, while showing GGT

to be persistently elevated.

In 1996, my employer started a voluntary wellness program,

offering annual fitness tests that include blood work. I was out of

shape but became (much to my own surprise) devoted to jogging. To

this day, I derive great satisfaction from working up a sweat for 30-

45 minutes, every other day or so. This has led to great HDL

numbers, a low resting pulse, and (my proudest and vainest moment)

an assessment of a " body age " of 27 when I was actually 43 years old.

So much for the good news. From 1996 forward, my GGT has

never been below twice normal, and ALT, AST, and AlkP became

increasing elevated, though never in a predictable pattern. Indeed,

readings of as much as five times normal have alternated with almost

normal numbers.

In 1997, I saw two hepatologists and had a CT scan of my

abdomen (normal). Both held out for the possibility of PSC, but also

agreed that there was time to wait and see, considering that I had

no symptoms and appeared to be in otherwise excellent health. In

1999 I saw one of them again. I was advised to come back for a liver

biopsy should my blood work show significant increases in liver

enzymes. That happened in 2001, but I did not go back. By this time

I had developed an " aw shucks " mentality about the whole thing.

Numbers be damned, I felt fine, didn't I?

This cavalier attitude was allowed to last until two months

ago. It struck me one day that I was constantly itching. Also, my

appetite was suppressed, food didn't settle well on my stomach, I

experienced mild chills, I had more frequent bowel movements (they

were also looser and of a lighter color than normal) and I was more

tired than normal. I lost a surprising ten pounds in about two

months.

When I went to see my GP, he ordered additional blood tests,

showing negative AMA, ANA, and ANCA. An ultrasound of the liver was

normal as well, with no evidence of bile duct dilations. I was put

on ursodiol, which may or may not have helped much so far. And, of

course, I was referred back to a specialist, a new one this time.

The specialist's observations: To his trained eye (and aided

by the special lighting in the examining room) I showed slight

jaundice and signs of recent muscle wasting. He compiled a

considerable list of things I do not (yet) have. The bad news: " My

impression is that the patient has chronic liver test abnormalities

recently becoming more cholestatic. I suspect that he has primary

sclerosing cholangitis. " So on to an ERCP I go, " for confirmation of

diagnosis and to exclude any remediable cause of bile duct

obstruction. " (Hmm.. Could we please retain remediable causes?)

Also, because I am not showing immunity to hepatitis A and B, I am

to get inoculations against those as a safety measure.

All is not bad: I still enjoy all of my meals. I am still

capable of my every-other-day jogs. I am sleeping well (and longer)

for the most part. And I especially enjoy spending time with my

family, but for how much longer? That is my big question: Am I

looking at one year, three, ten? I have many other questions, and

have started digging through the thousands of posts from this group.

Professionally, I am a scientist by training and am employed

in an engineering field. For the time being, I have decided to

correspond under an assumed name, because I am only at the beginning

of assessing the impact this all is going to have on my life, my

family, my career, and my insurance coverage. But, I look forward to

getting to know people in this group.

Drukker (jdruk59@...), December 10, 2004

December 13, 2004

Welcome ,

Sorry I'm so late answering and that you have reason to find this

group. Everyone reacts differently to Psc, but since I see a couple of

parallels in our stories, i will tell you some of mine. The first i knew

anything was wrong was in 1974 when i started to itch uncontrolably. i was

33 and, like you, had 2 little kids. Long story short, it progressed slowly

until my tx in Aug of 2002. i kept getting more and more fatigued but did

not recognize that or other things until after the fact. All of a sudden it

dawns on you that you are too tired to work or play the way you always have.

I was mis dx for a number of yrs until the itching and fatigue was getting

the better of me and i went to a liver specialist, where I wish i had been

yrs earlier. So, there is hope that this will progress slowly for you and

the knowledge about PSC keeps growing.

Do you have UC, IBS or Crohns? Tim L Original Message -----

To: < >

Sent: Saturday, December 11, 2004 7:18 AM

Subject: New to PSC (but learning)

or the most part. And I especially enjoy spending time with my