Anyone with Sclerosing Cholongitis?

[Jack Crow]

Hello,
     I have recently been diagnosed with Primary Sclerosing Cholongitis (PSC) and am interested to find out if any one has experience with this disease. From everything I have been told and have read so far, it seems that these is nothing that can be done, but to wait until it gets bad enough to require transplant. My Dr. seems optimistic about transplant results for me, but estimates that it may be needed withing 2 to 3 years. If anyone has information or experience with this, I would appreciate some information! Thanks.

[imkindly]

I don't have sclerosing cholangitis, but we do have some information about it on our board.

You can go to
http://forums.delphiforums.com/liverfailure/messages

Then scroll down the column on the left side of your screen, and click on the (purple) topic "Primary Sclerosing Cholangitis"

That will make about 70 posts on that topic appear. Click on any that interest you.


P.S.- anyone can enter that board as a "guest" to read posts.
But in order to post any comments or questions there, you have to make up a name and password. (all free)

Hope this helps.

[Jack Crow]

Hi and thank you for replying. I went to this site and did find some nice posts, but on the left I only saw a listing for PBC. If there is something I missed, please let me know, and thanks again.

[Wools]

Jack, sorry to hear about your PSC.  I know how rare the disease
is and it has been my son's battle for almost 1 year. He is 23 and was in perfect health until one day in Oct 2000 when he started itching and turning yellow.  An ERCP to diagnosis the disease caused him to get pancreatitus (pancreatitis).  That put him in ICU for 63 days. After 3 months in the hospital he was discharged. He
lost 50 lbs. His PSC was confirmed. He not only had scaring of the internal bile ducts but also had a dominant stricture in the main duct. He was given a stint that allowed bile to drain outside the body and later converted to drain internal. They left the drain in outside incase the internal one plugged. He was put on a transplant list as soon as he was well enough to withstand surgery.  He was on actigal to help thin the bile.  He had the tube replaced 4 times in the next 6 months.  Our biggest concern was getting a liver in time.  In June of 2001 he received his new liver and is doing wonderful. We just celebrated his 1 year mark this week.  I don't mean to scare you by telling you all this. I'd be happy to answer any questions you might have.  You might find a transplant center and get on the list under Inactive.  Learn about how the list works and how to acquire time. That way if you start to get sick you'll have
time in your account built up.  The system worked for us and we feel very very LUCKY.

[imkindly]

Hi Jack

I think maybe you just saw the "General" topic there.

Delphi messageboards (like that one) are a little strange....because they have a seperate column on the left side of the screen that you can "scroll".

(It scrolls seperately from the section on the right.)

Hope this isn't confusing!  Give it another try.  

If you go there, you'll notice the narrow column on the LEFT side of your screen (only about 3 inches wide) has it's own "scroll".
If you scroll through that column, you'll see that there's 16 different PURPLE topics.

You need to scroll down (just that narrow column on the left!) to where you see "Primary Sclerosing Cholangitis" in PURPLE.

Give it a try.

[imkindly]

If you still have trouble with that,

I was experimenting just now to try to see if there was a way to give you a URL to just that topic (on that board).....and this one might work to do that:

http://forums.delphiforums.com/n/mb/list.asp?webtag=liverfailure&ctx=1048576&cacheTag=x19-1

[Jack Crow]

Thank you both for taking the time to help. Wools, maybe you can point me in a direction to find out information about building time on a waiting list like you mentioned. I have the feeling I am going to be fighting with the Dr.'s to get motivated. Unfortunately they don't seem all that worried about getting anything done at this point. Maybe the specialist will help, and the specialist is a part of the transplant team. Tell your son that I am glad to hear that he is doing well. Things are starting to worry me, but I have to trust God and let him handle things. I appreciate the information, and I have definately learned to value the good time I have now, as it seems things will not be so hot down the road. Thanks and let me know if there is information out there that can help.

[Wools]

Jack....After I posted the last comment I started feeling like I probably scared you to death.  PSC is such a rare disease and there are so many questions that arise.  They figure my son had is about 10 years before he got so sick.  I guess we were lucky in that we didn't know it existed until he was in liver failure.
You need to learn everything you can about the transplant process. There are two areas to learn about, receipants and donors.  You can learn about all the transplant centers through the UNOS website.  Read about the numbers and learn about the
time aquired and blood types on the center of your choice.
Do this before you get on the list so that you make a good choice
where the center is aggressive in doing lots transplants. We were at one center and waiting for a predicted 2 years. We then transferred to another that was more aggresive (aggressive) and got a liver in weeks.  Also learn about the procurement program that supports your center.  Go to the local organ donation organization and talk to them about the volume of organs they receive and see if they have an aggressive program in place.
This area is just as important as the transplant center. Some centers let you "double list" which allow you to be on two lists
at one time.  We did this against the wishes of one center.
You must make decisions based on saving your life even though it
means breaking some hospital rules.  The internet is full of
articles that help you make important decisions for your future.
We feel God has protected our son throughout this past year and a half. Once you make it through, you sure appreciate each day.
I now work with the local organ donation program because I know the importance of every single donor.This past week my son had his one year liver biopsy and they found no sign of rejection and so sign of reoccurance of PSC. My son is back to college and work and feeling great. May God bless you in your journey.

[imkindly]

Jack, the advice that Wools gave you is good advice (checking out centers, OPOs, etc.). Very good advice.

One thing though---

UNOS switched over to the "MELD" score system as of February 2002.

Highest MELD gets the next organ available.

Waiting time (time accrued on the list) no longer factors into who gets the next transplant.  (Waiting time is only used now to "break a tie" between two patients with the same MELD score.)

MELD was approved November 2001, and went into effect February 2002.

[Wools]

To Imkindly: Thanks for the new info. Where can I learn about the
new criteria for the receipients of transplants? Does UNOS still exist? What does MELD stand for? My how things have changed in a years time....

[imkindly]

Yes, UNOS is still in charge.
They came up with a new system to allocate livers. It's called MELD (Model for Endstage Liver Disease).

MELD uses 3 things to calculate a "score":
1. the patient's INR (from blood tests)
2. the patient's total bilirubin (from blood tests)
3. the patient's creatinine (from blood tests)

they also give extra points if you're on kidney dialysis.

The INR is a ratio that's related to blood clotting time. (INR rises, the sicker someone gets)
The bilirubin also rises, the sicker someone gets.
And when someone gets really ill, they can go into 'hepatorenal syndrome' (kidney failure caused by liver failure)....creatinine rises as kidneys fail.


UNOS put out a news release at
http://www.unos.org/Newsroom/archive_policy_20020214_3.6.4.4_notice.htm

UNOS has an online "MELD Score Calculator" at:

http://www.mayo.edu/int-med/gi/model/mayomodl-5-unos.htm



P.S.- Not everyone was thrilled with this changeover. There was alot of debate about it last year. There's some articles (from last year) at

http://organtx.org/tx/ctp-meld.htm

[Laurel's mom]

Hi my daughter has has primary sclerosing cholongitis for 6 years and had the whipple procedure done 6 years ago.   Her enzymes have been going up and down.  now her g.i. specialist is suggesting they are having good results using urso biosalts [bile from a bear!].  he feels she may be a good candidate, but he must still do further research. has anyone heard of this .. sounds drastic, but could spare her liver hopefully.   thanks!

[Laurel's mom]

Hi my daughter has has primary sclerosing cholongitis for 6 years and had the whipple procedure done 6 years ago.   Her enzymes have been going up and down.  now her g.i. specialist is suggesting they are having good results using urso biosalts [bile from a bear!].  he feels she may be a good candidate, but he must still do further research. has anyone heard of this .. sounds drastic, but could spare her liver hopefully.   thanks!