Primary Sclerosing Cholangitis

[Patti]

My husband has PSC, diagnosed in Sept/99....are there any advancements in the treatment of this disease?  He is on 1200 mg. of Urso daily, also taking milk thistle, vit. B complex, lecithin, multivitamins, alpha-lipoic acid, and SAMe....he took the naturals before starting Urso and saw a decline in liver functions, since starting Urso, his eyes seem to have a yellow tinge.  How long before Urso works?  He has been taking it for 6 weeks now.
We are desperate to find something to slow down the progression of this disease.  I've read that they are doing trials with oral nicotine, we are in Canada and can't find any clinical trials.
I'm sorry to ask so many questions but it's hard to find answers.
Thank you so much.

[wendy vincent]

my husband was just diagnosed with PSC on a week ago. he is having pain on the right upper quadrant of the abominal area and he feels cold are these syptoms side affects of the PSC? how fast does this disease progress?

[DenverD]

Many of your questions can be answered if you join (free) the PSC-Support group at www.eGroups.com or by sending a blank email to psc-support-***@****

[Cindy]

I WAS DIAGNOSED WITH PSC 3 YEARS AGO.  I WAS ASYMPTOMATIC UNTIL 2 WEEKS AGO.  IN DEC. I HAD A TOTAL PROCTOCOLECTOMY WITH ILEO-ANAL ANASTOMOSIS (J POUCH) FOR ULCERATIVE COLITIS.  I HAD THE ILEOSTOMY TAKE DOWN 3 MONTHS AGO TOMORROW.   2 WEEKS AGO, I STARTED ITCHING. I CALLED MY GI AND HE SAID HE DIDN'T THINK IT WAS LIVER RELATED BUT HE WOULD DO BLOOD TESTS TO RELIEVE MY ANXIETY.  WELL HELLO - ALK PHOS WAS OVER 700 NEARLY 800 AND MY BILIRUBIN WAS 2.8 NOT TO MENTION A FEW OTHER TESTS THAT WERE ELEVATED.  HE HOSPITALIZED ME THAT SAME DAY, PUT ME ON CIPIRO OVER THE WEEKEND AND THEN SCHEDULED AN ERCP. THE ERCP CONFIRMED A BILE DUCT BLOCKAGE.  THEY DIDN'T DRAIN OR STENT.  THEY PUT ME ON ACTIGALL AND CIPRO AND SENT ME HOME.  I WAS ON STRONG BENADRYL WHILE I WAS IN THE HOSPITAL, BUT THE ITCHING STARTED AGAIN ONCE I GOT HOME.  I AM SO FATIGUED, AND THE MEDICATION (I THINK) IS SWIPING MY APPETITE.  I HAD LOST OVER 25 POUNDS FROM MY COLON SURGERY AND NOW I LOST ANOTHER 2 IN THE HOSPITAL AND 4 MORE OVER THE WEEKEND.

I GET SO DISCOURAGED FROM READING INFORMATION BECAUSE NONE OF THE INFORMATION HAS PROVIDED ME WITH HOW TO COPE, WHAT TO PHYSICALLY EXPECT.  I DESPERATELY NEED TO TALK WITH OTHERS WITH PSC BECAUSE I JUST FEEL LIKE I HAVE BEEN CONDEMNED TO DIE WITHIN 2-5 YEARS.  ALL THE LITERATURE I HAVE READ IS LEADING ME TO EITHER THE MAYO OR CLEVELAND CLINIC.  I WENT TO CLEVELAND FOR MY COLON SURGERIES, BUT INFO SAYS MAYO HAS MORE EXPERIENCE WITH LIVER PROBLEMS.

[Dick A. Hawkins]

Hi Lisa and Patti,,
I believe PSC reoccurs in approximately 20-25% of people who are transplanted, including myself. I'm six years post-transplant, had a tube placed in my abdomen after one year, and am presently having my bile ducts dilated every four months through that tube. I play tennis twice a week and am looking forward to the 2000 U.S. Transplant Games in June. My complete story, including my wife's story, is on the Web: http://www.sadiehawkins.com/liver.htm

Urso thins the bile so it flows better and therefore lowers liver function tests. It is, however, no cure. I am aware of nothing in the near future which will eradicate this terrible disease.

[Jim Hines]

What wonderfully upbeat people you all are. My three-year-old son has been diagnosed with PSC for the last year now. I feel for you all, and your families. There is a terrible lack of research on this silent killer and while there is some knowledge about the disease's effects in adults, there's very, very little in children.

Additionally, given that to my knowledge, no one really knows what the true cause of the disease is, you (and we) can only treat the side effects of the advancement.

There is a site called e-groups.com which hosts a PSC support group. It's very active and usually full of reasonably good information. However, many different medical opinions are needed I believe, when dealing with this stupid (*)(#$)(*#$) disease. 14 months ago, I didn't know PSC existed. And although I wish I'd never heard of it, anyone dealing with it (either personally or family-wise) is a great deal stronger than the average bear.

Please feel free to drop me an e-mail at ***@**** if you would like to chat about PSC and its implications. Not that I have any knowledge or miracles, but sometimes a good listening ear is worth almost as much as good medical texts.

Reagards,

jim

[Kelly -To Wendy,Eric and Patti]

Please write, I am here to help....  I have a bookshelf full of information.  And, at this time, I am on sebatical, so I have extra time to help.  Kelly  ***@****

[kelly]

Dear Eric,  don't give up.  It is a hard road, but, there is alot of hope!  Like any disease, there are going to be good days and bad days.  And I know what you are dealing with since I have a spouse with the same disease.  Have you had a second opinion yet?  We had a couple of doctors that said that they could not perform an ERCP because of the narrowing bile ducts.  But, it can be done by a VERY skilled GI doctor.  And, if they can not perform an ERCP, then the doctor should look at doing what is called a PTC.  It is a very common procedure and I have material on the procedure if you would like me to fax it to you.  We have one of the top transplant doctors in the US.  And, we have also did enough studies on the topic, that we feel like doctors.  I am also a volunteer for the Neb. Health systems organ recovery dept., so needless to say, I have a wealth of information and contacts if you would have any other questions...Here to help.
Kelly

[Kelly Hirsh]

Message to everyone that has had questions.  I am here to help
as best that I can!!!  For the last four years, I have studied
PSC.  My husband became ill in 1996.  We were sooo frustrated.
No one knew what was wrong with him for months.  We have been very fortunate to have some of the best medical personel in the midwest!  My husbands doctor is one of the transplant doctors in the US.  My husband has also been to the mayo clinic to research alternative drug treaments.  At this time my husband is feeling very well.  He refuses to be ill! Good for him, but, I,on the other hand am a worry wart.  To make myself feel better, I study this PSC.  I also volunteer for the organ recovery dept at NHS in Neb..  This is the treatment and procedures that he has been through so far:  Actigal (first two years), Benidryl, ERCP, PTC, and now he is taking cholestyramine light.  The cholestyramine is working very well.  We are also waiting for the FDA approval
on a new drug (not new, but new for pre transplant) called
"cell-cept".  The Mayo clinic is now finalizing the "red tape" for that program and we are hopeful that he will be able to start that soon...  P.S.  I would like to visit with the doctor(
HFHSM.D-Gastro RF!!!!  You, whoever you are, did not even give one piece of encouraging advise to anyone that had questions.  Are you really "up" on this?????  P.S.S.  I am not a doctor by no means, but, I also believe in alternative meds.  My husband has taken Milk thistle and loves it, but, with the new cell cept program, the mayo wants his whole system to be clear, so to speak. Write me at:***@**** if you need help with your questions!  God Bless

[Eric Harner]

I think the time is near, but our doctors
refuse to guage how far along she is. It
is so frustrating, my wife is a 29 year old
special educaton teacher, never drank or
smoked. And all we can get out of our
doctors is that her byle ducts are thread like
so much that they can"t even perform the
ercp procedure. Now we are dealing with
what the doctor believes is esophagual spasms.
She gets these horrific stomach pains, that
make it's way around her back. For the past
3 monthes every other day she gets these
terrible aches.She recieved 4 different
scripts from the doctor and he told us to
keep a diary of what worked, only thing is
nothing is working. Is their anything at all
I can do for her,reguarding this awful pain,
and reguarding this terrible disease PSC ????

[Wendy]

answer to Eric,
this is new for my husband and i also.
what our physician told us is that the liver will have to be about 80%non functional before he will be able to be on the transplant list. he is about 20-30%non fuctional now.
Has she had a procedure called E.R.C.P? if not the doctor should be able to tell aproximately has advanced the disease is.
our doctor is giving him 10-15 years before any significant changes will occur. but i think it progresses diffrently for everyone even though i have been told it moves slowly.

[Kathy]

My mother had a liver transplant Nov. 27, 1999.  She had PSC.  The ducts are narrowing again and she is going back on the transplant list.  The disease took approx. 15 years initially.  This time it's only 4 months.  What should we expect?  Rapid decline?  Stabalization possible?  Any info. is appreciated.

[Olga Cornell]

I was told I had PSC in 1986 I have not had any symtoms so far four years ago my doctor at Cleveland Clinic told me I was in the first stage of cirrosis with grade 1 Verices in the esophogus I have just had my annual check up with him Liver scan endoscope and colonoscopy (colitis) and the verices are now grade 2 I have taken actigall for the last four years and now he wants me to take blood pressure medication I feel fine I am 56 I keep myself in shape by exercising three times a week  I am hoping this will keep me as healthy as possibly untill I will be on the transplant list I am not afraid of having a liver transplant but I am afraid of being very ill and not being able to do the things I like (travel,garden,cook,take care of grandchild etc.

[Eric Harner]

My wife has PSC, and I was wondering how
far along the disease has to be before
she goes on the transplant list? These are very
trying times, and I just would like to know
how to guage just how far along she really
is?

[Eric Harner]

My wife has PSC, and I was wondering how
far along the disease has to be before
she goes on the transplant list? These are very
trying times, and I just would like to know
how to guage just how far along she really
is?

[Lisa Baird]

I had a liver transplant 6 months ago and I was wondering how long the average transplanted liver lasts and how common it is for the disease that caused my liver failure(psc)to reappear?

[HFHSm.D.-Gastro-rf]

Dear Patti,
Iam sorry but there are no new medical therapies for primary sclerosing cholangitis.  Urso remains the only drug given by many liver specoalists.  BY now your husband should have seen some change in liver tests with treatment.

There are reports in the surgical literature of surgery to relieve some strictures and of liver transplantation to improve the patient.  Decisions regarding these approaches are best made by the doctors who have access to the entire medical record