Re: Just diagnosed

[Guest guest]

> My partner has just been diagnosed with PSC.

Welcome to the group. You will probably learn more than you bargained

for in this group and some of the information can be scary. Not

everyone needs a transplant and not everyone has every symptom or

complication. THe fantastic thing is that this group is very active

and very supportive and if they can't answer every question, they can

help you find it. AS for marriage and kids and all things wonderful,

live your life for each moment (but take care of your body), no regrets.

Kate in Seattle

Caregiver/Partner for Deb

PSC UC Jpouch listed for TX 2/2002 and currently in the ICU for a GI

bleed. Meld score creeping up to a 17!

[Guest guest]

The only constant with this disease is its inconsistency. I lived a

fairly symptom free life for about four years before having real

problems and ultimately being listed for transplant, but am currently

doing well and aspire to keep my god-given liver as long as it wants

to hang around. Many here have gone much longer than that, some for

more than twenty years.

Please don't panic. First diagnosis is a very scary time. There's

not much optimistic information about PSC available on the net. It

paints a pretty grim picture. However, the fact that you've found us

will give you a much more realistic view of the disease. Make no

mistake, it is a serious condition which CAN lead to transplant, but

it's certainly not an automatic death sentence.

My third child was born after my diagnosis (she's two now) and I fully

intend to be around for her graduations, wedding, etc. If your

partner has done well adapting to his UC, then he can certainly adapt

to the PSC. In fact, many people find their IBD to be more difficult

to manage than their PSC.

I guess the point I'm trying to make is that you can't let the disease

define you (or him) and you can't let it dictate your life. Sure, at

some point it may dictate some limitations, but don't automatically

accept them. I highly recommend Klug's book " To the Edge and

Back " and also suggest you do as much reading on the disease as

possible. The definitive resource for PSC info is Dave ' PSC

Literature website (www.psc-literature.org). Dave is a frequent

poster here, co-founder of the PSC Partners Seeking a Cure Foundation,

and all around good guy.

Feel free to ask any questions you may have as well. The first

casualty of this disease is modesty. We've all been poked, prodded,

stented, biopsied, and examined so much that no procedure has been

invented yet that someone here hasn't been through. We'll all be glad

to share our experiences at any time.

Peace,

Bill Wise

PSC '00, Listed Tx 11/04

[Guest guest]

Being just diagnosed is a scarry thing. Some of your husband's story sounds like mine. My blood tests were first screwy when I was 26 or 27. Since then, I got married and have two beautiful daughters and have not had any problems caused by PSC.

Once you come to terms with the diagnosis, get back to living your life. The last thing you want to do is let PSC tell you how to live and if you should have kids. What if 25 years from now, your husband wakes up still feeling good and you missed out on the best joys life has to offer.

Fight back with URSO and supplements and tell PSC not to bother you - you got better things to do now. I know it is easier said than done but you'll find the ability to do it.

in Clevelandpsc7654 wrote:

My partner has just been diagnosed with PSC.

[Guest guest]

Sorry to hear about your partners PSC diagnosis. I was first

diagnosed in 1989 and it was quite a shock. I vowed not to let PSC

run my life, not completely anyway. I have had two sons since my

diagnosis, in 1991 and 1994. I have also had two liver transplants

since then, 1997 and 2004. There is nothing scarier then having major

surgery when you have young children, but they also help give me

strength to want to live everyday. Don't let PSC stop you from doing

what you want. No one can predict what can happen and take the good

with the bad. Good luck and best wishes.

PSC 89 tx1 97, tx2 08/04.

He is only 25 and I am very worried about the long term

> prognosis. We want to get married, have kids etc - how is PSC going

to

> affect his life? And will he definitely need a liver transplant?

> Thanks for your advice.

[Guest guest]

I am sorry to hear of your partner's diagnosis of PSC, but I'm glad

you found the group. I hope we can answer some of your questions.

There's a form of PSC called " small-duct PSC " , where the liver looks

normal in the ERCP, and I'm wondering whether this might fit your

partner's situation, since you mentioned that his liver

looks " beautiful " ? Apparently, small-duct PSC has a much better

prognosis than " classic PSC " (which affects the large ducts):

Hepatology. 2002 Jun;35(6):1494-500.

Small-duct primary sclerosing cholangitis: a long-term follow-up

study.

Angulo P, Maor-Kendler Y, Lindor KD.

Division of Gastroenterology and Hepatology, Mayo Clinic and

Foundation, Rochester, MN 55905, USA.

Some patients with inflammatory bowel disease (IBD) have chronic

cholestasis and hepatic histology compatible with primary sclerosing

cholangitis (PSC) but normal findings on cholangiography. These

patients with small-duct PSC have remained largely unstudied. Our aim

was to determine the prevalence and long-term outcomes of patients

with small-duct PSC. Eighteen patients with small-duct PSC (7 female

and 11 male patients; mean age, 39.9 +/- 15.3 years [range, 13-68

years]) seen over a 4-year period were matched blindly by age and sex

to 36 patients with classic PSC and followed up for 32.5 years. Small-

duct PSC represented 5.8% of patients (18 of 309) with sclerosing

cholangitis. Subsequent endoscopic retrograde cholangiography (ERC)

performed in 5 patients with small-duct PSC showed progression to

typical PSC in 3 patients at 4, 5.5, and 21 years of follow-up. None

of the patients with small-duct PSC but 4 of the patients with

classic PSC developed hepatobiliary malignancy. There were 3 deaths

(17%) or liver transplantations in patients with small-duct PSC (2

after progressing to classic PSC) and 15 (42%) in the classic PSC

group. Survival free of liver transplantation was significantly

greater in the small-duct than in the classic PSC group (P =.04).

Compared with the general U.S. population, survival in patients with

small-duct PSC was similar (P =.4) but significantly lower in

patients with classic PSC (P <.001). In conclusion, small-duct PSC

may represent an earlier stage of PSC associated with a significantly

better long-term prognosis. Some patients, however, progress to

classic PSC and/or end-stage liver disease with the consequent

necessity of liver transplantation. PMID: 12029635.

Because " small-duct PSC " might be an early stage of " classic PSC " it

might be good to consider getting your partner some medications that

might delay disease progression? One promising drug is the bile acid,

ursodeoxycholic acid, which at high doses (25-30 mg/kg/day) tends to

normalize liver biochemistry (i.e. liver function tests) in PSC

patients. In primary biliary cirrhosis, a disease that mostly affects

women, and specifically affects the small-ducts of their livers,

early intervention with ursodeoxycholic acid has been shown to delay

time to transplantation.

Best regards,

Dave

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

> My partner has just been diagnosed with PSC. He has had Ulcerative

> Colitis for about 3 years but hasn't really had too much trouble

with

> it. He goes to the toilet a lot and has had diarrhoea consistently

> throughout the 3 years but it has never stopped him from doing

> anything. He copes very well. He was having blood tests for some

> medication he was taking (Azathioprine I think - he reacted very

badly

> to it and has come off this now) but it transpired from the blood

tests

> that there was something funny going on with his liver so he had an

> ERCP. The x-rays showed a " beautiful " liver according to the

doctor

> and if it wasn't for the blood tests we wouldn't know he even had

PSC.

> The doctor seemed very calm about it and said he may start needing

> treatment for it in around 5 years but there is nothing to worry

about

> right now. I have been reading about PSC on the web and it has

scared

> me a bit. He is only 25 and I am very worried about the long term

> prognosis. We want to get married, have kids etc - how is PSC

going to

> affect his life? And will he definitely need a liver transplant?

> Thanks for your advice.

[Guest guest]

PSC7654

PSC can be scary at first. Keep in mind, the disease usually progresses s l

o w l y, and the world changes daily. Probably in your lifetime, and

certainly in mine liver transplantation has went from an experiment done on

pigs to highly successful for those of us with PSC. In fact we are the

" poster children of success " . Also am wondering how sure the diagnosis could

be when the ERCP was negative, and no reference to a liver biopsy being

troublesome. Lots of things can raise liver blood tests. I suppose this has

been answered to your satisfaction?

All the Best

jd, 44

UC 1973, Jpouch 2000, Chronic Pouchitis 2001, PSC 2004, Stage 3

ston City, IL

krmpotich@...

Just diagnosed

> My partner has just been diagnosed with PSC. He has had Ulcerative

> Colitis for about 3 years but hasn't really had too much trouble with

> it. He goes to the toilet a lot and has had diarrhoea consistently

> throughout the 3 years but it has never stopped him from doing

> anything. He copes very well. He was having blood tests for some

> medication he was taking (Azathioprine I think - he reacted very badly

> to it and has come off this now) but it transpired from the blood tests

> that there was something funny going on with his liver so he had an

> ERCP. The x-rays showed a " beautiful " liver according to the doctor

> and if it wasn't for the blood tests we wouldn't know he even had PSC.

> The doctor seemed very calm about it and said he may start needing

> treatment for it in around 5 years but there is nothing to worry about

> right now. I have been reading about PSC on the web and it has scared

> me a bit. He is only 25 and I am very worried about the long term

> prognosis. We want to get married, have kids etc - how is PSC going to

> affect his life? And will he definitely need a liver transplant?

> Thanks for your advice.

>

>

>

>

>

>

>

[Guest guest]

This is pretty typical of early PSC - liver looks fine, bile ducts not so

good. There are people in this group that have had PSC for 30+ years, so

you really don't know. The standard rule of thumb is 10 years from

diagnosis to transplant, but it's been suggested that there are a LOT of

people with PSC that have no symptoms.

I am currently asymptomatic since common bile duct (CBD) balloon dilatation

and stenting in the spring of 2001 (and possibly related, cholecystectomy 4

months later).

He should get standard LFT blood tests (liver function) every six months or

so to help show progress, if any. The usual treatment for PSC is Ursodiol

(Ursofalk), although efficacy is not yet proven.

My hepatologist typically says " You may need a liver transplant in the next

10 years - based on what I see now, it may be never " . Not much help, but

it sums up the situation pretty well.

Hang in there, and keep asking questions - this is the place for answers!

Arne

53 - UC 9/77 - PSC 4/00

Alive and (mostly) well in Minnesota

-----Original Message-----

From: [mailto: ] On

Behalf Of psc7654

My partner has just been diagnosed with PSC. He has had Ulcerative Colitis

for about 3 years but hasn't really had too much trouble with it. He goes

to the toilet a lot and has had diarrhoea consistently throughout the 3

years but it has never stopped him from doing anything. He copes very well.

He was having blood tests for some medication he was taking (Azathioprine I

think - he reacted very badly to it and has come off this now) but it

transpired from the blood tests that there was something funny going on with

his liver so he had an ERCP. The x-rays showed a " beautiful " liver

according to the doctor and if it wasn't for the blood tests we wouldn't

know he even had PSC.

The doctor seemed very calm about it and said he may start needing treatment

for it in around 5 years but there is nothing to worry about right now. I

have been reading about PSC on the web and it has scared me a bit. He is

only 25 and I am very worried about the long term prognosis. We want to get

married, have kids etc - how is PSC going to affect his life? And will he

definitely need a liver transplant?

Thanks for your advice.

[Guest guest]

My husband was diagnosed in 1999 at the age of 22 just about the

time we got married. We were told at that time it could be 5 years

or 15 until he needed a transplant. This seems likes years away at

the time. He basically had no symptoms from 1999 until 2003 when he

had his gallbladder removed. Once again, no symptoms until

September when he had part one of two step J pouch surgery. At that

time the colorectal surgeon said his liver looked terrible. Up

until this point he took Actigal 3 times a day and basically went

about his life with more problems for the UC than the PSC. After

this the GI sent him back to his hep. and began to look more closly

at his MELD. It was somewhere in the mid teens until the last

couple of months. It is now a 17 and we go back to Vanderbilt for

transplant evaluation June 8-10. The reason I'm telling you all of

this is to let you know that you can sit around and worry or go on

with your life. We have a lovely daughter, 9 months old, that was

born 2 weeks before his J pouch surgery. You will know when things

progress to the point where you are ready for the transplant. I was

scared to death when his GI broke all of this to me 6 years ago, now

I'm pushing the doctors for a transplant. I would never have

thought this a few years ago.

[Guest guest]

I recently joined the group myself. My husband is 26 and we have been

together for 3 1/2 yrs (we were just married in July) and have a one yr

old boy. It sound like we are in a similar situation. I don't know a

lot about the disease, even though he was diagnosed in 1999. He has

been fairly healthy until a few recent complications that we are

working with the doctor on now.

No matter what happens, I don't regret one moment of my life with

. I can't tell you the future of your partner, I only have advice.

Live every day to it's fullest, and take a moment to cherish the small

things in life. Look on the bright side - medical research and

advancement is happening every day - it can only move forward!

My best wishes to you and yours,

Hancock

(wife of - PSC 1999)

Mt. Vernon, WA