Primary Sclerosing Cholangitis

My daughter had a diagnoses of PSC
and UC at the same time. She had some high liver numbers so they ran some tests.They gave her Ursodiol (a bile salt she takes daily) and do blood work every few months. She has had normal blood work ever since. Her JPouch is new and we have been dealing with a few complications with it. Both diagnoses came just two years ago so we too are new to both the j pouch and cholangitis.

I have both PSC and a J pouch the PSC is a back ground issue as long as I keep my drinking to a normal amount and stay away from most pesticides and herbicides it leaves me alone. My J pouch is what it is.

I have PSC and J pouch as well,and manage them separately. I took Ursodiol (actigall) at the Mayo study experimental dose for many years. ( so many mg. per kg. ) It didn't really do anything for me,and on the recommendation of my hepatologist,I stopped taking it about 3 years ago. It's also very expensive !
The main thing to be aware of is a cholangitis attack.This is when there is a dominant stricture in the a bile duct,and the bile backs up in the liver,causing severe chills and fever. It's like the flu, only 10 times worse ! In which case, you would need to get to the Hospital,get labs to verify high biliruben and alkphos. Then have an ERCP, with dilation and stent to open the stricture.
Some people go years before developing any symptoms.

Thank you all for responding! I have had my meeting with my GI. My GI talked about Ursodiol but said that it really does not work. She has informed me that I need to go to a specialist in this area at the Mass. General in Boston. Just waiting on appointment. I have an abscess (first one) that I am addressing right now with my colorectal surgeon. He drained it but I believe not all the way so, I will see him next week for a follow up. Also, was informed that I have to have my gallbladder out. I see that surgeon on February 2nd.

Techwrench or Murray do either of you have your gallbladder still?

I guess I started the new year off with one hell of a bang!!!!

Roberta
UC 2007
proctocolectomy w/ileostomy and j pouch 2/2012
takedown 4/2012
lst obstruction 3/2014
PSC 1/2015
abscess 1/2015

Yes I do still have my gall bladder.

I still have my gall bladder. The first two ERCP's gave me terrible pancreatitis. It is a complication to be aware of.

I also have PSC and manage the pouch and the PSC separately. Both doctors that see me (my hepatologist and the surgeon) are both aware of both issues and do ask me about each thing when I see them. I see my pouch surgeon 1x/year (2 years post-op) and see my hepatologist every 3-6 months, as long as I'm not having any symptoms. I'm pre-transplant.

PSC can be a disease that you will have  in the background) for a long time as I have ! Do find a good PSC liver specialist, not a General Dr., and stick with them even if you must travel for appts to find one.  It will be a good thing to have them at your side when your PSC does become more of an issue in your life..

 

I was diagnosed with PSC through very high alko liver numbers and the UC shortly after over 20 years ago. 11 years with a j-pouch. I'm doing very well but still pre-transplant. 

 

Two years ago this Fall I headed to the hospital for what we thought was a cholangitis liver attack, but turnout to be a gallbladder attack. I had the gallbaldder out by my (future) liver transplant team as they wanted to make sure the bile ducts connected to the gallbladder were preserved with an eye toward transplant.. If I had had to have emergency surgergy by a general surgeon not familiar with Liver transplant issues, I could have unknowingly risked a result that made me not suitable for transplant! I would never have thought of that!  

 

Since I have had my gallbladder removed, I have gone two years with no cholangitis liver attacks. So we think some of the preceding liver attacks were probably aggreviated by my failling gallbladder- it was/ is difficult to know.

20 years out, my liver is in good shape but my bile ducts are not! When the bile ducts are clear I do very well!

 

I know it takes awhile to wrap your head around a liver disease that may mean a liver transplant someday, but my j-pouch has had more of an impact on my day to day life than my PSC.

 

Good luck to you. 

I have posted before about "having PSC."   Long story very short, I recently inquired of my GP because I thought a doctor mentioned quite a while ago that I had PSC.  However, after a gallbladder attack, hospital stay, ERCP, nobody mentioned PSC, which I thought was unusual.

 

My GP said that in the reports a while ago it was a POSSIBILITY I had it because liver numbers were elevated after surgery, but they have calmed down.  He said I do not have PSC.  I mention this as it seems as if post big surgery we can have numbers that indicate potential PSC, but after watching them it can later be ruled out.  

 

However, I do realize that IBD-ers have a great incidence of PSC ...

 

Roberta, I just noticed this thread.  My son was just diagnosed with PSC and we are being seen in NYC at Weill Cornell.  I noticed you asked the question of a doctor in the Boston area.  I'm sure by now you have found one, but I have a friend who is a transplant doctor at Leahy Clinic.  His name is dr. Pompselli.  I've known him since he was a kid!  But he is some hotshot over there, and thankfully he was able to get us in to see doctors in NYC.  If you need anything, and I can help, let me know.

dianne

KNKLHEAD, Just a note to your post clarify for others new to PSC...

 

PSC is often diagnosed after a blood test with very high Liver Numbers. But as you said high liver blood tests numbers can point to many other issues as well.

 

The way PSC it is verified this through an ERCP. In the ERCP, for PSC, the Doctor can see that the bile ducts are abnormally narrowed. The many large and small bile ducts, eventually,  all through out the liver will be scarred and this creates an image on the screen that the doctor can see that looks like a string of pearls... Along the links of my ducts the go from the size of a pencil to the  size of a needle and back- creating a beading effect. I assume this is more evident in cases such as mine that as at least 15+ years out.

Beth, I didn't realize you also had PSC.  That was a good explanation of the disease.  Help clarify for me.  How are you feeling?  Right now Jeff is doing well at the moment.

 

I was also diagnosed with PSC along with UC.  After I had the surgery j-pouch  I was being seen by a liver specialist.  after 1 year on medication she took me off.  My numbers have been good and what I have been told it is a long acting disease.  I had surgery 5 years ago and have no problem so far  with my numbers.  Grace

Grace, thank you.  I'm still in shock, but I'm happy to hear good news from someone!  I'm so happy to hear you are doing well.  This disease has me scared to death for my Son.  And most of what I read on the PSC sites, while some people are doing well, there always seems to be something going on.

 

thank you again, I feel a little better.  

 

Dianne

Hi there.

Im new to this site tonight so just browsing. Was very interested seeing a PSC thread as its something that I never see talked about anywhere (this is the first medical condition forum I have joined)

Im a 34 year old male from the UK and ive lived with UC since I was 8. When I was around 11 I was diagnosed with PSC. When I reached 15 I was put on the list and received a transplant successfully. I continued in good health until my liver started to reject and I received a second transplant aged 20.

Since then I have lived a very normal life with a wife and two young children.

Despite having the 2 transplants by far and away UC has caused me way more health problems than PSC ever did.

My memory may be a bit hazy but if you ever have any questions about it please ask.

Raider, hi there.   my son has had UC since he was 6 and was just diagnosed with PSC.  There are several good sights for PSC, two of which are connected through Facebook.  There is also another one started by me, I forget what I called it.  I'm so happy to hear that you are doing so well and that your UC is more bothersome than the PSC. If you read through my posts, I am terrified that I'm going to lose my son.  I'm not what is worse, me dying first, and him dying without me, or losing hm first and having to suffer through that.  But I'm beginning to believe there is hope than he could possibly have a good life ahead of him.  He is 24 now.  If you look through my posts, you will see that he had a very difficult time, especially the last year.  I am so happy you are doing well.  I hope Jeff is as fortunate.  If you google PSC, but spelled out, you will find a few forums.  I wish you continued good health!

dianne

RLC, how are you doing?  I see you started this journey last February, we just started in May.  You said you were seeing someone at Mass General.  That's a huge place.  If needed we have a family friend that is a transplant guy at Leahy, just outside of Boston, that's how we were able to get into weill Cornell so quickly.  I live n NJ, so NY was an obvious option.

 

hope you are doing well

 

dianne