Cholangitis? WTF?

There is another treatment which is having excellent success. You can contact the Dr in the article's office directly (Dr Cox). I have two friends whose kids have had uc/psc for years but are now in full remission (of both) b/c of this treatment.
http://stanmed.stanford.edu/2011spring/article6.html

Sorry you got this bad news, especially since it appears you are fairly well along with the disease. This is typical though, since there are no symptoms until the ducts are blocked enough to cause them. That said, it is pretty rare, as only about 5-10% of UC patients get it. The strange thing is that a very large percentage, I think like 70-80% of the PSC patients have a history of UC. Sometimes the PSC even shows up first. So, just because you have a history of UC, it does not mean everyone should get an ERCP. This is an invasive test and not without risk. Plus, it could show nothing. The first step is to periodically get your liver enzymes checked. That is a simple blood test and part of good preventive medicine.

I hope they come up with some treatment for you to help keep you comfortable and also I hope this is very slow. The vancomycin treatment looks promising, but I don't know if it is only for pediatric patients.

Jan

Good point Jan. It is a pediatric study but that's only because the hepatologist who figured out the vancomycin treatment is a pediatric doc. I spoke to him over the phone and he said he gets calls from around the world weekly from children and adults. I don't know if the results are the same (usually it's the opposite -treatments studied on adults and then used, often off label, on kids). I don't think their efficacy is usually different? Either way, just thought it was worth a look. Good luck to you and I hope you find something that works!

I did find this link to a current study underway, and it includes both children and adults. It is at Stanford and they are currently recruiting subjects.
http://clinicaltrials.gov/ct2/show/NCT01802073

Jan

I also have PSC, diagnosed two years ago at the same time as my UC diagnosis. A really great place to get some support for that is on Facebook, search PSC Partners Seeking A Cure. That group, in and of itself, made my fears of the disease diminish. It's a great place to go if you have any questions about the disease. Hope the consult with the Transplant Center goes well.

Jeff

PSC is a strange disease and may be a heterogenous collection of conditions. With your history of pancreatitis you might want to do a google search on "ig4 pancreatitis cholangitis". You will find a lot of information about a rarer overlap disease that may be responsive to steroids.

Thanks for your thoughts, everyone. AV8, I went on that facebook group. You're right-it does look like a great place to go, as does their website.

Durk, you gave me something to think (worry) about. My GO thinks that my pancreatitis was caused by medication I was taking at the time, not by auto-immune pancreatitis. But we'll see. I'll definitely get more familiar with what you suggest.

JeffDC - I also have PSC and only found out because I was going to see my GI doc for my annual appt before my colonoscopy (I had UC). My liver enzymes were high, which resulted in an MRI, which resulted in an ERCP and bammm...PSC diagnosis.

In addition to the Facebook group, there is also a yahoo group that is VERY helpful and full of information and people just like you...and different.

It has been just 2 years since I was diagnosed, but I have normal LFTs now and no other symptoms. I have never heard of "remission" for this disease, as there is no way to change the scarring that has already occurred in the bile ducts. This is a very slow progressing disease in most people...but everyone seems so different and you never know at what stage you are when you get diagnosed.

Check out the Yahoo group as well as Facebook. I think you will find both resources very useful.

Oh, and I also do NOT recommend everyone get an ERCP. From what I have heard/been told/experienced myself, you only get one done to get a stent, do balloon dilation or something else that's really needed. It is risky and invasive. Usually an MRI with contrast and lab tests of liver enzymes is all you need. (BTW, I have had 3 ERCPs and 2 stents)

I have just been diagnosed with mild primary sclerosing cholangitis.....feeling battle weary. I am just so frustrated. I had the colonectomy 4 years ago and then my kidneys were not happy so was struggling with keeping them from going into stress and now this! I so wanted to be able to enjoy what was my new found energy and now my energy has fallen dramatically over the past 6 months. When they started investigating they found the PSC. I am so glad I have this site to go to and be able to vent, get support and then also to get information about where to go for more information and support. I am flagging with my optimism right now (actually having a pity party). Tomorrow is another day ....I will then start checking out the information I found on this discussion forum. Thanks for reading this. Any encouragement welcome. Sigh......

Oh....I forgot to ask....does anyone here know of a really good Hepatologist located in BC or Alberta?

To have gone through Ulcerative Colitis,and the surgerys,it is a shock to get the PSC diagnosis.The disease treats everyone a little differently.I once knew a man who just became suddenly ill,went to the ER,and had an emergency Liver transplant within a few weeks. Others,like myself go years with ERCP's,dilation and stent therapy ,surveillance MRI's ect. My diagnosis was in '99,a year after the j pouch surgery's.I have been listed as a transplant candidate for going on 10 years.I can explain how the transplant listing process works,and my experiences with PSC and the treatments if anyone is interested.This disease has been like a roller coaster.Back in '07 we had a colangiocarcinoma scare,but it turned out to be scar tissue. I should mention,the first 2 Ercp's gave me horrible pancreatitis,probably from the small cut called a sphincterotomy,they have to make at the duodenal opening to the common bile duct.So be prepared.I've had too many ERCP's to count...probably around 15 ??

Really impressed with the caring way sites are being recommended to go to for others. Just a good example of everyone helping each other.Exactly what we are all about.

A friend has a little one with UC, a failed j pouch, and an ileostomy who has PSC, and they're going to head to CA to see the peds guy who spearheads the Vanc study.

Thanks for your responses. How is it that your have been on the transplant list for 10 years Techwrench2? I thought you can't get on a transplant list until things have progressed to a very serious point? Maybe there is a different process for getting on the transplant list in Canada? I am definitely interested to hearing/reading your story regarding PSC. I am feeling a bit like I have just had the rug pulled out from under me. I have done a lot of crying over the past couple of days (grieving process) that I know will ease up as I process and move forward with the business of living.

To get listed one has to undergo a transplant evaluation.That consists of an abdominal CT scan,pulmonary function test,sonogram of the heart,a meeting with the transplant coordinator,and a Q&A meeting with the surgeons.They want to make sure you don't have any undiscovered medical issues that would compromise a transplant.The MELD (model for end stage liver disease)scoring system is 6 thru 42.Your score is determined from 5 liver function lab values. Biliruben,alkline phosphtase,creatine(kidney function),pro time (a clotting time) etc.A meld calculator (you can find on the internet) calculates those values and gives a MELD score.Understand those values can fluctuate with time.For example I was first listed at score of 9,and have fluctuated between 9 and 13 over the years.Unfortunately,the dirty little secret with PSC,is that a cholangiocarsonoma will not raise your MELD score.The transplant committee can meet and give you more points at their discretion.It's very tricky,you can have a lower MELD score,with a small cholangiocarsonoma (1.5 mm is max size to be considered for transplant) and you won't meet the minimum score of 25 for a cadaveric transplant.PSC patients tend to receive more living donor transplants,for this reason.There is no minimum score requirement for living donor.One can be listed as long as you have insurance,meet the minimum MELD score of 6,and comply with UNOS (United Organ Sharing Network)requrements: MELD lab work every 90 days,see your doctor at least once per year,and comply with other requirements like MRI's when recommended.For 2 years after my cholangiocarsinoma scare, I had MRI's every 90 days,until they determined it was probably scar tissue,and bumped the schedule to every 6 months,then once per year.In about 2003,they changed the system,and the length of time on the list is not as relevant,since the MELD system is a better gauge for determining the sickest patients.This is how I've been listed for 10 years.

Ive had PSC for 5 years now when first showed I had 2 groups of doctors fighting over weather I should have the liver out or wait. I went with wait and every thing settled and I still have MY liver. This year they stopped the bi yearly appointments and went to as needed. So take it easy and don't let them push you into any thing.

It truly is a roller coaster ride.Back in 2004,my gastro. at the time said, "by next summer you will probably need a transplant,so we need to get you listed". That's when I went through the transplant evaluation,and listing process.PSC is a difficult disease to navigate,and predict.With the high risk of cholangio carsonoma,I think that's why doctors are over cautious and jumpy.My hepatologist said they don't want to prematurely transplant anyone,because with the risks,and the side effects of the imunosuppressives,you are trading one disease,for another.The immunosuppressives are very hard on the kidneys.PSC can also return in a transplanted liver,and if it does, usually within ten years.So,live in the present,and deal with things as they they come.You will get overwhelmed thinking about things that have not happened,and may not happen.Make a plan with your doctor to monitor the disease, and live your life.A good book to read on PSC is TO The Edge and Back by Chris Klug,the olympic snowboarder.

Tech, you've been put through the wringer. I hope some good news happens for you soon.

My last MELD score was a 12, but I am not on a list. So I have my fingers crossed.

A lot of the lessons I learned when fighting UC are helpful, and you comments are in line-having the right mental outlook is key.

I went through the whole transplant workup just to find out the PSC Doc who said lets wait and see was right. cost me $6000 to find out that the Liver wasen't in as bad of shape as the transplant teem hoped.



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