Question about relative rate of progression

I get the sense that one of the big questions that we all seem to have, at least those with new diagnoses, is how fast will the disease/liver damage progress.

I have looked at several of the “PSC Prognostication Calculators out there like the Mayo Clinic one, and the UK one, but they are lacking in utility.

So, my basic question for those that have had this disease is whether so far I am progressing rapidly, slowly, or normally … I know that past progression isn’t necessarily a sign of the future and all the other caveats. I get the sense that so far I am progressing relatively rapidly.

I have had UC for 37 years, and Type 1 diabetes for 24 years.

In 2016 my Alk Phos started being slightly elevated, so with the UC history, they did a MRCP and found fatty liver but no signs of PSC.

Did another MRCP, which showed signs of PSC, and then the Fibroscan scores showed stage 3 Fibrosis (12.8 kPa) and late fatty liver (400 dB/m). My liver blood tests are all still normal other than the Alk Phos, which is always slightly elevated.

My newbie sense is that this has progressed rather quickly over the last 5 years … from no signs to stage 3 Fibrosis in 5 years.

What are the thoughts of those who have been dealing with this for longer than I have been as to this rate of progression relatively speaking … fast? Slow? Normal?

Thanks!

Joe,
From what you have described I do not get the sense that your PSC is progressing rapidly. I believe we would see your AST, ALT & Alk Phos off the charts. What is your INR and bilirubin looking like these days?
Also, plug in your latest labs to this MELD calculator and let us know what your current MELD is. Although not a gold star for what’s really going on as PSC doesn’t always show high MELD, it at least gives us some idea. MELD Calculator - OPTN

Mark
PSC 2011 / Liver Transplant 2015

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Joe,
When first got diagnosed, I read that the average person with PSC goes for about 12 years from diagnosis to transplant.

I lasted 4 years and two months. Others have lived for 15-20+ years. But we all have to be ready to deal with the symptoms when they arise.

Aa Mark said, the MELD is the best, if imperfect, method docs use to determine how close a person is to transplant.

There are quite a few people here who also have UC, so you’re in good company. I had UC in 2006 but never could get it in remission, so I had a pouch put in later that year.

Jeff

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Hi Jtrombo,
I was diagnosed with PSC in 2006. Doctor told me I had end-stage IV liver cirrhosis at that time. I had a gallbladderectomy in 2000 and had stage III cirrhosis. PSC patients today do not seem to get told about the condition of their liver by their doctor. I would ask to know what stage you are in. If you know your history (trend over time) about cirrhosis, you can use that as a predictor of how long you have.

When I was diagnosed in 2006, my hepatologist could see from my surgical records of the gall bladder removal that the surgeon had taken a liver biopsy in 2000, had it tested in the lab and it was recorded I had stage III cirrhosis. The pathology report said I had PSC in 2000. Not the surgeon or my gastroenterologist told me of this diagnosis.
In 2006, when I was officially diagnosed, I had no PSC symptoms. I was very active and working about 50 hours a week at age 62. They could not explain how I was able to function so normally with stage IV cirrhosis. The PSC doctors told me my health would not last, that some patients hit a plateau and stay their the rest of their lives, while others worsen. Less than two years later, I had full-blown symptoms of PSC and going down hill fast. They put me on the transplant list. My MELD never got higher than 16, so it was not a good predictor of my disease. I had liver failure that hospitalized me 8 times between 2008 and 2011. Each time, I recovered. I got listed at two other hospitals and finally had a living donor transplant in 2012. At that time, death was eminent. My Hepatologist said when one of these liver failure episodes happen, I would not respond to treatment and my MELD would go from 16 up to 39-40. At that time, they would have to search for a cadaver liver match and transplant me before I died. Good luck!

It is very difficult how it will develop in you. Get informed about your cirrhosis stage and look at MELD. Your blood chemistry can give you indicators, but I would let the doctors read and interpret your scores and what they mean.

Not knowing was a very confusing time for me. It affects each PSCer differently. I don’t mean to scare you with what happened to me. You need to know there is risk you must face. I turned to Jesus for comfort and confidence to get me through. I hope this and what others will write will be helpful to you. Best wishes.
Paul

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I am not a hepatologist, but having only one liver enzyme slightly elevated after 5 years doesn’t sound like rapid progression to me. My heps didn’t really do staging-- they always used MELD score, which I think is more precise.

I went 14 years from dx to tx, but I was not symptomatic at the time of dx. I found out by accident after I had my gallbladder removed and they found my main duct dilated and beading in the smaller ducts in the liver.

Your disease may progress very slowly and you may never even need a transplant. Or, you have probably heard how some people “go off the cliff” and have a sudden, rapid progression for no discernible reason.

PSC is so weird in how it manifests differently and different people. You are already doing the best you can do by staying in touch with your doctors and keeping an eye on your labs.

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Thanks everyone. So what I am gathering from everyone’s comments is that if the liver bloodwork is ok, and my MELD score is low, I shouldn’t worry too much about the MRCP and Fibroscan going from no physical signs to stage 3 Fibrosis in 5 years. Am I interpreting that correctly?

Joe,
I’d be more concerned if the MRCP was showing increased beading in the bile ducts and if your LFT’s were climbing. If there was a blockage your bilirubin would be really going up. I personally don’t give a lot of credence to the fibroscan. I’m sure it does give them some idea of progression, but labs and MRCP’s plus ERCP’s if needed really tell the truth of the matter from my experience. I wish you many years of slow progression.

Mark
PSC 2011 / Liver Transplant 2015

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I was diagnosed in 2015, so six years, and my disease is not advanced, based on the most recent liver biopsy and MRCPs. Be careful how much stock you place in those Fibroscans. I have had probably 6-8 fibroscans as a result of being in a study, and the numbers tend to move around quite a bit. My first one was showing significant fibrosis, but subsequent ones showed the score maybe half of what that first one was, My hepatologist said to take those scores with a grain of salt.

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Thanks Mark – What exactly is “beading” and what does it mean?

The report for the MRCP states:

"relative left lobe hypertrophy with suggestion of subtle contour nodularity …subtle beading and irregularity is suggested in the visualized proximal intrahepatic bile ducts. Distal intrahepatic bile ducts are dimunutive in caliber … small lymph nodes are seen in the porta hepatis.

is the visualization of lymph nodes normal?

My LFTs have been normal or slightly elevated, but relatively stable. A couple of months ago the Bili was 0.7, and it was 0.6 last summer. The Alk Phos was 161, the AST was 41, and the ALT was 72 last summer, but back in August they had come down to 138, 19, and 42.

Joe

Beading is where the bile ducts become irregular in shape, making it difficult for bile to properly drain from the liver. If you go to google and search “psc beading” and look at images, you can see what this looks like.

That is what happens when a bile duct shows the effect of PSC-the duct shrivels up and dies.
I came up with a theory, brought about by a macabre sense of humor, that whenever I had a RUQ pain, I thought, " Well, there goes another bile duct.!"