Recent PSC, My Story

Welcome New Member Introductions
1

Greetings to all,

Let me start with an introduction. I'm Russ, recently (Feb. 2015) diagnosed with PSC. Also diagnosed with UC in Jan. 2014.

Thank you to everyone here who have been so open with your personal lives. I've been reading a lot of your posts. You're truly an inspiration. Now where do I begin? I'm guessing the beginning is always helpful.

Back in Sept. 2013 I began to experience some severe gastrointestinal problems. Without getting into too much explicit detail (not a pretty picture) I finally went to see a GI specialist sometime in November 2013. So I suffered for a couple of months before determining that this was no passing malady. The GI doc did blood workup and I was to see him again in December. This is where the proverbial manure hit the rotary blades..

We had a snowstorm here in NY mid December. I was outside clearing snow off the cars. Just a broom cleaning as I can't do anything strenuous due to severe chronic back problems. I mention this because this was the last thing I did before my life was turned upside down. Upon entering the house I began to feel a pain in my chest. It felt like I was having a heart attack (from what I hear, as this was the first time I've ever had a pain like this). Note, at the time I was 54 y/o. I told my wife what was going on. We called 911.

Fast forward to the emergency room. My heart was and still is fine. No heart attack. However, they did a plethora of tests, both blood and sonograms. This is when I was informed that my alkaline phosphatase was 687. I was released and told I needed to see both a cardiologist for a nuclear stress test (bad back wouldn't do well with the standard cardiac stress test) and a Gastroenterologist. Stress test fine. Cleared to proceed with the GI test. At this time the GI doc did a colonoscopy and endoscopy. Feb. 2014, diagnosis is UC, diverticulosis, swollen sphincter of Oddi. Treatment was prednisone for about one month. Alk Phos came down. Prescribed Lialda for the UC. It has worked great to this date. Stopped the prednisone and the levels began to rise. GI doc is doing his due diligence and not jumping to any quick diagnosis. Lots of blood tests (every month at this point), ERCP, MRCP, liver biopsy, and probably more things I just can't recall anymore. It was at this time he recommended going to a hepatologist.

The hep doc started doing blood tests every two weeks. Put me back on prednisone. He did another liver biopsy with his team as well as reviewing the first one of course. Switched me over to budesonide instead of the prednisone. Enzyme levels came down slightly. Nothing to impress the hep doc. He then added 6MP to the budesonide. Enzyme levels began getting worse. Now he was afraid of drug induced liver disease. Stopped 6MP.

It is now Feb. 2015, about 14 months since my 'heart attack' episode. The hepatologist finally felt comfortable labeling my symptoms as PSC. He gave it a stage 1 which is why I believe he had such a difficult time diagnosing it. Various issues with this particular doctors support staff/office so I switched hepatologists in June 2015.

Had my quarterly visit with the new hep doc last week. My enzyme levels have spiked to levels that are very concerning to me, but apparently not to her. My alk phos is back up to 578 with my ALT at 225 and AST at 202. Do any of you folks feel these numbers are a red flag that warrant a more serious discussion with my doc? I have not had any jaundice, vomiting, diarrhea. The only noticeable symptoms I can claim are fatigue and itching (on my back, mostly at night). Other than that the only indicator that I have a diseased liver is because my doctors tell me it is.

I have adjusted my diet. Unfortunately it is a severely limited diet because of the combination of PSC, UC, and diverticulosis. I'm quite sure most everyone here is aware of the dietary restrictions. It doesn't lend itself to much of an appetite. I dropped 40 lbs since Feb. I feel as good as I have felt in 10 years. I've begun to swim again. I was a competitive swimmer back in HS in the 1970's, so swimming is a natural sport for me. I can't do any other sport due to my back, so this was a blessing. I figured if I have to be put on a list one day I didn't want them to tell me its time to get in shape. I want to be a good candidate for a transplant as well as be as fit as I can to make recovery as problem free as I possibly can. I'm finding there are so many things in this life I don't have control over, the few things I can control I MUST control (meaning my fitness/weight).

My wife is amazing. I bring her to every doctors appointment because I listen to the doctors, but it doesn't appear that I hear the doctors. She is introduced to them as my PCP (Primary Care Person). Without her I'd be a goner. I've been blessed with her.

Back to my current levels. They scare me and I'm hoping someone (or a few of you) can add some insight to this spike.

Thank you all for listening to my rant. Thank you all for your support. Thank you all for the compassion I've seen through your posts.

Please all, be well,

Russ

PS - Happy Halloween

2

Hi Dennis,

Thanks for your reply. I was taking budesonide and my levels were lower. My hep doc said she wanted me off it to determine if it was masking the symptoms or helping the condition. Would you think this is a proper course of action if it were you? I understand you're not a Dr. I'm just trying to be my own best medical adviser.

Thnx again......

Dennis said:

Hi Russ, your enzyme levels are quite similar to mine upon time of diagnosis. However, urso has helped to cut them roughly in half.

3

Maybe you can try oral vancomycin…
it has helped many people…at least Stanford University is using that, probably east coast has doctors familiar with that too…

4

I’ve never heard of oral vancomycin. I will definitely do some research on it.

Dennis, my enzyme levels were lower on budesonide (another classification of steroid) as yours were on Urso. My doc wanted to see if it was helping or masking. As we know, long term steroid use is not recommended.

5

Hi Russ,

Are the steroids for the UC or the PSC? Generally speaking, steroids do not work to help PSC with the exception of fringe cases like AIH/PSC overlap and IgG4 sclerosing cholangitis.

PSC progression is unpredictable and a change in LFTs may be significant (usually accompanied with a change in symptoms; ex bacterial cholangitis) or may just be a random fluctuation. If LFTs spike up and persist, this may warrant a non-invasive look via MRCP to check for any obvious strictures, but generally speaking a rise in LFTs by itself usually isn't an emergency or anything to lose too much sleep over.

Some people persist at 600+ ALP for years without any notable progression, others proceed to needing transplant after a short time at much lower numbers. The one thing we do know about these numbers is that for any time given time out from diagnosis, those with ALP of <160ish are substantially less likely to reach an end point (transplant, cancer, death) than those with higher ALP. While the standard PSC treatment is to do nothing, anything you do that can pull your ALP down to near normal will improve survival (whether this happens spontaneously, through Urso or Vanco, or by any other treatment).

Most doctors have very little experience with PSC and are unlikely to devote a ton of time researching the latest and greatest for their one PSC patient. It will be your job to become the subject matter expert and to help guide your doctor in the right direction.

Fat soluble vitamin deficiency is very common for people with PSC. If your doctor hasn't done so, suggest a blood test for fat soluble vitamins (ADEK). Even if there is no deficiency, a bone density scan to test for osteoporosis is not a bad idea every couple years.

If your doctor hasn't done so, have a discussion about what to do in the case of bacterial cholangitis at your next office visit. Become familiar with the symptoms and come up with a game plan with your doctor (on hand script for antibiotics; proceed directly to ER, etc.). Know what this is and what to do about it before it happens the first time.

6

jtb,
Thank you very much for such a detailed response.

1 - the steroids were supposed to address the elevated ALP +/- 600. Seemed to work. This treatment was started before the actual diagnosis of PSC. Lots of ‘fishing’ in those exploratory days. Discussion of AIH and PSC were running rampant back then.

2 - LFT’s? Please excuse my ignorance, I’m guessing LFT’s mean liver function tests? Not sure so please correct me if I’m wrong.

3 - You stated, ‘It will be your job to become the subject matter expert and to help guide your doctor in the right direction.’. This is one of the reasons I’ve taken to this wonderful site. I’m hopeful that comingling everyone’s experiences can help us all progress.

4 - Bone density scan? This is the first time I’ve heard any mention of osteoporosis. I will certainly bring this up at my next consult.

5 - Bacterial cholangitis, once again, first mention of this. I need to do much more reading to understand this. I thought I had PSC exclusively. What is this bacterial cholangitis you speak of?

Again, thank you so much for all your insight. It really means a lot to me. Sorry for the delayed response on my part.

7

Sorry for the acronyms. You are correct: LFTs stands for liver function tests and usually include ALT, AST, ALP or GGT, and bilirubin.

As PSC progresses and strictures form, liver bile gets stuck in the liver and doesn't flow as well out into the intestines. One issue is that less dietary fat gets absorbed because most fat must be broken down by liver bile first before it can be processed. This also means that less fat soluble vitamins (ADEK) will get absorbed. It is important to keep tabs on these vitamins to identify and supplement any deficiencies. Osteoporosis is a very common side effect of PSC.

Another issue with liver bile lingering in the liver is that it can become infected. This is called a cholangitis attack and is a big deal because it can develop into sepsis if it is not treated. Symptoms are different for everyone but generally if your PSC symptoms get a lot worse and if you develop chills or a fever, you may be having a cholangitis attack. Treatment is with antibiotics, either oral at home or IV at the hospital. It is important to come up with a game plan with your doctor ahead of time.

8

jtb,
That was the most concise and comprehensive explanation of PSC and what I should be concerned about I’ve heard since day one. THANK YOU!

My next Dr’s appointment is already shaping up to be quite a visit.

9

Hello Masterswimmer,

Just reading over some old posts and were wondering how you were getting along with your PSC? We'd be glad to hear an update from you if you are up to it. I trust things are staying pretty stable for you. Please let us know if there's anything we can do on our end. Take care.

Mark Wilson, Moderator

PSC 2011 / Liver Transplant 7-2015

10

Hi Mark,
Thanks for reaching out with concern. I just had my four month hepatologist visit this past week. Overall good visit with encouraging blood workup.

Alk Phos went from 576 in Oct’15 to 416 in Feb’16.
ALT & AST both just over 200 in Oct’15 to both in the 90’s Feb’16.

Like I said, encouraging. However, my doctor put me on URSO starting this past week. Not 100% sold on it yet. Hope to see good results. Time will tell. Anybody here have good or bad concerns with URSO?