New UC/PSC patient seeking guidance

Hi everyone,

Dan from Ottawa, Canada here. I am a 34 year male who, this past spring, was diagnosed with ulcerative colitis and as of last month, also diagnosed with PSC. I’m seeking guidance from you all, the UC/PSC community, as to how I should proceed and navigate these newly confirmed diseases. First, a quick summary of my conditions:

All throughout 2019, I was suffering from sharp abdominal pains (lasting about 2 min each, multiple times a day) and frequent diarrhea (4 times a day, sometimes with small droplets of blood). My skin was developing all sorts of rashes (eczema) on my legs, stomach and groin and I would also have week’s long stretches of fatigue, particularly when I travelled. After switching family doctors, I was able to get a colonoscopy in January of 2020. The primary diagnosis was Crohn’s, but was changed to UC when I was assigned my GI specialist in March. I was placed on mesalamine for a few months, which seemed to minimize the sharp abdominal pains, but didn’t change my stool consistency and frequency. A 7-week round of prednisone was then prescribed in May, which provided immediate relief, but true remission was not achieved as UC symptoms returned once the steroid doses were reduced/tapered off. A second colonoscopy in July confirmed “stage 3” pancolitis; stage 3 refers to severe colitis with deep ulcerations. Mesalamine was continued until I was approved for Remicade infusions beginning at the start of August. I am hopeful the biologics, combined with azathioprine, will eventually provide intermediary remission from my UC symptoms.

However…two days after second Remicade infusion, and exactly one week after the birth of my first child, I began developing extreme upper-right quadrant pain that lasted for 10+ hours per night. For over a week, I would get these severe pains, beginning in the evening, and would be utterly paralyzed for the entire night, writhing in pain. During the day, I would be exhausted and have no appetite. After attempting to ease the pain with opioid painkillers, I was implored to check into the ER at the Ottawa General Hospital. I had become severely jaundiced (my bilirubin was 5x higher than normal) and a ultrasound showed inflammation in my bile ducts. An MRI/MRCP performed a couple days later indicated PSC. Since I was provided no direction from the doctors to ease the severe pains, I forced their hand with a second visit to the ER on September 1st. My GI doctor confirmed a PSC diagnosis and a ERCP was ordered for the next day.

Before and after the ERCP was conducted, my bilirubin dropped, but my liver functions numbers (AST, ALT, ALP) were 4x higher than normal, even the day after I was discharged from the hospital. I experienced some pain relief for one day, but soon after the pains returned. Because my lipase level is high, my GI suspects pancreatitis, although she cannot explain the delayed onset (post-ERCP). She told me to stop taking the azathioprine for now as it can aggravate pancreatitis. The ERCP report claims I have my common bile duct is unremarkable, but that my hepatic bile ducts (left and right) are stricted and were dilated during the procedure. I am assuming this indicates small-duct PSC. I am seeing a hepatic biliary specialist on Oct. 8th; there is a “roundtable” discussion amongst the doctors to discuss my case, and I’m hoping to have some direction from them.

After receiving my third loading dose of Remicade last Thursday (with a saline IV, less than a week ago), my PSC-related abdominal pain symptoms subsided completely and my appetite finally returned. I had lost over 20lbs in three weeks and was already lean to begin with, so the weight loss was noticeably shocking to my family. I have been able to put a few pounds back on and my energy is up slightly; I feel the biologics might be finally resulting in some type of remission, but am still wary of the symptoms returning at any time. My most recent bloodwork has shown my liver function numbers have stabilized greatly, besides my ALP number.

I was hoping to have some guidance, advice and direction on my next steps:

  1. Has anyone been prescribed a combination therapy of Remicade and azathioprine? How successful has that medicine been for you? Does remission from UC provide relief from PSC symptoms as well?
  2. Should I have seen the PSC symptoms coming? Did anyone else experience sudden and severe pain when a PSC diagnosis was indicated? All my bloodwork collected previously to my painful episodes at the end of August did not indicate any abnormal liver function.
  3. Should I be pushing for any type of medication to help manage PSC, such as ursodiol? The scans I got indicate no liver damage (ie cirrhosis) at this time, but I don’t know the health or progression of my biliary tract.
  4. How should I go about finding my MELD score? I have most the numbers I need to calculate it on my own besides knowing my INR score. Is INR found using a different specific value in a blood sample?
  5. Has anyone else experienced the PSC stomach pain patterns occurring only at night? I found it very odd and disconcerning that I could predict when my pain would onset, always after 7pm.
  6. How long does the dilating effect of an ERCP usually last/relieve pain? I can’t imagine needing another one anytime soon unless another bout of severe pain kicks in.

Thank you for taking the time to read my story. Again, any direction or advice for me would be greatly appreciated.

Cheers,
Dan from Canada

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Dan,
Thanks for your post. I’m sure several will give you a reply soon to your inquiries. I plan to sit down and respond later today hopefully. But just to address one point. From your description of the ERCP, to me it is indicating a normal large duct PSC, not small duct. Large duct PSC involves strictures in the left and right hepatic ducts as well as the common bile ducts. Keep in mind with large duct PSC you start out with a perfectly healthy liver, but he disease hits the bile ducts keeping bile from flowing out. So over time, that bile has no where to go fast enough so just backs up which leads to cirrosis of the liver. The longer the dilation lasts before structuring the ducts again from the ERCP, the more bile flows, and the disease progression slows down. My first ERCP gave me relief for almost a year, but as the disease progresses the ducts will stricture sooner resulting in more frequent ERCP’s. There came a point with me where I had two in less than a month. I got transplanted a month later. It’s so different in every patient.

Chat with you soon.

Mark

quote=“DanStern55, post:1, topic:4222”]
I was hoping to have some guidance, advice and direction on my next steps:
[/quote]

  • Should I have seen PSC coming? You would think so but in my case, I certainly didn’t. I first discovered there was a problem when I had some blood work done when I was trying to increase my life insurance and abnormal liver numbers came back. I then noticed weight loss and some itching, then we pursued to discover what was going on. It was quite devasting at first, but after educating myself more I was able to find peace about how things stood. I believe having a very open and frank communication with family and close friends was key to me during my sickness. I really needed that support.
  • I believe that URSO is a very good medication to be on with PSC. My hepatologists kept me on it through my entire time. I took 1200mg a day which I had to adjust myself the amount and times of day as it can increase diareah. Keep a good supply of Imodium caplets on hand to control that as there are times I would get what I call a “bile dump” when it seems it all came at once and tore my stomach up.
  • You can determine your MELD score by going to this website and entering your lab results. https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/
  • The dilating effect of ERCP will vary in each patient and according to disease progression. For some in early stage PSC the dilation will last for some time, but as things progress the times between ERCP’s will be shorter. I made a practice to always use the same advanced endoscopist specialist each and every time. He knew my bile ducts and this lessened the chance of pancreatitis or worse.

Hope this helps Dan.

Mark

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Thank you for your quick and helpful responses, Mark. I will continue to seek out assistance when needed, to help myself and others work through the many challenges that a PSC diagnosis presents.

I will keep you all posted regarding my specialist consultation in October.

Cheers,
Dan

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Dan, welcome to the club! A lot of members here deal with multiple diagnoses. UC is bad enough, but throwing on PSC is tough. I had UC in 2006, resulting in a pouch. PSC came along in 2013.

To address your questions:

  1. Has anyone been prescribed a combination therapy of Remicade and azathioprine? How successful has that medicine been for you? Does remission from UC provide relief from PSC symptoms as well?

I had three Remicade infusions. They did not work for me but allowed me to take some much needed naps. I did not have the azathioprine.

  1. Should I have seen the PSC symptoms coming? Did anyone else experience sudden and severe pain when a PSC diagnosis was indicated? All my bloodwork collected previously to my painful episodes at the end of August did not indicate any abnormal liver function.

No. There is a significant percentage of UCers who develop PSC. What got me diagnosed was a trip to the ER that was prompted by a nagging pain between my belly button and sternum. Then it radiated to my back. I had that nagging pain three times before that lead to pancreatis. They found my bloodwork was off and an ultrasound showed a dilated bile duct. A ERCP the next morning showed PSC.

  1. Should I be pushing for any type of medication to help manage PSC, such as ursodiol? The scans I got indicate no liver damage (ie cirrhosis) at this time, but I don’t know the health or progression of my biliary tract.

Tough to say. I am on ursodial post-transplant. Some people swear by vancymocin.

  1. How should I go about finding my MELD score? I have most the numbers I need to calculate it on my own besides knowing my INR score. Is INR found using a different specific value in a blood sample?

Mark sent you the link already.

  1. Has anyone else experienced the PSC stomach pain patterns occurring only at night? I found it very odd and disconcerning that I could predict when my pain would onset, always after 7pm.

Mine could happen at any time. I played a macabre joke on myself when I had those abdominal pains-I told myself, “Well, there goes another bile duct.”

Could those evening pains be food related? Is there a pattern?

  1. How long does the dilating effect of an ERCP usually last/relieve pain? I can’t imagine needing another one anytime soon unless another bout of severe pain kicks in.

I would think it varies. If the situation calls for it, the doc can put in a stent into a duct that can help it stay open longer. These usually have to come out a few months later via another ERCP.

Hi Jeff,

Thanks for the response. I’m relieved others share similar experiences with me, I think the toughest part of this diagnosis is not having “all the answers” about the future progression of the disease. I’m crossing me fingers that the relief brought upon by the ERCP and Remicade treatments lasts several months/years before more issues arise. Again, I’m hoping to receive more answers/direction when I see a hepatic biliary specialist on Oct. 8th.

I appreciate you taking the time to detail your thoughts!
Cheers,
Dan

Hi Dan,
We live in Toronto, and my son has been diagnosed with UC/PSC back in 2016/17… he is in process of moving from SickKids to UHN as he is turning 18 in November.
Even though I know everyone’s PSC journey is unique, and I am not a medical expert by any means, I’d like to share with you how we have been able to manage my son’s disease and make it “stable” according to his doctors, but before I start, I encourage you to join “PSC partners seeking a cure- Canada” community. We meet once or twice a year and also have a couple of doctors from UHN to volunteer in the events and answer the questions. You can easily find this group by a simple google search.
Back in 2016, we used to go to ER almost every month (with symptoms like yours, including pancreatitis), but since we started the following, we have not been in ER for PCS/US even once since 2018 and his blood-work is mostly normal (it took almost 9 months to show its effectiveness).
Now, what we did and saw its benefit, compared to other members of the community (with the same age and gender), was a dramatic change in lifestyle:

  • Stopped eating out (cheat days every couple of months)
  • No “Junk food” (almost absolute zero)
  • No alcohol (Absolute zero)
  • Very low gluten (once a month) but no “gluten free” stuff (like GF bread, which are more dangerous than regular bread due to additives)… all natural “whole-food” GF.
  • Multi-strain, high dose probiotics and almost all of our food is “prebiotic”. (the most important)
  • No “night-shed” like potato and tomatoes.
  • Regular exercise.
    The only medication my son is taking now is Urso, but he is taking some supplement based on his doctors’ advice:
    Vitamin D, Curcumin, Omega 3, and also maintenance of multi-strain probiotics.

Sorry, I did not answer any of your questions directly, as I am not a medical professional, however, as a dad of a PSCer, I wanted to share our experience with you. I personally believe PSC is not a “disease” itself, but it is a “symptom” of something else that nobody is sure about yet.

Good luck with your journey, I wish you all the best and please be assured that you are not alone.

Take care.

Hi Bob,

Thank you very much for your insights into how your son has progressed. I’ll be sure to join the Partners Seeking a Cure group.

I adjusted my diet as you described a few months ago to help with my UC. Particularly, I’m on very low gluten and have cut alcohol out completely. Next is eating out and junk food, though I don’t consume too much of this either. I will definitely look into some type of high-dose probiotic. Do you recommend a certain type or brand?

Again, I appreciate your input!
Cheers,
Dan

Hi Dan,
You are very welcome, I’m glad that you have already started to modify your diet. According to my son’s PSC doctor, it has been seen in younger PCSers, that when the UC is under stable remission, the PSC progress is much slower. Therefore, I am positive that if you could keep your gut healthy, your PSC side would be more manageable.
On the probiotic recommendation, we tried several brands, and actually, as per my son’s UC doctor’s recommendation, we tried to be as diverse as we can. The main thing is that the dose should be at least 90 billion, and more the number of strains the better (we prefer capsules over powder to help the little guys pass through the stomach acid). Today, he is taking “120 billion 20 strain made by New Roots brand” and we bought it from Whole-foods Market near our place (on sales for $45/30 capsules). But we change the probiotics from time to time, and do not stick to a specific brand or store… as you need to have your microbiome as diverse as possible, we go for whatever has more potency and more strains and is on sales! As long as the store is a reputable one… I’m sure you can find good products in Ottawa.
But the other thing, and more important than probiotics is the “prebiotics”… according to doctors, when the “ecosystem” of the gut bacterias is established, you just need to maintain them, and more importantly, feed them by prebiotics foods. As long as you have not been on antibiotics, your gut bacteria are having their time in your body. If you feed good bacteria with the good foods, they do you good, and if you feed bad bacteria by the bad food, they start to harm you… read about “leaky gut”… it’s important to know about it.

One last thing, as you have experienced pancreatitis, I share our experience… late in 2017, my son was on Solfasalazin for his UC, and one night we had to take him to the ER for severe abdominal pain… the diagnosis was Pancreatitis… my wife is an avid reader about our son’s condition, by complete chance she came across the idea that people who have an allergy to Fava beans, show pancreatitis to certain medications. She asked the IBD doctor to stop Sulfasalazine and do a
“Glucose-6-phosphate dehydrogenase deficiency test”… this test is not a routine protocol and few doctors care to do it… sure enough, my son had this issue and actually sulfasalazine was very dangerous for him… so, what I am trying to say is that your knowledge is your best friend and do not hesitate to challenge your doctors for additional test… there are many unknowns about this disease and unfortunately, there are very few specialists in this field.

Please try to reduce your stress, I’m sure it is easier to say than done, given the new addition to your family and also the PSC/UC emotional “roller coaster” you are going through…

And, please be sure we are in this thing together…

All the best,
Cheers,

Hi Bob,

Wow, I really do appreciate your insights. Thank you for taking the time to describe the ups and downs of your son’s journey.

I will definitely look into a multi-strain, high count probiotic to use. I will also increase my prebiotic food consumption; I already eat a fair amount of oats, beans, bananas, onions and garlic, but I will try to include some of the other suggested foods in my diet.

And yes, I have learned through this long process that I need to advocate for my own health concerns, especially when dealing with specialists who seem overwhelmed by the amount of patients they need to track. I find they are very wary to try anything “outside the book”, which seems to me the reason they haven’t suggested a single medicine, supplement or dietary change to help manage my PSC. Hence, why I’m reaching out to you all, the PSC community!

I am hopeful that I will continue to trend in the right direction, and with some guidance, can stave back a quick disease progression.
Cheers!
Dan

You are welcome Dan,

Just last point, be careful about beans (and all the legumes), they can be a double-edged sword, read more about them and talk to an expert. All other foods you mentioned, are our favorites too!

Good luck, take care and stay safe,
Cheers,

Totally agree with everything you say it worked for me here in UK .My addition to the list would be remove stress from life.

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My daughter had a similar reaction to azathioprine. I will send you a PM so that I can send you information.