New and confused

General
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Hello, my name is Alex. I’m 39 years old, and my husband, son, and I just moved to paris, France, from Houston in June. I’ll make a very long story short-I have had loose bowels since March, but only in the mornings. I had full labs in March and mid-May, and all was totally normal. I had a CT scan of the abdomen in mid-September, and it was negative for anything wrong. I came down with a virus October 3, and by that Saturday, October 6 (?), I was itching like crazy. I was also exhausted, nauseous, and lethargic. Itching continued, I saw my GP, and she ordered labs drawn. My liver enzymes were through the roof-ALT and AST 900/780 respectively. GGT 74, ALP 143. Bilirubin is measured differently here, but it wasn’t that high. Ended up in the hospital that Sunday for a bout of weird pain (lower down-felt like labor pains), so one week after the itching started, and everything had dropped a bit except for the conjugated bilirubin, which went up. Had ultrasound in the hospital that was totally normal. Labs the following day showed same trend, as did labs that Wednesday. Had an EUS last Thursday. They told me my bile duct was inflamed, and they started me on questra and Urso. I went to see my gastro today (after emailing to ask for clarification), and he said again that it was “inflamed”. I asked specifically if there was beading or scarring, and he said that the walk was thicker. I think he had no idea what I meant. I asked if it looked like PSC and he said, looking at me like I was crazy, “Well, yes, it’s PSC. We have ruled out all autoimmune diseases and the hepatitises, so it is PSC. But you’re at the beginning, and it progresses slowly. So don’t worry”. Ummmmm…yeah, not a good answer. I got more labs. Everything else has continued to drop (four days on Urso, five on Questra), except my bilirubin, which is still climbing. I’m itchy, yellow, and my poop is pale. So, here’s my questions:

  1. Has anyone gone back home to the states to get a care plan and then moved back to another country?
  2. I would go to NYC for treatment. Has anyone heard of Brett Fortune or Thomas Schiano?
  3. I have NO other symptoms besides climbing bilirubin-no pain, fatigue is fine, no fever, etc. This all came on in the space of 3 weeks. Is that possible?
  4. Would I be better off going to London for treatment or follow up, or should I go back home?
  5. Is my climbing bilirubin dangerous? What should I do?

We just got to Paris. We love it, and most importantly, my son loves it. He is 7 and a pediatric cancer survivor, and I don’t want to leave this city and pull him from his school if I don’t have to. I don’t have a lot of faith in my doctors here, though, so I need to at least devise a plan without them. We are thriving here, and it’s the opportunity of a lifetime, and I don’t want this to dictate the trajectory of our lives right now.

Thank you for your help and advice!! :slight_smile:

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Alex,
For one I think your GI doctor doesn’t know much about PSC. Didn’t he even recognize the fact that PSC “IS” an auto-immune disease so he can’t rule out autoimmune if he’s saying PSC. You need a hepatologist. I do know that University of Birmingham has a transplant program. I’d seek them out to meet with a hepatologist there. Just to introduce you a bit to their program, here’s a testimonial video from a PSC patient who had a transplant there. https://www.futurelearn.com/courses/liver-transplant/1/steps/64848

You state your bilirubin is climbing. What is your bilirubin now? Have you ran your MELD score yet? Go to this web site and input your latest lab data and share with the group if you don’t mind. https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/

As far as a recommendation in New York City, I would encourage you to contact Mariel Carr who has a small business in helping folks connect with the right transplant hospitals for not only transplants but for treating their liver disease. Her husband had a transplant a few years ago and she knows her stuff. http://www.comparetransplantcenters.com/index.html

I know I didn’t answer all your questions but hopefully this is a good start. Take care.

Mark

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Alex, of the blood levels you mentioned, the one I used to pay the most attention to was the bilirubin. That seems to be the best indicator of direct liver trouble.

There is a sizable contingent of Western Europeans on this forum, so I hope you get some good info on your remaining questions.

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Thanks for this information, Mark. My bilirubin is 88, but this is by European standards. I don’t know the equation to convert it to American bilirubin. I also can’t get my MELD score for this reason.

Unfortunately, I think Birmingham will be difficult for me to get to, so I would likely go to London if we decide to go that route.

I’m freaked out that my bilirubin keeps rising, and I’m unsure what to do about it. I’m hesitant to take a cross-Atlantic flight with something going drastically wrong in my body, but I don’t know how to get it down in the meantime. My doctor today threw out the possibility of steroids, but I don’t have a lot of faith that he knows what the heck he’a doing.

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Thanks for this, Jeff. I’m so puzzled as to how all my other levels were already dropping and my bilirubin keeps rising. Initially they said “mild inflammation”, but the rising levels lead me to believe it’s so inflamed nothing is getting through?

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Are you having any RUQ (right upper quadrant) pain and does any of the pain go around to the right side of your back? Have you had an ERCP done? This is an endoscopic procedure where they go into the common bile duct and up into your left and right hepatic ducts?

You really need to get to a specialist as soon as possible. It doesn’t sound like your doctor where you are currently is skilled enough to handle this. If you start running a fever over 101.5 degrees, chills, shaking, you need to go to the ER. Tell them you have PSC and that you believe you are having a cholongitis attack. They will need to put you on iv antibiotics, etc. You also need to keep a prescription filled of a broad range antibiotic like Cipro so you can start that immediately should you have an attack.
I’m not trying to panic you or anything but you do need to see a specialist. I’d contact London and try and get in with one of their major hospitals there as soon as you can. Keep in touch.
In the meantime go to this web site I found. Using your number of 88, your us bilirubin is 5.14. That tells me you probably have a bile duct blockage that needs to be cleared. If that keeps increasing, with increased RUQ pain, itching, etc. that’s another tell tale sign you need intervention. And please don’t let your local GI do that procedure. It’s an advanced procedure and there are risks but an expert in the field that does it day in and day out tied with a transplant hospital is your best option. I had 5 of those done between diagnosis and transplant. Good luck.

Mark

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If you come to London, the royal free hospital has an excellent hepatology department. This is where I have had all my procedures and there are many doctors that know all about PSC, many of which you can find on YouTube speaking on the subject. They have excellent Mrcp imaging, ultrasound, fibroscan, biopsy procedure and good gastroenterologists peforming the endoscopies.

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Hello!

Sorry to hear about your misfortunes in Paris. Wanted to specify a few things:
I believe the doctor meant “wall thickening” of the bile ducts which means the duct is dilated. The beading appearance is usually seen in MRCP and not with EUS.

Also, which hospital in Paris did you go to? Have you tried to go to any clinic? I’d advise to maybe call the Ambroise Paré / Hartmann (it’s a group of clinics, not sure which one has the hepatology department), here is a number: 01 46 39 89 89

I’m not sure if they speak english or anything but it’s worth a try to explain your situation and see what they say before deciding to fly out of the country.

One very specific question I’d ask is why haven’t they made any cholangiography (ERCP or MRC). The EASL clearly specifies: "A detailed cholangiographic assessment of the biliary tree is essential in making a diagnosis of PSC ". And, as Mark wrote above, with your symptoms (pale feces, jaundice, itching), it seems you have a bile duct blockage and an ERCP could seal the diagnosis AND help with the situation by opening the bile duct.

PS: I’m french so if you have any question related to it, please ask

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I agree with Mark 100%. You should seek a Hepatologist ASAP. Most GI docs go their whole career and never run into a patient with PSC, so they are very inexperienced.

Like others said, the “beading” you’re looking for wouldn’t usually show up on other scans. An MRCP would provide more detail and allow them to see it.

Also, having antibiotics on hand like Mark said is key. I was in the hospital left and right for months. My doctor gave me a prescription of Cipro to keep on hand for colangitis attacks, and it stopped me from ending up in the ER all the time. I just took the medicine at first sign of trouble (pain in liver area and fever). Vancomycin also helped keep me stable for a few years.

When I was diagnosed, I was fortunate enough to have a doctor who was honest and told me that I’d be better off seeing a Hepatologist who worked in a transplant program, not her. I had complications over the years, as my case was bad. Had I not had someone experienced, I’d not be here today.

And yes, I say my case WAS bad. I had a transplant 6 months ago, and so far there is no sign of reoccurrence. While it is a big surgery, keep hope, if you are diagnosed, there is a cure! Don’t think you can’t have a normal life after transplant…if it ever comes to that.

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Thanks, Rick. I emailed them and am waiting to hear back.

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Thanks, Frank, for your kind words of encouragement. I’m hoping to get to London next week. Aside from the itching, I feel completely and totally fine.

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Thanks so much, Ldde. I went to the American Hospital of Paris. I think I will go to London initially to get full work up/diagnosis, because I don’t want the language/cultural differences to get in the way. I was so startled at the lack of empathy (and, frankly, understanding!) shown by my gastro.

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Alex,
If you do travel to NYC, Dr. Fortune is my son’s heptologist. EXCELLENT doctor, transplant specialist. I know he is handling 1 or 2 other psc’ers on this site. We were just out to see him Monday.

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Hi Alex, it is definitely the right track to quickly see a hepotologist with PSC experience, but I wouldn’t conclude at this point that PSC is the culprit. If you have any historic blood work (even if it is years old) you should gather it up to present to the liver doc. I’m sending positive thoughts your way.

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I love everyone’s detailed responses. This site is so great! When they were first figuring out what I had, my ALT was at 750, supper high! Urso and Lialda work for me currently. Also, I had weird lower cramps recently , like labor cramps and they ended up being fibroids! I recently had 9 fibroids removed from my uterus! Hope you are feeling better and get the care you need to be comfortable :hugs:

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Hi, Ill through in my two cents…my daughter is a patient of Dr. Fortune and he is one of only about 8 doctors in America who will prescribe oral vancomycin. It has been a miracle for my daughter, not only stopping the progression, but allowing her liver to heal from near cirrhosis to completely normal. Please research oral vancomycin and PSC and, if it works on you, it will be a game changer. Good luck! Joanne

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Hi Alex

You definitely need to seek a second opinion. I am though sure there are plenty of knowledgeable specialists in France. The reason I say a second opinion would be worthwhile is that from what you describe doesn’t seem to sit well with my understanding of what you would expect to see with PSC.

Firstly the speed at which everything seems to have happened is a bit at odds with PSC. Things don’t tend to go from everything being absolutely normal to diseased within such a short space of time. It’s a bit more of a slow burner if that makes sense.

Secondly, with the blood tests you’ve had, the one I would expect to be raised would be the ALP. This is the marker that hints more to cholestatic liver disease, of which PSC belongs. Most people with PSC would tend to have this marker raised on most occasions irrespective of any flare up taking place. If you’re is generally normal then perhaps another diagnosis may be more likely.

From what you were describing, it looks more to resemble some form of acute attack on your liver than the chronic attack you get from PSC. It looks more to resemble an attack from some form of infection. The fact that most of your markers are decreasing but your bilirubin is still increasing does though warrant further investigation. Something clearly isn’t right and a better diagnosis is needed. Hopefully it will be something relatively straight forward to treat. You do though need a more informed opinion than you currently seem to have been given. Hopefully, fingers crossed, it does turn out to be something other than PSC.

Hope that’s helpful. If nothing else to open up other avenues to explore. There are many things that could result in what you describe so hopefully you’ll get a more robust diagnosis soon.

Best wishes.

K