PSC Caregiver with questions

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Hello! My husband was diagnosed officially with U.C. in 2011 and PSC in Feb 2016. His UC causes issues sometimes, which needs prednisone/meds (just a couple of times in the last 6 years). His PSC has been asymptomatic for most of the time.

Beginning in February of this year, his liver numbers began to rise, specifically his ALP (from 636 in Feb to 844 in June down to 729 last week). His ALT and AST did as well but his bilirubin never went over 1.2. From my research, it seems they don’t seem to worry as much about these numbers, but I can’t imagine it’s good for the body/liver/ducts after this long of a period of time. He doesn’t take any meds for his PSC.

Two weeks ago this Wednesday, he finally had an ERCP (first one). He had an MRCP a month or so before and they couldn’t “see” the common bile duct. The ERCP doctor took a look before he did the ERCP and said he could see it just fine but it was pretty narrowed.

The ERCP was done and there was a dominant stricture in the CBD along with some other minor narrowings in both L/R intrahepatic ducts. The doctor said his dilation wasn’t that bad. The 2mm camera couldn’t get through it and when they shot the contrast, some went up into the pancreatic duct. Thankfully, he didn’t develop pancreatitis.

They put a 5 Fr by 9 cm stent “9 cm into the right hepatic duct”. I’m not sure how large 5 fr is, but 9cm is longer than I expected. lol When he showed me the pictures, I couldn’t believe how small his CBD is. (He told me normal sizes are between 5mm-7mm.) The whole thing is the same size and it’s barely bigger than the stricture itself.The report said “main bile duct contained a diffuse irregularity and was of very small caliber–2mm maximal diameter”. Even with the stent, I can’t imagine it being opened up that much more. He’ll have it removed this coming Wednesday (exactly two weeks later). Hoping and praying it stays open. <3

Hubby does remember before the stent was put in that he couldn’t eat a lot and now his appetite is back again (since the stent was put in). Beyond that, he never had any symptoms (no itching or pain). Most of the time, if he has pain or discomfort, he usually blames it on the UC anyway. lol

He did get a fibroscan done and he has mild fibrosis, which isn’t too bad, I don’t think. Better than cirrhosis. They did a brushing and FISH as well. We’ve not heard those results, but I assume if it was bad, we’d have heard by now.

So, question, is it unusual for a long stent to be put in like his? I understand PSC causes narrowing of the ducts, but is this much narrowing common or just inevitable? The doc said there isn’t much they can do for him except make him as comfortable as possible. (yes, he said that, otherwise, he was super nice and answered all of my questions…I think I’ll keep him anyway). Of course, that seems like he’s on death’s door step, which we all know isn’t necessarily true. Anything can happen. :smiley:

Also, the report said “the sphincterotome could not be passed through the stricture-too tight”. Does anyone know what that means? They made a 9mm biliary sphincterotomy, but I thought that was to put the stent in. (More questions to ask on Wednesday, I’m sure.)

Otherwise, we’re doing good. We have two kids: 10-year-old girl and 7 (almost 8) year-old boy who keep us very busy.

Thanks for listening to my rambling! :smiley: Can’t wait to get to know more people who understand (because it’s real hard to find people who get it) and are willing to listen. :slight_smile:

lovesstorms

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Hi lovesstorms ! Welcome! I can’t say that I know much about the degree of narrowing of the ducts, since I’m new to the disease myself (diagnosed last September). It does sound familiar and I think it does differ in each of us with PSC. I hope someone else can offer some insight for you. This is a great place to vent or ask questions, so I’m glad you found it. :blush:

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Lovestorms,
Hello and welcome to this PSC forum. Thank you for your post. I have a few questions and suggestions for you to consider.

  1. Was his ERCP performed by his local GI doctor or one chosen by a transplant hepatologist at a major transplant hospital? The reason I ask is that it is often the case that it is difficult or impossible sometimes to gain access to a particular bile duct one time, but they can sometimes wait a week or so and go back in and gain access. That was the case with me a few times. A major transplant hospital’s advanced endoscopic team does ERCP’s day in and day out, often the doctor performing this is all he does in some cases or other related endoscopic procedures. You really need an advanced endoscopist that knows the ins and outs, and tricks of the trade to sometimes gain access to these strictured ducts. I was glad to read that he’s going to take the stent out in two weeks, that’s a must or you risk some pretty serious infections as the stent will get clogged up eventually due to the PSC.
  2. I don’t buy this “well we just need to make him as comfortable as we can.” What’s his MELD score right now based on recent labs? You can check this at this link.
    https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/
  3. Is he on URSODIOL? With these significant blockages I’d ask for it. I was on 1200 mg a day for 4 years. It does make a difference to help with thinning the bile. No, it’s not a cure for PSC, but it does aid quality of life.
  4. Finally, is his doctor a transplant hepatologist or a regular GI? He needs to be under the care of a hepatologist! This is a primary need for PSC patients.

Take care and we are here for you if you need us.

Mark
PSC 2011 / Liver Transplant 2015

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Hi, Mark! I’ve seen you around and you’ve been through a lot, so I’m glad you still take the time to help all of us out. :slight_smile:

  1. No, currently, he’s at a liver transplant hospital in our state (the only one and it’s a university hospital). We travel two hours to get there. He’s having another ERCP tomorrow (2nd one) to remove the stent they put in. He’s one of the best ERCP doctors in the state. We also see a hepatologist down there. Our ERCP doc said he’s the best in the state as well. :smile: So, we’re good on that end.

Hopefully, we’ll get good news and the stent will have opened up his duct like they want and we won’t need to go back for a long time. At least that’s what this doctor said last time was the plan. I will say I may have felt a little like I was being patted on the head, though. Like things were worse than they appeared, but he didn’t want to get us upset. You know what I mean? Maybe not, though. Sometimes I imagine things. :slight_smile: No results yet on the brushings/FISH, but I assume like I said that if it was bad, we’d have heard by now.

  1. lol I wish that was a joke, but that’s exactly what he said. My husband didn’t have any major issues except his ALP was high and his appetite was low, which was never even mentioned to the ERCP doc (like I said, he attributed that last one to the UC). I can’t get a MELD score as he only gets the Hepatic Function Panel, which I can’t see some of the required items on (IE: INR, Serum Sodium, Serum Creatinine). So, I have no idea, plus his fibroscan showed only some fibrosis, so I doubt he’s even close to the liver transplant stage.

  2. No, he’s not. I believe the hepatologist hasn’t considered hubby’s condition bad enough (he treats many others with PSC), so he’s been kind of ignoring him, in my opinion. I’m not saying the URSO couldn’t aid quality of life, but beyond the not feeling like eating, he didn’t have any other major symptoms, which makes me think this whole thing isn’t nearly as bad as it seems.

I’m not sure hubby wants to add another med when he doesn’t necessarily feel like he needs it right now. Maybe the hepatologist will say differently, though. I’ve gone back and forth on it myself. I mean, anything to help the bile flow better is always good. But if it doesn’t prolong the liver’s life, then I’m not sure about it. But then if he has symptoms, I’d want him to take it. (lol…believe me…I’ve thought about this in the past) Currently, even after all these tests and such, we don’t have an appointment with the hepatologist until January. Ugh. Perhaps it’ll be moved up, though. He may be waiting for all the tests to come back first.

  1. I’m not sure if the hepatologist is a transplant doctor or not. He’s at the university hospital in the transplant area and his online profile says it’s one of his “subcategories”. So I assume he is, but we know there are other hepatologists there, too. Hubby wanted to give this doc another chance (because I don’t like the way he’s been treated…his emails being ignored, for instance). I’ve only met him once like over a year ago. I can ask the ERCP doc tomorrow about the few questions I had here and about the other doc.

To be honest, I kind of haven’t really been paying attention to this whole thing until we went to the PSC conference in Cleveland this year. (Even despite our getting into a serious accident on the way there, we still continued our journey because it was important. Thankfully, we had only minor injuries except for our poor car, which was totaled.)

And thanks again for talking with me. Sometimes we just need to talk it out and plow through the sludge of information to some understanding. :smile:

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In one way the doc was right. They can (usually) only treat the symptoms until it is time for a transplant. That is what the doc said who did my initial ERCP and met with me afterwards.
But there are some horrible ways to say that info, and doctors differ in their bedside manner.
Sounds like your doc could use some improvement.

Be glad his bilirubin is down. The high AST and ALP are somewhat troubling, but it is the bilirubin that really got my doc’s attention as it is a more true indicator of current liver problems.

I’m sorry both you and he have to deal with UC and PSC at the same time. One is bad enough. I had UC eleven years ago, ending up with a j-pouch. Going through that year with PSC rearing is ugly head would have driven me nuts. But the lessons I learned that year helped me with psc: have a sense of humor, be patient and focus on the long term.

Remember that the stent is only temporary, good for a few months if that.

Jeff

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So true. Patience, focus, and a HUGE sense of humor is most definitely key. :wink:

Hubby did get his stent out yesterday (only in for 2 weeks as they know many PSC’ers develop infections longer than that). His 2mm wide bile duct is flowing nicely and a new stent was not placed. His less than 1mm stricture (didn’t know that until yesterday) has opened up nicely as well, so the ERCP doc was happy with it.

The doc said he’d sent the report for the cytology and the FISH results to us (via mail). However, we never received them (still haven’t as of today). So, he told us that information yesterday.

The cytology result from the last ERCP showed negative, which is good. They did another one yesterday and we’ll receive that information next week. They usually throw the stents away, but in this case, they actually sent it off to the lab as well to look for cancer cells. He said sometimes they like to hide inside.

The FISH results showed a positive for both polysomy & trisomy. (He seemed somewhat grim when he told us this.) I’m not super sure what to think, to be honest, but I know my husband is pretty devastated. I’ve heard some people say this means nothing while others say it’s practically pre-cancerous. Don’t know, but it doesn’t sound good. The doc did say he planned on checking for cancer via ERCP every 6 months.

Last night, hubby and I discussed the thought of doing an ERCP every 3 months, but we’d need to talk to his hepatologist to see if he’d allow this or feel it’s warranted. We have no idea. I mean, bile duct cancer is pretty aggressive. I called him today and the nurse said the FISH results hadn’t even been flagged for the doc to see. So, I’m glad I called. Hopefully, we’ll get some answers soon. Our appt isn’t until January and we’d really like to see him sooner, if possible.

Thanks to all of you for helping us by answering my silly questions and listening.

Heather

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Heather,
Be assured of our thoughts and prayers for your husband during these days. Having an ERCP every three months will put your husband through a lot, but the doctor I’m sure will act in his best interest and advise you best. Hopefully the news will not turn out as bad as it sounds.

We are here for you if we can help in anyway. Take care.

Mark

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