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Posted 5/16/2014 4:18 PM (GMT 0)
Hi,

I'm a new member of this wonderful site. I was introduced to what happens when your liver decides not to play nice last year. Like a typical guy, I ignored what were increasingly strong signs that something wasn't quite right until I ended up in the hospital, blown up like a giant water balloon along with peripheral edema.

After a CT and MRI, they found a tumor in my liver but weren't sure what it was. Did a couple of paracentesis procedures, gave me some diuretics and a beta-blocker, and send me on my way a week later minus 80 lbs. The doctors just had a puzzled look about what was going on but were definitely going to followup.

They then did the followup MRI and now they were pretty sure it was cholangiocarcinoma. I'd never heard of it so I looked it up - bile duct cancer, sounds lovely. Biopsies were inconclusive but then the pathologist said this guy's got PSC with severe portal-to-portal fibrous - MRCP & colonascopy were then done. So now we know where the PHT and tense ascites was coming from and why we're looking at an apparent cholangiocarcinoma tumor.

Also, over the past couple of years, I'd progressively grown weaker with constant joint/muscle pain and debilitating fatigue. I found that liver diseases (cholestatic forms especially) can cause all this but this seemed beyond what was to be expected. So now we get a Rheumy involved and she suspects a CTD disease is in play as well - postive ANA, etc.

I've worked in a scientific/engineering research lab my whole career but knew next to nothing about medical things. So I've spent the last 6 months in an crash course on the liver and biliary systems. Very amazing stuff but unfortunately not working out so well for us.

I know this is a hepatitis forum but I hope to get and offer additional insight into our various liver and extrahepatic diseases. Hopefully there are other PSC members here as well. Thank you and sorry for the long introduction.

Post Edited By Moderator (MamaLama) : 5/21/2014 2:06:36 PM (GMT-6)

Posted 5/16/2014 5:21 PM (GMT 0)
Hi bb, Welcome. I am not familiar with PSC....so I checked into PSC liver, and find the Ulcerative Colitis forum at HW has some members with PSC. I quickly searched and found some have been told the will need liver transplant. You might want to look there as well as this Hep forum. We do have people with all sorts of liver disease posting here. Alcoholic cirrhosis, PBC, HCC, and autoimmune hep.

My husband is on a transplant list because of the Hep C cirrhosis he had decades before he knew he had Hep C. Big Hugs, and again...Welcome!
Posted 5/16/2014 5:40 PM (GMT 0)
My bad! I should have said what it was. PSC, Primary sclerosing cholangitis and yes, I do need a new liver soon since I'm in the ESLD phase. Unfortunately that can't happen until the cholangiocarcinoma issue is resolved. Kinda complicated ...
Posted 5/16/2014 5:48 PM (GMT 0)
Hi there and welcome

As you have probably been told PSC is autoimmune in nature but attacks the bile ducts rather than the liver as in autoimmune hepatitis. Steroids and immunosuppressant treatment doesn't seem to help PSC although many patients find a drug called ursodeoxycholic acid helps.

Unlike many other autoimmune diseases it seems to affect more men than women and is often linked to inflammatory bowel disease.
I can't remember anyone here with it - I think the member called syxx may have been told he may have it though.

I gather in some people it doesn't progress but others go on to need transplants. Has your hep stated what is likely to be the next step for you or is he/she just monitoring you?

I am based in UK and there is an excellent support group here that welcomes people from overseas. It is called PSC Support.org.uk. I think there are a couple of online groups in the US including psc partners.org so it may be worth looking at those.

Ann
Posted 5/16/2014 5:49 PM (GMT 0)
I typed my question before I saw your latest post as you may have guessed!
Posted 5/16/2014 8:18 PM (GMT 0)
Thank you and I'll certainly check the other groups as well. As you mentioned, PSC attacks the bile ducts and this is reflected in my biopsies and MRCP. In the non-cirrhotic portions of my liver (peripheral parts are cirrhotic), the portal tracts are a war zone but the rest of the liver lobules are normal with well preserved central veins. This causes my MELD to stay low (due to still having good liver function) and means I'll probable have to be on the transplant list for quite a while before getting a new liver, not fun. That's if I ever get on the list of course, can only hope right now.
Posted 5/18/2014 1:50 AM (GMT 0)
Bblbt,
Welcome to the forum. It sounds like you're in a tough spot. Would a transplant not be the best way to remove the cholangiocarcinoma? How are they going to treat the carcinoma, what's the plan on that front?
Posted 5/18/2014 2:55 AM (GMT 0)
Finding a liver should get easier as time goes on, what with people getting cured of Hep C. Most liver transplants go to people who have Hep C.
Posted 5/18/2014 2:58 AM (GMT 0)
Dany -

Unfortunately I have intrahepatic, mass-forming cholangiocarcinoma and resections are the only real possibility of a curative solution. Even with a clean resection, metastatic CC is very likely but they can usually hit the smaller recurrences with ablation or radiation to help stomp it out for good. They can't do a transplant because of CC's nasty habit of coming back and the anti-rejection therapy weakening you, preventing your body from fighting the cancer. Meanwhile the PSC is busy beating up what remains of the good portions of your liver.

I'm not sure what they'll be doing yet. I'm a big risk for surgery due to the PHT issue, single kidney and diminished hepatic reserve. But, I simply do what I can to stay healthy and keep fighting the chronic parts of my problems, PSC and whatever CTD I seem to have as well. Hopefully, the acute issue, CC, will be dealt with in the near future. Life sure has a way of making things interesting.
Posted 5/18/2014 4:10 AM (GMT 0)
I hope so David, seems liver are a very precious commodity currently.
Posted 5/19/2014 7:40 PM (GMT 0)
bblbt,

Sorry to be late in welcoming you to the forum.

Your case sounds pretty complicated. It sounds like you have studied your various conditions pretty carefully. Fortunately the new radio frequency ablations and chemo embolizations are working well for many patients.

Let us hear from you as time goes by...

Mama Lama
Posted 5/19/2014 9:19 PM (GMT 0)
I posted something a few days ago but lost it, and did not have time to go back and repost.

I have not been confirmed with any diagnosis. I have to many hep doctors disputing my diagnosis to get anything firm other than there has been significant improvement in my numbers since they stented me up a year ago. I woke up morning extremely yellow. At first they were thinking viral hep A, but were not sure. Test negative, blah blah blah. Anyways, after getting told I was an alcoholic due to my age, 31 at the time and the severity of my liver cirrhosis I got a second opinion. He thought I had an obstruction or PSC. After an MRCP I had an emergency ERCP where they put in 2 or 3 stents, not sure. At the time he found a huge mass on my bile duct. He said it was not vascular, so he thought cancer was not an issue but wanted me to be aware it was a possibility. Within about 6 weeks my jaundice was gone and took about 6 months to get my liver function back to my bili being normal and other labs close to normal.

The doctor who did the ERCP said that even though there were stones that the likely hood that I had bile duct stricture that caused no pain and this advanced damage without me noticing it was impossible. He said I had to have PSC. My primary hep, who works in the same clinic as the doc who did the ERCP said no that this could happen. Well lets say, they want my liver now as it is a first for them.

A few things I have learned from my team and from months of research. You are right, your MELD stays pretty low, as PSC ravages your liver. I would suggest strongly to look for a living donor, PSC patients do extremely well with living donors. I know it is risky for the donor, but most PSC patients I have found to have living donors. Also, find a transplant area with a low MELD transplant score so you have a better chance. Most PSC patients only hover around 12 to 13 before they get to sick for transplant.

Sad thing is, PSC patients have one of the highest recovery rates and it is extremely rate for it to come back in the transplanted liver unlike many other auto-immune liver diseases.

If you have turned jaundice, ask about stenting until they do a resection. Even with the PHT, getting the cancer out is needed. Bile duct cancer is high in PSC patients and is nothing to mess around with. The sooner you do it, the better. I think the risk is worth it, you just have to find a doctor who will do it. There is typically things they do before surgery to prep you. Load you up on vitamin k, band all varicies etc. Surgery for people with PHT can be done, and do not let them talk you out of it.

The surgeon I saw who was going to cut the cyst out of me, before they realised it was inflammation and mysteriously disappeared, said that even though it was risky it was more risky to let it turn to cancer and not get it out.

I know of a forum where there are people with PSC and there is a group on facebook for it.

Like I said, I am not confirmed with it, but its a 50-50 shot for me. I hope to just keep going the way I am. Oh, caffine is good for PSC patients. Not sure why, but they say it is.

I do know there is some really strong research out there showing decrease in firbous bile ducts out there with PSC. It is still 2 to 3 years out though minimum.

You sound well informed, I think that is really important. Learn as much as you can, as sometimes doctors are not on top of everything going on out there.

Posted 5/21/2014 7:07 AM (GMT 0)
Certainly seems strange to hear the doctors keep referring to decompensated when discussing things and then seeing the MELD bouncing around 12 or so. But, it does "feel" like the MELD is much higher than it is. My bile ducts are very damaged as shown in the cholangiography. Strange things these bile ducts ... never really knew what they were or existed until they got my undivided attention.
Posted 5/21/2014 11:04 AM (GMT 0)
SyXx - In regards to your diagnosis dilemma, did they ever do a liver biopsy to help determine the underlying disease(s)? I know it's not always definitive in PSC but it would certainly have helped with the additional info. My pathologist were amazing in what they extracted from the multiple samples from several regions.
Posted 5/21/2014 5:57 PM (GMT 0)
I had 2 biopsies, MRCP, MRI, CT, ERCP. The biopsies were reviewed by 10 different pathologists across the US. I know 2 of them were done at the U of Minnesota, 1 at Mayo and it was also reviewed by a surgeon at Abbott NOrthwestern as well as the best pathologist in MN who works at Fairview Ridges in Burnsville. They said there was no signs of anything other than bile was in my liver tissue.

The MRCP showed a something blocking my bile flow. The ERCP showed a 4 inch mass, he said it was biggest he had ever seen but he was certain it was not cancer. Luckily it was not, they put stents in and within 6 months the mass was gone. When I first went in for the emergency ERCP my INR was like 2.2 and my bili was around 33. He said normally he would not even attempt it, but he said he knew something had to be done or I would dead or needing a transplant in the next few weeks.

There was 6 stones in my bile duct, but its just a matter of who you talk to. Some believe it had to be the stones, other say there is no way since I had no pain.
Posted 5/21/2014 8:00 PM (GMT 0)
Very interesting but not a lot of fun for you SyXx. When we get to see things fail like we do on sites like this, it's really seems pretty amazing that the body actually functions as well as it does. Glad I'm able to interact with others like yourself in this forum.

Post Edited (bblbt) : 5/21/2014 5:04:09 PM (GMT-6)

Posted 5/22/2014 3:14 AM (GMT 0)
Caffeine has anti-inflammatory properties. My hep fully supports my caffeine consumption. He says he drinks it by the bucketfuls too tongue
Posted 5/22/2014 11:54 AM (GMT 0)
Yeah, I read a webinar about caffeine. They are not sure how it works, but it does improve liver function a little in PSC patients. The doctor who wrote it said to drink lots of coffee pretty much.
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