Progressive slecrosing chlongitis anyone ??????please

[ezz2012]

Hi everyone for years I have had raised liver fuction tests recently had a biopsy as I was told I had auto immune hepatitis but the results came back I have a very rare auto immune disease and there is no cure and very little is known about it it affects the bile ducts and they become blocked and stuff and inflamed and so does the liver it developes liver failed eventually but can take decades or be quick it really is all down to luck some will need a liver transplant also 75 percent of patients will have colitis which I also have and there is high chance of colon cancer and could also develope osteoporosis has anyone got this disease? I am only 32

[Bikafella]

Hi ezz2023 - there's no need to be too despondent as treatment, chemistry and methods of dealing with the associated conditions of UC are changing all the time. Liver transplants (outside of Covid restrictions) are now considered as as a straightforward and almost common treatment in the fight against liver disease. It's true that there is no cure for PSC (Primary Sclerosing Cholangitis) but they can deal with it very well. PSC is normally only found in UC sufferers. It can take upto 20 years to get to the liver failure stage. I've had UC since childhood (60 years ago) my PSC started to manifest itself in 2001 and I needed a liver transplant in 2017 and so far so good. Since I was assessed for the transplant I was put on Calcium and Vit D3 to combat osteoporosis. The important thing is to eat a good balanced diet and get yourself physically fit to help combat the disease.

[ezz2012 in reply to Bikafella]

Thank you for your reply I would say I am quite healthy apart from my diagnosis I have always kept quite fit with exercise happy you replied thank you

[Kristian]

Hi ezz,

Welcome to the PSC club. Its quite an exclusive establishment by all accounts, lol.

I was eventually diagnosed with PSC post transplant but the suspicions were there from the early stages of things not being quite right. For me it took about 8 years to go from fibrosis stage to transplant. However, I know many people in the PSC community that have lived with the disease for 20 plus years and still no where near even the cirrhosis stage. So yes, it really can be a bit of pot luck how things progress, but, as you've already discovered, not everyone with PSC goes on to even need a transplant. This is now a very different story to a few years back where everything you read gave an average life span without transplant of 12 years. So just be careful what you read on the Internet. Those early studies were not representative of the whole PSC community, only those in specialist centres. They therefore didn't take account of those whose progression was much milder or slower.

There is an Organisation in the uk called PSC support that have good advice and they also have a Facebook group that is worth joining too. Its a very friendly community and well supported by PSC support UK. It therefore has quite a lot of up to date info shared.

The amount of knowledge around PSC is increasing all the time. It seems to have become an area of liver disease that has got a bit sexier in recent years. There are much more studies and trials into treatments going on now than ever before and some are looking very promising with early outcomes.

Hope thats helpful.

[Hidden]

Hi ezz2012, you have already received lots of good advice and support from others on the forum but I'll attach a link to the British Liver Trust information, if it's helpful. If you are in the UK, our nurse led helpline is open 10am to 3pm Monday to Friday on 08006527330.

britishlivertrust.org.uk/in...

Take care,

Trust10

[Liverandlife]

I have a very similar profile to "bikafella". I have had U C all my life but not diagnosed until I was 15 when I nearly lost my colon. Fortunately my mother suggested a dietary solution which helped greatly, BUT no-one ever mentioned the possible liver connection; perhaps in 1962 they didn't know. Following a major spike in symptoms 4 years ago I was referred for a liver transplant, but age and other complications including U C have ruled it out. I am now taking a broad suite of medication including prophylactic abx and diuretics. The P S C and Ascites are currently under control, and the cirrhosis has stabilised.

[Bikafella in reply to Liverandlife]

Interesting reply Liverandlife! The consultant gastroenterologist who diagnosed my PSC and treated me for 16 years told me that there's a strong school of thought that young people with UC (obviously depending on how severe it is) are in some cases being recommended to have a full or partial colectomy (?) (i.e. removal of the offending part or all of the colon) to avoid more serious liver problems in later life. I did have that suggested to me in the late 1970s but it sounded very extreme at that time and stage of my life late 20's) most of my problems then we're colon related (if you know what I mean).😜

[ezz2012]

Do you mind me asking your age ? I’m so happy everything under control 🙏

[Liverandlife]

I am 75. What saved my colon was the suggestion reluctantly followed up by the surgeon that I had a sensitivity to "dairy". Once the hospital put me on a dairy free diet my condition began to improve immediately. I believe it is a specific protein that occurs only in cow's milk and eggs that causes the problem - sheep and goats milk/cheese etc. are fine (and delicious!). I have however never been entirely free of the U C, but sufficiently so to have been able to live a normal life until the liver problem became critical. But I cannot stress how important it is to ensure any treatment you have is tailored specifically to your needs - my specialist has made it clear that my combination of medical problems is both complex and, in his experience, unique.I sincerely hope you manage to find an empathetic source of medical advice to get you on the right path. It is possible that I am alive today because the gastroenterologist who was looking after me when my liver first started to cause problems* had a few weeks prior to a consultation been to a conference where he learnt about the UC / cirrhosis / P S C connection. He has now retired but I am eternally grateful that he was able to refer me to a doctor he had met who had the appropriate skills. As kristian said medical knowledge and skills are advancing all the time. Although the implication of bikafela's reply is that dissemination is patchy and I have had to educate myself so I can help the doctors by asking the right questions.

*btw my g.p. thought it was hapatitus.

[Liverandlife]

A thought has just struck me that, as a 15 year old kid, the surgeon may not have thought it necessary to explain why the complete removal of my colon was necessary.