PBC and progression

[Ablack98]

With having PBC, if you respond to the medicines, will you definitely still need a liver transplant say if you have PBC for 30-40 years? How much does Urso slow the progression since no matter what PBC always progresses?

[Hidden]

Hi Ablack98

I think that it is different for everyone as there are people on here who have had for years and never needed transplant or likely to but others that the progression is more aggressive.

Xx

[butterflyEi]

I cannot say to what will happen in 30-40 years they may have found a cure or alternative medication in that time as there is currently a good deal of research being done. URSO has been found to slow the progression and there is now Obetacolic Acid (OCA) for those who do not respond. In some cases Bezafibrate is being used by specialists in the right individuals. Each of us may share PBC but all of us seem to present slightly differently so I think it is impossible to say.

What works for me is a clean varied diet rich in vegetables low in salt. No processed foods or fizzy drinks like cola. It has also been proven and is recommended by the PBC Foundation that movement is medicine, I try to walk every day but it has been too hot for me recently so I have tried to swim when I can.

[kimphoebe]

Hi there,

As it happens spoke to my consultant from the QE yesterday and asked her a similar question. I’m a nonreponder to uso so now taking bezafibrates aswell. I seemed to have progressed to cirrhosis in only 6 years so I asked could I stay like this or get worst but she couldn’t give me the answer because everyone is different. She just said they will keep a close eye on it which is Okay with me. Bloods at the moment are normal so it’s a good sign. 👌

[Hidden in reply to kimphoebe]

Hi,

I’m about to start bezafibrate myself, I have been on urso for almost 2.5 years but it’s never brought my alp down. How are you getting on with bezafibrate?

[kimphoebe in reply to Hidden]

Hi there,

I seem to be ok on it, still get very tired and having a little more joint pain but apart from that nothing 👌

[Hidden in reply to kimphoebe]

Thanks for replying, good to hear, I think I’ll have to wait a couple of weeks for my prescription to be sent to my GP, hopefully it will do the trick with my ALP.

[kimphoebe in reply to Hidden]

Hi rosemadder9

When I started bezafibrates I had to have blood tests every 4 weeks for 3 months. Think it can sometimes effect your kidneys so please ask your gp bout that. 👌

[Hidden in reply to kimphoebe]

Thank you for letting me know, yes I will ask about that if instructions aren’t included in the letter from my consultant.

[ninjagirlwebb]

I agree with the replies thus far as each person is unique so can’t generalize.

Additionally, it also depends on how soon you were diagnosed. If pbc is caught at an early stage, and there is responsiveness to the meds, and it continues to progress at a slow rate, chances are you won’t need a transplant.

Most people diagnose with pbc at an early stage with responsiveness to urso or meds have the same life expectancy as the general population. This is confirmed by research that I’ve read.

[HermansMom]

I don't think there's a definite answer to this question, as like most others have said, the progression can vary greatly between individuals, even those that share similar stories (response to medication, same medication, age of diagnosis, stage at diagnosis, lifestyle, etc.). However, I do believe that certain factors can have an impact on possible outcomes, such as early vs. late diagnosis, stage at diagnosis (zero cirrhosis vs significant cirrhosis) and response to Urso.

I believe my PBC went un-diagnosed for about 16 years (when I first noticed symptoms) and I was stage 4 PBC (significant cirrhosis at time of diagnosis), however I appear to be responding well to Urso so far (been on it for about 2 months) and my LFT are lowering significantly and my bilirubin and albumin have always been normal, showing my liver, although diseased, is still functioning adequately. Although I may be more advanced than some others here, I do not suffer from the itch problem many struggle with, but I do have severe fatigue, joint pain and stomach issues.

I wish you the bet outcome. Since PBC is a slow, chronic condition, try (I know it's hard!) not to think too far into the future, but celebrate each positive outcome (lowered LFT's, response to medication(s), lack of some symptoms, etc) and focus on healthy living.

[Slm66]

pbc progresses differently not everyone with pbc requires a transplant

i was a non responder to urso and this led to my pbc progressing i had a liver transplant within 5 yrs of diagnosis im back on urso now again as the pbc is back 8 yrs post and it seems my liver is responding

ive known others with pbc that have been stable and have no progression of the disease

this is why blood tests are so important

[PBCRobert Partner]

Whilst every patient is different there are still generalisations.

Between 60&70% people with PBC respond to Urso.

If you do not respond to Urso, there is OCA (50% response in trials) and fibrates (30ish% response from memory). There are also new meds available.

If all else fails, there is the possibility of transplant.

If you do respond to Urso, we would anticipate normal life expectancy.

In terms of transplant (which is enormously successful in PBC and brings up the average for all liver transplant survival numbers):

There are approx 22,000 PBC patients in UK. Annual PBC liver transplant numbers sit at about 70.

Seventy. Per year.

That’s less than 0.3%.

Now even if nothing else changes and we remain at 70 transplants per year. That is stilll less than 3% of patients over 10 years.

Again, assuming there are no other improvemements, that would make under 9% over a 30 year period.

That is genuinely not a lot. Too many, absolutely. Yet still very small figures.

And we still need to factor in new improvements. We have not seen what OCA and fibrates will do to transplantation rates. It is now best practice to go on Urso again immediately after transplant which makes a difference (even in people who had not previously responded to Urso).

I hope that helps.

As ever, please see our range of webinars that were on PBC Day for more information.

[510amr]

I've known I have PBC for 28 years. My doctor says I will never need a transplant. Have used Urso all these years and stayed at about 1.5 times upper normal ALP. This past year I added Fenofibrate and now am in normal range for all liver panel reports/

[Odieweb]

That is a very good question so thanks for raising it. I myself often wonder about how the PBC will progress for me, and am lucky it was found early, so both taking URSO and slightly shifting my lifestyle and food intake, I hope I don't need a liver transplant. But as others have wisely said, we are best to live in the present and take one day at a time. After all there are many more things that could impact us - other health issues/accidents/etcetera - so trying to remain positive and proactive in one's health is what is important. I'm hoping to live for many years, as I love this gift of life. And if I need a transplant, and am fortunate enough to get one, so be it. What an amazing time we live in, when transplants are possible, and early diagnosis happens, and there is lots of research going on for PBC so who knows what treatments or possibly cures or preventions are ahead.