Exactly What Is PBC ?

[sapphire73]

Can anyone tell me exactly what PBC?

Also is it hereditory?

[Brummi]

Hi, PBC is an autoimmune disease , where the body's own defence system attacks the liver. Why the body does this is not known, also it is not hereditary. It happens to females more then males. I had this and was transplanted in June 2013.

[sapphire73 in reply to Brummi]

Brummi,

Thankyou very much !!!

You have put it direct... I will post again when I get all test results back.

2yrs since your Transplant "Congradulations" Keep up the Good Life. xxx

[youngatheart in reply to Brummi]

My consultant is of a different view. Yes it can be hereditary. Believes on the maternal side only. Two years ago I lost two cousins within 6 months of each other due to having the disease for many years. They were sisters. I had only found out that I had the disease two years previous to that not knowing they had it too. At least I had someone to discuss with.

[Brummi in reply to youngatheart]

Hi, I think this goes to show just how little we know about the disease and the difference of opinions in doctors . I had been ill for years and never knew it. It seems strange that one can be so ill and know nothing about it...

[youngatheart in reply to Brummi]

Think it is because it is not part of blood test. Only found out because I was getting bad joint pains. Got tested for arthritis. Came back PBC. god knows how long I've had it.

[Brummi in reply to youngatheart]

Sort of creeps up on you does'nt it. When I got told I was also told it was mainly a females illness I went straight home and checked my birth certifcate....

[bluetopia86 in reply to youngatheart]

Similar story here! I was scheduled for a gallbladder removal and when they opened me up half my liver was chirosed. I was 20/21 at the time. They told me I was probably born with it, but since most babies are born jaundice it went unnoticed.

[Curlymac]

Actually the liver can be perfect with PBC. It's the bile ducts that lead from the liver that are affected. They become clogged and scarred. Therefore can not carry the bile from the liver effectively to the pancreas and other areas. It's like having a perfectly good sink ( the liver) and clogged drain pipes ( the bile ducts). Eventually because the pipes don't work and will not drain the sink, the sink becomes corroded and will need replacement. So the draino to clear the drain pipes would be represented by the pill called Ursodiol which acts to clear the build up in the ducts. It clears away the walls to make them work more effectively. Same drug used to blast away gall stones in some cases.

[Hidden in reply to Curlymac]

Curlymac, Love your description of PBC.

[Maribella in reply to Curlymac]

Clever analogy Curlymac, I'm impressed!

[Prakash_hathi]

also, transplant may not be required in every case.

my PBC detection (april 2013) was way too early and i'm on urso since then. there's been remarkable improvements and just last week my doctor told me that there's a chance that i may not need a liver transplant!

[Hidden in reply to Prakash_hathi]

Regarding liver transplants. I prefer not to think a bout jumping steps ahead and choose not to go there as to me it is something that in the majority of cases will never be. So why worry about something?

I was fortunate that the consultant I saw briefly outlined PBC when I was diagnosed. On my first return visit 2 months after starting urso (he got my GP to inform me he had was diagnosing PBC and write the urso scripts out), he just ended his short chat (as I had informed him that I already was a bit in the know information-wise about PBC) with 'it can in some cases lead to transplant'. He said no more. I got the feeling he is a doctor who will deal with things as and when and that is my aspect of having PBC.

[Sassyr]

I was incredibly lucky, went to GP after much persuasion from family and friends because I was constantly tired, I had blood tests and then they were repeated - all along I expected to be told I was anaemic. My GP picked up on the tests that she thought I possibly had PBC, referred me to a consultant and I've been on urso since then. During some of my testing it was picked up that I had an aneurysm in my brain. This led to a coiling op with a stent, unfortunately I had a a haemmorhage but I couldn't have been in a better place to have it dealt with. Consider myself to be very fortunate indeed.

[liver-bird]

Curlymac's analogy is spot on. PBC is thought to be an auto immune malfunction and auto immune problems tend to run in families. My mother and two of her siblings had rheumatoid arthritis (another auto immune) I had a cousin with MS (another ai) and I have pbc.

[Hidden in reply to liver-bird]

I have PBC and I'm sure my father had it too. He had fatique and joint pain just like me but he also had very itchy skin which luckily I don't.

[cazer]

Ive been diagnosed since1999.been on urso since then.my understanding is that pbc is an autoimmune disorder where the bodies own rogue antibodies attack the bile ducts in the liver which gradually furs up the drainage for the liver.the anti bodies are anti mitochondrial and mitochondria are the bits in cells which make energy....hence im assuming the is where the fatigue comes from. Autoimmune conditions e.g

Pbc ms diabetes etc run infamilies so hence its sort of hereditary

Pbc is more common in women but may not go directly from generation to generation. Hope this is helpful

[Hidden]

If you've not already done so, check out Liver North's website, they can supply free of charge a dvd all about PBC.

They also have their back issues of newsletters online too.

There is also the PBC Foundation. They do have their binder online these days too.

I'm not sure about the hereditary part. I think in today's world it seems easy to just pin certain things with labels. There are conditions out there that can be passed on to one's offspring (haemophillia is one) but it's not to say that every generation will succumb.

I just don't think about things like hereditary as once we are born without our parents knowing or being aware that one day they might acquire a certain condition (I was 46 when I was informed I had PBC. I had 2 grown-up children by then. I can't say if my mother might have developed PBC as she died aged 43 of something totally unconnected). I choose to get on with life now, I'm not one for moping about wondering how this PBC will become. I think life is a lot of things that may or may not be and we often here the phrase, "the wrong place at the wrong time", etc and that we live quite by chance anyway.

livernorth.org.uk/

[normadawnreid in reply to Hidden]

A note to say I have adapted your very helpful attitude over the past year. This time last year I was in a flat spin, just being diagnosed, gone through all the emotions including denial. I rely on the the basics, rest, eating well and walk/run most days. I was exhausted before starting urso last year but I feel much better with lifestyle changes (eating more frequently and listening to my body). Your attitude of living your life and not getting caught up in what may never happen is what I've learned, and this was from you. Thank you. I still wonder if taking the brand name urso, is best. It is very expensive although my insurance just agreed to pay after getting a note from my doctor. I had to change to the generic as it took time to convince them, but there doesn't seem to be much difference on either. any opinions on that. Thanks again

[Hidden in reply to normadawnreid]

Hello normadawnreid.

Thanks for the posting. I try not to look at PBC in a bad way. I can remember back to 2010 when I started itching and every time I saw the GP which was frequently, he would undertake yet another blood check for something and turn up nothing and then say the LFTs were still coming up abnormal until he then did some blood check to decide it was liver-related and not bone-related.

I stumbled across PBC in a library book and then looking on internet came across the PBC Foundation first. I hoped I didn't have PBC and never said anything to my GP as I didn't want to have more than a temporary helath issue that would go away.

Sadly for me like everyone else on this site I was to be informed I had PBC by the end of 2010. The reading I did prior to ever being diagnosed came across to me as not being so bad in one sense and I had read the wording to the effect that PBC is 'slow progressing' and that it is highly likely PBC patients would more than likely die of something unrelated than the PBC.

I took the diagnosis not as shockingly as I thought I might have looking back but I think that was because I had been informed what I had and from then I could start to get on with life and knew that for the present I would just have simple basic health checks and take the urso. I'm still here over 4 years on and feel pretty much normal again.

I think if one is to think of other things that they might have noticed before they were diagnosed with PBC and then realise they no longer seem to have those minor issues to me it can be a pretty good sign. I know back in 2010 and probably just before this year, my eyes had pretty bad shadows beneath them and when I was tired if I rubbed my eyes I would end up making them like someone had given me a right hook. I have noticed in the last year my eyes look normal, the shadows are very faint and at times if I do rub my eyes I don't see the effects of it afterwards. I have also noticed that bruises that I occasionally encounter from a knock into something seem to vanish pretty quickly and even odd knicks and cuts (I tend to acquire frequent paper and even cuts from plastic) heal rapidly these days. I think due to even better care of myself and also the fact that by taking urso it has improved things greatly for me.

I originally took a brand in urso in 300mgs (I was on 600mgs per day) and I did quite well with those. Then in summer 2013 they were withdrawn here in the UK due to some production issue. I had to be prescribed 150mgs so now I have 4 of these tablets daily. I was originally given generic at the pharmacy but I suffered dreadful heartburn that persisted continually. I did encounter this in the beginning of urso 300mg tablets but it did vanish after a few months. I ended up asking the pharmacy if they had any other 150mgs as I had read on this site some were on a different one that was branded (Destolit here in the UK). The pharmacy obliged though my prescription has always been written generically (600mgs daily of ursodeoxycholic acid). Then when I returned 3mths later for the next script, I was told I could not have the brand, I had to have generic. I went back to see my GP and explained, she said that in my case and due to side-effect that I wasn't encountering with the Destolit I could have the brand script and that is how I have it written now. I put it down to the filling agents used in our urso that is the cause there.

I think if you are doing well with any of the urso available then I personally wouldn't change. I'd have had no issue with the generic 150mgs I was presribed but for heartburn returning on taking like it did with my originaly 300mgs. I do know that brands are dearer to the NHS here in the UK than generics but in my opinion if a patient is deemed to have some medication for life and it halts or permanently ceases any other NHS intervention then that to me has to be the cheaper option. I have to pay the prescription charge to the NHS that we pay for each item so I refuse to think any other way here with regards to the urso.

I had to fight to get the urso in a more lengthy prescription though. I originally managed to get one GP to write it out for 90 days but then another GP got to sign the repeat and he questioned it and rang me stating I could only have 28 days. I went in to see him as I was not happy. He gave me some convoluted reply that I knew was wrong (I had meanwhile wrote to my MP about this so-called 28 days being a month's supply of tablets and receive a copy of the Health Minister's reply). I managed to get him to write the script for 84 days and got those for 2 prescriptions. I then went back to see the GP I was seeing for PBC last summer and explained that it was

daft 84 days as the tablets come in boxes and I always have to receive a broken box as they are in boxes of 15 days. She relented and I receive the 90 days again.

Generic urso is supposed to be the same as in the active ingredient (in this case the ursodeoxycholic acid) but as I have noticed on checking different urso (a lot seem to take 250mg or 500mg tablets, they are by a different pharma), the only difference is the fillling agents, some contain talc for instance, some don't.

[cazz22]

I also love curly macs description.will borrow it for future use😃😃

I disagree it's hereditary.both my parents are 90/91 and disgustingly well.i take careful note on their routine blood tests with envy.my much older brother is ok so are all aunties and uncles.im interested in genetics anyway and have gone back generations.you would think I stood a great chance of immortality as g g grand parents grand parents all lived to 90/100 fit and active too!

I live in south yorks very close to Hickleton pit(now derelict)for the last couple of decades I have walked twice daily on the nature reserve there with my dogs.cant prove it but I think that triggered mine

[Curlymac]

Gee thanks Cazz22 and Liver-bird for liking my quirky description of PBC. Hope you all have a great weekend and stay well. :). Spring has sprung in British Columbia and always makes me feel renewed.

[sheilaro]

I also like Curlymacs description. My route to diagnosis began 18 years ago. I had a severe cough which persisted and led to three broken ribs over 18 months. I felt awful, was dreadfully tired and treated for asthma but I knew it was not like other peoples' asthma!! The chest consultant made me feel like I was neurotic and looking for attention but my GP (a family friend) knew me well and referred me to rheumatology and haemotology. It was then I was informed that potentially I may have PBC because of the prescence of Antimitochondrial Antibodies.

The Gastro enterologist told me I very probably had PBC but did not want to do a biopsy straight away. He likended the liver to an Oil refinery with all the pipes involved in processing the oil. If one pipe got blocked or malfunctioned the refinery could often keep going without intervention. The engineers monitored the situation ( blood test) and would eventually intervene to confirm problem (biopsy). This analogy was quite helpful at the time but your one about the sink is better!! I was put on Urso straight away and had my Biopsy at the end of 2000. I hope I never have need a transplant and consider myself very fortunate as progress has been slow. My cough improved fairly quickly after going on Urso (no real explanation !!) and am a 9 year survivor from Breast Cancer. I turned down the chemotherapy, because of the effect it imay have had on my liver and have no regrets.

Great believer in keeping positive!!