Primary Biliary Cirrhosis: Basics, 1

My last post was a personal account of my ongoing experience with primary biliary cirrhosis (PBC), and I’ll be offering more of that, but first I need to explain a bit about this condition. I have yet to encounter someone outside the medical world who knows about it, although “PBC is considered to be an uncommon disease, but not rare,”  according to MedicineNet, the source I will most rely on here.

Of course there are a number of sites like MedicineNet which have been written and reviewed by the medical profession. This is no substitute for those by any means. But I’ve had some experience with the kinds of questions that people who are not worried about personally being affected ask first, while the health sites I think are usually written for people who have been diagnosed and need more systematically organized info.

First, the name. The unfortunate name.

The primary is OK — it is primary because not secondary or tertiary. It isn’t a result of another condition.

Same goes for biliary: it involves the bile ducts.

As for cirrhosis, it’s unfortunate on two counts.

To begin with, it isn’t entirely accurate. The condition eventually leads to cirrhosis; it isn’t a special variety of cirrhosis.

Secondly, cirrhosis is a loaded word. I bet most people think they know what causes cirrhosis of the liver: chronic alcohol abuse. Some might come up with another cause: hepatitis C (and what causes that? Dirty needles?). And, yes, these are the two most common causes of cirrhosis, and both are, to a greater (chronic alcohol abuse) or lesser (hepatitis C) extent  related to choices people make. And we all know that there are those who lack sympathy for alcoholics’ or IV drug users’ health problems.

There are, by the way, a number of other causes of cirrhosis of the liver besides those two, including, in addition to PBC, others that have nothing to do with substance abuse, like haemochromatosis (too much iron in the blood) and Wilson’s Disease (too much copper).

The problem is that

… the name primary biliary cirrhosis is actually a misnomer for patients in the earlier stages of the illness. The more technically correct and ponderous term for PBC, chronic non-suppurative destructive cholangitis. . . has never been widely used and is unlikely to replace PBC.

Chronic non-suppurative destructive cholangitis: I’ve got to agree, that’s not likely to catch on, is it?

So what does cause PBC?

Most of the introductions I’ve read say it is an autoimmune condition, but MedicineNet is a little more cautious in answering the question:

The cause of PBC remains unclear. Current information suggests the cause may involve autoimmunity, infection, or genetic (hereditary) predisposition, acting either alone or in some combination.

. . . PBC is presumed by most experts to be an autoimmune disease, which is an illness that occurs when the body’s tissues are attacked by its own immune (defense) system. (Auto means self.)

. . . Despite strong evidence to support the concept that PBC likewise is an autoimmune disease, some features of PBC are uncharacteristic of autoimmunity. For example, all other autoimmune diseases occur in both children and adults, while, as already mentioned, PBC has never been diagnosed in childhood.

 

Next time: Who gets it

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8 thoughts on “Primary Biliary Cirrhosis: Basics, 1

  1. UAB is a good place to go — that’s where I’ve been going since I was diagnosed in December 2006. They do things a bit differently than some others — I have never had a biopsy. Their theory is that the results of a biopsy wouldn’t change the treatment approach (I’ve responded — at least my bloodwork has improved for what it’s worth [which may not be much] — to Urso Forte). Having esophageal varices isn’t what I fear most — it’s having them and not knowing I do. I have had 3 bleeds and maybe 8 endoscopies. I had varices banded in October, but had another bleed early March. I had an endoscopy April — no varices to band. Now I’m having one tomorrow at the local hospital, just in case. I know we will never beat this thing, but I’ve dug in my heels and am going to give it hell.

  2. Hello Everyone. I was diagnosed with PBC 13 years ago. Went thru numerous tests, blood work and etc. Then a liver biopsy. Yes, PBC raised its ugly self. I have been for my second biopsy three months ago and waiting for my appt to UAB liver hospital in Alabama. Iam in Florida appx five hours away. Don’t really know what to expect but will be checked for Esophagus Varices. If shows even a trace thats stage four. Scared to death. Actigal and numerous meds did nothing for me. Scared to death but “gotta do whatcha gotta do”. God luck to All of you, cause you have a long tired road to travel.

  3. Dear Brooks,
    If you read materials from before 2000, the life expectancy predictions are very bleak. Once the drug Ursodiol (Urso Forte) entered the picture, life expectancy predictions for those who responded to it improved markedly — some even say that there is no significant difference between people who were caught in the early stage and respond to Urso, and the normal population.
    If you look on my PBC page here on the blog, you will find a list of the sources I found useful.
    What I think is most important is finding a hepatologist (not just a GI) with experience with PBC, even if it means traveling to do so since you might not be making that frequent of visits (at first I was seen at a 3-month interval, then six, then a year for three years, now back to six months).
    I have never been “staged” — my doctor doesn’t seem to think that is useful, preferring instead to deal with individual cases instead of placing individuals into groups.
    Don’t panic — you are in for a long haul. Good luck and best wishes.

  4. I was diagnosed yesterday…The Dr. did not tell me what stage, but it has taken 6months for them to diagnosis. He did tell me that I had this in 2005 and wasn’t caught. I have several of the symptoms that I have read about on the internet. I am in quite a panic….Which websites are the mosst informative?? What about literature … I have read that life expectancy is 7.5 years, 10 years, 15 years??? Do you know if any of this is true?

  5. My Specialist told me that I have got BPC last April 2011. He put me on Actigall, which gave me so much side-affects and side-afftects remind me of BPC constantly.

  6. Yes, and thank-you. I plan to mention the work pbcers.org is doing and the support offered in their forums very soon. Best wishes.

  7. Your blogpost came up in my Google alerts. Thanks for helping spread the word about PBC. I have stage one, no symptoms. I’m hoping it stays that way! Do you know about http://www.pbcers.org? I read their Yahoo group posts daily and it really helps me feel that I’m not alone.

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