I’m pausing today before getting into how I was diagnosed with PBC, aka my year of living dreadfully. If you saw the kind comment for my next-to-last post, you probably want to know more about pbcers.org, and I’m happy to oblige.
The first stop site for those who have been diagnosed with PBC, suspect such a diagnosis is coming their way, or have a loved one with PBC is the Primary Biliary Cirrhosis Organization: pbcers.org. This is their statement of purpose:
The Primary Biliary Cirrhosis Organization aka PBCers is a wonderful source of support and education for PBC patients, family members, friends and other autoimmune liver disease patients. Formed in 1996, the PBCers is the largest online PBC support group with almost 3,000 members worldwide.
As a group, we discuss medical information, pain management, medications, research, ask questions, transplantation, vent our anger & fears, speak freely on the ups and downs of daily living, share our personal experiences and build lasting friendships.
On its website, you will find a chat room, doctors panel Q & A, contact info for local support groups, conference announcements, and other services. Educational materials are also there to download; one of the organization’s aims is to increase awareness of what PBC is.
To learn more about other people’s experiences with PBC, I’d start with these two services at pbcers. org:
- Yahoo Group Daily Digest: Because this is a daily digest of that day’s forum postings, you get a single email a day instead of an email each time someone posts a comment. There is also an archive of the past nine years’ messages on the sign-up page.
- PBCers Organization on Facebook.
Yesterday when I wrote about factors associated with increased risk for PBC, I don’t think I was very clear. These are not causes of the disease. If they have any relevance at all, it is that they may be triggers for whatever mechanism sets the autoimmune process in motion. My understanding is that you can have a set of people all of whom have a genetic predisposition for an autoimmune condition, but just a few ever actually develop one. So the mystery is what triggered the conditions in those who got sick — how do they differ from those who didn’t? Something — or more likely some combination of things — must have happened to light their fuses.
Another source of information:
There’s a good resource, copyright free, on at the National Digestive Diseases Information Clearinghouse, part of the National Institutes of Health of the U.S. Department of Health and Human Services.
Here’s their summary statement, and a diagram showing those bile ducts you’re hearing so much about in these posts:
Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear. Bile is a liquid produced in the liver that travels through
the bile ducts to the gallbladder and then the small intestine, where it helps digest fats and fat-soluble vitamins A, D, E, and K. When the bile ducts become damaged from chronic inflammation, bile builds up in the liver, injuring liver tissue. . . .
Primary biliary cirrhosis develops over time and may ultimately cause the liver to stop working completely. Most people are diagnosed early, before the disease progresses. Early treatment delays—but does not stop—the eventual onset of cirrhosis and liver failure. When a person has end-stage liver disease, a liver transplant is necessary for survival.
And these are their “Points to Remember”:
- Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear.
- Injured liver tissue from chronic inflammation and the buildup of bile leads to cirrhosis, a condition in which the liver slowly deteriorates and malfunctions.
- The cause of primary biliary cirrhosis is unknown. Most research suggests the disease is an autoimmune condition.
- Primary biliary cirrhosis is more common in people who have a parent or sibling—articularly an identical twin—with the disease.
- Most people are diagnosed early, before the disease progresses. The disease is often discovered when routine blood tests to check liver function are abnormal.
- Many people with primary biliary cirrhosis do not have symptoms until after the disease is diagnosed.
- The first and most common symptoms of the disease are itching, called pruritus, and fatigue. Other symptoms include dry eyes and mouth and jaundice.
- The anti-mitochondrial antibody (AMA) blood test, the alkaline phosphatase blood test, and a liver biopsy may be necessary to confirm a diagnosis of primary biliary cirrhosis.
- Some of the complications of primary biliary cirrhosis are osteoporosis and maldigestion.
- Early treatment delays—but does not stop—the eventual onset of cirrhosis and liver failure.
- Ursodiol (Actigall) is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.
- Liver transplantation is the only treatment that will cure primary biliary cirrhosis. A liver transplant is considered when complications cannot be controlled by treatment.