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What is PBC?

© Elizabeth Batt

A few short months ago I didn't know what Primary Biliary Cirrhosis was. I'd never heard of the disease and did not know that this disease existed. It was only after receiving a phone call from my dad who told me he had been diagnosed with Stage 4 PBC that I began to delve into what this awful disease entailed.

People hear the word cirrhosis and often immediately equate it with alcohol. What most fail to realize is that cirrhosis is a scarring of the liver that can be caused by things other than alcohol. Hepatitis C for instance can also cause liver scarring, as can PBC.

What my dad first told me about the disease was that there was no cure. While medications can slow the progression of PBC, there is no magic formula for a restoration of health. As the disease progresses and the liver becomes unable to function, a liver transplant is often the only option. A transplant does not cure PBC but it is known to buy a sufferer time. Please note though, PBC is a slow moving disease and some sufferers are symptom free for years. Those without symptoms often have a very good prognosis in that PBC may never affect their overall health.

It was while conducting research to enable me to better help my dad, that I discovered that PBC may run in families. While it isn't classed as hereditary as such, it has been found in mothers and daughters, as well as siblings. One of the symptoms of early PBC is pruritus - intense itching, usually on the palms of the hands and the soles of the feet. Seeing as I had presented myself at the doctors with this some month's prior, I figured the best course of action was to get myself tested. On November 25, 2003 after various tests, I was also diagnosed with PBC.

What is PBC?
Although unsure of the causes, specialists term PBC an autoimmune disease. This means that the body's own defense system attacks the tissues of the body, in this case the liver. As the liver is attacked, the small bile ducts of the liver become inflamed and then destroyed. These ducts carry bile to the small intestine. Bile is a substance that aids digestion and carries away waste products. When the small bile ducts are damaged, they block the flow of bile and thus the bile becomes backed up in the liver. As the inflammation progresses, it begins to spread to liver cells and coupled with the toxicity of the bile, scarring (cirrhosis) occurs. When cirrhosis is evident, a person is seen to have end stage PBC.