Primary biliary cirrhosis

PBC

I’ve been asked by a friend who suffers from this disease to help create awareness. I’ve never heard of it so had to look it up, and have summarised it below.

For those who are non-medical it can be described in a few words;
You look like shit, feel like shit, can’t shit, and are not even allowed a drink to drown your sorrows. 

Despite everything, my friend manages to put on a brave face and keep her sense of humour. Keep smiling Karen. Let’s hope greater publicity helps in finding a cure. 

 

Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver.

It is marked by the slow progressive destruction of the small bile ducts. When these ducts are damaged, bile and other toxins build up in the liver and over time can lead to scarring, fibrosis and cirrhosis.

PBC was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3–4,000 people and in the ratio of at least 9:1 female to male.

There is no known cure, but medication may slow the progression.

In advanced cases, a liver transplant, if successful, may result in a favourable prognosis.

Those with the disease suffer from the following;

Debilitating Fatigue

Pruritus (itchy skin)

Jaundice (yellowing of the eyes and skin)

Xanthoma (local collections of cholesterol in the skin, especially around the eyes

Complications of cirrhosis and portal hypertension

Fluid retention in the abdomen

Enlarged spleen 

Oesophageal varices 

Hepatic encephalopathy, including coma in extreme cases in more advanced disease.

In up to 80% of cases it is associated with an autoimmune disorder such as rheumatoid arthritis or Sjögren’s syndrome.

Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E,

As in all liver diseases, consumption of alcohol is unadvisable.

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