Biliary atresia is a disease of the liver and bile ducts that affects newborns. There are two distinct forms of biliary atresia. The most common form is thought to develop very shortly before or very shortly after birth and is sometimes called postnatal biliary atresia. The less common form of biliary atresia (just 10-35% of cases) is called embryonic or prenatal biliary atresia and appears to begin very early in embryonic development and is sometimes associated with other abnormalities, such as multiple spleens or situs inversus (left-right location of internal organs is reversed). In both types of biliary atresia, inflammation of the bile ducts outside the liver occurs first and progressively the ducts scar and eventually wither and close. Once the ducts are scarred shut, bile cannot flow normally to the small intestine. Liver cells produce bile at the normal rate, but it is trapped inside the liver, causing progressive liver damage.
My name is Dorothy and my youngest son Nick is the inspiration for this website. Nick was born in 1994 and diagnosed with biliary atresia at the age of 8 weeks. He’s 15 years old this year and still buzzing along nicely with his “original equipment.” Although he hasn’t needed a liver transplant we know that can change, and probably will. I try not to dwell on it too much, but biliary atresia and liver transplants are topics that I think about, read about, and research on a regular basis.
