Biliary atresia is a disease of the bile ducts that affects only infants. It is rare, affecting about one out of every 18,000 infants.The disease is more common in females, premature babies, and children of Asian or African American heritage, according to the National Digestive Diseases Information Clearinghouse.
In biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile – a digestive liquid that is produced by the liver -- to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver. It is the leading cause for liver transplants in children.
The cause of this disease is not known. In some infants, the condition is most likely congenital, meaning present from birth. About one in 10 babies with biliary atresia have other congenital conditions. Some research indicates that an early viral infection may be linked to biliary atresia.
Scientists do know that biliary atresia is not hereditary; parents do not pass it on to their child. It is also not contagious, and it is not preventable. It is also not caused by anything an expectant mother did or did not do.
Symptoms of biliary atresia usually appear between two and six weeks after birth. The baby will appear jaundiced, with yellowing of the skin and whites of the eyes. The liver may harden and the abdomen may become swollen. Stools appear pale grey and the urine may appear dark. Some babies may develop intense itching.
Since other conditions have symptoms similar to those of biliary atresia, doctors must perform many tests before a conclusive diagnosis can be made. These may include blood and liver tests, an ultrasound examination, X-rays and a liver biopsy, in which a small amount of liver tissue is removed with a needle for examination in a laboratory.
Unfortunately, there is no cure for biliary atresia. The only treatment is a surgical procedure in which the blocked bile ducts outside the liver are replaced with a length of the baby’s own intestine, which acts as a new duct. This surgery is called the Kasai procedure and is used to allow drainage of bile from the liver into the intestine through the new duct. The operation is completely or partially successful about 80 percent of the time if performed early (before three months of age). In babies who respond well, jaundice and other symptoms usually disappear after several weeks.
In cases where the Kasai procedure does not work, the problem often lies in the fact that blocked bile ducts are “intrahepatic,” or inside the liver, as well as “extrahepatic,” or outside the liver. No procedure, except for liver transplant, has been developed to replace blocked intrahepatic ducts.
When the Kasai procedure is successful, the child may recover and live a normal life. However, in most cases, even when surgery is successful, patients will suffer gradual damage to the liver. These children will need specialized medical care throughout their lives, and many will ultimately need a liver transplant.
But children with biliary atresia can and do lead normal lives. Nick, John and Grace can attest to that. Read their stories here.
For more information about biliary atresia, call the American Liver Foundation’s National HelpLine – 1-800-GO-LIVER (1-800-465-4837) or visit us at www.liverfoundation.org.