Biliary Atresia

What is Biliary Atresia?

 Biliary Atresia is a serious disease that occurs in young infants. It results in inflammation and obstruction of the ducts which carry bile from the liver into the intestine. Since bile cannot flow normally, it backs up in the liver. this results in jaundice, or a yellowing of the skin, and causes cirrhosis, in wich healthy liver cells are dstroyed and replaced with scar tissue. The scarring interferes with blood flow through the liver, causing more cell damage and scarring.

The cause of biliary atresia has not yet been discovered. It affects approximately one infant in every 15,000 live births. It is not known to be a hereditary condition.  It also is not caused by anything done by a parent or sibling.

What is treatment for biliary atresia?

There is no cure for biliary atresia. The most successful treatment for biliary atresia to date is a type of surgery that allows drainage of bile from the liver when the ducts have become completely obstructed.  This operation is called the Kasai procedure.

In the Kasai procedure, the surgeon removes the damaged ducts outside of the liver and replaces them with a length of the baby's own intestine, which acts as a new duct.

The aim of the Kasai is to allow excetion of bile from the liver into the intestine via the new duct. The operation accomplishes this about 50 percent of the time.  In the other 50 percent, the problem lies in the fact that the obstructed ducts are inside the liver.  There has been no procedure developed to correct this condition other than liver transplant.

About 2/3 of the patients who are diagnosed and have this operation will go on to require a liver transplant by the age of 20. Depending on the success of the operation and how well the recovery goes will determine the amount of time until a transplant is required.  The other 1/3 essentially get a transplant at some point later in life.


Will there ever be a cure?

There cannot be a cure until a cause is known.  Research is the key to solving the mystery.


All of the above info was provided in a pamplet put out by the American Liver Foundation.  For more info, visit their site at liverfoundation.org.


What this all means for our family:

We don't know at this point.  We know that she will have this surgery and that we will have to plan on a tranplant at some unknown point in her life.