Faye's journey through Biliary Atresia

What is Biliary Atresia?

Biliary Atresia is a rare liver disease that only affects infants. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats.
In biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.

What causes BA:
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 defects. 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.

How is BA diagnosed:
Since other conditions lead to symptoms similar to those of biliary atresia, doctors must perform many tests before a conclusive diagnosis can be made. These tests 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.

How is BA treated:
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 after Dr. Morio Kasai, the Japanese surgeon who developed it.
The aim of the Kasai procedure is 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 3 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.
The Kasai procedure is most successful in babies younger than 3-months-old, so early diagnosis is important.
If the Kasai procedure is not successful, the only other option is a liver transplant. However, a suitable donor organ must be found quickly, before damage to the liver from the backed-up bile becomes deadly.

Babies with BA:
Without successful treatment, few children with biliary atresia live beyond age two. In some cases, where the Kasai procedure is completely 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.

Transplant:
In children with biliary atresia, a liver transplant is generally not attempted unless the Kasai procedure has been tried. In cases where that operation is unsuccessful, the child may become a candidate for a liver transplant. Success depends on the timely availability of a matching donor liver. Fortunately, survival rates for liver transplant recipients have increased dramatically with improved surgical techniques and the development of drugs that help overcome the problem of organ rejection.

Faye's journey through Biliary Atresia

Thursday, September 6, 2012

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