Sheriff Sarah

Sheriff Sarah

Monday, March 11, 2013

Pediatric Liver Disease Profile #1: Biliary Atresia

Throughout the coming months I'll be writing profiles of different pediatric liver diseases. This world used to be invisible to me before Sarah's diagnosis. I'm grateful to be part of a wonderful community of people who care for each other, advocate for their children and try to bring awareness to their own little parts of the world.

Up first, the disease that affected my family: biliary atresia.

Biliary atresia is a rare pediatric liver disease that affects infants in about every 10,000-20,000 births. Race can factor into this number, and females are generally affected more than males. 

The essence of biliary atresia is the liver's inability to excrete bile (the "garbage"). This is due to bile ducts being too small to be efficient or completely non-existent. Without the ability to rid itself of bile, the liver is poisoned, leading to a hardening of the organ. If not diagnosed within 8-12 weeks of age, serious complications can occur. The disease is already life-threatening to begin with but if diagnosis lags, the danger increases. 

Hardening of the liver (as in cirrhosis) can lead to impeded blood flow which causes varices, as the blood must find "alternate" routes through the body to the heart. These varices (essentially, internal varicose veins) first cause the spleen to increase to an abnormal size before before making a new path through the spleen to the esophagus and beyond. In the absence of immediate care, these varices can burst with serious ramifications. 

The first step in restoring the liver's ideal function is the Kasai surgery. The Kasai takes a piece of the small intestine and attaches it directly to the liver in order to allow bile drainage. Sometimes this is enough to restore the liver's health and no transplant is necessary. 

If the liver has been too badly damaged to continue for the long term, transplant is the only viable option. 

Standard symptoms include jaundice (body and eyes), distended belly, lack of appetite, white stool and lethargy. These may or may not ALL be present but if you have a question, you should request your pediatrician request blood tests. 

Links for Reference and Education:

National Digestive Diseases Information Clearinghouse

Cincinnati Children's Hospital

American Liver Foundation

American Pediatric Surgical Association

The Children's Hospital of Philadelphia

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