1. Surgery: Kasai procedure
The Kasai procedure is the preferred treatment for biliary atresia at The Children's Hospital of Philadelphia and has been performed here for more than 30 years. The operation connects the bile draining from the liver directly to the gastrointestinal (GI) tract. It is most successful when done early in the disease process.
The specific age to obtain the best results from the Kasai procedure is unknown because biliary atresia is a rare disease and manifests differently in every child. Researchers have reported varying results over the past three decades. Some suggest the Kasai procedure is best completed before the infant is four weeks old, while others say the best rest results are produced when the operation is completed by the time the baby is 8-10 weeks old.
While the Kasai procedure is not a cure for biliary atresia, it is the preferred treatment because it allows a child to grow and remain in good health for several years. A successful Kasai procedure delays the need for a liver transplant. In rare cases, the Kasai can actually eliminate the need for a liver transplant.
2. Nutrition
With biliary atresia, not enough bile reaches the intestine to assist with the digestion of fats in the diet. Protein deficiencies may occur due to liver damage. Vitamin deficiencies may also occur. Children with liver disease require more calories than a normal child because they do not absorb the same amount of calories and nutrition. Your physician may recommend that a pediatric nutritionist make recommendations regarding your child's diet. Nutritional guidelines may include the following:
__Provide your child with a good, well-balanced diet. Sometimes special infant formulas are required.
__MCT (medium-chain triglyceride) oil may be recommended to add extra calories to the diet and help your child grow. Medium-chain triglycerides are more easily digested without bile than other types of fats. MCT oil can be added to foods and liquids that your child eats.
__Supplement your child's diet with vitamins, as directed by your child's physician.
__Provide your child with high-calorie liquid feedings, as directed by your child's physician. Some children with liver disease become too sick to eat normally. In this case, your physician may recommend your child have liquid feedings to help meet his/her body's requirements. These feedings are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus, and into the stomach. A high-calorie liquid can be given through the tube to supplement your child's diet if he/she is able to eat only small amounts of food, or to replace meals if your child is too sick to eat. These special diets are especially important in the first year of life when the child’s growth is most rapid.
3. Liver Transplant
A liver transplant removes the damaged liver and replaces it with a new liver from a donor. The new liver can be:
__A whole liver, received from a donor who has died
__Part of a liver, received from a donor who has died
__Part of a liver, received from a living relative or other person whose tissue types match the child's tissue type
After surgery, the new liver begins functioning and the child’s health often improves quickly.
After a liver transplant, children will need to take medications to prevent the body from rejecting the new organ. Rejection occurs due to one of the body's normal protective mechanisms that helps fight against an invasion of viruses, tumors or other foreign substances. Anti-rejection medications are taken in order to prevent this normal response of the body from fighting against the transplanted organ.
Frequent contact with the physicians and other members of the transplant team is crucial after a liver transplant.