Biliary atresia
At 7-weeks-old, Michael was diagnosed with
biliary atresia. His parents turned to our world-class pediatric liver transplantation team for the procedure that would save his life.
Read more.
Treatment for biliary atresia
Specific treatment for biliary atresia will be determined by your child's physician based on the following:
- The extent of the problem
- Your child's age, overall health, and medical history
- Your child's tolerance for specific medications, procedures, or therapies
- Expectations for the course of the problem
- The opinion of the physicians involved in the child's care
- Your opinion and preference
Biliary atresia is an irreversible problem. There are no medications that can be given to unblock the bile ducts or to encourage new bile ducts to grow where there were none before. Until that happens, biliary atresia will not be curable. However, two different operations can be done that will allow the child with biliary atresia to live longer and have a better quality of life. Your child's physician can help determine whether either of these operations are an option.
Kasai portoenterostomy
- This operation connects the bile drainage from the liver directly to the intestinal tract. It is most successful when done before an infant is 8 weeks old. The Kasai procedure is helpful because it can allow a child to grow and remain in fairly good health for several years.
- Eventually, cholestasis (backup of bile in the liver) will occur, causing liver damage. Up to 80 percent of children who undergo the Kasai portoenterostomy will eventually need to have a liver transplant.
Liver transplant
A liver transplant operation removes the damaged liver and replaces it with a new liver from a donor. The new liver can be either:
- A whole liver, received from a child who has died.
- Part of a liver, received from a child who has died.
- Part of a liver, received from a 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 invasion of viruses, tumors, and 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. More here on liver transplants.
Nutrition and biliary atresia
Before your child has either one of these operations, nutrition may be a problem. 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 of a faster metabolism. 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.
- Supplement your child's diet with vitamins, as directed by your child's physician.
- MCT (medium-chain triglyceride) oil or infant formulas with MCT (Portagen® or Pregestimil®) may be recommended to add extra calories to 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.
- 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 that your child have liquid feedings given 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.
- After surgery, your child's digestion may return to normal, or you may still need to give extra vitamins and/or work with your child's diet. Please consult your child's physician for recommendations.
What is the long-term outlook for a child with biliary atresia?
Many factors affect the long-term outlook for these children. Some of them include:
- The extent of bile duct damage
- The age at which either a Kasai portoenterostomy or liver transplant is done
- The extent of liver damage that has occurred
- The overall health of your child
- More than 65 percent of children who have the Kasai portoenterostomy eventually require a liver transplant.
- After liver transplant, the child's health will usually improve; however, a rigorous medical regimen must be followed.