There is no cure for Alagille syndrome. Management of the disorder is aimed at preventing complications, increasing the flow of bile from the liver, maintaining normal growth and development and reducing blood cholesterol levels.
Medications. Medicine may be used to increase bile flow and to relieve severe itching caused by buildup of bile in the blood and skin. These same medicines may be used to treat high cholesterol levels that cause the hard, whitish nodules that develop in the skin in Alagille syndrome patients.
Vitamin supplements. Reduced bile flow can lead to difficulty digesting fat and vitamins from a child's diet. Fat-soluble vitamin supplements (A, D, E and K) may be used.
Baby formula rich in triglyceride. Baby formula rich in a type of fat that can be digested despite reduced bile flow may be used to help with fat absorption in infants.
High-calorie diet. Alagille syndrome patients often have difficulty absorbing the calories they eat. To prevent malnutrition and growth failure, a high-calorie diet with a lot of protein may be recommended. A feeding tube that delivers large quantities of nutrients overnight may be used.
Liver transplantation. A small percentage of patients will develop cirrhosis (severe damage to the liver). Liver transplantation is the only option for these patients.
A liver transplant also may be the last resort for patients with severe itching, portal hypertension (high blood pressure) and severe growth failure that is not responding to medication. Liver transplantation has been used successfully in these cases.