Cystic fibrosis (CF) is a genetic disease in which thick secretions cause chronic lung disease. Bile also may be too thick to flow properly, scarring the liver. In most CF patients, the mild scarring does not cause clinical problems. In a small percentage of children significant liver disease develops. As the liver develops cirrhosis, blood backs up into the spleen and esophagus. Bleeding, an accumulation of abdominal fluid (ascites), and other problems can develop. In advanced disease, jaundice and poor blood clotting are seen.
Children with CF who have liver complications should be treated by a pediatric GI specialist. Medicines and nutritional support are prescribed to keep them as healthy as possible. A medicine Urso (Actigall) may be used to promote bile flow. If cirrhosis and liver failure occur, liver transplant may be an option. Liver transplantation has been done successfully in patients with CF liver disease.
The Riley Hospital for Children Gastroenterology at Indiana University Health division participates in a multi-center research study investigating cystic fibrosis liver disease. Talk to your child’s doctor if you would like to know more about the study or if you have questions about your child participating.
For More Information
Riley Hospital for Chlldren Cystic Fibrosis Center at Indiana University Health