Cystic fibrosis (CF) is a genetic condition affecting the lungs, liver, intestines, pancreas and reproductive organs. In the lungs, thick mucus has difficulty getting out of the lungs causing chronic lung disease. In the liver, bile is thick and tenacious and also has difficulty getting out of the liver. This may lead to inflammation and scarring of the liver. Advanced liver disease may result in cirrhosis or permanent scarring of the liver. Fortunately, only a small percentage of children with CF develop severe CF-related liver disease or cirrhosis.
Many children with CF liver disease do not show symptoms until late in the course of the disease. Sometimes the liver and spleen will be enlarged on physical exam. With progressive disease, the liver becomes hard and blood cannot flow through the liver well leading to an increase in pressure in the portal vein (the main vein going into the liver). This is called portal hypertension. This can lead to further complications including ascites, or fluid accumulation in the abdomen, or bleeding from enlarged veins in the esophagus or stomach. Sometimes, patients will become jaundiced, which means a yellowing of the skin and eyes.
CF liver disease can be diagnosed with blood tests, findings on physical exam and radiologic tests such as an abdominal ultrasound. Some patients may have a liver biopsy to aid in the diagnosis.
Doctors at Riley at IU Health can perform the following exams and tests to make a diagnosis of CF liver disease:
There are no treatments to prevent or cure CF liver disease. Your child will need to be closely followed by a pediatric gastroenterologist in addition to his or her pulmonary doctor. There are some medicines such as ursodiol which might help improve bile flow from the liver. If your child develops complications of liver disease, such as fluid in the abdomen, there are other medications to help control this. Ensuring optimal nutrition is important in patients with CF liver disease. If the scarring in the liver leads to liver failure, some children may require a liver transplant.
Visit the websites below to find support groups and services and learn more about cystic fibrosis liver disease.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
Riley at IU Health participates in a multicenter 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.
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