Around 40% of people with cystic fibrosis (CF) will have some liver abnormalities, although only around 5–10% of these will experience problems with their health as a result. The majority of patients with cystic fibrosis-related liver disease will experience no or mild symptoms and signs related to their liver disease. As children are screened from a young age, a diagnosis of CF-related liver disease can be based on some changes on the liver ultrasound scan or mildly abnormal liver blood test results in the absence of any signs or symptoms.
The outlook for most people with cystic fibrosis who experience liver problems is generally very good. Most will show little or no deterioration over time, and often a clinic review once a year with a scan and blood tests is all that’s required. Complications can be managed with careful monitoring and treatment, although a small minority do deteriorate and require transplantation. Anyone with CF-related liver disease should avoid any further damage from alcohol, obesity and/or viral hepatitis; additionally it would be advisable to discuss hepatitis A and B vaccination with their clinicians.