Cystic fibrosis and liver disease

Quick Summary - Cystic fibrosis-related liver disease

• Cystic fibrosis is a common genetic condition that causes thick, sticky mucus to build up in the body’s tubes and passageways.
• Cystic fibrosis can make bile in the body become thicker. This may lead to blockage of the bile ducts, which can damage the liver.
• Most children and young people with cystic fibrosis will not develop any serious problems with their liver. Around 5-10% will develop liver problems that have an impact on their health.
• We cannot predict who will get cystic fibrosis-related liver disease.
• Tests for cystic fibrosis-related liver disease include physical examination, blood tests, scans and occasionally liver biopsy.
• The symptoms and severity of cystic fibrosis-related liver disease can vary from child to child.
• In a small number of cases, damage to the liver can become worse over time.
• There is no cure for cystic fibrosis-related liver disease but dietary support, medicines and medical procedures can help reduce the symptoms and complications.
• Only a small number of children with cystic fibrosis develop advanced liver disease that will lead to a liver transplant.

Special thanks

Information reviewed: July 2024

Next review date: July 2027

We would like to thank staff at each of the specialist paediatric liver centres: Birmingham Children’s Hospital, King’s College Hospital and Leeds Children’s Hospital. We would also like to thank the families who helped us produce this information.

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