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The second most common cause of death in people with cystic fibrosis (CF) is liver disease. Between six to eight percent of people with cystic fibrosis have potentially fatal liver disease requiring liver transplantation. About 25 percent of people with cystic fibrosis over the age of four have serious issues with liver function.
What causes liver disease?
Liver disease in people with cystic fibrosis is likely caused by abnormally thick fluids in the liver. Cystic fibrosis results in decreased fluid flow, thereby increasing the concentration of bile components and contributing to the abnormal fluid thickness in the liver. Recent research has shown that a particular ‘modifier gene’ (a gene that is not responsible for cystic fibrosis, but influences the course of the disease) increases the probability of a person with cystic fibrosis developing liver disease.
How is liver disease screened and diagnosed?
Liver disease is screened through blood tests measuring the level of specific liver enzymes. Current research shows that signs of liver disease usually develop at or before puberty. Jaundice (yellowing of the skin) – a hallmark symptom of liver disease – is not normally evident in people with cystic fibrosis. A liver biopsy is one of the few effective ways to diagnose the disease and its severity, but is very invasive to perform.
How is liver disease treated?
Medical management, including certain drugs and nutritional support, may slow the progression of liver disease. Liver transplantation can also play a role in treating severe liver disease. Patients who undergo the procedure typically make excellent recoveries and notice a significant improvement in respiratory function.