The second most common cause of death in people with cystic fibrosis is liver disease. Between 6-8% of people with CF have potentially fatal liver disease requiring liver transplantation. About 25% of people with CF over the age of four have serious issues with liver function.
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 CF, but influences the course of the disease) increases the probability of a person with CF developing liver disease.
Because of an increase in liver testing, doctors are more frequently diagnosing liver disease associated with cystic fibrosis. With current methods of testing, however, it is difficult to diagnose liver disease. So the true risk of developing CF liver disease is underestimated. 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 CF. A liver biopsy is one of the few effective ways to diagnose the disease and its severity but is very invasive to perform.
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.
For more information, visit http://www.liver.ca