Bile is naturally produced by the liver and is drained by the bile ducts into the gallbladder and small intestine where it helps with digestion. People with CF have thicker bile which is more likely to plug the bile ducts in the liver and to form stones in the gall bladder than in people without CF. CF liver disease is probably more common than we realize. In some studies, liver changes have been present in over 70% of adult patients with CF but these changes may not have an impact on how the liver functions. Liver damage can be difficult to recognize because many people do not have symptoms until late into the stage of disease. Liver blood tests can be elevated because of slow flow of bile. Some people with CF can develop fatty liver (hepatic steatosis)
Blockage of the bile ducts in the liver causes inflammation and progressive scarring (fibrosis) of the liver and eventually this leads to liver failure or cirrhosis in about 5% of people with CF. Patients with end stage liver disease may require a liver transplantation.
Gallstones form in 10-15% of people with CF and some people will form stones in the ducts draining the liver and pancreas.