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Approximately five percent of CF patients will encounter significant liver problems related to their disease. Liver problems result when small ducts in the liver become blocked with thick, sticky mucus characteristic of CF. Bile can no longer drain from the liver, damaging the organ. The resulting liver disease can be very mild, causing only slight variations in function, or very severe, leading to liver failure. CF patients are screened for liver problems with blood tests once a year. Medical Therapy
When blood tests show elevated liver function, a medication called ursodeoxycholic acid (Actigall® or URSO®) may be recommended. This medication helps to decrease clogging of the bile ducts, allowing more bile to flow from the liver. If a patient is diagnosed with liver dysfunction, fat-soluble vitamin levels need to be monitored closely. These vitamins may not be properly absorbed and additional supplements may be required. Patients and their CF team must also ensure that vaccinations for Hepatitis B are up to date. Also, alcohol consumption, and products like acetaminophen, herbal supplements or over-the-counter remedies can worsen liver damage and function. Liver Transplantation
Depending upon the severity of a patient's liver disease, transplantation may be an option. Patients with mild lung disease and severe liver disease are usually the best candidates for liver transplants. In children and young adults, the estimated one-year survival after transplant is approximately 75-80 percent. Patients who have undergone successful liver transplants usually have better nutritional status and improved quality of life. About 10 CF patients receive liver transplants each year in the U.S.
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