Medical Therapy
When blood tests show that your liver is working harder than it should be, a medication called ursodeoxycholic acid (Actigall^® or URSO^®) may be given to you. This helps decrease clogging of the bile ducts or tubes, allowing more bile to flow from the liver. Ursodeoxycholic acid normally helps your liver to not work quite so hard. If you are diagnosed with liver problems, your fat-soluble vitamin levels need to be watched. Because your body may not absorb these vitamins fully, you may have to take additional vitamins. You and your CF team must also make sure your vaccinations for Hepatitis B are up to date.

Liver Transplantation
Depending upon how bad a patient’s liver disease is, a liver transplant may be the answer. Patients with mild lung disease and severe liver disease are usually the best candidates for liver transplants. In children and young adults, the estimated 1-year survival after transplant is approximately 75-80 percent. Patients who have a successful liver transplant usually are healthier and have a better quality of life. Approximately, 10 CF patients receive liver transplants each year in the U.S.