Cystic fibrosis (CF) is a multisystem disease caused by mutations in a single gene (cystic fibrosis transmembrane conductance regulator) and is the most frequent potentially fatal inherited condition in whites, occurring in 1 in 2,000 births. Progressive pulmonary disease is the major contributor to morbidity and mortality, and CF is the most common indication for lung transplantation in adolescents. Liver disease occurs in many patients with CF, but progression to cirrhosis is uncommon, occurring in 3 to 7% of all patients and 10% of patients over 16 years of age. Of these, still fewer progress to end-stage liver disease that necessitates consideration for liver transplantation (LT). In the first 32 months after the introduction of the Model for End-Stage Liver Disease (MELD) and Pediatric End-Stage Liver Disease models, 38 patients with the diagnosis of CF underwent LT (approximately 14 patients per year). The mean age at transplantation was 15.7 years. Of the 38 transplants, 31 were liver transplant only, 4 were liver-lung transplant, 2 were liver-pancreas transplant, and 1 was liver-kidney transplant.
Outcome data from several small series in the literature suggest that the 1-year survival rate (approximately 90%) in patients with CF receiving LT alone is similar to that for other diagnoses. 1,2 Fridell and colleagues 3 achieved a 5-year survival rate of 75%. Late mortality is generally related to progression of pulmonary disease. However, most studies have demonstrated significant improvement from pretransplantation status in pulmonary function in patients undergoing LT alone. 4 CF patients with advanced liver disease and significantly compromised lung function (forced expiratory volume in 1 second ฯฝ40%) may be candidates for combined liver-lung transplantation. 5 Anecdotally, it appears that as hepatic insufficiency