Lung transplantation in cystic fibrosis

Lung transplantation has emerged in the last decade as a therapy for selected patients with end-stage lung disease. Although the presence of highly resistant organisms in the airway and sinuses of patients with cystic fibrosis (CF) increases the perioperative risk of infection following lung transplantation, transplant procedures can be undertaken in properly selected CF patients with acceptable morbidity and mortality at experienced centers. A modification in the technique of double-lung transplantation has resulted in improved operative survival of CF patients undergoing bilateral lung transplantation. Although problems with posttransplant infection and obliterative bronchiolitis contribute to morbidity, it is clear that lung transplantation can offer CF patients with end-stage lung disease improvement in both the quality and quantity of life, with perioperative mortality similar to that for other prospective recipients of lung transplantation. The most serious impediment to more widespread application of this therapy in CF is the inadequate number of pulmonary donors.