Lung transplantation in cystic fibrosis

Lung transplantation has emerged in the last decade as a valid therapeutic endeavor for 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. The introduction of a modification in the technique of double-lung transplantation has resulted in improved operative survival of CF patients undergoing lung transplantation. Whereas problems with posttransplant obliterative bronchiolitis, infection, and lymphoproliferative disorders 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. The most serious impediment to more widespread application of this therapy in CF is the inadequate number of donor organs.