Lung Transplantation for Cystic Fibrosis

Heart–lung and lung transplantation have now been successfully applied to patients with end-stage cystic fibrosis (CF) for more than a decade. Initial concerns about overwhelming posttransplant infection in this population with chronic suppurative sinopulmonary disease proved to be exaggerated and fears that the primary defect would recur in the allografts have faded with insights into the genetic basis for the disease. Successful transplantation offers the CF patient the prospect of a more normal and active lifestyle, unencumbered by dyspnea and free of the need for laborious airway clearance measures. Nonetheless, lung transplantation remains an imperfect technique. Survival beyond 5 years is realized by only half of the recipients and the risk of infection and rejection are ever present. In the desperate situation of advanced, life-threatening lung disease, patients and their families are frequently blinded to the significant limitations of transplantation, focusing only on the immediate need for the patient to survive. It is essential that the physician counseling the patient provide a realistic impression of both the potential benefits and the significant shortcomings of this procedure so that an informed decision can be made and unrealistic expectations avoided. This article discusses issues of importance in maximizing successful application of lung transplantation to the CF population, including the appropriate timing for referral of patients, selection of suitable candidates, and expansion of the current donor pool through the controversial use of living donors. The documented benefits of transplantation and the common complications limiting the success of the procedure are then reviewed. Finally, the article looks toward the future in a discussion of active research efforts that promise to transform lung transplantation into a more widely applicable and successful procedure.