Cystic Fibrosis and Bronchiectasis: Selection, Timing, and Outcome

Abstract

Of the nearly 30,000 Cystic Fibrosis (CF) patients alive in the United States, more than 300 die each year. Of CF deaths at least 75% are from respiratory failure due to bronchiectasis. CF patients make up about 40% of the patients undergoing bilateral transplant. Because of the shortage of donor organs relative to the demand for them, it is important to choose candidates who are in the "transplant window.'' International criteria have been developed to assist in proper selection of potential candidates, but new data suggest that some refinement of current criteria may improve this process. Appropriate prognostic models, however, are not yet completely validated. Bronchiectasis patients are usually evaluated according to the same protocols as CF patients because no large studies exist that would allow us to derive separate prognostic indicators. Outcomes for CF patients are comparable to those for other diagnoses, except in the case of living donor recipients, which are slightly lower. CF patients may face particular issues following transplant-like infections and some immunosuppression complications possibly related to their underlying disease process. In addition, CF patients seem to be at particular risk of posttransplant lymphoproliferative disorder.