Selection of patients with cystic fibrosis for lung transplantation

Lung transplantation is the most aggressive therapy available for end-stage lung disease from cystic fibrosis (CF). A new predictive survival model of CF uses demographic, FEV₁, nutritional, microbiologic, and acute exacerbation data to produce precise estimates of 5-year survival. The model improves the ability to select patients most likely to have survival benefit from transplantation. We discuss potential application of the survival model to four distinct groups of patients with CF: (1) candidates for cadaveric transplantation, (2) potential living donor recipients, (3) patients infected with multiply-resistant organisms such as Burkholderia cepacia, and (4) patients critically ill and dependent on mechanical ventilation. Measuring the impact of transplantation on quality of life remains a difficult task, and further studies are needed to determine whether lung-transplantation-derived survival benefit implies quality-of-life benefit. However, judicious use of the survival model to select patients for transplantation is likely to improve survival outcomes.