Prediction of mortality and timing of referral for lung transplantation in cystic fibrosis patients

Lung transplantation (Tx) is an optional treatment for cystic fibrosis (CF) patients with end-stage lung disease. The decision to place a patient on the Tx waiting list is frequently complex, difficult, and controversial. This study evaluated the current criteria for lung Tx and assessed additional parameters that may identify CF patients at high risk of death. Data were extracted from the medical records of 392 CF patients. Forty of these patients had a forced expiratory volume in 1 s (FEV ₁) less than 30% predicted, and nine of these 40 patients were transplanted. A comparison was performed between the survival of those transplanted (n=9) and those not transplanted (n=31), by means of Kaplan-Meier survival curves. The influence on survival of age, gender, nutritional status, sputum aspergillus, diabetes mellitus, recurrent hemoptysis, oxygen use, and the decline rate of FEV ₁, were investigated by means of univariate and multivariate analyses. The rate of decline of FEV ₁ was evaluated employing the linear regression model. CF patients with a FEV ₁<30% and who did not receive a lung transplant had survived longer than CF patients who did receive a lung transplant (median survival 7.33 vs. 3.49 yr, 5-yr survival 73% vs. 29%). Two factors - rate of decline in FEV ₁ values and age <15 yr - were found to influence the mortality rate, while the other parameters examined did not. Our results indicate that the current criterion of FEV ₁<30% predicted, alone is not sufficiently sensitive to predict the mortality rate in CF patients and time of referral for Tx, as many of these patients survive for long periods of time. Additional criteria to FEV ₁<30%, should include rapidly declining FEV ₁ values and age <15 yr.