Longer Survival of Patients with Cystic Fibrosis

During the past 2 decades, survival among patients with late-stage cystic fibrosis (CF) has lengthened substantially. In this retrospective cohort study, U.K. investigators evaluated survival of 276 patients (53% male; mean age, 26) with CF whose lung function had deteriorated to forced expiratory volume in 1 second (FEV₁) <30% of predicted.

Patients were divided into seven 2-year cohorts (1990–1991, 1992–1993, etc.) from 1990 to 2003 according to the time when their FEV₁ was first recorded as <30% of predicted. Median survival increased from 1.2 years in the 1990–1991 group to 5.3 years in the 2002–2003 group, with a marked difference in survival after 1994 — the year that nebulized recombinant human (NRH) DNase was licensed for use in the …