Pediatric Pulmonology 46:393–400 (2011) Pulmonary Exacerbations Are Associated With Subsequent FEV1 Decline in Both Adults and

Summary. Background: Patients with cystic fibrosis (CF) frequently experience pulmonary exacerbations that may lead to a faster subsequent decline in pulmonary function; however, this relationship has not been clearly established. The purpose of this study was to determine the association between the frequency of pulmonary exacerbations and subsequent forced expiratory volume in 1 sec (FEV1) decline in adults and children with CF. Methods: Cohort study of subjects followed in the Cystic Fibrosis Foundation Patient Registry from 2003 through 2006. Mixed effects modeling was used to estimate differences in rates of decline in FEV1 in 2004–2006 for patients with 0, 1, 2, or 3þ pulmonary exacerbation(s) in 2003. Results: Of 8,490 subjects wh