Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis

Background In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis ( Responders ) is associated with better growth and less lung disease at age 6 years. This study examined whether these benefits are sustained through 12 years of age. Methods Longitudinal growth from 76 children with CF enrolled in the Wisconsin CF Neonatal Screening Project was examined and categorized into 5 groups: R 12 , R 6 , and R 2 , representing Responders who maintained growth improvement to age 12, 6, and 2 years, respectively, and I 6 and N 6, representing Non-responders whose growth did and did not improve during ages 2–6 years, respectively. Lung disease was evaluated by % predicted forced expiratory volume in one second (FEV 1 ) and chest radiograph (CXR) scores. Results Sixty-two percent were Responders. Within this group, 47% were R 12 , 28% were R 6 , and 25% were R 2 . Among Non-responders, 76% were N 6 . CF children with meconium ileus (MI) had worse lung function and CXR scores compared to other CF children. Among 53 children with pancreatic insufficiency without MI, R 12 had significantly better FEV 1 (97–99% predicted) and CXR scores during ages 6–12 years than N 6 (89–93% predicted). Both R 6 and R 2 experienced a decline in FEV 1 by ages 10–12 years. Conclusions Early growth recovery in CF is critical, as malnutrition during infancy tends to persist and catch-up growth after age 2 years is difficult. The longer adequate growth was maintained after early growth recovery, the better the pulmonary outcomes at age 12 years.