Association between practice patterns and body mass index percentile in infants and young children with cystic fibrosis.

Background: Few guidelines focus on the preventive and maintenance care of infants with cystic fibrosis (CF). We explored how practice variability at accredited CF centers affected nutritional outcomes.

Methods: A retrospective cohort study using Cystic Fibrosis Foundation registry data (1993-2004) from three CF centers compared the initial management with respiratory, antimicrobial, and nutritional agents in infants. Further, we examined the association between dornase alpha use prior to two years of age and BMI percentile over time accounting for several possible factors including gender, race, CF center, presentation, age at diagnosis, sweat value, F508del status, first Pseudomonas aeruginosa infection age, second-year weight percentile, supplemental feedings use, and pancreatic enzymes use.

Results: Patient characteristics and prescribed therapies were similar at all sites for 165 patients who met inclusion criteria. However, one CF center prescribed dornase alpha significantly more frequently, 82% vs. 10% (p<0.001), and supplemental feeds significantly less frequently, 56% vs. 78% (p=0.04). Dornase alpha prescription prior to age two was associated with a 10-percentile increase in BMI through age six compared to infants not prescribed dornase alpha.

Conclusions: Treating infants less than two years old with dornase alpha may improve nutritional outcome through age six.