Improvements in Lung Function Outcomes in Children with Cystic Fibrosis are Associated with Better Nutrition, Fewer Chronic Pseudomonas aeruginosa Infections, and Dornase Alfa Use

Objective

To compare lung function and nutritional outcomes in cystic fibrosis (CF) for 2 birth cohorts in our CF center.

Study design

Patients with CF born between 1985 and 2000 treated in our CF center before age 5 years were included. The patients were divided into 2 equal birth cohorts for comparison: birth cohort 1 (born between 1985 and 1992) and birth cohort 2 (born between 1993 and 2000). To compare lung function, we used forced expiratory volume in the first second (FEV₁)% predicted and FEV₁% predicted slope from age 6 to 12 years. We hypothesized that we would find significant improvements in lung function and nutritional outcomes in our patients with CF.

Results

The patients born between 1993 and 2000 (birth cohort 2) had better lung function, a slower rate of decline in lung function, and better nutritional outcomes compared with those born between 1985 and 1992 (birth cohort 1). Factors associated with a slower rate of decline in lung function in both groups were a higher baseline body mass index (BMI)%, a slower BMI% rate of decline, absence of chronic Pseudomonas aeruginosa respiratory infection, and initiation of dornase alfa (Pulmozyme) therapy before age 9 years.

Conclusion

Our results demonstrate dramatically improved lung function and nutritional outcomes in the children with CF in our center. The improvements in lung function outcomes are associated with better nutrition, fewer chronic P aeruginosa infections, and dornase alfa therapy.

Abbreviations: BMI, Body mass index, BMI%, BMI percentile, CF, Cystic fibrosis, FEV₁, Forced expiratory volume in the first second, Height-for-age%, Height-for-age percentile, Weight-for-age%, Weight-for-age percentile