OBJECTIVE. Previous analyses of the Epidemiologic Study of Cystic Fibrosis database revealed that sites with the highest average patient lung function monitor patients and treat with antibiotics more aggressively than those where average lung function is lowest. The aim of this study was to assess whether patterns of care for infants at cystic fibrosis sites with superior average lung function in 6- to 12-year-old children showed any differences from those at the lowest outcome sites. METHODS. We divided cystic fibrosis sites with ≥20 patients who were 6 to 12 years of age into quartiles on the basis of median forced expiratory volume in 1 second of that age group in 2003 and compared demographic and clinical characteristics and treatment patterns during the first year of enrollment for patients who were aged 0 to 3 years at those sites in 1994 to 1999. The analysis included 755 infants from 12 upper quartile sites and 743 infants from 12 lower quartile sites. RESULTS. Upper quartile sites had more infants whose disease was diagnosed by family history or newborn screening, fewer infants with symptoms at diagnosis, higher weight for age at enrollment, more white patients, and more ΔF508 homozygotes. Medical conditions and respiratory tract microbiology differed between sites. Infants at upper quartile sites had more office and sick visits; more respiratory tract cultures; and more frequent use of intravenous antibiotics, oral corticosteroids, mast cell stabilizers, and mucolytics; but they received less chest physiotherapy, inhaled bronchodilators, oral nutritional supplements, and pancreatic enzymes. CONCLUSIONS. Both enrollment characteristics and infant care patterns are associated with lung function outcomes in later childhood. Our analysis suggests that pulmonary function of older children may be improved through specific interventions during the first 3 years of life.
- Cystic fibrosis
- Early practice patterns
- Lung function outcomes
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health