Characterization of Inpatient Cystic Fibrosis Pulmonary Exacerbations

Abstract

BACKGROUND AND OBJECTIVES: Pulmonary exacerbations lead to significant morbidity and mortality in patients with cystic fibrosis (CF). National consensus guidelines exist, but few studies report current practice in the treatment and monitoring of pulmonary exacerbations. The goal of this study was to characterize consistency and variability in the inpatient management of CF-related pulmonary exacerbations. We focused on the use of guideline-recommended maintenance therapies, antibiotic selection and treatment regimens, use of systemic corticosteroids, and frequency of lung function testing. We hypothesized that significant variability in these treatment practices exists nationally. METHODS: This trial was a retrospective cross-sectional study. It included patients with CF aged ≤18 years hospitalized for pulmonary exacerbations between July 1, 2010, and June 30, 2015, at hospitals within the US Pediatric Health Information System database that are also Cystic Fibrosis Foundation–accredited care centers. One exacerbation per patient was randomly selected over the 5-year study period. RESULTS: From 38 hospitals, 4827 individual pulmonary exacerbations were examined. Median length of stay was 10.0 days (interquartile range, 6–14.0 days). Significant variation was seen among centers in the use of hypertonic saline (11%–100%), azithromycin (5%–83%), and systemic corticosteroids (3%–61%) and in the frequency of lung function testing. Four different admission antibiotic regimens were used >10% of the time, and the most commonly used admission antibiotic regimen comprised 2 intravenous antibiotics with no additional oral or inhaled antibiotics (29%). CONCLUSIONS: Significant variation exists in the treatment and monitoring of pulmonary exacerbations across Pediatric Health Information System–participating, Cystic Fibrosis Foundation–accredited care centers. Results from this study can inform future research working toward standardized inpatient pulmonary exacerbation management to improve CF care for children and adolescents.