Characterization of Inpatient Cystic Fibrosis Pulmonary Exacerbations

Abstract

Background/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. We aimed 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 Retrospective cross-sectional study of CF patients’ ≤18 years hospitalized for pulmonary exacerbations between July 1st, 2010 and June 30th, 2015 at hospitals within the U.S. Pediatric Health Information System (PHIS) database that are also Cystic Fibrosis Foundation (CFF)-accredited care centers. One exacerbation per patient was randomly selected over the five-year study period. Results From 38 hospitals, 4,827 individual pulmonary exacerbations were examined. Median length of stay was 10.0 days (IQR 6–14.0 days). Significant variation was seen among centers in use of hypertonic saline (11–100%), azithromycin (5–83%), and systemic corticosteroids (3–61%), and in lung function testing frequency. Four different admission antibiotic regimens were used over 10% of the time, and the most commonly used admission antibiotic regimen was two IV antibiotics without additional oral or inhaled antibiotics (30%). Conclusions Significant variation exists in the treatment and monitoring of pulmonary exacerbations across PHIS-participating, CFF-accredited care centers. Results from this study can inform future research working towards standardized inpatient pulmonary exacerbation management to improve CF-care for children and adolescents.

Cystic fibrosis year in review 2017

“…Patients who had >6 courses of oral antibiotics during the study period had the steepest rate of decline in FEV1. In another study, significant variability in treatment of exacerbation was seen when assessed via the Pediatric Health Information System, which includes 38 US pediatric hospitals with CFF‐accredited centers, accounting for 4827 exacerbations over the years 2010‐2015 . Length of stay ranged from 5 to 13 days, and frequency of spirometry varied from less than every 3 days to more than every 7 days.…”

Reevaluating approaches to cystic fibrosis pulmonary exacerbations

“…Risk factors for failure to return to baseline for PEx treated with IV antibiotics include familiar demographic and disease characteristics (poor nutritional status, infection with P. aeruginosa , Burkholderia cepacia, or methicillin‐resistant S. aureus , female gender, Medicaid insurance status) but certain modifiable aspects of treatment are notable: length of prodromal symptoms prior to treatment, depth of drop in ppFEV1 from baseline to treatment initiation, and outpatient versus inpatient treatment of PEx . Nonetheless, great variation exists across CF care programs regarding triggers of treatment, site of treatment (inpatient vs outpatient), as well as duration of treatment, choice of antibiotics, and use of adjunctive therapies (Figure ) …”

Serum and Sputum Iron Trends Distinguigh Responders and Non-Responders to Treatment of Cystic Fibrosis Pulmonary Exacerbation

“…Studies have also determined that the magnitude of FEV1% recovery is higher when PEXs are treated in the hospital rather than in the outpatient setting (14,15) and that 30-day risk of retreatment with IV antibiotics is higher if patients are never treated in the hospital (16). Because clinical practices vary among, and even within, CF care centers (13,17,18), and PEX treatment outcomes remain heterogeneous, there is an unmet need to characterize patient phenotypes and correlative biochemical indices that clinicians might use to improve the e ciency and accuracy with which they diagnose and treat PEXs and to personalize therapies (19,20). Trends in these indices could complement serial symptom scores in individuals with poor baseline lung function who are less likely to have signi cant treatment-related increases in FEV1% (13), thus providing additional objective evidence of recovery that might be leveraged to reduce overtreatment.…”