Original ArticleProbability of Treatment Following Acute Decline in Lung Function in Children with Cystic Fibrosis is Related to Baseline Pulmonary Function
Section snippets
Methods
ESCF is a large multicenter, longitudinal, prospective observational study of the clinical course of patients with CF in the US and Canada from 1994-2005.12 Data collected on each patient included clinical demographics, signs and symptoms of lung disease, information on growth and nutrition, results of spirometry, results of respiratory tract cultures, use of routine therapies, and information on pulmonary exacerbations and their treatment. Informed consent was obtained based on decisions by
Results
Of all children and adolescents enrolled in ESCF between 1994 and 2005, 16 524 had sufficient FEV1 data. Of these, 11 276 (68%) patients had 3 stable baseline FEV1 values, were 6-17 years of age at the first of these values, and demonstrated at least 1 sudden decline event. We excluded 388 patients because either (1) during the moving average period, they had no respiratory cultures or no encounters; or (2) they were hospitalized before or during all their sudden decline events. This left
Discussion
This study demonstrates that the likelihood of therapeutic intervention after a substantial and clinically meaningful decline in lung function is inversely related to the level of baseline lung function. Overall, the rate of IV antibiotic therapy or hospitalization for treating patients with CF was surprisingly low when using a threshold of a 10% relative decline in FEV1% predicted. In fact, these patients experienced large absolute declines in lung function in less than a single year. The mean
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2018, Journal of Cystic FibrosisImpact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis
2018, Journal of Cystic FibrosisCitation Excerpt :Surveillance for and treatment of pulmonary exacerbations is a cornerstone of contemporary CF care [9]. However, several observational database analyses have found that this is done inconsistently, especially among patients with higher baseline FEV1, and that the consequence of missed diagnosis and non-treatment is a subsequent loss of lung function [10, 11]. Moreover, several studies have found that CF centers that have greatest success in preserving patients' lung function are more consistent and possibly more aggressive in their treatment of pulmonary exacerbations [6, 7].
Pulmonary exacerbations and acute declines in lung function in patients with cystic fibrosis
2018, Journal of Cystic Fibrosis
Sponsored by Genentech, Inc (San Francisco, CA). W.M., J.W., and M.K. have received honoraria from Genentech for serving as members of the Scientific Advisory Group for the Epidemiologic Study of Cystic Fibrosis and have served as consultants to Genentech; no compensation was provided to these authors in exchange for creation of this manuscript. D.P. and S.M. are employees of ICON Late Phase and Outcomes Research, which was paid by Genentech, for providing analytical services for this study. A.Y. and J. W. were employees of Genentech.
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A list of the Scientific Advisory Group members, investigators, and coordinators of the Epidemiologic Study of Cystic Fibrosis is available at www.jpeds.com (Appendix).