Pulmonary exacerbations and acute declines in lung function in patients with cystic fibrosis

Jeffrey S. Wagener, Michael J. Williams, Stefanie J. Millar, Wayne J. Morgan, David J. Pasta, Michael W. Konstan

Research output: Contribution to journalArticlepeer-review

15 Scopus citations


Background: Patients with cystic fibrosis (CF) who experience acute declines in percent predicted FEV 1 (ppFEV 1 decreased ≥10% relative to baseline) are often not treated with antibiotics for pulmonary exacerbations (PEx), whereas other patients are treated even when they have not experienced a decline in lung function. Methods: We analyzed 2 patient cohorts using 3 years of Epidemiologic Study of CF data. Cohort 1 (12,837 patients) experienced a ≥10% acute decline in ppFEV 1 (n = 22,898) and Cohort 2 (10,416 patients) had a clinician-diagnosed PEx (n = 20,731). Results: 70.7% of ≥10% decline events were treated with antibiotics; with intravenous antibiotics used 67.1% of the time. 32.0% of clinician-diagnosed PEx declined <10%; with intravenous antibiotics used 36.9% of the time. Conclusions: A clinician's decision to diagnose a PEx and treat with antibiotics often is not defined by measured lung function: a ≥10% FEV 1 decline is not considered an absolute indication of a PEx and the lack of a decline does not contraindicate a PEx. Clinicians appear to use the history of prior PEx plus other variables as factors for diagnosing PEx.

Original languageEnglish (US)
Pages (from-to)496-502
Number of pages7
JournalJournal of Cystic Fibrosis
Issue number4
StatePublished - Jul 2018


  • Cystic fibrosis
  • Lung function
  • Pulmonary exacerbations

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine


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