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Cystic Fibrosis Pulmonary Guidelines

Treatment of Pulmonary Exacerbations

¹ Departments of Medicine and Pediatrics, Medical University of South Carolina, Charleston, South Carolina; ² Department of Pediatrics and ³ Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland; ⁴ Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, Washington; ⁵ Department of Internal Medicine, University of Texas Southwestern Medical School, Dallas, Texas; ⁶ Division of Pharmacy Practice and Science, University of Kentucky, Lexington, Kentucky; ⁷ Cystic Fibrosis Foundation, Bethesda, Maryland

Correspondence and requests for reprints should be addressed to Patrick A. Flume, M.D., Medical University of South Carolina, 96 Jonathan Lucas Street, 812-CSB, Charleston, SC 29425. E-mail: flumepa{at}musc.edu

ABSTRACT

The natural history of cystic fibrosis lung disease is one of chronic progression with intermittent episodes of acute worsening of symptoms frequently called acute pulmonary exacerbations These exacerbations typically warrant medical intervention. It is important that appropriate therapies are recommended on the basis of available evidence of efficacy and safety. The Cystic Fibrosis Foundation therefore established a committee to define the key questions related to pulmonary exacerbations, review the clinical evidence using an evidence-based methodology, and provide recommendations to clinicians. It is hoped that these guidelines will be helpful to clinicians in the treatment of individuals with cystic fibrosis.

Key Words: aminoglycosides • IV antibiotics • drug synergism • Pseudomonas • respiratory therapy