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Cystic Fibrosis Pulmonary Guidelines Treatment of Pulmonary Exacerbation

Patrick A. Flume, Peter J. Mogayzel, Jr., Karen A. Robinson, Christopher H. Goss, Randall L. Rosenblatt, Robert J. Kuhn, Bruce C. Marshall

American Journal of Respiratory and Critical Care Medicine, Volume 180, Issue 9, November 2009, p802-808

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 exacerbationstypicallywarrantmedicalintervention.Itisimportant that appropriate therapies are recommended on the basis of avail- able 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 recommenda- tions to clinicians. It is hoped that these guidelines will be helpful to clinicians in the treatment of individuals with cystic fibrosis.

Keywords: aminoglycosides; IV antibiotics; drug synergism; Pseudo- monas; respiratory therapy

Cystic fibrosis (CF) is a complex genetic disease affecting many organs, although 85% of the mortality is a result of lung disease (1). CF lung disease begins early in life with inflammation and impaired mucociliary clearance and consequent chronic infec- tion of the airways (2). There is progressive decline of lung function with episodes of acute worsening of respiratory symptoms, often referred to as ‘‘pulmonary exacerbations.’’ Although a generally applicable prospective definition of a pul- monary exacerbation has not been developed, clinical features of an exacerbation may include increased cough, increased sputum production, shortness of breath, chest pain, loss of appetite, loss of weight, and lung function decline (3). Pulmo- nary exacerbations have an adverse impact on patients’ quality of life and a major impact on the overall cost of care (4). Identifying optimal treatment methods for these events could produce significant improvements in quality and length of life for patients with CF. To identify the best treatment practices, the CF Founda- tion’s Pulmonary Therapies Committee, comprising individuals knowledgeable in all the major facets of CF care, conducted a search of published results of controlled trials of common treatment methods for exacerbations. It is not our intent to define a pulmonary exacerbation, nor to discuss relative sever- ity, but to evaluate the evidence supporting therapies and approaches for the management of a health decline determined by a CF specialist to represent an exacerbation of CF lung disease. This systematic review allowed the Committee to make specific treatment recommendations and to determine areas that need additional study. The guidelines presented are designed for general use in most individuals with CF, but should be adapted to meet specific needs as determined by the individual, their family, and their health care provider.

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