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Cystic Fibrosis Pulmonary Guidelines Chronic Medications for Maintenance of Lung Health

Peter J. Mogayzel, Jr., Edward T. Naureckas, Karen A. Robinson, Gary Mueller, Denis Hadjiliadis, Jeffrey B. Hoag, Lisa Lubsch, Leslie Hazle, Kathy Sabadosa, Bruce Marshall

American Journal of Respiratory and Critical Care Medicine
Volume 187, Issue 7, April 2013, p680-698

 

Rationale :

Cystic fibrosis (CF) is an autosomal recessive disease char- acterized by abnormal airways secretions, chronic endobronchial infection,andprogressiveairwayobstruction.Theuseofmedicationsto slowtheprogressionoflungdiseasehasledtosignificantimprovement in survival. An evidence review of chronic medications for CF lung disease was performed in 2007 to provide guidance to clinicians in evaluating and selecting appropriate treatment for individuals with this disease. We have undertaken a new review of the literature to update the recommendations, including consideration of new medications and additional evidence on previously reviewed therapies. A multidisciplinary committee of experts in CF pulmonary care was established to review the evidence for use of chronic medications for CF lung disease and make treatment recommendations. Published evidence for chronic lung therapies was systematically reviewed and resulting treatment recommendations were graded based on the United States Preventive Services Task Force scheme. These guidelines provide up-to-date evidence of safety and efficacy of chronic treatments of CF lung disease, including the use of novel therapies that have not previously been included in CF pulmonary guidelines.

Keywords: antibiotics; antiinflammatory agents; bronchodilators; CFTR modulators; hypertonic saline

To aid care providers in the use of chronic medications, the Cystic Fibrosis (CF) Foundation established the Pulmonary Clinical Practice Guidelines Committee, which published guidelines on chronic medications for the maintenance of lung heath in 2007. Since this publication, two novel medications have been approved for use in the United States and additional data have been published on therapies previously reviewed. To consider this new evidence, as well as additional and revised questions on the use of therapies, the committee conducted an assessment of the current evidence to develop the updated recommendations presented here.

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