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Semin Respir Crit Care Med 2019; 40(06): 751-761
DOI: 10.1055/s-0039-1698462
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Treating the Airway Consequences of Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction

Demet Toprak
1   Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Seattle Children's Hospital, Seattle, Washington
,
Chelsea Davis
1   Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Seattle Children's Hospital, Seattle, Washington
,
Margaret Rosenfeld
1   Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Seattle Children's Hospital, Seattle, Washington
› Author Affiliations
Further Information

Publication History

Publication Date:
28 October 2019 (online)

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Abstract

In cystic fibrosis (CF), absent or dysfunctional CF transmembrane conductance regulator (CFTR) on the surface of airway epithelial cells causes abnormal mucociliary clearance, leading to chronic endobronchial infection and inflammation, in turn resulting in life-shortening progressive obstructive lung disease and structural airway damage. Fortunately, CF-specific therapies have been developed that improve lung function and reduce pulmonary exacerbations, contributing significantly to improved survival over the past 4 decades. Therapies not originally developed for CF, such as bronchodilators and corticosteroids, are also widely used by people living with CF. Therapies to be reviewed in this article include mucolytics, airway surface liquid hydrators, anti-inflammatory medications, bronchodilators, inhaled and oral antibiotics, and airway clearance techniques. Determining which therapies to utilize can be challenging, as there is variable evidence for each treatment, differing national guidelines, few head-to-head studies, potential for drug–drug interactions, and synergistic toxicities, as well as issues with burden of care. In this review, we summarize the mechanism of action and available evidence, and compare national guidelines for each major medication used to treat the airway consequences of CFTR dysfunction.

Keywords

cystic fibrosis - therapies - pulmonary
 

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