CF Airway Clearance

Airway clearance is an important mainstay of CF therapy. Ideally it includes two arms: mucolytic agents and airway clearance techniques (ACTs). Aerosolized recombinant human DNase (RhDNase, dornase alfa) is the most widely used mucolytic agent. It decreases sputum viscosity and should be considered in all CF patients ages 6 and older to help reduce pulmonary exacerbations (PEs) and improve lung function. Hypertonic saline, which serves to promote clearance by hydrating the aqueous surface layer (ASL), can also be used as monotherapy or as an adjunct to dornase alfa. Although the evidence to support the use of hypertonic saline is less robust than that available for dornase alfa, it is an affordable and widely available therapy that appears to have positive effects on respiratory symptoms and lung function. There are a variety of ACTs available, none of which has been found to be clinically superior to another. Because of this, treatments are often individualized based on patient age, preference, and ease of use. ACTs serve either to increase laminar flow or to create shearing forces which help mechanically clear airway secretions and improve respiratory function.