Airway Clearance Therapy in Cystic Fibrosis Patients Insights from a Clinician Providing Cystic Fibrosis Care.

Abstract

Cystic fibrosis (CF) is a genetic disease characterized by an accumulation of thick layers of mucus, leading to airway obstruction and air trapping. Poorly cleared mucus leads to frequent respiratory infections that produce chronic cough and dyspnea. The presence of infected mucus induces progressive inflammation. The resulting damage anatomically distorts airways leading to development of bronchiectasis. Bronchiectasis is irreversible and results in progressive respiratory function decline over time. Impaired mucociliary clearance together with tenacious mucus makes expectoration with cough alone problematic. Clinicians providing effective care for CF patients must have knowledge of the wide variety of treatment options currently available. Knowledge of these techniques will enable clinicians to prescribe airway clearance therapy (ACT) where necessary and provide treating physicians the ability to adapt to changing patient treatments as necessary. Training programs frequently do not provide in-depth knowledge of ACT technologies in CF patients resulting in knowledge gaps once physicians are in practice. This paper reviews strategies for ACT. It is specifically targeted for clinicians who frequently provide care for patients with CF.