ABSTRACT
Current therapies for CF address the symptoms of the disease, and their systematic use has lead to steady and remarkable improvements in patient longevity. The rationale for all approved therapies is rooted in reducing disease symptoms, despite differences in how and when they are applied (1). For example, mucus obstruction and airway plugging is addressed by airway clearance methodologies, such as daily chest physiotherapy, nebulization of rhDNase (Dornase alfa, to reduce sputum viscosity), and more recently the addition of nebulized hypertonic saline (to hydrate mucus) as part of daily treatment regimens (2). Bacterial infections are treated with pathogen-directed antibiotics, and the choice of delivery route and antibiotic class is based on the infectious agent and the nature of the infection. Intravenous antibiotics directed toward Pseudomonas aeruginosa during pulmonary exacerbations helps restore lung function. Alternatively, cycled aerosolized tobramycin in patients with chronic P. aeruginosa infection suppresses bacterial growth and stabilizes lung function (2). New symptom-based therapies under development will continue to allow CF healthcare providers and patients to choose from diverse and effective treatment options for many years to come.
