Optimizing outcomes of pulmonary exacerbations in cystic fibrosis

Pulmonary exacerbations are described as worsening of the daily symptoms of cystic fibrosis airways disease, typically with increased cough and sputum production. There are often associated signs such as weight loss and reduced lung function. These events occur frequently and are associated with considerable cost and morbidity. Although approved maintenance therapies are shown to reduce exacerbations, they still occur and are associated with poor outcomes despite treatment. Guidelines to define best practices found a paucity of evidence upon which to base recommendations.

There are ongoing studies that are trying to build the evidence upon which to improve our practice. Antibiotics remain a core aspect of treatment, but there is high variance in practice patterns including selection of antibiotics and duration of therapy. In addition, there is a discordance between antibiotic susceptibility test results and clinical outcomes, suggesting we need better approaches to guide antibiotic selection.

Treatment durations are highly variable but recent evidence has demonstrated worse outcomes with shorter durations; longer durations may be associated with complications of treatment, suggesting an optimal duration could be identified. New studies aim to define best practices to improve outcomes with treatment of pulmonary exacerbations.