Pulmonary Exacerbations

Pulmonary exacerbations (PEx) are a frequent occurrence in the lives of individuals with cystic fibrosis (CF) and are associated with worsened morbidity, mortality, and quality of life. Approximately 25–35% individuals with CF don’t recover to 90% of baseline lung function after treatment for a PEx. Currently, there is scant evidence upon which to base guidelines for detection and management of PEx; therefore, the CF Foundation convened a working group to design and conduct clinical trials in order to establish evidence for best practices (Standardized Treatment of Pulmonary Exacerbations, STOP). The STOP program has conducted an observational study which has helped define the phenotype of PEx, characterize physician treatment practices, and evaluate clinical endpoints to use in future clinical trials. STOP-2 is an ongoing clinical trial of duration of antibiotics, which should guide establishment of best practices on duration. This chapter reviews the definition, epidemiology, current treatment practices, prognosis, and associated outcomes related to CF PEx. A patient perspective highlights the burden of PEx on the lives of individuals with CF. Current research is reviewed to clarify future directions in PEx. Upcoming clinical trials of PEx should provide robust clinical data in order to provide clear guidelines on detection and treatment of PEx