Outpatient management of pulmonary exacerbations in children with cystic fibrosis.

Introduction: Pulmonary exacerbations are common events in children with cystic fibrosis (CF) and are usually treated with oral antibiotics on an outpatient basis. Even these mild clinical events are clinically meaningful and contribute to the progression of lung disease.

Areas covered: This review discusses the challenges in diagnosing pulmonary exacerbations in children in the absence of a standardized definition. It describes an approach to the management of these events and emphasizes knowledge gaps and areas of future research directions. Information to write this narrative review was collected from 1) a PubMed search [keywords: exacerbation, children, cystic fibrosis] that was not limited by date 2) a hand search of references of retrieved literature 3) personal expertise of the literature and the management of cystic fibrosis.

Expert opinion: Pulmonary exacerbations require prompt diagnosis and management to preserve lung function. More work is needed to understand the impact of CFTR modulators on the frequency and severity of these events and how they influence approaches to management. In a new era of CF care, there is a need to incorporate sensitive outcome measures into clinical care to inform treatment decisions and track treatment response.