Outpatient management of pulmonary exacerbations in children with cystic fibrosis

ABSTRACT

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.

KEYWORDS:

• Children
• antibiotics
• outpatient
• diagnosis
• management
• cystic fibrosis
• pulmonary exacerbations
• acute respiratory events

Article highlights

• Most pulmonary exacerbations in children present with mild symptoms or increased cough either on its own or with an increase in chest congestion or sputum

• Even mild events treated with oral antibiotics are important and can be associated with lung function decline

• Given the lack of an established definition or evidence-based management guidelines, treatment strategies for pulmonary exacerbations are highly variable across CF centers.

• There is a need for more sensitive tests that can identify pulmonary exacerbations associated with lung function worsening that warrant intervention

• The lung clearance index (LCI) is more sensitive than FEV₁ in detecting lung function abnormalities and could have a role in the management of pulmonary exacerbations in children with mild disease

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.