Special considerations for the treatment of pulmonary exacerbations in children with cystic fibrosis

ABSTRACT

Introduction: Cystic fibrosis (CF) is a disease characterized by recurrent flares of respiratory symptoms, known as pulmonary exacerbations (PExs), which have a cumulative, detrimental effect on lung function decline and overall mortality. Although much research has been done on the effects of PExs in adults with CF, considerably less is known about these events in young children with CF.

Areas covered: This review describes the typical presentation of PExs in children and their impact on long-term clinical outcomes. Traditional and new monitoring techniques, such as pulmonary function testing using multiple breath washout, radiographic modalities and microbiological screening methods are reviewed. Finally, the choice, administration and duration of antimicrobial treatment as well as the potential use of antiviral therapy is discussed.

Expert commentary: Although it is now well recognized that a significant proportion of patients do not recover their lung function following PExs, to date, little progress has been made to improve outcomes in this group. Additional therapies, to complement antimicrobials, may be required to treat infection and inflammation during PExs. Trials of anti-inflammatories such as corticosteroids or other novel drugs need to be done in the setting of PExs with the goal of complete lung function recovery for all individuals with CF.

KEYWORDS:

• Cystic fibrosis
• pulmonary exacerbations
• antibiotics

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.

Additional information

Funding

This paper was not funded.