The use of multiple breath washout for assessing cystic fibrosis in infants

ABSTRACT

Introduction: Lung Clearance Index, measured using the multiple breath washout (MBW) technique, may be a useful test in infants with Cystic Fibrosis (CF). However, the requirement for specialised equipment and a number of important technical and methodological considerations relevant to testing in infants have complicated matters, and to date prevented its widespread translatability in this age group.

Areas covered: We review the current status of infant MBW testing in CF, focusing on Lung Clearance Index. This includes a review of recent developments in the field relevant to testing methodology in the infant population, use in evaluating lung disease in CF in infancy, and the associated challenges which remain.

Expert commentary: The challenges of infant MBW are not limited to those associated with testing equipment, but also gaps in our understanding regarding the interpretation of MBW indices in infants. This includes their relationship to underlying physiology and pathology, and tracking over time. Recent advances in understanding and improving the infant MBW test set up (including both hardware and software) relevant to infants will greatly progress the field.

KEYWORDS:

• Cystic fibrosis
• infant lung function
• lung clearance index
• multiple breath washout
• clinical trials

Declaration of interest

P. Aurora is a consultant for Vertex Pharmaceuticals in relation to a future trial in infants with CF using LCI as an endpoint. He has no other 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. The authors have no other 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 apart from those disclosed. This article presents independent research funded by the National Institute for Health Research (NIHR). The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health.

Additional information

Funding

G. Davies is supported by a National Institute for Health Research (NIHR) Academic Clinical Lectureship at University College London, and along with PA is supported by the NIHR Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London.