Developments in multiple breath washout testing in children with cystic fibrosis

Abstract

Background: Lung clearance index (LCI) is becoming recognized as an important addition in the monitoring of pediatric cystic fibrosis (CF). The non-invasive technique is easy to perform in all ages, reproducible and increasingly being used in clinical trials. There is interest in utilizing it within the clinic setting but its current use is mostly as a research tool. The procedure is highly dependent on skilled operators and a relaxed testing environment is key to obtaining good quality measurements.

Conclusions: Standardization of LCI is part of an ongoing collaborative, multicenter process. This review describes the background to LCI, discusses technical issues and limitations and provides examples of its utility in clinical and research contexts.

Keywords:

• Child
• cystic fibrosis
• lung clearance index
• multiple breath washout
• spirometry

Transparency

Declaration of funding

This study was not funded.

Declaration of financial/other relationships

C.Sa., K.B., S.I., C.Sh., A.B., and J.C.D. have disclosed that they have no significant relationships with or financial interests in any commercial companies related to this study or article.

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

Acknowledgements

AB is supported by the National Institute for Health Research Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London. JCD, CS & CS provide Core services on behalf of the ECFS Clinical Trials Network including training/ certification of site teams and over-reading of traces using SOPs developed for the Exhalyser D (Ecomedics). ECFS and a number of commercial sponsors conducting CF clinical trials have contributed funding for this work. JCD serves as a clinical advisor for several companies developing new therapies for CF, any associated fees are not for personal benefit.

Note

Notes

1 ORKAMBI is manufactured for Vertex Pharmaceuticals Incorporated