Improving lung function testing methods in children with neuromuscular disorders

Each year, ELF gives travel grants to the two best abstracts in patient-centred research submitted to the ERS International Congress. The aim is to highlight impactful examples of patient involvement in medical research and to encourage greater awareness of the importance of doing research with patient-centred outcomes at the heart of the project.

Grace Pettigrew was one of the winners of this award. Grace is a researcher at the Telethon Kids Institute in Perth, Australia.

Below is a summary of her research.

What did the study look at?

The study looked at respiratory health in children with neuromuscular disorders (NMD). Children with NMD have muscle weakness which leads to difficulty breathing. Parents and families of children with NMD identified an important need to improve how lung function is measured, as current lung testing practices, such as spirometry, are not well suited for children with NMD.

The study explored the possibility of measuring the Lung Clearance Index (LCI) of children with NMD using a test called multiple breath washout (MBW). MBW tests measure how efficiently gas mixes in the lungs.

Patient-centred aspects:

The Neuromuscular Consumer Reference Group was formed in partnership with Muscular Dystrophy Western Australia (MDWA), which established the study methods and platforms. This helped to make sure the research was designed in a way that minimised any discomfort for the children taking part. The researchers engaged with and consulted the neuromuscular disorder community throughout the study.

What did the results show?

The results showed that it is possible to use LCI testing in children as young as 6 years old to monitor their lung health.

Why is it important?

Monitoring lung function is vital for children with NMD. However, there are no reliable outcome measures to predict changes in lung health. Current tests can be difficult for children to do. Improved methods of monitoring lung function could help patients to have a better quality of life. The LCI could provide a less burdensome and tiresome monitoring method.

Grace produced a poster and an introductory video about the research.

View the poster and video.

Find out more about submitting abstracts to the ERS Congress.

Find out more about ELF abstract grants.