Multisystem and autonomic complications of NMD on NIV
Research type
Research Study
Full title
Growing up on non-invasive ventilation: assessment of multisystem and autonomic complications of neuromuscular and neurological disorders in young adults
IRAS ID
266270
Contact name
Anita Simonds
Contact email
Sponsor organisation
Royal Brompton and Harefield NHS Foundation Trust
Duration of Study in the UK
1 years, 6 months, 2 days
Research summary
Neuromuscular and neurological disorders (NMD) starting in childhood are rare conditions which significantly impair muscle function. As well as muscles controlling limb movement, those involved in vital functions such as breathing and heart function are often affected. Due to progress in NMD management, particularly the use of non-invasive ventilation (NIV) to support breathing, many individuals are now surviving into adulthood. However, because these conditions often progress, older individuals are developing potentially fatal complications: these often involve the heart e.g. sudden cardiac death, gastrointestinal (GI) tract and respiratory system.
The autonomic nervous system (ANS) is a network of nerves vital to controlling how the body responds to short-term physiological changes (e.g. sleep, exercise, stress, emotions, eating etc.) It is particularly important in controlling heart rate and blood pressure, which are in turn affected by breathing and ventilation. The ANS is also important in controlling functions of the GI tract. Previous studies largely in children and adolescents suggest individuals with NMD may develop problems of the ANS - it is likely some complications are related to these problems which is what this study aims to better understand.
This study will use non-invasive tests during wake and sleep to investigate ANS function and how it relates to complications in adults with NMD on long-term NIV. Understanding these problems will enable identification of individuals with and at risk of complications. Also, it is important to understand how NMD progresses so when new specific treatments are developed, researchers can better understand their effectiveness. As conditions progress, adults with NMD may have to make decisions about their care: decisions may be difficult, particularly when complex and/or regarding major health decisions such as preferences for resuscitation if health deteriorates. This study will use qualitative methods to evaluate experiences and support needs of individuals in making such decisions.
REC name
Yorkshire & The Humber - South Yorkshire Research Ethics Committee
REC reference
19/YH/0306
Date of REC Opinion
30 Sep 2019
REC opinion
Further Information Favourable Opinion