Can imaging of airway dynamics optimise assisted coughing in children with spinal muscular atrophy?

Research Question Can imaging of airway dynamics optimise assisted coughing in children with spinal muscular atrophy? Background Spinal Muscular Atrophy(SMA) is a life-limiting condition affecting children’s ability to move limbs, swallow, breathe and cough effectively, impacting quality of life and life expectancy. Patients develop increased secretions, causing recurrent chest infections and hospital admissions. Respiratory physiotherapy relies on Mechanical Insufflation Exsufflation (MI-E) devices to help prevent infections. MI-E delivers positive pressure promoting deep inhalation followed by a rapid switch to negative pressure to mimic a cough. MI-E are programmed on specific settings for children to use at home. However, there is limited understanding of what occurs in the child’s airway when breathing, coughing, and using MI-E. Concerns include potential airway collapse and MI-E effectiveness. Understanding upper airway response to using MI-E is vital to improve patient outcomes Aims Investigate reasons for variability in effectiveness of MI-E in children, establishing optimal settings for MI-E in patients with SMA Assess variations in lung function with optimised MI-E settings Evaluate tolerance and acceptance of new assessment techniques Objectives Work Package(WP)1a: Assess the upper airway dynamics using Transnasal Fibreoptic Laryngoscope(TFL) in children with SMA WP1b: Evaluate/optimise upper airway response patterns to MI-E WP2: Assess changes in respiratory patterns/lung volumes utilising opto-electronic plethysmography(OEP) with baseline and optimised MI-E settings WP3: Measure patients'/carers' tolerance and acceptability of assessment techniques Method An interventional quantitative study consisting of three WPs 45 patients will be recruited. In WP1 Patients will be assessed with TFL during normal breathing and use of home MI-E settings. Airway status will then be analysed with MI-E being adjusted for optimal airway status. In WP2, OEP will measure differences in breathing patterns and lung volumes, before MI-E, at baseline MI-E settings and on adjusted MI-E settings. During WP1 and WP2, patients/carers will rate tests in terms of tolerance and acceptance on a visual scale (WP3). Statistical analysis will be conducted. Data synthesis will confirm challenges, strengths, and areas for improvement. Timelines for delivery WP1, 2 and 3: 9-18Months Knowledge mobilisation, dissemination, and impact Improved secretion management will reduce hospitalisations improving health and wellbeing. The study provides insight into airway dynamics and will translate into other paediatric conditions. Results will be shared via podcasts and meetings with parent support groups/charities, and presentations at meetings/conferences, throughout the UK and internationally, including submission to appropriate peer reviewed journals. Research Inclusion The study focuses on paediatric patients with SMA, which as a rare disease serves an under-represented population from all cultural backgrounds. It is supported by community steering groups. Adjustments have been made to ensure patients with behavioural and communicative concerns are able to participate. Patient information sheets will be available to all and adapted for different communication styles. An animated video has been created to aid inclusion. Characteristics related to inclusion of participants will be reported. Ongoing reviews will ensure equality, diversity and inclusion are considered.