Abstract
BACKGROUND: Children with neuromuscular diseases (NMD) are at risk of morbidity and mortality because of progressive respiratory muscle weakness and ineffective cough. Inspiratory muscle training (IMT) aims to preserve or improve respiratory muscle strength, thereby reducing morbidity and improving health-related quality of life (HRQoL). OBJECTIVES: To describe the safety and feasibility of a 6-week IMT programme using an electronic threshold device (Powerbreathe®). Any adverse events and changes in functional ability, spirometry, peak expiratory cough flow (PECF), inspiratory muscle strength and HRQoL (Pediatric Quality of Life [PedsQL]) were recorded. METHODS: A convenience sample of eight participants (n = 4 boys; median [interquartile range {IQR}] age: 12.21 [9.63-16.05] years) with various NMD were included in a pre-experimental, observational pre-test post-test feasibility study. Training consisted of 30 breaths, twice daily, 5 days a week, for 6 weeks. RESULTS: There were significant pre- to post-intervention improvements in upper limb function and coordination (p = 0.03) and inspiratory muscle strength: maximum inspiratory mouth pressure (Pimax) (p = 0.01); strength-index (p = 0.02); peak inspiratory flow (PIF) (p = 0.02), with no evidence of change in spirometry, PECF or HRQoL. No adverse events occurred and participant satisfaction and adherence levels were high. CONCLUSION: Inspiratory muscle training (at an intensity of 30% Pimax) appears safe, feasible and acceptable, in a small sample of children and adolescents with NMD and was associated with improved inspiratory muscle strength, PIF and upper limb function and coordination. CLINICAL IMPLICATIONS: Larger, longer-term randomised controlled trials are warranted to confirm the safety and efficacy of IMT as an adjunct respiratory management strategy in children with NMD.