Inspiratory muscle training in severe spinal muscular atrophy: a case report

Abstract

Background/Aims Inspiratory muscle training aims to preserve or improve respiratory muscle strength in children with neuromuscular diseases in order to prevent or minimise pulmonary morbidity. The aim of this study was to determine the effect of inspiratory muscle training on clinical outcomes and health-related quality of life in a child with advanced neuromuscular disease and severe pulmonary restriction. Methods A one patient pre-test post-test study design was implemented. General function, spirometry, peak expiratory cough flow and health-related quality of life were measured at baseline and after a 6-week inspiratory muscle training programme. Inspiratory muscle strength (maximal inspiratory mouth pressure and sniff nasal inspiratory pressure) was measured every 2 weeks. The patient used a tapered flow threshold inspiratory training device (POWERbreathe K3) at an intensity of ± 30% of maximal inspiratory mouth pressure twice a day, 5 days per week. Findings The non-ambulatory 10-year-old girl with type 2 spinal muscular atrophy initially had a forced vital capacity of 18% predicted and peak expiratory cough flow of 60 litres/minute. A substantial improvement was seen in inspiratory muscle strength between baseline and 4 weeks. Patient health-related quality of life improved and patient satisfaction was high, with a score of 9/10. The patient developed a lower respiratory tract infection towards the end of the inspiratory muscle training period. No other adverse events occurred. Conclusions Improved inspiratory muscle strength and health-related quality of life was associated with inspiratory muscle training in a child with advanced spinal muscular atrophy. Controlled clinical trials are recommended to determine the safety and efficacy of inspiratory muscle training in children with advanced spinal muscular atrophy and severe respiratory muscle weakness to inform clinical practice.