Inspiratory muscle training in children and adolescents with
neuromuscular disorders: a cross-over randomized controlled trial
Abstract
Progressive respiratory muscle weakness and ineffective cough
contributes to morbidity and mortality in children with neuromuscular
disorders (NMD). Inspiratory muscle training (IMT) aims to preserve or
improve respiratory muscle strength and reduce respiratory morbidity.
This study intended to determine the safety and efficacy of IMT in
children with NMD. A randomized cross-over study compared three-month
intervention (IMT) with control periods (no IMT). Children diagnosed
with NMD (5-18 years) performed 30 breaths (at 30% of inspiratory
muscle strength (Pimax)) with an electronic threshold device, twice
daily. During the control period participants did not perform any IMT.
Twenty-three children (median (IQR) age of 12.33 (10.03-14.17) years),
mostly male (n=20) and non-ambulant (n=14) participated. No adverse
events related to IMT were reported. No difference in median patient
hospitalization and respiratory tract infection rates between control
and intervention periods (p=0.60; p=0.21) was found. During IMT, Pimax
and peak cough flow improved with a mean (SD) of 14.57 (±15.67) cmH
2O and 32.27 (±36.60) L/min, compared to 3.04(±11.93)cmH
2O (p=0.01) and -16.59 (±48.29) L/min (p = 0.0005)
during the control period. Spirometry, functional ability and total
health-related quality of life scores following intervention did not
show a significant change. Patient satisfaction with IMT was high
(median 8/10 (IQR 5-10)) and adherence was good. A three-month IMT
programme in children with NMD appears safe and well-tolerated, with
significant improvement in respiratory muscle strength and cough
efficacy. The use of IMT could be considered as an adjunct to
respiratory management in children with NMD.