Inspiratory muscle training in children and adolescents living with neuromuscular diseases: A pre-experimental study.

Anri Human,Brenda M Morrow

doi:10.4102/sajp.v77i1.1577

Abstract

BackgroundChildren 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).ObjectivesTo 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.MethodsA 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.ResultsThere 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.ConclusionInspiratory 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 implicationsLarger, 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.

Highlights

Children with neuromuscular diseases (NMD) often present with respiratory morbidity because of underlying progressive respiratory muscle weakness (Chatwin et al 2018; Mayer et al 2017; Park et al 2010)
Two patients presented with cardiopulmonary comorbidity, including right ventricular hypertrophy and pulmonary hypertension; whilst one patient had a confirmed diagnosis of attention deficit hyperactivity disorder (ADHD) and mild cognitive impairment, but was still able to follow instructions
We found a significant improvement in the ability to create airflow (PIF) when taking a deep breath (p = 0.02), despite all participants presenting with restrictive lung disease of varying severity

Summary

Introduction

Children with neuromuscular diseases (NMD) often present with respiratory morbidity because of underlying progressive respiratory muscle weakness (Chatwin et al 2018; Mayer et al 2017; Park et al 2010). The combination of poor cough, decreased airway clearance, restrictive lung disease and recurrent respiratory infections leads to a progressive decline in pulmonary function and respiratory failure (Boentert, Wenninger & Sansone 2017; Farrero et al 2013; Mayer et al 2017; Toussaint et al 2018). It is hypothesised that inspiratory muscle training (IMT) could ameliorate the decline of inspiratory muscle strength, thereby preserving pulmonary function and cough ability for longer and delaying the onset of respiratory muscle failure (Aboussouan 2009; Chatwin et al 2003; Yeldan, Gurses & Yuksel 2008). Inspiratory muscle training (IMT) aims to preserve or improve respiratory muscle strength, thereby reducing morbidity and improving health-related quality of life (HRQoL)

Objectives

Methods

Results

Discussion

Conclusion