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Human A.1,2, Corten L.2, Jelsma J.2, Morrow B.3
1Sefako Makgatho Health Science University, Physiotherapy, Garankuwa, Pretoria, South Africa, 2University of Cape Town, Health and Rehabilitation Sciences (Division Physiotherapy), Observatory, Cape Town, South Africa, 3University of Cape Town, Paediatrics and Child Health, Rondebosch, Cape Town, South Africa
Background: Patients with neuromuscular diseases (NMDs) are at risk of morbidity and mortality due to respiratory compromise, caused primarily by diaphragmatic and intercostal muscle weakness. Inspiratory muscle weakness can lead to decreased lung volumes; altered and ineffective breathing; decreased thoracic and shoulder mobility, chest expansion and alveolar ventilation; poor secretion clearance and ultimately respiratory failure. The use of inspiratory muscle training (IMT) to improve or preserve respiratory function in NMD patients remains controversial.
Purpose: To determine the safety of IMT and its effects on morbidity, adverse events, muscle strength and endurance, pulmonary function, dyspnoea and health-related quality of life (HRQoL), in children and adolescents with NMDs.
Methods: The Cochrane methodology for systematic reviews was followed (Prospero CRD42014013875). In October/November 2014, fifteen online databases were searched for published and on-going studies, using pre-specified search strategies. Randomised, quasi-randomised, cross-over and clinical controlled trials were included if they assessed the use of an external IMT device compared to control or alternative IMT intervention in children aged five to 18 years with NMDs. Data from included studies were extracted using a pre-structured data extraction form. The methodological quality and risk of bias of included studies were independently assessed using a standardised tool. Data were analysed using RevMan software (version 5.2).
Results: Seven studies (n=168) were included for analysis and synthesis. The majority of the studies were conducted on males diagnosed with Duchennes Muscular Dystrophy. The intensity, repetitions, frequency, rest intervals and duration of IMT differed between studies, but most used threshold IMT devices with mouthpieces over a medium to long-term period (3-6 months). The frequency of hospitalisations, use of antibiotics, HRQoL or any adverse events related to the intervention were not reported. Six studies showed no significant changes in pulmonary function tests between experimental (respiratory muscle training) and control groups (no intervention/sham, breathing exercises or peak flow meter). Two studies reported significant improvement in inspiratory muscle endurance compared to no treatment. Four studies reported a significant improvement in inspiratory muscle strength in experimental groups compared to either no treatment, breathing exercises or use of a peak flow meter. Meta-analysis of two comparable studies revealed a significant, large effect size (p .001) for mean maximum inspiratory muscle strength (Pimax/MIP) in cmH2O, favouring the experimental (IMT) group. Other outcome measures could not be pooled due to heterogeneity.
Conclusion(s): There is no clear evidence for or against the use of IMT in children and adolescents with NMDs, despite some indications of statistical improvement in Pimax/MIP and individual improvement in endurance associated with IMT. It has to be noted that improvement in Pimax can be attributed to a learning effect caused by training with a threshold device. There is currently no consensus regarding optimal respiratory muscle training devices, protocols and methods to ensure clinically relevant changes for the NMD population.
Implications: Insufficient evidence exists to guide clinical IMT practice amongst children and adolescents with NMDs. High-quality clinical research is needed to determine the effect of IMT on clinical long-term outcomes, including respiratory function, morbidity and HRQoL among various types of NMDs.
Funding acknowledgements: This work is supported by the URC Equipment Grant (Western Cape); SMU Research Development Grant; and the SASP PhD grant.
Topic: Cardiorespiratory
Ethics approval: Human Research Ethics Committee (UCT): 513/2015
All authors, affiliations and abstracts have been published as submitted.
