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      Every breath counts! : Inspiratory muscle training in children with neuromuscular diseases: a cross-over randomised controlled trial

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            Abstract

            Progressive respiratory muscle weakness and ineffective cough contributes to morbidity and mortality in children with neuromuscular diseases (NMD). Inspiratory muscle training (IMT) aims to preserve or improve respiratory muscle strength and reduce respiratory morbidity. This study aimed to determine the safety and efficacy of IMT in children with NMD. A randomised cross-over study compared three-month intervention (IMT) with control periods. During the intervention, children with NMD (5-18 years) from two provinces in South Africa 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 participants (median (IQR) age of 12.33 (10.03-14.17) years), mostly male (n=20) and non-ambulant (n=14) were included. No adverse events related to IMT were reported. There was no evidence of a difference in median patient hospitalisation and respiratory tract infection rates between control and intervention periods (p=0.60; p=0.21). 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 a change of 3.04(±11.93)cmH 2O (p=0.01) and -16.59 (±48.29) L/min (p=0.0005) during the control period. There was no evidence of change in spirometry, functional ability and total health-related quality of life scores following intervention. 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 is well tolerated, appears to be safe and is associated with a significant improvement in respiratory muscle strength and cough efficacy.

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            Journal
            ScienceOpen Posters
            ScienceOpen
            6 September 2022
            Affiliations
            [1 ] Department of Physiotherapy, School of Health Care Sciences, University of Pretoria, South Africa.
            [2 ] School of Health Sciences (Physiotherapy), University of Brighton, Eastbourne, United Kingdom.
            [3 ] Physiotheray department, Red Cross War Memorial Children’s Hospital, Rondebosch, South Africa.
            [4 ] Department of Paediatrics and Child Health, University of Cape Town, South Africa.
            Author notes
            Author information
            https://orcid.org/0000-0002-5960-6256
            Article
            10.14293/S2199-1006.1.SOR-.PPAVCQ0.v1
            718a3841-f81b-4348-8510-187d7af0dca1

            This work has been published open access under Creative Commons Attribution License CC BY 4.0 , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Conditions, terms of use and publishing policy can be found at www.scienceopen.com .

            History
            : 6 September 2022

            The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.
            Medicine
            Inspiratory muscle training,neuromuscular diseases,children,paediatric,inspiratory muscle strength,cough ability,randomised controlled trial

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