Abstract

BackgroundApproximately one in every 1200 South Africans is affected by a neuromuscular disease (NMD). Weak respiratory muscles and ineffective cough contribute to the development of respiratory morbidity and mortality. Early identification of individuals at risk of respiratory complications, through peak expiratory cough flow (PCF) measurement, may improve patient outcomes through timely initiation of cough augmentation therapy.ObjectivesThe aim of this study was to investigate the relationship between peak expiratory flow (PEF), forced vital capacity (FVC) and PCF in South African children with neuromuscular disorders.MethodsA retrospective descriptive study of routinely collected data was conducted.ResultsForty-one participants (aged 11.5 ± 3.6 years; 75.6% male) were included. There was a strong linear correlation between PCF and PEF (R = 0.78; p = 0.0001) and between PCF and FVC (R = 0.61; p = 0.0001). There was good agreement between PCF and PEF, with intraclass correlation coefficient of 0.8 (95% confidence interval, 0.7–0.9; p < 0.0001). Peak expiratory flow < 160 L.min−1 and FVC < 1.2 L were significantly predictive of PCF < 160 L.min−1 (suggestive of cough ineffectiveness), whilst PEF < 250 L.min−1 was predictive of PCF < 270 L.min−1, the level at which cough assistance is usually implemented.ConclusionPEF and FVC may be surrogate measures of cough effectiveness in children with neuromuscular disorders.Clinical implicationsPEF and FVC may be considered for clinical use as screening tools to identify patients at risk for pulmonary morbidity related to ineffective cough.

Highlights

  • Children with neuromuscular disorders (NMD) typically present with mild to severe muscle weakness that may extend to the respiratory muscles, depending on the condition and stage of the disease

  • There was good agreement between peak expiratory cough flow (PCF) and peak expiratory flow (PEF), with intraclass correlation coefficient of 0.8 (95% confidence interval, 0.7–0.9; p < 0.0001)

  • Peak expiratory flow < 160 L.min–1 and forced vital capacity (FVC) < 1.2 L were significantly predictive of PCF < 160 L.min–1, whilst PEF < 250 L.min–1 was predictive of PCF < 270 L.min–1, the level at which cough assistance is usually implemented

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Summary

Introduction

Children with neuromuscular disorders (NMD) typically present with mild to severe muscle weakness that may extend to the respiratory muscles, depending on the condition and stage of the disease. Respiratory muscle weakness contributes to rib cage deformity and, together with reduced mobility and potentially poor sitting posture, may result in chest wall deformities, which further impacts respiratory function (Panitch 2009:S215–S218). Shortening and fibrosis of the chest wall muscles, because of an inability to fully expand the chest as a consequence of inspiratory muscle weakness, result in a progressive decrease in chest wall compliance, leading to an eventual restrictive pattern of respiratory disease (Gozal 2000:141–150; Panitch 2009:S215–S218). Identification of individuals at risk of respiratory complications, through peak expiratory cough flow (PCF) measurement, may improve patient outcomes through timely initiation of cough augmentation therapy

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