Ventilator Support in Children with Neuromuscular Disorders

Children with neuromuscular disorders can develop respiratory complications such as respiratory failure, recurrent pulmonary infections and secretion clearance difficulties related to weakness of inspiratory and expiratory muscles, swallowing problems and chest wall disorders. It is increasingly possible to identify children at risk of these problems, monitor their progress and intervene with targeted measures such as non-invasive ventilation and cough assist techniques. These have, in many groups, resulted in improved survival, reduced hospitalisations and enhanced quality of life. In other groups, non-invasive ventilation may be used to palliate symptoms and facilitate discharge to home. Prognosis in Duchenne muscular dystrophy has improved so that these individuals typically live into their late twenties/early thirties and some to middle age, and children with neuromuscular disorders who were previously expected to die in the paediatric age range are now successfully transitioning to adult care. This has important implications for decision-making, service planning, adult pulmonary teams and the patient and family.