Respiratory problems in spinal muscular atrophy in the paediatric age group

• Published in: Paediatrics and child health 2009-12, Vol.19, p.S123-S126
• Main Authors: Testa, M.B. Chiarini, Paglietti, M.G, Pavone, M, Schiavino, A, Pedace, C, Cutrera, R
• Format: Article
• Language: English
• Subjects: mechanically assisted cough; neuromuscular disease; non-invasive ventilation; Pediatrics; respiratory physiotherapy; respiratory problems; spinal muscular atrophy
• ISSN: 1751-7222; 1878-206X
• DOI: 10.1016/j.paed.2009.08.017

Abstract The patient with neuromuscular disability (spinal muscular atrophy, muscular dystrophy, myopathy, amyotrophic lateral sclerosis, post-poliomyelitis syndrome or neuropathy) represents a typical example of a child with chronic respiratory disease requiring either hospitalisation or domiciliary assistance. For these patients, respiratory disease represents the main cause of morbidity and mortality, due primarily to weakness of the respiratory muscles, an ineffective cough and abnormal swallowing. A multidisciplinary approach, with the aim of improving the patient's quality of life, is therefore required. Such an approach involves various strategies, for example use of the cough-assist machine (a non-invasive respiratory physiotherapy that safely and consistently removes secretions in patients with an ineffective ability to cough), monitoring of nutritional status, the prevention of chest deformities, and the use of mechanically assisted ventilation – both non-invasive and invasive ventilation via a tracheotomy.