Respiratory problems in spinal muscular atrophy in the paediatric age group

M. B Chiarini Testa, M. G. Paglietti, M. Pavone, A. Schiavino, C. Pedace, R. Cutrera

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
JournalPaediatrics and Child Health
Volume19
Issue numberSUPPL. 2
DOIs
Publication statusPublished - Dec 2009

Keywords

  • mechanically assisted cough
  • neuromuscular disease
  • non-invasive ventilation
  • respiratory physiotherapy
  • respiratory problems
  • spinal muscular atrophy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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