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Neuromuscular Respiratory Failure

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Pediatric Critical Care Medicine

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Abstract

A number of acute and chronic disorders, congenital and acquired, can affect muscular function, control of breathing, and the mechanics of respiration. Consequently, the leading cause of death among children afflicted with a neuromuscular disorder (NMD) is respiratory insufficiency. Even with excellent chronic management, the lives of these children may be punctuated by episodes of acute respiratory failure necessitating management in the pediatric intensive care unit (PICU). As long term survivorship with these disorders is increasing, there is an expectation of recovery to baseline functional status following acute crises. This requires the critical care specialist to have a broad understanding of the implications of neuromuscular disease and its management, that are unique and characteristic to this group. This chapter comprehensively details the anatomic and physiological disturbances particular to neuromuscular respiratory failure. Interactions between respiratory muscles, the chest wall, and the lung are highlighted. The management approach for this group of patients is then presented with a basis in the available evidence and the underlying pathophysiology. This includes secretion clearance, feeding and nutrition, non-invasive and invasive ventilation, extubation strategies, decision making regarding tracheostomy and palliative care. The significant defining features and treatment options for the major neuromuscular disorders that the critical care specialist encounters are also presented.

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Authors and Affiliations

  1. Department of Pediatrics and Adolescent Medicine, Pediatric Pulmonology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    R. Paul Boesch DO, MS

  2. Division of Pulmonary Medicine, Cincinnati Children’s Hospital Medical Center, MLC 2021, 3333, Burnet Avenue, Cincinnati, OH, 45242, USA

    Hemant Sawnani MBBS, MD

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  1. R. Paul Boesch DO, MS
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  2. Hemant Sawnani MBBS, MD
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Correspondence to R. Paul Boesch DO, MS .

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Editors and Affiliations

  1. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA

    Derek S. Wheeler

  2. Div. of Critical Care Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA

    Hector R. Wong

  3. Pediatric Critical Care Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA

    Thomas P. Shanley

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© 2014 Springer-Verlag London

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Boesch, R.P., Sawnani, H. (2014). Neuromuscular Respiratory Failure. In: Wheeler, D., Wong, H., Shanley, T. (eds) Pediatric Critical Care Medicine. Springer, London. https://doi.org/10.1007/978-1-4471-6356-5_16

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  • DOI: https://doi.org/10.1007/978-1-4471-6356-5_16

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