Dispositivos ventilatórios não invasivos em criança portadora de amiotrofia espinhal do tipo 1: Relato de caso

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Abstract

Introduction: the spinal muscular atrophy type 1 (AME1) is an autosomal recessive genetic disorder that promotes cell death of motor neurons located in the anterior horn of the spinal cord and motor nuclei of the brainstem. Early mortality is linked to the bulbar dysfunction and respiratory failure, requiring hospitalization and invasive mechanical ventilation (IMV). The purpose of this study is to promote the maintenance patients with SMA type 1 without invasive ventilation support and individualized physical therapy protocol, improving child's quality of life and integration with their families. Description: this report relates a child diagnosed with AME1, who at 11 months was admitted and underwent IMV for 76 days, succeeding extubation after respiratory therapy protocol application using cough machine and noninvasive ventilation (NIV). Discussion: despite the difficulties and complications observed, there was persistence of the proposed assistance, reaching goal of withdrawal of IMV and transfer to home care with noninvasive ventilation devices. The IMV for tracheostomy is considered treatment of choice in Brazil. Notwithstanding, families need to be clarified of the irreversibility of the disease and strategic possibilities of therapeutic strategies (IMV, NIV and palliation) for the management of the child with severe SMA 1.

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Magalhães, P. A. F., d´Amorim, A. C. G., Mendes, A. P., Ramos, M. E. A., de Almeida, L. B. S., & Duarte, M. C. M. B. (2015). Dispositivos ventilatórios não invasivos em criança portadora de amiotrofia espinhal do tipo 1: Relato de caso. Revista Brasileira de Saude Materno Infantil, 15(4), 435–440. https://doi.org/10.1590/S1519-38292015000400007

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