Palliative care in children with spinal muscular atrophy type I: What do they need?

Title

Palliative care in children with spinal muscular atrophy type I: What do they need?

Creator

García-Salido A; de Paso-Mora MG; Monleón-Luque M; Martino-Alba R

Publisher

Palliative & Supportive Care

Date

2014

Subject

spinal muscular atrophy

Description

Objective: Our aim was to describe the clinical evolution and needs of children with spinal muscular atrophy type I treated in a domiciliary palliative care program. Method: We undertook a retrospective chart review of nine consecutive patients. Descriptions of the clinical and demographic profile of children with spinal muscular atrophy (SMA) type I were referred to a pediatric palliative care team (PPCT). Results: Six males and three females were admitted to the PPCT, all before six months of age, except for one afflicted with SMA type I with respiratory distress. The median time of attention was 57 days (range 1-150). The domiciliary attention mainly consisted of respiratory care. The patient with SMA type I with respiratory distress required domiciliary mechanical ventilation by tracheotomy. In all cases, a nasogastric tube (NT) was indicated. As end-of-life care, eight required morphine to manage the dyspnea, four received it only by enteral (oral or NT) administration, and four received it first by enteral administration with continuous subcutaneous infusion (CSI) later. Three of the four patients with CSI also received benzodiazepines. While they were attended by the PPCT, none required hospital admission. All the patients died at home except for the one attended to for just one day. Significance of Results: Domiciliary care for these patients is possible. The respiratory morbidity and its management are the main issues. Application of an NT is useful to maintain nutritional balance. Morphine administration is necessary to manage the dyspnea. Palliative sedation is not always necessary.
2014-02

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Pages

1-5

Citation

García-Salido A; de Paso-Mora MG; Monleón-Luque M; Martino-Alba R, “Palliative care in children with spinal muscular atrophy type I: What do they need?,” Pediatric Palliative Care Library, accessed January 26, 2022, https://pedpalascnetlibrary.omeka.net/items/show/15018.

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