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

Published online by Cambridge University Press:  24 February 2014

Alberto García-Salido ,
María García de Paso-Mora ,
Manuel Monleón-Luque  and
Ricardo Martino-Alba
Alberto García-Salido*
Affiliation:
Pediatric Critical Care Unit and Pediatric Palliative Care Unit, Hospital Infantil Universitario Niño Jesús, Madrid, Spain
María García de Paso-Mora
Affiliation:
Pediatric Palliative Care Unit, Hospital Infantil Universitario Niño Jesús.Madrid, Spain
Manuel Monleón-Luque
Affiliation:
Pediatric Palliative Care Unit, Hospital Infantil Universitario Niño Jesús.Madrid, Spain
Ricardo Martino-Alba
Affiliation:
Pediatric Palliative Care Unit, Hospital Infantil Universitario Niño Jesús.Madrid, Spain
*
Address correspondence and reprint requests to: Alberto García-Salido, Pediatric Critical Care Unit, Hospital Infantil Universitario Niño Jesús. Avenida Menéndez Pelayo 65, Madrid, Spain. 34915035900. E-mail: citopensis@yahoo.es
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Abstract

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.

Keywords

PediatricsPalliative careSpinal muscular atrophy
Type
Original Articles
Information
Palliative & Supportive Care , Volume 13 , Issue 2 , April 2015 , pp. 313 - 317
Copyright
Copyright © Cambridge University Press 2014 

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