Symptom management and psychological support for families are the cornerstones of end-of-life care for children with spinal muscular atrophy type 1

Title

Symptom management and psychological support for families are the cornerstones of end-of-life care for children with spinal muscular atrophy type 1

Creator

Pede CD; Agosto C; Tommasi V; Gregorio A; Benini F

Identifier

10.1111/apa.14086

Publisher

Acta Paediatrica

Date

2017

Subject

Dyspnoea; End Of Life; Paediatric Palliative Care; Pain Relief; Spinal Muscular Atrophy Type 1

Description

AIM: This study described end-of-life care for children affected by spinal muscular atrophy type 1 (SMA1), which is characterised by progressive muscle weakness and develops in the first six months of life. METHODS: We retrospectively analysed 17 children (13 boys) who attended the University of Padua's paediatric palliative care centre in Italy from March 2000 to March 2015. All the children received supportive care without proactive respiratory intervention to prolong survival. RESULTS: The median age at admission was 3.57 months and the median age at death was 6.80 months. The most frequent symptoms were dyspnoea and pain. In the last 72 hours of life 15/17 children required more intense doses of morphine and, or, benzodiazepines for intractable dyspnoea and pain, but deep palliative sedation was not needed. Airway suction to manage secretions and nasogastric tubes were required in all cases. The place of death was previously planned by the parents in all cases - home, hospital or hospice - and 15/17 deaths occurred in that place. We also interviewed 16 of the 17 parents after their child died. CONCLUSION: Our study found that symptom management and psychological support for families were the cornerstones of end-of-life care for children with SMA1. This article is protected by copyright. All rights reserved.

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).

Citation List Month

November 2017 List

Notes

1651-2227
Pede, Chiara Di
Agosto, Caterina
Tommasi, Valentina De
Gregorio, Alessandra De
Benini, Franca
Journal Article
Norway
Acta Paediatr. 2017 Sep 23. doi: 10.1111/apa.14086.

Citation

Pede CD; Agosto C; Tommasi V; Gregorio A; Benini F, “Symptom management and psychological support for families are the cornerstones of end-of-life care for children with spinal muscular atrophy type 1,” Pediatric Palliative Care Library, accessed October 26, 2021, https://pedpalascnetlibrary.omeka.net/items/show/11024.

Social Bookmarking