Palliative care in children with spinal muscular atrophy type 1: How do they die? Results from a French multicentric study (National Hospital clinical Research Program)

Title

Palliative care in children with spinal muscular atrophy type 1: How do they die? Results from a French multicentric study (National Hospital clinical Research Program)

Creator

Hully M; Barnerias C; Vanesse S; Viallard ML; Desguerre I

Publisher

European Journal Of Paediatric Neurology

Date

2017

Subject

clinical research; palliative therapy; Werdnig Hoffmann disease; 50-48-6 (amitriptyline); 52-26-6 (morphine); 57-27-2 (morphine); 549-18-8 (amitriptyline); Amitriptyline; benzodiazepine derivative; Child; Clinical Article; clinical practice; Diagnosis; Drug Therapy; Female; follow up; Human; infant; Intensive care unit; Interview; Male; Morphine; multicenter study; nasogastric tube; Noninvasive Ventilation; psychologist; quantitative analysis; Resuscitation; time of death

Description

Objective: The national Hospital Clinical Research Program (PHRC) called Assessment of clinical practices of palliative care in children with Spinal Muscular Atrophy Type 1 (SMA 1) was conducted to depict palliative practices in that fatal disease, in which death up to now occurs few weeks or months after the diagnosis. We here report data about the conditions of death for the patients included. Methods: In this French multicentric study, patients were included from june 2012 to june 2016. Parents and physicians filled in a specific health book during the follow up, the physician in charge filled in a survey concerning the patient's management over the last 48 hours before death, then a semidirected interview of the parents was conducted by a trained psychologist 6 to 18 months after the child's death. We here report the quantitative analysis of data obtained from the survey about patient's management around death. Results: 38 patients were included in the study (17 centres), data were available for 36 dead patients. Median age at inclusion was 3 months (0,6-10,4), death occurred at a median 5,5 month of age (1,5-16,4), i.e a median follow-up of 2 months (0,2-12,8). 39% of patients died at home, 6% in an intensive care unit. At the time of death, patients received morphine (56%), benzodiazepines (39%), amitriptyline (39%). Treatments were given through a nasogastric tube (83%), and oxygenotherapy was delivered (76%). 6% patients received noninvasive ventilation at the time of death. No resuscitation recommendations had been prepared for most patients (97%), written in 85%, after a multidisciplinary meeting in most cases (79%). Conclusion: Our data confirm current knowledge about natural outcome in SMA 1, death occurring very soon after the diagnosis, claiming for an effective palliative management of the patients, including the involvement of parents in medical care at home.

Rights

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Citation List Month

January 2018 List

Collection

Citation

Hully M; Barnerias C; Vanesse S; Viallard ML; Desguerre I, “Palliative care in children with spinal muscular atrophy type 1: How do they die? Results from a French multicentric study (National Hospital clinical Research Program),” Pediatric Palliative Care Library, accessed July 28, 2021, https://pedpalascnetlibrary.omeka.net/items/show/11229.

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