Decision-Making Regarding Ventilator Support in Children with SMA Type 1-A Cross-Sectional Survey among Physicians.


Journal

Neuropediatrics
ISSN: 1439-1899
Titre abrégé: Neuropediatrics
Pays: Germany
ID NLM: 8101187

Informations de publication

Date de publication:
12 2019
Historique:
pubmed: 14 8 2019
medline: 30 5 2020
entrez: 14 8 2019
Statut: ppublish

Résumé

Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by muscle atrophy and severe proximal muscle weakness. In the absence of curative treatment, it has been controversial whether critically ill infants with SMA type 1 should receive ventilator support. The aim of this study was to investigate the process of decision-making regarding ventilator support in children with SMA type 1 from the perspectives of physicians. A web-based survey with 17 questions and 2 case vignettes was conducted in 671 physicians in Germany and Switzerland from 12/2016 to 03/2017. The survey focused on factors influencing the decision about ventilator support and the content in informed consent discussions. Additionally, physicians were asked about their general attitude towards mechanical ventilation in children with SMA type 1 and their hypothetical clinical management in emergency settings using case vignettes. Hundred and sixty-five physicians participated in the survey (50.3% child neurologists, 18.8% specialists for ventilator support, 6.1% pediatric palliative care physicians, and 6.1% with more than one of these specializations). Of all physicians, 44.2% confirmed to have experience with SMA type 1 patients using ventilator support. In summary, our results show that physicians' attitudes and experiences about mechanical ventilation in children with SMA type 1 vary considerably and are likely to influence the outcome in informed consent discussions and the hypothetical management in emergency settings.

Identifiants

pubmed: 31408889
doi: 10.1055/s-0039-1694986
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

359-366

Informations de copyright

Georg Thieme Verlag KG Stuttgart · New York.

Déclaration de conflit d'intérêts

AP reports personal fees for presentations and training activities from Biogen, outside the submitted work; TL participated in a sponsored workshop by Biogen. JK reports grants, personal fees, and nonfinancial support from Biogen; grants, personal, fees and nonfinancial support from Roche; personal fees and nonfinancial support from Avexis, outside the submitted work.

Auteurs

Astrid Pechmann (A)

Department of Neuropediatrics and Muscle Disorders, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Thorsten Langer (T)

Department of Neuropediatrics and Muscle Disorders, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Janbernd Kirschner (J)

Department of Neuropediatrics and Muscle Disorders, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Department of Neuropediatrics, University Hospital Bonn, Bonn, Germany.

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