Tracheotomy and children with spinal muscular atrophy type 1

Rul, Brigitte; Carnevale, Franco A.; Estournet, B.; Rudler, M.; Hervé, Christian

doi:10.1177/0969733011429014

Cited by 24 publications

(12 citation statements)

References 32 publications

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“…We repeated this analysis focusing on children <3 years with neuromuscular disease and no craniofacial/airway abnormalities (ie, conditions that would make caregivers favor IV over NIV). Whether to offer LTV and what modality to offer these children are debated (7, 22–24). To help ensure counts could be reasonably compared across sites, these latter two analyses included sites that contributed 10 or more quarters of data.…”

Section: Methodsmentioning

confidence: 99%

Children and Young Adults Who Received Tracheostomies or Were Initiated on Long-Term Ventilation in PICUs*

Edwards

Houtrow

Lucas

et al. 2016

Pediatric Critical Care Medicine

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Objectives To characterize patients who received tracheostomies for airway compromise or were initiated on long-term ventilation for chronic respiratory failure in pediatric intensive care units (PICU), and to examine variation in the incidence of initiation, patient characteristics, and modalities across sites. Design Retrospective cross-sectional analysis. Settings Seventy three North American PICUs that participated in the Virtual Pediatric Intensive Care Unit Performance System. Patients PICU patients admitted between 2009 and 2011. Interventions None. Measurements and Main Results Among 115,437 PICU patients, 1.8% received a tracheostomy or were initiated on long-term ventilation; 1034 received a tracheostomy only, 717 were initiated on invasive ventilation (IV), and 381 were initiated on noninvasive ventilation (NIV). Ninety percent had substantial chronic conditions and comorbidities, including more than 50% with moderate or worse cerebral disability upon discharge. Seven percent were initiated after a catastrophic injury/event. Across sites, there was variation in incidence of tracheotomy and initiation of long-term ventilation, ranging 0–4.6%. There also was variation in patient characteristics, time to tracheotomy, number of extubations prior to tracheostomy, and the use of IV versus NIV. Conclusions While the PICU incidence of initiation of tracheostomies and long-term ventilation was relatively uncommon, it suggests that thousands of children and young adults receive these interventions each year in North American PICUs. The majority of them have conditions and comorbidities that impose on-going care needs, beyond those required by artificial airways and long-term ventilation themselves.

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Section: Methodsmentioning

confidence: 99%

Children and Young Adults Who Received Tracheostomies or Were Initiated on Long-Term Ventilation in PICUs*

Edwards

Houtrow

Lucas

et al. 2016

Pediatric Critical Care Medicine

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“…[23][24][25] While most physicians would accept noninvasive ventilator support in children with SMA type 1, tracheotomy is discussed even more controversially. 16,17,26 In the consensus statement, tracheotomy ventilation should only be an option in selected patients in whom noninvasive ventilation is insufficient. 10 In our survey, only about half of physicians would inform families about invasive ventilation in case of chronic or acute respiratory failure in informed consent discussions.…”

Section: Discussionmentioning

confidence: 99%

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

2019

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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.

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“…In contrast, a United Kingdom consensus statement recommended that invasive ventilation was inappropriate once a SMA 1 diagnosis had been confirmed [22]. Additionally, while a consensus exists to provide 'proactive nutritional supplementation as soon as inadequate oral intake is recognized', no consensus exists as to how supplemental nutrition should be provided [7].…”

Section: Discussionmentioning

confidence: 99%

“…These results stand in contrast to literature that describes a much broader spectrum of practice from the United States, France and Japan. 11,[18][19][20][21][22] Despite SMA 1 having a well-described prognosis, approximately 40% of patients did not have a documented advance care plan, and 20% did not have a documented DNR. No association was found between the absence of advance care directives and having invasive interventions.…”

Section: Discussionmentioning

confidence: 99%

Management of children with spinal muscular atrophy type 1 in Australia

Tassie

Isaacs

Kilham

et al. 2013

J Paediatrics Child Health

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Aims: To (1) estimate the prevalence of Spinal Muscular Atrophy Type 1 (SMA 1); (2) describe what practices characterise end-of-life care of patients with SMA 1; (3) ascertain whether a consistent approach to the management of these patients exists in Australia. Methods: An audit of the Australasian pathology laboratories offering the diagnostic SMN1 deletion test was conducted for patients diagnosed with SMA in Australia for 2010 and 2011. In addition, a retrospective clinical audit was conducted in eight major Australian paediatric hospitals of the end-of-life care provided to children with confirmed SMA 1 from 2005 to 2010. Results: 35 children were included in the clinical audit, accounting for an estimated 61% of children diagnosed with SMA 1 from 2005-2010. 26% were ventilated invasively, only two of whom were intubated after the diagnosis was confirmed. No children were ventilated longterm (>90 days) or had a tracheostomy performed. Nasogastric tube feeding was a common measure to support adequate nutritional intake. Total parenteral nutrition, gastrostomy and fundoplication were not provided for any children. Conflict over end-of-life care decisions was documented in one instance, without the involvement of a guardianship tribunal.Conclusion: There appears to be a consistent approach in the management of children with SMA 1 in Australia, which can be characterised as 'actively managed dying.' This study could contribute to the development of Australian consensus guidelines for the management of these children. These results also highlight a number of ethical issues related to the management of children with SMA 1.

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Tracheotomy and children with spinal muscular atrophy type 1

Cited by 24 publications

References 32 publications

Children and Young Adults Who Received Tracheostomies or Were Initiated on Long-Term Ventilation in PICUs*

Children and Young Adults Who Received Tracheostomies or Were Initiated on Long-Term Ventilation in PICUs*

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

Management of children with spinal muscular atrophy type 1 in Australia

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