http://hdl.handle.net/2123/14931
Title: | The management of children with Spinal Muscular Atrophy Type 1 in Australia |
Authors: | Tassie, B Isaacs, D Kilham, H Kerridge, I |
Keywords: | Australian consensus guidelines Spinal Muscular Atrophy Type 1 (SMA 1) end-of-life care of patients SMN1 clinical audit children diagnosed with SMA 1 actively managed dying ethical issues |
Issue Date: | 2013 |
Publisher: | Wiley |
Citation: | Tassie B, Isaacs D, Kilham H, Kerridge I. The management of children with Spinal Muscular Atrophy Type 1 in Australia. J Paediatr Child Health. 2013, 49:815-9. |
Abstract: | 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 long-term (>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. |
Description: | postprint |
URI: | http://onlinelibrary.wiley.com/doi/10.1111/jpc.12291/abstract http://hdl.handle.net/2123/14931 |
Type of Work: | Article |
Appears in Collections: | Research Papers and Publications. Sydney Health Ethics |
File | Description | Size | Format | |
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the-management-of-children-PP-2013.pdf | 520 kB | Adobe PDF |
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