Back to a podcast already. This time there is a brand new guest, the very excellent Dr Gail Wong. Gail works at The Children’s Hospital at Westmead but also spent a number of years at Sick Kids’ in Toronto. Starting with the sort of case none of us relish, the chat covers the mucopolysaccharidoses, management of tricky airways and the ways modern treatments have made the old descriptions of kids with these conditions less relevant.
Anyway, here it is.
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Gail kicks off with a case she’s had prior clearance to discuss.
The intro mix is by Blank & Kytt and the outro bit is by Breakmaster Cylinder and is provided under an Attribution Licence/Creative Commons set up on the Free Music Archive.
The image is shared under Creative Commons and is unchanged from the flickr post by Jason Tong.
There is plenty of extra reading to check out to get a more comprehensive story about kids with these conditions than we could cover in a quick chat.
Here’s the one by Walker and crew mentioned in the conversation:
Kirkpatrick K, Ellwood J, Walker RWM. Mucopolysaccharidosis type I (Hurler syndrome) and anaesthesia: the impact of bone marrow transplantation, enzyme replacement therapy, and fiberoptic intubation on airway management. Pediatr Anesth. 2012;22:745-51.
Here’s a couple of reviews:
Hack HA, Walker R, Gardiner P. Anaesthetic implications of the changing management of patients with mucopolysaccharidoses. Anaesthesia Intensive Care. 2016;44:660-8.
Walker R, Belani KH, Braunlin EA, et al. Anaesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36:211-9.
Here are a couple covering longer-term outcomes:
Aldenhoven M, Wynn RF, Orchard PJ, et al. Long-term outcome of Hurler syndrome patients after hematopoietic cell transplantation: an international multi-centre study. Blood. 2015;2164-72.
Lum SH, Stephen KM, Ghosh A, et al. Long term survival and cardiopulmonary outcome in children with Hurler syndrome after haematopoietic stem cell transplantation. J Inherit Metab Disease. 2017;40:455-60.
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