Transcript Duchenne Muscular Dystrophy: Considerations for Surgery

Duchenne Muscular Dystrophy:
Emergency Care Considerations
Introduction
• In an emergency situation, a range of factors should be
taken into account
– Diagnosis, current medication, respiratory and cardiac
status, and associated medical disorders should be made
clear from the outset to the emergency room staff
– Many health professionals are not aware of management
strategies available for DMD: the current life expectancy
and expected good quality of life should also be explained
to reduce the risk of therapeutic nihilism
– Anaesthetic issues relating to DMD need to be taken into
account at all times if surgery or sedation is needed
Steroids
• Chronic steroid use needs to be made clear to
emergency staff, including:
– How long patient has been using steroids
– Whether any doses have been missed
– Any previous use of steroids
• Treating clinicians should also be made aware of
the concomitant steroid risks of
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Reduced stress response
Masking of infection, and possible
Increased risk of gastric ulceration
(Rarely) other steroid complications
Breathing Problems
• Risk of respiratory failure supervening during an intercurrent
infection is high in those with borderline respiratory function
• Care in the use of opiates and other sedating medication is
essential, as is care in the use of oxygen without ventilation
owing to the risk of hypercapnia.
• If nocturnal ventilation already being used, then access to the
ventilator is essential during any acute event or intervention.
• For those who are already ventilated, the team involved with
respiratory care should be involved as soon as possible.
• The family should record the latest breathing test results (e.g.
FVC), which is helpful for doctors in the event of acute illness
Heart Function
• Awareness of the risk of heart arrhythmias
and cardiomyopathy is important
• The family should keep a record of the latest
heart test results (e.g. left ventricular ejection
fraction, LVEF) and any medications – these
are helpful for doctors in the event of acute
illness
Broken Bones
• Early mobilisation in the event of a fracture is
very important to maintain ambulation
• Internal fixation may be preferred to cast to
allow weight bearing
• Physio/ neuromuscular doctor should be
consulted in management of fractures
References & Resources
• The Diagnosis and Management of Duchenne
Muscular Dystrophy, Bushby K et al, Lancet
Neurology 2010 9 (1) 77-93 & Lancet
Neurology 2010 9 (2) 177-189
– Particularly references, p186-188
• The Diagnosis and Management of Duchenne
Muscular Dystrophy: A Guide for Families
• TREAT-NMD website: www.treat-nmd.eu
• CARE-NMD website: www.care-nmd.eu