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Duchenne Muscular Dystrophy: Emergency Care Considerations

Duchenne Muscular Dystrophy: Emergency Care Considerations. Introduction. In an emergency situation, a range of factors should be taken into account

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Duchenne Muscular Dystrophy: Emergency Care Considerations

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  1. Duchenne Muscular Dystrophy:Emergency Care Considerations

  2. 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

  3. 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 • Reduced stress response • Masking of infection, and possible • Increased risk of gastric ulceration • (Rarely) other steroid complications

  4. 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

  5. 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

  6. 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

  7. 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

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