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 – 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 (1) & Lancet Neurology (2) – Particularly references, p The Diagnosis and Management of Duchenne Muscular Dystrophy: A Guide for Families TREAT-NMD website: CARE-NMD website: