TABLE 1

Overview of Key Issues Related to DMD Emergency Care

Advance directives, history, and contacts
 Determine whether there are restrictions on resuscitation
 Ask for the patient’s emergency card and baseline test results, including ECG results
 Obtain a brief history with a focus on baseline respiratory and cardiac status, including the use of relevant devices and medications
 Determine whether the patient is treated with chronic steroid therapy
 Contact the patient’s neuromuscular specialist
Breathing problems
 Ask about respiratory symptoms and home equipment
 Monitor Spo2 levels via pulse oximetry; even mild hypoxemia (Spo2 <95% in room air) is a concern; do a blood gas analysis if necessary
 Treat with noninvasive ventilation and frequent application of a cough assistance device (or manual assisted coughing if device is unavailable); use the patient’s home equipment when available
 Obtain a portable chest radiograph
 Obtain early consultation with a respiratory therapist and respiratory physician
Cardiac problems
 Ask about cardiac symptoms
 Monitor heart rate and rhythm
 Obtain an ECG (this is typically abnormal and Q waves might be expected) and portable chest radiograph
 Measure blood levels of B-type natriuretic peptide, troponin I, or both, as indicated
 Consider worsening cardiomyopathy, congestive heart failure, and arrhythmias
 Obtain an echocardiogram when necessary
 Obtain early consultation with a cardiologist
Endocrine problems
 Determine whether stress steroid dosing is necessary
 For critical adrenal insufficiency, administer intravenous intramuscular hydrocortisone: 50 mg for children <2 y old; 100 mg for children ≥2 y and adults
 In less critical situations, consult the PJ Nicholoff Steroid Protocol
 Obtain early consultation with an endocrinologist
Orthopedic problems
 Assess for long-bone or vertebral fractures as indicated
 Review critical precautions related to sedation and anaesthesia if applicable (see text)
 Consider fat embolism syndrome if individual has dyspnea or altered mental status
 Obtain consultation with an orthopedic specialist early in the process
Disposition after discharge from emergency care
 Be aware that most patients will need hospital admission (eg, to initiate or intensify respiratory or cardiac therapy, or to manage fractures)
 Early in the process, initiate emergency transport to a center specializing in the care of patients with DMD, in cooperation with the individual’s neuromuscular specialist
  • This table is adapted with permission from Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, an update, part 3: primary care and emergency medicine, psychosocial care, and transitions of care across the lifespan. Lancet Neurol. 2018;17(5):447. B-type, brain; Spo2, blood oxygen saturation.