Emergency Care Considerations

  • If you find yourself needing to go to the hospital in an emergency situation, there are a range of factors that should be taken into account.
  • Your primary care provider or NMS should call the hospital before you arrive. This will allow the hospital staff to receive, and appropriately care for you
  • If you find yourself in accident and emergency (A&E), be sure to call your NMS or neuromuscular team
  • Your diagnosis of Duchenne, current medications, presence of any respiratory and cardiac complications, and the people who are your normal key medical team should be made clear to the admitting unit
  • As many health professionals are not aware of the potential management strategies available for Duchenne, the current life expectancy and expected good quality of life should also be explained

Ideally, you should always carry an emergency card and a have access to a brief medical summary provided by your NMS that includes your:

  • Diagnosis
  • List of medications
  • Baseline medical status including the results of recent pulmonary and heart function studies
  • Any past history of recurring medical problems (i.e. pneumonia, heart failure, kidney stones, gastroparesis [delayed stomacy emptying])
  • Brief discussion of initial assessment and management

There is also information about what to do during an emergency on the PPMD mobile app for smartphones (www.ParentProjectMD.org/App), and in the UK from Action Duchenne in collaboration with others:  https://duchenneemergency.co.uk/.

Box 8. Major Points of Emergency Care

  1. Bring your emergency information card or alert card (found at https://www.musculardystrophyuk.org/) when talking to doctors, nurses and hospital administration
  2. Contact your NMS after the initial assessment to discuss disease management
  3. Advise the emergency staff if you are taking steroids
  4. If you can, bring copies of your most recent heart and pulmonary test results, such as FVC, ECG, and LVEF
  5. If you have a cough assist device and/or breathing equipment (i.e. BiPAP), bring them with you to the hospital
  6. If your oxygen level drops, the staff must be very careful giving oxygen without breathing support (ventilator). This can cause a situation where the urge to breathe is taken away
  7. If you have a broken bone, insist that they speak with your NMS or physiotherapist. Watch for signs and symptoms of fat embolism syndrome (FES)

Chronic daily steroid use needs to be made clear to the accident and emergency (A&E) providers. Tell the A&E staff how long you have been using daily steroids and if a dose(s) has been missed. It is also important to let the A&E staff know if you have used steroids in the past, even if you aren’t taking them now.

This may be important because:

  • Steroids can decrease the body’s response to stress, so extra stress doses of steroids may be needed in A&E
  • Steroids can increase the risk of stomach ulceration; this should be considered by the A&E staff
  • Rarely, other steroid-related complications can present acutely and should be considered by the A&E staff


  • Recent test results about your heart function can be helpful (e.g. ECG, echo or MRI results); bring those if you can
  • Remind the accident and emergency (A&E) staff that, for patients with Duchenne, the ECG is typically abnormal at all ages; bring a copy of your ECG with you can
  • During acute illness, continuous cardiac monitoring may be needed to be sure there is not an issue with your heart rate or rhythm


  • Recent test results about your respiratory function can be helpful (e.g. forced vital capacity, FVC); bring those if you can
  • It is important to bring any equipment that you use (cough assist, BiPAP, etc.) with you to the hospital in case they do not have equipment for you to use. Insist that the A&E staff use your equipment to assist in your care
  • If you use a ventilator at home, the hospital’s respiratory care team should be involved as soon as possible
  • Care in the use of opiates, other sedating medication and muscle relaxants is essential – they may affect your breathing rate and depth, making breathing shallower and slower
  • If anesthesia is needed, IV anesthesia should be used and inhaled anesthesia should be avoided; succinylcholine is strictly contraindiciated in Duchenne and should not be given

Breathing and coughing muscles are further weakened when you are ill, and the risk of these complications dramatically increases. Thus, if you have significantly weak breathing muscles:

  • Antibiotics may be needed for chest infections
  • Breathing support with a non-invasive ventilator is likely to be needed or needed for longer than usual
  • If oxygen is needed, use caution (see Box 9)
  • Continuous use of noninvasive ventilation while awake, interrupted as necessary for frequent assisted cough, should be a standard approach to respiratory illnesses in accident and emergency

Box 9. Oxygen - Caution!

  • We all breathe to inhale oxygen (O2) AND exhale carbon dioxide (CO2)
  • If you have reduced pulmonary function, giving oxygen may reduce the body’s drive to breathe and lead to high levels of carbon dioxide (called “CO2 retention” or “respiratory acidosis”). This can be dangerous, and may even be life threatening. Giving supplemental oxygen should be done with extreme caution and carbon dioxide should be monitored
  • Blood levels of carbon dioxide should be checked if oxygen saturation in the blood (measured by finger pulse oximetry) has decreased to <95 percent. If the carbon dioxide in the blood is elevated, manual and mechanically assisted cough and non-invasive breathing support are necessary
  • If oxygen is needed, it should be given with non-invasive ventilation (bi-level positive pressure ventilation) and with blood CO2 carefully monitored

People living with Duchenne are at risk of broken bones. Breaking a leg bone may make it difficult to walk again, especially if walking was becoming more difficult before the break. Let your neuromuscular team, especially your physiotherapist, know if there is a fracture so they can talk to the surgeons if necessary.

  • Surgical fixation, if it is appropriate for your fracture, is often a better option than a cast for a broken leg if someone is still walking (weight bearing is often sooner with surgical fixation than casting)
  • Input from a physiotherapist is crucial to make sure you get back on your feet as soon as possible
  • If the broken bone is one of the vertebrae (backbones) and there is pain in your back, input from your bone doctor or endocrinologist is needed to provide the right treatment

Fat embolism syndrome (FES) is a risk in Duchenne and presents a medical emergency. Immediately let the emergency staff know that you suspect a fat embolism. Symptoms of FES include:

  • Confusion and/or disorientation
  • “Not acting like yourself”
  • Rapid breathing and heart rate
  • Shortness of breath