Emergency Care

Considerations

Duchenne Muscular Dystrophy (DMD) is a relatively unfamiliar diagnosis within the South African Community of healthcare professionals; we recommend you read this and bookmark it on your phone or print a copy to put in your “My Emergency Assistant” file for when an emergency may occur.

General Recommendations & Precautions

  • Keep immunizations up to date and get influenza vaccine annually
  • People taking daily, long term steroids should avoid live vaccines when possible
  • Always wear seat belts – in the car/wheelchair/scooter
  • Invest in a stylish yet functional medical bracelet

Medical Alert Bracelets

QJDMD knows first-hand how stressful an emergency situation with Duchenne can be. Medical bracelets provide you with the peace of mind that in case of an emergency your medical details (and a contact number of a next of kin) will be provided to the care team.

Breathing Problems

If an individual with DMD complains of difficulty breathing, you should seek emergency care
  • Only give oxygen with close monitoring of CO2 levelsbreathing may need to be supported (with BiPAP, for example).
  • Take your equipment (cough assist, BiPAP) with you to the hospital/emergency room (ER); alert your neuromuscular team that you are going to the ER/hospital.

Emergency Preparedness

QJDMD has created the “My Emergency Assistant”, to assist all parents with boys with Duchenne, to have all information readily available – if you purchase your binder – most of the emergency will be covered – this could be updated after appointments and kept close in case of emergency. Our binders ensure you will not be caught off guard with any information the emergency department might require. 

In addition please insert your emergency preparedness plan and all contact details in the front of your file.

Broken / Suspected Fracture/Fall/Trauma

If you suspect that you/your child may have broken a bone or has a fall/trauma, remember:
  • If ambulatory: Ask if internal fixation/surgery rather than casting, may be possible. Surgery may help preserve walking.
  • If your child has had a fall or a leg injury, and has rapid onset shortness of breath/difficulty breathing, changes in alertness (confusion, agitation, disorientation) this is an emergency; go immediately to the ER and alert staff that symptoms could be due to Fat Embolism Syndrome (FES).

Anaesthesia

  • Local and intravenous (IV) anaesthesia are generally well tolerated. Make sure the emergency and/or anaesthesia team know the patient has Duchenne muscular dystrophy..
  • Caution should also be used with inhaled anaesthetics.
  • Because cardiomyopathy affects all individuals with DMD, we encourage you to talk to providers about cardiac anaesthesia precautions prior to sedation.

Dental Procedures

If you/your child is having a dental procedure, remember:
  • Dentistry generally can, and should, be performed with the minimal amount of anaesthesia possible while providing the patient maximal physical and emotional comfort.
  • Local anaesthetics, nitrous oxide, and an oxygen “wash out” are safe for most patients with Duchenne, especially patients who are ambulatory with normal pulmonary function (normal breathing).
  • Patients with Duchenne who have pulmonary dysfunction (abnormal breathing) should consider receiving dental care requiring general anaesthesia in a hospital or surgery centre staffed with an anaesthesiologist, and equipped to monitor intra-operative respiratory functioning and to manage potential respiratory and cardiac emergencies.

Pain

If you/your child is having severe pain, remember:
  • In Duchenne, there are many issues that may be related to pain.
  • It is important to take information about you/your child with you, so that the emergency room staff can do a thorough evaluation, diagnose the cause of pain quickly, and help manage the pain appropriately.

Surgery

If you/your child is scheduled for surgery, or having emergency surgery, remember:
  • Avoid inhaled anaesthesia
  • IV anaesthesia is considered to be safe (with close monitoring)
  • People with Duchenne should NOT receive succinylcholine
  • Local anaesthetics & Nitrous Oxide are safe for minor dental procedures

Vomitting and/or unable to take daily Corticosteroids

If you/your child is vomiting and/or unable to take daily corticosteroids for 24 hours, remember:
  • Call your neuromuscular specialist and let them know you are going to the emergency room and why
  • Request substitute IV corticosteroid until oral medications are tolerated 
  • Remind clinicians that high liver enzymes (AST/ALT) are normal for people with Duchenne

Steroids Dosing

Steroids such as prednisone or deflazacort should never be stopped suddenly.

Additionally, individuals experiencing a medical crisis such as severe illness, trauma, or surgery may require “stress dosing”