Health emergencies are very stressful and something we hope all families can avoid. However, you never know when one will occur.
Thank you to our Canadian clinicians, Dr. Amin, Dr. Ward and Dr. McAdam, who helped co-create the information and resources below, and a special thank you to Parent Project Muscular Dystrophy in the United States for allowing us to adapt their material.
In an emergency remember to T.H.I.N.K:
Don't cut it close, take an extra dose.
- Rachael Witzke-Prendergast (Duchenne Mom), Ottawa Valley, ON
Emergency Care Guidelines
Risk: Respiratory Failure
- If you/your child is having trouble breathing/coughing, the oxygen saturations are low, and/or having more secretions and trouble managing them, please contact your respiratory care team and/or call 911 depending on the severity of symptoms.
- Bring your equipment (cough assist, BiPAP, etc.) and all medicines (including your steroids) with you to the hospital/ER.
- In ambulance or ER, medical providers must only give Oxygen with close monitoring of CO2 levels; breathing may need to be supported.
- Call your neuromuscular team and tell them you are going to the hospital/ER (do not depend on the ER staff to do this).
Risk: Pain, Loss of Ambulation, and Fat Embolism
Leg or Arm Fracture:
- If still ambulatory, tell doctor. Standing and walking ASAP may preserve ambulation (i.e. internal fixation surgery with rapid weight bearing, no casting).
Fat Embolism Syndrome:
- May develop after fall or fracture. If you your child has difficulty breathing, seems confused, or is less alert after a fall/fracture, this is an emergency; go immediately to the ER and alert staff that symptoms could be due to Fat Embolism Syndrome (FES)
Risk: Adrenal Crisis
- Remember to tell the doctor if you/your child is taking steroids.
- If you/your child has sustained an injury or is unable to take steroids daily, take intramuscular hydrocortisone and then proceed to the nearest emergency department. (6 mg of deflazacort = 5 mg prednisone = 20 mg hydrocortisone) – see the video below on how to take intramuscular hydrocortisone
- Bring all equipment/medications and the PJ Nicholoff Protocol with you.
- Watch for signs of adrenal crisis during times of server illness, injury, or surgery. Symptoms of adrenal crisis can include any of the following:
- Abdominal pain
- Confusion or coma
- Dizziness or light-headedness
- Flank pain (pain in one side of the body between the upper belly area (abdomen) and the back.)
- High fever
- Loss of appetite
- Loss of consciousness
- Low blood pressure
- Profound weakness
- Rapid heart rate
- Rapid breathing
- Slow, sluggish movement
- Unusual and excessive sweating on face or palms
Risk: Rhabdomyolysis (massive breakdown of skeletal muscle that may be life threatening)
Inhaled anesthesia can cause rhabdomyolysis among other serious complications (i.e., cardiac arrest) in patients with Duchenne. If possible, inhaled anesthesia should be avoided.
IV anesthesia is considered safe with close monitoring.
Succinylcholine should never be used.
Local anesthesia and nitrous oxide are generally safe for minor dental procedures.
Rhabdomyolysis occurs when massive amounts of muscle fibers breakdown and release their myoglobin (a muscle protein) into the bloodstream. This release of myoglobin can cause serious complications such as kidney failure. If the kidneys fail, they can not get rid of harmful waste in the body such as potassium, which can lead to dangerous heart rhythms. Rhabdomyolysis can happen with too much physical activity and dehydration or with exposure to certain types of inhaled anesthetics (i.e., during surgery or a medical procedure).
Signs and symptoms of rhabdomyolysis include:
Muscle pain in the shoulders, thighs, or lower back
Increased muscle weakness or trouble moving arms and legs
Nausea or vomiting
Fever and rapid heart rate
Confusion, dehydration, or changes in consciousness
Dark red or brown urine or decreased urination
- Abdominal pain
Key Points to Remember
- When you show up at an ER, hospital, or doctor’s office that is not part of your/your child’s regular care team, you are the expert!
- For children under 18 years old, try to get care in a pediatric hospital if possible.
- Call your/your child’s neuromuscular team and tell them you are going to the ER/hospital (do not depend on the ER staff to do this).
- Remember stress dosing if you/your child are on steroids.
- Take IV hydrocortisone in an emergency, during severe stress, or if you/your child can not take steroids orally.
- Keep immunization up to date, including, Pneumonia, COVID, and annual influenza (flu) vaccines.
- Always wear seat belts in the car AND chairs/wheelchairs/scooters/shower chairs.
- A fracture could be caused by a minor event.
- Know your/you child’s baselines, i.e., heart rate, blood pressure, and liver enzymes (ALT/AST- in Duchenne are higher than normal).
- Wear your medical alert bracelet, including:
- Medical condition (Duchenne muscular dystrophy)
- Medications taking (i.e., steroid dependant)
- No Succinylcholine or inhaled anesthetics
- If there is room on the ID plate, also include: Name, Birthday, and ICE medical contact info (in case of emergency)
Emergency Care Resources
Click the buttons below to read more about Duchenne muscular dystrophy emergency care protocol as created by Parent Project Muscular Dystrophy (PPMD) in the United States.
Our friends at the Children’s Hospital of Eastern Ontario (CHEO) have shared their helpful video on how to administer a hydrocortisone injection:
Order Emergency Care Cards
The following emergency care resources are available at no cost to Duchenne families across Canada; however, quantity limits are set to help us manage the cost associated with producing and distributing such materials. If you have additional quantity needs or to inquire about international shipping, please call us or send us an email.
While Defeat Duchenne Canada is happy to provide these resources free of charge, we always appreciate your donations to support our mission to defeat Duchenne. Click here to make a gift.
This simple yet vital card for your wallet will help you talk to doctors, nurses, and hospital administration if your child suddenly becomes sick or injured. Available in English and French.
Tearproof Information Card
The information card is larger, tearproof, and waterproof. It includes a chain for easy attachment to backpacks, scooters, strollers, wheelchairs, and other mobility devices. The card is two-sided; one emphasizes essential information for families and emergency contact information, and the other is directed towards healthcare professionals/first responders taking care of you or your child. Available in English and French.