Emergency Care

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
  • Shock
  • Confusion or coma
  • Dehydration
  • Dizziness or light-headedness
  • Fatigue
  • Flank pain (pain in one side of the body between the upper belly area (abdomen) and the back.)
  • Headache
  • High fever
  • Loss of appetite
  • Loss of consciousness
  • Low blood pressure
  • Nausea
  • Profound weakness
  • Rapid heart rate
  • Rapid breathing
  • Slow, sluggish movement
  • Unusual and excessive sweating on face or palms
  • Vomiting

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