Duchenne

Emergency information – for parents of boys with Duchenne MD

(Parent Project Muscular Dystrophy)

Name: ___________________________________________________________

Date of birth: ______________________________________________________

Emergency contact: ______________________________________________

GP name: ______________________________________________________

GP contact: ____________________________________________________

If presenting at an emergency department, contact the neurology/neuromuscular team and respiratory team at: ________________________________________________________________

as soon as possible on: _____________________________________________________________

LEG FRACTURE:

  • 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 or difficulty breathing and 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).

RESPIRATORY CARE:

  • Risk: Respiratory failure. Please only give oxygen with close monitoring of CO2 levels; breathing may need to be supported (with BiPAP, for example)..
  • If oxygen levels are low, assisted coughing (with cough assist machine or Amby bag) may help.
  • Take your equipment (cough assist, BiPAP, etc.) with you to the hospital/emergency room (ER); alert your neuromuscular team that you are going to ER/hospital.

GENERAL RECOMMENDATIONS AND PRECAUTIONS:

  • Keep immunisations 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 AND on the wheelchair/scooter.

ANAESTHETIC PRECAUTIONS:

  • Avoid inhaled anaesthesia.
  • IV anaesthesia is considered to be safe (with close monitoring).
  • People with Duchenne should NOT receive succinylcholine.
  • Local anaesthetics and nitrous oxide are safe for minor dental procedures.

IF VOMITING AND/OR UNABLE TO TAKE DAILY CORTICOSTEROIDS FOR 24 HOURS:

  • Go to a hospital emergency room; bring the PJ Nicholoff Steroid Protocol (ParentProjectMD.org/PJ).
  • Request substitute IV corticosteroid until oral medications are tolerated (6 mg of deflazacort equals 5 mg of prednisone).
  • Remind clinicals that high liver enzymes (AST/ALT) are normal for people with Duchenne MD.