Last modified: 26th April 2023
Reasonable to avoid suxamethonium in view of theoretical risk of rhabdomyolysis.
All general anaesthetic agents have been used safely at appropriate doses.
Reducing the substrate available to form propionic acid is the main focus for perioperative care.
Substrates include: protein catabolism due to fasting, certain neuromuscular blocking drugs, NSAIDS, and propofol.
These patients are at risk of QT prolongation.
Propofol has been used safely as a bolus and brief infusion, but prolonged infusion should be used with caution.
Reasonable to avoid suxamethonium in the setting of hypotonia, given the theoretical risk of hyperkalaemia and rhabdomyolysis