I was talking with a woman recently whose son had Duchenne muscular dystrophy. The boy was to have surgery and I wanted to be sure the doctors had discussed possible anesthetic problems with the mother. The woman had no idea what I was talking about and I was appallled. I wondered if her doctors didn't know about the risk of the disorder, Maligmnant Hyperthermia, with Duchenne muscular dystrophy and some of the muscle diseases or they just didn't want to worry the woman. I explained about the disorder and suggested the mother check with the anesthesiologist to be sure he or she knew about Malignant Hyperthermia and the relationship to Duchenne dystrophy. The anesthesiologist should always have a special tray with medications ready in case there is a problem and succinylcholine or halothane must not be used as anesthetic agents.
Children have more problems with Malignant Hyperthermia than adults and the disorder can be fatal if it is not recognized and immediately treated. Sixty percent of patients die if the disorder develops full-blown symptons. Of particular concern are the patients who have Central Core disease because both disorders have the same genetic locus. Central Core disease is in the group of Congenital Myopathies and is easily recognized on muscle biopsuy if the biopsy is done and processed correctly