Table 5.

Treatment of Manifestations in Individuals with Emery-Dreifuss Muscular Dystrophy

Manifestation/
Concern		TreatmentConsiderations/Other
Cardiac Specific for cardiac issue in the individual; can incl antiarrhythmic drugs, cardiac pacemaker, implantable cardioverter defibrillator, & both pharmacologic & non-pharmacologic therapy for heart failureHeart transplantation may be necessary in end stages of heart failure; some persons may not be candidates for transplantation due to assoc severe skeletal muscle & respiratory involvement.
Respiratory Use of respiratory aids (respiratory muscle training & assisted coughing techniques, mechanical ventilation) if indicated in late stages
Musculoskeletal
  • Orthopedic surgeries to release Achilles tendons & other contractures or scoliosis as needed
  • Mechanical aids (canes, walkers, orthoses, wheelchairs) as needed to help ambulation
  • PT & stretching exercises to promote mobility & help prevent contractures

PT = physical therapy

From: Emery-Dreifuss Muscular Dystrophy

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