Table 4.

Treatment of Manifestations in Individuals with Calpainopathy

  • Passive PT program & stretching exercises instituted early following diagnosis to promote mobility, prolong walking, & slow disease progression by maintaining joint flexibility 1
  • Affected persons usually benefit from strengthening & gentle, 2 low-impact aerobic exercise (swimming, stationary bicycling) w/supervised submaximal effort to ↑ cardiovascular performance, ↑ muscle efficiency, & ↓ muscle fatigue.
  • Additional treatment per PT & OT 3
  • Maintain appropriate weight for height w/nutrition mgmt as needed.
  • Mobility aids for loss of certain motor abilities; canes, walkers, orthotics, & wheelchairs enable affected persons to regain independence.
  • Knee-ankle-foot orthoses while sleeping to prevent contractures
  • Consider need for positioning & seating devices, as scoliosis occurs mainly after wheelchair dependence. 4
  • Surgical intervention as needed for orthopedic complications (foot deformities, scoliosis, Achilles tendon contractures)
  • Scapular fixation may be required for problematic scapular winging.
  • Annual influenza vaccination
  • Prompt treatment for chest & respiratory infections w/ mechanical in-exsufflator when needed 5
  • Nocturnal ventilator assistance (noninvasive ventilation by nasal masks) for those w/nocturnal hypoventilation &/or respiratory failure
  • Respiratory aids for those w/chronic respiratory insufficiency
  • Wheelchair-bound persons may develop weak cough efforts, placing them at risk of atelectasis, pneumonia, progressive mismatch, & respiratory failure.
  • Nocturnal ventilator assistance may be lifesaving in severely affected persons. 6
  • Respiratory aids may be indicated to prolong survival. 7
Cardiac Treatment per cardiology recommendations as needed
Social & family
Social & emotional support to ↑ quality of life, maximize sense of social involvement & productivity, & ↓ social isolation 8
Anticipate & facilitate decision making for affected persons & their families as disease progresses incl:
  • Decisions regarding loss of mobility;
  • Need for assistance w/ADL, medical complications, & end-of-life care. 9

From: Calpainopathy

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