Muscular Dystrophy

  1. General information
    1. A group of muscular diseases in children characterized by progressive muscle weakness and deformity
    2. Genetic in origin; biochemical defect is suspected
    3. Types
      1. Pseudohypertrophic (Duchenne type): most frequent type
        1. X-linked recessive
        2. affects only boys
        3. usually manifests in first 4 years
      2. Facioscapulohumeral
        1. autosomal dominant
        2. mild form, with weakness of facial and shoulder girdle muscles
        3. onset usually in adolescence
      3. Limb girdle
        1. autosomal recessive
        2. affects boys and girls
        3. onset usually in adolescence
      4. Congenital
        1. autosomal recessive
        2. onset in utero
      5. Myotonic
        1. autosomal dominant
        2. more common in boys
        3. onset in infancy or childhood, or adult onset
        4. prognosis in childhood form is guarded
    4. Disease causes progressive disability throughout childhood; most children with Duchenne's muscular dystrophy are confined to a wheelchair by age 8-10 years.
    5. Death occurs by age 20 in 75% of clients with Duchenne's muscular dystrophy.
  2. Assessment findings (Duchenne type)
    1. Pelvic girdle weakness is early sign (child waddles and falls)
    2. Gower's sign (child uses hands to push up from the floor)
    3. Scoliosis (from weakness of shoulder girdle)
    4. Contractures and hypertrophy of muscles
    5. Diagnostic tests
      1. Muscle biopsy reveals histologic changes: degeneration of muscle fibers and replacement of fibers with fat.
      2. EMG shows decrease in amplitude and duration of potentials.
    6. Serum enzymes increased, especially CPK
  3. Nursing interventions
    1. Prepare child for EMG and muscle biopsy.
    2. Maintain function at optimal level; keep child as active and independent as possible.
    3. Plan diet to prevent obesity.
    4. Continually evaluate capabilities.
    5. Support child and parents and provide information about availability of community agencies and support groups.

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