Angelite Nurses: Muscular Dystrophy

Muscular Dystrophy

General information

A group of muscular diseases in children characterized by progressive muscle weakness and deformity
Genetic in origin; biochemical defect is suspected
Types

Pseudohypertrophic (Duchenne type): most frequent type

X-linked recessive
affects only boys
usually manifests in first 4 years

Facioscapulohumeral

autosomal dominant
mild form, with weakness of facial and shoulder girdle muscles
onset usually in adolescence

Limb girdle

autosomal recessive
affects boys and girls
onset usually in adolescence

Congenital

autosomal recessive
onset in utero

Myotonic

autosomal dominant
more common in boys
onset in infancy or childhood, or adult onset
prognosis in childhood form is guarded

Disease causes progressive disability throughout childhood; most children with Duchenne's muscular dystrophy are confined to a wheelchair by age 8-10 years.
Death occurs by age 20 in 75% of clients with Duchenne's muscular dystrophy.

Assessment findings (Duchenne type)

Pelvic girdle weakness is early sign (child waddles and falls)
Gower's sign (child uses hands to push up from the floor)
Scoliosis (from weakness of shoulder girdle)
Contractures and hypertrophy of muscles
Diagnostic tests

Muscle biopsy reveals histologic changes: degeneration of muscle fibers and replacement of fibers with fat.
EMG shows decrease in amplitude and duration of potentials.

Serum enzymes increased, especially CPK

Nursing interventions

Prepare child for EMG and muscle biopsy.
Maintain function at optimal level; keep child as active and independent as possible.
Plan diet to prevent obesity.
Continually evaluate capabilities.
Support child and parents and provide information about availability of community agencies and support groups.

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