A young boy has just been diagnosed with pseudohypertrophic (Duchenne's) muscular dystrophy. The management plan should include:
a. Recommending genetic counseling.
b. Explaining that the disease is easily treated.
c. Suggesting ways to limit the use of muscles.
d. Assisting the family in finding a nursing facility to provide his care.
ANS: A
Pseudohypertrophic (Duchenne's) muscular dystrophy is inherited as an X-linked recessive gene. Genetic counseling is recommended for parents, female siblings, maternal aunts, and their female offspring. No effective treatment exists at this time for childhood muscular dystrophy. Maintaining optimal function of all muscles for as long as possible is the primary goal. It has been found that children who remain as active as possible are able to avoid wheelchair confinement for a longer time. Assisting the family in finding a nursing facility is inappropriate at the time of diagnosis. When the child becomes increasingly incapacitated, the family may consider home-based care, a skilled nursing facility, or respite care to provide the necessary care.
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