A 4-month-old with significant head lag meets the criteria for floppy infant syndrome. A diagnosis of progressive infantile spinal muscular atrophy (Werdnig-Hoffmann disease) is made. What should be included in the nursing care for this child?
a. Infant stimulation program
b. Stretching exercises to decrease contractures
c. Limited physical contact to minimize seizures
d. Encouraging parents to have additional children
ANS: A
Werdnig-Hoffmann disease (spinal muscular atrophy type 1) is the most common paralytic form of floppy infant syndrome (congenital hypotonia). An infant stimulation program is essential. Frequent position changes, including changes in environment, provide the child with more physical contacts. Verbal, tactile, and auditory stimulation are also included. Contractures do not occur because of muscular atrophy. Sensation is normal in children with this disorder. Frequent touch is necessary as part of the stimulation. Werdnig-Hoffmann disease is inherited as an autosomal recessive trait. Parents should be referred for genetic counseling.
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