The therapist is performing an airway clearance technique to a 5-month-old child with a diagnosis of aspiration pneumonia. The child has a very weak cough and, although he appears alert, he cannot move. Which of the following disorders of the motor nerve should the therapist suspect?

Question

Quizplus · Accepted Answer

SMA type I, also called Werdnig-Hoffmann disease, is the acute infantile form, which usually presents within the first 6 months of life. In these children, limb weakness develops rapidly, whereas the facial muscles are slower to fail and the extraocular muscles are essentially spared. The result is a child who appears alert and responsive but cannot move. The respiratory effects of SMA type I include weakness of the bulbar, abdominal, and intercostal muscles, which makes feeding difficult and leads to aspiration and a weak, ineffective cough. A weak cough results in recurrent pneumonias and poor airway clearance. Even relatively minor viral infections result in severe airway and ventilator compromise. Without intervention, most infants will die of respiratory insufficiency and infection before reaching 1 year of age.

The Therapist Is Performing an Airway Clearance Technique to a 5-Month-Old