A preadolescent child with ataxia-telangiectasia is demonstrating an exacerbation of choreoathetosis. What should the nurse do to help this patient?

Question

A) Provide comfort measures.
B) Assist the patient to walk several times a day.
C) Encourage the patient to increase independence.
D) Recommend transferring the patient to a rehabilitation facility.

Answer 1

A
Feedback:
Ataxia-telangiectasia is a primary immunodeficiency disorder that results in progressive cerebellar degeneration. Telangiectasia or red vascular markings appear on the conjunctiva and skin at the flexor creases. Neurologic symptoms caused by the degeneration process can usually be detected in early infancy when developmental milestones are not met. Children develop an awkward gait when they begin to walk. Choreoathetosis or rapid, purposeless movements may develop. Unfortunately, there is no effective treatment, and children with this disorder often die in late adolescence of infection, respiratory failure, or a malignant brain tumor. The nurse should provide comfort measures when caring for this child. The patient may not be able to walk. It is an unrealistic expectation for this child to increase independence. A rehabilitation facility is not going to help with the long-term prognosis of this disorder.