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RN Nursing · Amyotrophic Lateral Sclerosis · Practice question

A nurse is teaching a client who has amyotrophic lateral sclerosis (ALS) about the end stages of the disease process. Which of the following information should the nurse include in the teaching?

Answer & explanation

Correct: 'You might require a machine to keep your airway open."

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that destroys upper and lower motor neurons, leading to progressive muscle weakness and eventual paralysis. In the end stages of ALS, the respiratory muscles become severely weakened, making it impossible for the client to breathe without assistance. Teaching should therefore include that the client may require mechanical ventilatory support, such as noninvasive positive-pressure ventilation or invasive mechanical ventilation via tracheostomy, to keep the airway open and maintain breathing. The option mentioning nutrition through a central venous access device is inaccurate because ALS clients who require nutritional support typically receive it via a percutaneous endoscopic gastrostomy (PEG) tube, not a central venous catheter, since their primary issue is dysphagia rather than a need for parenteral nutrition. Splints over affected joints during sleep are used earlier in the disease to prevent contractures, not specifically in the end stage. Dexamethasone is a corticosteroid that does not treat muscle atrophy in ALS; there is no corticosteroid-based therapy for ALS-related muscle wasting. The defining feature of end-stage ALS that nurses must teach is progressive respiratory failure requiring ventilatory support, which is the correct answer.

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