RML MAINS 2025
Medical & Surgical Nursing
Hard
A client with amyotrophic lateral sclerosis (ALS) is experiencing respiratory distress. What is the nurse's priority action?
Appeared in: RML MAINS 2025
Explanation
- The primary cause of respiratory distress in ALS is the progressive weakness of the diaphragm and intercostal muscles, leading to alveolar hypoventilation.
- Non-invasive ventilation (NIV), such as Bi-level Positive Airway Pressure (BiPAP), is the first-line treatment for respiratory insufficiency in ALS.
- NIV provides positive pressure to assist with inspiration and expiration, reducing the work of breathing, improving gas exchange (clearing CO2 and improving O2), and alleviating symptoms of distress.
- Initiating NIV is a priority action to stabilize the patient and prevent the immediate need for invasive mechanical ventilation (intubation).
Why Other Options Were Wrong
- Option A: Bronchodilators treat bronchospasm (airway narrowing), which is not the cause of respiratory distress in ALS. The problem in ALS is muscle weakness, not airway constriction.
- Option B: A patient in acute respiratory distress from ALS lacks the muscle strength to perform deep breathing exercises effectively. This action is not therapeutic in an emergency and delays definitive treatment.
- Option D: Administering a sedative is dangerous and strictly contraindicated. Sedatives depress the central nervous system and the respiratory drive, which would worsen the existing hypoventilation and could precipitate respiratory arrest.
Related Visual
Clinical Relevance
- Nursing practice connection: Safe nursing care depends on performing Priority nursing intervention for respiratory distress in a patient with Amyotrophic Lateral Sclerosis (ALS) in the correct sequence, documenting the action clearly, and monitoring for the expected response.
- Recognizing respiratory failure is a critical nursing skill in managing ALS patients. Early signs include morning headaches (due to overnight hypercapnia), fatigue, and shortness of breath.
- Nurses are pivotal in educating patients and families about NIV, including its benefits, proper use, and maintenance. This improves compliance and quality of life.
- What if? If the patient refused NIV due to anxiety or claustrophobia, the nurse's priority would be to provide reassurance, explain the benefits, try different mask types for comfort, and consider a low-dose anxiolytic only under strict monitoring and physician order, as NIV remains the essential treatment. The goal is to facilitate acceptance of the life-sustaining therapy.
How to Approach the Question
- First, identify the core patient diagnosis: Amyotrophic Lateral Sclerosis (ALS).
- Recall the primary pathophysiology of ALS: it's a progressive motor neuron disease causing muscle weakness.
- Connect the diagnosis to the presenting problem: Respiratory distress in ALS is caused by weakness of the respiratory muscles (diaphragm), not an airway obstruction or lung disease itself.
- Evaluate each option based on this pathophysiology. Ask: 'Does this intervention address weak respiratory muscles?'
- Eliminate options that don't fit: Bronchodilators treat airway size, not muscle strength. Deep breathing requires muscle strength the patient lacks. Sedatives would further weaken the respiratory effort.
- Select the option that directly supports the failing system: Non-invasive ventilation provides mechanical support to compensate for the weak muscles, making it the correct priority action.
Concept Tested & Keywords
- Concept Tested: Priority nursing intervention for respiratory distress in a patient with Amyotrophic Lateral Sclerosis (ALS).
- Stem keywords: amyotrophic lateral sclerosis (ALS), respiratory distress, priority action
- Lead-in keywords: priority action
- Clinical cues: Patient has ALS, a neurodegenerative disease causing muscle weakness.
- Clinical cues: Patient is in active respiratory distress, indicating a life-threatening situation.
Question ID
QHaYgS-oOvyR9lX4wiXcwq
Reference Book
E6 Medicine Davidson Principles Practice 24e p. 222-224
E6 Nursing Brunner Adult Health 3SA Vol 2 Part 2 p. 138-140
Practise the full RML MAINS 2025
Attempt every question from this paper in a timed mock, then review the full solution for each one.
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