Chapter 63: Amyotrophic Lateral Sclerosis (ALS) Flashcards
A 58-year-old male presents to the clinic with complaints of muscle weakness in his right hand, difficulty gripping objects, and frequent muscle twitching. He reports occasional choking while eating and mild slurred speech. Based on these symptoms, which diagnostic test is most appropriate to confirm amyotrophic lateral sclerosis (ALS)?
A. MRI of the brain
B. Electromyography (EMG) and nerve conduction studies
C. Muscle biopsy
D. Serum creatine kinase (CK) levels
B. Electromyography (EMG) and nerve conduction studies
Rationale: EMG assesses electrical activity in muscles, identifying motor neuron dysfunction typical of ALS. Nerve conduction studies differentiate ALS from other neuromuscular disorders. MRI rules out other causes, such as multiple sclerosis. Muscle biopsy is not required, and serum CK may be mildly elevated but is nonspecific.
A newly diagnosed ALS patient is prescribed riluzole (Rilutek). Which statement by the patient indicates a need for further teaching?
A. “This medication may help slow the progression of my disease.”
B. “I should take this medication on an empty stomach.”
C. “This medication will cure my ALS if I take it as prescribed.”
D. “I should have my liver function monitored while taking this medication.”
C. “This medication will cure my ALS if I take it as prescribed.”
Rationale: Riluzole slows the progression of ALS but is not a cure. It is taken on an empty stomach to improve absorption, and liver function monitoring is necessary due to hepatotoxicity risks.
Which of the following is the most common cause of death in patients with ALS?
A. Cardiac arrest
B. Renal failure
C. Respiratory failure
D. Sepsis
C. Respiratory failure
Rationale: ALS causes progressive respiratory muscle weakness, leading to respiratory failure, the most common cause of death. Cardiac and renal failure are not primary complications. Sepsis may occur secondary to aspiration pneumonia but is not the leading cause.
Which symptom should the nurse monitor closely in a patient with ALS to prevent aspiration pneumonia?
A. Muscle cramps in the lower extremities
B. Spasticity in the upper extremities
C. Emotional lability
D. Dysphagia
D. Dysphagia
Rationale: Dysphagia increases the risk of aspiration, a major complication in ALS. Muscle cramps and spasticity contribute to mobility issues but do not directly cause aspiration. Emotional lability affects mood but does not impact swallowing.
A 50-year-old male patient with ALS expresses concerns about his declining mobility. The nurse should recommend which intervention to maintain function as long as possible?
A. Strict bed rest to prevent muscle overuse
B. High-intensity weight training to preserve strength
C. Moderate-intensity, endurance-type exercises
D. Passive range-of-motion exercises only
C. Moderate-intensity, endurance-type exercises
Rationale: Moderate endurance exercises help maintain mobility and reduce spasticity without overfatiguing muscles. High-intensity exercise can worsen weakness. Passive ROM is beneficial but insufficient alone.
A patient with ALS develops progressive respiratory distress. Which intervention is the priority?
A. Encourage deep breathing exercises
B. Prepare for mechanical ventilation discussions
C. Position the patient supine to promote comfort
D. Administer a sedative for anxiety
B. Prepare for mechanical ventilation discussions
Rationale: As ALS progresses, respiratory muscles weaken, leading to ventilatory failure. Discussing ventilation early allows the patient to make informed decisions. Deep breathing exercises provide minimal benefit at late stages. The supine position may worsen respiratory distress.
The spouse of an ALS patient reports increased frustration due to caregiving stress. What is the best nursing intervention?
A. Recommend they consider placing the patient in a long-term care facility
B. Suggest respite care services
C. Encourage the spouse to “stay strong” for the patient
D. Teach relaxation techniques for stress management
A. Recommend they consider placing the patient in a long-term care facility
Rationale: Caregiving for ALS is physically and emotionally demanding. Respite care provides temporary relief. While relaxation techniques may help, respite care directly addresses caregiver burnout.
A nurse is educating a newly diagnosed ALS patient and family about the disease. Which statements should be included in the teaching?
(Select all that apply.)
A. “ALS affects motor neurons, leading to progressive muscle weakness.”
B. “Intellectual functioning remains intact.”
C. “Riluzole may extend survival by a few months.”
D. “Respiratory support may become necessary as the disease progresses.”
E. “There are many effective treatment options to cure ALS.”
A. “ALS affects motor neurons, leading to progressive muscle weakness.”
B. “Intellectual functioning remains intact.”
C. “Riluzole may extend survival by a few months.”
D. “Respiratory support may become necessary as the disease progresses.”
Rationale: ALS primarily affects motor neurons, leading to muscle weakness. Cognitive function is preserved, though some may experience mild cognitive impairment. Riluzole modestly extends survival, and respiratory failure is a late-stage complication. No cure exists.
Which of the following are appropriate nursing interventions for a patient with ALS?
(Select all that apply.)
A. Encourage a high-calorie, high-protein diet
B. Implement fall precautions
C. Educate on alternative communication methods
D. Discourage the use of assistive devices to maintain independence
E. Monitor for signs of respiratory decline
A. Encourage a high-calorie, high-protein diet
B. Implement fall precautions
C. Educate on alternative communication methods
E. Monitor for signs of respiratory decline
Rationale: ALS patients need high-calorie, high-protein diets to combat weight loss. Fall precautions are necessary due to weakness. Communication devices help as speech deteriorates. Monitoring respiratory function is critical. Assistive devices should be encouraged to maintain mobility.
A 62-year-old ALS patient is experiencing worsening dysphagia, frequent coughing during meals, and a 10-lb weight loss over the past month. What is the priority nursing intervention?
A. Refer the patient for a swallowing evaluation
B. Encourage the patient to drink more fluids with meals
C. Advise the patient to consume a high-protein diet
D. Educate the caregiver on feeding techniques
A. Refer the patient for a swallowing evaluation
Rationale: Dysphagia increases the risk of aspiration. A swallowing evaluation determines the safest diet consistency. Drinking fluids may worsen aspiration risk. While nutrition education is important, safety takes priority.
A 55-year-old male with ALS is admitted for respiratory distress. His oxygen saturation is 88% on room air, and he is using accessory muscles to breathe. What is the nurse’s priority action?
A. Administer a dose of albuterol via nebulizer
B. Prepare for intubation and mechanical ventilation
C. Obtain arterial blood gases (ABGs)
D. Position the patient upright and apply noninvasive ventilation
D. Position the patient upright and apply noninvasive ventilation
Rationale: Noninvasive ventilation (e.g., BiPAP) can help ALS patients with respiratory muscle weakness avoid immediate intubation. Upright positioning improves lung expansion. Intubation may be needed later, but noninvasive options are preferred initially. Albuterol is not indicated unless bronchospasm is present.
A nurse is assessing a patient with ALS. Which finding is most concerning?
A. Frequent muscle fasciculations in the upper extremities
B. Weak cough effort and shallow respirations
C. Dysarthria and slurred speech
D. Increased emotional lability
B. Weak cough effort and shallow respirations
Rationale: Weak respiratory muscles can lead to respiratory failure, the leading cause of death in ALS. Fasciculations, dysarthria, and emotional lability are expected symptoms but are not immediately life-threatening.
The spouse of a patient with ALS asks about hospice care. Which statement by the nurse is most appropriate?
A. “Hospice is only for patients who are bedridden and near death.”
B. “Hospice can provide comfort care and support as the disease progresses.”
C. “Your spouse is not eligible for hospice since they are still mobile.”
D. “Hospice is only for cancer patients, so we will refer you to palliative care instead.”
B. “Hospice can provide comfort care and support as the disease progresses.”
Rationale: Hospice focuses on symptom management and quality of life for terminal illnesses, including ALS. Patients do not need to be bedridden to qualify.
A patient with ALS is being discharged home with a percutaneous endoscopic gastrostomy (PEG) tube due to dysphagia. Which instruction should the nurse give the caregiver?
A. “Flush the tube with 5 mL of water before and after feeding.”
B. “Administer medications whole through the tube to prevent clogging.”
C. “Elevate the head of the bed to at least 30 degrees during and after feedings.”
D. “Bolus feedings should be given rapidly to prevent aspiration.”
C. “Elevate the head of the bed to at least 30 degrees during and after feedings.”
Rationale: Keeping the head of the bed elevated reduces the risk of aspiration. The tube should be flushed with 30 mL of water, not 5 mL. Medications should be crushed (if allowed) and mixed with water. Feedings should be given slowly to prevent aspiration.
A patient with ALS is prescribed edaravone (Radicava). What is the most important nursing consideration?
A. Monitor for signs of hepatotoxicity
B. Assess for infusion-related hypersensitivity reactions
C. Administer the medication subcutaneously
D. Monitor blood pressure closely after administration
B. Assess for infusion-related hypersensitivity reactions
Rationale: Edaravone is given IV and can cause serious hypersensitivity reactions, including anaphylaxis. Hepatotoxicity is more associated with riluzole.
