1. What additional information is needed to determine which “type” of ALS PG has?
A. Genetic testing
B. Family history
C. Results of magnetic resonance imaging
D. Concurrent medications
E. Unsure
2. Since diagnosis a few months ago, PG finds herself crying often and she has lost interest in activities she used to enjoy. She is also very stressed because she feels as though she cannot concentrate and get things done around her house. PG is most likely suffering from which of the following conditions?
A. Anxiety
B. Depression
C. Pseudobulbar affect (PBA)
D. Dementia
E. Unsure
3. The decision is made to start riluzole 50 mg twice daily. Which counseling point is important to discuss with PG?
A. Riluzole, in rare cases, can cause interstitial lung disease. Notify your doctor immediately if you notice your asthma worsening.
B. Riluzole can cause acute kidney injury. Notify your doctor if you experience decreased urine output, flank pain, dark urine, or swelling in the extremities.
C. Riluzole can cause new or worsening hypertension. Begin taking your blood pressure in the morning before breakfast.
D. Riluzole clearance is decreased in Hispanic patients. Notify your doctor if you experience any adverse effects from riluzole such as nausea or worsening weakness.
E. Unsure
4. PG usually picks up her prescriptions, but her husband is in the pharmacy today to pick up her riluzole and other medications. You inquire how she has been doing and her husband notes that she has not felt well and has been getting sick a lot in the past month. What should you recommend to PG's husband?
A. Start over-the-counter echinacea: ALS patients commonly get sick because of poor nutrition, so PG should begin taking a supplement for immune health
B. Follow up with PG's neurologist: severe neutropenia has been reported within the first few months of riluzole therapy, so she needs blood work
C. Request an antibiotic: PG likely has an infection and should be treated promptly
D. Start antiviral prophylaxis: ALS patients are at increased risk for upper respiratory tract infections
E. Unsure
5. KN was started on amitriptyline 50 mg at bedtime for insomnia and is now complaining of frequent choking episodes. She states it feels like she also has phlegm in her throat that she cannot cough up. She also feels as though her drooling is a little worse recently. What would be an appropriate recommendation to her ALS care team?
A. Consider adding atropine 0.1% ophthalmic drops (1-2 drops sublingually every 4-6 hours as needed)
B. Consider increasing amitriptyline to 100 mg at bedtime and placing a humidifier in her bedroom
C. Consider discontinuing amitriptyline and refer to her pulmonologist
D. Consider botulinum toxin injections into her salivary glands
E. Unsure
6. In addition to age and side effects, what other factors or risks are important considerations when deciding whether to continue or increase the dose of KN's amitriptyline?
A. Serotonin syndrome when amitriptyline is used in combination with baclofen
B. Increased amitriptyline exposure when amitriptyline is used in combination with dextromethorphan/quinidine
C. Decreased amitriptyline exposure when amitriptyline is used in combination with riluzole
D. Increased risk for seizures when amitriptyline is used in ALS patients
E. Unsure
7. The decision has been made to start edaravone. Which of the following is the recommended dosing schedule?
A. Initially: 60 mg intravenously (IV) once daily for 14 days followed by a 14-day drug-free period; subsequent cycles: 14-day drug period followed by a 10-day drug-free period
B. Initially: 60 mg IV once daily for 14 days followed by a 10-day drug-free period; subsequent cycles: 10-day drug period followed by a 14-day drug-free period
C. Initially: 60 mg IV once daily for 14 days followed by a 14-day drug-free period; subsequent cycles: 10-day drug period within a 14-day period followed by a 14-day drug-free period
D. Initially: 60 mg IV once daily for 14 days followed by a 10-day drug-free period; subsequent cycles: 10-day drug period within a 14-day period followed by a 10-day drug-free period
E. Unsure
8. Which of the following pieces of information was not provided but is needed prior to dispensing edaravone for infusion?
A. Kidney function
B. Liver function
C. Drug allergies
D. Respiratory function
E. Unsure
9. BT started edaravone and has been taking it for approximately 4 months. He does not believe it is effective because he is still tired every day. What counseling point should BT know?
A. Edaravone takes up to 6 months to become effective, so BT should continue it for at least 6 months to determine efficacy
B. Edaravone does not work effectively without concurrently taking riluzole; ensure BT is also taking riluzole daily
C. Edaravone's drug-free periods can be shortened to 5 days to increase efficacy in patients with no perceived benefit within the first few months
D. Edaravone is a disease-modifying therapy, meaning its goal is to slow disease progression, not to treat current symptoms
E. Unsure
10. BT has been doing well on his medications and has not had any symptom progression for approximately 3 months. He questions whether he actually has ALS and whether he needs to seek another opinion. What do you tell BT?
A. Progression has stopped because of the edaravone and riluzole
B. Patients with ALS can sometimes plateau and have periods of little to no disease progression
C. ALS always progresses despite disease-modifying therapy so he should seek another opinion
D. He should get genetic testing to confirm he has ALS
E. Unsure
Evaluation Questions
11. How confident are you in your decision about treatment for PG in the case above?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident
12. How confident are you in your decision about treatment for KN in the case above?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident
13. How confident are you in your decision about treatment for BT in the case above?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident
