1. SP was diagnosed with HbSS at the age of 5 years old. HbSS is one of two genotypes that are associated with severe complications in SCD. Which of the following best represents the other genotype that is associated with severe complications in SCD?
Click here for Patient Case for Questions 1-7

2. SP has been noncompliant with use of hydroxyurea. Which of the following does not represent a common reason for nonadherence of hydroxyurea among sickle cell patients?

3. SP has had 10 ED visits and 6 hospitalizations within the last year. Upon discharge from this hospitalization, his hematologist has convinced him to restart use of hydroxyurea to reduce the frequency of pain crises and risk of complications. The patient was restarted on hydroxyurea 500 mg by mouth twice daily 2 weeks ago and presents to the clinic today for a follow-up visit. SP endorses that he has remained compliant with use and has taken hydroxyurea as directed most of the time. He reports no signs or symptom of toxicity. His labs are reported as: white blood cell (WBC) 5.1, neutrophils 3500 mm3, Hb 8.2 g/dL, hematocrit 23.2, PLT 133, reticulocytes 120,000.

Which of the following best represents an appropriate dosing recommendation based on the laboratory values reported?

4. During this follow-up visit, the patient explains that he has a girlfriend and had “heard that hydroxyurea can be harmful to babies.” He endorses use of condoms during sexual intercourse and wanted to know if there was anything else that he should be doing to prevent pregnancy. Which of the following best represents information that should be provided to the patient pertaining to pregnancy prevention during use of hydroxyurea?

5. After receiving guidance on use of contraception while on hydroxyurea, the patient declines to continued use of hydroxyurea. He states that he and his girlfriend plan on having children in the near future and admits to difficulty remembering to take it twice daily. Which of the following represents the best therapeutic alternative that could be used in place of hydroxyurea in this patient?

6. SP has arrived at the clinic today to receive his first dose of crizanlizumab. During the infusion, the nurse reports that the patient experienced pruritis and nausea during the infusion. The nurse slowed the rate of the infusion and administered diphenhydramine to relieve itching. Which of the following drugs should not be used to manage symptoms associated with crizanlizumab-related infusion reactions?

7. In the SUSTAIN trial, which of the following population of patients had the most clinical benefit with use of crizanlizumab?

8. Patient Case for Questions 8-10
PA is a 32-year-old female patient who presents to the clinic today for a follow-up visit following her recent hospitalization for symptomatic anemia. She has a history of HbSS (diagnosed at 4 years old), avascular bone necrosis, acute chest syndrome, chronic anemia, depression, and seizures. She has used hydroxyurea for the past 15 years and has required monthly blood transfusions for the last 2 years. Her other medications include fluoxetine 20 mg by mouth daily and phenytoin 100 mg by mouth 3 times daily.

Which of the following medications would be the best option to use in addition to hydroxyurea to reduce the incidence of anemia and the need for blood transfusions in this patient?

9. Which of the following best represents the correct drug name and recommended starting dose that could be initiated in addition to hydroxyurea to reduce the incidence of anemia and frequency of blood transfusions in this patient?

10. Which of the following should be monitored to evaluate efficacy in reducing hemolysis in this patient?

Evaluation Questions

11. How confident are in your treatment choice for PA in question #8-10 above?

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