Journal Logo

CE Connection

Sickle cell disease

Where are we now?

Author Information
doi: 10.1097/01.NURSE.0000515478.93433.7a
  • Free

INSTRUCTIONS Sickle cell disease: Where are we now?


  • To take the test online, go to our secure website at
  • On the print form, record your answers in the test answer section of the CE enrollment form on page 35. Each question has only one correct answer. You may make copies of these forms.
  • Complete the registration information and course evaluation. Mail the completed form and registration fee of $12.95 to: Lippincott Williams & Wilkins, CE Group, 74 Brick Blvd., Bldg. 4, Suite 206, Brick, NJ 08723. We will mail your certificate in 4 to 6 weeks. For faster service, include a fax number and we will fax your certificate within 2 business days of receiving your enrollment form.
  • You will receive your CE certificate of earned contact hours and an answer key to review your results.
  • Registration deadline is April 30, 2019.


  • Send two or more tests in any nursing journal published by Lippincott Williams & Wilkins together by mail, and deduct $0.95 from the price of each test.
  • We also offer CE accounts for hospitals and other healthcare facilities on Call 1-800-787-8985 for details.


Lippincott Williams & Wilkins, publisher of Nursing2017 journal, will award 1.0 contact hour for this continuing nursing education activity.

Lippincott Williams & Wilkins is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's Commission on Accreditation.

Lippincott Williams & Wilkins is also an approved provider of continuing nursing education by the District of Columbia, Georgia, and Florida CE Broker #50-1223. This activity is also provider approved by the California Board of Registered Nursing, Provider Number CEP 11749 for 1.0 contact hour.

Your certificate is valid in all states.

Sickle cell disease: Where are we now?

GENERAL PURPOSE: To present an overview of SCD. LEARNING OBJECTIVES/OUTCOMES: After completing this continuing-education activity, you should be able to: 1. Differentiate between SCD and SCT. 2. Describe the pathophysiology of SCD. 3. Outline the management of patients with SCD.

  1. In sickle cell anemia, the patient has inherited one Hb “S” gene plus
    1. another Hb “S” gene.
    2. an Hb “C” gene.
    3. an Hb “D” gene.
  2. A person with SCT
    1. is designated as type HbSS.
    2. has SCD-related health problems.
    3. may pass the trait along to children.
  3. The ancestry of most of those affected by SCD is
    1. Mediterranean.
    2. African.
    3. South American.
  4. Which occurs when RBCs sickle?
    1. RBC deformability increases.
    2. RBC membrane permeability decreases.
    3. Blood viscosity increases.
  5. The lifespan of normal RBCs is
    1. 5 to 9 days.
    2. 10 to 20 days.
    3. 90 to 120 days.
  6. The shortened lifespan of sickle cells results in
    1. iron deficiency anemia.
    2. hemolytic anemia.
    3. sideroblastic anemias.
  7. VO and hemolytic anemia cause
    1. tissue infarction.
    2. metabolic alkalosis.
    3. hemorrhage.
  8. The pathogenesis of VO involves
    1. enhanced fibrinolysis.
    2. platelet activation.
    3. increased circulating anticoagulants.
  9. The pain of VOC is typically described as
    1. intermittent.
    2. gradual onset.
    3. throbbing.
  10. Goals of SCD management include
    1. decreasing circulating HbF.
    2. maximizing tissue perfusion.
    3. maintaining optimal body mass index.
  11. The primary effect of HU therapy is
    1. increased circulating reticulocytes.
    2. decreased RBC size.
    3. increased concentration of HbF.
  12. The nitric oxide released directly from HU metabolism may contribute to
    1. local vasodilation.
    2. decreased RBC adhesiveness.
    3. improved RBC permeability.
  13. Which statement about HU therapy is accurate?
    1. It's as effective as blood transfusion therapy in stroke prevention.
    2. It's FDA-approved for induction of HbF in adults with SCD.
    3. Its therapeutic effects are directly dose-dependent.
  14. Blood transfusions every 3 to 4 weeks
    1. decrease sickled RBCs.
    2. decrease HbA.
    3. increase HbSS.
  15. Patients with SCD are highly vulnerable to infection primarily due to
    1. asplenia.
    2. anemia.
    3. thrombocytopenia.
  16. Families of patients with SCD should be taught that a fever
    1. is a normal response to HU therapy.
    2. should be evaluated within 48 hours.
    3. is a medical emergency.
  17. Triggers for RBC sickling include
    1. normothermia.
    2. hypernatremia.
    3. euvolemia.
  18. Adults with SCD and moyamoya syndrome are at particular risk for which type of stroke?
    1. ischemic
    2. hemorrhagic
    3. cryptogenic
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.