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Managing sickle cell disease

doi: 10.1097/
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INSTRUCTIONS Managing sickle cell disease



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Managing sickle cell disease

GENERAL PURPOSE: To provide the professional nurse with an overview of SCD and its symptoms, diagnosis, and treatment.LEARNING OBJECTIVES: After reading this article and taking this test, you'll be able to: 1. Describe the pathophysiology of SCD.2. Identify symptoms of SCD and crisis. 3. Discuss medical and nursing management of SCD.

1. Typically, the two organs that are the most prone to damage from SCD are the

a. kidneys and spleen.

b. heart and liver.

c. lungs and gallbladder.

2. SCD is a result of

a. trauma to the spleen, which interrupts RBC production.

b. inadequate oxygen transport from the lungs into the RBCs.

c. a mutation in the gene responsible for hemoglobin production.

3. Which is the most common type of SCD?

a. hemoglobin SC disease

b. hemoglobin SS disease

c. hemoglobin SB thalassemia disease

4. The average life span of an individual with hemoglobin SS disease is

a. 45 years.

b. mid-60s.

c. 75 years.

5. A person with mostly hemoglobin S with a small percent of normal hemoglobin A has

a. hemoglobin SS disease.

b. hemoglobin SC disease.

c. hemoglobin SB thalassemia disease.

6. The child with sickle cell trait

a. will probably not exhibit symptoms.

b. may convert to full SCD later in life.

c. has two parents with SCD.

7. Which of the following is a trigger for SCD symptoms?

a. pseudoephedrine

b. folic acid

c. ketorolac

8. The first symptoms of SCD are usually noticed after the age of

a. 4 to 6 weeks.

b. 4 to 6 months.

c. 5 to 6 years.

9. Anemia and jaundice in SCD are caused by

a. deceased oxygen in tissues.

b. splenic sequestration.

c. RBCs breaking apart.

10. Among the most common causes of death in the young child with SCD are

a. septicemia and meningitis.

b. hand-foot syndrome.

c. stroke.

11. Proper management of the patient with SCD should include

a. applying ice to painful extremities.

b. antibiotics for infants to prevent pneumonia.

c. hydroxyurea to reduce pain and inflammation during crisis.

12. Adverse reactions of hydroxyurea include

a. iron toxicity.

b. worsened anemia.

c. increased stickiness of RBCs.

13. Which is currently experimental but has potential as a curative treatment?

a. bone marrow transplant

b. GMI-1070

c. gene therapy

14. Which isn't an indication for blood transfusion?

a. stroke

b. acute pain

c. splenic sequestration

15. Which symptom is least likely to be related to a complication of SCD?

a. swelling of the hands and feet

b. vision changes

c. lower right-sided abdominal pain

16. Which physical activity is best to recommend for an SCD patient?

a. biking

b. jogging

c. swimming

17. Erythema on the chest of a patient with acute chest syndrome may indicate

a. pulmonary embolus.

b. rib infarction.

c. pneumonia.

18. Which drug is especially helpful in managing the inflammatory pain of SCD crisis?

a. ibuprofen

b. opioids

c. ketorolac





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