Question

Sickle Cell Case Study Read the following case study and answer the questions below. Provide evidence with correct citations to support your answers.

V.M. is a 29 year old African American married man who has sickle cell disease (SCD) marked by frequent episodes of severe pain. His anemia has been managed with multiple transfusions. Six months ago he started showing signs of chronic renal failure. His regular medications are pentoxifylline (Trental), oxycodone-acetaminophen (Percocet), hydroxyurea (Droxia), and folic acid. In the hematology clinic this morning, V.M.’s hemoglobin measured 6.7 g/dL. He received 2 units of packed red blood cells (PRBCs) over 3 hours and went home. He developed dyspnea and shortness of breath approximately 1 hour later, and his wife called 911. The emergency medical system crew initiated oxygen at 8 L per nasal cannula and transported V.M. to the emergency department (ED).

1. What is SCD?

2. True or False: Only African Americans get SCD. Explain your response.

3. Which statement is true about the inheritance pattern of SCD?

a. If V.M.’s wife has sickle cell trait, each child will either have SCD or be a carrier.

b. If V.M.’s wife does not have sickle cell trait, each child has a 50% risk of having SCD.

c. If V.M.’s wife has sickle cell trait, each child will either have SCD or be normal.

d. If V.M. has children, each child will automatically have SCD regardless of his wife’s status.

4. V.M.’s hemoglobin measured 6.7 g/dL. Why is anemia common in patients with SCD?

5. Why is it difficult to crossmatch blood to transfuse V.M.?

6. What role does hydroxyurea (Droxia) play in managing V.M.’s SCD?

Answer 1

1. SCD is Sickle Cell Disease. ie nothing but sickle cell anemia constitutes a group of diseases termed as hemoglobinopathies. In SCD, hemoglobin A is replaced by abnormal sickle shaped hemoglobin S.

2. African Americans are having risk factors to get SCD. So the statement is true. The other risk factors are parents heterogeneous for hemoglobin S.

3.b) If VM's wife does not have sickle cell trait, each child has a 50% risk of having SCD.. This statement is true. Always as a nurse has to inform parents of the hereditary aspects of the disorder.

4. Anemia is very common in patients with SCD. Insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow. It leads to increased red blood cells destruction. So the patients will have profound anemia.