Sickle Cell Disease and Thalassemia

The nurse is explaining why deferasirox for thalassemia is being added to a patient's oral medication regimen. Which patient statement demonstrates the patient's need for further education?

"Deferasirox will prevent me from having frequent crises." Deferasirox is a medication that affects iron in the blood and should be given simultaneously with blood transfusions. Hydroxyurea will prevent a patient from having sickle cell crises. The patient has confused the effects of the two drugs, which indicates a need for further patient education.

Which nursing intervention has the highest priority for the nurse caring for a patient experiencing a sickle cell crisis?

Administer intravenous fluids. Hydration is important to decrease blood viscosity and prevent renal failure. This is the highest priority at this point in time.

A patient with sickle cell disease is admitted to the hospital for the second time in 4 months, has an oral temperature of 100.9° F, and is experiencing severe pain. Hydroxyurea is being added to the patient's medication regimen. Which patient outcome shows this medication is effective?

The patient experiences a reduced number of sickle cell crises. Hydroxyurea is the only drug shown to be clinically beneficial for antisickling. This drug increases the production of hemoglobin F (fetal hemoglobin), which is accompanied by a reduction in hemolysis, an increase in hemoglobin concentration, and a decrease in sickled cells and painful crises. The number of hospital admissions will be reduced if hydroxyurea is effective.

The nurse is receiving a report on a patient with sickle cell disease being admitted from the emergency department. Which question by the nurse exhibits an awareness of the primary symptom of the disease?

"When did the patient last receive pain medications?" Pain is the most common symptom of sickle cell disease. Therefore the nurse should know the last time pain medication was given.

The nurse is caring for an older adult patient who has been admitted with abdominal distension and has a history of sickle cell anemia. Which findings would the nurse expect to see on an abdominal x-ray report?

Small to nonexistent spleen The spleen in a sickle cell patient becomes infarcted, dysfunctional, and small because of repeated scarring. An abdominal x-ray may reveal a very small spleen in a patient with sickle cell disease.

A nurse is caring for a patient with sickle cell disease. The nurse realizes the patient's hypoxemia and dehydration are primarily caused by which characteristic of sickle cell disease?

Vessel occlusion Vessel occlusion occurs because sickled erythrocytes are prevented from passing through the capillaries.