NCLEX RN Practice Question # 816
NCLEX Examination.
Practice Question # 816.
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SICKLE CELL ANEMIA
Description
- Sickle cell anemia constitutes a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S.
- It is caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain.
- Risk factors include having parents heterozygous for hemoglobin S or being of African- American descent.
- Hemoglobin S is sensitive to changes in the oxygen content of the red blood cell.
- Insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow
Sickle Cell Crisis
- Vaso-Occlusive Crisis:
Caused by stasis of blood with clumping of cells in the microcirculation, ischemia, and infarction
Manifestations: Fever; painful swelling of hands, feet, and joints; and abdominal pain
- Splenic Sequestration:
Caused by pooling and clumping of blood in the spleen (hypersplenism)
Manifestations: Profound anemia, hypovolemia, and shock
- Aplastic Crisis
Caused by diminished production and increased destruction of red blood cells, triggered by viral infection or depletion of folic acid
Manifestations: Profound anemia and pallor
Interventions:
Maintain adequate hydration and blood flow through oral and intravenously administered
fluids; electrolyte replacement is also provided as needed.
Administer oxygen and blood transfusions as prescribed to increase tissue perfusion; exchange
transfusions, which reduce the number of circulating sickle cells and the risk of complications,
may also be prescribed.