NCLEX RN Practice Question # 816

NCLEX Examination.

Practice Question # 816.

 

nclex examination.

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.

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