Sickle Cell Anemia

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nurse alert

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a common term used in describing an abnormal CBC is shift to the left, which refers to the presence of immature neutrophils in the peripheral blood from hyperfunction of the bone marrow, as seen during a bacterial infection.

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anemia

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the term anemia describes a condition in which the number of red blood cells (RBCs0 or the hemoglobin (Hgb or Hb) concentration is reduced below normal values for age. As a result of this decrease, the oxygen-carring capacity of the blood is diminished, causing a reduction in the oxygen available to the tissues.

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anemia

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anemia is the most common hematologic disorder of infancy and childhood and is NOT a disease itself but an indication or manifestation of an underlying pathologic process.

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classification: anemias

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anemias are classified in relation to (1) etiology or physiology, manifested by erythrocyte and/or Hgb depletion, and (2) morphology, the characteristic changes in RBC size, shape, and color. For example, anemia with reduced Hgb concentration may be caused by a dietary depletion of iron, and the principal intervention is replenishing iron stores.

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consequences of anemia

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The basic physiologic defect caused by anemia is a decrease in the oxygen-carrying capacity of blood and consequently a reduction in the amount of oxygen available to the cells. When the anemia has developed slowly, the child usually adapts to the declining Hgb level.

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consequences of anemia

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growth retardation, resulting from decreased cellular metabolism and coexisting anorexia, is a common finding in chronic severe anemia and is frequently accompanied by delayed sexual maturation in the older child.

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diagnostic evaluation

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In general, anemia may be suspected from findings on the history and physical exam, such as lack of energy, easy fatigability, and pallor; however, unless the anemia is severe, the first clue to the disorder may be alterations in the CBC, such as decreased RBCs, and decreased Hgb and hematocrit (Hct) levels.

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therapeutic management

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The objective of medical management is to reverse the anemia by treating the underlying cause and to make up for any deficiency of blood, blood component, or substance the blood needs for normal functioning. For example, blood or blood cells are replaced after hemorrhage; in nutritional anemias, the specific deficiency is replaced.

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Nurse Alert - the following are suggested explanations for teaching children about blood components:

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Red blood cells--carry the oxygen you breathe from your lungs to all parts of your body
White blood cells--help keep germs from causing infection
Platelets--small parts of cells that help make bleeding stop; platelets help your body stop bleeding by forming a clot (scab) over the hurt area.
Plasma--the liquid portion of blood; has clotting factors that help make bleeding stop.

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sickle cell anemia

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sickle cell anemia (SCA) is one of a group of diseases collectively termed hemoglobinopathies, in which normal adult hemoglobin (hemoglobin A [HbA]) is partly or completely replaced by abnormal sickle hemoglobin (HbS). Sickle cell disease (SCD) includes all those hereditary disorders whose clinical, hematologic and pathologic features are related to the presence of HbS. Even though SCD is sometimes used to refer to SCA, this use is incorrect. Other correct terms for SCA are SS and homozygous sickle cell disease.