Sickle cell anemia is a genetic blood disorder that occurs when a patient has abnormal hemoglobin on their red blood cell called Hemoglobin S (normal hemoglobin type is A).
Hemoglobin S is very sensitive to low oxygen level. When a red blood cell with hemoglobin S experiences a decrease is oxygen it causes the red blood cell to change it’s shape from a round, smooth shape to a stiff, sickle-shaped, and sticky shape.
This change of shape in the red blood cell leads it to occlude circulation within the vessel, which can lead to various types of sickle cell crises (vaso-occlusive, spleen sequestration, hyperhemolyic, and aplastic crisis).
Sickle cell anemia is an autosomal recessive disorder that is homozygous. This means that both parents will each give their offspring one abnormal hemoglobin gene (Hemoglobin S) to make their child have hemoglobin SS. The parents are typically carriers of the disease without symptoms. The parents are heterozygous in that they each have sickle cell trait (in other words they each have hemoglobin AS), but not the actual disease.
African-Americans are most commonly affected with this disease. According to CDC.gov, 1 in 12 African-Americans carry the sickle cell trait. In addition, Middle Easter, Asian, Caribbean, and East Mediterranean patients are at risk as well.
Many patients with sickle cell anemia will have symptoms of anemia, but will not manifest major signs and symptoms until a sickling episode occurs. Factors that can cause a sickle cell crisis include: significant blood loss, illness, climbing or flying to high altitudes, stress, dehydration, fever, strenuous exercise, or extreme cold temperatures.
Sickle cell crises vary among patients with some patients experiencing random episodes with others experience them frequently. Sickle cell anemia is typically discovered in the young pediatric patient (age 6 months and onward). These patients will present with swelling of the hands and feet (hand-foot syndrome or Dactylitis), fever, and be extremely fussy.
Patients who have sickle cell anemia are at risk for the following complications: acute chest syndrome, stroke, splenomegaly, infection, leg ulcers (mainly older children), anemia, vision changes, gallstones etc.
Nursing interventions for sickle cell anemia include: educating the patient or parents about the factors that lead to sickle cell crisis, maintain hydration, administering oxygen, pain control, bedrest, prevent infection and assessing vaccination history of patient, elevating swollen extremities, removing occlusive clothing or objects etc.
A medication used to treat sickle cell anemia is Hydroxyurea, which helps make fetal hemoglobin. This will decrease sickling episodes and the need for blood transfusions. However, a low white blood cell count is a side effect of this medication.
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