Sickle cell disease treatment and Nursing Role

Sickle cell anemia

Sickle cell disease

It is associated with inherited defect in the Hb synthesis leading to formation of Hb S instead of Hb A.

Types:

  1. Sickle cell disease (severe type).
  2. Sickle cell trait (asymptomatic type).

Clinical feature of sickle cell disease:

The main clinical manifestation the patients may have are sickle cell crises:

  1. Vaso-occlusive crises(painful crises), in which the patients have severe pain in the abdomen, legs or any site in the body . The child also may have severepallor,fever,vomiting and severe flank pain with hematurea or the child manifestconvultion, stiff neck, coma and paralysis.
  2. Splenic sequestration crises.
  3. Hyperhemolytic crises (G6PD deficiency +sickle cell disease).
  4. Aplastic crises.

– In sickle cell trait there are no clinical symptoms.

Nursing role:

  1. Assisting the physician with the treatment.
  2. Educate the child and parents about the disease and advice them to increase fluid intake, to avoid extreme temp.(hot weather, cold weather),to avoid hypoxia and to treat any infection immediately.
  3. Genetic counseling is necessary.

Treatment:

Treatment consist of supportive measures as:

  1. blood transfusion when necessary and protection against infection .
  2. Treatment during crises includes codeine and aspirin to relieve pain and maintenance of hydration .
  3. Antibiotics may be necessary if bacterial infection is present.
  4. Oxygen is given if the child has symptoms of hypoxia.

In older children autosplenectomy may occur as a result of repeated thrombosis.

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Posted in Nursing Care Plans, Nursing Intervention, Pediatrics

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