Sickle cell disease
It is associated with inherited defect in the Hb synthesis leading to formation of Hb S instead of Hb A.
- Sickle cell disease (severe type).
- Sickle cell trait (asymptomatic type).
Clinical feature of sickle cell disease:
The main clinical manifestation the patients may have are sickle cell crises:
- 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.
- Splenic sequestration crises.
- Hyperhemolytic crises (G6PD deficiency +sickle cell disease).
- Aplastic crises.
– In sickle cell trait there are no clinical symptoms.
- Assisting the physician with the treatment.
- 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.
- Genetic counseling is necessary.
Treatment consist of supportive measures as:
- blood transfusion when necessary and protection against infection .
- Treatment during crises includes codeine and aspirin to relieve pain and maintenance of hydration .
- Antibiotics may be necessary if bacterial infection is present.
- Oxygen is given if the child has symptoms of hypoxia.
In older children autosplenectomy may occur as a result of repeated thrombosis.