Sickle Cell Anemia: Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by inherited abnormal hemoglobin (an oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells. The sickled red blood cells are fragile and prone to rupture.
When the number of red blood cells decreases from rupture (hemolysis), the result is anemia. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain.
The disorder mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In the UK, sickle cell disorders are most commonly seen in African and Caribbean people.
Because of the risk of the potentially life-threatening complications associated with sickle cell anaemia, it's important that you're aware of any signs or symptoms that your child’s health has suddenly worsened. Signs and symptoms to look out for are:
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fever (high temperature) of 38C (100.4F) or above
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severe pain that develops during a sickle cell crisis that can't be controlled using over-the-counter painkillers
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breathing difficulties
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severe abdominal pain or swelling
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severe headache, stiff neck or dizziness
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changes in mental state, such as appearing confused or drowsy
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episodes of priapism (*see below) that last longer than two hours
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seizures (fits)
(*Priapism is the medical term that describes a persistent and painful erection of the penis).
All babies born in the UK are tested for sickle cell anaemia as part of the heel prick screening test that's carried out by the midwife.
Contact your doctor immediately if your child develops any of the above symptoms.
