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Dear Dr Ombeva,
My younger brother was diagnosed with sickle cell disease last year. I am six months into my first pregnancy. I have never had signs of sickle cell disease, but I am afraid I could be having the sickle cell gene or that my children will get sickle cell. I am told it runs in families. How can I know if I have sickle cell, or if my child will be a sickler?
Mary
Dear Mary,
Sickle cell anaemia is an inherited disease of blood, in which red blood cells assume an abnormal, sickle shape. Blood is made of white cells, red cells and platelets. Normally, the red blood cells are disc-shaped and move easily through blood vessels. Red blood cells contain haemoglobin, a protein that carries oxygen from the lungs to the rest of the body.
Sickle cells contain abnormal haemoglobin called sickle haemoglobin (haemoglobin S), which causes the cells to develop a sickle shape. Sickle cells are stiff and sticky, and often block blood flow in the blood vessels of the limbs and other body organs. Blocked blood flow can cause pain and organ damage.
Due to the underlying disease, a sickler is prone to “sickle cell crisis,” and may suffer overwhelming infections due to a non-functional spleen. Some forms of “sickle cell crisis” include severe pain, swollen painful fingers and toes, acute chest syndrome among others.
If you have the sickle cell trait, you don’t show any signs of sickle cell disease, but you are a carrier for sickle cell gene. Note that adult humans have Haemoglobin A (considered AA), while those with the trait have AS, whereas sicklers have SS. A child inherits the disease from both parents. If your spouse is AS too, then some of your children (25 per cent) can become sicklers, 50 per cent will be AS like you while the rest (25 per cent) would be normal (AA).
If one parent has sickle-cell anaemia (SS) and the other has sickle-cell trait then there is a 50 per cent chance of a child’s having sickle-cell disease and a 50 per cent chance of a child’s having sickle-cell trait. When both parents have sickle-cell trait a child has a 25 per cent chance of sickle-cell disease. You can find out if your partner, yourself or your child has sickle cell disease or is a carrier by having the haemoglobin electrophoresis test performed on your blood.
- Dr Ombeva Malande is a paediatrics and child health expert
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