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Dear Dr Ombeva,
Thank you for the article on sickle cell disease. I come from a family of sicklers, and my two siblings have the disease. I would like to know more about carriers of sickle cell. Are they the same with those who have sickle cell itself?
Sophie
Dear Sophie,
Thank you for your question. The disease of sickle cell is different from the trait. Sickle cell trait refers to a condition in which a person has one abnormal allele of the hemoglobin beta gene, but does not display the severe symptoms of sickle cell disease which occur in a person who has two copies of that allele.
Normal haemoglobin is called haemoglobin A, but people with sickle cell disease have only haemoglobin S, which turns normal, round red blood cells into abnormally curved (sickle) shapes.
Normally, a person inherits two copies of the gene that produces beta-globin, a protein needed to produce normal haemoglobin (hemoglobin A, genotype AA). If your child has the sickle cell trait, he does not show any signs of sickle cell disease, and is a carrier for sickle cell gene (allele). Normally, adult humans have Haemoglobin A (considered AA), those with the trait have AS, while sicklers have SS.
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. The blood test to confirm disease or trait is called haemoglobin electrophoresis. Sickle cell trait is protective against acquisition of malaria. The trait causes significantly fewer deaths due to malaria, especially due to Plasmodium falciparum.
In some cases, athletes with sickle cell trait do not achieve the same level of performance as elite athletes with normal hemoglobin AA, and must together with their instructors be aware of the dangers of the condition during extraneous exercises especially in hot and dehydrated conditions
— Dr Ombeva Malande is a paediatrics and child health expert
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