Sickle Cell Trait

Sickle Cell Trait

Much media attention has been given to the profile of someone who is likely to carry the sickle cell gene. Basically, each person inherits a gene from each parent that characterizes the behaviour of their haemoglobin. Haemoglobin is the blood’s oxygen transport system. If two healthy genes, known as Haemoglobin A (HbA) are inherited from the parents, haemoglobin functions as it should, carrying oxygen to the muscles that demand it without any complications of sickling, or shape shifting. Two haemoglobin A genes are usually referred to as HbAA.

If, on the other hand, a person inherits one healthy Haemoglobin A gene and one sickle gene, they become a carrier or are said to have the Sickle Cell Trait. This means that they are able to go about their daily routines without frequent hospitalizations from medical crises. There is much debate about the “sudden death syndrome” in people with Sickle Cell Trait who exercise violently on mountain tops where the air is thin. This myth has held sway for over three decades. However, the simple fact is that Sickle Cell Trait can never be considered as the cause of death. As the leading authority on Sickle Cell Disorders, Dr Konotey-Ahulu explains, blaming Sickle Cell Trait in cases of sudden death is wrong; a person who tests positive for sickle cell will not necessarily have the disease. An extra test, electrophoresis, will show conclusively whether the person has the trait or the disease.

Despite the media attention, there are still some myths surrounding the profile of someone with the Sickle Cell Trait. Generally speaking, the traits of Haemoglobin S, Haemoglobin C or Haemoglobin Beta Thalassaemia are well documented. However, there are other forms of haemoglobin that present the same complications when they are inherited with another sickle trait. These include Haemoglobin D Punjab, Haemoglobin O Arab, Haemoglobin Lepore and Haemoglobin E. People do not necessarily need to be from the Punjab, they don’t need to be Arabian or come from Lepore to carry those haemoglobin genes. Anyone from any racial background could carry a sickle haemoglobin gene, these are names given to categorize the different kinds of sickle trait; much like Neapolitan ice cream doesn’t necessarily come from Naples, it is a generic term that describes that kind of icecream so that someone who has it knows what characteristics to expect.

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Carrying the trait should not be considered as having the disease. People with the trait can and do live perfectly healthy lives.