Those who have Sickle Cell Disease (SCD) or others with a family member or friend who suffers from it, are keenly aware of the excruciating pain that comes with this illness. Few, however may be aware that SCD evolved as the body’s way of combating malaria, a health menace that has claimed millions of lives in Africa.
Before discussing the role that Sickle Cell Disease plays in malaria resistance, a brief overview of the disease may be a good place to start.
Sickle Cell Disease is a genetic condition in which the hemoglobin molecule, the molecule that carries oxygen in red blood cells, is affected. The blood cell disorder causes the patient’s blood cells to become abnormally shaped.
Red blood cells are naturally biconcave in shape. This gives them a greater surface area which allows them to absorb oxygen more efficiently. Sickle cells on the other hand, are shaped like crescent moons which causes them to get stuck in the blood vessels and leads to many complications.
According to Hilal Abuzahra M.D., UCR Health Pediatric hospitalist and UC Riverside School of Medicine Assistant Clinical Professor, chronic anemia is a feature of the disease and it happens in 100 percent of cases of those with SCD, and the complications of this condition can get much worse.
It becomes difficult for sickle cells to pass through small blood vessels such as capillaries, which leads to blockage, and can result in ischemia—a loss of blood flow. Ischemia results in chronic pain and pain crisis.
Yellowing of the eyes is also a common symptom of SCD because of the breaking down of blood cells. For someone living with this blood disorder, each day can be a constant struggle. Unfortunately, SCD also shortens one’s life expectancy.
According to the California Department of Public Health, African Americans are more prone to this disease, as they have a one in 14 chance, of carrying the sickle cell trait. Among Hispanics, the chances of carrying the trait are one in 192, and for Whites the prevalence is 1 in 630. The group with the lowest possibility of carrying the SCD trait are East Asians at 1 in 6,072 people.
Why is SCD more common in the African American population? Dr. Abuzahra indicated geographical location plays a key role in this. “If you look at a map” he said, “it makes perfect sense. The population where malaria is endemic will have the highest likelihood of having the trait.” In ancient times, malaria was a prominent killer for people in regions like North Africa, the Mediterranean, the Middle East and Puerto Rico.
According to Dr. Abuzahra. “Carrying one of the sickle cell genes means you are resistant to malaria. This also means that people are able to live longer and have children, that allows the sickle cell trait to get to a higher concentration.”
It is important to note that for genetic disorders such as sickle cell, you need to inherit two of the affected genes, one from each parent. Therefore, if you only carry one sickle cell gene you would not have the disease, but because you carry the gene it is possible you can pass it on to your children.
For couples in which both partners carry the trait, they have the option of seeking genetic counseling prior to having offspring; who can inform them about the likelihood of their children developing a genetic disease such as sickle cell anemia.
In California, screening for SCD is done at birth. Newborns are tested to see what type of Hemoglobin they carry. Babies carry fetal hemoglobin and adults carry adult hemoglobin. “However, those individuals with a sickle cell trait will have a sickle cell variant that will be picked up by the newborn screen. Symptoms however, do not begin to show until the sixth month of age, because a baby will use their fetal type of hemoglobin , which is not affected by sickle cell disease,” says Dr. Abuzahra. “By six months of age, a baby will transition to adult hemoglobin, which is affected by the disease.”
For those suffering from SCD, Dr. Abuzahra recommends good hand hygiene, drinking plenty of water, and taking all the medications necessary and on time to maintain the healthiest life possible. In addition to those recommendations, patients should follow-up with their pediatric hematology specialist. It is also important that individuals are aware if they are over exerting their bodies.
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Lilian Ortiz is a student intern with the Center for Healthy Communities at the UCR School of Medicine.