For a genetic disorder that has affected families and spousal selection, Nigerians do not know enough about the Sickle Cell Disease (SCD) or Sickle Cell Anemia (SCA). It is quite easy to throw around the letters AA, AS and SS, without a fundamental understanding of what they mean and how a cross between these genotypes can affect an offspring.
Today, 19th of June, 2018 is Sickle Cell Awareness Day and here is all you need to know about Sickle Cell, brought to you by Happenings.
Sickle Cell Anemia or Sickle Cell Disease, is a genetic condition in the blood, passed on from parents to offspring and it has no known cure. The genetic disorder of SCD or SCA, makes it impossible for enough healthy red blood cells to carry adequate oxygen throughout the body. Even worse this lifelong disease causes red blood cells to become misshapen, hard and inflexible, thus it is difficult for them to travel in small blood vessels. Oftentimes they block these blood vessels and the flow of Hemoglobin rich blood to vital organs leading to a crisis, excruciating pain, damage of vital organs and eventual death. While normal blood vessels are round, soft and flexible, the red blood cells affected by the sickle cell disease are shaped like hooks or tiny crescent moons , they are hard, sticky and not flexible enough to travel through blood vessels in the body.
Interestingly, the SCA or SCD is a genetic mutation prevalent in people of African descent and it is quite rife in sub-Saharan Africa. While this lifelong disease has no cure, it can be effectively managed, to relieve pain and organ damage when in crisis.
An individual with the AA genotype has no affiliation with the sickle cell disease, while one who is AS carries the trait of the sickle cell disease but is not a sufferer. However when individuals who are AA cross with those who are AS, there are chances that their offspring will carry the sickle cell trait but they will not be sufferers. If two individuals who carry the sickle cell traits cross, their offspring may either have the AA, AS and the SS gentoype. It is those who have the SS genotype, that become what we call ‘sicklers’.Thanks to modern medicine, a foetus in the womb can get screened to see if they carry the SS genotype.
A sufferer of the Sickle Cell disease may go into crisis when subjected to stress, dehydration, infection, temperature changes, and high altitude. This could cause cells with the sickle cell disease to block some blood vessels, leading to a shortage of red blood cells(Anemia) , when parts of the body are deprived of oxygen rich blood, they begin to die.
An individual who suffers from the SCD when in crisis will feel fatigue, periodic episodes of pain e.g joints, stomach, bones; painful swelling of stomach, hands and feet; ulcers, bone and organ damage, paleness of the skin, yellowness of the eyes and nail beds.
In extreme cases, a crisis may cause stroke (when parts of the brain are denied hemoglobin rich blood), blindness (when parts of the retina are denied hemoglobin rich blood), organ damage of the lungs, spleen and pulmonary hypertension. A male sufferer of the SD may suffer Priapism (long lasting painful erections when the sickle cells block the blood vessels in the penis. This can damage the penis and lead to impotence.
When a crisis is severe, the sufferer of the Sickle Cell Disease may be hospitalized, while SCD has no known cure, sufferers must avoid crisis triggers by staying hydrated, taking antibiotics and taking proper care of themselves. Parents who carry the sickle cell trait, must see a genetic counselor before trying to conceive, as this can help them understand the risks of having a child with sickle cell anemia. Thus, they are properly acquainted with possible treatments, preventive measures and reproductive options.
Happy Sickle Cell Awareness Day.