Sickle Cell Anemia is one of the few diseases most Africans know affects them in higher numbers compared to other ethnic groups. Surprisingly however, other ethnic groups, particularly those of Mediterranean and Middle Eastern descent, are also at risk. There is much mythology about Sickle Cell, its causes and prevention that modern science has demystified in recent years.
What Is Sickle Cell Disease?
Sickle Cell Anemia occurs when red blood cells that in healthy individuals are fat, round, flexible and donut–shaped, become thin, elongated, brittle and sickle–shaped. This change in form and function interferes with the cell’s ability to do its primary work – delivering oxygen to other cells and vital organs of the body. Instead, these red blood cells clump together and become sticky and immobile. They clog the walls of blood vessels and prevent other cells from receiving vital nutrients they need to regenerate and keep us healthy. Red blood cells affected by this disease do not live as long as normal red blood cells. They die after about 20 days and often leave an anaemic patient with a weakened immune system in their wake. Sickle cell patients often suffer from deep fatigue and are at risk for numerous infections, stroke and circulatory problems.
How is Sickle Cell Disease Transmitted?
The presence of two defective genes is needed for Sickle Cell Anemia. If each parent carries one sickle cell gene and one normal gene, each child has a 25% chance of inheriting two defective genes and having Sickle Cell Anemia. Children also stand a 25% chance of inheriting two normal genes and not having the disease and a 50% chance of being an unaffected carrier like the parents.This complex of possibilities points out the critical need for regular testing.
Blood test specifically for the sickle cell gene before marriage of both partners is highly recommended if the couple intends to have children. Testing results can inform Black couples of their risk of having children affected with Sickle Cell Anemia.
Symptoms & Treatment
People who have Sickle Cell Disease are prone to have a “sickle cell crisis” from time to time. During these periods, patients can experience extreme fatigue, body aches and swelling, particularly in the arms and legs. These symptoms are often treated at home with pain medication, lots of fluids and bed rest. In a more serious crisis, hospitalization may be in order where blood transfusions to increase oxygenation in the blood and antibiotic therapy to halt the spread of infections are routine treatments. In the most serious cases, some sickle cell patients receive bone marrow transplants.
A daily regimen of folic acid supplements and pain control medications are typical treatments most sickle cell patients live with for their entire lives. If you have the disease, take precautions to avoid over–exertion, extremes of heat or cold, dehydration and exposure in places like hospitals and public spaces where germs and contaminants that could lead to a serious infectious disease run rampant.
Science is making great strides in genetic testing that may one day lead to a cure for Sickle Cell Disease. In the meantime, with proper oversight, most patients can lead close to normal lives. Africans should endeavour to give blood often to local blood banks. Since our community is disproportionately affected by Sickle Cell Disease, your blood donation may help a child, a neighbor or family member improve their chances of leading a normal life
Photo credit: Medicinenet