Sickle cell disease is a genetic condition that runs in families and is more common in certain races, especially Black people. It is caused by a point transformation in the B-hemoglobin gene leading to the blend of sickle hemoglobin, which polymerizes when deoxygenated, distorting and injuring the red blood cell.
Patients with sickle cell disease are homozygous for this change and they suffer from acute vaso-occlusive events, hemolytic anemia, organ damage and failure, and an average lifespan reduction in developed countries of more than three decades.
Its known symptoms are fatigue, headaches, dizziness, short of breath and coldness in the hands and feet. Other symptoms include jaundice, swollen hands and feet, severe chronic pain and severe infections.
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According to the World Health Organisation, figures cannot be placed on people living with the disease in the United States, however, with its work with partners, Center for Disease Control, approximately, 100,000 Americans have SCD. It also projects that SCD occurs amongst 1 of every 365 Black or African-American births, SCD occurs amongst 1 of every 16, 300 Hispanic-American births, about 1 in 13 black or African babies are born with sickle cell trait. With all these towering figures, the economic cost in medical expenditure for children with SCD in the US averaged about $11.702.
Despite all the figures coming out of the US, the prevalence of SCD is more in Africa and within the black race. Reports indicate that in the African continent, 300,000 babies are born with the disease annually, the majority of them coming from South Saharan Africa.
Amazingly, the world’s SCD population is centered and concentrated in three countries, two of which is from Africa – Nigeria and DR Congo. Reports say that 2 percent of the population from these countries are affected by the disease.
Why is SCD prevalent in the black race?
Abstract studies suggest that the inheritance of some genetic diseases largely depends on ancestry. This is a detail that must be studied when interpreting SCD with the black race.
Findings by www.ncbi.nlm.nih.gov compared the genetic profiles of African Americans with sickle cell disease to those of Black Africans and Caucasian populations of European descent and found that they are less genetically admixed than other African Americans and have an ancestry similar to Yorubas, Mandinkas and Bantu.
They further stated that a transformation of the sickle hemoglobin reached polymorphic frequency in areas in Africa, while the others can be found in the HbS gene in the Middle East and India where malaria is prevalent.
As the world celebrates World Sickle Cell Day, the World Health Organisation has declared Sickle Cell Disease a public health priority as 6 million Africans will be living with SCD if average survival reaches half the African norm.
While the US and UK are reducing the mortality from 3 to 0.13 per 100 people with some drastic interventions such as comprehensive care, newborn screening and prevention of infections, countries in Africa have been delayed by lack of information.
Here are some misconceptions about this disease;
- Although the red blood cells in a person with sickle cell are sickled in shape, the disease is actually named after a C-shaped farming tool called a sickle.
- Sickle cell disease is not a communicable disease as opposed to the perception that it can be spread through contact.
- Both parents must carry the sickle cell gene to have a child with sickle cell disease.
- People living with sickle cell disease can live past 60-years as opposed to public opinion that they cannot grow to be adults.