The United Nations General Assembly established World Sickle Cell Day in 2008 to raise public awareness of sickle cell disease (SCD) and its treatment. Countries worldwide commemorate World Sickle Cell Day every year on June 19. The purpose of this day is to raise awareness of sickle cell anaemia as a public health issue and encourage people to learn more about this inherited illness. This year’s World Sickle Cell Day theme is ‘Shine the Light on Sickle Cell.’
Sickle cell disease is a set of hereditary red blood cell abnormalities. Haemoglobin, a protein that transports oxygen, is found in red blood cells. Red blood cells are spherical and flow through small blood channels to transport oxygen to all body regions in a healthy state. Haemoglobin is aberrant in people with SCD, causing red blood cells to become stiff and sticky, resembling a C-shaped farm tool called a “sickle.” The sickle cells die early, resulting in a chronic lack of red blood cells. They also get caught and obstruct the blood flow when travelling through small blood arteries.
SCD is inherited from parents in the same way we inherit blood type, hair texture, eye colour, and other physical characteristics. Individuals inherit one from each parent, as they do with most genes. The forms of haemoglobin produced in red blood cells are determined by the haemoglobin genes inherited from both parents.
For instance, all of the children will have sickle cell trait (AS) if one parent has sickle cell anaemia (SS) and the other has normal blood (AA). On the other hand, if one parent has SS and the other parent has AS, there is a 50 percent probability (1 chance out of 2) of having a baby with sickle cell illness or sickle cell trait with each pregnancy. In comparison, each pregnancy has a 25 percent probability (1 chance out of 4) of having a child with sickle cell disease and a 50 percent probability of having AS if both parents have AS.
A healthy carrier of a sickle-cell disease gene makes up about 5 percent of the world’s population. In some areas, the percentage of persons who are carriers of the gene is as high as 25 percent.
These diseases are prevalent in tropical areas, although they have spread to most countries due to population movement.
In Africa, sickle-cell disease is the most common, with Nigeria being the epicentre, with 4-6 million people living with the disease, and 1 in every 4 Nigerians has a sickle cell trait. This implies that Nigeria has the highest number of people suffering from SCD worldwide, with about 40 million people having the disease.
Every year, approximately 300,000 children with SCD are born worldwide, of which sub-Saharan Africa accounts for over 75 percent of the total. In Nigeria, 100,000-150,000 newborns are diagnosed with SCD (33 percent of the global burden of SCD) every year. As a result, from a worldwide perspective, Nigeria has a critical position in the epidemiology of SCD.
The reason most of those born in poor and middle-income nations have SCD may be due to illiteracy and ineffective government awareness programmes about the disease.
A sickle cell crisis happens when a sudden discomfort can last from a few hours to as long as many days. It occurs when sickled red blood cells obstruct small arteries carrying blood to the bones. The discomfort can be throbbing, acute, dull, or stabbing in nature and can affect the back, knees, legs, arms, chest, and stomach. This can also result in pain and other major health issues such as infection, acute chest syndrome, and stroke.
Although there may be no idea of what prompted a sickle cell crisis; however, there are frequently multiple causes for a crisis. As we know that prevention is better than cure, there are a few things that can be done to help avoid a crisis which include: avoiding alcoholic beverages, avoiding smoking, regular exercise, drinking plenty of water while exercising, reducing stress, early treatment of infection, wearing of warm clothing in cold weather and fabulous dress in hot heat, avoid swimming in cold water, and think positively about yourself.
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In addition, the treatment of sickle cell disease is lifelong. However, the simple techniques that can be used to treat sickle-cell disease include high fluid intake, a nutritious diet, folic acid supplementation, pain medication, and vaccinations. Antibiotics are also available for infection prevention and treatment and various other therapeutic procedures.
Furthermore, sickle cell disease poses a lot of economic burden on society as the cost of treatment for SCD increases. SCD also interferes with the education, work, psychosocial development, and basic survival of people with SCD.
Thus, the way forward is for the government to maintain the appropriate policy that will guide and reduce the prevalence of SCD in the country. This can be done by giving people adequate genetic counselling as marriage counselling is not the same as genetic counselling.
Also, SCD should be treated with care in the community. People with SCD or healthy gene carriers may feel stigmatised if they don’t disclose, deny, or lie about their haemoglobin genetic status. This situation may be counterproductive to efforts to control the disturbance in the community. Therefore, there should be proper education and counselling for people and families of people with SCD to avoid stigmatisation.
Busayo Aderounmu is an economist and researcher
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