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Friday, March 6, 2015

Preventing Sickle Cell Disease Through Genetic Counselling

Sickle cell chart

According to the World Health Organization (WHO), over 500,000 babies with several forms of sickle cell disorders are born worldwide, making it the commonest inherited disorder in the world, with majority of cases occurring in underdeveloped and developing countries, especially in African countries like Nigeria. The world health’s study shows that Nigeria accounts for about 75 per cent of infant sickle cell cases in the continent with over 100,000 children dying from the scourge every year in Nigeria while 40 million Nigerians are carriers and an estimated one million persons living with sickle cell disorder. A prevalence rate that has resulted in Nigeria commonly referred to as the country with the highest level of sickle cell disorder in Africa. Even with its high prevalence rate in the country, there is presently no dedicated sickle cell centre in any of the 36 states of the federation, including the federal capital territory, according to available reports.

It is on this note that the Research Director, Fair Needs Africa, Lagos, Dr. Reuben Ogala, sought the need for serious awareness campaign among every citizen in the country, especially among intending couples and youths generally. He is of the opinion that reducing the scourge in the country requires genetic counseling so as to present the facts about sickle cell disorder to whoever is of child bearing age in other for them to be able to make

informed choices on who to marry and who not to marry, especially on issues of genotype. Ogala, said that when both parents have the AS genotype, there is a possibility of one in every four of their children having the SS genotype which is the sickle cell disorder; a diseases such a person will suffer throughout his or her entire life. 

He explained that a child born of the AS genotype has the sickle cell trait and can as well give birth to someone with the trait or someone who has the sickle cell disorder, if his or her spouse has the sickle cell trait (AS genotype) or who has the sickle cell disorder (SS genotype).

He believed that someone with AS genotype poses no threat until he or she mates with someone who also has the AS genotype or worse still, someone with SS genotype. According to him, there are other situations where the sickle cell trait is combined with other hemoglobinopathies; abnormalities of the hemoglobin in the blood, which in itself poses a threat. He said that the major issues emanating from sickle cell disorder affects children more, as their tolerance level is low compared to that of an adult, making them more at risk of the disease. According to experts, sickle cell anemia is a disorder of the blood that causes the red blood cells to blow into a sickle shape. These cells however do not carry oxygen efficiently to areas of the body where it is needed. Normal red blood cells have a 120-day life span, but people born with sickle cell disease have sickle-shaped blood cells that usually live not more than 20 days. These sickle cells can get stuck in blood vessels, blocking blood flow and less blood flow can damage the body's organs, muscles, and bones, sometimes leading to life-threatening conditions.

On why there is high incidence of the disease in Nigeria, Ogala said it is a genetic disorder common in some parts of the world, especially in malaria prone parts of the world like Nigeria and most parts of Africa. Explaining further, he said “sickle cell disease is especially common in Nigeria and some Africa countries because it has been observed in areas where there is significant incidence of malaria. This is because the pattern of distribution of plasmodium which causes malaria has a high relationship with the pattern of the incidence rate of the sickle cell disease. So it has been hypothesised that the sickle cell itself is an adaptation to the presence of malaria in the environment,” he added.

Shedding more light on how to tackle the scourge, the former permanent secretary, Ministry of Health, Lagos state, and pioneer Chief Medical Director, Lagos State University Teaching Hospital (LASUTH), Dr. Olufemi Olugbile explained that due to the medical care available now, people hardly die young as it used to be because majority are living into full adults and even growing old.

According to him, people should be aware of their own genotype and that of their prospective partners. “Knowing the risks involved hopefully reduces the prevalence of it. Inasmuch as counseling has a major role to play, one cannot make a law that, if the partners’ genotype is not compatible, then they cannot get married. It should only be advisory. People should either take it or reject it. But generally, awareness is the key to reducing the disease,” he added.

According to the advanced medical dictionary, Eugenics is the process of wanting to have perfect children, thereby eliminating imperfection by terminating certain pregnancies. It has also been associated with racism and preference of having one human type over another. While noting the importance of religious bodies, organizations and clubs in creating awareness on the need for people to know he risks involved, Olugbile therefore called on the government to establish sickle cell clubs where interactions can take place between persons with the disease. He advised that these clubs should be in schools, hospitals and other related institutions.


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