Debunking myths about sickle cell disease
Nigeria with over 180 million people has the largest burden of Sickle cell disorder in the world’s with over 150,000 babies born with the serious condition every year, where it causes millions of avoidable deaths and suffering, particularly in children, which some may perceived as a spiritual ailment known as “Ogbanje” in Igbo and Yoruba “Abiku”.
Sickle Cell Disease (SCD) is a group of inherited blood conditions that affect the red blood cells in the blood. Red blood cells contain a protein called haemoglobin that carry oxygen throughout the body. Haemoglobin is normally disc-shaped and flexible, so it can be easily carried by the red blood cells in the blood stream. In sickle cell disease, haemoglobin containing red blood cells become shaped like crescent moon or a ‘sickle’ because of an abnormal haemoglobin gene. Hence the name sickle cell disease.
Sickle cells get stuck in the blood vessels and disrupt or block blood flow, thus, parts of the body where the blood flow is blocked do not get the required amount of oxygen. This can cause intense pain that can last from hours to a few weeks. This is known as sickle cell crisis
Olu Akinyanju, chairman, Sickle Cell Foundation of Nigeria, said “one in four or over 40 million Nigerians are health carriers of the sickle cell gene- Haemoglobin AS (Hb AS) while 1 in 50 or over 150,000 children are born each year with sickle cell anaemia (Hb SS)”.
“However, it is sad, but true, that control of SCD is not amenable to ad hoc and seemingly simple interventions. This is because these often lack a clear understanding of the complexity of the problem and of the ethics and objectives of effective genetic counselling, he noted.
According to World Health Organisation, Sickle Cell Disease (SCD) is a major genetic disease in most countries in Sub-Saharan Africa; trait is now known to be widespread, reaching its highest prevalence in parts of Africa as well as among people with origins in equatorial Africa, the Mediterranean basin and Saudi Arabia.
The Prevalence levels in countries such as Cameroon, Republic of Congo, Gabon, Ghana and Nigeria, is between 20% and 30% while in some parts of Uganda it is as high as 45%.
In countries where the trait prevalence is above 20% the disease affects about 2% of the population. The geographic distribution of the sickle-cell trait is very similar to that of malaria.
The sickle cell trait has a partial protective effect against malaria, and this may explain why it has been maintained at such high prevalence levels in tropical Africa.
Those who inherit the gene from both parents do not have this protection. In addition, they suffer from severe effects of SCD and many die before they reach reproductive age.
Adeolu Ogunsanya an Obstetrics and Gynaecology based in Lagos said, a child suffering of Sickle cell is affected as well as the family and recurrent sickle-cell crises delays the education, psychological growth of the child.
He further said, more Nigerians need to be aware of their genotypes before choosing a partner because majority of these children with the most severe form of the disease die before the age of five, usually from an infection or severe anaemia.
“Drinking lot of fluids will help patients suffering sickle cell so as not to dehydrate and wearing appropriate clothing to avoid catching cold will help to avoid trigger crisis”
“Nigeria still needs more awareness, government and stakeholders should help promote the masses”, said Ogunsanya.
Anthonia Obokoh