Sickle cell disorder has robbed me of my happiness. This disorder has killed three of my children as they had the ‘SS’ genotype. I spent a lot of money to ensure they stay alive but that never happened, no thanks to the ignorance of my wife and I.
These were the words of Demola Adebayo, a banker who unfortunately has the same ‘AS’ genotype like his wife. Adebayo is one of many Nigerian parents undergoing similar harrowing experiences.
As the situation is a global phenomenon, the World Health Organisation (WHO) has said that about five percent of the world’s population carries the trait genes for haemoglobin disorders, mainly sickle-cell disease (SCD) and Thalassaemia.
Haemoglobin disorders are genetic blood diseases that emerge due to the inheritance of mutant haemoglobin genes from both generally healthy, parents with the disorder affecting how oxygen is carried in the body.
While WHO notes that over 300,000 babies with severe haemoglobin disorders are born annually with the health burden of haemoglobin disorders, the global body says the burden can be effectively reduced through management and prevention programmes.
In an interview with BusinessDay, Olu Akinyanju, chairman, Sickle Cell Foundation Nigeria, said that over 40 million Nigerians live with the disorder, making Nigeria the country with the highest sickle cell burden in the world.
Explaining the disorder, Akinyanju said sickle cell is characterised by a modification in the shape of the red blood cell from a smooth, donut-shape into a crescent or half-moon shape cells lack plasticity and can block small blood vessels, impairing blood flow.
“This condition leads to shortened red blood cell survival, and subsequent anaemia, often called sickle-cell anaemia. Poor blood oxygen levels and blood vessel blockages in people with sickle-cell disease can lead to chronic acute pain syndromes, severe bacterial infections, and necrosis (tissue death). Sickle-cell disease predominates in Africa while thalassaemias are common in Asia, Mediterranean basin, and the Middle East,” the professor explained.
He hinted on the need to support sickle cell disorder sufferers by implementing the policies of Nigerian Sickle Cell Expert Advisory Committee (NISEAC) recommended since April 2008. Akinyanju stated that the aim is to increase their average life-expectancy from less than 20 years to what obtains among affected persons in the US: 57 years and above.
“These recommendations include introduction of newborn/infant screening programme for SCD to foster early diagnosis, provision of free prophylactic measures for infections that they are prone to and which frequently cause severe illness and deaths in children with SCD (oral penicillin and pneumococcal vaccines), training of sickle cell specialist nurses to increase access to skilled health workers, development of standard Blood Transfusion Services throughout the country and prevention of fraudulent marketing of remedies of unproven benefit to sickle cell affected population,” Akinyanju added.
Recent medical advancement has shown that stem cell transplant is an antidote to addressing the disorder which until now is deemed incurable. With stem cell transplant, a feat achieved recently by Nosakhare Bazuaye, consultant haematologist and coordinator, Stem Cell Transplant Centre, University of Benin Teaching Hospital (UBTH), the cost of transplant observers believe, most sickle cell sufferers may not be able to afford in the country.
BusinessDay findings show that it costs over N1.76 million ($110,000) per patient to carry out a bone marrow transplant, with an average of three months needed to carry out the procedure for transplant with series of tests and checks involved.
With the feat being the first of its kind in West Africa and third in Africa after Egypt and South Africa, calls on more sickle cell dedicated centres in the six-geopolitical zones of the country have been advocated. For instance, in the United States of America and United Kingdom, there are dedicated sickle cell centres with specialised nurses and doctors to offer medical services. India also have about 20 sickle cell centres.
