It is disheartening to observe that Nigeria has the highest number of sickle cell patients in the world and that out of 250,000 children born annually with sickle cell disease worldwide, about 100,000 are Nigerians, meaning that there could be between 80,000 and 100,000 new sickle cell patients born every year in the country. At any given time, there may be up to 1 million or 1.5 million sickle cell patients in Nigeria. The figure shows that Nigeria constitutes 40 per cent of the burden of sickle cell every year, the largest for any country in the world.
Sickle-Cell Disease, SCD is a major genetic disease in most countries in Sub-Saharan Africa. Majority of children with the most severe form of this disease die before the age of five, usually from an infection or severe anaemia. And only 5 per cent of the children live past the age of 10 in Nigeria as compared to over 96 per cent surviving into adulthood in the United Kingdom and the United States. Oftentimes, the survivors remain vulnerable to exacerbation of the disease as well as the recurrent pain and complications of the disease interfering with many aspects of the patient’s life- education, employment and psychological development.
Health experts attribute this disease to a genetic disorder that affects the haemoglobin within the red blood cells called haemoglobin S, Hb S instead of haemoglobin A, Hb A. Haemoglobin in the red blood cell is responsible for the transportation of oxygen in the body. The red blood cells (Erythrocytes) may contain normal haemoglobin AA only, a mixture of A and S (Hb AS) or S only. So persons with AS haemoglobin have sickle cell traits, and are referred to as sickle cell carriers, while those with haemoglobin SS only (Hb SS) suffers from sickle cell disease and those with AA are normal without sickle cell traits.
Today, the most effective way to curb ignorance surrounding this disease is through an aggressive public sensitisation and enlightenment campaign. Such information should include the fact that carriers should not marry AS or SS as well as education about inheritable nature of the disease. Towards this end, there is need for public education to determine genotype, so that incompatible people do not fall into love let alone marry each other. So, the best option for now is sensitisation which should be at the head of other strategies.
However, since there is presently no known cure for the disease, except the existence of a cost effective treatment for the pain and other related-aspects of the disease, the most crucial components of the treatment include early interventions with analgesics, antibiotics, rest, good nutrition, folic acid supplementation and high fluid intake. At times, all-encompassing procedures such as blood transfusion and surgery may be needed.
Also, there is sufficient evidence that neo-natal screening for sickle-cell disease when correlated to timely diagnostic testing, parental education and comprehensive care, clearly reduces morbidity and mortality in infancy and early childhood. Nonetheless, the most simple, inexpensive and cost-effective procedures remain the use of penicillin to prevent infections, which sadly are not readily available to most patients.
Interestingly, the World Health Organisation, WHO, under the DNC Department, helps to supervise and coordinate interventions relating to the prevention and management of SCD globally. By so doing, WHO intends to support primary prevention (genetic counselling, general public knowledge); early detection – screening; reduce morbidity; chemo-prophylaxis, vaccines, clinical care of special groups (children, pregnant women); capacity building Human resource; improve quality of life of patients and build partnerships.
With the stunning revelation therefore, we urge President Muhammadu Buhari to create sickle cell management centres across the 774 local governments of the federation so as to reduce the pains and trauma experienced by the sufferers. And since it is a preventable disease, policy makers should enact a legislation making it compulsory for everybody to undergo genotype tests before marriage. The change efforts of the present administration can only be meaningful if it includes healthcare needs of all Nigerians.