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WHAT IS SICKLE CELL DISEASE?

Sickle-cell anemia (also known as sickle-cell disorder or disease) is a common genetic condition that occurs due to a haemoglobin disorder – inheritance of mutant haemoglobin genes from both parents. Such haemoglobinopathies (thalassaemias and sickle-cell anaemia) are globally widespread.

About 5% of the world’s population carries genes responsible for haemoglobinopathies. Each year about 300 000 infants are born with major haemoglobin disorders – including more than 200 000 cases of sickle-cell anaemia in Africa. Globally, there are more carriers (i.e. healthy people who have inherited only one mutant gene from one parent) of thalassaemia than of sickle-cell anaemia but the high frequency of the sickle-cell gene in certain areas leads to a high rate of affected newborns.

Sickle-cell anaemia is particularly common among people whose ancestors come from sub-Saharan Africa, India, Saudi Arabia and Mediterranean countries. Migration raised the frequency of the gene in the American continent.

SCD is widespread in sub-Saharan Africa and among descendants of Africans worldwide. Sufferers have no visible symptoms, but periodically experience severe pain crisis and are also highly prone to anemia because the blood cells break down after only 10-20 days, rather than the usual 4 months.

In some areas of sub-Saharan Africa, up to 2% of all children are born with the condition. In broad terms, the prevalence of the sickle-cell trait (healthy carriers who have inherited the mutant gene from only one parent) ranges between 10% and 40% across equatorial Africa and decreases to between 1% and 2% on the north African coast and <1% in South Africa.

The public health implications of sickle-cell anemia are significant. Its impact on human health may be assessed against the yardsticks of infant and under-five mortality. As not all deaths occur in the first year of life, the most valid measure is under-five deaths. Increasing proportions of affected children now survive past 5 years of age but remain at risk of premature death. When health impact is measured by under-five mortality, sickle-cell anaemia contributes the equivalent of 5% of underfive deaths in Africa, more than 9% of such deaths in west Africa, and up to 16% of under-five deaths in individual west African countries.

In West African countries like Ghana and Nigeria, the frequency of the trait is 15% to 30% whereas in Uganda it shows marked tribal variations, reaching 45% among the Baamba tribe – west of Uganda.

Frequencies of the carrier state determine the prevalence of sickle-cell anemia at birth. For example, in Nigeria, by far the most populous country in the sub region, 24% of the population are carriers of the mutant gene and the prevalence of sickle-cell anemia is about 20 per 1000 births. This means that in Nigeria alone, about 150 000 children are born annually with sickle-cell anemia.

From available statistics, 100,000 infants die from sickle-cell disease in Nigeria annually out of  the 150,000 who are born with Sickle Cell Disease, making it the number one sickle-cell endemic country in Africa  [KANO, 21 August 2008 (IRIN) ].

Based on World Health Organization [WHO] indices, Nigeria accounts for 75 percent of infant sickle-cell cases in Africa and almost 80 percent of infant deaths from the disease in the continent. More than 5 million of the total population in Nigeria has been discovered to be suffering from the deadly disease while 25 million others carry the genes which they pass to their offspring (Falusi, 2012).

There are, however, no firm data on the survival of patients with sickle-cell anemia on the African continent.  The statistics in Nigeria though alarmingly high may just be a skeletal representation of the real situation to unavailability of adequate statistics.

Sickle Cell disease affects nearly one hundred million people in the world and is responsible for over 50% of deaths in those with the most severe form of the disease.
In Nigeria, according to the Federal Ministry of Health, sickle cell disease is among the ten (10) priority non- communicable diseases (NCD) and it contributes significantly to both child and adult morbidity and mortality. Presently, Nigeria stands out as the most sickle cell endemic country in Africa with an annual infant death of 100,000 representing 8% of infant mortality in the country. It is estimated that about 24% Nigerian adults carry the Sickle Cell trait.
As alarming as the facts of the situation confronting the nation about this condition is, our response to the ordeal has been sluggish and very minimal. The mere mention of the disorder makes people cringe and postpone the discussion, yet it’s a reality that continues to worsen.

 The recurrent pain and complications caused by the disorder can interfere with many aspects of the individual’s  life, including education, employment and psychosocial development. Presently, the only cure for SCD is the bone marrow transplant. However, cost-effective treatment exists for the pain and other aspects of the disease. The most important components of this treatment are early intervention with analgesics, antibiotics, rest, good nutrition, folic acid supplementation and high fluid intake. At times, invasive procedures such as blood transfusions and surgery may be required.

Counseling and prevention of the causes and early treatment of infections are simple measures not readily accessible to most individuals . As a result, the majority of children with the most severe form of the disorder die before the age of five, usually from the complications associated with infections or severe anemia. The survivors remain vulnerable to exacerbations of the disease and the complications mentioned above. Unfortunately, this situation is further compounded by the lack of basic facilities to manage patients. Systematic screening for SCD using a simple blood test is not a common practice, and diagnosis is usually only made when a severe complication occurs.

SCD has major social and economic implications for the affected child as well as the family. Recurrent sickle-cell crises interfere with the individual’s life, especially with regard to education, work and psychosocial development and other related cultural traumas. Through repeated failures in the quality of care, sickle cell anemia can become a chronic illness i.e. an extremely expensive and highly stressful health-care crisis. At such times, primary caregivers (often parents) are exasperated and might want to give up in despair; even desiring the death of the child at times, as a source of release for both the child and his care-givers.  This Foundation seeks to ameliorate the harsh psychosocial trauma that patients and care-givers experience.