The Sickled One

I often joke that everyday could be a United Nation's day. June 19 is World Sickle Cell Day a day set aside to create awareness for the disease. A disease so common, yet little knowledge of it is circulated. Most people in my country (Nigeria) are full blown adults-at the verge of marriage before they realize their genotype. Another group is oblivious, not even knowing the difference between a blood group and genotype.

Sickle Cell Disease is a genetic disease worldwide present on four continents: sub-Saharan Africa and in the Maghrib, Asia, Middle-East, the Americas, the South and in Southern Europe . UNESCO estimates that FIVE HUNDRED THOUSAND are born every year with this severe and invalidating condition and that up to 50% of them will die before the age of 5 years.

Often times by no fault of theirs, people are given false results and are not aware they carry the 'S' gene. My older sister was sickly as a child and upon tests the blood work revealed she had the sickle cell disease, so the journey began. She would fall ill regularly (at least once every other month), had a fragile frame and had resigned to her fate. My parents never saw the need for a confirmatory test so she went on taking folic acid and getting the care anyone with sickle cell would. Fast forward several years later; now she doesn't fall ill as often and is a lot healthier, my sister is in her first year in the University studying Micro biology and decides to run a test on herself....the result was UNBELIEVABLE, she was now 'AA'. What had happened to the 'S' gene she lived most of her childhood fearing? obviously a mis-diagnosis or wrong result (as several confirmatory tests confirmed.). She is married and has two children of her own (all healthy).

My point is, how do we begin to advocate a disease so common yet not know? In modern days when genetics counselors are brought into prevention of the disease we cannot play dumb.

As much as the world thinks the rural communities need education on sickle cell disease, I have come to realize even the guy in the suit in the urban area is either clueless or ignorant of the disease.

The idea of using celebrities and influencial persons as ambassadors for the cause may be a good idea but sufferers of the disease also need to be brought into advoMy president's daughter Zarah Buhari was recently recognized for her work in promoting free genotype testing and genetic counselling in Nigeria. US ambassador to Nigeria,James Entwistle, gave the special recognition to Zahra for promoting genotype testingas an important early intervention in the prevention sickle cell disease.

Misconceptions about the disease, myths and facts need to be clearly labeled. In Nigeria, the mother of an 'Ogbanje' child or children is probably losing children from complications of sickle cell disease. the Term 'Ogbanje' in Igbo language is a term for what was believed to be an evil spirit that would deliberately plague a family with misfortune. Its literal translation in the Igbo is \"children who come and go\".

In some countries patients find it difficult getting proper treatment at emergency rooms...one doctor has found that patients in sickle cell crisis often aren't treated properly by doctors and nurses. They erroneously think the patients may be running scams, seeking drugs.

It is hoped that while people get genetic counselling and testing, counselling for sufferers of the disease and their families should also be adapted.