Intervention for sickle cell anaemia

The acronym, BMTCs, is not some concoction made from some sacred tree barks indigenous to “Kotopo” or my village at Itori in Ewekoro Local Government Area of Ogun State. Rather, BMTCs is an acronym for Bone Marrow Transplant Centres. Other than the basic concepts we learnt back in the days of Integrated Science at African Church Grammar School in Abeokuta, Ogun State, there has not been a new vocabulary other than danger yet added to the consequences of the union of a couple with the sickle cell trait (common cases being one with the Hb AS or AC genotype marrying another with same Hb AS or AC genotype).

We know there is a 25 per cent chance that such a couple may birth a baby with the sickle cell disorder. Musings of a regular partially educated couple may erroneously explain the 25 per cent chance to mean – for every four kids, there may be a sickle cell baby; then, such a couple may choose to stop at having three instead. The rationale is so grossly fallacious and the scientific truth is that such a couple could in fact unfortunately have all three babies with the sickle cell disorder. The 25 per cent chance actually means that on the birth of one single child, if you are to pick among four genotypic options, there is one in four odds that the baby’s genotype could be SS – depending on each independent allele contributed by both heterozygous parent (who are commonly Hb AS or AC).

The scientific front has evolved over the years from the days of just taking a lackadaisical approach to addressing such disease like sickle cell anaemia. Folic acids and hydroxyurea could just do their ameliorative bits in the disease management, but the genetic components of the disorder in affected individuals still remain over the years. Frequent blood transfusions as a treatment option can also only last a quarter because the foundational mechanisms controlling hematopoeisis see to it that normal Red Blood Cells do not live beyond 120 days before they are cleared off the circulatory system.

The two preventive causes often advocated for are: To seek genetic counselling prior to getting married should there be the sickle cell trait in a family line; or to seek help with assisted conception to select the genotype of an embryo prior to the commencement of pregnancy – should an affected couple still decide to proceed with such marriages. Either choice is costly. And for the already affected families with sickle cell babies, youngsters and adults – a preventive cause may just be like a screeching broken record. But the truth is, sickle cell disorder actually does have a cure!

Science in itself is a conception of faith and does not always sentence its opposition to fate. I am a young scientist and also one of the healthy 40 million Nigerians with the sickle cell trait (Hb AS). I have lost two friends and a cousin to sickle cell disease while growing up, and I am friends with a Nigerian family here in the United States whose two healthy kids had access to a successful bone marrow transplant to revert their sickle cell status to just being a carrier – like I am. I understand the consequences of working with the odds of a 25 per cent chance in a heterozygous union (example being Hb AS/AS or AC/AS or AC/AC), but then I would not gamble with such odds if there were not a definitive scientifically proof already. For now, bone marrow transplant seems to be the remedy; and an adequate understanding that it is not an outrageously evasive treatment only limited to the western world – is yet another mentally liberating force to a lasting solution for a country with the highest sickle cell cases in sub-Saharan Africa. We could keep blaring the horns to the Federal Government till forever – to create for her citizens such Bone Marrow Transplant Centres that could help families suffering in silence with the scourge of sickle cell anaemia, to live past their challenge if they are able to access the right intervention programmes; but there may not be enough help to trickle down to every nook and cranny of the nation.


-- Souce: http://www.punchng.com/opinion/letters/intervention-for-sickle-cell-anaemia/