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Early signals of sickle cell disorder in print

Traffic signs are static, but they are consistent. They are meant to instruct road users, whether transporters or pedestrians. Their interpretation remains the same, irrespective of tribe, culture, your mood or disposition. Red means stop, yellow means get ready and green means go. You must obey these traffic signals if you must pass that way.

Where laws are strictly enforced, a violation of the rules set on traffic light could result in accident, for the violator or for other unintended victims, or for both. The consequences of such a violation could be injury, disability or death: and these have the potential of attracting penalties, sometimes leading to years of jail terms depending on severity of offence.

It won’t matter how justified you felt, a violation of traffic rule still attracts due penalty. If your opinion about traffic light does not change the inherent sense that informed its application, so should your opinion not count much on the scientifically established facts about genes, genotypes, genetics and heritable conditions that have implications for individual health, life expectancy, longevity, family life and general wellbeing.

As the book on early warning signals on Sickle Cell Anaemia seeks to bridge an existing but wide knowledge gap in homes, families, medical and social circles, it is important to point out the danger and devastation of marriages with incompatible genotype combinations and underscore the need for people to understand that neither money nor science can yet cure the disorder and its complications. This is what the book is out to emphasise, while also warning against every mistake that could lead to such clinical conditions.

The writer, Pastor Emmanuel Dickson Ibekwe, burning with zeal and concern for the populace, made vulnerable by ignorance and selfwill sometimes, decided to follow up on an earlier book on this sensitive, but often ignored, subject matter, titled Cruel Wounds of Sickle Cell Anaemia: a Family Experience, which was launched about this time last year. He told his own story, shared his family experience and counselled various categories of people therein. This priceless service appears yet to be appreciated by many homes as the message, from all indications, needs to spread further afield, to prevent more families from falling into this pathetic and sometimes life-long ordeal of clinical conditions caused by sickle cell disorder for which no medical relief has been found. The writer adopted a different approach in writing the new book on early warning signals. A picture is worth a thousand words.

If the first book on Cruel Wounds seems – to a reader – too voluminous and laden with prose, the new book on Early Warning Signals is less in volume and richer in pictorial and graphical illustrations, easy enough to graph, with less stress and with greater capacity of receptivity and retention.

A cursory reader can pick this new book up and read all through within less than an hour. With all the coloured pictorial representations, the troubling signs of sickle cell disorder are vividly and succinctly captured for ease of understanding.

The author warned that, to those who have not come to terms with this condition and its potential to do damage, it is best to heed the voice of reason and run away from getting in, much less thinking of managing the consequences. An ounce of prevention is worth a pound of cure.

Unfortunately, in the sickle cell disorder, the road to cure still remains long, tortuous and endless, making prevention the most assured way out. For this reason, so much emphasis has been placed on premarital decisions, after which the consequences could only be managed. It is important, according to Pastor Ibekwe, to take advantage of the opportunity afforded by the disclosures in this book. Much attention is to be paid to avoiding decisions superficially made and marital plans that ignore scientific underpinning of a matrimonial union.

In this new book on Warning Signs, he has again sounded out his warning to those who are intending to get married. He has warned again that they should look out very well: or, in a current Nigerian parlance, to “shine their eyes well, well.” To them, he has written: “Let good conscience guide you in that engagement or courtship, otherwise you will be the worse for it. Do not allow any person, by flattery or falsehood, bring destruction on your lovely and innocent children. Your AS plus AS partner may betray you.” Going by personal experience, he has asked: “Could anything be more depressing?” You clearly have no reason to fall into this lifetime trap.

Economics of management of sickle cell anaemia makes it prohibitive for the poor. The stem cell transplantation carried out by a team of medical doctors in Nigeria recently that converted a seven years old patient from SS to AA is a very welcome development because Nigeria has the largest endemic population of sickle cell disorder. Many Nigerians have the trait while over 150,000 children are born each year with the disorder (WHO 2006) and over 100,000 children last year. So the breakthrough that helped one out of a million is highly appreciated by all well-meaning advocates of the eradication, prevention and management of sickle cell disorder. Unfortunately the enormous potential cost in Nigeria (N2.5 to N5million) is no doubt capital-intensive.

Therefore over a quarter of a million Nigerians with this disorder need assistance. Apart from stem cell transplantation a few other preventive steps that can reduce mortality rate can be taken. The book on Early Warning does not pretend to say everything to be known about sickle cell disorder, but proffers some ideas drawn from experience for families to properly manage the sickness. It also encourages sicklers, telling them to exercise faith in God. It goes further to highlight that it requires collective effort to provide the resources needed for the optimal maintenance of health in persons with sickle cell anaemia and to bring joy and peace into such homes. I believe that many that are planning marriage and child birth would read the book and run to the laboratory. Let the book incite an unprecedented degree of excitement for genotype confirmation.

While young people still get into marriage against medical advice, mostly through ignorance and refusal to conduct necessary tests, there is a possibility that some might be going in through wrong professional advice and provision of false sense of security.

This brings to the fore the possibility that there are either cases of false negative results or incompetent scientists putting the lives and future of young would-be couples in permanent jeopardy. In days when the sensitivity and specificity of laboratory results have been enhanced through sophisticated, state-of-the-art equipment, there are growing indications that some medical caregivers and service providers are endangering people’s lives.

While they need to play a proper role in providing appropriate signals to those who need counsel before deciding on marriage, it is rather disgusting to note that professional incompetence is already costing many of their clients very dearly. It is important that the message in this book goes far and wide, helping people to stave off the ailment at all cost and providing succour and coping strategies for the sufferers. I recommend this book as a handbook to be kept handy, read and read again as the tips being provided should help the readers and others they may

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