Sickle Cell Disease - The Tragedy of Ignorance!

Everyday we hear about HIV/AIDS, malaria and other communicable diseases. Granted, the aforementioned illnesses deserve the best of media coverage because they've been largely responsible for the grim health statistics in Nigeria. Unfortunately, there are other critical health challenges so often ignored. One of these is Sickle Cell Disease (anaemia)! Many Nigerians are now familiar with the disease. That wasn't the case decades ago when the disease, its cause, prevention and management has not been fully elucidated. Yet, with so much known about the disease, the ignorant attitude of many towards the disease is pervasive and heartrending. This is especially so in Northern Nigeria.

When I was a child the stories of the 'Ogbanje child' was everywhere. It made best-selling novels and movies. These are children who were said to die young only to be reborn or reincarnated to die again. We hear of a mother who keeps losing her children at tender ages. Some would lose four, five, six children in the same manner. Nobody knew the cause of the death of these children and so some came up with myths to explain it. Hence, the talk of the 'Ogbanje child:' a child who is wicked and seeks only the downfall of the family. Some were regarded as witches and wizards seeking the doom of their immediate family. Even a convulsing child or an epileptic teenager was regarded as an 'Ogbanje.' So, there were different traditional yet medieval methods cooked up to handle these children. One could only imagine the horror faced by these poor innocent children. Thanks to God: modern science came to the rescue! Many of those children suffered from sickle cell disease, a genetic illness which they inherited from their parents. It was explained that if both parents are carriers of the AS genotype, in every pregnancy, there is a one-in-four chance of them giving birth to a sickle cell disease child. This means while the children of a certain carrier couple will all have the disease, another couple will have carrier children mixed with those who have the disease. These children were derogatorily called 'Sicklers!' More appropriately, they are called sickle cell disease (anaemia) patients.

Are you are a sickle cell disease patient? Do you know a sickle cell disease child? Have you been close to a family with a sickle cell disease child? In my pediatric out-patient unit, about one in every five children I see is a sickle cell child. You will see the sheer burden of pain and stress borne by the sickle cell child or patient. You will see the exhaustion, frustration and anxieties borne by the parents and guardian. You will see beautiful young mothers looking like old women, some even abandoned by their husbands because of the excessive time, energy and resources expended on the care of the sickle cell child. I have seen marriages collapse because of the birth of a sickle cell child. I have seen mothers who forsook promising carriers to take care of their children. I've seen couples who would have been the pride of town been pitied by others because of the toll sickle cell disease has taken on the well-being of their family. Have you seen a child with severe vaso-occlusive crises, acute chest syndrome, et cetera, complications of sickle cell disease that leaves them crying and breathing with pains and much difficulty for long? Why do these children have to go through the pains of sickle cell disease in the 21st Century when it can easily be avoided?

It's not love but cruel injustice to the unborn child if a couple fail to know their genotype or worst still choose to marry when their genotype is not compatible. It's a travesty of true love and a miscarriage of justice! If not for anything else, think of that unborn child whom you are bringing into this wicked world to have an unfair share of its suffering. The world is hard enough as it is, why make it worse for them? Think of the cycles of vicious pains that child will suffer. I have a friend who was in love with a young lady back in the days. He was AS. He later discovered the lady was AS too. My friend started making series of research on the recent discoveries about sickle cell disease and was almost hopeful that he could go through with the marriage. That was until he met a single mother of two sickle cell disease children who lived in the neighbourhood for advice; a woman who was abandoned by her husband because he couldn’t deal with the reality of raising two sickle cell disease kids. When he left that woman's place, he knew much better and he ended that relationship respectfully with the mutual consent of the lady involved. Today, he is happily married to an AA lady (meaning no sickle cell disease gene) with a beautiful and lovely child with certainly no threat of sickle cell disease. If you are young and you are in love, it's a fine thing but think of 5, 10, 20 or more years time when the fairy tale of romantic passions start waning. We often say it's good to plan for one’s future, which is why we do a lot of things like going to school and working hard to make a living. In the same vein, plan for your marriage and your unborn child by knowing your genotype today. Let it be one of the cardinal things that will guide you in choosing a life partner. Please, in the name of the unborn child, do this today!

Sickle cell disease children are born beautiful and lovely like every other child. As he grows up, though some are slow, he can be exceptionally gifted and brilliant. He can be smarter than his peers. He does so well in school. His story is a glorious one to tell until he comes down with a crisis, until he spends so much time in the hospital that it can be called his second home, until that dreadful day when his fragile body can't take the pain no more and he slips away in the cruel hands of death. If you are one of his parents, what will you tell him is the cause of his problems? How will he feel when he grows up to learn that you once had a choice of bringing him into this world a better person but you chose otherwise? Many years ago when I was in medical school, there was the story of a medical student I will live to remember because I had the privilege of seeing him while he was alive. Poor me, I have forgotten his name. He was a very brilliant young man who was on top of his class. He was handsome and chubby. He was everything a young man would dream of save for the fact that he had sickle cell disease. He was in his twenties and about to complete his undergraduate medical training, least to say he was in his prime when he became very sick. He was so sick that he had to be transferred from one teaching hospital (tertiary health facility) to the another. He died on the way. I learnt when friends and colleagues went to pay his parents a condolence visit, someone rose to speak. Short of nothing to say, that person said 'God gives and God takes'. The grieving father asked the speaker to keep quiet. Think of it, God has made it possible for us to know about this disease and how it can be prevented, how anyone can dare blame God for their own wrong choices and undoing!

The story of sickle cell disease in Northern Nigeria! There is prevailing illiteracy and lack of formal education in most parts of Nigeria but the picture is worse in the north for several reasons. There is a burgeoning population in the North with scarce human and material resources to meet the educational needs of its populace. Then, there are the nomadic populations whose occupation warrants them to move from place to place. Aside from education, there are some attitudes and anecdotes in the Northern narrative which are inimical to promoting certain health issues. There is also the issue of consanguineous marriages amongst family members which is common among certain tribes in the north. The Garki Project conducted by Molineaux et al in Kano State in 1979 showed a prevalence of the sickle cell trait at 24.2% in those under the age of one and over 28% in other age groups (http://garkiproject.nd.edu/static/documents/garkiproject.pdf). While the efforts at eradicating Polio are commendable, in my own opinion, sickle cell disease poses a greater Public Health problem than Polio. I pray here that the media, International bodies, NGO's and the government would give more attention to the campaign and effort to stem this disease.

I want to use this medium to pass a message. Even if you can’t change the world, you can surely pass on a message of change. I’m a doctor who’ve seen and managed patients with sickle cell disease. I have friends and colleagues who have and who have lost loved ones suffering from sickle cell disease. So, I’m writing from a professional and a personal point of view. In law, it's often said ignorance is no excuse. In the matter of sickle cell disease, ignorance whether deliberate or not is absolutely inexcusable. An unborn child deserves the best as a gift from God. A newborn child comes to this world with cries of life and smiles of innocence. As he grows older, he expects the world to be the best place to live in. He dreams of a life full of meaning, joy, hope and love. Let's help make every child have the fullest potential to explore the amazing world around him and thus fulfill his dream to the best of his abilities. Know your genotype today and make his world a better place!