When the German author, Oscar Auliq-Ice said “everything you have worked for can go to waste with only one wrong decision,” he might have had in mind some young would-be couples who take irrational decisions in the name of love.
Miss Evelyn Agbontaen, (not real name), is beautiful, elegant and brilliant. She is in her late 20s. She has fallen in love with the man of her drea – good-looking with a good job and an assured future.
But there is a clause in her quest to tie the nuptial knot with her ideal man. Her genotype is AS – same as that of her husband-to-be – Olumide. She appears not to know the implication, or she is just not bothered.
She was apparently not interested in running a test to reveal her compatibility with her man. According to the lady, their love was strong enough to conquer any fear. But after much pressure from Evelyn’s parents to know her genotype as well as that of her suitor, she reluctantly accepted and the result was out in a matter of days.
The outcome of the result was the genesis of a protracted conflict between the lady and her parents. She insisted on proceeding with the proposed marriage, but her parents fiercely kicked against it. All counsel from Evelyn’s relatives and friends fell on deaf ears. She stood her ground and went ahead with the wedding. Twelve years down the line, she is full of regrets for her decision but it is rather too late – she has already lost one of her children to sickle cell anaemia, after having spent so much money to keep him alive.
There is a story of another adamant lover, who clearly knew the genotype of his wife-to-be. But he was not deterred; he moved on to marry the woman he desired. But the marriage metamorphosed into disaster, anguish, regrets and the death of their second daughter. And it ended in divorce after endless accusations and counter-accusations between the couple.
Sickle cell disease (SCD) is a hereditary problem that causes a faulty haemoglobin in red blood cells. A sickle cell crisis is pain that can begin suddenly and may last from hours to several days. It happens when sickle red blood cells block small blood vessels that carry blood to the bones. One might have pain in the back, knees, legs, arms, chest or stomach. The pain can be throbbing, sharp, dull or stabbing. How often and how bad the pain gets varies a lot from person to person and from crisis to crisis.
SCD is said to the most commonly inherited blood disorder. That means it is passed down through families; it is not something you catch or develop later in life. According to sufferers’ accounts, the ailment is dangerous and can cause extreme pain, anaemia, and other symptoms.
It is estimated that about 200,000 children are born with SCD yearly in Nigeria, representing the highest number of persons born with the disease across the globe.
As gathered, many of the children born with the ailment do not grow into adulthood, especially in Nigeria, where awareness and management of the disease is slow. Stakeholders have called for Nigeria to be in the forefront of finding a lasting solution to the burden, especially as the country reportedly has the highest burden of the disease in the world.
Contrary to beliefs in some parts of the country that the disease is caused by witchcraft or ‘ogbanje’, experts have corrected different myths around it, stating that through counselling, it can be prevented. They emphasised that the genotypes of both parents plays the role on whether their child will have the disease or not.
On how to manage the situation, a general practitioner in Lagos, Tochukwu Chima, told the reporter that the only way to manage the situation was to marry a partner who inherited the wrong haemoglobin. He described all the deaths arising from the crisis as 99 per cent preventable.
“You have virtually nothing to lose if you don’t marry that man or woman who you claim to be in love with. It is the campaign we have embarked upon for years now. Why should you allow your child or children to suffer pains as a result of your foolish decision or by mere ignorance? We have lost thousands of Nigerians to the disease, yet people keep making the same mistake. Some couples will tell us that there is nothing God cannot do. Bringing religion into it is very laughable.
“But for those who are already trapped, most of the time, you won’t know what causes your sickle cell crisis. A crisis usually has more than one cause. However, you can do several things that might keep a crisis from occurring. Some of the triggers are heavy consumption of alcohol, smoking, stress, hard labour jobs and other medical conditions. A patient is expected to have breathing problems, extreme tiredness, headache or dizziness, painful erections in males, weakness or a hard time moving some parts of your body and yellowish skin colour (jaundice).
“Some of the things to do to control the pain are hot baths, rest or massage. Do a physical therapy to relax and strengthen your muscles and joints. That might lessen your pain. Individual counselling, self-hypnosis and activities to keep you from thinking about your pain (such as watching television or talking on the telephone) might also help,” Chima said.
Speaking on factors that lead to the disease, the doctor said parents need to pass the abnormal haemoglobin gene on to the child in order for him or her to develop the disease. He said if both parents carry the defective gene, one has a one in four chance of inheriting the disease and becoming sick with it.
“If a child is born with one defective haemoglobin-beta gene, he may become a carrier of the disease. Carriers usually don’t develop SCD symptoms. But, they can pass the disease on to future children if their partner also carries the sickle cell trait. The person with AS should kindly avoid a partner with AS or SS when planning to get married,” he advised.
According to him, there are several different forms of sickle cell disease, and he explained that the type you or your child inherits depends on many things, including the specific type of abnormal haemoglobin you have.
In his words: “Haemoglobin SS, also called sickle cell anaemia, is usually the most severe type of this disorder. Other common forms include: Haemoglobin SC (usually mild), Haemoglobin Sβ thalassemia, while the rare types are haemoglobin SD, haemoglobin SE and haemoglobin SO.”
In many developed countries, newborn screening programmes require that all babies are tested for sickle cell disease shortly after birth.
In March 2018, the chairman of Association of Sickle Cell Anaemia Patients in Gombe State, Malam Isa Barkama, called for compulsory genotype test for intending couples.
He said that for intending couples to go for only HIV/Aids test before marriage was not enough considering the hardship anaemia patients have been going through.
“People only present HIV/Aids test and do not like genotype test, thereby cheating children yet unborn. What is the need for giving birth to children who will spend their entire life suffering, while there is a way out,’’ he queried.
As a reaction to the association’s demand, the Gombe State House of Assembly on March 20 passed a motion, which compels traditional and religious leaders as well as parents to ask for genotype screening result before joining intending couples as husband and wife in the state.
According to the motion, which was passed as a policy of the government, individuals set to be wedded will have to provide results of genotype screening, just like that of HIV/AIDS which is now the norm for every wedding in the state.
Alhaji Muhammad Bello, member representing Gombe South at the state House of Assembly, who presented the motion, said that until the pronouncement by the House, couples were only required to present results from HIV/AIDs tests before their wedding, not minding the genotype screening result, which seems to be more hazardous than HIV.
“My attention was drawn to the issue by two of our scholars in Gombe, who told me the implications of not including the screening as part of the requirement for marriage. I then studied their plea and realised that having one sickle cell patient in a family can jeopardise the wellbeing of the family. I once lived with a neighbour whose son was a sickle cell patient; I can recollect what they went through caring for the kid then. And such phenomenon may, perhaps, be prevented,” the lawmaker said.
However, the policy will be to advise the intending couples on ills of not going for screening and adhering to the professional counsel given after the screening.
The assistant pastor at Fountain of Knowledge International Ministry, Lagos, Mr. Ephraim Chidiebere, said it was mandatory at his parish for intending couples to produce a genotype screening result before being wedded.
“Once the result is against what our doctors have advised, we will not conduct the marriage. At the same time, we cannot stop the lovers from doing so in another church if they insist. The reason we don’t approve such marriage is because it will amount to deliberately causing the parents and their children to suffer avoidable pains,” the cleric said.