A patient undergoing a transcranial screening at the National Sickle Cell Centre
Solomon Elusoji probes into the sickle cell disease and writes that while it is important to prevent it, carriers shouldn’t live a life of gloom and doom
Funmiwo, the main character in Tunde Kelani’s 2014 movie, Dazzling Mirage, wanted to live a normal life. Yes, she had her issues (she had sickle cell anaemia), but she wasn’t going to let that stop her from doing what non-sickle cell anaemia patients do. She fought hard to keep her job, nurture lasting friendships, and build a home. Although things were never smooth-sailing, in the end, Funmiwo’s story resonates with a vibrant, vivacious hope that shines through dark, difficult days.
In Nigeria, according to statistics from the World Health Foundation (WHO), at least 10,000 babies die from sickle cell disorder every year. This figure puts Nigeria as the worst hit country, in terms of sickle cell deaths, in the whole of Africa.
The depressing sickle cell deaths statistics raises much cause for alarm with WHO indicating that Nigeria has the largest population burden for the disease, with over 40 million Nigerians as carriers and an estimated 1,000,000 living with sickle cell disorder in the country.
But what exactly is sickle cell anaemia? Simply put, according to Healthline.com, it is “a genetic disease of the Red Blood Cells (RBCs). Normally RBCs are shaped like a disk. This gives them the flexibility to travel through even the smallest blood vessels. However, in people with sickle cell, the RBCs have an abnormal crescent shape. This makes them sticky and rigid. They can get trapped in small vessels and block blood from reaching different parts of the body. This can cause pain and tissue damage.”
To have sickle cell anaemia, patients must have two copies of the sickle cell gene (SS, SC, CC). People who have only one copy of the sickle cell gene (AS, AC) are only said to have the sickle cell trait. They do not have sickle cell anaemia. In fact, the sole sickle cell gene present in their bloodstream provides them with better ammunition to be able to fend off malaria. Genetic experts have linked the ubiquity of the sickle cell gene (since it can fight malaria) in Sub-Sahara Africa, to the prevalence of Malaria vectors in the region.
Patients who have sickle cell anaemia experience bouts of severe pain in the bones, due to blocked vessels in different areas of the body. Complications that may arise because of the condition include: delayed growth, blindness, skin ulcers, priapism, gallstones.
But, contrary to popular belief, sickle cell anaemia is not a death sentence. Although it is true that in this part of the world, a lot of sickle cell anaemia victims die at a tender age, the reasons are not linked to the deadly nature of the disease.
“I have colleagues who have sickle cell and are doing very fine,” a senior medical officer at Igando General Hospital, Dr. Nitiri Aghogho, told THISDAY. “The problem is that there is a lack of awareness and sensitisation on what this disease is all about. Nobody is talking about it. Look at our educational institutions, how many children in secondary school know their genotypes? In more developed climes, everybody carries a card that contains their genotype and blood group, in case of emergencies. But it is not like that here.”
The caveat is this: two carriers of the sickle cell gene are advised not to make a baby, because there is, at least, a one in four chance that they are going to produce a baby with sickle cell anaemia. But despite the consequences, a lot of sickle cell gene carriers still go ahead and mate with one another.
“I think most people do it because they are in love,” a relationship writer who lives in Lagos, Miracle Adebayo, told THISDAY. “Then because they are in love, they back it up with religious faith and presume God is going to save them. Then there are those who don’t even know their genotypes and are not aware of the consequences.”
Lacking a sense of responsibility, according to Dr. Nitiri, is also another reason why these types of couples choose to have babies. “I think a lot of people don’t really appreciate the consequences of raising a sickle cell child. And the truth is that it is the women who suffer more. The men can run away. And that is another reason why we need to have more girl-child education, so that women can be able to make informed decisions in situations like this,” she said.
A Hypothetical Question
Segun and Funmi met during their days at the University, and went on to date for six years. When it was time to tie the knot, they went for marriage counselling in their church and were required to go for genetic testing. That was when they discovered they were both carriers of the sickle cell gene. What should they do?
Of course, the story of Segun and Funmi is not premised on real life, but it succinctly illustrates the sort of dilemma in which a lot of couples in Nigeria find themselves today. Should they break up and protect their yet unborn generation or damn the consequences and roll the dice.
“There is no law in medicine that says they can’t get married,” Dr. Nitori told THISDAY. “The only thing a medical counsellor will do is to make you aware of the consequences of your choice. It’s a one in four chance. If you are ready to take the risk, go ahead. If not, don’t.
“That’s why it’s good for people to know their genotype very early. In fact, I have a colleague who, whenever he meets a woman, asks her for her genotype. He knows that he is a sickle cell carrier, so he doesn’t even wait for romance to blossom before he knows your status, and whether you guys can tag along.”
A housewife and mother of one, Rachael Abiodun, told THISDAY that she would never advise such a couple to get married. “It’s a very wrong decision, because they will be putting the health of their unborn child at risk. It is not advisable for them to continue at all.”
If the couple decide to go ahead with the marriage and have babies, they will have different options in preventing or, as the case may be, managing the sickle cell phenomenon. First, immediately the woman gets pregnant, tests can be run to ascertain whether the baby will have sickle cell anaemia. If the baby will, it can be aborted.
“But you have to understand that abortion is illegal in Nigeria,” Dr. Nitori pointed out, “except in rare cases where the woman’s life is in danger. And also, a lot of religious faiths do not support the act.”
Although there is no widely available cure for sickle cell anaemia, the disease has been found to be curable in some children who have been successfully treated with blood stem cell, or bone marrow transplants. So,
secondly, if the couple have the funds, they can arrange for this procedure to be performed.
“Actually, I have a friend who was a sickle cell anaemia patient, she went to Germany, had this procedure performed, and today she is AA; all her children too her AA. I am not sure if we have the capacity to do that in Nigeria – I think there are a few institutions that can – but the point is, it is very curable,” Dr. Nitori said.
Thirdly, if the couple conceive a sickle cell child, they will have to be ready to give her special attention. Sickle cell anaemia patients who receive adequate medical and family attention have the same chances, as a non-sickle cell anaemia patient has, to survive.
“There is hope,” Dr. Nitori said. “People should know that. There are a lot of sickle cell patients who have lived long lives. So, people should stop being superstitious about the entire thing.”
She also went on to note that, due to wrong diagnosis by some laboratories, a lot of people who are sickle cell carriers have been misled to believe they are not. She implied that people should double-check their test results, and ensure that no errors have been made.
Meanwhile, the National Director of Sickle Cell Foundation of Nigeria (SCFN), Annette Akinsete, has noted that sickle cell disease can be reduced in the country through genetic counselling.
She said: “Genetic counselling is a process by which people with sickle cell disorder and their relatives and healthy carriers are provided with accurate, unbiased information in a structured, coherent, and co-ordinated manner in a caring relationship.
“Genetic counselling is a gateway for effective care and management of sickle cell. This is one of the services rendered by the centre, by highly qualified genetic counsellors in sickle cell centres. They deliver generic counselling every day of the week to individuals, couples and families.”
At the end of Dazzling Mirage, Funmiwo wins. She becomes an epitome of how the life of a sickle cell anaemia patient should be lived. She shows us that it doesn’t have to be all shadows and gloom. She doesn’t just show us hope, she gives it to us.