'What's the worst that could happen?': Love in the sickle cell capital of the world

The prevalence of sickle cell disease is changing how Nigerians date, marry and plan their lives. And as genetic testing becomes more common, prospective parents across the world will face similar questions by Krithika Varagur

Subomi Mabogunje fell for Nkechi Egonu within hours of meeting her in 2004. They were working at a state-run TV station in Ijebu Ode, a trading hub in south-west Nigeria. While Subomi was thin and bespectacled, Nkechi was petite and voluptuous, with her hair in a ballerina bun, and coldly immune to the stares that trailed her across the office. Her swaggering personality was also the opposite of his reserved one, and she was quickly promoted to programme presenter. She was the most exciting person, Subomi felt, who had ever walked into his home town.

He found the courage to speak to Nkechi one weekend when they were assigned to do community service, clearing overgrown grasses near a government building. Subomi went, despite his habitual avoidance of strenuous physical activity. “You’re too good for this kind of work, ehe?” Nkechi teased. With his hollow cheekbones, frail body and elongated fingers, Subomi was clearly what some uncharitable onlookers would call a “sickler” – one of up to 6 million people in Nigeria with sickle cell disease (SCD), a group of inherited blood disorders that turn red blood cells from soft discs into rigid crescents, leading to blood clots, pain episodes called “crises” and serious complications in most major organs. But Nkechi never shied away from him. Within a few weeks of their first conversation, they were inseparable. 

From the beginning, Nkechi knew that she and Subomi had “no business dating”. His genotype was SS: he had two abnormal S genes for haemoglobin, the oxygen-carrying protein in his blood. Nkechi’s genotype was AS: she had one abnormal S gene and one normal A gene. Like an estimated quarter of all Nigerians, she was a “silent carrier” of the disease. So there was a 50% chance that any child they had would be born with SCD. This was no light prospect. Subomi’s childhood had been marred by secrecy and shame over his condition, and Nkechi had lost four SS cousins to it. But those deaths happened in the decades before widespread genetic testing. Today, there was a growing consensus – particularly in their university-educated, upper-middle-class milieu – when it came to passing on two sickle-cell genes: don’t risk it.

But how, and when, do you weigh risk against attachment? Nkechi revealed her genotype just days after meeting Subomi, and he didn’t stop loving her. By the year’s end, Nkechi had fallen in love with Subomi, too – but she thought it shouldn’t last. In March 2005, she moved to Lagos alone. Subomi simply followed her. In April 2006, she broke up with him at a fried chicken restaurant, screaming about how stupid, how uneducated, it would be for them to stay together; they reconciled a week later. She broke up with him again in January 2008, sitting in his parked car; that separation lasted a month.

Nkechi was just shy of 30 when she initiated their third breakup, in late July 2009. As the eldest of six, she was starting to feel uneasy that neither she nor her siblings had married or started families, unusual for Nigerians of their generation. She had even talked to a genetic counsellor at the Sickle Cell Foundation Nigeria, in Lagos, who had advised her to end the relationship. (A representative from the foundation told me that this is against their policies.) Nkechi and Subomi talked and cried for three hours as the sun set, and she kept crying on the lurching bus ride home. They were tears of grief, but also relief. She had finally, and responsibly, severed this unwieldiest of attachments.

Subomi didn’t sleep much that night. Early the next morning, he started driving to his father’s house in Ijebu Ode. Something told Nkechi to call him in the afternoon, to make sure he’d got there. Subomi’s boss picked up the phone.

“I was actually just about to call you,” Nkechi recalls him saying, over a crackling connection. “Subomi’s had an accident.”

Less than an hour outside Lagos, Subomi’s sedan had been rear-ended, hit a truck and flipped over. Paramedics had taken him to a hospital in the city of Ibadan. Nkechi’s mouth went dry. At dawn, she began a long bus journey to the hospital.

Subomi was in the ICU, in a coma. Nkechi held his mother’s hand as the doctor explained his prognosis. It could be hours, days, weeks or months before he woke up, if he woke up at all. Nkechi had to work on Monday, but she returned to Ibadan the following Friday, inaugurating a regular weekend pilgrimage.

On the first Saturday in September, she showed up as usual and was stunned to find Subomi awake. She screamed with joy and clasped his hands. He was soon discharged, but still faced a long road to recovery. Nkechi substituted her weekend trips to Ibadan with visits to his mother’s house in Lagos. She guided him through complicated stretches, gave him sponge baths and crawled on the floor beside him as he learned how to walk again. Remarkably, he was on his feet within six months. Whenever Nkechi left his side, he started to ask her when they were getting married.

Nigeria is the sickle cell capital of the world. Its residents account for about half of all new cases of severe haemoglobin disorders worldwide. And SCD is one of the world’s most prevalent autosomal recessive genetic disorders; the sickle cell trait is more than six times more common in Nigerians than the cystic fibrosis gene is among people of northern European descent, or the Tay-Sachs gene among Ashkenazi Jews.

In the 1950s, a number of scientists speculated that the sickle cell trait confers some resistance to malaria – now a widely accepted theory – which would account for the prevalence of the gene in sub-Saharan Africa, home to more than 90% of all malaria cases in the world. Over millennia, according to this hypothesis, as more AS than AA children survived acute malaria infections and reached reproductive age, they passed on their single S genes. But for those with two such genes, the potential complications include acute pain episodes, acute chest syndrome, strokes, priapism, jaundice, numb chin syndrome, an enlarged spleen, leg ulcers and blindness.

The heritability of sickle cell anaemia, the most common and severe form of SCD, is as follows: if both parents are carriers (AS/AS), their children have a 25% chance of having the disease. If one parent has the disease (SS) and the other has no sickle cell genes (AA), there’s a 100% chance their kids will be carriers (AS) and a 0% chance they will have the disease. A couple like Nkechi and Subomi, where one parent has the disease and one is a carrier, has a 50% chance of giving birth to an SS child and a 50% chance of an AS child.

Olufemi Akinyanju, an 86-year-old Nigerian haematologist, started identifying sickle cell patients as a young doctor in Lagos in the 1960s, fresh out of medical school in London. In 1994, he founded the Sickle Cell Foundation Nigeria in Lagos, now one of the region’s pre-eminent treatment and diagnosis centres. His generation of doctors helped SCD testing take root in Nigeria. The most common blood test is haemoglobin electrophoresis, in which electrical currents are passed through a blood sample, separating different types of haemoglobin into discrete bands. The test is now commonplace in urban centres such as Lagos and Abuja. Testing in a high-end facility can cost up to US $40, but there are also free and cheap clinics, which can provide results in as little as 30 minutes.

Nkechi, who was born in 1979, has known her genotype since she was seven. But testing is less accessible outside major cities, especially in the country’s poorer northern states. And in less well resourced clinics, results are often inaccurate. Some find this out the hard way, like Ezekiel Ogbu, a 36-year-old bus driver with SCD who lives in Lagos. In 2018, he discovered, a few weeks before his wedding, that his fiancee was not AA, as she had long believed, but AS. They broke up, and called off a wedding with more than 500 guests. Many Nigerian sickle cell activists now recommend two or three different blood tests to be sure.

Determining your genotype is one thing, but puzzling out its implications is another. Given Nigeria’s emphasis on marriage, its relatively low incidence of prenatal testing and the illegality of abortion in most cases, a social norm is rapidly consolidating to dissuade two people with sickle cell genes from marrying, or even dating. (Not all Nigerians share this attitude. A 2015 study found that one in five people who realised they were in AS-AS relationships from their premarital screenings got married anyway.) But genotype screening is now a requirement to get married in many Nigerian churches and mosques. And states in the north and the south have passed measures to mandate premarital testing. In 2020, the Nigerian Senate debated an SCD management bill that would encourage premarital testing nationwide. During these discussions, Chukwuka Utazi, a senator from Enugu State, implied that he had suffered a genotype-related breakup of his own. He understood what a painful choice it could be. But in Africa, he maintained, “we marry for children, we don’t marry for love”. Another senator chimed in: “We will not allow love to take away the best part of our marriages.”

Heterosexual Nigerian couples who come to these genetic crossroads are not merely grappling with whether to break up or get married. They are also considering what makes a good life, for themselves and for their prospective children. SCD is not a death sentence – especially in a city like Lagos – but it is usually a life sentence. Its two most effective treatments, bone marrow transplants and gene editing, are out of reach for ordinary people. Most Nigerians with SCD must deal with painful crises, procure expensive medications, seek out specialists and confront stigma, including obstacles to someday starting a family of their own. Should such people come into existence at all? The question sounds crude or even amoral, but when Nigerian carrier couples split up, their implicit answer is no.

Their concerns are also broadly relevant beyond SCD. As genetic testing becomes more common, many prospective parents, in many countries, will find themselves asking: which diseases should we test for? Which conditions truly compromise a child’s future, and which are ultimately manageable? How much can you really control? And what is worth sacrificing to be with the person you love? In Nigeria, these questions are already part of daily life.