Love in the Time of Sickle Cell Disease, by Krithika Varagur – Harper’s Magazine

Illustrations by Katherine Lam

Subomi Mabogunje fell for Nkechi Egonu within hours of meeting her in 2004, in his hometown of Ijebu-Ode, a trading hub in southwest Nigeria. They worked at a state-run broadcast TV station, thrown together by the National Youth Service Corps. He was speechless on the day Nkechi first walked into work. While Subomi was thin and bespectacled, she was petite and zaftig, 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; she was outspoken in the stations weekly news meeting, and top brass quickly promoted her to program presenter. She was the most exciting person, Subomi felt, who had ever walked into his hometown.

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. Youre too good for this kind of work, ehe? Nkechi teased, furrowing her brows. Locals discount, he joked, and she laughed. With his hollow cheekbones, frail body, and elongated fingers, he was clearly what some uncharitable onlookers would call a sicklerone of up to six million people in Nigeria with sickle cell disease, a group of inherited blood disorders that turn red blood cells from soft discs into rigid crescents, frequently leading to blood clots that can cause pain episodes, called crises, and serious complications in most major organs. But Nkechi never shied away from him. A few days later, the offices radio transmitter stopped working, so Nkechi and Subomi had nothing to do except talk. After work, they made a beeline for a local dive bar housed in a car wash. Within a few weeks, 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 hemoglobin, the oxygen-carrying protein in his blood. Nkechis 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. There was a 50percent chance that any child they had would suffer from sickle cell disease like their father. This was no light prospect. Subomis own childhood had been marred by secrecy and shame over his condition. Nkechi, meanwhile, had lost four cousins to the disease. Those deaths might be understood as products of an earlier, benighted time, when the average Nigerian knew far less about genetic testing and disease management. Today, however, there was a growing consensusparticularly in their college-educated, upper-middle-class milieuwhen it came to passing on two sickle cell genes: dont risk it.

But how, and when, do you weigh risk against attachment? Nkechi revealed her genotype just days after meeting Subomi, and he didnt stop loving her. He liked that she held her ground, didnt worry about the future, and could make even his stolid father laugh. She also had a quick temper, but he preferred her foot-stomping, door-slamming outbursts to his quieter family life, shuttling between two parents who often lived apart. But why, dude? Nkechi asked him in those early months, in her broad, Americanized diphthongs. He wasnt sure, he said. She was something that had happened to him, like a coconut dropping on your head while youre walking down the street.

By the end of her service year, Nkechi had fallen in love with Subomi, too, but she thought it shouldnt last. In March 2005, she moved back 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 thirty when she initiated their third breakup in late July 2009. She met him on a Friday evening at home and calmly told him that they had to find other people to marry before it was too late. As the oldest 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 counselor at the Sickle Cell Foundation Nigeria (SCFN), in Lagos, who had advised her to end the relationship. (Though a representative from the foundation told me that this is against their policies.) There were too many obstacles. A marriage is not just between two people, Nkechi reminded Subomi. They talked and cried for three hours as the sun set, and she kept crying all the way home on the lurching bus. They were tears of grief, but also relief. She had finally, and responsibly, severed this unwieldiest of attachments.

Subomi didnt sleep much that night. Early the next morning, he started driving to his fathers house in Ijebu-Ode. That afternoon, something told Nkechi to call him to make sure he had arrived safely. Subomis boss picked up the phone.

Is Subi there? she asked, confused.

Oh, its you, Nkechi. I was actually just about to call you, Nkechi recalls him saying over a crackling connection. Subomis had an accident.

Less than an hour outside Lagos, Subomis sedan had been rear-ended, hit a truck, and flipped over. Paramedics had taken him to the hospital and dialed the last received call on the phone they fished from Subomis pocket. Nkechis mouth went dry. At dawn, she began a long bus journey to the hospital, where she ran into Subomis mother. They found Subomi in the ICU, in a coma.

Nkechi held his mothers hand as the doctor explained Subomis 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 on Friday afternoon, inaugurating a regular weekend pilgrimage. She sat by Subomis bed all day, napping at a nursing students dorm nearby. Perhaps, his doctor offered, playing Subomis favorite songs or movies could help. Nkechi made him a mixtape with his favorite songs by Kenny G, Enya, and the Nigerian gospel singer Tim Godfrey, which she had played on a loop day in and day out. His mother started to visibly relax whenever Nkechi walked through the hospital doors. Nkechi, for her part, put their breakup out of her mind.

On Saturday, September 5, she showed up as usual and was stunned to find Subomi awake. She screamed with joy and clasped his hands.

Happy birthday, Nkechi, he told her.

Everyone near his bed turned to her and paused. Was he crazy? But it was, in fact, her thirtieth birthday; Subomi had glanced at the date on some paperwork. Nkechi started to laugh. Everyone then agreed that he could go home soon, and he was discharged a few weeks later.

Subomi still faced a long road to recovery, and he was intermittently bedridden. Nkechi substituted her weekend trips to Ibadan with visits to his mothers 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 monthsand, despite everyones concern, didnt suffer any sickle cell crises during his convalescence. When Nkechi got up to leave, he started to ask her when they were getting married.

We are broken up, she would remind him.

I have no recollection of that, he would say. Or did you forget I was in a monthlong coma? It was hard for her to gauge his sincerity.

In the new year, Subomi could not only walk but drive again, and as he regained strength, the flame of Nkechis attraction flickered. She didnt try to put it out. For once, none of their family members were asking probing questions about the future. One afternoon in August, when Nkechi was making sure Subomis bed was comfortable, he pulled a pink tourmaline ring from his pocket.

Later, Nkechis mind would cycle through the past six yearstheir chance meeting in Ijebu-Ode, those afternoons at the car-wash bar, breaking and making up in Lagos, the accident, his convalescenceand she would wonder if they were making the right choice. But in that moment, and without hesitation, she said yes.

Nigeria is the sickle cell capital of the world. Its residents account for about half of all new annual cases of severe hemoglobin disorders worldwide. Sickle cell disease is one of the worlds most prevalent autosomal recessive genetic disorders; the sickle cell trait is over 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 Fifties, a number of scientists speculated that the sickle cell trait confers some resistance to malarianow a widely accepted theorywhich would account for the prevalence of the gene in sub-Saharan Africa, home to over 90percent of all malaria cases in the world. Over millennia, per this hypothesis, as more AS than AA children survived acute malaria infections and reached reproductive age, they passed on their single S genes, too. 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 damaged blood cells in the retina, which can lead to blindness.

The heritability of sickle cell anemia, the most common and severe form of SCD, is about as straightforward as a Punnett square from a middle school biology textbook: if both parents are carriers (AS/AS), their children have a 25percent chance of having the disease. If one parent has the disease (SS) and the other has no sickle cell genes (AA), theres a 100percent chance their kids will be carriers (AS) and a 0percent 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 50percent chance of giving birth to an SS child and a 50percent chance of an AS child. Every year, a growing number of people in Nigeria consider breaking up because of this calculation.

Dr. Olufemi Akinyanju, an eighty-six-year-old Nigerian hematologist, started identifying sickle cell patients as a young doctor in Lagos in the Sixties, fresh out of medical school in London. The patients had often never heard of the disease, and he went on to spend most of his career studying it. In 1994, he founded the Sickle Cell Foundation Nigeria in Lagos, now one of the regions preeminent treatment and diagnosis centers. His generation of doctors helped SCD testing take root in Nigeria. The most common blood test is hemoglobin electrophoresis, in which electrical currents are passed through a blood sample, separating different types of hemoglobin into discrete bands. The test is now commonplace in urban centers like Lagos and Abuja. Testing in a high-end facility can cost up to forty dollars, but there are also free and cheap clinics, which can provide results in as little as thirty minutes.

More and more Nigerians are now being encouraged to test at an early age. Nkechi, who was born in 1979, has known her genotype since she was seven. Testing is less accessible outside major cities, especially in the countrys poorer northern states. And in lower-end clinics, results are often inaccurate. Ezekiel Ogbu, a thirty-six-year-old bus driver with SCD in Lagos, found this out the hard way in 2018, when he discovered that his fiance was not AA, as she had long believed, but AS. They broke up two weeks before their wedding, following her third blood test, and he informed more than five hundred guests that the ceremony was off. 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. Absent strict or uniform laws, decisions about dating often fall to individuals. Genotype screening is now a requirement to get married in many Nigerian churches and mosques, but some young people are starting higher upstreamas early as the first date. Given Nigerias emphasis on marriage, combined with 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. But not everyone makes this choice. A 2015 study found that one in five people who realized they were in AS-AS relationships from their premarital screenings got married anyway.

Some lawmakers have tried to intervene in this gray area. States in both the north and the south have passed measures to mandate premarital testing; in 2020, the Nigerian Senate debated an SCD management bill that, among other things, would also encourage premarital tests. 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 dont marry for love. (Nigeria already polices the intimate sphere: public displays of same-sex affection are illegal, for example, and punishment for same-sex intercourse can range from prison time to execution by stoning.) 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, both for themselves and for their prospective children. SCD is not a death sentenceespecially in a city like Lagosbut 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 broadly relevant to the rest of us who live in the age of genetic testing and its novel theaters of decision-making. Prospective parents find themselves asking: Which diseases should we test for? How risky is it to pass on a certain trait? Which conditions truly compromise a childs future, and which are ultimately manageable? How much can you really control? And what is worth sacrificing to be with the person you love? As genetic testing continues to spread, many more people, in many more places, will try to answer these questions. In Nigeria, they are already part of daily life.

Perched on the Bight of Benin, Lagos is home to some fifteen million people and is the center of Africas largest economy. The stretch marks of the developing world are visible everywhere: cheap cell phone data sold at hand-painted stalls, piles of refuse, strip malls that pop up overnight like mushrooms, awe-inspiring traffic, and unflinching sales-children who dash through it. The city abuts the Lagos Lagoon, where a vast slum is held up on stilts and fishermen still use dugout canoes to catch red snapper and mackerel. Sometimes, as the Nigerian playwright Wole Soyinka once wrote, one feels that the worlds discards, the detritus of the constantly surging ocean, eventually come to rest on the beaches of Lagos.

Last year, I made several trips to this amphibious megacity to meet Nigerians who have navigated dating, love, and marriage under the star of their sickle cell genotype. Its one of the likeliest places in Nigeria to have a few partners before marriage; it is also where the greatest number of Nigerians go to work. Like all cities, its filled with strivers trying to make rational decisions about their future; a 2021 study of over 1,300 city-dwelling Nigerians found that 29percent of respondents had ended a relationship due to genotype incompatibility. In this city rife with choice and its discontents, there may be more than 3.5 million people with the sickle cell trait.

Among the Lagosians I met were: several individuals who had broken up with their carrier boyfriend or girlfriend in their twenties; a forty-something couple who, upon discovering their daughter was SS, belatedly realized they both had AS genotypes; a single mother of three whose husband left her after two of their SS children started having severe crises; several older single women with the sickle cell trait who had given up on marriage altogether; and a woman in her seventies with SCD who had been happily married for over fifty years. I heard about people who had forged their genotype test results, people who hid their status from their spouses, and couples who lied to get married in their house of worship. People explained their weighty decisions to me straightforwardly, as if recalling what they had for lunch. Everyone I spoke to was religiouseither Christian or Muslim, Nigerias two major faithsand expressed the belief that any outcome, be it heartbreak, late-in-life romance, or a child with surprise SCD, had, to some degree, been willed by God.

Its not that young Lagosians are pure fatalists or cold-blooded optimizers; some do allow themselves genetically risky attachments, even if theyre not meant to last. I met Blessing Assam, a twenty-five-year-old pastors daughter and accounting student with dreams of running a large business, who has an AS genotype. Blessing has worked a series of tough jobs to put herself through university, including serving as a live-in care worker for a woman with severe SCD. She has been dating Samson, a handsome twenty-nine-year-old entrepreneur, who also has an AS genotype, for two years. They video chat every night; he speaks to her in a soft voice and lets her order anything she wants whenever they go out without glancing at the price. But both told me they will have to break up. Blessing saw severe SCD at work, and three of Samsons family members have died of the disease. They dont want to risk it. I need to wake up from my dreamworld, she told me over lunch. She made a noose gesture and cocked her head to one side.

Other Lagosians who have SCD themselves dream of a relationship as an escape to a better life. Princess Samuel, a thirty-year-old with cut-glass cheekbones and a silvery voice, grew up sharing her churchs spare room with her mother and younger siblings after her fathers death. Her family could barely afford mild painkillers, but she stoically endured frequent vaso-occlusive crises, which happen when sickled red blood cells congeal and deprive tissues of oxygen. At university, she met and fell in love with a medical student who promised he didnt care about her genotype. For three years, they kept her status a secret from his less tolerant parents. Then, in 2017, she had an acute crisis and was rushed to the hospital. Her boyfriend feared for her life and called in favors to fast-track her treatmentincluding with his mom, who was a nurse at the hospital where Princess was admitted. She helped Princess get priority treatment, and then promptly demanded that they break up. They did, and he married someone else last October.

Princess has had a few flirtations since then, but all have faded when she has been sick or hospitalized. Last spring, she quit her job and moved back in with her mother, who lives in a cinder-block house on the citys outskirts. Every Sunday, Princess wakes up as early as 4:30am to commute to The Elevation Church in Ikorodu, which has a dedicated service for unmarried congregants. Last year, she had three more vaso-occlusive crises. The most serious one, in March, left her with a hospital bill that cost nearly as much as her mothers yearly rent. SCD is expensive, both acutely and chronically: prevention regimens of vitamins, painkillers, and antibiotics (up to $30 a month, for Princess), hospital admissions (sometimes $150 per day at a private hospital), blood transfusions (often over $100). These all hit harder given Nigerias flailing economy and rapidly weakening currency. Thats not just for patients, but also for their prospective partners and in-laws. People think the cheapest is just to walk away, really, Timi Edwin, a thirty-five-year-old SCD advocate who has the disease, told me. She speaks from experience: Timis fianc left her six years ago because of her condition.

Princesss mother is still optimistic. Sure, she says, its a little harder for people with SCD to marrybut maybe Princess should also try a little harder to meet single men. They are marrying, she mused, about sickle cell patients shes met through her church. Some even to wealthy people who are able to take care of them. She went on: I believe God will give her a wealthy husband too, she said. Im praying seriously toward it.

Nkechi and Subomi got married twice: first in an Igbo wedding in her familys village in Mbaise, in the same house where her parents married, and then in a Yoruba ceremony in Ijebu-Ode. The Igbo wedding was on December 3, 2010, and Nkechi turned out, against expectations, to be a calm and happy bride. She wore a lilac crepe wrapper, tied like a skirt, and a coral-bead headdress. Subomi wore a shirt printed with roaring lions, a traditional Igbo motif. A week later, in Ijebu-Ode, Subomis cousin washed Nkechis feet as she stepped in, as a bride, to a house that she already knew so well. They skipped a third church wedding, which also let them sidestep premarital genotype testing requirements.

Nkechis sister Uche met Subomi several times when they dated, and found him strikingly thoughtful. Once, when he couldnt make it to a lunch they had planned in Lagos, he mailed her a check so she could take herself out on her interns salary. My entire familys only concern, really, was the genotype, Uche told me. I asked her several times, are you readyreally readyif you have an SS child? And was she very sure his family knew she was a carrier too? But then she did a fantastic job taking care of him after the accident, Uche said. It felt silly to keep probing. Whereas an accident like that, for me, would have been a time to reevaluate things, said Chijioke Nwamara, a childhood friend, for Nkechi it just strengthened her resolve to press ahead.

As a wedding present, Subomis parents gave them a two-bedroom house in their Lagos compound. Both families were in high spirits through Christmas, including Nkechis one-hundred-year-old grandmother, who was overjoyed that her grandchild was finally embarking on her adult life. Nkechi and Subomi didnt tell her, or anyone else, that they had decided, as a condition of staying together, not to have children.

As newlyweds, they could finally live together, and they loved their city. They spent weekends at Lagoss nightclubs and urban beaches, and went to separate churches on Sunday mornings. Sometimes, they collaborated on graphic design work. They were modern, unconventional, independent; colleagues, freelancers, best friends. But things started to change around their first anniversary. More nights were spent staying home to watch TV than out on the town. Subomi, who had grown up lonely, started to feel lonely again, like there werent enough people at home. He began twisting Nkechis arm: What if they tried for a baby? Theres got to be more to life than this, he told her. Please? Just one?

They found themselves attending their friends babies naming ceremonies and birthdays, and then Nkechis younger brothers started having children, too. Her resolve to remain child-free gradually waned. She struck up tentative conversations with doctors, who told her that the science of SCD management had advanced in leaps and bounds, and that babies born with an SS genotype could live to old age. At thirty-two, Nkechi decided to stop taking her birth control pill. Looking back, she thinks she was inviting fate to intervene in a battle of the wills between two awfully stubborn people.

Nothing happened for four years. Nkechi knew she was on the older side for pregnancy, but she started to avoid the subject with Subomi, who could slip into a dark mood when he ruminated on his unfulfilled dreams of fatherhood. But in early 2016, after days of a sore throat and nausea, a pharmacist forced Nkechi to take a pregnancy test. It was positive. When she told Subomi, he burst into tears.

But they had to think of the big question in the same breath. Testing the fetuss sickle cell genotype would require an invasive test at the Sickle Cell Foundation Nigeria, and Subomi begged her not to do it, both because the procedure posed a small risk of miscarriage and because it had taken them so long to conceive. Nkechi called family members on both sides for advice, and she remembers many of them saying the same thing: there had been vast improvements in SCD management, and anyway, a 50percent chance was far from a guarantee. A week passed as Nkechi mulled it over, during which time the fetus started to kick, and she realized she could not go through with an illicit abortion, whatever the genotype was. Well, she told Subomi, here we go. Whats the worst that could happen?

Nkechi went into labor the night of Donald Trump and Hillary Clintons last presidential debate, which she and Subomi watched in their living room. When the sun came up on October 20, he drove her to the hospital. By noon, she was admitted for a cesarean section. The baby had nestled close to her rib cage and had to be massaged out by two nurses. Nkechi woke up from the anesthesia in a cold sweat and learned that her babyher sonwas in another room, where nurses were starting him on a ventilator after he had ingested his own stool on the way out. When Nkechi saw him for the first time, he was on intravenous antibiotics. She didnt expect to feel so attached to this small personso responsible for him. They brought him home two days later. The name Subomi had picked out for him was Oluwagbotemi, Yoruba for God heard my cry. But given the complicated birth, he decided to revise his sons name to Momoreoluwa, meaning I know the grace of God. Momo, for short.

Subomi was overjoyed. He spent all day with Momo, rambling to him about current events, changing his diapers, bathing him, and watching him sleep. He started reciting Yoruba phrases well before Momo could talk, so hed become worthy of his florid name. He took to calling him his mini me. But Subomi was also on edge until they brought three-month-old Momo for a sickle cell screening. When it indicated that he was just a carrier, they were walking on air.

Seven months later, Momo woke up sobbing, with swollen hands. It was, as Nkechis mother quickly recognized, dactylitis: swelling of the fingers or toes due to blocked blood vesselsits a classic early symptom of SCD. But what about the genotype test? His fetal hemoglobin, which lingers for months, may have confused the results. His parents had been advised to bring him in for another test on his first birthday, but they had hoped it would be perfunctory. Nkechi bundled Momo into their car and drove to the hospital where hed been born. A new blood test confirmed that he was SS. That night, he started his daily program of antibiotics and antimalarial pills.

Subomi was too distressed to be of much help. Part of his grief, Nkechi knew, came from the painful memories of his own childhood, when distant relatives told him hed die before he was ten, and his parents, wary of stigma, warned him to avoid mentioning his disease. He used to sit miserably on the sidelines at soccer games and take countless days off from school. But Momos case was different from Subomis. He had been diagnosed early, life expectancies for patients had risen, and his parents knew much more about managing the disease. Subomi had never developed a solid regimen of preventive medication as a child; Momo was started on one before his first birthday. And Nkechi was going to be open about her sons condition, not hide it. You can do better for Momo than your parents did for you, she told Subomi. They were both anxious after Momos diagnosis, but after months of keeping infections at bay, they relaxed slightly. They even felt cautiously optimistic. Maybe they hadnt been totally reckless, after all, in rolling the genetic dice. Maybe the worst that could happen wasnt that bad.

The age of relatively cheap genetic testing has created a new term for those in Nkechi and Subomis situation: genetically at risk. Genetic risk does not imply resignation in the face of an implacable biological destiny, wrote the sociologists Carlos Novas and Nikolas Rose in 2000; it induces new and active relations to oneself and ones future. Such thought also reshapes prudence and obligation, because those who are genetically at risk must make the right choices, both for their own sakes and for those of their families. These actors try to exercise genetic responsibility, a concept coined in 1974 by the medical researchers Peter T.Rowley and Mack Lipkin Jr. They argued that the nascent field of genetics would compel more and more individuals to act responsibly, especially by avoiding the spread of genetic diseases. While the science may be new, they wrote, the problem is not. Whether to suffer or to not live at all has been debated since Job.

There are not yet universal standards for genetic testing, but a narrow consensus has emerged around specific conditions. The first prenatal diagnosis for Down syndrome was made in 1968. Since then, screenings have become routine for pregnant women in many parts of the world. In Iceland, for instance, the vast majority of women choose to be screened, and up to 85percent of those who learn of an increased likelihood of the disorder choose to terminate the pregnancy. This shared idea of genetic responsibility has resulted, locally, in the conditions virtual disappearance, though a few babies with Down syndrome are still born there each year. But the polarized debates around the practice show that genetic responsibility is far from a neutral, or standardized, concept. The situation in Iceland has drawn global ire, and some have argued that these abortions offer a backdoor to eugenics.

But people in societies where abortion has been illegal or discouraged have also created local norms, in which decisions are made earlier in the process of family formation, and often more communally. That was precisely the case with beta-thalassemia, another recessive genetic blood disorder, in Cyprus. In the Sixties, up to eighty babies with the condition were born there each year, and in the Seventies, local physicians began to strongly encourage carrier screening, counseling, and prenatal testing, eventually pressuring the Cypriot Orthodox Church to request premarital screening certificates. In 1986, the number of babies born with beta-thalassemia dropped to zero. Tay-Sachs disease is another example. In 1983, an ultra-Orthodox Brooklyn rabbi who lost four young children to Tay-Sachswhich causes damage to nerve cells and leads most patients to die before age fivestarted Dor Yeshorim. The organization, whose name is Hebrew for upright generation, is a premarital screening service that has rapidly become central to matchmaking in many ultra-Orthodox communities. Today, Dor Yeshorim representatives draw blood samples from Orthodox students, test them for several genetic diseases, and store the results in a confidential database. Early in a prospective match, the couple can call Dor Yeshorim, and if the individuals are both carriers for the same disease, they are simply told that the match is not advisable.

Both of these examples were enacted in relatively small, homogenous communities. But its hard to closely follow either playbook in a massive, multiethnic democracy like Nigeria. Instead, over the past half-century, Nigerians have forged their own notions of genetic responsibility.

In 1986, Dr. Akinyanju led the first trainings of genetic counselors in Lagos. In the beginning, they worked primarily with families who already had SS children, but, by the Nineties, they started to see far more prospective couples, according to Ayo Otaigbe, who was among Nigerias first class of trained genetic counselors. Over five hundred SCFN-trained counselors now work in a number of African countries where SCD is prevalent, from Ghana to Tanzania, and they are the most professionalized tip of an iceberg that includes public health campaigns, widespread testing, and rising awareness of SCD in popular culture. (Sickle cell been show me crises, the Afropop singer Adekunle Gold sang in last years single 5 Star, which he has remixed with Rick Ross. I will never forget the Nineties/Many nights I go dey beg for mercy.) The watchword of SCFN-trained counselors is non-directive: they provide information but do not compel specific action, and they especially avoid discouraging marriage or procreation. This fits with the global rise of non-directive genetic counseling in the Fifties, which emphasized voluntary measures in the shadow of Nazi atrocities. The manual used by SCFN to train counselors advises them to avoid phrases like if I were you and Im terribly sorry for you.

Sickle cell counseling never took off in the same way in the United States, even though about one in thirteen black babies are born with the sickle cell trait. In 1972, Congress passed an act that encouraged voluntary sickle cell genotype testing among black Americans, and sickle cell screening was even a part of the Black Panther Partys public health initiatives. But people started hollering genocide pretty fast, recalls Dr.Wally Smith, who now directs Virginia Commonwealth Universitys SCD program. He says the idea of a public health program directed at black Americans was hampered by widespread distrust of the government and medical authorities. Smith believes they might have given up too soon, lamenting a 2017 poll in which only 36percent of the black Americans surveyed even knew that they had an elevated risk of SCD.

Nigerian experts are split on the future of SCD in their country. In just a couple generations, awareness has skyrocketed and free clinics have proliferated. Though statistics are scarce, those working in the field believe the number of cases has fallen, especially in urban centers. But the uptake of testing and shift in social norms has been polarized along socioeconomic lines. Fewer children in middle- and upper-class homes seem to be born SS, observed Toyin Adesola, the executive director of an SCD advocacy organization. That means the disease is slowly concentrating in poorer communities. In Kano State, part of the less-developed, Muslim-majority north, prevalence of the sickle cell trait has been reported to be as high as 41percent, compared with about 25percent nationwide.

We dont see eradication as the goal, at least for now, said Dr. Annette Akinsete, the national director of the Sickle Cell Foundation. And she does not think laws to stop carrier unions will ever find a foothold. Instead, she hopes to see more widespread newborn screening so that patients can get a head start managing the condition, as well as free or subsidized antibiotics and supplements.

In high-income countries, the median survival age for a patient with SCD has risen to between forty and sixtymore than double what it was in the Seventies. In such places, SCD has evolved from a life-threatening pediatric condition to a chronic disease among adults. The average survival age in Nigeria is much lowerlikely under five, though statistics are not widely availablebecause of the high infant mortality rates in poor regions. But a middle-class Nigerian patient with access to resources, especially in Lagos, has a very good chance of managing their condition into adulthood, says Dr. Akinsete. Most Nigerian medical professionals dont see their goal as zero future SCD cases, but somewhat fewer of them, and a much higher quality of life for those born with the condition.

In the United States today, pregnant women can find out whether their unborn child has cystic fibrosis, fragile X syndrome, spinal muscular atrophy, or many other conditions. Prospective parents can quickly determine their carrier status for hundreds of disorders, from primary congenital glaucoma to maple syrup urine disease to a bone disease that increases the likelihood of fractures. People with no thoughts of parenthood, too, can get a basic genetic profile in a matter of weeks; for about two hundred dollars,23andMe can detect ones carrier status for over two dozen conditionsand also estimate the likelihood of someday developing breast cancer or Alzheimers. Without public health initiatives, its unlikely that these advancements will dramatically reduce the prevalence of any particular condition. They will, however, put more people in positions like that of Nkechi and Subomi: having genetic information in the back of their minds while dating, falling in love, and starting a familyand deciding whether theyre willing to intervene on the basis of that information.

When science moves faster than moral understanding, as it does today, men and women struggle to articulate their unease, the political philosopher Michael J.Sandel has written on parenting after the genomic revolution. In liberal societies, they reach first for the language of autonomy, fairness, and individual rights. But this framework quickly reveals its limits. In developing and developed countries alike, many families, engaging in the most optimistic of enterprises, eventually reach a point where meaningful choices evaporate, confronting all manner of shortcomings both before and after birththose stemming from health outcomes, but also those of resources, community, and chance. In the age of genetic testing, more information does not always mean fewer obstacles; conversely, it may entrench parents struggle for control. Discrete decisions tend to give way, as Sandel puts it, to questions about the moral status of nature, and about the proper stance of human beings toward the given world.

Momo grew the same whorled hair on his head that Nkechi has on her arms, but he had Subomis dark complexion, slim feet, dark lashes, and occasional indignant frown. He refused to walk until he was two, and then quickly proceeded to somersaults. And he refused to eat solid food unless Nkechi took off her shirt, as if she were about to breastfeed him; only then could she shovel forkfuls of rice into his mouth, drawing peals of laughter from Subomi. Momo cheerfully moved from lap to lap at church and was sanguine when Nkechi left him with his aunt, who disciplined him alongside her own children. He turned two without another crisis. Nkechi decided to bring him to Mbaise for Christmas in 2018. Subomi stayed in Lagos to celebrate with his mom and sister.

When Nkechi got home, she noticed something on Subomis leg as they got ready for bed: a yellowing ulcer, the size of a grape, over his left ankle. Come on, man, she said. I leave you for a week and this happens? It was nothing to worry about, he protested. It didnt even hurt. Leg ulcers are relatively common in adults with SCD. Subomi resisted going to a doctor, and Nkechi hoped it would resolve itself. Instead, it expanded to the width of a lime. Then Subomis feet started to swell, and in February, a pinprick-size ulcer opened up over his right ankle. It grew into an equally large crater by Valentines Day, when they could not make it out of the house for dinner.

On February 21, Subomi woke up at around a quarter to six, screaming in pain. A friend drove him to the hospital, where he was immediately admitted to the emergency ward. Nkechi was relieved; she had won the battle of convincing Subomi to see a doctor at all. Momo was in high spirits when he waved goodbye to his dad.

When Nkechi arrived an hour later, doctors had started Subomi on antibiotics and dressed his wounds. He told her that he would be discharged in a few days. It could have been much worse, said the doctor, and they had brought him in at the right time. Why didnt you bring me strawberry yogurt? Subomi ribbed Nkechi. You better get home soon, she warned him. Momo is waiting for you.

The next morning, Nkechi woke up earlier than usual, around 5am. She called Subomis nurse, and then his doctor, but neither picked up. She felt a surge of adrenaline. As if on autopilot, she put on a T-shirt and quietly drove to the hospital. When she got there, the doctor told her that Subomi had suffered a sudden embolism in his intestine. He was dead.

Nkechis ears started to ring.

It can happen to anyone, the doctor continued. The direct cause, in Subomis case, remains impossible to ascertain. Blood disorders like SCD are straightforward risks for blood clots, which are the primary cause of embolismsbut so are factors like age, certain medications, and high cholesterol. Its not the kind of thing that can be proved conclusively; someone with an AA genotype could have suffered the same fate.

The details didnt interest Nkechi. Her husband was dead. The doctor started expressing his sympathy, but she cut him off. She told her family, who wailed at the news, and then returned home to tell Momo, who, like his mother, was stoic. Her heart was racing and her internal monologue ran on an obsessive loop: Nkechi, whats your next move? She was now a single mother, with a son who needed intensive medical supervision. Would she relocate somewheremaybe California, where her brother lived? Would she stay where she was? Would she continue their business? There had to be a funeral next week.

Subomi was buried in Ijebu-Ode, in front of friends who came from as far as Canada. Nkechi sleepwalked through it. None of her friends or family remember her crying.

Three months after his father died, Momo had a crisis: splenic sequestration. Nkechi noticed that he had suddenly become very quiet, and his stomach was unusually bloated. He stayed in the hospital overnight and received a blood transfusion. Some time later, Subomis father called Nkechi to ask about the house he had given the couple as a wedding present; he was considering renting the place out, though he never did. In 2021, Momo had another crisis: sepsis. Its trigger was a bacterial infection of unknown provenance, and he spent ten days in the hospital on antibiotics. Nkechis mother-in-law dropped in for a brief visit on the fifth day.

When Nkechi had married Subomi a decade prior, the bride price his family paid made her, on paper, a daughter of Ijebu-Ode. But few arrangements are permanent, and today, raising Momo is largely Nkechis solo undertaking. In 2021, she withdrew Momo from his paternal grandmothers school and enrolled him in a different one two miles away. Nkechi and Momo now go to Mbaise for holidays. They rarely find occasion to test their status, by marriage and by birth, as daughter and son of Ijebu-Ode.

By the time I met Momo, shortly before his sixth birthday, the acute dramas of his diagnosis had subsided. Nkechi has told her son that he has special blood. Momo is about a meter talla little small for his age, but not by muchand his slightly distended stomach is visible under his butter-yellow school uniform. Nkechi lectures all his teachers about keeping him hydrated and avoiding intense sports. Momo isnt shy about his condition, even if he doesnt fully comprehend it. Ive been to the hospital like a hundred times! he told me cheerfully. (Nkechi brings him to a government pediatric hospital every three months.) He and Nkechi have settled into their new equilibrium. I asked him about his last crisis, when he was on intravenous antibiotics. I was on the drrrip! he exclaimed, trilling his rs. Sometimes, Momo is the one to remind his mom to give him his pills, which he crunches like candy. Money can be tight, so Nkechi prioritizes Momos medications and economizes elsewhere. As far as she knows, Momo is the only student in his kindergarten class with the disease. But some parents are weird about this, she admitted. So who knows.

Momo has a sunny temperament and speaks with the diction of someone who interacts mainly with adults. I miss the old days, he said one day, out of the blue, with my dad! Nkechi raised an eyebrow. Shes not convinced he actually remembers Subomi. Momo is an in-demand playmate at church and the ringleader of his many maternal cousins. When home alone, hes resourceful: a paper fan is a baseball bat, crumpled paper a soccer ball. One of his plastic toy soldiers is named Subomi.

The goal of SCD management, Dr. Akinyanju has written, is extending the steady-state period between crises. Momo has cleared the early-life danger zone for infections. But children with fewer resources can be far less lucky. In Lagos, I also met Tawakalitu Ayinde, a poor, unemployed single mother of four daughters, the last of whom has SCD. Nine-year-old Oluwaseyifunmi had seven strokes between 2017 and 2022; the last one, in January 2022, left one of her legs paralyzed and her speech garbled. She is still not on any regular medications.

As Momo gets older, malaria may trigger crises, but its unlikely to be fatal. Viral hepatitis will remain a lifelong risk. He might reach puberty later than his peers. Recent research has suggested a genetic component to leg ulcers, which means Momo may be at higher risk for them, so he may start wearing compression bandages. And someday, he will have to accommodate the fact that stress, like the kind that comes from relationships or work, may trigger crises too. It all seemed far away, until I noticed Momo kissing a T.rex puppet over Christmas in Mbaise. Hold on, he told me, Im doing something romantic.

Even today, while Nkechis friends and family almost universally disapprove of carrier marriages, they concede that Momo doesnt really look like a sickler. During Nkechis sisters wedding in Mbaise, a family friend, Sunny Opara, explained why he thought that educated people shouldnt risk having SS children while Momo and his cousin streaked by. I pointed out that Momos life chances were vastly better than those of previous generations with the disease, but Opara insisted that prevention is better than the cure. As Momo tore through the house, filled with four generations of people who loved him, I wondered what everyone would really prefer: a world in which genetically-at-risk Nkechi had made the supposedly responsible choice to break up with Subomi, or this one, in which she took a chance.

Someday, Momo might be a candidate for a bone-marrow transplant. The Sickle Cell Foundation Nigeria has just started evaluating patients for its first ever in-house transplant program, but so far, only a handful of well-to-do Nigerians have received the treatment, usually abroad, paying up to $500,000. Nkechi isnt counting on this. Throughout her life, her great strength, and great flaw, has been her laser focus on the present. She never wants Momo to feel the shame that racked her husband. Just three months after Subomi died, Nkechi started doing outreach again for the Sickle Cell Advocacy and Management Initiative. I know he would have supported me speaking out, she said. Though maybe thats a low bar, because he supported everything I did.

As Nkechi and Momo left church one Sunday, a male friend in the parking lot warned her about the effects of single motherhood on a young boy like Momo. Dont bring him up as a woman! he joked.

Momo stood up in the back seat and screamed, I am strong!

Nkechi snorted, and stepped on the gas. What do they know?

Read more:

Love in the Time of Sickle Cell Disease, by Krithika Varagur - Harper's Magazine