They lost three daughters to sickle cell. Can they save a fourth?

The hip pain from sickle cell disease was so intense that Sadiya Haruna, age 9, could not walk, so she rode on her sister’s back. She settled among dozens of children and mothers on a concrete bench outside a clinic here in northern Nigeria.

Her anguished mother, Mariya Haruna, sitting beside her, had already seen sickle cell squeeze the life out of three of her daughters. She was frightened that Sadiya was hurtling toward the same fate.

Sadiya’s mother was determined to ease her daughter’s bouts of agony, caused by crescent-shaped — or sickled — blood cells clogging her vessels. And she hoped the staff of the pediatric sickle cell unit at Murtala Mohammed Specialist Hospital in Kano would have answers. As a public hospital, it is the only place many families can afford to take their children to get treated for a disease that can cause severe pain, organ failure and strokes.

As Sadiya waited to be seen on a sweltering July day in 2019, families filed into the clinic. In one corner, nurses scribbled down patients’ vital information. In another, children tested their motor skills — hopping on one foot, holding out their arms. In yet another, a doctor, sitting in a torn office chair, evaluated children with bloated stomachs, flaccid limbs and labored breathing. The clinic represents progress in battling the disease in Nigeria. Thirty years ago, a single nurse ran it. Now it has a staff of seven.

Just three years ago, nurses still used mercury thermometers and had no equipment to take a child’s blood pressure. Thanks in part to philanthropic funding and research, a better-trained staff now collects those vital signs and can detect whether a child is at high risk of suffering a stroke.

“When you compare what we offer sickle cell patients to what a lot of other hospitals offer, we have gone very far,” said Dr. Safiya Gambo, the head of pediatrics there.

 

Mariya Haruna holds a picture of her daughter Zaliha, left, who is studying to be a nurse, and another daughter, Aisha, who died of sickle cell in 2008, in Kano, Nigeria, July 27, 2019. (KC Nwakalor/The New York Times)

Still, there are not nearly enough doctors to provide individualised care to the roughly 18,000 pediatric sickle cell patients who are enrolled there. And the children’s fate often depends on relatively inexpensive drugs that families often struggle to afford, and screenings and treatments that can be difficult to access.

Africa is the global epicenter of sickle cell disease, a genetic blood disorder that, while relatively rare, is devastatingly familiar across the continent. In Nigeria, where research suggests sickle cell is most prevalent, roughly 150,000 children are born with the disease each year. In the United States, in contrast, about 100,000 people suffer from it, most with African ancestry.

In this oil-rich nation, plagued by underinvestment in health care and deep income inequalities, doctors and nurses battle to keep children alive. Nearly 1 out of 2 children in Nigeria with sickle cell dies before his or her fifth birthday, according to a study in The Lancet in September that examined births and deaths from 2003 to 2013. An estimated 35,000 children younger than age 5 died from sickle cell each of those years.

Haruna Shaaban, a pediatric nurse at a sickle cell clinic in Kano, Nigeria, July 26, 2019. (KC Nwakalor/The New York Times)

Simply screening all newborns for sickle cell could greatly reduce premature deaths — but such tests remain rare in Nigeria and across Africa. Likewise, a 12-cent-a-day regimen of three drugs could slash the death toll, but that, too, is often out of reach in Nigeria, where 2 out of 5 people live on less than $1.90 per day.

On the other hand, the United States, one of the world’s wealthiest nations, has the technologies and capacity to better care for people with the disease. And life expectancies for them have improved in recent decades, as Americans with the disease live into their 50s. Still, the American health care system, marred by stark racial inequities, often fails to provide basic care for people with sickle cell.

“The disparity in survival in sickle cell patients in high-income versus low-income countries is injustice,” said Dr. Shehu Abdullahi, an associate professor of pediatrics at Aminu Kano Teaching Hospital and Bayero University in Kano.

The inconsistent income that Sadiya’s parents earn is not always enough to pay for her medical needs. The family sometimes has to choose medicine over food. During almost weekly pain crises, she wails through the night and struggles to walk. Her mother worries that she may end up like the daughters she lost at ages 9, 7 and 6.

“I’m always scared whenever she says she’s sick,” said Haruna, 46. “I feel like she’s going to die like the other ones.”

Prevalent but overshadowed

The disease seemed to strike their daughters out of nowhere.

Khadija, 9, had suffered a fever for a few days. It didn’t seem severe until she closed her eyes forever on a Friday in 2003.

Two months later, 7-year-old Sadiya was sucking on sugar cane one morning and seemed to be recovering from a fever. But her temperature suddenly spiked, and she was gone before her parents could get her to the hospital. (Her parents gave her name — and those of their other daughters who died — to daughters they have since had.)

Five years after that, Aisha, 6, was at Murtala Mohammed for a mild fever and doctors ordered her to get a blood transfusion. The hospital was full, and she succumbed on the way to another one.

Accurate data on the prevalence and deadliness of sickle cell in Nigeria and other African countries is scarce — but the disease’s devastation is clear in experiences like these. Most newborns are not screened for the disease because of cost and logistical challenges, said Dr. Obiageli E. Nnodu, the director of the Center of Excellence for Sickle Cell Research and Training at the University of Abuja in Nigeria.

Those who suffer from sickle cell are often stigmatised. And it is also often overshadowed by other devastating diseases including malaria, HIV and pneumonia.

“I’m sorry to say, sickle cell disease, nobody wants to pay any attention,” said Hadiza Danisa, a nurse who ran the sickle cell clinic at Murtala Mohammed from 1991 until retiring this year.

Hadiza Danisa, a nurse who ran the sickle cell clinic from 1991 until this year at Murtala Mohammed Specialist Hospital in Kano, Nigeria, July 25, 2019. (KC Nwakalor/The New York Times)

Yet for Danisa and her staff, sickle cell has become their lives — two nurses continued going to the clinic during the strict pandemic lockdown so they could provide care.

Sadiya was 1 year old when her parents first took her to be screened there and has returned ever since for her regular medical care.

The benefits of early screening are clear.

Nnodu recalled two babies born around the same time last year at a hospital in Abuja. Both were screened and found to have sickle cell. One family accepted treatment, while the other insisted their child was not sick.

The child who received care celebrated her first birthday in October. The other baby died within weeks.

Reasons for hope

Ten years ago, Dr. Michael R. DeBaun, a professor of pediatrics at Vanderbilt University who specialised in treating strokes in children with sickle cell, visited Nigeria at the invitation of a Nigerian-born pediatrician. During a two-day retreat for girls with sickle cell, DeBaun determined that six of them had suffered a stroke.

“We were devastated and really said, ‘We have to figure out a way to address this problem in a systematic way,’” he said.

That was the spark for three yearslong studies initiated in Kano. They established that the risk of strokes was greatly reduced in children with sickle cell if they took a moderate daily dose of hydroxyurea, an inexpensive, generic chemotherapy drug.

The studies, funded by the National Institutes of Health, helped persuade the state governments of Kano, Kaduna and Katsina to provide free hydroxyurea to children with sickle cell at high risk for strokes. The drug is made in Nigeria, by Bond Chemical, a locally owned company, which started producing it several years ago as a less expensive alternative to imports.

The studies also brought a vital resource to the region: DeBaun’s team provided 18 Transcranial Doppler ultrasound machines to several medical facilities. The devices, rare in Nigeria, have long been used in the United States to detect high-risk stroke patients, though the American medical system often fails to screen children who need them.

In Nigeria, there is now more hope for detecting the disease early. New blood screening tests that require only a finger prick and cost less than $2 each have brought the possibility of testing every newborn for sickle cell disease closer to reality.

There are also efforts to prevent the births of children with the disease.

Increasingly, Nigerians are being urged to get a simple blood test to see if they carry the mutation to a single gene that puts them at risk of passing on the disease to their children. If both parents carry the mutation, there is a 1 in 4 chance that their children will inherit it from both parents and have sickle cell disease.

It’s common for Nigerians to consider whether a potential romantic partner has the mutation before moving forward with a relationship. Many clinicians and religious leaders discourage marriages of people who both carry the sickle cell trait. Some states have passed laws requiring couples to get genetic testing before they marry.

And in some clinical settings, the training and technology for treating children with sickle cell have vastly improved.

Murtala Mohammed has access to two Transcranial Doppler ultrasound machines because of DeBaun’s study, and nurses are now trained to detect strokes through physical exams. Through the ultrasounds and physical exams, nearly 650 children since 2016 have been identified as at high risk for strokes or as having had one and been placed on hydroxyurea.

But significant challenges remain.

Sadiya, now 11, arrived at the clinic with her mother last month for a routine checkup and treatment of her leg, which was hurting badly. They got in line with about 50 other families. Nearly two hours later, she was finally able to see a nurse.

In the United States, sickle cell patients with pain as intense and chronic as Sadiya’s are routinely given narcotics and prescribed hydroxyurea, which has been shown to reduce pain episodes.

But at Murtala Mohammed, most children who get hydroxyurea are at high risk of stroke — which Sadiya is not. There aren’t enough doctors to prescribe the drugs more widely, and to monitor the dosage and impact. And patients’ histories are difficult to track because medical records are handwritten and kept in green file folders.

“No matter how well meaning the families are and how dedicated the staffs are, there’s a resource limitation that cannot be overcome,” said DeBaun, who is working to increase the capacity to treat children with sickle cell in Kano through Vanderbilt’s fundraising efforts.

So the advice that Sadiya’s mother got last month was to take her daughter to an orthopedist, which she had already done, and give her ibuprofen.

A family’s fight

Sadiya’s Islamic school was throwing a celebration of Mawlid, the birth of the Prophet Muhammad, and she could not have been more excited. She put on a purple wrap dress with gold and blue diagonal stripes bought for the occasion. She painted her hands and feet with henna. She was bright and buoyant and ready to go.

But then a pain crisis struck.

“That is all! The day is over for my innocent daughter!” Haruna recalled exclaiming on that day in November.

And so began another struggle to get Sadiya’s pain under control.

Two years ago, Sadiya was coming off an eight-month hospital stay, with pain so bad that she hadn’t walked in a year. Her challenges remain.

One moment she is joking and bouncing around with her siblings. The next she is writhing in agony, her mouth in a frown, her lips twitching. She walks with a limp.

Caring for her is a family enterprise.

When pain crises hit, her mother and siblings drop what they are doing to massage her, give her medicine — and sometimes rush her to the emergency room.

The family’s modest concrete home is nestled along a narrow dirt road on the western end of Kano, a sprawling metropolis of 4 million people.

The family had long struggled to pay for Sadiya’s medical care, and it has only gotten harder because of the pandemic. Her father, Haruna Aliyu, 60, lost his stable income selling kerosene and now makes a meager living loading trucks. Her mother sells clothes and picks up odd jobs. Her older brother, Abubakar Aliyu, moved to Dubai, where he cares for parrots on a farm to make money to send home.

Sadiya has been prescribed the 12-cent-a-day, three-drug regimen that is standard for children with sickle cell in Nigeria: folic acid, penicillin and an anti-malarial. But she sometimes misses doses when money runs out, especially when she is taken to the ER and needs painkillers, lab tests and other treatments the family can’t afford. Her parents have held off on getting surgery on her leg, recommended by orthopedists, in part because of the cost.

“I dare not stay idle or risk losing my beloved daughter,” Haruna said. “We owe her this responsibility as parents.”

Haruna, an observant Muslim who wears a gentle expression, is strong-willed and optimistic, constantly referring to God’s grace when discussing Sadiya’s condition. Yet she has no illusions about the toll of sickle cell. When her husband said in an interview that he wanted to have more children, she firmly said that they would not.

“My mother’s condition worries me a lot because every ounce of strength in her has been drained both financially and emotionally because of my sibling’s health condition,” said Zaliha Haruna, 20.

So Zaliha has decided to take up some of the burden: She’s studying to be a nurse.

“Sadiya is a strong and brilliant girl with a positive mind,” Zaliha said, sobbing. “But she always falls sick and suffers a lot. I assume she is going to die, too.”

For now, Sadiya enjoys the little things in life. She watered a plant in a patch of dirt in the family’s courtyard one afternoon last month. “I like flowers,” she said smiling, adding what she hoped for out of life:

“I just want to be all right.”