They Lost Three Daughters to Sickle Cell. Can They Save a Fourth?

Sadiya Haruna, age 9, was unable to walk because of the hip pain from her sister's disease. She was among a group of children and their mothers on a bench outside a clinic.

Her anguished mother, Mariya Haruna, was sitting beside her, and she had already seen the effects of the disease on three of her daughters. She was afraid that Sadiya was about to go the same way.

The mother of Sadiya was determined to ease her daughter's bouts of agony caused by crescent-shaped blood cells. She hoped the staff of the children's unit at Murtala Mohammed Specialist Hospital would have answers. Many families can't afford to take their children to the public hospital because it is the only place they can afford to get treated for a disease that can cause severe pain, organ failure and strokes.

Families waited for Sadiya to be seen on a hot July day. In one corner, nurses wrote down vital information. Children were testing their motor skills by hopping on one foot. A doctor sitting in a torn office chair evaluated children with bloated stomachs, flaccid limbs and labored breathing.

The clinic shows progress in the fight against the disease. Thirty years ago, a single nurse ran it. There is a staff of seven.

Three years ago, nurses still used mercury thermometers and had no equipment to take basic information like a child's blood pressure. Thanks to philanthropic funding and research, a better-trained staff now collects those vital signs and has equipment to detect whether a child is at high risk of suffering a stroke.

The head of pediatrics at Murtala Mohammed said that they have gone far when it comes to offering patients with the disease.

There aren't enough doctors to provide individualized care for the 18,000 children who are there. The fate of the children is dependent on drugs that families can't afford, and screenings and treatments that are hard to access.

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Mariya Haruna and her daughter, Sadiya, went to the clinic to get their legs checked out. She has been receiving care there since she was a baby.

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The clinic has 18,000 children and has benefited from improved technology and training. There are still challenges.

Africa is the epicenter of a genetic blood disorder that is devastatingly familiar across the continent. 150,000 children are born with the disease in Nigeria each year, according to research. Most of the 100,000 people who suffer from it in the United States have African ancestry.

Doctors and nurses battle to keep children alive in this oil-rich nation, which is plagued by underinvestment in health care and deep income inequalities. A study published in The Lancet in September found that nearly one out of two children in Nigeria with the disease die before their fifth birthday. 35,000 children under 5 years old died from sickle cell each year.

Screening all newborns for sickle cell could greatly reduce premature deaths, but it's rare in Nigeria and across Africa. In Nigeria, where two out of five people live on less than $2 per day, a 12-cent-a-day regimen of three drugs is often out of reach.

The United States, one of the world's wealthiest nations, has the technologies and capacity to better care for people with the disease. Americans with the disease live into their 50s, as life expectancies have improved in recent decades. The American health care system often fails to provide basic care for people with sickle cell.

Dr. Shehu Abdullahi, an associate professor of pediatrics at the Bayero University in Kano, said that the disparity in survival in sickle cell patients in high-income versus low-income countries is injustice.

Sadiya's parents don't always have enough money to pay for her medical needs. The family has to make a decision. She struggles to walk during almost weekly pain crises. Her mother is worried that she will end up like her three daughters who died.

Ms. Haruna said she was always scared when she said she was sick. She is going to die like the other ones.

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Mariya Haruna has a photo of her two daughters, Zaliha, 20, who is studying to be a nurse, and Aisha, 6, who died of a blood disorder.

Their daughters seemed to be struck by the disease.

The 9-year-old had a cold for a few days. She closed her eyes on a Friday in 2003 and it didn't seem as bad.

After two months, 7-year-old Sadiya seemed to be getting better and was sucking on sugar cane. She was gone before her parents could get her to the hospital. Her parents gave her name and those of her other sisters who died to daughters they have since had.

After five years, a doctor ordered a blood transfusion for the 6-year-old girl. She died on the way to another hospital.

The prevalence and deadlines of the disease in Nigeria and other African countries are not easy to find. Most newborns are not screened for the disease because of cost and logistical challenges, according to the director of the Center of excellence for Sickle Cell Research and Training.

People who suffer from the disease are stigmatized. Malaria, H.I.V. and pneumonia are some of the diseases that are overshadowed by it.

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A nurse is examining a patient. The clinic used to have a single nurse, but now there are seven.

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Hadiza Danisa was a nurse and ran the clinic for a decade. She did it by herself when she started, but now there are other people involved.

Hadiza Danisa, a nurse who ran the sickle cell clinic at Murtala Mohammed from 1991 to this year, said that nobody wants to pay attention to the disease.

Ms. Danisa and her staff have become dependent on the two nurses who went to the clinic during the Pandemic to provide care.

When Sadiya was a year old, her parents took her to be screened there and she has returned ever since for her regular medical care.

There are clear benefits to early screening.

Two babies were born at the same hospital last year. Both were found to have the same disease. One family accepted treatment while the other insisted their child was fine.

The child who received care celebrated her first birthday. The other baby was dead within weeks.

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A doctor at a university examined a boy at Murtala Mohammed. He came to Nigeria a decade ago and ran trials that helped improve care for patients with the blood disorder.

Ten years ago, a Nigerian-born doctor invited a professor of pediatrics at the University of Tennessee to visit Nigeria to treat strokes in children with a rare blood disorder. Dr. DeBaun found that six of the girls had suffered a stroke during the retreat.

Dr. DeBaun said that they had to figure out a way to address the problem in a systematic way.

Three yearslong studies were initiated in Kano after that. They found that the risk of strokes in children with sickle cell was greatly reduced if they took a moderate daily dose of hydroxyurea.

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. Bond Chemical started making the drug in Nigeria several years ago as a less expensive alternative to foreign imports.

Several medical facilities in the region received 18 Transcranial Doppler ultrasound machines from Dr. DeBaun's team. In the United States, the devices have been used to detect high-risk stroke patients, but the American medical system often fails to screen children who need them.

There is hope for detecting the disease early in Nigeria. New, easy to administer blood screening tests that only require a finger and cost less than $2 have brought the possibility of testing every newborn for sickle cell disease closer to reality.

Efforts are made to prevent the births of children with the disease.

A simple blood test that can tell if a person carries a single gene that puts them at risk of passing on the disease to their children is becoming more and more popular. One in four children will inherit it from their parents if they carry the same genetic abnormality.

It is common for Nigerian people to consider whether a potential partner has the same genes as them. People with the same trait are discouraged from marrying each other. Some states require couples to get genetic testing before they get married.

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A research nurse at Murtala Mohammed is giving a Transcranial Doppler Scan to a young girl to see if she is at high risk for having a stroke.

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Parents are waiting for their children to be seen at the clinic.

The training and technology for treating children with a blood disorder has vastly improved.

Dr. DeBaun's study gave Murtala Mohammed access to two Transcranial Doppler machines, and nurses are now trained to detect strokes through physical exams. Since the beginning of the year, nearly 650 children have been identified as high risk for strokes or had one placed on hydroxyurea, through the use ofechocardiography and physical exams.

There are still significant challenges.

A month ago, Sadiya and her mother went to the clinic for a checkup and treatment of her leg, which was hurting badly. They were in line with many other families. She was able to see a nurse nearly two hours later.

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

Most children who get hydroxyurea are at high risk of stroke at Murtala Mohammed. There isn't enough doctors to prescribe the drugs more widely and to monitor the impact on patients. Medical records are handwritten and kept in green file folders.

Even though the families are well meaning and the staffs are dedicated, there is a resource limitation that cannot be overcome.

The advice that Sadiya's mother received last month was to take her to an orthopedist and give her some medicine.

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Sadiya was given a special dress by her mother for the school celebration of the birth of the Prophet Muhammad. She was unable to attend the event because of a pain crisis.

She was very excited to attend the celebration of the birth of the Prophet Muhammad. She wore a purple wrap dress with gold and blue diagonal stripes. She painted her hands and feet. She was ready to leave.

When the time came to leave, Sadiya was frightened. There was a pain crisis.

That is all! The day is over for my daughter. Ms. Haruna exclaimed on that day.

And so began another struggle to get Sadiyas pain under control.

Two years ago, Sadiya was coming off an eight-month hospital stay, and she hadn't walked in a year. Her challenges are not over.

She is bouncing around with her siblings. The next she is in pain, her mouth agape. She has a limp.

A family enterprise is caring for her.

When she is in pain, her mother and siblings give her medicine and sometimes rush her to the emergency room.

The family's modest concrete home, built around a small courtyard, is nestled along a narrow dirt road on the western end of the city of four million people.

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While sitting in the courtyard of the family's home in Kano, Sadiya got her head wrapped by her older sister Zaliha.

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Ms. Haruna rubbed Sadiya's legs and feet with a balm.

The family has been struggling to pay for medical care for a long time, and it has only gotten harder because of the Pandemic. Her father, Haruna Aliyu, 60, lost his income selling kerosene and now makes a living loading trucks. Her mother is a clothes seller. Abubakar Aliyu, her older brother, moved to the United Arab Emirates to care for parrots on a farm.

The standard regimen for children with sickle cell in Nigeria is a 12-cent-a-day, three-drug regimen that includes penicillin, an anti-malarial and folic acid. She misses her doses when the family can't afford the treatments she needs. Her parents have held off on the surgery because of the cost.

Ms. Haruna said that she risked losing her daughter. We owe her this responsibility as parents.

Ms. Haruna, a Muslim who wears a gentle expression, is always optimistic and willed when talking about Sadiya's condition. She doesn't have any illusions about the toll of the cell. She said that they wouldn't have more children when her husband said he wanted to.

Zaliha Haruna said that her mother's condition worries her a lot because every ounce of strength in her has been drained because of her sibling's health condition.

Zaliha is studying to be a nurse.

Zaliha cried as she said that Sadiya is a strong and brilliant girl. She suffers a lot and always falls sick. I think she is going to die as well.

Sadiya likes the little things in life. She watered a plant in a patch of dirt in the courtyard. She said that she hoped for out of life to be flowers.

I just want to be okay.

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Sadiya is waiting to be seen. Her chronic pain causes her to go to the hospital frequently.