A rural Ugandan community is a hotspot for sickle cell disease. But a patient gives hope

MBALE, Uganda – Barbara Nabulo was one of three girls in her family. But when a sister died, her mother lamented at the funeral that she was left with only one and a half daughters.

One half was the sickly Nabulo, who at the age of 12 understood what her mother meant.

“I hated myself so much,” Nabulo said recently, recalling the words that preceded a period of illness that left her hospitalized and fed through a tube.

The scene highlights the lifelong challenges for some people with sickle cell disease in rural Uganda, where the disease remains poorly understood. Even Nabulo, despite knowing how the disease weakens the body, repeatedly spoke about “the germ I was born with.”

Sickle cell disease is a group of inherited diseases in which red blood cells – normally round – become hard, sticky and crescent-shaped. The deformed cells obstruct blood flow, which can cause infections, excruciating pain, organ damage, and other complications.

The disease, which can stunt physical growth, is more common in malaria-prone regions, particularly Africa and India, because having the sickle cell trait helps protect against severe malaria. Global estimates of how many people have the disease vary, but some researchers put the number at between 6 million and 8 million, with more than 5 million living in sub-Saharan Africa.

The only cure for the pain that sickle cell disease can cause is a bone marrow transplant or gene therapies like the one commercially approved by the U.S. Food and Drug Administration in December. A 12-year-old boy last week became the first person to enter therapy.

These options are beyond the reach of most patients in this East African country, where sickle cell disease is not a public health priority despite the burden it places on communities. There is no national database of sickle cell patients. Funding for treatment often comes from donor organizations.

In a mountainous part of eastern Uganda that is a hotspot for sickle cell disease, the main referral hospital cares for hundreds of patients who arrive from nearby villages to collect medicines. Many are given doses of hydroxyurea, a medication that can reduce periods of severe pain and other complications, and researchers are studying its effectiveness in Ugandan children.

Nabulo, now 37 years old, is one of the hospital’s patients. But she also approaches others like her as a caregiver.

After dropping out of elementary school, she emerged in recent years as a counselor to other patients, speaking to them about their survival. Encouraged by hospital authorities, she makes weekly visits to the ward which has many children cared for by exhausted-looking parents.

Nabulo says she was diagnosed with sickle cell disease at two weeks old, but is now a mother of three, including twins.

This message gives hope to those who feel discouraged or worried that sickle cell disease is a death sentence, said Dr Julian Abeso, head of pediatrics at Mbale Regional Referral Hospital.

It is known that some men divorce their wives – or neglect them when looking for new partners – when they discover that their children have sickle cell disease. Frequent deaths in the community due to complications from the disease reinforce the perception of it as a scourge.

Nabulo and health professionals call for opening up and testing children for sickle cell anemia as early as possible.

Abeso and Nabulo became close after Nabulo lost her first baby hours after giving birth in 2015. She cried in the doctor’s office as she spoke of her desire “to have a relative that I can call my own, a descendant that can help me,” she recalled. Abeso.

“At that time, people here were very negative about sickle cell disease patients having children because the complications would be so many,” the doctor said.

Nabulo’s second attempt at having a child was difficult, with some time in the ICU. But her baby is now a 7-year-old boy who sometimes accompanies her to the hospital. The twins came last year.

Speaking outside the one-room house she shares with her husband and children, Nabulo said many people appreciate her work despite the numerous indignities she faces, including unwanted glances from people on the streets who point out the woman with the “big head.” ”, a manifestation of the illness in her, her siblings often behave as if they are ashamed of her, she said.

She once heard about a girl in her neighborhood whose grandmother made frequent visits to the clinic because of an undiagnosed illness in the child. The grandmother was hesitant to test the girl for sickle cell anemia when Nabulo first asked her. But tests later revealed the disease and now the girl is receiving treatment.

“I return to Nabulo for help because I cannot control the disease that affects my grandson,” said Kelemesiya Musuya. “She may feel pain and starts crying, saying, ‘It’s here and it’s getting bigger and it’s hurting here and here’.”

Musuya sometimes seeks reassurance. “She asked me: ‘Even you, when you’re sick, does your legs, chest, head hurt?’ I tell her this, yes, it’s painful like that,” Nabulo said.

Nabulo said he was happy because the 11-year-old girl still goes to school.

The lack of formal education is detrimental to Nabulo, who has difficulty writing her name, and is a source of shame for her parents, who repeatedly apologize for letting her drop out of school while her siblings studied. One brother is now a doctor and runs a clinic in a town not far from Nabulo’s home.

“I’m so happy to see you,” said her mother, Agatha Nambuya.

She remembered the swelling of Nabulo’s head and limbs when he was a baby and how “these children used to die so young.”

But now she knows other people with sickle cell disease who have become doctors or whatever they wanted to be. She expressed pride in Nabulo’s work as a counselor and said her grandchildren make her feel happy.

“At that time,” she said, remembering Nabulo as a child, “we didn’t know.”

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