Doctors have cured an Odisha boy of sickle cell disease by transplanting him with the partially-matched stem cells of his kid sister. The first such bone marrow transplantation (BMT) in the western region is being hailed for not just curing the boy of a debilitating disease with a half-match donor, but also opening the door of possibilities for many who can’t find full match donors.
The parents of 18-year-old Manish Bhoi, residents of Sambalpur in Odisha, are hopeful that their son will finally lead a life free from pain and uncertainty. His father Arun Kumar, an official in a private bank, said his son was detected with sickle cell disease within six months of his birth and has since lived with excruciating pain that struck parts of the body. The pain attacks had aggravated since last year.
“Every alternate day he would land up in the hospital with insufferable pain. He would wake up screaming at night as if someone was breaking his bones. He would get attacks in schools, in the middle of taking a shower, or just about anywhere,” said the father, adding that his son started suffering from depression after the disease disrupted his schooling. “He started losing hope,” said Arun.
Sickle cell disease is a blood disorder that changes the structure of hemoglobin (oxygen-carrying protein in red blood cells). The genetic defect causes red blood cells to become stiff and assume the shape of a sickle that start blocking blood flow, giving rise to severe pain, organ damage and stroke. Studies say 20% of children with the disease die by the age of 2.
BMT is the only known cure as it eliminates the genetic defect by replacing the diseased bone marrow with healthy bone marrow. But the challenge is in finding a compatible sibling, who doesn't have the genetic defect. In Manish’s case, the search started from his family and extended to registries across the US and Germany, but there was no full match. That's when doctors at Kokilaben Hospital decided to turn to his 10-year-old sister, who was a half-match but didn’t have the congenital defect.
“Recent advances have made transplants possible from half-matched brothers and sisters. Called haploidentical BMT, they offer the chance of a cure to a vast majority of sickle cell patients,” said Dr Santanu Sen, pediatric oncology consultant at Kokilaben. “Here, conditioning the patient before the transplant is crucial. Like, we had replaced all of Manish’s blood plasma with albumin to bring down antibodies and the possibility of rejection,” he said. Affordability, therefore, could be a challenge for many, said Arun, who got significant funding from his employer. The cost of the procedure is upwards of Rs 30lakh.
Dr Mamta Manglani, director of BMC-run Comprehensive Thalassemia Care, said they avoid doing haploidentical transplants because of the higher chances of complication.
"Also, there is a need to manipulate the stem cells to prevent rejection, which cost a lot, and maybe a challenge in the public sector," he said.
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