An Edmonton woman appears to be the first adult in Canada to be cured of sickle cell disease through a stem cell transplant, giving her hope of a new life without the life-threatening and painful disease.
Sickle cell disease is a genetic condition in which the body’s normally round red blood cells become sticky and shaped into crescents. The deformed cells are unable to properly carry oxygen through the body and impede blood flow.
There is no cure for the disease but researchers have been having success with offering stem cell transplants to children with the disease. The treatment is intense and involves destroying a patient’s bone marrow by giving them high doses of chemotherapy-like medications, and then replacing their cells with healthy marrow from a donor.
Because the process suppresses a patient’s immune system, the treatment leaves them at risk of potentially fatal infections.
Agyepong’s sister Stephanie Amoah read about stem cell transplants in children and wondered whether Agyepong might qualify too. Agyepong wanted to know whether Stephanie, who doesn’t have the disease, might be able to be a donor. So they underwent compatibility testing called HLA tests. “We hoped for the best, crossed our fingers, and then, on her birthday – which is crazy — we got the best news ever; that she was a 10 out 10 match,” Agyepong told CTV Calgary. In November, Agyepong underwent the complicated procedure at the Tom Baker Cancer Centre under the direction of Dr. Daly. The procedure has proven successful.
“Over the past few months, what we’ve seen is that Revée’s sister’s bone marrow has taken over the production of Revée’s red blood cells,” Dr. Daly said in a statement.
Blood tests confirmed the amount of sickle cell hemoglobin in her bloodstream is down almost to zero, essentially making her sickle cell disease-free. Agyepong still needs to take anti-rejection drugs, which leaves her immune system compromised, and the side effects of the drugs are expected to persist for another year. But Agyepong says she no longer experiences episodes of pain and exhaustion.