Alberta woman cured of sickle cell disease thanks to sister’s stem cells
Published Wednesday, April 4, 2018 8:29AM EDT
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.
That puts patients at risk of stroke, organ damage, and early death. It also causes “crises” of severe pain that send many patients regularly to hospital.
For Edmonton resident Revée Agyepong, 26, the disease had her living in a world of pain.
“Simple things for everyone else, like going swimming, for me, could end up in a sickle cell crisis just because of the cold temperature of the water, or changes in temperature,” she told CTV Calgary on Tuesday. “I even went skating one time in Banff and ended up in the hospital later that day.”
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.
While several children have successfully undergone the transplant, doctors have been reticent to offer it widely to adults, since their immune systems tend to be less resilient than children’s.
Agyepong’s sister Stephanie Amoah read about stem cell transplants in children and wondered whether Agyepong might qualify too.
“We were like, ‘Yes, this is exciting. There's got to be a way, a way for her to finally get rid of this pain and have a better quality of life,” Amoah remembers. “But then when we looked into it, they said she's too old.”
Agyepong was undaunted and reached out to Dr. Andrew Daly, who leads Alberta’s bone marrow transplant program, trying to convince him that she could handle the treatment.
“When Revée approached us, we had coincidentally been thinking about adult stem cell transplant for sickle cell disease, based on the remarkably good outcomes that Alberta Children’s Hospital has been seeing with transplants in the pediatric population,” Dr. Daly said in a statement.
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.
On Tuesday, 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.
“I can see the light at the end of the tunnel,” she said.
Her longtime hematologist, Dr. Bruce Ritchie, is also excited about what Agyepong’s success means for other patients.
“This is great news and has led to a big change in how we practice,” he said in a statement. “We are now trying to move ahead with transplants for other suitable patients with matched sibling donors.”
With files from CTV Calgary’s Kevin Green