Meet Canada’s first adult cured of sickle cell anemia
Speaking softly, she has a conference room full of international medical experts hanging on her every word. There is barely a sound aside from a quiet, thoughtful voice sharing a remarkable story of pain, resilience and recovery.
Agyepong is Canada’s first adult cured of sickle cell disease. She was born with sickle cell anemia, a hereditary disease in which blood cells change into a sickle shape and block blood vessels, leading to constant and severe pain, and a risk of stroke and organ damage. It’s caused by one abnormal molecule which wraps her body in constant pain.
“It made my life miserable. There’s no other word for it,” she tells the gathering of sickle cell specialists during a conference in Banff in June 2018. They have come to hear her story and collaborate to find better ways to treat the disorder which affects about 5,000 Canadians.
At a certain point, she thought a long life no longer seemed possible.
With hundreds of hospital visits starting from the age of two, Agyepong can repeat the dates of her blood transfusions, pain medications, and emergency hospital admissions. While other young adults talked about friends, fashion and Netflix, Agyepong worried about the levels of nitric oxide in her blood to keep it flowing properly and hydroxyurea treatments, a drug to stimulate critical hemoglobin production.
“I really thought this was it, I could no longer go on like this,” she says.
“My life is different now.”
A smile breaks on Agyepong’s face. She laughs and takes a deep breath before starting to speak again to the audience. “It’s like I was given a reboot.” In the last few months Agyepong has been living a life she once thought impossible. She does all the activities she loves and no longer worries that she will faint or suffocate.
That transformation happened in Alberta, using a stem cell transplant therapy in November of 2017 that was adopted and refined at the Alberta Children’s Hospital (ACH) under Dr. Greg Guilcher, MD, an associate professor in the departments of Paediatrics and Oncology and member of the Alberta Children’s Hospital Research Institute at the Cumming School of Medicine. Agyepong was administered stem cells or specialized cells that can produce healthy hemoglobin from an infusion of her sister’s blood drawn through a process called apheresis. Not many people find a family donor whose blood is a match — but her sister was perfect.
The transplant took place at the Tom Baker Cancer Centre in Calgary under the direction of Dr. Andrew Daly, MD, who leads Alberta’s bone marrow transplant program.
It was a complete success. Agyepong’s blood tests show no signs of the disease and her story has become something of a media sensation.
“I was so surprised that my news touched people all over the world, that others were really interested in sickle cell disease and this transplant,” she says.
While children under Guilcher’s care have successfully undergone the transplant at the ACH, doctors have been hesitant to offer it to adults because of the potential for injury. As part of the procedure, Agyepong took drugs to suppress her immune system and a course of low-dose radiation. “I’m so proud of how we’ve been able to help Revée and others like her,” says Guilcher.
A world leader in our own backyard
Many with sickle cell anemia can trace their heritage to places where malaria is common. That’s because having the mutation for sickle cell also helps prevent malarial infection. Agyepong’s parents are from Ghana, a small African country. But how did Calgary become one of the leading centres in the world for advanced sickle cell therapies?
Guilcher got involved in sickle cell research several decades ago when he enrolled in a medical elective offered in Uganda, a country in eastern Africa with a high rate of the disease.
His work in Africa continues to this day through a partnership between the CSM and the Mbarara University of Science and Technology.
A dedicated clinician researcher in oncology and transplant, Guilcher recognized that he could take his cancer expertise and apply that knowledge to children with other serious blood disorders. He has been advancing stem cell transplants for children with leukemia for many years, building Calgary’s collective expertise.
He’s now working with the Children’s National Medical Center and the National Institutes of Health in the United States building on a pilot project in Calgary to treat children with sickle cell using a non-invasive therapy. It is dramatically changing how kids are cared for.
ACH is one of only two Canadian hospitals participating in the Sickle Transplant Alliance for Research (STAR), a group of transplant researchers from 20 pediatric hospitals across North America.
A first in North America, Guilcher developed a treatment curing sickle cell disease in children within a month through a transfusion. There are 18 children who have received this treatment and all are living normal lives.
Guilcher gives Agyepong a hug at the conclusion of her talk and thanks her for sharing her story. The two embrace and smile. They are connected by a disorder, but both have a vision for the future that is very bright. Agyepong is now a registered nurse and a thoughtful advocate for young ‘sickle cell warriors’ in Alberta and beyond.
What is sickle cell disease?
It’s a genetic disorder, inherited from your parents resulting in the production of atypical red blood cells. The disease varies from person to person and can change over time. But it often leads to severe episodes of pain, painful swelling of hands and feet, frequent infections, stroke, delayed growth, vision problems, organ failure and even death. Families of African, Indian or Middle Eastern origin are most likely to carry the gene.
The research by the Hematology, Oncology, Blood and Marrow Transplant Program at the Alberta Children’s Hospital is supported by community donations through the Alberta Children’s Hospital Foundation.