National Sickle Cell Awareness Day on June 19 highlights the need to raise awareness about sickle cell disease to improve the quality of life of affected individuals and their families.
The disease is an inherited blood disorder that can shorten a person’s life span by more than 30 years. It is caused by abnormally shaped red blood cells that can impair blood flow, causing strokes, lung disease, organ damage, inflammation and pain.
In Calgary, plans are underway to expand expertise in less-intensive bone marrow transplants to help sickle cell disease patients at centres across Canada. Dr. Greg Guilcher, MD, a paediatric cancer specialist at Alberta Children’s Hospital (ACH) and a Canadian research leader in this area, is working toward sharing this expertise globally, so children around the world can access a cure in their home countries.
“I get contacted often from families in Africa, where the disease is prevalent, desperately seeking a cure in Canada and it breaks my heart,” says Guilcher, an associate professor in the departments of paediatrics and oncology and a member of the Alberta Children’s Hospital Research Institute at the Cumming School of Medicine (CSM).
Specialists at ACH developed nation-leading expertise in administering reduced intensity conditioning (RIC) bone marrow transplants as a cure for the disease. This technique involves low-dose radiotherapy, immunotherapy and notably no chemotherapy to suppress the child’s immune system before infusing healthy donor cells.
Patients who have a matched sibling donor are able to tolerate the RIC transplant with few effects. Calgary is leading the way in this form of treatment, and sharing the knowledge with other centres around the world.
To date, 22 children have been cured of sickle cell disease in Calgary. Last year, an Alberta woman was one of the first adults in Canada to be cured of sickle cell anemia through a stem cell transplant at Alberta Health Services’ Tom Baker Cancer Centre.
Building partnerships and programs to help others
The World Health Organization and the United Nations have recognized sickle cell disease as a global health priority. More than 300,000 children are born with the disease each year in Africa, affecting millions.
In some parts of sub-Saharan Africa, 50 per cent of children with sickle cell disease die by age five, says Guilcher. Various supportive measures can make a dramatic difference in terms of life expectancy and quality of life, but they are not readily available there.
Guilcher and his colleagues are promoting partnerships that will make sickle cell disease education and expertise available to countries in need, where resources are scarce, including the Eastern and sub-Saharan regions of Africa.
Among the steps they are taking to create and strengthen bridges between UCalgary, ACH and program participants in Africa is the creation of a new global working committee for bringing bone marrow transplants to the region. The committee includes participants from Africa, Europe, Canada and Brazil.
Guilcher is also the international study lead for the new Project Sickle Cure, an initiative through the Sickle Cell Transplant Advocacy and Research Alliance (STAR) that plans to establish sickle cell disease’s only data and bio-specimen bank for curative therapies.
“I started my work with bone marrow transplants with sickle cell disease at a time when there has been a large migration of African families to Calgary over the past decade,” says Guilcher. “It has created an opportunity to help others.”
The Global Health and International Partnerships unit at the Cumming School of Medicine is leading the way for many Canadian institutions in a comprehensive and holistic approach to health that is global and local. It works to raise awareness of the importance of global health by providing opportunities for global health education, research and capacity-building.