Lanre Tunji-Ajayi M.S.M
President/Chief Executive Officer, Sickle Cell Awareness Group of Ontario
In 2008, the United Nations recognized June 19th as World Sickle Cell Day, reflecting the global impact of Sickle Cell Disease (SCD) as an international public concern. Around the world, approximately 500,000 infants are born every year with this severe, debilitating congenital condition, and nearly half of them will die before the age of 5.
Sickle Cell Disease is most prevalent in the populations of sub-Saharan Africa and in the Maghrib, Asia (Middle-East, Arabian-peninsula, India), and Southern Europe (Southern Italy and Sicily, Greece, Turkey), and among their diaspora around the world, particularly in North, Central and South America.1
Though there is no national sickle cell disease registry in Canada, a recent study revealed that at least 3500 individuals in Ontario and 6000 people across the country live with the disease.2 Yet, individuals living with SCD have access to significantly less comprehensive care, patient support and research compared to similarly prevalent or less prevalent chronic health conditions.
What is Sickle Cell Disease?
Sickle Cell Disease is a serious inherited blood disorder where red blood cells become rigid, sickle-shaped and fragile. This causes them to break easily, leading to anemia and adhesion to blood vessel walls and blockage of blood flow. Tissues beyond the blockage become starved of oxygen and over time become irreversibly damaged.
When these blockages occur, patients often experience excruciating pain in their bones, chest and abdomen. Eventually, the blood vessels themselves become damaged and patients can then develop permanent organ dysfunction and premature death.
Sickle Cell Disease Care in Ontario
The Sickle Cell Awareness Group of Ontario (SCAGO) recently conducted a study of individuals with SCD and their caregivers on the type of care received in the emergency department or inpatient units of Ontario hospitals when presenting with vaso-occlusive crisis. The result showed that 54 per cent of the respondents (mostly adults) believed they received sub-optimal care. Those who reported receiving optimal care are predominantly those who sought care in the pediatric hospital setting.3
Sickle cell disease predominantly affects racialized Canadians, especially Black Canadians. As a result, many SCD patients continue to experience systemic racism and inequity in health care. Many Ontario care providers have limited knowledge of the disease and its presentation, which directly contributes to poor and less-than-optimal patient care. Sadly, due to the under-treatment of sickle cell disease in Canadian hospitals, many patients continue to suffer preventable complications including premature death.
Greater Awareness of Sickle Cell Disease is Essential
The ongoing challenges and barriers faced by individuals living with SCD and their families demonstrates the urgent need for greater awareness of the disease and the care that patients need and deserve. In 2017, the Government of Canada recognized June 19th as National Sickle Cell Awareness Day.
On June 3rd, 2021, a Private Member’s Bill co-sponsored by Andrea Khanjin (MPP, Barrie-Innisfil) and Rudy Cuzzetto (MPP, Mississauga-Lakeshore) has received royal assent in the Ontario Legislature. Bill 255, the Sickle Cell Disease Awareness Day and Thalassemia Awareness Day Act, also proclaims June 19th as Sickle Cell Disease Awareness Day, and May 8th as Thalassemia Awareness Day in Ontario.
According to Lanre Tunji-Ajayi M.S.M, the president/CEO of the Sickle Cell Awareness Group of Ontario, “the passing of Bill 255 is a landmark victory for the thousands of individuals in Ontario living with this complex, life-altering condition and their families, most of whom belong to Ontario’s Black and South Asian communities. By creating the much-needed awareness of Sickle Cell Disease, we can reduce stigma, remove barriers to equitable care, and bring attention to the important need of life-saving comprehensive care for patients across Ontario”.