Bill S-211: Recognizing June 19 as the Canadian Sickle Cell Awareness Day

Bill S-211: Recognizing June 19 as the Canadian Sickle Cell Awareness Day

Sickle Cell Disease (SCD) is the most common genetic disease in the world. According to the World Health Organization (WHO) estimates, sickle-cell anemia affects nearly 100 million people throughout the world. Tragically, the majority of these individuals will die in childhood due to lack of basic care, and those who survive into adulthood too often face a life of chronic disability and premature death unless disease-modifying therapy can be provided. In Canada, estimated 5000 persons live with SCD and up to 25%1 of Canadians from ethnic roots in regions of the world traditionally affected by malaria may carry the trait. RECOGNITION OF SCD IN CANADA The World Health Organization (2006) and United Nations (2008) have designated the 19th day of June every year as World Sickle Cell Day (WSCD). Canada has yet to recognize this date. The province of Nova Scotia, cities of Markham and Toronto and town of Ajax (Ontario) are the only jurisdictions recognizing June 19th as WSCD. On December 8th 2015 Hon. Jane Cordy introduced Bill S-211- An Act respecting National Sickle Cell Awareness Day. This bill has passed 1st reading and is currently in 2nd reading. The World Health Organization (WHO) and the United Nations have recognized SCD as a global public health priority. The WHO therefore urges countries affected by SCD to establish health programs at the national level and promote access to medical services for people affected by the disease2 3

WHAT IS SCD? SCD or sickle cell anemia is a hereditary genetic disease characterized by the presence of abnormal crescent-shaped red blood cells. People with SCD have abnormal hemoglobin (called hemoglobin S or sickle haemoglobin) in their red blood cells. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. The abnormal shaped red cells cause a lack of tissue oxygen, which in turn can cause sudden attacks of excruciating pain, called pain crises. These pain attacks can occur without warning, with levels of pain often described as the same or worse than childbirth labour pain and cancer related pain. The red cell sickling and poor oxygen delivery can also cause organ damage. Over a lifetime, SCD can harm a person s spleen, brain, eyes, lungs, liver, heart, kidneys, joints, bones, or skin. It can cause stroke in even children as young as two years old! At the present time, hematopoietic stem cell transplantation is the only cure for SCD. Canadians affected by sickle cell disease come from diverse ethnic backgrounds - African, Caribbean, Mediterranean, Middle East, South America, and South Asia. What is Sickle Cell Trait (SCT) People with the SCT have inherited a copy of genes for normal haemoglobin from one parent and genes for sickle cell hemoglobin from the other parent. While these individuals are not sick themselves, they run the risk of passing the gene to their own children. Sickle cell trait occurs in approximately 300 million people worldwide, with the highest prevalence of approximately 30% to 40% in sub-saharan Africa3. 4

CURRENT STATE OF SCD IN CANADA If living in Canada with SCD, you, your child or family member: May not have access to comprehensive care including lifesaving treatments, drugs and knowledgeable care providers May be stigmatized and branded as drug seekers when presenting for medical treatment for vaso-occlusive pain crises, and then denied access to effective pain medications Often have quality of life that is lower than your friends and other Canadians without SCD Have a life expectancy that is significantly reduced by about 25 to 30 years compared to other Canadians without SCD May have your children born with the condition without diagnosis until after a critical window to provide life-saving treatment has passed. This is because new born screening for SCD is not universal and currently provinces of Alberta, Manitoba, Saskatchewan, Newfoundland & Labrador and Northwest territories are not screening newborns for hemoglobinopathies. May have children born with sickle cell trait, even in provinces with newborn screening, and not be aware that they, too, are at risk of having an affected child of their own WHAT WE NEED FROM YOU: Vote in support of Bill S211- An Act Respecting June 19th as the National Sickle Cell Awareness Day 5

Bill S-211 Currently in 2nd reading, Bill S-211 was introduced by Senator Jane Cordy on December 8th 2015. It is an Act respecting National Sickle Cell Awareness Day. WHY MUST BILL S-211 PASS AS LAW? Currently Canada witnesses about 120 new births yearly and number continues to rise International migration further increases the population prevalence in Canada Canada continues to lose many youths and children to preventable complications of SCD. The first step towards reducing the number of new SCD births is increasing awareness of the disease. Establishing a national awareness day will be an important step towards identifying at risk individuals, providing them preventative care, and decreasing the burden of illness that would otherwise result for both family members and the medical system as a whole. About the SCDAC The Sickle Cell Disease Association of Canada Established in 2012, SCDAC is a national patient association advocating for comprehensive healthcare system across Canada for individuals and families with sickle cell disease (SCD). Through its educational programs, the SCDAC is improving knowledge translation among health care providers and management of SCD among patients and families. SCDAC works with governments, researchers, clinicians and industry to increase awareness of SCD and advance the current care and treatments with the goal of improving the quality of life of affected individuals and their families. References: SCAGO. (March 2007). Sickle Cell Disease within the at Risk Communities. Sickle Cell Awareness Group of Ontario. Retrieved from sicklecellanemia.ca United Nations. (December, 2008). Adopting Consensus Text, General Assembly Urges Member States, United Nations System To Raise Awareness Of Sickle Cell Anaemia On 19 June Each Year. United Nations 2014, New York. Retrieved from http://www.un.org/press/en/2008/ga10803.doc.htm Tsaras, Geoffrey. Owusu-Ansah, Amma. Owusua Boateng, Freda. Amoateng-Adjepong, Yaw. (June, 2009). Complications Associated with Sickle Cell Trait: A Brief Narrative Review.The American Journal of Medicine, Vol 122, No 6. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/19393983 6

Lanre Tunji-Ajayi, President and Executive Director Sickle Cell Disease Association of Canada/Association d Anémie Falciforme du Canada (SCDAC/AAFC) Unit 34, 260 Adelaide St. E, Toronto, ON M5A 1N1 Canada E-mail: president@sicklecelldisease.ca Copy: communication@sicklecelldisease.ca