Statement made on 22 June 2011 by Senator Joan Fraser
Hon. Joan Fraser:
Honourable senators, Monday of this week was Sickle Cell Awareness Day. Sickle cell disease is a painful, incurable and deadly genetic blood disorder that affects about 100,000 people in North America. It is one of two main hemoglobin disorders along with thalassemias, which are also inherited. Worldwide, more than 300,000 babies are born with these disorders each year.
Sickle cell disease affects red blood cells, which change from their normal ovoid shape to a long, narrow crescent, or sickle, shape. The changed cells adhere to the walls of blood vessels, where they block the normal flow of blood. Sickle-cell anemia occurs because the cells do not live long and the body cannot replace them fast enough.
People with sickle cell disease are extremely vulnerable to infections and can suffer brain and lung damage. They have periodic health crises that cause great pain and difficulty in breathing. Their lifespan can be reduced by as much as 30 years.
Sickle cell disease generally affects people whose ancestors came from sub-Saharan Africa, India, Saudi Arabia and Mediterranean countries. Thalassemias affect mainly those of Asian, Mediterranean or Middle Eastern ancestry. As Canada's population becomes ever more diverse, the incidence of these diseases will rise. The impact is devastating, not only for the victims but also for their families through all the long years.
What should we do? According to the World Health Organization, the best approach is a strategic balance of disease management and prevention programs. Prevention would include blood tests to determine which couples are at risk of having affected children and counselling for those couples.
Thalassemias are mostly managed with regular blood transfusions and treatment of the effects of transfusions on organs. Management of sickle cell disease includes such simple items as a healthy diet, vaccinations to prevent infections, and pain management. Sadly, many physicians remain unfamiliar with sickle cell disease and in particular with the excruciating pain it can cause. Education is needed for doctors as well as the public. We need screening programs, medical guidelines, awareness of best practices and social supports.
In the other place, Dr. Kirsty Duncan has presented a private member's bill calling for a comprehensive national strategy. We all know how few private members' bills make it into law, but honourable senators, this bill offers a fine opportunity to begin making Canadians aware of this scourge that blights the lives of so many.