Make an impact this September (Sickle Cell Awareness

Month) as we call for a National Framework for Sickle Cell

Disease

Long considered a rare disease within Canada, sickle cell disease is in fact the most
common genetic disease in the world, with an estimated 100 million affected individuals
world-wide and another 275,000 babies with SCD born annually1. The number of
affected Canadians numbers in the thousands and additional 100 or more children with
the condition are born in this country each year.

What is SCD?
Sickle cell disease is an inherited condition that results in an abnormality of haemoglobin, the
substance that carries oxygen within red blood cells. This abnormal haemoglobin can lead to
red blood cell deformation and break down, increased blood viscosity or “sludginess”, and both
acute and chronic organ dysfunction. Typical manifestations in patients with sickle cell disease
include:
 Severe pain episodes due to bone marrow necrosis
 Susceptibility to infections
 Increased risk of stroke and vision loss
 Need for frequent blood transfusions
 Reduced life expectancy of approximately 30 years compared to normal
population.2

Because sickle cell disease is considered the quintessential “multi-system disease”,

patients often require comprehensive care to prevent and manage complications.3

SCD in Canada
Because sickle cell gene mutation emerged in response to malaria, individuals with
ethnic backgrounds associated with equatorial regions have a greater likelihood of
carrying at lease one copy of the genetic mutation (a condition known as being sickle
cell trait or being a sickle cell carrier), if not the disease itself. These individuals
therefore tend to be of African, Middle Eastern origin or from the Indian subcontinent
(India is second only to Nigeria), but due to centuries of inter-marriage and emigration,
individuals of virtually any ethnic background may be affected. Universal newborn
screening for children with sickle cell disease will soon be available in every province,
but Canada continues to lag behind the United States in its lack of a national strategy
for managing this condition.
According to Dr. Jacob Pendergrast, chair of the Sickle Cell Disease Association of
Canada’s Medical Advisory Board, “A National Framework would help ensure that all
patients in Canada with sickle cell disease receive evidence-based, comprehensive
care. This would not only ensure that patients born with this condition live longer and
healthier lives, but would also save the health care system substantial costs by shifting
the medical focus from inpatient treatment of emergencies to outpatient preventative
care.”

Furthermore, the president of the Association, Ms. Lanre Tunji-Ajayi notes that many
patients have reported an apparent lack of familiarity with sickle cell disease amongst
the physicians who treat them. In many instances this results in delayed and
incomplete care, but occasionally patients experience life-threatening complications and
even death due to failure to provide appropriate management. “In a country as well-
resourced and diverse as Canada, this is simply unacceptable. Young adults with this
disease are dying at a time when other countries with comparable populations are
having fewer or no deaths due to the provision of comprehensive care programs. Over
the last 10 years, repeated pleas for improved quality of care for patients with sickle cell
disease have been sent to hospitals, regional health authorities and Ministries of Health
by various sickle cell organizations across the country. While these pleas have been
met with sympathetic responses, there has been little actual mobilization of resources.”
Continued Ms. Tunji-Ajayi, ”It would be extremely beneficial to the patient community
and save the health care system considerable expense long term if more
comprehensive clinics for Sickle Cell Disease could be established in strategic locations
across the country. This would allow more patients to attend regular clinics and stay
healthier longer.”

Cost of Sickle Cell Disease in Canada

The lifetime costs for a patient with sickle cell disease have been estimated at $9
million.4 As there are approximately 5 000 patients with sickle cell disease in Canada,
the total costs to their health care system may be as high as $4.5 billion. Much of this
cost reflects the frequent hospitalizations required by patients with sickle cell disease
when they are denied comprehensive, preventative care: a one-week non-ICU
hospitalization, for example, costs $20 000 on average, and patients with sickle cell
disease may require hospitalizations several times per year if they are not treated with
inexpensive medical interventions such as vaccinations and the medication
hydroxyurea. The human cost of sickle cell disease, on the other hand, both to the
patient and their family, is unquantifiable, and includes missed school days and
employment opportunities, social isolation and stigmatization, and psychological strain.
About Sickle Cell Disease Association of Canada/Association d’Anémie
Falciforme du Canada (SCDAC/AAFC): Established in 2012; it is its mandate to
ensure among other purposes that every Canadian living with Sickle Cell Disease has
access to comprehensive care and treatment. To this end, SCDAC/AAFC is actively
engaging different ministries of health across the country.

For media interviews; please contact us at: communication@sicklecelldisease.ca

References:

1. Modell, B. and M. Darlison, Global epidemiology of haemoglobin disorders and derived

service indicators. Bulletin of The World Health Organization, 2008. 86:480-487

2. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: life expectancy
and risk fac- tors for early death. N Engl J Med. 1994;330: 1639-1644

3. Chakravorty S, Williams TN. Sickle cell disease: a neglected chronic disease of

increasing global health importance. Arch Dis Child 2015;100:48–53

4. Ballas SK. The cost of health care for patients with sickle cell disease. Am J Hematol
2009; 84:320–322.