Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP,...
Transcript of Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP,...
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Sickle Cell Disease – An Overview
Richard Ward, MSc, MRCP, FRCPath
Director, Red Blood Cell Disorders Program, UHN
Assistant Professor, Hematology, University of Toronto
Chair, Canadian Haemoglobinopathy Association
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About Your Blood
Hemoglobin binds with Oxygen and carries it around the body
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The Cells Really Are Sickle Shaped
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• RBCs break apart (“hemolyse”) – Anemia (low Hb level) – Jaundice (yellow eyes from bilirubin pigment)
• Block and damage blood vessels (vaso-occlusion)
– Oxygen starvation: pain, organ failure – Formation of new small, fragile blood vessels to try to
increase oxygen supply (neovascularisation)
Why Sickling Makes you Sick
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Why Halifax, NS?
~80-90 SCD births /yr in Canada ~500,000 Blacks in Ontario >1 in 10 carrier rate in Toronto, as both Caribbean and West African popn in GTA
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It’s in the Family
C b
3 main forms of SCD
HbSC HbS/b Thalassemia
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=Newborn or prenatal screening!
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Getting Tested – Patient or Donor
=CBC, Hb electrophoresis
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Why Worry About SCD?
Quinn, et al., Blood 2010
High rate of childhood death
from infections
>10% children suffer a stroke,
25% of adults
=NBS!
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Sickle Cell is not Simply Pain
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Sickle Cell is for Life
?
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Don’t Let SCD Define You Patient self-management
Healthy living
Employment, relationship, housing issues
Monitoring and treatment of complications
Less damage to the body
Reduced hospital dependence
Normal life expectancy
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We Can Beat It (Together)
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www.sicklecelldisease.ca www.canhaem.org