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,...
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
About Your Blood
Hemoglobin binds with Oxygen and carries it around the body
The Cells Really Are Sickle Shaped
• 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
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
It’s in the Family
C b
3 main forms of SCD
HbSC HbS/b Thalassemia
=Newborn or prenatal screening!
Getting Tested – Patient or Donor
=CBC, Hb electrophoresis
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!
Sickle Cell is not Simply Pain
Sickle Cell is for Life
?
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
We Can Beat It (Together)
www.sicklecelldisease.ca www.canhaem.org