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