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

Transcript of Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP,...

Page 1: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

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

Page 2: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

About Your Blood

Hemoglobin binds with Oxygen and carries it around the body

Page 3: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

The Cells Really Are Sickle Shaped

Page 4: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,
Page 5: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

• 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

Page 6: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

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

Page 7: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

It’s in the Family

C b

3 main forms of SCD

HbSC HbS/b Thalassemia

Page 8: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

=Newborn or prenatal screening!

Page 9: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

Getting Tested – Patient or Donor

=CBC, Hb electrophoresis

Page 10: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

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!

Page 11: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

Sickle Cell is not Simply Pain

Page 12: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

Sickle Cell is for Life

?

Page 13: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

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

Page 14: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

We Can Beat It (Together)

Page 15: Sickle Cell Disease An Overvie · Sickle Cell Disease – An Overview Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology,

www.sicklecelldisease.ca www.canhaem.org