What is sickle cell disease?
A plain-language guide to the most common inherited blood disorder in the world, written with warriors and clinicians.
Four things to know
What it is
Sickle cell disease (SCD) is a group of inherited red blood cell disorders. Healthy red blood cells are round and flexible. In SCD they become rigid, sticky and crescent shaped, so they get stuck in small blood vessels and slow or block the flow of blood and oxygen.
Who it affects
SCD is most common in people whose families come from sub-Saharan Africa, the Caribbean, the Middle East, South Asia and the Mediterranean. In parts of sub-Saharan Africa, up to 40 percent of people carry the sickle cell trait.
How it feels
Symptoms vary from person to person and over time: anemia and fatigue, sudden pain crises when blood flow is blocked, a higher risk of infection, and over time, damage to organs like the lungs, kidneys, eyes and joints.
How it’s treated
Care includes vaccines and antibiotics to prevent infection, pain management, hydroxyurea and blood transfusions. A stem cell transplant can cure some people, and gene therapy is now authorized in Canada for some patients.
Round cells move. Sickled cells get stuck.
A healthy red blood cell
Round and flexible, it slips through the smallest blood vessels to carry oxygen around the body.
A sickled red blood cell
Rigid, sticky and crescent shaped, it can block blood flow, causing pain and, over time, damage to organs.
The most common types
Your type is found with a blood test. It helps your care team plan, but every person’s experience is different.
HbSS
Sickle cell anemia
The most common and usually the most severe form.
HbSC
Sickle hemoglobin C disease
Often milder, but serious complications can still happen.
HbSβ
Sickle beta thalassemia
Severity depends on the type of beta thalassemia gene inherited.
It takes two copies of the gene
Sickle cell disease is inherited. A person with one sickle gene has the trait and usually no symptoms. When two people with the trait have a child, each pregnancy has the same odds.
In each pregnancy: a 1 in 4 chance of sickle cell disease, and a 1 in 2 chance of the trait.
Treatments, explained
TreatmentAug 20, 20264 min read
Hydroxyurea for sickle cell: how it works and what to expect
The daily medicine has been studied in sickle cell for decades. What it can reduce, who is offered it, why it needs regular blood tests and why it takes months to work.
TreatmentSep 8, 20264 min read
Stem cell transplant for sickle cell: how the cure works and who it is for
A transplant replaces the bone marrow that makes sickled cells. It can cure sickle cell, carries serious risks and depends on finding a match, which is where donors come in.
TreatmentSep 24, 20264 min read
Gene therapy for sickle cell in Canada: what Casgevy is and where access stands
In its main trial, Casgevy kept nearly everyone free of severe crises for at least a year. How it works, what treatment involves and why access in Canada is still limited.
