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What
is Sickle Cell Disease ?
Sickle
Cell Disease is a group of inherited red blood cell disorders.
Normal red blood cells are round like doughnuts, and they move
through small blood tubes in the body to deliver oxygen. Sickle
red blood cells become hard, sticky and sickle shape. When these
hard and pointed red cells go through the small blood tube, they
clog blood flow and break apart. This can cause pain, tissue
damage and a low blood count, or anemia.
What
makes the red cell sickle?
There
is a substance in the red blood cell called hemoglobin that
carries oxygen inside the cell. One little change in this
substance causes the hemoglobin to form long rods in the red
blood cell when it gives away oxygen. These rigid rods change
the red bloods cells into a sickle shape.
How
do you get sickle cell anemia?
You
inherit the abnormal hemoglobin from parents if both are
carriers of the sickle cell trait. The abnormal hemoglobin
can also be inherited if one of your parents has sickle cell
disease. Sickle cell disease is not contagious. You are born
with the sickle cell hemoglobin and it is present for life.
How
can I be Tested?
A
simple blood test called the hemoglobin electrophoresis can be
done by your doctor or local sickle cell foundation. This test
will tell if you are a carrier of the sickle cell trait or if
you have the disease. New born babies can also be screened
for sickle cell disease.
What
is sickle cell trait?
A
person with sickle cell trait carries one sickle hemoglobin
producing gene and one
normal hemoglobin gene. Normal hemoglobin
is called type A. Sickle hemoglobin called S. Sickle cell
trait is the presence of hemoglobin AS on the hemoglobin
electrophoresis. This will NOT cause sickle cell disease. Other
hemoglobin traits are AC and AE traits.
What
are the other forms of sickle cell disease?
There
are three common types of sickle cell disease.
- Hemoglobin
SS or or sickle cell anemia
- Hemoglobin
SC disease
- Hemoglobin
sickle beta-thalassemia
Each
of these can cause sickle pain episodes and complications, but
some are more common than others. An increase in fetal
hemoglobin can protect the red blood cells from sickling in all
of these forms of sickle cell disease. The medication
hydroxyurea increases fetal hemoglobin.
Frequently
Asked Questions About Sickle Cell
Sickle
Cell and Nutrition
Managing
Sickle Cell Pain
Prevalence
in Canada
Sickle
Cell Summary for Patients
What
Teachers and Employers should Know
Online
Books and Handouts for Patients and Families
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