Sickle cell disease is a hereditary disorder caused by a defect in the hemoglobin gene of the red blood cells.
Fact: We need blood to live. The main purpose of red blood cells is to deliver oxygen throughout the body.
Blood circulating through the body delivers oxygen and nutrients to the cells and tissues of the body.
The blood travels throughout the body via a vast network of tube-like blood vessels, which become
smaller and smaller as they reach a hungry cell.




Normal red blood cells are disc or doughnut-shaped, flexible,




and easily pass through small vessels. There is a protein in our
red blood cells called hemoglobin; it makes our blood red. This protein is responsible for carrying
oxygen you breathe in your lungs, to your heart, and out to the rest of your body - eventually realeasing it to your hungry cells.
One little change in your hemoglobin can change the shape of your blood cells; when you have sickle hemoglobin, it forms long rods
when it gives up oxygen. The genes you inherit from your mother and father determine what type of hemoglobin you make.



Sickle red blood cells start out disc-shaped, but become sickle or crescent-shaped



after releasing oxygen. Sickle blood cells are hard, sticky, and pointed; they look like bananas, cresent moon,
or the sickles that farmers use to cut tall grass (hence the name).



Sickled cells are rigid and have difficulty passing through small vessels. When these hard and pointed
red blood cells go through the small artreries, they block flow, then break apart too quickly.



Sickled cells block small blood vessels and delay the delivery of oxygen to tissues.


This blockage causes severe pain, organ damage, and a low blood count, or anemia.
Anemia can result from a variety of reasons, but in a person with sickle cell disease, anemia usually occurs because the red blood cells can not be replaced as fast as they are destroyed.
Anemia can cause fatigue (tiredness) and may result in an inability to participate in strenuous activities for a long period of time.


People with sickle cell must get enough sleep to help aviod a pain crisis.
Normal red blood cells last 90-120 days. (3-4 months).
Sickle red blood cells last 10-20 days.
Yes, there are different types of sickle cell disease, all of them named for the "shape changeing" hemoglobin (Hb).
Hb SS, (called sickle cell anemia)
Hb SC,
Sickle Beta Thalassemia
Hb SD-Punjab
Hb SO-Arab
Hb SE
Hb S-HPFH (hereditary persistence of fetal hemoglobin)
All of these combinations have sickle complications, but some are more severe than others.
There are many genetic differences within these hemoglobin combinations that are not yet fully
understood that make some have a milder course and some have a more severe course and a shorter life span.
Sickle Beta Thalassemia
Is an inherited disorder of beta globin production, a part of the hemoglobin molecule.
Decreased hemoglobin production causes smaller than normal red blood cells, which
look like bull's eye targets under a microscope.
Beta thalassemia can combine with the sickle hemoglobin to cause sickle beta thalassemia.
Most people with beta thalassemia have fewer problems with infections, fewer pain episodes,
and less damage to the organs early in life. They often do vey well when they get older.
No, Sickle Cell is found in many other nationalities and races, including Arabs, Greeks, Italians, Latin Americans, and Indians.
But Sickle cell is most prevalent in people of African decent and Latinos/Hispanics.
Males and females have different life spans and this is true for people with sickle cell disease as well.
Some people with sickle cell disease live longer than the average life span and some people live less than the average life span.
People with different types of sickle cell disease have different average life spans and different complications from the disease.
> A female with Hemoglobin SS disease will live an average of mid 30's to mid 40's.
> A male with Hemoglobin SS disease will live an average of mid 30's to 42 years.
> A female with Hemoglobin SC disease will live an average of 68 years.
> A male with Hemoglobin SC disease will live an average of 60 years.
Related Facts:
People with Sickle Beta Zero Thalassemia have similar complications as those with homozygous sickle cell disease (SS),
so their average life spans are the same.
You are probably already aware of the dangers of alcohol, smoking and using "street" drugs.
These are harmful to everyone. But for people with sickle cell disease,
even occasional drinking, smoking (even second hand smoke) or drug use can lead to severe sickle cell complications.
Alcohol + fluid loss + dehydration = PAIN!
Smoking = less oxygen to lungs = lung infection and permanent lung damage!
"Street" drugs = serious stress to body = damage to major body organs – heart, lungs and brain!
You are in control over what you put in your body. Please choose wisely.
Yes, A Bone Marrow Transplant is the only cure for
Sickle Cell Anemia. However this cure is not available or practical for
most sicklers due to the risk of rejection.
A sibling who closely matches the patient or a stem cell transplant provides
the best chance for a successful transplant, because the posibility of rejection
by the patient's body will be greatly reduced.
Umbilical cord blood
stem cell transplants
are less prone to
rejection.
(A bone marrow transplant using stem cells taken from cord blood.)