Sickle Cell Disease Association of Florida, Incorporated
What Is Sickle Cell Anemia?
Sickle cell anemia is a blood disorder that causes shortage of red
blood cells and periodic pain. In people with sickle cell anemia, not
all of their hemoglobin (a molecule in red blood cells that carries
oxygen to other parts of the body) works properly. Some of the
hemoglobin forms long, rod-like structures that cause the red blood
cells to be sickle-shaped and stiff. These cells can clog small blood
vessels, preventing some organs or tissues from receiving enough
oxygen. When this happens, it can cause episodes of severe pain
or damage organs and tissues, and may lead to other serious
What is Sickle Cell Trait?
Sickle Cell Trait is not a disease. It is a carrier state of the disease
in which a gene for abnormal hemoglobin is inherited. About 1 in 10
Black Americans are born with Sickle Cell Trait. Sickle cell trait occurs
when a person who carries one sickle hemoglobin producing gene
inherited from their parents 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. Sickle Cell trait will NOT cause sickle cell disease.
How is Sickle Cell Disease detected and prevented?
• Screening for sickle cell disease
• Newborn Screening
• Individual and Family Screenings
• Genetic Counseling
• Simple Blood Test
Sickle Cell anemia 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
shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they
clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia. There is a substance in
the red 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 cell when it gives away oxygen. These rigid rods change the red cell into a
sickle shape instead of the round shape.
How is Sickle Cell Disease Treatment?
Aggressive medical treatment is necessary. The focus of medical treatment is
on prevention of infection, the decrease of symptoms and research. The most
commonly used treatment is oxygen, fluids (oral and intravenous), antibiotics,
and pain medication. A drug called hydroxyurea, which raises fetal
hemoglobin levels, also reduces painful crises, acute chest syndrome and need
for frequent blood transfusions. However, none of these methods is a cure. In
some cases, bone marrow transplant and cord blood transplantation are
recommended. There is no cure for Sickle Cell Disease.
More about sickle cell disease
Sickle cell disease is an inherited condition. Two genes for the sickle hemoglobin must be inherited from one's parents
in order to have the disease. A person who receives a gene for sickle cell disease from one parent and a normal gene
from the other has a condition called "sickle cell trait". Sickle cell trait produces no symptoms or problems for most
people. Sickle cell disease can neither be contracted nor passed on to another person. The severity of sickle cell
disease varies tremendously. Some people with sickle cell disease lead lives that are nearly normal. Others are less
fortunate, and can suffer from a variety of complications. Sickle cell disease is extremely varied in its manifestations.
This includes both the organ systems that are affected as well as the severity of the affliction. A study of the natural
history of sickle cell disease indicated that about 5% of patients account for nearly one-third of hospital admissions
(Platt et al., 1991). A significant number of patients with the disease have few admissions and live productive and
relatively healthy lives. The average life-span of people with sickle cell disease is shorter than normal, however,
reflecting increased mortality due to the complications of the disease.
Complications from the sickle cells blocking blood flow and early breaking apart include:
yellow eyes or jaundice
kidney damage and loss of body water in urine
painful erections in men (priapism)
blood blockage in the spleen or liver (sequestration)
low red blood cell counts (anemia)
Where Did The Disease Come From?
It is believed that Sickle Cell Trait began in countries where Malaria was common because people with the trait were
protected against the severe form of malaria. People who originated in the Mediterranean area are most commonly
affected. Sickle cell is in many nationalities including African Americans, Arabs, Greeks, Italians, Latin Americans and
those from India. You can be Caucasian and have sickle cell disease or trait. All races should be screened for this
hemoglobin at birth.
What Is The Difference Between Sickle Cell Anemia and Sickle Cell Disease?
Sickle Cell Anemia is the most severe form of Sickle Cell Disease. Other Sickle Cell Diseases are: Sickle Cell
Thalassemia (Cooley's Anemia), Sickle Cell C Disease, Sickle Cell D Disease, and any other abnormal hemoglobin
combined with sickle hemoglobin.