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What is hemoglobin?

Hemoglobin (Hb) is the special protein within the red blood cells that carries oxygen from the lungs to the rest of the body. Hemoglobin is what makes your blood look red in color.

Where does your hemoglobin come from?
Your hemoglobin type is inherited through family genes. The color of your hair, the color of your eyes, and your hemoglobin type are all examples of things that are determined by genes. You receive one gene for hemoglobin type from your mother and one from your father. Hemoglobin A or normal adult hemoglobin is the most common type. There are over 500 different types or variations of hemoglobin.

What is hemoglobinopathy?
Hemoglobinopathy is a term used to describe disorders caused by the presence of abnormal hemoglobin production in the blood.

What is sickle cell trait?
Sickle cell trait occurs when a person inherits a normal hemoglobin gene (Hb A) from one parent, and a sickle hemoglobin gene (Hb S) from the other parent. This results in hemoglobin AS or sickle cell trait. A person with sickle cell trait may also be called a sickle cell carrier. Sickle cell trait is not a disease and usually does not affect one's health, but individuals with sickle cell trait can pass the sickle hemoglobin gene to their children.

How will I know if I have sickle cell trait?
A simple blood test called a hemoglobin electrophoresis will detect sickle cell trait. This test will tell if you are a carrier of the sickle cell trait or if you have the disease.

Where can I be tested for sickle cell trait?
Your physician can give you the test or the test may be obtained at some hospitals, sickle cell centers, and health department clinics.

What is sickle cell disease?
Normal and Sickled Red Blood CellsSickle cell disease is a group of inherited blood disorders caused by the presence of hemoglobin S in red blood cells. The red cells change to a sickle (banana) shape and can clog blood vessels causing damage to the body's tissues and organs.
Sickle cell anemia (Hb SS) is the most common type of sickle cell disease. There are other hemoglobin types such as hemoglobin C or hemoglobin E, that in combination with the gene for sickle hemoglobin can result in different forms of sickle cell disease (Hb SC, Hb SE, Hb S/beta thalassemia).

What are "sickle cell conditions"?
They are sickle cell trait and the various types of sickle cell disease.

How do you get sickle cell anemia or trait?
You inherit the abnormal hemoglobin from your parents. You cannot catch it from someone else or pass it to another person like a cold or other infections. You are born with sickle cell hemoglobin and it is present for life. If you inherit only one sickle gene, you have sickle cell trait and will not develop the disease. If you inherit two sickle cell genes, you have sickle cell anemia.

How common is sickle cell anemia?
Sickle cell anemia affects millions of people throughout the world. It is particularly common among people whose ancestors come from Africa; Spanish speaking regions (South America, Cuba, Central America); Saudi Arabia; India; and Mediterranean countries, such as Turkey, Greece, and Italy. In the United States, most people who have sickle cell disease are African American. In Missouri, the disease occurs in approximately 1 in every 400 African-American births, and approximately 1 in 12 African Americans carry the sickle cell trait.

How is sickle cell anemia detected?
Most states now perform a blood test for sickle cell disease on all newborn infants. Early diagnosis of sickle cell anemia is critical so that children who have the disease can receive proper treatment. Since May 1989, all babies born in Missouri are tested for sickle cell conditions through the Missouri Newborn Screening Program (MNSP). This test is performed at the same time and from the same blood samples as other routine newborn screening tests. If the test shows the presence of sickle hemoglobin, a second blood test is performed to confirm the diagnosis. These tests also tell whether the child carries the sickle cell trait.

How is the testing done?
A small blood sample is taken from the infant's heel, usually before the baby leaves the hospital. The blood sample will show whether the baby has sickle cell trait, sickle cell disease or any other abnormal hemoglobin.

What are the chances of having a baby with sickle cell trait?
One parent with sickle cell traitWhen ONE parent has sickle cell trait and the other parent has normal hemoglobin the child may inherit:
two normal hemoglobin genes, or
one normal hemoglobin gene and one sickle cell gene.

With EACH pregnancy there is a:
50% chance that the baby will have normal red blood cells (AA).
50% chance that the baby will have sickle cell trait (AS).

What are the chances of having a baby with sickle cell disease?
Both parents with sickle cell traitWhen BOTH parents have sickle cell trait (AS), the child may inherit:
two normal hemoglobin genes, or
one normal hemoglobin gene and one sickle cell gene, or
two sickle cell genes

With EACH pregnancy there is a:
25% chance that the baby's red blood cells will be normal (AA).
50% chance that the baby will have sickle cell trait (AS).
25% chance that the baby will have sickle cell disease (SS).

What medical problems are caused by sickle cell disease?
Sickle shaped red blood cells tend to get stuck in narrow blood vessels blocking the flow of blood. This can cause damage to organs including the spleen, liver and kidneys. Other complications include pain episodes, lung problems and stroke.

What should future parents know?
People who are planning to become parents should know whether they are carriers of the sickle cell gene. If they are, they may want to seek genetic counseling to find out what the chances are that their child will have sickle cell trait or sickle cell anemia.

How can I be tested?
Your doctor can do a simple blood test called a hemoglobin electrophoresis. This test will tell if you are a carrier of the sickle cell trait or if you have the disease.

Credit: http://health.mo.gov/living/families/genetics/sicklecell/faqs.php#1