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What is "Sickle Cell"?

Sickle Cell is a genetic blood disorder that is passed from the parent to the child. Genes are what determine a child’s physical traits such as skin, eye and hair color. Genes also affect hemoglobin. Hemoglobin is a protein in the red blood cells that caries oxygen from the lungs to other parts of the body. People with normal hemoglobin received two normal genes denoted as AA.

Abnormal genes are common and can also be transferred to children. Both parents contribute one gene for each trait. When a person has Sickle Cell Trait (SCT) the gene type is AS, which is one normal gene and one abnormal gene. A person who has Sickle Cell Disease (SCD) has a gene type of SS.

If one parent has SCT (AS) and the other parent has normal hemoglobin (AA) there is a 50% or 1 in 2 chance that each of their children will have the Sickle Cell gene, creating SCT (AS). If both parents have AS, there is a 25% or 1 in 4 chance that each of their children will have two Sickle Cell genes, creating SCD (SS). For a visual understanding of the family risk associated with Sickle Cell Disease and Trait, please CLICK HERE.

The abnormal hemoglobin causes the red blood cells to sickle and lose their oxygen. When red blood cells sickle they become hard, sticky, and crescent shaped. These cells create blockages in blood vessels. These blockages make it difficult for normal red blood cells to pass through the blood vessels with vital oxygen and nutrients the body needs.

If a person has SCD, they are prone to more serious health conditions. Examples of health complications include the following:

• Pain Episodes
• Organ Damage
• Stroke
• Anemia
• Leg Ulcers
• Acute Chest Syndrome
• Jaundice
• Pneumonia
• Slowed Growth and Delayed Puberty in Children
• Heart Problems
• Joint Damage
• Increased Risk of Infections

While the disease is often debilitating and even deadly, successful treatments are increasing life expectancy with the advancement of medical science.

The following are just a few available medical treatments:

Hydroxyurea - is an oral medication that is used to increase fetal hemoglobin (HbF) in the body. When Hbf is increased, it also raises the total hemoglobin in the body. Fetal hemoglobin is used to promote healthy blood cells in the body and reduce the risk of cells sickling.

Blood Transfusions - A blood transfusion provides new blood with red blood cells and oxygen to the body. When this happens, it can reduce the amount of blockage that can occur with sickled cells, allowing oxygen rich blood to pass through unrestricted.

Bone Marrow Transplant - This therapy replaces a person’s bone marrow. The bone marrow is where the production of red blood cells occurs.


FAQs

Q: What is Sickle Cell Disease?
A: Sickle Cell Disease is a group of inherited blood disorders that cause red blood cells to change shape. The three most common forms are SS (Sickle Cell Anemia), SC (Sickle C Disease) and Sickle B-Thal (Sickle Beta Thalassemia).

Q: Who can have Sickle Cell?
A: Historically, Sickle Cell has erroneously been categorized as a “Black” disease but it is now known to affect persons from virtually every ethnicity.

• About one of every ten African Americans has Sickle Cell Trait.
• Approximately 1 in 375 persons of African descent are affected by the disease.
• Hispanic and other ethnic groups are documented carriers of SCD and the SCD Trait. In fact, 1 in 1,400 Hispanics have SCD.
• As many as 1 in 725 East Indians and 3.6 in 10,000 Native Americans have SCD.

Q: Is Sickle Cell Contagious?
A: No. Sickle Cell is hereditary (passed from parent to child). Everyone inherits two copies of the hemoglobin gene (one from mom and one from dad). When the hemoglobin gene is changed, the gene cannot work properly.

Q: I am not African American, should I still get tested?
A: Yes! Sickle Cell is not a minority disease. In fact, people in many ethnic groups can have Sickle Cell Trait or Disease. It’s most common among African Americans, but people of Central and South America, Southern Europe, Central and Southern India and African ancestry may have it too. Remember, Sickle Cell is a genetic blood disorder that can affect anyone.

Q: Is there a cure for Sickle Cell Disease?
A: Medical research has indicated that Bone Marrow Transplants may lead to a cure for up to 85% of young patients who are fortunate enough to receive them. Bone Marrow Transplants are very expensive and require Bone Marrow Donors. Consequently, this potential cure is not readily available for the majority of Sickle Cell patients at this time.

Therefore, it is important to become a registered Bone Marrow Donor and to donate money to make it possible for more Sickle Cell patients to receive Bone Marrow Transplant. For more information on how you can register to be a donor, please CLICK HERE

For more information on Sickle Cell join us at one of our educational presentations listed on the calendar. To setup a presentation for your group, school, church, or company please contact Sherry Coleman at scoleman@themartincenter.org or 317-927-5158 x103

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