What is sickle cell disease (SCD)?
Sickle cell disease is group of inherited or passed down diseases that affect the shape of hemoglobin in the blood. The presence of abnormal, “sickle” hemoglobin can produce complications. There are many different types of sickle cell. The most common type of sickle cell is HbSS or “sickle cell anemia.” Other types include HbSC, Hb beta + thalassemia, and Hb beta 0 thalassemia.
How do you get sickle cell?
- Sickle cell disease happens when both parents have sickle cell trait, one parent has sickle cell and the other has trait, or when both parents have sickle cell.
- We all have two sets of hemoglobin traits in our DNA – normal hemoglobin is referred to as hemoglobin A. Someone without sickle cell, sickle cell trait, or another abnormal hemoglobin has AA hemoglobin. Someone with sickle cell trait has AS hemoglobin.
- Sickle cell occurs when the S hemoglobin is passed down from both parents. Different types of sickle cell, such as SC and Beta Thalassemia can occur when one parent has sickle cell trait and the other carries a different abnormal hemoglobin. Sickle cell disease is not contagious; You are either born with the disease or you will never have it.
Who is affected by sickle cell disease?
- Sickle cell disease is found throughout the world. In addition to the United States, it is often found in parts of Africa, the Middle East, India and the Mediterranean region.
- In the United States, sickle cell disease mostly affects people of African descent and a few of Latino descent.
- About 1 out of every 400 African Americans has sickle cell disease.
- About 1 out of every 13 African Americans carries sickle cell trait.
- Roughly 100,000 people with sickle cell disease live in the United States. About 10,000 people with sickle cell disease live in North Carolina.
- Each year in North Carolina, over 100 infants are born with sickle cell disease.
What are the symptoms of sickle cell disease?
- The most common symptoms of sickle cell disease are fatigue and painful episodes called vaso-occlusive crisis.
- Sickle cell disease can affect all organs in the body.
- People with sickle cell disease can lead full, happy, and productive lives with treatment.
How is sickle cell disease treated?
- Routine visits with doctors specializing in sickle cell called hematologists. Your sickle cell doctor will determine which treatments are right for you.
- Hydroxyurea is a daily medication used in patients with sickle cell.
- Penicillin prophylaxis is used in children to lessen the risk of infection.
- Many patients receive blood transfusions regularly or as needed.
- There are many other medications used to treat the various symptoms of sickle cell such as: L-glutamine, voxelotor, and crizanlizumab.
How can I help someone with sickle cell disease?
- Donate blood at your local Red Cross.
- Join the bone marrow donor registry at bethematch.org
- It is especially important for African Americans without sickle cell to donate blood and join the bone marrow registry as they are the most likely match for sickle cell patients in need of blood or bone marrow transplant.
Where can I learn more about sickle cell disease?
- We encourage patients to learn about their disease and stay up to date on treatments and research studies. Your sickle cell doctor is always willing to answer questions or help you understand more clearly.
- Here are some websites we trust to provide good information:
What is thalassemia?
Thalassemia is an inherited or passed down blood disorder where the body is unable to make enough healthy hemoglobin. A person with thalassemia may have “beta” or “alpha” thalassemia depending on the part of the hemoglobin that is not being properly made in the red blood cells. Thalassemia may also be categorized severity by specifying “trait”, “intermedia”, or “severe”.
Who is affected by thalassemia?
Thalassemia commonly affects people or families who come from Mediterranean countries such as Greece and Turkey and those from Africa, Asia and the Middle East.
What are the symptoms of thalassemia?
Anemia, fatigue, enlarged spleen, and the need for blood transfusions.