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    What do you know about Sickle Cell Disease?

    by Ngozi Osuagwu, MD | June 16th, 2024

    What do you know about Sickle Cell Disease?

    I remember one of my friends telling me about one of her pregnant patients whose blood test showed she had hemoglobin AS. The patient knew it was a sickle cell trait and was healthy without problems. She thought only black people had sickle cell trait.

    The lab test was a hemoglobin electrophoresis, which measures the different types of hemoglobin in the blood. When a patient has hemoglobin AS, one of their hemoglobin is normal and the other is sickle. This indicates the patient has sickle cell trait, a condition where they have one copy of the altered hemoglobin gene. It is important to note that people with sickle cell disease have two copies of the altered genes, which can manifest as hemoglobin SS, Hemoglobin SC, or hemoglobin Sbeta-thalassemia. While most people with sickle cell trait will never experience any medical complications, in rare instances, some may experience complications during intense physical exercise.

    June 19 is World Sickle Cell Day, and the purpose of this day is to increase the knowledge about sickle cell disease. Sickle cell disease (SCD) is a group of inherited red blood cell disorders. SCD affects 90,000 to 100,000 people in the United States, mainly Blacks or African Americans. The disease occurs among about 1 of every 500 black or African-American births and among 1 out of every 36,000 Hispanic-American births. SCD affects millions of people worldwide and is particularly common among those whose ancestors come from sub-Saharan Africa; regions in the Western Hemisphere (South American, the Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy.

    Healthy red blood cells are round and move through small blood vessels carrying oxygen to all body parts. In SCD, the red blood cells become rigid and sticky and look like a C-shaped farm tool called a “sickle.”  Compared to healthy blood cells, sickle cells can get stuck in small blood vessels and block blood and oxygen flow to organs in the body. These blockages cause repeated episodes of severe pain, organ damage, serious infections, or even strokes.

    The CDC wants you to know five facts about sickle cell disease:

    1. A child gets sickle cell disease when they receive two sickle cell genes – one from each parent.
    1. Sickle cell disease has many faces. The disease affects millions of people worldwide.
    1. SCD can be cured for specific patients – We have bone marrow transplants and gene therapies.
    1. Anemia is a common effect of SCD, but it can be treated.
    1. A person with SCD can live a long and high-quality life by doing the following:
    2. Getting regular checkups with their doctor
    3. Following treatments prescribed by their doctor, such as taking a medication called hydroxyurea
    4. Preventing infections by taking simple steps, including washing your hands
    5. Practicing healthy habits like drinking  8 – 10 glasses of water daily and eating healthy food.

    Please click here to learn more about how one inherits sickle cell disease from parents.

    One Response to “What do you know about Sickle Cell Disease?”

    1. A friend’s friend only just discovered in her 60s that she has SCD and is only now experiencing symptoms. I did not know that was possible. Is this unusual?

    Leave a Reply

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