Sickle Cell disease can be a very scary issue to deal with however, with proper monitoring and care, many people are able to live their life to its full capacity with minor complications.
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Definition
According to the National Heart, Lung and Blood Institute, “Sickle cell disease is a group of inherited red blood cell disorders that affect hemoglobin, the protein that carries oxygen through the body. Normally, red blood cells are disc-shaped and flexible enough to move easily through the blood vessels. In sickle cell disease, red blood cells become crescent- or “sickle”-shaped due to a genetic mutation. These sickled red blood cells do not bend or move easily and can block blood flow to the rest of the body.
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The blocked blood flow through the body can lead to serious problems, including stroke, eye problems, infections, and episodes of pain called pain crises.”
Also, sickle cell is a lifelong illness and affects approximately 100,000 people in the United States and 20 million people worldwide.
In the United States, most people who have sickle cell disease are of African ancestry or identify themselves as Black:
- About 1 in 13 Black or African American babies are born with sickle cell trait.
- About 1 in every 365 Black or African American babies are born with sickle cell disease.
Symptoms can range from severe anemia to heart and lung problems.
Q&A with doctor
Back in 2019, KPRC 2 spoke with Medical Director, Dr. Idowu, at UT Physicians Comprehensive Sickle Cell Center and asked the following questions.
Q: What is sickle cell disease? How do you get sickle cell disease?
A: Sickle cell disease is an inherited group of blood disorders that causes red blood cells to become misshapen or sickle-shaped. A red blood cell’s normal shape makes it flexible and allows it to easily move through blood vessels to deliver oxygen. When cells are sickle-shaped, they stick to vessel walls preventing blood and oxygen flow, which ultimately causes severe pain. These episodes of pain, known as “pain crises” can last a week or longer in some patients.
Q: How do you know if you have sickle cell disease?
A: A blood test can check for hemoglobin S — the defective form of hemoglobin that underlies sickle cell anemia. In the United States, this blood test is part of routine newborn screening done at the hospital. But older children and adults can be tested, too.In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where it’s screened for hemoglobin S.If the screening test is negative, there is no sickle cell gene present. If the screening test is positive, further tests will be done to determine whether one or two sickle cell genes are present.
Q: What are some of the signs and symptoms of sickle cell disease?
A: Acute pain crisis, severe anemia, stroke, bone infarct, splenic infarct, risks for infections, retinal damage, priapism, leg ulcers, heart problems, lung problems (acute chest syndrome), kidney disease, liver complication and multiple organ dysfunction or even multiple organ failure.
Q: How is sickle cell disease treated?
A: Bone marrow transplant, also known as stem cell transplant, offers the only potential cure for sickle cell anemia. It’s typically reserved for people younger than age 16 because the risks increase for people older than 16. Finding a donor is difficult, and the procedure has serious risks associated with it, including death. As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. Babies and children age 2 and younger with sickle cell anemia should make frequent visits to a doctor. Children older than 2 and adults with sickle cell anemia should see a doctor at least once a year, according to the Centers for Disease Control and Prevention. However, since patients actually have progressive organ damage as they grow older, adult SCD specialists recommend that patients should continue to see a doctor at least every 3 to 6 months. Treatments might include medications to reduce pain and prevent complications, and blood transfusions, as well as a bone marrow transplant.