About Sickle Cell Disease

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About Sickle Cell Disease

Sickle cell disease (SCD) is an inherited blood disorder in which red blood cells may become sickle-shaped and harden, causing serious and potentially fatal complications. People with sickle cell disease are born with this condition. It can cause chronic, severe and unpredictable pain. It is not contagious, and there is no universal cure.


About 1 in 13 African Americans carry the sickle cell trait, and many do not know they have it.


An estimated 100,000 people in the U.S. have SCD.


Approximately 2,000 babies are born with SCD annually in the U.S.


On average, diagnosis is made at birth.


People of many ethnic backgrounds can have SCD.


Latinos have the second most common incidence in the U.S.

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Understanding Sickle Cell Disease

People with sickle cell disease have genes that tell their body to produce abnormal hemoglobin. Hemoglobin is the main substance of red blood cells, and it helps those cells carry oxygen from the air in our lungs to all parts of the body. Normal blood cells contain hemoglobin A, which allows the cells to be soft and malleable. Sickled red blood cells contain hemoglobin S, which causes the cells to become stiff and distorted. When sickle-shaped cells block small blood vessels, less blood is circulated throughout the body. This process causes most sickle cell complications.

Normal Red Blood Cells

  • Contain hemoglobin A
  • Are soft and round
  • Can easily squeeze through blood vessels
  • Live for about 120 days

Sickled Red Blood Cells

  • Contain hemoglobin S
  • Become stiff and distorted in shape
  • Have difficulty passing through blood vessels
  • Live for about 16 days

Understanding the Role of Genetics

Just like your hair color or your eye color, your sickle cell status is determined by your parents’ genes. Like most genes, hemoglobin genes are inherited in two sets, one from each parent. Let’s look at some potential family trees to better understand this concept.

If one parent has sickle cell disease and the other is normal, all of their children will have sickle cell trait.

If one parent has sickle cell anemia and the other has sickle cell trait, with each pregnancy there is a 50 percent chance their child will have sickle cell disease or sickle cell trait.

There are several types of sickle cell disease. The most common are:

  • Hemoglobin SS Disease
  • Hemoglobin SC Disease
  • Sickle Beta-Plus Thalassemia
  • Sickle Beta-Zero Thalassemia

Most individuals with sickle cell disease have hemoglobin S, but some have a different type of abnormal hemoglobin like hemoglobin C, hemoglobin D or hemoglobin E. Also, some individuals can have sickle cell disease because of co-inheritance of beta thalassemia.

  • Chronic, severe and unpredictable pain
  • Anemia
  • Frequent infections
  • Swelling in extremities
  • Fatigue
  • Delayed growth
  • Vision problems/blindness
  • Lung tissue damage
  • Kidney disease
  • Stroke
  • Shortened life expectancy
  • Damage to hip joint
  • Gallstones
  • Jaundice
  • Priapism
  • Antibiotics
  • Pain management
  • Intravenous fluids
  • Blood Transfusion
  • Surgery
  • Medications like hydroxyurea
  • Folic acid
  • Gene therapy