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Sickle Cell Disease - Test Your Knowledge

Sickle Cell Health

Sickle cell disease, or sickle cell anemia is a serious lifelong, genetic (inherited) disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.

When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. Blocked blood vessels can cause severe pain (crises), serious infections, organ damage and stroke. Bone marrow/stem cell transplant can cure sickle cell disease, however, there is currently no universal cure for this condition. Forty years ago, the life expectancy of people with sickle cell disease was 14 years, however, due to improved treatment and care, people who have sickle cell anemia are now living into their fifties, or longer.

Sickle cell trait (AS) is an inherited condition in which both hemoglobin A and S are produced in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy. However, It is important for people to get tested because if they have the trait it is possible that they experience complications at high altitudes (flying, visiting high altitude cities), and a person with sickle cell trait can pass the disease on the their children. People with sickle cell disease can mitigate sickling of red blood cells by staying hydrated, getting enough oxygen, preventing or quickly treating infections.

Sickle cell disease is inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets, one from each parent.

If one parent has sickle cell anemia and the other is normal, all of the children will have sickle cell trait.
If one parent has sickle cell anemia and the other has sickle cell trait, there is a 50% chance (or 1 out of 2) of having a baby with either sickle cell disease or sickle cell trait with each pregnancy.
When both parents have sickle cell trait, they have a 25% chance (1 of 4) of having a baby with sickle cell disease with each pregnancy.

A simple blood test followed by a laboratory technique called hemoglobin electrophoresis will determine the type of hemoglobin you have. This technique differentiates between normal hemoglobin (A), sickle hemoglobin (S), and other different kinds of hemoglobin.

Sickle cell disease is more common in people of African and Mediterranean descent. It is also occurs in people from South and Central America, the Caribbean, and the Middle East. The prevalence of the disease in the United States is approximately 1 in 5,000, however, about 1 in 375 African American newborns have sickle-cell disease, and 1 in 12 Black Americans have sickle cell trait.

Sickle Cell Q&A


		Which of the following statements about sickle cell disease is not true?
		a) Sickle cell disease, or sickle cell anemia is a serious genetic blood disorder that affects red blood cells.
		b) Sickle-shaped (crescent shaped) red blood cells have difficulty passing through small blood vessels.
		c) Blood vessels that are blocked by sickled cells can cause severe pain, serious infections, and organ damage.
		d) Sickle cell disease can easily be cured with proper treatment.


		People with sickle cell trait usually do not have any of the symptoms of the disease.
		c) True
		d) False


		If both parents have sickle cell trait, what is the chance of having a baby with sickle cell disease with each pregnancy?
		a) All of the children will have sickle cell trait.
		b) 75%
		c) 50%
		d) 25%


		How can people with sickle cell disease mitigate sickling of their red blood cells?
		a) By staying hydrated.
		b) Getting enough oxygen.
		c) Preventing/quickly treating infections.
		d) All of the above


		Why is it important for Black people to get test for sickle cell disease?
		a) The disease is more common in people of African and Mediterranean descent.
		b) About 1 in 500 Black newborns in the U.S. have sickle-cell anemia.
		c) Approximately 1 in 12 African Americans have sickle cell trait.
		d) All of the above

Correct answers will be marked with a red asteric '*'