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Sickle Cell Anemia

What is Sickle Cell Anemia?

Sickle Cell is an inherited, chronic blood disease where the red blood cells become crescent shaped and function abnormally. When the blood cells become cresent/sickle shaped, they are unable to deliver adequate amounts of oxygen to other cells. The Sickle Cell Disease results in anemia (low blood counts), episodes of pain and increased susceptibility to infections.

Sickle Cell produces a chronic anemia which may become life-threatening when hemolytic crises (the breakdown of red blood cells) or aplastic crises (bone marrow fails to produce blood cells) occur. If repeated crises occurs, damage of the kidneys, lungs, bone, liver, and central nervous system may result. Occasionally, acute painful episodes may occur. These acute episodes may last hours to days affecting the bones of the back, the long bones, and the chest.

What makes the red blood cell become a sickle shape?

"Bad" Hemoglobin causes the red blood cell to develop a sickle shape. "Bad" hemoglobin is hemoglobin that contains a rod shaped substance. The red blood cell develops the sickle shape when it attaches itself to the "bad" hemoglobin. The red blood cells have a normal shape until they attach themselves to a "bad" hemoglobin.

Sickling of the red blood cells in persons with sickle cell results in cells of abnormal shape and flexibility. The sickling is promoted by conditions which are associated with low oxygen levels, increased acidity, or low volume (dehydration) of the blood. These conditions can occur as a result of injury to the body’s tissues, dehydrating states, or anesthesia.

How do you get Sickle Cell?

Sickle cell is inherited from your parents. It occurs in people who have inherited the gene hemoglobin S from both parents. In order to inherit the disease from your parents, each parent must be a carrier for the sickle cell trait. You can also inherit sickle cell anemia if one of your parents is a carrier for the trait and the other parent has sickle cell.

Sickle Cell cannot be "caught" by touching someone who has it. It can only be inherited from your parents. If you have Sickle Cell Anemia, you are born with it. It will not go away.

Symptoms of Sickle Cell

There are many symptoms for sickle cell. Individuals with sickle cell may not experience all of these symptoms. The symptoms include: family history of sickle cell anemia, fatigue, breathlessness, rapid heart rate, delayed growth and puberty, susceptibility to infections, ulcers on the lower legs (in adolescents and adults) jaundice, attacks of abdominal pain, weakness, joint pain, fever, vomiting, bloody (hematuria) urination, excessive thirst, excessive penis pain, priapism, chest pain and decreased fertility.

Symptoms may not appear until 4 months after birth and are most severe during an acute episode.

Is Sickle Cell Found only in African Americans?

No. Although African Americans have a high occurrence of Sickle Cell, many other nationalities suffer from the disease.

Can I be tested for Sickle Cell?

You can be tested for sickle cell. Your doctor can perform a simple blood test called the hemoglobin electrophoresis to determine whether you are a carrier for sickle cell or have sickle cell. This test can also be done at a local sickle cell foundation.

Sickle Cell Statistics

  • 1 out of 400 African Americans has sickle cell
  • Sickle Cell affects 8 out of 100,000 people
  • 2 million people carry the sickle cell genetic trait in America

It is most common in West and Central Africa.

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