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Diseases - Sickle Cell Anemia

What is Sickle Cell Anemia?
How can I carry the trait for Sickle Cell Anemia?
What are the signs and symptoms of Sickle Cell Anemia?
How can Sickle Cell Anemia be treated?
How can I get more information on Sickle Cell Anemia?

What is Sickle Cell Anemia?

Sickle Cell Anemia affects about 50,000 African Americans. About 9 % of African Americans carry the trait for Sickle Cell Anemia. Sickle Cell Anemia is a disease caused by a problem with the body's red blood cells. The genetic defect affects the red blood cells hemoglobin. The hemoglobin's job is to carry oxygen to all the cells and tissues of the body. Normal red blood cells are soft and round like little doughnuts. The normal red blood cells bend and flow through the tube-like blood vessels to deliver oxygen to all of the body parts. Sickle cells are different. The sickle cell red blood cells are stiff and curved in shape and difficult to flow through the blood vessels.

The hard, curved sickle cells clog the inside of small blood vessels and keeps blood from delivering oxygen to all the body parts and cause pain. Normal red blood cells live for 120 days and the body makes new red blood cells. The life span of the sickle cell is much shorter.

What is the Sickle Cell trait?

Sickle cell anemia is an inherited disease. A person will be born with sickle cell disease if they receive the genes from both the mother and the father. If the gene is inherited from one parent the person is considered a sickle cell carrier. A carrier has an increased chance of having a child with sickle cell disease. The person with sickle cell trait has received a message for normal and sickle cells.

What are the signs and symptoms of Sickle Cell Anemia?

Symptoms and complications of Sickle Cell Anemia vary with each individual. The symptoms include the following:

  • Anemia: the ability of red blood cells to carry oxygen is reduced due to the sickle shape of the red blood cells. The body will become dehydrated and the individual may have a fever. Severe anemia will make the individual pale and tired. The body's ability to heal, and normal growth and development may be delayed.

  • Pain Crisis (Sickle Crisis): When the blood flow is blocked to an area due to a sickled cell, pain will occur. The pain may occur anywhere, most often in the chest, arms or legs. Any interruption in blood flow to the body will result in pain, swelling, and possible death of the tissues not receiving blood and oxygen.

  • Acute Chest Syndrome: When sickling is in the chest. This is life threatening. It occurs suddenly, when the body is under stress from infection, fever and dehydration. The sickled red blood cells stick together and block the flow of oxygen to the tiny vessels in the lungs. Permanent lung damage may result.

  • Splenic Sequestration (pooling): The spleen may become enlarged and painful due to increase blood volume in the spleen. There is a sudden drop in Hemoglobin (iron containing pigment in the blood that carries oxygen from lungs to tissue). There is a risk for infection due to the spleen not working properly.

  • Stroke: The sickle cells may block the blood vessels carrying blood to the brain. Without oxygen from the blood a stroke will occur, resulting in devastating brain damage.

  • Jaundice (yellowing of the skin eyes or oral mucosa): The sickle cell red blood cells do not live as long as normal red blood cells and are dying too fast for the liver to filter them out. Billirum (which causes the yellow color) will build up in the blood system causing jaundice.

All major organs are affected by sickle cell disease. The liver, heart, kidneys, gallstones, eyes, bones and joints can suffer damage. Problems include:

  • Increased infections
  • Leg ulcers
  • Bone damage
  • Gallstones
  • Kidney damage and loss of water in urine
  • Eye damage.
How can Sickle Cell Anemia be treated?

Early diagnosis and prevention of complication is critical in sickle cell treatment. Treatment options may include:

  • Pain Medication for sickle cell crisis
  • Drinking plenty of water daily (8 to 10 glasses) or receiving fluid intravenously to prevent and treat pain crisis.
  • Blood transfusions for anemia and to prevent stroke.
  • Penicillin to prevent infections.
  • Folic Acid to prevent severe anemia.
  • Hydroxyurea is a medication used to reduce frequent pain crisis and acute chest syndrome.
  • Bone Marrow transplant has been effective in curing some children with sickle cell.

The life expectancy for individuals has greatly increased. Individuals with Sickle Cell Anemia are now living into their mid-40s and beyond.

How can I get more information on Sickle Cell Anemia? http://www.fda.gov/fdac/features/496_sick.html
http://www.kidshealth.org/kid/health_problems/blood/sickle_cell.html
http://www.rhofed.com/sickle/
For more information, contact:
Rosetta Swinton, RN
Episcopal Director of Health
Ph: (843) 876-1949
Fax: (843) 792-7476
swintonr@musc.edu