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Frequently Asked Questions
 
What is Sickle Cell Disease?

Sickle cell disease is an inherited blood 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.

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What causes Sickle Cell Disease?

Sickle cell anemia is an inherited condition. People with sickle cell anemia inherit two copies of the sickle cell gene, one from each parent.
The sickle cell gene makes abnormal hemoglobin. Hemoglobin is the protein inside red blood cells that carries oxygen to all parts of the body and gives blood its red color.
In sickle cell anemia, the abnormal hemoglobin sticks together when it gives up its oxygen to the tissues. These clumps are like liquid crystals that cause red blood cells to become stiff and shaped like a sickle, or “C.” It takes two copies of the sickle cell gene for the body to make the abnormal hemoglobin found in sickle cell anemia.

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Sickle Cell Disease vs Sickle Cell Trait.

People who inherit only one copy of the sickle cell gene (from one parent) will not have sickle cell anemia. They will have sickle cell trait.
People who have sickle cell trait generally have no symptoms and lead normal lives. Like people with sickle cell anemia, however, they can pass the sickle cell gene on to their children. The following image shows how two parents with sickle cell trait pass along the sickle cell gene.

 

Listing of the most Frequently Asked Questions

Sickle Cell Disease Diagnosis.

A blood test can check for hemoglobin S — the defective form of hemoglobin that underlies sickle cell anemia. In most states 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.

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History of Sicle Cell Disease.

The symptoms related to sickle cell crises were known by various names in Africa, long before they were recognized in the western hemisphere 1 . Symptoms of sickle cell anemia could be tracked back to year 1670 in one Ghanian family 2 . It was in 1910 when James Herrick 3 observed, “peculiar elongated sickle shaped RBCs” in the blood of an anemic black medical student, and then the scientific community came to know about it.

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Children and Sickle Cell Disease.

Most children with sickle cell disease feel and look fine most of the time. When they do have medical problems, they can become sick very quickly and with little warning. This is why it is important to test newborns. Identifying infants with sickle cell disease in the first weeks of life can help prevent some of the serious medical problems of the disease.

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Pregnancy and Sickle Cell Disease.

Women with sickle cell disease who become pregnant are at higher risk for complications, but serious problems have dropped significantly over the past decades. A 2001 study reported a higher risk for premature birth and low birth weight in the baby, and a higher risk for infections and hospital visits in the mother after delivery.

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What are Sickle Cell Disease Signs and Symptons?

People with sickle cell trait have one gene for the disease. They don't develop
the disease and usually have no signs and symptoms. Approximately one in 12
black Americans has sickle cell trait.

People with sickle cell anemia have two genes for the disease — one from each
parent. They usually show some signs and symptoms after 4 months of age. Some
people with sickle cell anemia have few symptoms. For others, the disease is
more severe and they may require repeated hospitalizations.

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Sickle Cell Disease and Stroke.

Stroke is an important and common complication of sickle cell disease (SCD), affecting children as well as adults. Clinically evident stroke, usually brain infarction, is usually associated with stenosis or occlusion of the intracranial arteries of the Circle of Willis

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Testing for Sickle Cell Disease.

A blood test can check for hemoglobin S — the defective form of hemoglobin that underlies sickle cell anemia. In most states 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.

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Treatment for Sickle Cell Disease?
Bone marrow transplant offers the only potential cure for sickle cell anemia. But very few people have a suitable donor for transplant. As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications.

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Incident of Sickle Cell Disease?

Sickle cell anemia affects millions throughout the world. It is particularly common among people whose ancestors come from sub-Saharan Africa; Spanish-speaking regions (South America, Cuba, Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy. In the Unites States, it affects around 72,000 people, most of whose ancestors come from Africa. The disease occurs in about 1 in every 500 African-American births and 1 in every 1000 to 1400 Hispanic-American births. About 2 million Americans, or 1 in 12 African Americans, carry the sickle cell trait.

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Stem Cell Research and Sickle Cell Disease?

Red Cell Substitute May Work Against Sickle Cell Disease

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