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What Is Sickle Cell Anemia?
Sickle cell anemia (uh-NEE-me-uh) is a serious disease in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a "C."
Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move easily through your blood vessels. Red blood cells contain the protein hemoglobin (HEE-muh-glow-bin). This iron-rich protein gives blood its red color and carries oxygen from the lungs to the rest of the body.
Sickle cells contain abnormal hemoglobin that causes the cells to have a sickle shape. Sickle-shaped cells don’t move easily through your blood vessels. They’re stiff and sticky and tend to form clumps and get stuck in the blood vessels. (Other cells also may play a role in this clumping process.)
The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ damage.
Overview
Sickle cell anemia is one type of anemia. Anemia is a condition in which your blood has a lower than normal number of red blood cells. This condition also can occur if your red blood cells don’t have enough hemoglobin.
Red blood cells are made in the spongy marrow inside the large bones of the body. Bone marrow is always making new red blood cells to replace old ones. Normal red blood cells last about 120 days in the bloodstream and then die. They carry oxygen and remove carbon dioxide (a waste product) from your body.
In sickle cell anemia, a lower-than-normal number of red blood cells occurs because sickle cells don’t last very long. Sickle cells usually die after only about 10 to 20 days. The bone marrow can’t make new red blood cells fast enough to replace the dying ones.
Sickle cell anemia is an inherited, lifelong disease. People who have the disease are born with it. They inherit two copies of the sickle cell gene—one from each parent.
People who inherit a sickle cell gene from one parent and a normal gene from the other parent have a condition called sickle cell trait. Sickle cell trait is different from sickle cell anemia. People who have sickle cell trait don’t have the disease, but they have one of the genes that cause it. Like people who have sickle cell anemia, people who have sickle cell trait can pass the gene to their children.
Outlook
Sickle cell anemia affects millions of people worldwide. The disease has no widely available cure. However, there are treatments for the symptoms and complications of the disease. Bone marrow transplants may offer a cure in a small number of cases.
Over the past 30 years, doctors have learned a great deal about sickle cell anemia. They know its causes, how it affects the body, and how to treat many of its complications.
Sickle cell anemia varies from person to person. Some people who have the disease have chronic (long-term) pain or fatigue (tiredness). However, with proper care and treatment, many people who have the disease can have improved quality of life and reasonable health much of the time.
Due to improved treatment and care, people who have sickle cell anemia are now living into their forties or fifties, or longer.
Other Names for Sickle Cell Anemia
- Hemoglobin SS disease
- Hemoglobin S disease
- HbS disease
- Sickle cell disorders
- Sickling disorder due to hemoglobin S
- Sickle cell disease
What Causes Sickle Cell Anemia?
Sickle cell anemia is an inherited disease. People who have the disease inherit two copies of the sickle cell gene—one from each parent.
The sickle cell gene causes the body to make abnormal hemoglobin. Hemoglobin is the iron-rich protein that gives blood its red color and carries oxygen from the lungs to the rest of the body.
In sickle cell anemia, the hemoglobin sticks together when it delivers oxygen to the body’s tissues. These clumps of hemoglobin are like liquid fibers. They cause the red blood cells to become stiff and shaped like a sickle, or “C.” The sickled red blood cells tend to stick together and get caught in the blood vessels. (Other cells also may play a role in this process.)
Two copies of the sickle cell gene are needed for the body to make the abnormal hemoglobin found in sickle cell anemia.
Sickle Cell Trait
If you inherit only one copy of the sickle cell gene (from one parent), you will not have sickle cell anemia. Instead, you will have sickle cell trait.
People who have sickle cell trait usually have no symptoms and lead normal lives. However, they can pass the sickle cell gene to their children.
When each parent has a normal gene and an abnormal gene, each child has: a 25 percent chance of inheriting two normal genes; a 50 percent chance of inheriting one normal gene and one abnormal gene; and a 25 percent chance of inheriting two abnormal genes.
Who Is At Risk for Sickle Cell Anemia?
Sickle cell anemia affects millions of people worldwide. It's most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.
In the United States, sickle cell anemia affects about 70,000 people. It mainly affects African Americans. The disease occurs in about 1 out of every 500 African American births. Sickle cell anemia also affects Hispanic Americans. The disease occurs in 1 out of every 36,000 Hispanic American births.
About 2 million Americans have sickle cell trait. The condition occurs in about 1 in 12 African Americans.
To read the rest of this article from the National Heart,
Lung and Blood Institute, please click here: http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhoIsAtRisk.html
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