WORLD SICKLE CELL DAY

African American Women & Sickle Cell Anemia

Sickle cell anemia (uh-NEE-mee-uh) is a disease affecting many African-Americans. It causes problems with the red blood cells. Normal red blood cells are round and smooth and move through blood vessels easily. Sickle cells are C-shaped and become hard and sticky. They can get stuck in small blood vessels, blocking blood flow. This can cause pain and organ damage. Sickle cells die quickly, but your body cannot make new red blood cells to replace them fast enough. This leads to anemia. People who have sickle cell anemia need constant treatment to prevent severe pain and serious health problems.

Each year, about 1 in 500 African-Americans is born with sickle cell disease, which runs in families. This means that the gene that causes sickle cell anemia is passed down from parents to children. People who have sickle cell disease got the sickle cell gene from both parents. People who have only one sickle cell gene are said to have the sickle cell "trait." They do not have the disease, but they can pass the gene to their children. About 1 in 12 African-Americans has the sickle cell trait.

If you want to have a baby, you and your partner can get a simple blood test to see if you have the sickle cell trait. If you both have the trait, your child will have a 1 in 4 chance of getting sickle cell anemia. If you both have the trait, talk to your doctor about your options. All 50 states test newborn babies for sickle cell anemia as part of their newborn screening programs.

What Is Sickle Cell Anemia?

Sickle cell anemia (uh-NEE-me-uh) is the most common form of sickle cell disease (SCD). SCD is a serious disorder in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a crescent.

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 an iron-rich protein called hemoglobin (HEE-muh-glow-bin). This protein carries oxygen from the lungs to the rest of the body.

Sickle cells contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle hemoglobin causes the cells to develop a sickle, or crescent, shape.

Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain, serious infections, and organ damage.

Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin. Figure B shows abnormal, sickled red blood cells blocking blood flow in a blood vessel. The inset image shows a cross-section of a sickle cell with abnormal (sickle) hemoglobin forming abnormal strands.

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 contain 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 live 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, the number of red blood cells is low 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 genes for sickle hemoglobin—one from each parent.

People who inherit a sickle hemoglobin gene from one parent and a normal gene from the other parent have a condition called sickle cell trait.

Sickle cell trait is different than 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 sickle hemoglobin gene on to their children.

Outlook

Sickle cell anemia has no widely available cure. However, treatments can help with the symptoms and complications of the disease. Blood and marrow stem cell transplants may offer a cure for a small number of people.

Over the past 100 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 treatments and care, people who have sickle cell anemia are now living into their forties or fifties, or longer.

Other Names for Sickle Cell Anemia

• HbS disease
• Hemoglobin S disease
• Hemoglobin SS disease
• Sickle cell disease (a broad term that includes sickle cell anemia)
• Sickle cell disorders (a broad group of conditions that includes sickle cell anemia)
• Sickling disorder due to hemoglobin S

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