Sickle cell anemia is a group of inherited red blood cells disorders. This condition occurs when there isn’t enough healthy red blood cells to carry adequate oxygen throughout your body.
Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With sickle cell disease, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells. The cells are supposed to be disc-shaped, but this changes them into a crescent, or sickle, shape.
There’s no cure for most people with sickle cell anemia. But treatments can relieve pain and help prevent problems associated with the disease.
Causes f Sickle Cell Anemia
The cause of sickle cell is a defective gene, called a sickle cell gene.People with the disease are born with two sickle cell genes, one from each parent.
The sickle cell gene is passed from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means that both the mother and the father must pass on the defective form of the gene for a child to be affected.
For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. In the United States, it most commonly affects black people.
Who gets sickle cell disease?
In the United States, most of the people with sickle cell disease are African Americans:
- About 1 in 13 African American babies is born with sickle cell trait
- About 1 in every 365 black children is born with sickle cell disease
Sickle cell disease also affects some people who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds.
Symptoms of sickle cell disease, which vary from person to person and change over time, include:
- excessive fatigue or irritability, from anemia
- jaundice, which is yellowing of the eyes and skin
- Painful swelling of hands and feet.
- frequent infections
- bed-wetting, from associated kidney problems
- pain in the chest, back, arms, or legs
- delayed growth
- Vision problems
Complications of Sickle Cell Anemia
Sickle Cell Disease can lead to many other complications. These are:
- Blindness – Sickle cells can block tiny blood vessels that supply your eyes. Over time, this can damage the portion of the eye that processes visual images (retina) and lead to blindness.
- Leg ulcers- Sickle cell anemia can cause open sores, called ulcers, on your legs.
- Gallstones – The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in your body can lead to gallstones.
- Sickle cell can cause the damage of other organs in the body. This will be as a result of oxygen not reaching those organs.
- People with sickle cell can easily develop high blood pressure especially in adults.
- A stroke can occur if sickle cells block blood flow to an area of your brain. Signs of stroke include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness.
- Men with sickle cell anemia can have painful, long-lasting erections, a condition called priapism.
Before birth, it is important to see important to see a genetic counselor before getting pregnant. You will be checked to see if you and your partner might have traits of sickle cell. You might be given preventive measures and education on how to avoid giving birth to a child with sickle cell anemia.
When to see a doctor if you have sickle cell
You need to see a doctor right away when you experience any of the above symptoms. People with sickle cell will be educated more on the disease and other measures will be put in place to help them.
Treatment of Sickle Cell Anemia
The only cure for sickle cell disease (SCD) is bone marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister.
There are treatments that can help relieve symptoms, lessen complications, and prolong life:
- Antibiotics to try to prevent infections in younger children
- Pain relievers for acute or chronic pain
- Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the blood. This medicine is not right for everyone; talk to your health care provider about whether you should take it. This medicine is not safe during pregnancy.
- Childhood immunizations to prevent infections
- Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications.
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