Sickle cell anemia is a blood disease that causes abnormal red blood cells. Red blood cells carry oxygen from the lungs to the rest of the body. A chemical in the red blood cells called hemoglobin helps the cells carry oxygen. If your child has sickle cell anemia, most of his red blood cells have an abnormal type of hemoglobin called hemoglobin S. Hemoglobin S changes the shape of red blood cells. Instead of being soft and round, the cells are a stiff crescent (sickle) shape. This shape makes it harder for the blood cells to pass through small blood vessels. Sickle cells tend to clump together, which blocks the flow of oxygen to the muscles, bones and organs of the body. This causes severe pain and can damage the body tissues.
Normal blood cells usually last about 4 months and are constantly being replaced. The abnormal blood cells are fragile and only last a few weeks. Your child’s body may not be able to make new red blood cells fast enough. This can cause a shortage of red blood cells. The shortage of red blood cells is called anemia.
Sickle cell anemia is inherited. Genes are in each cell of the body. They contain the information that tells the body how to develop and work. Changes in the genes can be passed from parents to children. If your child inherited the gene from both parents, it is likely your child will have the disease. If your child inherited the faulty gene from just 1 parent, usually your child will not have the disease, but can pass the gene on to his children.
Africans, African Americans, and East Indians have a high risk of the disease. It is also more common in people from Italy, Greece, the Middle East, the Caribbean, and Central or South America.
Babies are born with a kind of hemoglobin that protects the red blood cells from sickling right away. Around age 4 to 5 months, this newborn hemoglobin is replaced by "adult" hemoglobin and the cells start to sickle. After 4 months of age, symptoms are often brought on by an infection.
The symptoms are different from person to person and can be mild to severe. Most people with sickle cell anemia have times when they have symptoms and times when they do not have symptoms. The most common symptoms are related to anemia and pain.
Symptoms of anemia may include:
When abnormal red blood cells block the flow of blood and cause symptoms, it is called a crisis. The most common symptom of a sickle cell crisis is pain. The pain is usually felt in the part of the body where the sickling cells are blocking blood flow. Crises may start suddenly and last from a few days to several weeks. The pain may be mild to severe. Although not as common as a crisis, you may also have chronic (ongoing) pain, which is often felt in the bones.
Toddlers and children may have frequent pain with or without any other signs of illness.
Sickle cells crises can cause damage to many organs. Damage to the spleen can make it easier to get an infection and harder to fight infections. Symptoms may include:
Abnormal red blood cells can cause problems in any part of your child’s body. Some of the possible problems are:
In the US, sickle cell anemia is usually diagnosed at birth with a newborn screening test.
If your child has never been screened for sickle cell anemia, your child may have blood tests to look for anemia, sickle-shaped red blood cells, and abnormal hemoglobin.
If your child has sickle cell anemia, it is very important for you to work with healthcare providers who have experience treating sickle cell disease.
Treatments may include:
To try to prevent sickle cell crises, your child should avoid things that lower the blood oxygen, such as:
Also try to avoid dehydration. A loss of too much fluid from your child's body can cause sickling. Your child may lose too much fluid from:
Follow the full course of treatment prescribed by your child’s healthcare provider. Ask your provider:
Make sure you know when your child should come back for a checkup.
People who have the gene may pass the disease on to their children. Couples can get tested for the gene that causes sickle cell disease. If one of you has the gene, you will need to decide whether to take the chance of passing the disease on to your children. You may want to talk with a genetic counselor if anyone in your family has sickle cell anemia.
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