Have you ever seen a sickle? It's a farm tool with a curved, sharp edge for cutting wheat. Sickle cell anemia (say: sih-kul sell uh-nee-mee-uh) is a disease of the blood. It gets its name because a person's red blood cells are shaped like sickles, or crescent moons, instead of their usual round, flat shape.
Round and flat is the healthiest shape for red blood cells because they can move easily through the body. Red blood cells carry oxygen around your body. And every part of your body needs oxygen to work properly.
Red blood cells are made inside the bones in the soft, spongy area called the bone marrow (say: mar-o). Every time you take a breath, you breathe in oxygen. And your red blood cells carry oxygen to every cell in your body. Any time a person's body doesn't have enough red blood cells, it's called anemia. When the cause is the sickle shape of the red blood cells, it's called sickle cell anemia.
When red blood cells are shaped like sickles or moons, they can get stuck and die, especially inside smaller blood vessels. This keeps blood from flowing properly in the body and it also causes a lot of pain. Important organs like the brain, heart, and kidneys need constant blood flow to work properly.
A person's body knows that the sickle cells aren't good, so it attacks and destroys them. But the body can't make new blood cells fast enough to replace the old ones.
Signs and Symptoms
Kids who have sickle cell anemia may feel pain in their chest, stomach, or bones when a blood vessel gets clogged with sickle cells. The pain can last a few minutes or several days, and it might hurt a lot or just a little. When this happens, it's called a sickle cell crisis (a crisis means a time of trouble). Nobody knows exactly when sickle cells might get stuck or which blood vessels might get clogged. Certain conditions, like if a person gets too cold or gets sick, can lead to a sickle cell crisis. Doctors and nurses can help by giving strong medicine to relieve the pain.
Because kids with sickle cell anemia don't have enough normal red blood cells, they get tired more easily. They also get infections more often than other kids do. They may not grow as fast or feel as hungry as their friends.
Sometimes the whites of their eyes have a yellowish color, known as jaundice (say: jon-dus), and they may have to go to the bathroom a lot. In little kids - usually those under age 2 - sickle cell anemia can cause their hands and feet to swell and hurt.
What Causes Sickle Cell Anemia?
Sickle cell anemia is an inherited (say: in-hair-uh-ted) disease. That means you can't catch sickle cell anemia like you can catch a cold or the flu. Kids are born with the disease when parents pass along the sickle cell anemia gene to their children.
More African Americans have sickle cell anemia than any other group of people. About one out of every 500 African Americans has the disease. But some people whose ancestors came from countries around the
Some scientists think sickle cell anemia may be connected to malaria (say: muh-lar-ee-uh), a serious and sometimes deadly disease that was very common in those countries. It is believed that people who carry the gene for sickle cell anemia are less likely to catch malaria. So more of these people survived and passed on the sickle cell gene to their children.
How Does a Doctor Know a Kid Has It?
Special blood tests can tell a doctor if a kid has sickle cell disease. The tests will show the doctor the type of hemoglobin (say: hee-muh-glow-bin) in the kid's red blood cells.
Hemoglobin is the part of the blood that carries oxygen to different parts of the body in the red blood cells. There are different types of hemoglobin, including hemoglobin A and hemoglobin S. Normal red blood cells contain hemoglobin A, but people with sickle cell disease have mostly hemoglobin S (the "S" stands for sickle) in their red blood cells.
How Is Sickle Cell Anemia Treated?
Most kids with sickle cell anemia take penicillin (say: peh-nuh-sih-lun), a drug that helps prevent infections. A doctor may also prescribe a vitamin supplement called folic acid. Folic acid helps the body make new red blood cells.
Taking penicillin or vitamins does not cure sickle cell anemia, but it can help keep a kid with sickle cell anemia from getting sick. Pain medicine also helps kids with this disease. A kid with sickle cell anemia may need to go to the hospital if he or she has a lot of pain or a serious infection. At the hospital, the kid can get IV fluids, antibiotics, or other medicine.
Sometimes kids with sickle cell anemia need blood transfusions (say: tranz-fyoo-shuns). That's a way to put healthy blood cells right into a kid's body. A blood transfusion raises the amount of normal hemoglobin in the blood. It also decreases the chances that blockage, or crisis, will happen.
Can Sickle Cell Anemia Be Cured?
In some cases, a bone marrow transplant can cure sickle cell anemia. Bone marrow transplants replace the sickle cells with healthy cells from a donor (say: doe-nur). A donor is a person who gives healthy bone marrow or other organs or body parts to someone else who needs it. Not just any bone marrow will do. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister.
What Can a Kid Do to Stay Well?
Taking penicillin every day helps prevent infections in kids who have sickle cell anemia. It's important for kids with sickle cell anemia to stay as healthy as possible. That means eating healthy foods and drinks and getting plenty of sleep. Kids with the disease can play and exercise, but they should not get too hot, too cold, or too tired.
And if a kid with the disease gets a fever, pain, or any other problems, he or she needs to tell an adult right away. Fast treatment is very important, so the kid can get back to feeling good again soon.
Updated and reviewed by: Barbara P. Homeier, MD
Date reviewed: June 2005
Originally reviewed by: Steven Dowshen, MD
Source : http://www.kidshealth.org/kid/health_problems/blood/sickle_cell.html
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