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Sickle Cell Disease in Children

What is sickle cell disease?
Sickle cell disease, also called sickle cell anemia, is a hereditary (meaning that children can inherit it from their parents) problem that causes a type of faulty hemoglobin in red blood cells. Hemoglobin carries oxygen in the blood.

Normal red blood cells are disc-shaped and very flexible. In sickle cell disease, some red blood cells can change shape so that they look like sickles or crescent moons. Because of their shape, they don't move well through the smallest blood vessels. This can stop or slow blood flow to parts of the body, causing less oxygen to reach these areas. The sickle cells also die earlier than normal blood cells, which can cause a shortage of red blood cells in the body.


Who gets sickle cell disease, and how?
Sickle cell disease is most common in people whose ancestors came from Africa, Central America (especially Panama), South America, Caribbean nations, Mediterranean countries, India or Near Eastern countries.

To get sickle cell disease, a child has to inherit the sickle hemoglobin gene from one parent and a sickle gene or another abnormal hemoglobin gene from the other parent.

People with one normal gene and one sickle gene are carriers of the abnormal gene. This means that they have the sickle cell trait but not sickle cell disease. A child born to parents who both have sickle cell trait has a 25 percent chance of getting sickle cell disease.

If a child has one parent who has sickle cell trait and the other parent has a gene for another kind of abnormal hemoglobin, the child has a chance of getting a different kind of sickle cell disease.


What happens to red blood cells in sickle cell disease, and what problems can this cause?
Picture 1.
Normal red blood cells.
When the red blood cells of people with sickle cell disease don't get enough oxygen, these cells change shape. They become longer and curved. Some people think they look like the blade of a cutting tool called a "sickle." Picture 1 shows normal red blood cells, and picture 2 shows sickle cells.

Sickle cells can get stuck in blood vessels and keep blood from reaching parts of the body. This causes pain and can damage the body's internal organs. Blocked blood vessels in the arms, legs, chest or abdomen can cause strong pain. Children with sickle cell disease might get more infections because their spleen is damaged by sickle cells. (One of the spleen's main jobs is to protect against infection.) When sickle cells block blood flow to organs and cause pain and other problems, this is called a "sickle cell crisis," or a "pain crisis."


Blocked blood vessels in the brain can cause a stroke. This can cause brain cells to die. Strokes affect about one in every 10 children with sickle cell disease.

Your doctor can do a special test to see if your child is at risk of a stroke. If your child is two years or older, you should ask your doctor if the test is needed. If the test shows a higher risk of stroke, your doctor will talk with you about the use of regular blood transfusions.

If your child has weakness in an arm or leg, has slurred speech, refuses to walk, or has unusual behavior, it may be a sign of a stroke; take him or her to the doctor right away.


How can my doctor tell if my baby has sickle cell disease?
If you are at risk because of your family history or ethnic group, ask your doctor to check you and your sexual partner for sickle cell trait or disease before you get pregnant. Then you will know if you might have a child with sickle cell disease. Your doctor might want you to get genetic testing. If you are already pregnant, you might get testing for your fetus.

Most states test newborn babies for sickle cell disease. If your state does not do this, your doctor can get a special blood test for your baby. This test will show if your baby has sickle cell disease.


How is sickle cell disease treated?
If your child has sickle cell disease, he or she is at risk for some infections, lung problems and pain. Your child may need to take an antibiotic (usually penicillin) to prevent bad infections. Also, certain vitamins, like folic acid, can help your child's body replace damaged blood cells. Your child needs to have all of the recommended shots for children. Your child will also need a few special shots.

Your child will need to see your family doctor often for blood tests and to be checked for damage to internal organs. If your child has pain, fever, weakness or trouble breathing, he or she may need IV (intravenous) fluids (liquids given through a needle placed in your child's vein) and antibiotics. Your child may also need oxygen, blood transfusions and strong pain medicines. Special treatments will be needed if your child has organ damage.


When should I call my child's doctor?
You should call your doctor right away if your child has any of these signs:


    -
Swollen hands or feet

    -
Sudden paleness of the skin or nail beds

    -
Yellow color of the skin or eyes

    -
Fever or signs of infection

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Swelling in the abdomen (tummy)

    -
Sudden tiredness with no interest in what is going on

    -
Erection of the penis that won't go away

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Trouble hearing or seeing

   - Weakness on one side of the body or a sudden change in speech

   - Headache

   - Trouble breathing

   - Joint, stomach, chest or muscle pain, or limping


Can sickle cell disease be cured?
Generally, no. But with good care, people with sickle cell disease can live a mostly normal life. Bone marrow transplants can cure the disease in a small number of people.

They are you website people!
Disclaimer: Information on this web site is provided for informational purposes only and is not a substitute for professional medical advice. You should not use the information on this web site for diagnosing or treating a medical or health condition. You should carefully read all product packaging. If you have or suspect you have a medical problem, promptly contact your professional healthcare provider.
Article courtesy of
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