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Sickle Cell Anemia

What is sickle cell anemia?

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.

What is the cause?

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.

What are the symptoms?

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:

  • Feeling tired all the time
  • Shortness of breath, dizziness, headaches, coldness in hands or feet
  • Skin more pale than usual
  • Yellowing of skin or eyes

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:

  • Ongoing fever higher than 101°F (38.3°C)
  • Cough that produces mucus
  • Vomiting
  • Diarrhea
  • Stomach pain

What are the complications of sickle cell anemia?

Abnormal red blood cells can cause problems in any part of your child’s body. Some of the possible problems are:

  • Infections such as pneumonia or meningitis
  • High blood pressure in the lungs that can cause trouble breathing and heart damage.
  • Acute chest syndrome, which is a life-threatening lung problem that causes fever, chest pain or pressure, cough, or shortness of breath
  • Kidney or bone infection
  • Gallstones
  • Loss of vision caused by damage to blood vessels in the eyes
  • Hip and shoulder joint damage
  • Stroke
  • Damage to tissues in the penis
  • Painful erection of the penis that does not return to normal within 4 hours
  • Too much blood collecting in the spleen
  • Painful leg sores

How is it diagnosed?

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.

How is it treated?

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:

  • Pain medicine
  • Medicine to try to prevent sickle cell crisis. Taken daily, this medicine may help your child have fewer crises. It can also shorten how long each crisis lasts.
  • Vaccines such as Haemophilus influenzae type b, hepatitis A and B, flu, and pneumococcal vaccines to prevent infection. People with sickle cell anemia are more likely to have serious infections, ranging from flu to pneumonia. Infections can cause a sickle cell crisis.
  • Blood transfusions
  • Antibiotics
  • Folic acid to help make red blood cells
  • Bone marrow transplant
  • Medicine to remove extra iron in your child’s body. Your child should not take iron supplements. Sickle cell anemia is not caused by too little iron in the blood. Taking iron supplements can be harmful because the extra iron builds up in your child’s body and can damage his organs.

How can I take care of my child?

To try to prevent sickle cell crises, your child should avoid things that lower the blood oxygen, such as:

  • Strenuous exercise
  • Extreme hot or cold temperatures
  • High altitude
  • Tight clothing
  • Things that can make the blood vessels narrower, such as:
    • Some medicines
    • Cigarette smoking
    • Illegal drugs, such as cocaine

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:

  • Heavy sweating
  • Urinating a lot, which can happen with uncontrolled diabetes or taking diuretics (water pills)
  • Fever
  • Vomiting or diarrhea

Follow the full course of treatment prescribed by your child’s healthcare provider. Ask your provider:

  • How and when you will hear your child’s test results
  • How long it will take for your child to recover
  • If there are activities your child should avoid and when your child can return to normal activities
  • How to take care of your child at home
  • What symptoms or problems you should watch for and what to do if your child has them

Make sure you know when your child should come back for a checkup.

How can I help prevent sickle cell anemia?

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.

For more information, contact:

Developed by RelayHealth.
Pediatric Advisor 2015.2 published by RelayHealth.
Last modified: 2014-06-03
Last reviewed: 2014-06-03
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
Copyright ©1986-2015 McKesson Corporation and/or one of its subsidiaries. All rights reserved.
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