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Sickle Cell Anemia In Children


What is sickle cell anemia?

Sickle cell anemia is a disease that causes red blood cells (RBCs) to be sickle (crescent) shaped instead of round. This causes your child's body to break down the RBCs faster than usual. RBCs carry oxygen to all the organs and tissues. When RBCs break down faster than usual, less oxygen reaches your child's body. Sickle cell anemia is a serious form of sickle cell disease. Your child's risk increases if both his parents have the gene for sickle cell anemia. Your child's healthcare provider can confirm your child has sickle cell anemia with a blood test.

What are the signs and symptoms of sickle cell anemia?

  • Pale skin
  • Swollen hands and fingers
  • Pain throughout his body

How is sickle cell anemia managed?

Your child may need ongoing screening for conditions that can develop because of sickle cell anemia. Examples include kidney disease, hypertension (high blood pressure), retinopathy (eye problems), and problems with his lungs.

  • Medicines may be given to decrease pain or to decrease sickling of your child's RBCs. Your child may also need medicine to treat or prevent a bacterial infection.
  • NSAIDs , such as ibuprofen, help decrease swelling, pain, and fever. This medicine is available with or without a doctor's order. NSAIDs can cause stomach bleeding or kidney problems in certain people. If your child takes blood thinner medicine, always ask if NSAIDs are safe for him. Always read the medicine label and follow directions. Do not give these medicines to children under 6 months of age without direction from your child's healthcare provider.
  • Acetaminophen decreases your child's pain and fever. It is available without a doctor's order. Ask how much to give your child and how often to give it. Follow directions. Acetaminophen can cause liver damage if not taken correctly.
  • Do not give aspirin to children under 18 years of age. Your child could develop Reye syndrome if he takes aspirin. Reye syndrome can cause life-threatening brain and liver damage. Check your child's medicine labels for aspirin, salicylates, or oil of wintergreen.
  • A blood transfusion replaces blood with RBCs that are not sickle shaped. Your child may need a blood transfusion more than once.
  • Surgery may be done to remove part or all of your child's spleen or gallbladder.

How can I help my child manage pain?

  • Monitor your child for signs of pain. Watch for redness or swelling in his hands or feet. If he is too young to talk, watch his face and look for other signs of pain. If he is old enough to talk, ask him where he feels the pain and how bad it is. Have him use a pain scale to show you how much pain he feels.
    Pain Scale
  • Apply heat to areas of pain. Use a heating pad or place your child in a warm bath. Do this for 20 to 30 minutes every 2 hours for as many days as directed by your child's healthcare provider. Heat can help decrease your child's pain.
  • Massage areas where your child feels pain. This can help decrease pain and may help him relax.
  • Help your child balance rest and exercise. Have him rest during a sickle cell crisis. Over time, he can increase his activity to a moderate amount. Activity may help decrease pain. Ask your child's healthcare provider which activities are safe for your child. He may need to avoid activities that increase his risk for injury, such as football.

What can I do to prevent a sickle cell crisis in my child?

  • Give your child vitamins and minerals as directed. Folic acid can help prevent blood vessel problems that can occur with sickle cell anemia. Zinc may decrease how often your child has pain.
  • Give your child liquids as directed. Dehydration can increase his risk for a sickle cell crisis. Ask how much liquid he should drink each day and which liquids are best for him.
  • Keep your child out of the cold. Do not let him go from a warm place to a cold place quickly. Keep him warm in the winter. Do not let him go swimming in cold water.
  • Ask about vaccinations your child needs. Vaccinations can help prevent a viral infection that may lead to a sickle cell crisis. Take him to get a flu shot every year as directed. He may also need a pneumonia vaccine every 5 years.
  • Talk to caregivers, teachers, and others in your child's school. Tell them that your child has sickle cell anemia. Teach them the signs and symptoms of a crisis, acute chest syndrome, and infection. Teach them what to do if they see any signs or symptoms of these problems.
  • Talk to your child about harmful behaviors. Tell your child not to smoke or be around secondhand smoke. Tell him not to drink alcohol. Smoking cigarettes or drinking alcohol increases his risk for a sickle cell crisis.

Call 911 for any of the following:

  • Your child has a cough , shortness of breath, or chest pain.
  • Your child has a new, different, or more severe headache.
  • Your male child's penis stays erect for 2 hours.
  • Your child says that he cannot see out of one or both eyes.
  • Your child is confused, has problems speaking, or has weakness or numbness in his arm, leg, or face.

When should I seek immediate care?

  • Your child is sleepy, pale, and has low energy.
  • Your child has abdominal pain, is bloated, or is vomiting a lot.
  • Your child is not acting like himself, he has a seizure, or he faints.
  • Your child's pain does not decrease after you give him pain medicine.
  • Your child tells you that he cannot cope with his pain or the disease, or that he wants to die.

When should I contact my child's healthcare provider?

  • Your child has a fever higher than 100.4°F (38°C).
  • Your child has a fever, or cold, or the flu.
  • You see blood in your child's urine.
  • Your child has new symptoms.
  • Your child is constipated or has diarrhea.
  • Your child has changes in his vision.
  • You have questions or concerns about your child's condition or care.

Care Agreement

You have the right to help plan your child's care. Learn about your child's health condition and how it may be treated. Discuss treatment options with your child's caregivers to decide what care you want for your child. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2016 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.