Sickle Cell Disease In Children

What is sickle cell disease?

Sickle cell disease is a genetic disease that causes your child's body to break down too many red blood cells (RBCs). RBCs carry oxygen to all the organs and tissues of your body. Your child is at risk for sickle cell disease if both of his parents have the gene for sickle cell disease. Your child's healthcare provider can confirm he has sickle cell disease from the shape of his RBCs.

What are the signs and symptoms of sickle cell disease?

  • Pale skin

  • Swollen hands and fingers

  • Frequent headaches

  • Shortness of breath

  • Pain throughout his body

What are the risks of sickle cell disease?

  • Sickle cell disease increases your child's risk for infection. Your child may not grow as fast as other children. He may reach puberty at a later age than other children. He may also go through times when he has increased pain and swelling. Your child may have a sickle cell crisis. This may cause severe pain and damage to organs, such as his kidneys or spleen. Sickle cell disease may also cause jaundice (yellowing of his skin and eyes).

  • Sickle cell disease may lead to long-term lung, eye, or bone problems. It may cause boys to have painful erections (priapism). Sickle cell disease increases your child's risk for a seizure or stroke. Even with treatment, sickle cell disease increases the risk for organ failure, such as liver or kidney failure. These conditions may become life-threatening.

How is sickle cell disease treated?

  • Medicines decrease pain. Medicine may also be given to decrease sickling of your child's RBCs. Your child may also need medicine to treat or prevent a bacterial infection.

  • NSAIDs help decrease swelling and pain or 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 doctor.

  • 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.

How can I help my child manage pain?

Follow the directions of your child's healthcare provider for medicine to manage your child's pain. The following may also help monitor, manage, and decrease your child's pain:

  • Monitor your child for signs of pain. Watch for redness or swelling in his hands or feet. If he is young, 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. Staying active 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.

How can I 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 disease. 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 which vaccinations your child needs. Vaccinations can help prevent a viral infection that may lead to a sickle cell crisis. He should get a flu shot every year. He may also need a vaccine to protect him the hepatitis B virus.

  • Talk to caregivers, teachers, and others in your child's school. Tell them that your child has sickle cell disease. 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 that he should not smoke or be around secondhand smoke. Tell him to avoid alcohol. Smoking or drinking alcohol increases his risk for a sickle cell crisis.

When should I contact my child's healthcare provider?

  • Your child has a fever, a cold, or the flu.

  • There is 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.

When should I seek immediate care or call 911?

  • Your child has a cough, shortness of breath, or chest pain.

  • Your child has a new, different, or more severe headache.

  • Your child is confused, has problems speaking, or has weakness or numbness in his arm, leg, or face.

  • Your child is sleepy, pale, and his stomach is swollen.

  • 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 says that he cannot see out of one or both eyes.

  • Your child tells you that he cannot cope with his pain or the disease, or that he wants to die.

  • Your son's penis stays erect for 2 hours.

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.

© 2014 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.

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