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Sickle Cell Disease In Children

What is sickle cell disease?

Sickle cell disease occurs when red blood cells (RBC's) form abnormal curved shapes called sickles. Sickle-shaped red blood cells break down much faster than normal cells. Fast red blood cell breakdown leaves your child with fewer red blood cells than he needs. Not having enough red blood cells causes problems such as anemia. The sickle shape of these cells also causes problems, because the cells tend to get stuck in blood vessels. They block blood vessels, and do not let enough blood flow to where it needs to go in your child's body.

What are red blood cells and how are they made?

  • Red blood cells are round cells that are made in the spongy filling (marrow) of many bones in your child's body. The job of red blood cells is to carry oxygen to all parts of the body. Normally, when red blood cells get old or damaged, the spleen helps break them down. The spleen is an organ on the upper left side of the abdomen. Red blood cells are made from a protein that contains iron. The iron from old red blood cells is used by the body to make more red blood cells. The liver is an organ in the upper right side of the abdomen. The liver helps remove waste from broken-down red blood cells.

  • With sickle cell disease, the hemoglobin in your child's red blood cells is abnormal. Hemoglobin is a protein in healthy red blood cells that carries oxygen. Sickle cell hemoglobin can cause red blood cells to change their shape and get sticky. These sticky cells can block blood vessels. With sickle cell disease, red blood cells also die much quicker than normal red blood cells. When this happens, your child may not have enough red blood cells.

What causes sickle cell disease?

Sickle cell disease is passed from parents to children through their genes. A child gets sickle cell disease if both of his birth parents have the gene for sickle cell disease. Genes are little pieces of information in the body that tell it what to do, or what to make. If your child has a gene from only one birth parent, he has sickle cell trait. Children with sickle cell trait can pass the gene for sickle cell disease on to their own children.

What are the signs and symptoms of sickle cell disease?

  • Pain: Pain is often felt by children who have sickle cell disease. Your child may feel pain in his back, stomach, and chest. The bones in his arms and legs may also hurt. Pain can be there almost all of the time, or it can come and go. The pain can stay in one body area, or spread to other areas. The pain can last for minutes, hours, or longer than a week. Your child may not know when the pain will come. The pain may be mild or severe (very bad). A pain scale can be used by children to tell you how bad the pain is.

  • Headaches: Your child may get headaches. Headaches that are new or different can be a sign of a sickle cell crisis.

  • Trouble breathing: Your child may have trouble breathing when he is active and when he is resting.

  • Swollen hands and fingers: Swollen hands and fingers are called dactylitis. This problem happens most often in infants with sickle cell disease.

What medical problems can be caused by sickle cell disease?

  • Anemia: With anemia, your child will feel more tired than usual. He may not feel like doing activities and sports. He may have trouble breathing, and his skin color may fade to very light or white.

  • Infections: With sickle cell disease, your child may get many infections in his lungs and other body areas. Infections can cause fevers (high body temperature), and swelling. Trouble breathing and coughing are signs of a lung infection. Your child may get wounds (sores) on his legs that do not heal. These sores may get infected and be painful.

  • Episodic painful crisis: This is also called a sickle cell crisis, or vaso-occlusive crisis. This happens when RBC's get stuck in blood vessels, blocking blood flow and oxygen from reaching body areas. This causes severe (very bad) pain, and can damage body organs such as the kidneys. A stroke and other medical problems can also be caused by blocked blood flow.

  • Jaundice: With jaundice, your child's skin turns yellow in color, and the white areas of his eyes change to yellow.

  • Acute chest syndrome: Chest pain and trouble breathing are signs of acute chest syndrome. You may hear wheezing when you child breathes, and he may have a cough. He may also cough up blood, have a fever and chills, and have abdominal (belly) pain.

  • Priapism: This is when a boy's penis is very painful when it is erect. This can be a sign of a sickle cell crisis. This problem may go away without treatment. If it does not go away, the pain can be severe, and can lead to long-lasting problems.

  • Acute splenic sequestrian crisis: Signs and symptoms of this problem can come on suddenly. Your child's abdomen will swell up, and his skin will change to white or light-colored. Your child may feel very sleepy and complain of stomach pain. If you have been taught to feel your child's abdomen to check the size of his spleen, you will find that it is larger than it should be.

  • Growth delays: Children with sickle cell disease may not grow as fast as other children. Your adolescent child may not mature sexually as early as other teenagers his age.

How is sickle cell disease diagnosed?

Caregivers learn if your child has sickle cell disease by testing a sample of his blood. Sickle cell disease is usually found at birth. Sickle cell disease may be found later in life if a child has signs or symptoms of the disease. Urine tests, x-rays, computed tomography (CT) scans and other tests can show if the disease is getting worse. These tests can also show if the disease is causing other medical problems.

What medicines may be used to treat sickle cell disease?

  • Pain medicine: Pain medicine may be needed to help your child cope with his pain. Medicines such as acetaminophen or ibuprofen may be suggested by your child's caregiver for mild pain. You may need to give your child stronger pain medicine if the pain is worse. Ask you child's caregiver what to use to treat pain. Ask when to take your child to the hospital if the pain does not decrease or go away.

  • Antibiotic medicine: This medicine is given to help prevent or fight infections.

  • Bronchodilators: This medicine may help your child breathe more easily.

  • Vitamins: Your child may need to take a vitamin called folic acid. Folic acid can help prevent blood vessel problems that can come with sickle cell disease. Your child may also need to take a multivitamin every day.

What else may be used to treat sickle cell disease?

Treatment can decrease the amount of pain your child feels. Certain treatments may also decrease his risk of having problems such as a stroke or a sickle cell crisis. Treatments can also give him more energy to do activities. Your child should get enough rest and sleep. If he is having pain and trouble walking, he may need to use crutches or a wheelchair for a time. Ask your child's caregiver about new treatments for sickle cell disease that are being tested. Your child may also need one or more of the following treatments:

  • Blood transfusions:

    • Your child may need blood transfusions to treat and help prevent anemia. One or more intravenous (IV) tubes are placed in your child's blood vessels to give blood transfusions. During a transfusion, blood that has been donated by another person is given to your child. Whole blood, or only red blood cells may be given. Blood transfusions may help prevent sickle cell crisis, a stroke, and acute chest syndrome. Blood may be given in an emergency if your child is having severe pain. Blood transfusions can help your child grow and develop, and can make him feel better by giving him more energy. A blood transfusion may also be given before your child is going to have surgery.

    • Caregivers may suggest an exchange transfusion. This is when some of your child's blood is removed, and he is given donated blood to replace it. You may be worried that your child will get AIDS, hepatitis, or West Nile Virus from blood transfusions. The risk of this happening is rare. Blood banks test all donated blood for AIDS, hepatitis, and West Nile Virus.

  • Intravenous fluids: Liquid may be given through an IV tube. Dehydration (not having enough body fluid) is a common problem with sickle cell disease. IV liquids may help prevent a sickle cell crisis, and provide the liquid that your child needs.

  • Oxygen: Your child may need oxygen to help him breathe more easily. It can be given through a mask that is placed over his mouth and nose or through a nasal cannula. A nasal cannula is a pair of short, thin tubes that are placed in your child's nostrils.

  • Therapy: Your child may need to have physical therapy if he has bone problems that make it hard for him to walk. If he has had a stroke, he may need speech therapy to help him talk. He may also need occupational therapy to help him do daily activities.

  • Surgery: In some cases, a child may need to have part or all of his spleen removed. If stones have grown in your child's gallbladder, he may need to have them removed. If your child is a boy who gets priapism, he may need to have a procedure done to treat it. Problems with your child's bones may need to be treated with surgery. Ask your child's caregiver for more information if your child needs surgery.

What can I do to decrease my child's pain, and help him cope with sickle cell disease?

  • Help your child cope with pain.

    • Putting a heating pad on painful areas may help decrease your child's pain. Do not use ice or cold packs to treat pain. Gently massaging (rubbing) painful areas may also decrease pain. Gentle activity, such as walking, can help your child cope with pain. Taking a warm bath can help decrease pain for some children. Some children will feel less pain if they are not scared and anxious. Help your child learn ways to relax. This can include lying down and slowly tensing and relaxing his muscles, starting at the top of his body and moving downward.

    • If your male child has times of priapism, offer and encourage him to drink more liquids. He may try going to the bathroom, or taking a bath or shower. Exercise, such as walking, may also help make priapism go away. Give him pain medicine as your caregiver has told you to.

  • Stay with your child when he is in pain, and when he needs to see a caregiver or go to the hospital. The pain that your child feels may be less if you stay with him. When you are with your child, help him to think about things other than his pain. Younger children can color pictures and do crafts to help them forget about the pain for a while.

  • Know the early signs of infection and other emergencies. Learn the signs of infection. Do not wait to take your child to his caregiver if you see these signs. Ask caregivers when you should take your child to the hospital if pain treatment at home has not worked. Ask caregivers to teach you how to feel your child's abdomen. You can do this to check if his spleen is larger than it should be. By finding this problem, you may be able to quickly get help for your child. Do not keep reptiles and pets that are more likely to carry germs in your home. Germs from these animals can cause diseases, and make your child very ill.

  • Make sure your child gets the vaccinations that he needs. Vaccinations are shots that are given by caregivers to help prevent some infections and diseases. It is very important that children with sickle cell disease get the vaccinations that they need. Ask your child's caregiver what vaccinations your child needs.

  • Talk to 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.

  • Take your child to see a counselor.

    • It can be very hard for a child to cope with sickle cell disease. The pain that he feels can make him change the way he acts. He may have trouble with others at school, and he may need to miss school because of medical problems. This can make him feel very sad, or angry. Older children may not feel good about themselves. Your child may feel frustrated because he has no energy to do what he enjoys. He may feel helpless because he cannot make the pain go away.

    • Caregivers may suggest cognitive-behavior therapy. This therapy teaches your child ways that he can change his behavior. Ask your child's caregiver for more information about cognitive-behavior therapy.

  • Learn about sickle cell disease. Talk to older children about sexual activity and how to be safe from sexually-transmitted diseases. Teach them about the risks of having sickle cell disease if you are pregnant. Talk to older children about smoking. Smoking can make it harder for your child to breathe and lead to medical problems. If you smoke around your child, stop. Breathing in the smoke from someone else (second-hand smoke) is very harmful to a child. Learn about the risks of using street (illegal) drugs, and drinking alcohol (such as beer, wine and other adult drinks). Talk to your child, and have your child's caregiver talk to him about these risks.

What can I do to help prevent my child from having a sickle cell crisis?

  • Prevent dehydration. Give infants and young children healthy liquids to drink. For most children, this includes water, juice and milk. Offer and encourage older children to drink healthy liquids, especially if they are outside in warm weather, or playing sports. Dehydration can happen if your child does not drink enough or loses too much body fluid. This problem is more likely to happen in children who have sickle cell disease. Dehydration can cause a sickle cell crisis to begin.

  • Balance rest and exercise. Children should rest during a sickle cell crisis. Help your child increase his activity as he feels better. Your child should not do sports that have a greater risk of injury, such as football. He should not lift heavy weights for exercise. Talk to your child's caregiver about the best exercise plan for him.

  • Avoid cold places. Help your child stay warm, but not too hot. Make sure he is dressed warm enough in cold weather. Do not let your child move quickly from a warm area to a cold area. Do not let your child dive into or swim in cold water. If your child gets too cold, this can start a sickle cell crisis.

  • Offer a healthy diet. Children with sickle cell disease should get enough folic acid. This vitamin is found in vegetables and fruit. Ask your child's caregiver if you need to change your child's diet. Have caregivers talk to an older child about the best eating plan for him.

What are the risks of my child having sickle cell disease?

  • Your child may often feel pain, and he may get many infections. He may get anemia, and feel weak and tired. Your child may not grow as fast as other children. He may reach puberty (sexual maturity) at a later age than other children. His skin and the white parts of his eyes may turn yellow. Your child may have trouble seeing, and over time he may not be able to see. Your son may have painful erections (priapism), which can lead to sexual problems.

  • With sickle cell disease, your child may get sores (ulcers) on his legs, and he has a risk of having seizures (convulsions). Stones may grow in your child's gallbladder. He may need surgery to remove the stones, or to remove his gallbladder. His pancreas may get infected, and he may need surgery to correct his problem. Your child may have a stroke and not be able to speak clearly or move parts of his body.

  • He may get bone problems such as avascular necrosis, osteomyelitis, or arthritis. These problems cause bone pain, and make walking and other activities hard to do. Your child may be allergic to the medicine he takes to treat sickle cell disease. Your child may have a bad reaction to the blood used in transfusions. He may have too much iron in his body from the blood transfusions, and need medicine to treat this problem. Your child may not be able to do activities that he enjoys. Your child's liver, spleen or other organs may stop working, and he may die.

Where can I get support and more information?

  • Sickle cell disease can be hard for your child and your family to cope with. Pain and medical problems can make your child feel very sad and worried. He may miss school because of these problems, and have problems making and keeping friends. Other children in your family may feel angry or worried. As a parent, you may feel helpless and very sad. Learn about sickle cell disease. Help your family cope with the disease by teaching them about it. Help your child learn how to tell caregivers about his pain. Arrange for your family to meet with caregivers to talk about their feelings.

  • Be sure other people know that your child has sickle cell disease. Make sure your child keeps a wallet card that has his medical information with him all of the time. This will tell people about your child's medical problem in case of an emergency. Ask caregivers where to get this card. Contact the following for more information:
  • American Academy of Pediatrics
    141 Northwest Point Boulevard
    Elk Grove Village , IL 60007-1098
    Phone: 1- 847 - 434-4000
    Web Address: http://www.aap.org

When should I call my child's caregiver?

Call your child's caregiver if:

  • There is blood in your child's urine.

  • Your child has any new sign or symptom, even if it goes away.

  • Your child has diarrhea (loose, watery stools).

  • Your child is constipated (having hard, dry stools less often than usual, that are difficult to pass).

  • Your child tells you that he cannot see well.

When should I seek immediate care for my child?

Seek care immediately or call 911 if:

  • Your child has a cough, more trouble breathing, or chest pain.

  • Your child has a fever (high body temperature).

  • Your child has a new headache, or a headache that is worse or different from those he has had in the past.

  • Your child has a new rash, itchy skin, or feels sick after starting to use a new medicine.

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

  • Your child is more sleepy, and his skin looks lighter or whiter than usual, or he suddenly turns very pale, and his belly is swollen.

  • Your child is not acting like himself, he has a seizure, or he loses consciousness (passes out).

  • Your child's pain does not decrease or go away after trying to treat his pain at home.

  • 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, and that he wants to die.

  • Your son's penis stays erect and painful for two 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.

© 2013 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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