Sickle Cell Disease In Children


  • 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. This blocks the blood vessel, and does not let enough blood flow to where it needs to go in your child's body. A child gets sickle cell disease if both of his birth parents have the gene for sickle cell disease.

  • Your child may have pain, trouble breathing, and other problems from sickle cell disease. Blood tests are used to learn if your child has the disease. Computed tomography (CT) scans and other tests are used to check if the disease is causing other medical problems. Medicine, blood transfusions, fluids, and surgery may be used to treat sickle cell disease. Your child may need help coping with pain and other symptoms. 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 may give your child enough energy to play sports and be active.


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.


  • 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 feel very sad and worried. He may miss school because of health problems, and not be able to do activities that he enjoys. Your child's liver, spleen or other organs may stop working, and he may die.


Informed consent

is a legal document that explains the tests, treatments, or procedures that your child may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your child's medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done to your child. Make sure all of your questions are answered.

Intake and output:

Your child's caregiver may need to know how much liquid your child is drinking. They may also need to measure his urine. Your child may need to urinate into a container. If your child wears diapers, save them so a caregiver can weigh them. Do not throw away diapers or flush urine down the toilet before asking your child's caregiver. Caregivers may also want to know what your child eats, and if he had a bowel movement (BM). Caregivers may also weigh your child every day.

Pulse oximeter:

A pulse oximeter is a device that measures the amount of oxygen in your child's blood. A cord with a clip or sticky strip is placed on your child's foot, toe, hand, finger, or earlobe. The other end of the cord is hooked to a machine. Never turn the pulse oximeter or alarm off. An alarm will sound if your child's oxygen level is low or cannot be read.


Your child may wear a mask, or have tubes that go into his nose. He is given oxygen through the mask or tubes. This extra oxygen will help his body get the oxygen that it needs.

Vital signs:

This includes taking your child's temperature, blood pressure, pulse (counting his heartbeat), and respirations (counting his breaths). To take your child's blood pressure, a cuff is put on his arm and tightened. The cuff is attached to a machine which gives your child's blood pressure reading. Caregivers may listen to your child's heart and lungs by using a stethoscope.


Caregivers may use one of more types of medicine to treat your child. Ask your caregiver to tell you about the medicines that are being used to treat your child. The following are some of the medicines that may be used to treat sickle cell disease:

  • Pain medicine: Pain medicine may be given to help your child cope with his pain. Certain pain medicines will also decrease a fever.

    • Patient controlled analgesia: Your child may be given pain medicine with a special pump. This is called patient controlled analgesia (PCA). Caregivers set up the PCA pump to give your child small amounts of pain medicine. The PCA pump has a cord coming from it, with a button on the end. When your child feels pain and pushes the button, he will get pain medicine. The pump is set so that your child cannot give himself too much medicine. Ask caregivers for information about using a PCA pump.

  • 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 be given a vitamin called folic acid. Folic acid can help prevent blood vessel problems that can come with sickle cell disease.


Your child may need one or more of the following tests. Ask caregivers for more information about these and other tests that you child needs.

  • Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.

  • CT scan: This test is also called a CAT scan. An x-ray and computer are used to take pictures of your child's body. Your child may be given dye, also called contrast, before the test. Tell the caregiver if your child is allergic to dye, iodine, or seafood.

  • MRI: An MRI uses a powerful magnet and radio waves to take pictures of the inside of your child's body. Caregivers may use the MRI to look at your child's brain, muscles, joints, bones, or blood vessels. Your child will need to lie still during his test. Never enter the MRI room with any metal objects. This can cause serious injury.

  • Ultrasound of the head: This test uses sound waves to see the blood vessels in your child's head on a TV-like screen. This test may be used to see if your child is at risk of having a stroke.


  • Intravenous fluids: Your child may need to get fluids through an intravenous (IV) tube. This is a small tube that is placed into a blood vessel. Extra liquids may help prevent a sickle cell crisis, and give your child the liquid that he needs.

  • Blood transfusions:

    • Your child may need blood transfusions to treat and help prevent anemia. Blood is given through one or more IV tubes. 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. They 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.

  • Incentive spirometer: Your child may be taught to use an incentive spirometer to help him take deep breaths. He should use this device as often as caregivers say, when he is awake.

  • Therapy: Physical therapists are caregivers who may work with your child if he has bone pain, trouble walking, or if he has had a stroke. Hydrotherapy is a treatment that uses water, such as a whirlpool bath. Heating pads may be placed on painful areas of your child's body. Caregivers may help your child walk around and be active while he is in the hospital.

  • Counseling:

    • 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 feel very sad or angry. Older children may not feel good about themselves. Your child may have 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 to change his behavior. Ask your child's caregiver for more information about cognitive-behavior therapy.

  • 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 male child 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 surgery is needed.

© 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.

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.

Learn more about Sickle Cell Disease In Children (Inpatient Care)