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Sickle Cell Disease In Children

WHAT YOU NEED TO KNOW:

Sickle cell disease (SCD) causes your child's red blood cell (RBC)s to be sickle-(crescent) shaped. The sickle shape is caused by abnormal hemoglobin within the RBC. Hemoglobin carries oxygen to all tissues in your child's body. Sickle-shaped RBCs can get stuck inside blood vessels. This can stop or slow blood flow, and prevent oxygen from getting to tissues. When this happens, it is called a sickle cell crisis. SCD also causes RBCs break apart and die faster than healthy RBCs. This causes low red blood cell levels (anemia).

WHILE YOU ARE HERE:

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.

Emotional support:

Stay with your child for comfort and support as often as possible while he is in the hospital. Ask another family member or someone close to the family to stay with your child when you cannot be there. Bring items from home that will comfort your child, such as a favorite blanket or toy.

Intake and output:

Your child's healthcare providers may need to measure how much liquid your child is drinking. They may also need to measure his or her urine. Your child may need to urinate into a container. If your child wears diapers, save them so a healthcare provider can weigh them. Do not throw away diapers or flush your child's urine down the toilet before asking your child's healthcare provider.

Oxygen:

Your child may need extra oxygen if his or her blood oxygen level is lower than it should be. He or she may get oxygen through a mask placed over his or her nose and mouth or through small tubes placed in the nostrils. Ask before you take off the mask or oxygen tubing.

Medicines:

  • Pain medicine may be given. Do not wait until your child's pain is severe before you ask for more medicine.
  • NSAIDs help decrease pain and fever.
  • Antibiotics treat or prevent a bacterial infection.
  • Hydroxyurea helps your child's body make red blood cells that are less likely to sickle. This may help decrease his or her pain.

Tests:

  • Blood tests check the shape and number of your child's RBCs. They also show liver and kidney function and give information about your child's overall health.
  • Pulmonary function tests show how your child's lungs are working.
  • An x-ray, ultrasound, or MRI may show if SCD has affected organs, such as your child's lungs, liver, or kidney. Your child may be given contrast liquid to help the organs show up better in the pictures. Tell the healthcare provider if your child has ever had an allergic reaction to contrast liquid. Do not let your child enter the MRI room with anything metal. Metal can cause serious injury. Tell the healthcare provider if your child has any metal in or on his or her body.

Treatment:

  • IV fluids help treat or prevent dehydration.
  • An incentive spirometer is a device to help your child take deep breaths. Put the plastic piece into your child's mouth. Tell him or her to slowly take a breath a deep breath. Tell your child to hold his or her breath as long as he or she can. Then tell your child to let out the breath. Have your child use the incentive spirometer 10 times every hour when he or she is awake.
  • A blood transfusion increases the number of healthy RBCs in your child's blood. Your child may need a blood transfusion more than once.
  • Surgery may be done to remove your child's spleen or gallbladder. Surgery may be needed if RBCs often get stuck in your child's spleen or he or she has gallstones.

RISKS:

Sickle cell disease (SCD) increases your child's risk for infections. Sickle cell crisis may cause severe pain, breathing problems, and damage to organs, such as your child's kidneys or spleen. SCD may increase your child's risk for a heart attack or stroke. Even with treatment, sickle cell anemia increases your child's risk for organ failure, such liver or kidney failure. These conditions may become life-threatening.

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.

© 2017 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.

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