What is sickle cell disease?
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Sickle cell is a condition in which red blood cells change shape. Instead of being round and smooth, they form a “c” shape like a crescent moon. They can get stuck in blood vessels and block blood flow, which can cause pain or swelling and keep the body from fighting infection.
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The abnormally shaped red blood cells do not live as long as regular cells, so children with sickle cell disease have a low blood count and must make new red blood cells more quickly.
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Children are born with this condition and have it for life. Some children are more severely affected; some have a milder form.
How common is it?
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There are about 72,000 people in the United States with sickle cell disease. It is most common in people of African or Mediterranean descent.
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People with sickle cell
trait do not have the disease and are generally healthy. Children with sickle cell trait usually have no special requirements. About 2 million Americans have sickle cell trait.
What are some characteristics of children with sickle cell disease?
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Children with sickle cell disease may have a yellow tint to their eyes because of a by-product of the breakdown of red blood cells. They may be small or slender for their age.
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Children with sickle cell disease may have increased absences because of complications and may need to be hospitalized for treatment. Some complications include
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Pain
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Pain can happen in any part of the body but often occurs in the hands, feet, or joints.
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Chest pain can be especially serious. Signs of acute chest syndrome include cough, difficulty breathing, and fever.
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Fever
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Children with sickle cell disease can have a hard time fighting infection. The abnormal cells can interfere with the body’s ability to clear out and destroy bacteria.
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Fevers must be evaluated urgently by the child’s health care professional. Treatment includes laboratory blood studies and antibiotic administration to avoid complications.
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Pneumonia can be very serious in children with sickle cell disease.
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Splenic sequestration
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Splenic sequestration is an emergency.
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The spleen is an organ in the upper left section of our abdomen next to the stomach. The spleen acts to strain the blood and remove damaged cells and infection. Sickled cells can clog up the spleen and keep it from working properly. Sometimes sickled cells get especially clogged in the spleen and cause the blood to back up. The spleen can get very big if this happens and can sometimes break open, which is a life-threatening emergency.
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Aplastic crisis
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Abnormal blood cells have a shorter lifespan, so the body needs to make new blood cells very quickly. If something such as a viral infection prevents the body from keeping up with making new blood cells, cell count can drop and the child can get a dangerously low blood count very quickly. If this happens, the child can appear very pale and tired.
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Strokes
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If sickled cells block the blood flow to the brain, a stroke can occur.
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Signs include headache, weakness of a body part, seizure, or speech problems.
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Strokes require emergency evaluation and treatment.
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Skin ulcers
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These need to be treated promptly if they develop.
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Priapism
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Boys with sickle cell disease may get painful penile erections related to poor blood flow that require emergency treatment.
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Some children with more severe disease require regular blood transfusions.
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There are other blood diseases that may share some characteristics of sickle cell disease. These are called hemoglobinopathies, in which red blood cell proteins are abnormal.
Who is the treatment team?
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Children with sickle cell disease may have their primary medical care with the primary care provider in their medical home or with a specialty clinic. Check with parents/ guardians about who the first point of contact should be.
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Hematologists are the specialists who care for children with blood diseases.
What adaptations may be needed?
Medications
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Children with sickle cell may take penicillin from 2 months until 5 years of age to help prevent infection. Erythromycin may be substituted in children who are allergic to penicillin. Pain crisis is treated with medications like acetaminophen (eg, Tylenol), codeine, and ibuprofen (eg, Motrin, Advil). Extra amounts of folic acid may be required because of the extra red blood cells that are needed.
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Note on vaccinations: Children with sickle cell disease may need special vaccines such as pneumococcal or early meningococcal as well as routine immunizations. All children should receive annual flu vaccinations, but this is especially important for children with sickle cell disease.
Dietary considerations
Children with sickle cell disease should have at least 8 cups of water or fluid per day.
Physical environment
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Hydration helps to prevent sickling, so allowing the child to have a water bottle is a good idea.
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Children with sickle cell disease may need increased bathroom breaks.
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Most children with sickle cell disease have normal activity, but allow them to rest if they tire easily with anemia.
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Avoid extreme temperatures, hot and cold.
Transportation Considerations
Special consideration should be given to transport to and from child care or school because vehicles can be very warm or cold, either of which can increase sickling of red cells.
What should be considered an emergency?
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Call parents/guardians immediately for
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Fever
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Pain that does not improve with medication and rest
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Cough or mild chest pain
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Abdominal pain or swelling
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Paleness or increased tiredness
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Painful erection
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Call emergency medical services/911 if
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Difficulty breathing.
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Seizure or loss of consciousness.
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Headache or dizziness.
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Change in vision.
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Numbness or inability to move a body part.
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Severe pain.
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The spleen gets enlarged (sequestration).
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Prolonged erection.
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What types of training or policies are advised?
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Recognizing impending crisis and signs and symptoms of complications
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Responding to emergencies
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Medication administration
What are some resources?
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The Sickle Cell Information Center,www.scinfo.org/teacher.htm
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National Heart, Lung and Blood Institute,www.nhlbi.nih.gov
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Regional sickle cell centers (contact local resources or children’s hospitals for more information)
Source: Managing Chronic Health Needs in Child Care and Schools: A Quick Reference Guide.
Products are mentioned for informational purposes only. Inclusion in this publication does not imply endorsement by the American Academy of Pediatrics.
Listing of resources does not imply an endorsement by the American Academy of Pediatrics (AAP). The AAP is not responsible for the content of external resources. Information was current at the time of publication.
The information contained in this publication should not be used as a substitute for the medical care and advice of your pediatrician. There may be variations in treatment that your pediatrician may recommend based on individual facts and circumstances.