Participation in school and peer groups are important for development of self-esteem during school years. Striving for independence can pose special challenges.
Sickle Cell Disease Specifics
During the elementary school years, parents should:
- Get to know your child’s sickle cell team: doctor, nurse, social worker, psychologist
- Know what to expect for routine visits (every few months) and sick visits (emergency room and hospital). The stress of being in the emergency room or hospital can cause children of this age to act out or be very fearful. Talk to the Sickle Cell Program staff if your child’s behavior continues to be different after discharge.
- Keep your child’s identification card updated and ask the Sickle Cell Program staff about current educational materials.
- Know important phone numbers and who to call for concerns.
- Know that dehydration, low oxygen levels, (as with asthma), stress, exposure to cold temperatures and water, such as during swimming and/or playing in the snow, can all increase sickling and cause pain – work to avoid triggers!
- Make sure to have:
- Thermometer – have one on hand anywhere your child spends time
- Pedialyte – used for extra hydration in times of vomiting or diarrhea
- Humidifier – useful if your child has nasal congestion
Sickle Cell Medical Concerns
- Fever – every episode of fever of 101 degrees Fahrenheit (38.4 Celsius) needs medical evaluation immediately. Do not wait. With temperatures of 99–100 degrees Fahrenheit, call for advice. Do not give Tylenol, Motrin or other medicines to reduce a fever – call for advice.
- Stroke – sickling to the brain can cause lack of blood flow, which can result in obvious, as well as more subtle strokes. Headaches, lack of attention, weakness of extremities or facial features, and visual changes can all be signs. Prompt evaluation is needed. Transcranial Doppler Ultrasound (TCD) is recommended for screening in patients with certain types of sickle cell disease.
- Priapism is a prolonged, painful erection caused from sickling. Immediate medical intervention is necessary for episodes lasting more than 30-45 minutes to prevent long-term damage.
- Pain can occur anywhere in the body, arms, legs, back, chest, hands or feet (dactylitis) as a result of reduced blood flow from sickling. May be treatable at home if not accompanied by fever. Call for advice. Work on coping skills along with medical treatment.
- Bedwetting can be a common problem in sickle cell disease. Many treatment options exist.
- Growth/puberty can be delayed by sickle cell disease. Eventually children catch up, but may be several years behind their peers.
Call to be seen right away if:
- Fever – 101F (38.4C) or higher
- Color – very pale
- Behavior – isn't acting like usual self; won't wake up
- Stomach – vomits/has diarrhea more than once
- Color – jaundice (eyes or skin look yellow)
- Hands/Feet – swelling, tenderness, pain
- Nose – runny or stuffy nose
- Chest – cough without fever or chest pain
Medications/Doses
- Penicillin – extremely important in preventing serious infection in children with sickle cell disease. For children age 3 and older, a dose of 250 mg (liquid or tablets) is given twice daily. Liquid needs to be kept in the refrigerator and must be refilled at the pharmacy about every 10 days. Tablets can be broken or crushed and taken with food. DO NOT mix liquid penicillin with other drinks; it will stick to the inside of the cup and not get into the child. Talk with the sickle cell nurse if your child doesn't take penicillin well.
- Folic Acid – B vitamin that helps your body to make healthy red blood cells. Many packaged and prepared foods, such as cereal and bread, have added extra folic acid, so extra folic acid in tablet form may not be necessary, depending on your child’s diet and type of sickle cell disease.
- Tylenol/Acetaminophen – may be used for treatment of pain. Do not give for fever unless directed by a sickle cell doctor or nurse.
- Motrin/Advil/Ibuprofen – may be used for treatment of pain. Do not give for fever unless directed by a sickle cell doctor or nurse.
- Hydroxyurea (Hydrea) – helps to reduce painful episodes and chest syndromes when taken regularly for a few months. It is a longer-term treatment for sickling episodes, and is available in either capsules or liquid.
Immunizations
- Children should receive all of the routine pre-school booster immunizations from their primary pediatrician.
- Prevnar and Pneumovax – both help to fight infections from bacteria that can cause pneumonia, blood infections and meningitis (infection in the spinal column). Prevnar is given to all children. Pneumovax is given at age two and five for children with sickle cell disease.
- Children should receive a flu shot every year after six months of age.
Screenings/Special Tests
- TCD (Transcranial Doppler) – ultrasound of blood flow to the brain that screens for increased risk of stroke. Done every year, sometimes more often depending on results.
- Complete blood count (CBC) and Reticulocyte count (“Retic”) – done at all well and sick visits. Each patient will establish a baseline level that is important to know.
- Liver and kidney function tests and a urine test are done every year after age one.
- Hemoglobin electrophoresis (“sickle cell test”) – used to tell the difference between certain disease types. We can also help to arrange testing for family members.
- Oxygen saturation (O2 Sat) – measurement of oxygen in tissue. Checked as part of routine vital signs in the clinic, and in the ER/Hospital when sick. Baseline level when feeling well is important to know.
Notify the Sickle Cell Program staff if your child:
- Has any special procedure or surgery planned and will need to be put to sleep (“general anesthesia”). Special lab tests and other arrangements will need to be made 1–2 weeks before the procedure.
- Will be traveling. We will give you a travel letter to use in case medical care is needed.
Other
- For information about developmental and social milestones at this age, visit Children’s Child Health Library.
- Children’s Sickle Cell Program staff can assist parents with communicating with their child’s school and their own employers about your child’s unique issues.
- Sign your school-age child up for Camp Escape, Children’s annual summer camp for kids with sickle cell disease established in 1999.
- For helpful information about managing your child’s behavior, see Helpful Links.
- For information about helping your child’s success in school, see Helpful Links.
- For more news and information about sickle cell disease, see Helpful Links.
- For information about Special Needs Pen Pals, visit: www.childrenswishes.org.
The contents of Children’s Hospital of Pittsburgh of UPMC’s Web site are for informational and educational purposes only. Children’s does not give specific medical advice on its Web site. The content of this Web site is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because information contained on this Web site. The links provided on this web page are for reference purposes only. Children’s does not manage or expressly endorse the contents of third party websites.
