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Parents need to know what to look for
Sickle cell disease is genetic, yet often parents do not know that they are carriers of the disease until they have a child who has it, says Robin Pitts, MN, C-FNP, a sickle cell nurse practitioner at Children's Healthcare of Atlanta. Thus, education often begins at ground zero. "We spend a lot of time explaining the probability of offspring having the disease based upon the disease state of the parents," she says.
When two people have the sickle cell trait or gene, they can produce a child with sickle cell disease. Sickle cell disease affects red blood cells, making them hard and pointed rather than soft and round. Sickle cells cause anemia, pain episodes, and other medical problems.
Children with sickle cell are at risk for infection due to an impaired immune system; therefore, parents must be taught when to call their physician. This includes any time the child has a fever above 101° F. Other symptoms include: lethargy, pale, not eating, having nausea/vomiting, experiencing a pain crisis not relieved by oral medications at home, or any change in their routine behavior, says Pitts.
When a child is left at day care or goes to a friend's house, a responsible adult must be told what to look for, says Jackie George, MPH, a counselor at the Sickle Cell Foundation of Georgia in Atlanta. Parents must learn how to help the child live with the disease. For example, because of circulation problems, the child may need to carry a sweater to wear in air-conditioned stores during the summer. "The best thing for parents of a child with sickle cell disease is education, says George.
She advises parents to keep a diary of the child's pain episodes so if they have to go to the emergency department, the information is in writing. The child's medications should be listed in the diary as well, says George.
Parents should also know their child's baseline hemoglobin level. Because hemoglobin levels vary greatly depending on disease state, illness, and the individual child, there are no normal levels, says Pitts. Therefore, parents are instructed to follow their child's result with each clinic visit.
Managing pain is another important part of the education process. "It is vitally important for the children to stay well hydrated because this decreases blood viscosity and helps prevent vaso-occlusive crisis (VOC). VOC occurs when the sickle-shaped cells clump together and are unable to deliver oxygen effectively, thus causing tissue ischemia," explains Pitts.
Parents are taught that when the child complains of pain, they should begin administering pain medication around the clock, alternating Tylenol with Motrin every two hours. At these times, they also know to increase fluid intake as well as use massage and heat on the affected area. If the pain can't be controlled, the child must see a physician.
To help more people learn about sickle cell disease, the Sickle Cell Foundation of Georgia travels to middle and high schools to provide education and conduct screenings. Its health educator speaks to the children at assemblies or in classrooms to explain that sickle cell disease is a hereditary disorder; and it is important that when they decide to have children, that both they and their mate be tested. "We want them to be aware and educated so they can make an informed decision," says Beverly Sinclair, health educator for the Sickle Cell Foundation. To be tested, the children must have a consent form from their parents.
When a child with sickle cell disease is born, Pitts spends a lot of time explaining the probability of other children having the disease based on their disease state. If the parents do not know their disease state, a hemoglobin electrophoresis is performed to check it. This lab test measures and quantifies the different types of hemoglobin present in the blood by measuring the electrical charge of the hemoglobin molecule, says Pitts. "It is the definitive test to use to diagnose sickle cell disease," she says.
For more information on educating parents about sickle cell disease, contact:
• Jackie George, MPH, Counselor, and Beverly Sinclair, Health Educator, Sickle Cell Foundation of Georgia, 2391 Benjamin Mays Dr. S.W., Atlanta, GA. Telephone: (404) 755-1641. Web: www.sicklecellatlaga.org.
• Robin Pitts, MN, C-FNP, Sickle Cell Nurse Practitioner, Children's Healthcare of Atlanta, 1001 Johnson Ferry Road N.E., Atlanta, GA 30342. Telephone: (404) 250-2556. E-mail: email@example.com.