Appropriate reporting 6. Reporting of preliminary screening results is discussed in Chapters 2 and 3. Initiation of comprehensive care including penicillin prophylaxis and immunization 7. Children with sickle cell disease identified on screening examination should be referred to a comprehensive care program without delay. Because confirmatory testing may not be complete for several weeks or months, it is important not to delay the basic elements of care, as described in nodes 9-12. The panel concluded that prophylaxis against pneumococcal infection is Confirmatory testing positive? 8. All positive screening tests for sickle cell disease require a second blood sample to confirm the initial hemoglobin phenotype. A definitive diagnosis should be established by the infant's physician. Counseling and education of parents 9. Parents of infants with sickle cell disease must be counseled concerning the implications of their child's condition. Specifically, parents should be informed about the need for close vigilance with respect to the development of signs and symptoms that could indicate a serious medical problem. Any of the following warrant immediate medical consultation: (1) fever, (2) symptoms of respiratory tract infection, (3) increasing pallor, (4) increasing spleen size or abdominal distension, (5) weakness or numbness of an extremity, and (6) painful swelling of hands and feet. Information also should be provided concerning diet and adequate hydration. Parents should be trained in home management skills and should receive genetic counseling (Chapters 3 and 4). Health maintenance and compliance 10. The schedule of health maintenance visits need not differ from that used for a well child. Strenuous efforts must be made by the health care provider to ensure compliance with penicillin prophylaxis (Chapter 3). Parent presents child for emergency care? 11. Parents should be encouraged to seek immediate medical attention whenever the warning signs described in node 12 are noted. Patient febrile? 12. Fever over 101°F (38.5°C) requires immediate medical evaluation. The parent also should be told that changes in behavior (unusual somnolence or irritability) or alimentation (refusing feeding, vomiting, or diarrhea) are other possible early signs of significant illness. Consider sepsis 13. It is critical that all health care providers who care for patients with sickle cell disease be knowledgeable about the significance of fever in these children. The importance of evaluating febrile sickle cell children promptly and administering broad-spectrum antibiotics are emphasized. Management of febrile children with sickle cell disease is discussed in Chapter 3. Pallor, lethargy, and abdominal symptoms? 14-15. Acute anemia emergencies are common in children with sickle cell disease, particularly acute splenic sequestration and aplastic crises. Diagnosis and management of these conditions are discussed in Chapter 3. Limping, paresis, or other symptoms compatible with stroke? 16-17. Although relatively infrequent, both parents and providers must be alert for the possibility of a stroke. Any loss of consciousness or weakness of an extremity should be evaluated promptly. Painful swelling of hands and feet? 18-19. The most frequent early complication of sickle cell disease is the hand-andfoot syndrome, or dactylitis (Chapter 3). Attachment B. Neonatal Hemoglobinopathy Screening Policies and Primary Laboratory Methods in 53 U.S. Jurisdictions as of Mid-1992 Jurisdiction Alabama Universal Mandatory CAE California Colorado Universal Mandatory IEF Universal Mandatory HPLC, IEF Screens in three regions with some regional private contracting. Screens for Arizona and Wyoming Sends hemoglobinopathy tests to Sends all tests to Oregon. Test performed at Howard University (along with tests from the Virgin Islands). IEF |