Health Supervision for Children With Sickle Cell Diseases and Their Families

Abstract

This set of guidelines is designed to assist the pediatrician in caring for the child in whom the diagnosis of a sickle cell disease (SCD) has been confirmed by hemoglobin electrophoresis. The SCDs include SS, SC, SB0 thalassemia (refers to β thalassemia mutations that do not produce any β-globin), SD, SE (the latter being noted after the influx of Southeast Asian immigrants), and others. Because some of these variants (eg, SC or SE) may be associated with minor symptoms, the information contained in these guidelines about complications, morbidity, and mortality may be overstated for these disorders. Therefore, this statement has been developed primarily for the health supervision and treatment of patients with SS disease. Because management issues may be complex, consultation, referral, and/or comanagement with a pediatric hematologist or comprehensive sickle cell program is advisable. Medical treatment, home environment, patient and family support, and education can significantly affect the clinical outcome of children and adolescents with SCD and can facilitate their transition to adulthood. The following outline is designed to help the pediatrician in caring for children with SCDs and their families. In addition to assuring compliance with the schedule, "Recommendations for Preventive Pediatric Health Care" of the American Academy of Pediatrics (AAP), the following areas require ongoing assessment throughout childhood and should be reviewed at each visit or periodically at appropriate ages (Table 1). 1. Establishment of baseline hemoglobin, white cell count, absolute neutrophil count, and reticulocyte count, which should be repeated periodically; 2. Monitoring growth parameters and nutritional status;