Identification and Management of Sickle Cell Trait by Young Physicians

Abstract

Sickle cell disease (SCD) is found in many ethnic groups, with the highest prevalence of heterozygote ' carriers (sickle cell trait [SCT]) in African Americans. SCT is associated with an increased risk of fatal exertional heat illness, renal papillary necrosis, and splenic infarction. Since 2006, all infants born in the United States are required to be screened for hemoglobinopathies as part of newborn screening (NBS). In 2010, as part of a legal settlement, the National Collegiate Athletic Association (NCAA) implemented SCT screening in division I athletes. Members of the American Academy of Pediatrics (AAP) Section on Young Physicians were sent up to 4 e-mail survey requests to evaluate SCT education during residency, current NBS follow-up practice, and awareness of the NCAA policy. Descriptive statistics and chi2 analyses were performed. Of 871 eligible participants, 355 (41%) completed the survey. Respondents were 70% female, 71% white, and 79% general pediatricians. Most had experience with SCD during residency and had been taught about the medical and reproductive implications of SCT. Virtually all pediatricians report SCT to families when identified during NBS, but only 59% order confirmatory testing (e.g., hemoglobin electrophoresis) to verify status. While 93% counsel about reproductive implications of SCT, only 71% counsel about other medical implications. Only 27% were aware of the NCAA policy. Despite formal SCT education, a significant number of pediatricians do not verify NBS results or counsel about the medical implications of SCT. More comprehensive AAP guidelines about SCT are needed and must be incorporated into residency education.