title: Newborn sickle cell screening in a region of Western New York State.
abstract: OBJECTIVE
  To assess local trends in the incidence of sickle cell disease (SCD) and hemoglobin (Hb) S trait. Hemoglobinopathy clinic follow-up and cohort mortality rates were also evaluated.
  
  
  METHODS
  A longstanding newborn hemoglobinopathy screening program was reviewed. Incidence rates were computed with information from a confidential database, specialty clinic/hospital data, and local birth statistics.
  
  
  RESULTS
  Over 27 years, the incidence of Hb SS in live black births was 0.163% or 1 in 615. Over 18 years, the incidence of Hb AS was 8.5% or 1 in 11.8. No significant differences in the incidence of Hb SS, Hb AS, and the S allele were found over time. Specialty clinic follow-up improved. Death before age 18 years was documented for 6 SCD cases (2.8%; mortality rate of 0.23 per 100 patient years).
  
  
  CONCLUSIONS
  Local screening activities may have had an impact on participation in specialized SCD care and the disease-associated mortality rate. The incidence of Hb SS has remained unchanged over 27 years, and that of Hb S trait and the S allele has been unaffected in the last 18 years. Trait notification goals and approaches should be reevaluated.
authors.affiliation: Department of Pediatrics, University of Rochester, Rochester, NY, USA. norma_lerner@urmc.rochester.edu
authors.forename: Norma B
authors.initials: NB
authors.lastname: Lerner
authors.forename: Bridget L
authors.initials: BL
authors.lastname: Platania
authors.forename: Sandra
authors.initials: S
authors.lastname: LaBella
mesh_terms: D001741:Black or African American; D000755:Anemia, Sickle Cell / Q000175:diagnosis* / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D015997:Neonatal Screening; D009518:New York / Q000453:epidemiology; D012805:Sickle Cell Trait / Q000453:epidemiology