title: Defining Sickle Cell Disease Mortality Using a Population-Based Surveillance System, 2004 through 2008.
abstract: OBJECTIVE
  Population-based surveillance data from California and Georgia for years 2004 through 2008 were linked to state death record files to determine the all-cause death rate among 12,143 patients identified with sickle cell disease (SCD).
  
  
  METHODS
  All-cause death rates, by age, among these SCD patients were compared with all-cause death rates among both African Americans and the total population in the two states. All-cause death rates were also compared with death rates for SCD derived from publicly available death records: the compressed mortality files and multiple cause of death files.
  
  
  RESULTS
  Of 12,143 patients identified with SCD, 615 patients died. The all-cause mortality rate for the SCD population was lower than the all-cause mortality rate among African Americans and similar to the total population all-cause mortality rates from birth through age 4 years, but the rate was higher among those with SCD than both the African American and total population rates from ages 5 through 74 years. The count of deceased patients identified by using population-based surveillance data (n=615) was more than twice as high as the count identified in compressed mortality files using SCD as the underlying cause of death alone (n=297).
  
  
  CONCLUSIONS
  Accurate assessment of all-cause mortality and age at death requires long-term surveillance via population-based registries of patients with accurately diagnosed SCD.
authors.affiliation: Public Health Institute, Oakland, CA.
authors.forename: Susan T
authors.initials: ST
authors.lastname: Paulukonis
authors.affiliation: Emory University, Atlanta, GA.
authors.forename: James R
authors.initials: JR
authors.lastname: Eckman
authors.affiliation: Georgia State University, Department of Public Management and Policy, Atlanta, GA; Georgia State University, Georgia Health Policy Center, Atlanta, GA.
authors.forename: Angela B
authors.initials: AB
authors.lastname: Snyder
authors.affiliation: University of California San Francisco Benioff Children's Hospital Oakland, Oakland, CA.
authors.forename: Ward
authors.initials: W
authors.lastname: Hagar
authors.affiliation: California Department of Public Health, Genetic Disease Screening Program, Richmond, CA.
authors.forename: Lisa B
authors.initials: LB
authors.lastname: Feuchtbaum
authors.affiliation: Georgia State University, Georgia Health Policy Center, Atlanta, GA.
authors.forename: Mei
authors.initials: M
authors.lastname: Zhou
authors.affiliation: Centers for Disease Control and Prevention, Division of Blood Disorders, Atlanta, GA.
authors.forename: Althea M
authors.initials: AM
authors.lastname: Grant
authors.affiliation: Centers for Disease Control and Prevention, Division of Blood Disorders, Atlanta, GA.
authors.forename: Mary M
authors.initials: MM
authors.lastname: Hulihan
mesh_terms: D000293:Adolescent; D000328:Adult; D001741:Black or African American / Q000706:statistics & numerical data*; D017677:Age Distribution; D000368:Aged; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000208:ethnology / Q000401:mortality*; D002140:California / Q000453:epidemiology; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D003644:Death Certificates; D005260:Female; D005845:Georgia / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008498:Medical Record Linkage; D008875:Middle Aged; D015997:Neonatal Screening; D011159:Population Surveillance / Q000379:methods*; D017678:Sex Distribution; D055815:Young Adult