• Join Today!

Become a member and connect with:

  • An Active Online Community
  • Articles and Advice on SCD
  • Help Understanding Clinical Trials
abstracts & posters

Sickle Cell Disease Mortality in California and Georgia 2004-2008

key information

source: Blood

year: 2014

authors: Susan Paulukonis, Todd Griffin, Mei Zhou, James R. Eckman, Robert Hagar, Angela Bauer Snyder, Lisa Feuchtbaum, Althea M Grant, Mary Hulihan


On-going public health surveillance efforts in sickle cell disease (SCD) are critical for understanding the course and outcomes of this disease over time. Once nearly universally fatal by adolescence, many patients are living well into adulthood and sometimes into retirement years. Previous SCD mortality estimates have relied on data from death certificates alone or from deaths of patients receiving care in high volume hematology clinics, resulting in gaps in reporting and potentially biased conclusions.

The Registry and Surveillance System for Hemoglobinopathies (RuSH) project collected and linked population-based surveillance data on SCD in California and Georgia from a variety of sources for years 2004-2008. These data sources included administrative records, newborn screening reports and health insurance claims as well as case reports of adult and pediatric patients receiving care in the following large specialty treatment centers: Georgia Comprehensive Sickle Cell Center, Georgia Regents University, Georgia Comprehensive Sickle Cell Center at Grady Health Systems and Children’s Healthcare of Atlanta in Georgia, and Children’s Hospital Los Angeles and UCSF Benioff Children’s Hospital Oakland in California. Cases identified from these combined data sources were linked to death certificates in CA and GA for the same years.

Among 12,143 identified SCD cases, 640 were linked to death certificates. Combined SCD mortality rates by age group at time of death are compared to combined mortality rates for all African Americans living in CA and GA. (Figure 1). SCD death rates among children up to age 14 and among adults 65 and older were very similar to those of the overall African American population. In contrast, death rates from young adulthood to midlife were substantially higher in the SCD population.

organization: Public Health Institute, Richmond; Georgia Dept. of Public Health; Georgia Regents University; Emory University Medical Center, Atlanta; Children's Hospital & Research Center Oakland; California Department of Public Health; National Center on Birth Defects and Disabilities, Division of Blood Disorders, Atlanta; Centers for Disease Control and Prevention, Atlanta

read more