Population based surveillance in sickle cell disease: methods, findings and implications from the California registry and surveillance system in hemoglobinopathies project (RuSH).

There are no population-based surveillance systems to determine prevalence, impact or outcomes in sickle cell disease (SCD). Estimates of the SCD population in California range broadly from 4,500 to 7,000, and little is known about their health status, health care utilization or health outcomes. A surveillance strategy was implemented using diverse data sources to develop a multi-dimensional, state-based surveillance system for SCD that includes adults and children and describes utilization, treatment and outcomes. Data from California newborn screening, inpatient and emergency room records, Medi-Cal/Medicaid claims and two SCD special care centers were collected for 2004-2008. A multi-step, iterative linkage process was used to link and de-duplicate these data sources, and case definitions were used to categorize cases. After linking and de-duplicating, there were 1,975 confirmed cases of SCD, 3,159 probable cases as well as 8,024 possible cases. Among individual data sources, newborn screening and data from clinics contributed the greatest number of unique cases to the total. Select analyses of utilization and treatments for the population are described. Using linked existing data sources, an estimate of the statewide count of the SCD population is possible. The approach can be used to create an in-depth health status profile of the affected population by aggregating utilization, treatment, and outcomes data including mortality and morbidity information. This effort sets the stage for development of an on-going, state-based surveillance system.

Paulukonis ST ，Harris WT ，Coates TD ，Neumayr L ，Treadwell M ，Vichinsky E ，Feuchtbaum LB ... -  《-》

Surveillance for Sickle Cell Disease - Sickle Cell Data Collection Program, Two States, 2004-2018.

Snyder AB ，Lakshmanan S ，Hulihan MM ，Paulukonis ST ，Zhou M ，Horiuchi SS ，Abe K ，Pope SN ，Schieve LA ... -  《MMWR SURVEILLANCE SUMMARIES》

A method to identify California's sickle-cell disease population and its linkage to the California Cancer Registry.

Brown M ，Brunson A ，Wun T 《-》

Newborn screening follow-up within the lifespan context: Michigan's experience.

Experience in using various data sources for surveillance systems and studies complements the growing knowledge base regarding requirements for newborn screening follow-up, which include integration with services other than clinical subspecialties. A proposed model for utilizing state resources to develop sickle cell disease surveillance across the lifespan is presented. This surveillance process should help evaluate the burden of sickle cell disease across the lifespan, and it could be used as a model for other hemoglobinopathies as well as other newborn screening disorders. Through the continued assessment and monitoring of prevalence, comorbidities, service utilization, cost, and patient outcomes, the newborn screening follow-up program will be able to inform public health policy.

Grigorescu V ，Kleyn MJ ，Korzeniewski SJ ，Young WI ，Whitten-Shurney W ... -  《-》

Defining Sickle Cell Disease Mortality Using a Population-Based Surveillance System, 2004 through 2008.

Paulukonis ST ，Eckman JR ，Snyder AB ，Hagar W ，Feuchtbaum LB ，Zhou M ，Grant AM ，Hulihan MM ... -  《-》