Search

Background 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. Procedure 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. Results 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. Conclusions 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.

Historic practice recommends slow transfusion for children with chronic anemia and hemoglobin less than 5.0 g/dl due to the theoretical risk of transfusion-associated circulatory overload (TACO). In our pediatric intensive care unit (PICU), we have been utilizing a more liberal transfusion practice in patients without underlying cardiopulmonary disease, and a faster transfusion rate appears safe in this population. Rate of transfusion must be based on multiple factors including convenience, timeliness of procedures and transport to an appropriate care facility, risk of alloimmunization and wastage of blood, stress for the family, and need for PICU monitoring.

Background Clinical care for children and adults living with sickle cell disease (SCD) is often provided in the emergency department (ED). Population-based surveillance data can be used to describe the ED utilization patterns of this patient population. Procedure A cohort of pediatric and adult California patients with SCD was identified from multiple data sources, and 10 years (2005–2014) of their treat-and-release ED utilization data were analyzed. Results Among a cohort of 4,636 patients with SCD, 4,100 (88%) had one or more treat-and-release ED visits. There were 2.1 mean annual visits per person for the cohort (median 0.7; range 0–185). In a single year (2005), 53% had 0 treat-and-release ED visits, 35% had 1–3 visits, 9% had 4–10 visits, and 3% had 11 or more visits; this highest utilization group accounted for 45% of all patients’ ED visits. ED utilization in this cohort was highest among young adults and also higher among older adults than pediatric patients. Conclusion The majority of identified patients in each of the 10 years did not go to the ED, but nearly all had one or more such visits over the full span of time. This study highlights the power and utility of a multisource longitudinal data collection effort for SCD. Further study of the segment of the population with highest ED utilization may highlight areas where changes in healthcare and health policy could improve and extend the lives of patients with SCD.