Emergency department utilization by Californians with sickle cell disease, 2005-2014.

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.

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

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.

The newborn screening educational gap: what prenatal care providers do compared with what is expected.

OBJECTIVE: The purpose of this evaluation was to examine prenatal care providers' knowledge of, attitudes towards, and barriers for providing information about newborn screening and tandem mass spectrometry (MS/MS) screening. STUDY DESIGN: We mailed a 12-question survey to 6197 prenatal care providers in California asking about their experiences with newborn and prenatal screening services. RESULTS: Although 4/5 of respondents believe newborn screening is very important for their patients, only 1/3 discuss it with all their patients. Over half believe either pediatricians (38%) or hospital staff (36%) will discuss newborn screening. Only 61% of providers give their patients the newborn screening educational booklet, even though California law requires that the booklet be provided to all pregnant women. CONCLUSION: Pregnant women and state professionals rely on prenatal care providers to educate pregnant women about newborn screening; however, many providers do not appear to view it as part of their responsibility. Therefore, the state needs to improve communication with both providers and the public about newborn screening.

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

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). CONCLUSION: Accurate assessment of all-cause mortality and age at death requires long-term surveillance via population-based registries of patients with accurately diagnosed SCD.