Medicaid Coverage in Early Childhood for Children With Sickle Cell Disease.

This cohort study examines patterns of Medicaid coverage in the first 3 years of life among children with sickle cell disease across 5 states.

Assessing Patterns of Telehealth Use Among People with Sickle Cell Disease Enrolled in Medicaid During the Start of the COVID-19 Pandemic.

Background: Telehealth can be defined as using remote technologies to provide health care. It may increase access to care among people with sickle cell disease (SCD). This study examined (1) telehealth use, (2) characteristics of telehealth use, and (3) differences between telehealth users and nonusers among people with SCD during the COVID-19 pandemic. Methods: This was a retrospective analysis of Medicaid claims among four states [California (CA), Georgia (GA), Michigan (MI), Tennessee (TN)] participating in the Sickle Cell Data Collection program. Study participants were individuals ≥1 year old with SCD enrolled in Medicaid September 2019-December 2020. Telehealth encounters during the pandemic were characterized by provider specialty. Health care utilization was compared between those who did (users) and did not (nonusers) use telehealth, stratified by before and during the pandemic. Results: A total of 8,681 individuals with SCD (1,638 CA; 3,612 GA; 1,880 MI; and 1,551 TN) were included. The proportion of individuals with SCD that accessed telehealth during the pandemic varied across states from 29% in TN to 80% in CA. During the pandemic, there was a total of 21,632 telehealth encounters across 3,647 users. In two states (MI and GA), over a third of telehealth encounters were with behavioral health providers. Telehealth users had a higher average number of health care encounters during the pandemic: emergency department (pooled mean = 2.6 for users vs. 1.5 for nonusers), inpatient (1.2 for users vs. 0.6 for nonusers), and outpatient encounters (6.0 for users vs. 3.3 for nonusers). Conclusions: Telehealth was frequently used at the beginning of the COVID-19 pandemic by people with SCD. Future research should focus on the context, facilitators, and barriers of its implementation in this population.

Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability - Sickle Cell Data Collection Program, 11 States, 2016-2020.

Sickle cell disease (SCD) remains a public health priority in the United States because of its association with complex health needs, reduced life expectancy, lifelong disabilities, and high cost of care. A cross-sectional analysis was conducted to calculate the crude and race-specific birth prevalence for SCD using state newborn screening program records during 2016-2020 from 11 Sickle Cell Data Collection program states. The percentage distribution of birth mother residence within Social Vulnerability Index quartiles was derived. Among 3,305 newborns with confirmed SCD (including 57% with homozygous hemoglobin S or sickle ß-null thalassemia across 11 states, 90% of whom were Black or African American [Black], and 4% of whom were Hispanic or Latino), the crude SCD birth prevalence was 4.83 per 10,000 (one in every 2,070) live births and 28.54 per 10,000 (one in every 350) non-Hispanic Black newborns. Approximately two thirds (67%) of mothers of newborns with SCD lived in counties with high or very high levels of social vulnerability; most mothers lived in counties with high or very high levels of vulnerability for racial and ethnic minority status (89%) and housing type and transportation (64%) themes. These findings can guide public health, health care systems, and community program planning and implementation that address social determinants of health for infants with SCD. Implementation of tailored interventions, including increasing access to transportation, improving housing, and advancing equity in high vulnerability areas, could facilitate care and improve health outcomes for children with SCD.

Case Ascertainment of Sickle Cell Disease Using Surveillance or Single Administrative Database Case Definitions.

OBJECTIVE: In the absence of access to surveillance system data, single-source administrative databases are often used to study health care utilization and health outcomes among people with sickle cell disease (SCD). We compared the case definitions from single-source administrative databases with a surveillance case definition to identify people with SCD. MATERIALS AND METHODS: We used data from Sickle Cell Data Collection programs in California and Georgia (2016-2018). The surveillance case definition for SCD developed for the Sickle Cell Data Collection programs uses multiple databases, including newborn screening, discharge databases, state Medicaid programs, vital records, and clinic data. Case definitions for SCD in single-source administrative databases varied by database (Medicaid and discharge) and years of data (1, 2, and 3 years). We calculated the proportion of people meeting the surveillance case definition for SCD that was captured by each single administrative database case definition for SCD, by birth cohort, sex, and Medicaid enrollment. RESULTS: In California, 7117 people met the surveillance case definition of SCD from 2016 through 2018; 48% of this group was captured by the Medicaid case definition and 41% by the discharge case definition. In Georgia, 10 448 people met the surveillance case definition of SCD from 2016 through 2018; 45% of this group was captured by the Medicaid case definition and 51% by the discharge case definition. These proportions differed by years of data, birth cohort, and length of Medicaid enrollment. PRACTICE IMPLICATIONS: The surveillance case definition identified twice as many people with SCD as the single-source administrative database definitions during the same period, but trade-offs exist in using single administrative databases for decisions on policy and program expansion for SCD.

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

PROBLEM/CONDITION: Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. Complications of SCD can include anemia, debilitating acute and chronic pain, infection, acute chest syndrome, stroke, and progressive organ damage, including decreased cognitive function and renal failure. Early diagnosis, screenings and preventive interventions, and access to specialist health care can decrease illness and death. Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. PERIOD COVERED: 2004-2018. DESCRIPTION OF THE PROGRAM: In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). Previously, surveillance for SCD was conducted by two short-term projects: Registry and Surveillance System for Hemoglobinopathies (RuSH), which was conducted during 2010-2012 and included 2004-2008 data, and Public Health Research, Epidemiology, and Surveillance for Hemoglobinopathies (PHRESH), which was conducted during 2012-2014 and included 2004-2008 data. Both California and Georgia participated in RuSH and PHRESH, which guided the development of the SCDC methods and case definitions. SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. These linkages serve to synthesize and disseminate population-based, longitudinal data for persons identified with SCD from multiple sources using selected International Classification of Diseases, Ninth Revision, Clinical Modification, and Tenth Revision codes and laboratory results confirmed through state newborn screening (NBS) programs or clinic case reporting. Administrative and clinical data sources include state Medicaid and Children's Health Insurance Program databases, death certificates, NBS programs, hospital discharge and emergency department records, and clinical records or case reports. Data from multiple sources and years are linked and deduplicated so that states can analyze and report on SCD population prevalence, demographic characteristics, health care access and use, and health outcomes. The SCD case definition is based on an algorithm that classifies cases with laboratory confirmation as confirmed cases and those with a reported clinical diagnosis or three or more diagnostic codes over a 5-year period from an administrative data source as probable cases. In 2019, nine states (Alabama, California, Georgia, Indiana, Michigan, Minnesota, North Carolina, Tennessee, and Virginia) were funded as part of an SCDC capacity-building initiative. The newly funded states developed strategies for SCD case identification and data linkage similar to those used by California and Georgia. As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. RESULTS: During 2004-2018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. The 2018 annual prevalence count was 6,027 cases for California and 9,141 for Georgia. Examination of prevalence counts by contributing data source during 2014-2018 revealed that each data source captured 16%-71% of cases in California and 17%-87% in Georgia; therefore, no individual source is sufficient to estimate statewide population prevalence. The proportion of pediatric SCD patients (children aged 0-18 years) was 27% in California and 40% in Georgia. The percentage of females with SCD in California and Georgia was 58% and 57%, respectively. Of the cases with SCD genotyping data available (n = 5,856), 63% of patients had sickle cell anemia. SCDC data have been used to directly apprise health care providers and policymakers about health care needs and gaps for patients with SCD. For example, an SCDC Georgia assessment indicated that 10% of babies born during 2004-2016 with SCD lived more than a 1-hour drive from any SCD specialty care option, and another 14% lived within a 1-hour drive of a periodic SCD specialty clinic only. Likewise, an SCDC California assessment indicated that during 2016-2018, most patients with SCD in Los Angeles County lived approximately 15-60 miles from hematologists experienced in SCD care. A surveillance capacity and performance assessment of all 11 SCDC states during 2020-2021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source. Nonetheless, methods for standardizing reporting were developed across all participating states. INTERPRETATION: This report is the first comprehensive description of CDC's efforts in collaboration with participating states to establish, maintain, and expand SCD surveillance through the SCDC program to improve health outcomes for persons living with SCD. Findings from California and Georgia analyses highlighted a need for additional SCD specialty clinics. Despite different approaches, expansion of SCDC to multiple states was possible using standardized, rigorous methods developed across all participating states for reporting on disease prevalence, health care needs and use, and deaths. PUBLIC HEALTH ACTION: Findings from surveillance can be used to improve and monitor care and outcomes for persons with SCD. These and other SCDC analyses have had a role in opening new SCD clinics, educating health care providers, developing state health care policies, and guiding new research initiatives. Public health officials can use this report as a guiding framework to plan or implement surveillance programs for persons with SCD. Both data-related activities (data sources; patient identifiers; and obtaining, transferring, and linking data) and the administrative considerations (stakeholder engagement, costs and resources, and long-term sustainability) are crucial to the success of these programs.

Vital Signs: Use of Recommended Health Care Measures to Prevent Selected Complications of Sickle Cell Anemia in Children and Adolescents - Selected U.S. States, 2019.

INTRODUCTION: Sickle cell disease (SCD), a group of inherited blood cell disorders that primarily affects Black or African American persons, is associated with severe complications and a >20-year reduction in life expectancy. In 2014, an expert panel convened by the National Heart, Lung, and Blood Institute issued recommendations to prevent or reduce complications in children and adolescents with the most severe SCD subtypes, known as sickle cell anemia (SCA); recommendations included 1) annual screening of children and adolescents aged 2-16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke and 2) offering hydroxyurea therapy to children and adolescents aged ≥9 months to reduce the risk for several life-threatening complications. METHODS: Data from the IBM MarketScan Multi-State Medicaid Database were analyzed. TCD screening and hydroxyurea use were examined for 3,352 children and adolescents with SCA aged 2-16 years and continuously enrolled in Medicaid during 2019. Percentage change during 2014-2019 and variation by health subgroups were assessed. Analyses were stratified by age. RESULTS: During 2014-2019, TCD screening increased 27% among children and adolescents aged 10-16 years; hydroxyurea use increased 27% among children aged 2-9 years and 23% among children and adolescents aged 10-16 years. However, in 2019, only 47% and 38% of children and adolescents aged 2-9 and 10-16 years, respectively, had received TCD screening and 38% and 53% of children and adolescents aged 2-9 years and 10-16 years, respectively, used hydroxyurea. For both prevention strategies, usage was highest among children and adolescents with high levels of health care utilization and evidence of previous complications indicative of severe disease. CONCLUSION AND IMPLICATIONS FOR PUBLIC HEALTH PRACTICE: Despite increases since 2014, TCD screening and hydroxyurea use remain low among children and adolescents with SCA. Health care providers should implement quality care strategies within their clinics and partner with patients, families, and community-based organizations to address barriers to delivering and receiving recommended care.

COVID-19 and Sickle Cell Disease-Related Deaths Reported in the United States.

Sickle cell disease (SCD) is associated with increased risk of poor health outcomes from respiratory infections, including COVID-19 illness. We used US death data to investigate changes in SCD-related mortality before and during the COVID-19 pandemic. We estimated annual age- and quarter-adjusted SCD-related mortality rates for 2014-2020. We estimated the number of excess deaths in 2020 compared with 2019 using the standardized mortality ratio (SMR). We found 1023 SCD-related deaths reported in the United States during 2020, of which 86 (8.4%) were associated with COVID-19. SCD-related deaths, both associated and not associated with COVID-19, occurred most frequently among adults aged 25-59 years. The SCD-related mortality rate changed <5% year to year from 2014 to 2019 but increased 12% in 2020; the sharpest increase was among adults aged ≥60 years. The SMR comparing 2020 with 2019 was 1.12 (95% CI, 1.06-1.19). Overall, 113 (95% CI, 54-166) excess SCD-related deaths occurred in 2020.

Surveillance for the Rare Condition of Sickle Cell Disease in Wisconsin.

INTRODUCTION: Despite universal newborn screening, there is no comprehensive surveillance system to understand the sickle cell disease population in Wisconsin. METHODS: We initiated the development of a sickle cell disease surveillance system by linking newborn screening data and electronic health records from 2 large tertiary health care institutions in Wisconsin: Children's Wisconsin and Froedtert Hospital. RESULTS: There were 1478 individuals within the 3 data sources. One hundred thirty-two (82%) of 159 identified by newborn screening from 2013 through 2019 received care at Children's Wisconsin. The majority of individuals with sickle cell disease at Children's Wisconsin and Froedtert Hospital resided in Milwaukee County. DISCUSSION: The new surveillance program will increase our understanding of the sickle cell disease population in Wisconsin and help improve quality of care and health outcomes.

Newborn Screening Practices for Beta-Thalassemia in the United States.

Beta-thalassemia, a heritable condition of abnormal hemoglobin production, is not a core condition on the United States Recommended Uniform Screening Panel (RUSP) for state and territorial newborn screening (NBS) programs. However, screening for sickle cell disease (which is on the core RUSP) also detects reduced or absent levels of hemoglobin (Hb) A and certain other Hb variants associated with beta-thalassemia and, thus, allows for a timely referral to appropriate healthcare to minimize sequalae of the disease. The Association of Public Health Laboratories' Hemoglobinopathy Workgroup administered a comprehensive survey of all U.S. NBS programs to assess beta-thalassemia testing methodologies, the cutoffs for defining beta-thalassemia major, and the reporting and follow-up practices. Forty-six (87%) of the programs responded. Thirty-nine of the 46 responding programs (85%) report some form of suspected beta-thalassemia; however, the screening methods, the percentage of Hb A used as a cutoff for an indication of beta-thalassemia major, and the screening follow-up vary widely. The standardization of technical and reporting procedures may improve access to specialty care prior to severe complications, increase genetic counseling, and provide data needed to better understand the public health impact and clinical outcomes of beta-thalassemia in the United States.

Newborn Screening Practices and Alpha-Thalassemia Detection - United States, 2016.

Alpha-thalassemia comprises a group of inherited disorders in which alpha-hemoglobin chain production is reduced. Depending on the genotype, alpha-thalassemia results in moderate to profound anemia, hemolysis, growth delays, splenomegaly, and increased risk for thromboembolic events; certain patients might require chronic transfusions. Although alpha-thalassemia is not a core condition of the United States Recommended Uniform Screening Panel* for state newborn screening programs, methodologies used by some newborn screening programs to detect sickle cell disease, which is a core panel condition, also detect a quantitative marker of alpha-thalassemia, hemoglobin (Hb) Bart's, an abnormal type of hemoglobin. The percentage of Hb Bart's detected correlates with alpha-thalassemia severity. The Association of Public Health Laboratories' Hemoglobinopathy Workgroup conducted a survey of state newborn screening programs' alpha-thalassemia screening methodologies and reporting and follow-up practices. Survey findings indicated that 41 of 44 responding programs (93%) report some form of alpha-thalassemia results and 57% used a two-method screening protocol. However, the percentage of Hb Bart's used for thalassemia classification, the types of alpha-thalassemia reported, and the recipients of this information varied widely. These survey findings highlight the opportunity for newborn screening programs to revisit their policies as they reevaluate their practices in light of the recently released guideline from the Clinical and Laboratory Standards Institute (CLSI) on Newborn Screening for Hemoglobinopathies (1). Although deferring to local programs for policies, the report used a cutoff of 25% Hb Bart's in its decision tree, a value many programs do not use. Standardization of screening and reporting might lead to more timely diagnoses and health care services and improved outcomes for persons with a clinically significant alpha-thalassemia.

A National Measurement Framework to Assess and Improve Sickle Cell Care in 4 US Regions.

OBJECTIVES: Coordinated measurement strategies are needed to inform collaborative approaches to improve access to and quality of care for persons with sickle cell disease (SCD). The objective of our study was to develop a multilevel measurement strategy to assess improvements in access to and quality of care for persons with SCD in 4 US regions. METHODS: From 2014 through 2017, regional grantees in the Sickle Cell Disease Treatment Demonstration Program collected administrative and patient-level electronic health record (EHR) data to assess quality improvement initiatives. Four grantees-covering 29 US states and territories and an SCD population of 56 720-used a collective impact model to organize their work. The grantees collected administrative data from state Medicaid and Medicaid managed care organizations (MCOs) at multiple points during 2014-2017 to assess improvements at the population level, and local patient-level data were abstracted from site-level EHRs at regular intervals to track improvements over time. RESULTS: Administrative data were an important source of understanding population-level improvements but were delayed, whereas patient-level data were more sensitive to small-scale quality improvements. CONCLUSIONS: We established a shared measurement approach in partnership with Medicaid and Medicaid MCO stakeholders that can be leveraged to effectively support quality improvement initiatives for persons with SCD in the United States.

Providers' Perspectives on Treating Patients With Thalassemia.

In recent years, California has experienced a steady rise in Asian immigration which has led to a corresponding increased prevalence of clinically significant thalassemia in this state. As part of the Public Health Research, Education and Surveillance for Hemoglobinopathies emoglobinopathies project, a survey was developed to collect information from California providers who care for thalassemia patients in an effort to better understand their practice patterns, barriers to providing care, and educational needs. When asked about educational needs, providers most frequently expressed a desire for care and management guidelines (65.3%), health educational materials for patients (47.2%), and information on complications and clinical outcomes (32.1%). Only one quarter of providers (24.0%) reported that all of their thalassemia patients have a coordinated care plan. The increase in California thalassemia cases highlights the importance of provider knowledge to effectively serve the patients in their communities. Provider education and dissemination of treatment standards can not only improve knowledge about the disease but also increase awareness about the importance of coordinating care among a multidisciplinary team of specialists. Improvement in these areas will help achieve the overarching goal of better outcomes and quality of life for patients with thalassemia.

CE: Understanding the Complications of Sickle Cell Disease.

: Sickle cell disease (SCD) is an autosomal recessive genetic condition that alters the shape and function of the hemoglobin molecule in red blood cells. While the overall survival rate among children with SCD has improved in recent years, pediatric rates of hospitalization, ED use, and mortality from complications of SCD remain high. Among patients ages 18 and older, hospital admission and ED usage are even greater-and the median age at death of people with SCD is considerably lower than that of the general population. Nurses who care for patients with SCD have an opportunity to improve health outcomes and quality of life for these patients by recognizing the major SCD-associated complications and providing patients and their caregivers with appropriate educational information. The authors discuss the genetic, hematologic, and clinical features of SCD and describe the major associated health complications. In addition, they review the nursing implications of each complication and provide online links to resources for clinicians, patients, and caregivers.

Effectiveness of Clinical Decision Support Based Intervention in the Improvement of Care for Adult Sickle Cell Disease Patients in Primary Care.

INTRODUCTION: Although most patients with rare diseases like sickle cell disease (SCD) are treated in the primary care setting, primary care physicians may find it challenging to keep abreast of medication improvements and complications associated with treatment for rare and complex diseases. The purpose of this study was to evaluate the effectiveness of a clinical decision support (CDS) -based intervention system for transfusional iron overload in adults with SCD to improve management in primary care. METHODS: An electronic medical record based clinical decision support system for potential transfusional iron overload in SCD patients in primary care was evaluated. The intervention was implemented in 3 family medicine clinics with a control group of 3 general internal medicine clinics. Data were collected in the 6 months before the intervention and 6 months after the intervention. There were 47 patients in the family medicine group and 24 in the general internal medicine group. RESULTS: There was no management change in the control group while the intervention group improved primary care management from 0% to 44% (P < .001). CONCLUSION: A CDS tool can improve management of SCD patients in primary care.

The accuracy of hospital ICD-9-CM codes for determining Sickle Cell Disease genotype.

Sickle cell disease affects more than 100,000 individuals in the United States, among whom disease severity varies considerably. One factor that influences disease severity is the sickle cell disease genotype. For this reason, clinical prevention and treatment guidelines tend to differentiate between genotypes. However, previous research suggests caution when using a claimsbased determination of sickle cell disease genotype in healthcare quality studies. The objective of this study was to describe the extent of miscoding for the major sickle cell disease genotypes in hospital discharge data. Individuals with sickle cell disease were identified through newborn screening results or hemoglobinopathy specialty care centers, along with their sickle cell disease genotypes. These genotypes were compared to the diagnosis codes listed in hospital discharge data to assess the accuracy of the hospital codes in determining sickle cell disease genotype. Eighty-three percent (sickle cell anemia), 23% (Hemoglobin SC), and 31% (Hemoglobin Sß+ thalassemia) of hospitalizations contained a diagnosis code that correctly reflected the individual's true sickle cell disease genotype. The accuracy of the sickle cell disease genotype coding was indeterminate in 11% (sickle cell anemia), 12% (Hemoglobin SC), and 7% (Hemoglobin Sß+ thalassemia) and incorrect in 3% (sickle cell anemia), 61% (Hemoglobin SC), and 52% (Hemoglobin Sß+ thalassemia) of the hospitalizations. The use of ICD-9-CM codes from hospital discharge data for determining specific sickle cell disease genotypes is problematic. Research based solely on these or other types of administrative data could lead to incorrect understanding of the disease.

CDC Grand Rounds: Improving the Lives of Persons with Sickle Cell Disease.

Approximately 100,000 Americans have sickle cell disease (SCD), a group of recessively inherited red blood cell disorders characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in the red blood cells. Persons with hemoglobin SS or hemoglobin Sß0 thalassemia, also known as sickle cell anemia (SCA), have the most severe form of SCD. Hemoglobin SC disease and hemoglobin Sß+ thalassemia are other common forms of SCD. Red blood cells that contain sickle hemoglobin are inflexible and can stick to vessel walls, causing a blockage that slows or stops blood flow. When this happens, oxygen cannot reach nearby tissues, leading to attacks of sudden, severe pain, called pain crises, which are the clinical hallmark of SCD. The red cell sickling and poor oxygen delivery can also cause damage to the brain, spleen, eyes, lungs, liver, and multiple other organs and organ systems. These chronic complications can lead to increased morbidity, early mortality, or both. Tremendous strides in treating and preventing the complications of SCD have extended life expectancy. Now, nearly 95% of persons born with SCD in the United States reach age 18 years (1); however, adults with the most severe forms of SCD have a life span that is 20-30 years shorter than that of persons without SCD (2).

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.

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.