The Lives Lost to Inequities: Avertable Deaths From Neurologic Diseases in the Past Decade.

BACKGROUND AND OBJECTIVES: Mortality rates for neurologic diseases are increasing in the United States, with large disparities across geographical areas and populations. Racial and ethnic populations, notably the non-Hispanic (NH) Black population, experience higher mortality rates for many causes of death, but the magnitude of the disparities for neurologic diseases is unclear. The objectives of this study were to calculate mortality rates for neurologic diseases by race and ethnicity and-to place this disparity in perspective-to estimate how many US deaths would have been averted in the past decade if the NH Black population experienced the same mortality rates as other groups. METHODS: Mortality rates for deaths attributed to neurologic diseases, as defined by the International Classification of Diseases, were calculated for 2010 to 2019 using death and population data obtained from the Centers for Disease Control and Prevention and the US Census Bureau. Avertable deaths were calculated by indirect standardization: For each calendar year of the decade, age-specific death rates of NH White persons in 10 age groups were multiplied by the NH Black population in each age group. A secondary analysis used Hispanic and NH Asian populations as the reference groups. RESULTS: In 2013, overall age-adjusted mortality rates for neurologic diseases began increasing, with the NH Black population experiencing higher rates than NH White, NH American Indian and Alaska Native, Hispanic, and NH Asian populations (in decreasing order). Other populations with higher mortality rates for neurologic diseases included older adults, the male population, and adults older than 25 years without a high school diploma. The gap in mortality rates for neurologic diseases between the NH Black and NH White populations widened from 4.2 individuals per 100,000 in 2011 to 7.0 per 100,000 in 2019. Over 2010 to 2019, had the NH Black population experienced the neurologic mortality rates of NH White, Hispanic, or NH Asian populations, 29,986, 88,407, or 117,519 deaths, respectively, would have been averted. DISCUSSION: Death rates for neurologic diseases are increasing. Disproportionately higher neurologic mortality rates in the NH Black population are responsible for a large number of excess deaths, making research and policy efforts to address the systemic causes increasingly urgent.

Use of Community-Engaged Research Approaches in Clinical Interventions for Neurologic Disorders in the United States: A Scoping Review and Future Directions for Improving Health Equity Research.

BACKGROUND AND OBJECTIVES: Evidence suggests a significant prevalence of race and ethnic disparities in the United States among people with neurologic conditions including stroke, Alzheimer disease and related dementia (ADRD), Parkinson disease (PD), epilepsy, spinal cord injury (SCI), and traumatic brain injury (TBI). Recent neurologic research has begun the paradigm shift from observational health disparities research to intervention research in an effort to narrow the disparities gap. There is an evidence base that suggests that community engagement is a necessary component of health equity. While the increase in disparities focused neurologic interventions is encouraging, it remains unclear whether and how community-engaged practices are integrated into intervention design and implementation. The purpose of this scoping review was to identify and synthesize intervention studies that have actively engaged with the community in the design and implementation of interventions to reduce disparities in neurologic conditions and to describe the common community engagement processes used. METHODS: Two databases, PubMed and CINAHL, were searched to identify eligible empirical studies within the United States whose focus was on neurologic interventions addressing disparities and using community engagement practices. RESULTS: We identified 392 disparity-focused interventions in stroke, ADRD, PD, epilepsy, SCI, and TBI, of which 53 studies incorporated community engagement practices: 32 stroke studies, 15 ADRD, 2 epilepsy studies, 2 PD studies, 1 SCI study, and 1 TBI study. Most of the interventions were designed as randomized controlled trials and were programmatic in nature. The interventions used a variety of community engagement practices: community partners (42%), culturally tailored materials and mobile health (40%), community health workers (32%), faith-based organizations and local businesses (28%), focus groups/health need assessments (25%), community advisory boards (19%), personnel recruited from the community/champions (19%), and caregiver/social support (15%). DISCUSSION: Our scoping review reports that the proportion of neurologic intervention studies incorporating community engagement practices is limited and that the practices used within those studies are varied. The major practices used included collaboration with community partners and utilization of culturally tailored materials. We also found inconsistent reporting and dissemination of results from studies that implemented community engagement measures in their interventions. Future directions include involving the community in research early and continuously, building curricula that address challenges to community engagement, prioritizing the inclusion of community engagement reporting in peer-reviewed journals, and prioritizing and incentivizing research of subpopulations that experience disparities in neurologic conditions.

Indirect Economic Burden of Sickle Cell Disease.

OBJECTIVE: This study aimed to quantify the indirect costs of sickle cell disease in the United States. METHODS: Adult patients from a sickle cell disease clinic at an urban academic healthcare system completed an adapted Institute for Medical Technology Assessment Productivity Cost Questionnaire related to the impact of their disorder on absenteeism, presenteeism, ability to contribute through unpaid work outside of employment, and other aspects of life. Additional data were collected from patient records about each participant's genotype, total hemoglobin level, and pain level. RESULTS: Of the 192 individuals, 187 who completed the survey reported experiencing vaso-occlusive crisis pain events during the last year that negatively affected their productivity at work and in daily roles. Three-fourths of respondents reported impairment in their ability to complete everyday tasks, such as caring for children, running errands, doing housework, shopping for groceries, and volunteer (unpaid) work. Only 30% of respondents reported being employed or self-employed. Of those employed, estimated costs of absenteeism and presenteeism attributable to pain events averaged $15 103 per person annually. Estimated total annual losses in unpaid work productivity averaged $3 145 862 for the study respondents and another $2 870 652 for their caregivers. CONCLUSIONS: Sickle cell disease affected the work productivity, nonwork productivity, and the daily lives of adults seen with the disorder in an academic medical center.

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects.

Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

Predictive Ability of Intermittent Daily Sickle Cell Pain Assessment: The PiSCES Project.

Background: Pain diary assessment in sickle cell disease (SCD) may be expensive and impose a high respondent burden. Objective: To report whether intermittent assessment could substitute for continuous daily pain assessment in SCD. Design: Prospective cohort study. Setting: Academic and community practices in Virginia. Patients. A total of 125 SCD patients age 16 years or older in the Pain in Sickle Cell Epidemiology Study. Measurements. Using pain measures that summarized all diaries as the gold standard, we tested the statistical equivalence of four alternative strategies that summarized diaries only from the week prior or the month prior to study completion; one week per month; or one day per week (random day). Summary measures included percent pain days, percent crisis days (self-defined), mean pain (0-9 Likert scale) on all days, and mean pain on pain days. Equivalence tests included comparisons of means, regression intercepts, and slopes, as well as measurement of R2. Results: Compared with the gold standard, the one-day-per-week and one-week-per-month strategies yielded statistically equivalent means of six summary pain measures, and the week prior and month prior yielded equivalent means as some of the measures. Regression showed statistically equivalent slopes and intercepts to the gold standard using one-day-per-week and one-week-per-month strategies for percent pain days and percent crisis days, but almost no other equivalence. R2 values ranged from 0.64 to 0.989. Conclusions: It is possible to simulate five- to six-month daily assessment of pain in SCD. Either one-day-per-week or one-week-per-month assessment yields an equivalent mean and fair regression equivalence.

Community Health Workers as Support for Sickle Cell Care.

Community health workers are increasingly recognized as useful for improving health care and health outcomes for a variety of chronic conditions. Community health workers can provide social support, navigation of health systems and resources, and lay counseling. Social and cultural alignment of community health workers with the population they serve is an important aspect of community health worker intervention. Although community health worker interventions have been shown to improve patient-centered outcomes in underserved communities, these interventions have not been evaluated with sickle cell disease. Evidence from other disease areas suggests that community health worker intervention also would be effective for these patients. Sickle cell disease is complex, with a range of barriers to multifaceted care needs at the individual, family/friend, clinical organization, and community levels. Care delivery is complicated by disparities in health care: access, delivery, services, and cultural mismatches between providers and families. Current practices inadequately address or provide incomplete control of symptoms, especially pain, resulting in decreased quality of life and high medical expense. The authors propose that care and care outcomes for people with sickle cell disease could be improved through community health worker case management, social support, and health system navigation. This paper outlines implementation strategies in current use to test community health workers for sickle cell disease management in a variety of settings. National medical and advocacy efforts to develop the community health workforce for sickle cell disease management may enhance the progress and development of "best practices" for this area of community-based care.

Development of a sickle cell disease readiness for transition assessment.

BACKGROUND: Instrumentation with established reliability and validity is not yet routinely utilized to assess readiness for transition from pediatric to adult care for youth and young adults with chronic conditions, including sickle cell disease (SCD). OBJECTIVE: The aim of this study was to develop a SCD specific readiness for transition assessment tool. SUBJECTS: Fifty-seven youths with SCD, ages 15-21 years, completed the initial version of the Transition Intervention Program - Readiness for Transition (TIP-RFT) assessment; 113 youths/young adults with SCD, ages 14-26 years, at two distinct sites of care completed a refined version of the TIP-RFT. METHODS: The TIP-RFT was constructed based on a literature review, provider and patient consensus and assessed domains including knowledge and skills in medical self-care, social support, health benefits and independent living and educational/vocational skills. We used principal components factor analysis to evaluate TIP-RFT responses and assessed differences in TIP-RFT scores in relation to age, gender, sickle cell diagnosis and site of care. RESULTS: The original TIP-RFT, which had demonstrated face validity, was reduced from 56 to 22 items. The revised instrument consisting of four subscales demonstrated good internal consistency reliability and construct validity. CONCLUSION: Our results support that the TIP-RFT is a valid and reliable tool for the assessment of transition readiness for youths with SCD. The TIP-RFT assessment can guide interventions to improve transition readiness and can provide a foundation for future research on other variables that might be associated with transition readiness.

Daily home opioid use in adults with sickle cell disease: The PiSCES project.

BACKGROUND: Although opioid prescribing in sickle cell disease (SCD) can be controversial, little is published about patterns of opioid use. OBJECTIVE: To report on home opioid use among adults with SCD. DESIGN: Cohort study. PARTICIPANTS: Adults with SCD (n=219) who completed daily pain diaries for up to 6 months and had at least one home pain day. MAIN MEASURES: Use of long-acting or short-acting opioids, other analgesics, or adjuvants; the proportion of home days, home pain days, and home crisis days with opioid use; these two outcomes according to patient characteristics. KEY RESULTS: Patients used opioids on 12,311 (78 percent) of 15,778 home pain days. Eighty-five patients (38.8 percent) used long-acting opioids with or without short-acting opioids and 103 (47.0 percent) used only short-acting opioids. Twenty-one (9.6 percent) patients used only non-opioid analgesics and 10 (4.6 percent) used no analgesics. Both pain intensity and pain frequency were higher among opioid users (analysis of variance [ANOVA], p<0.0001). Opioid users used hydroxyurea more often than nonusers, even when controlling for mean pain on pain days. Among all patients, significant relationships were found between any opioid use and somatic symptom burden, SCD stress, negative coping, and physical and mental quality of life (QOL); the relationship with SCD stress and physical QOL remained when controlled for mean pain. Among opioid users, similar associations were found between frequency of opioid use and some disease-related and psychosocial variables. CONCLUSIONS: In this adult SCD sample, opioids were used by the majority of patients. Pain was the overwhelming characteristic associated with use, but disease-related and psychosocial variables were also associated.

Daily assessment of pain in adults with sickle cell disease.

BACKGROUND: Researchers of sickle cell disease have traditionally used health care utilization as a proxy for pain and underlying vaso-occlusion. However, utilization may not completely reflect the amount of self-reported pain or acute, painful episodes (crises). OBJECTIVE: To examine the prevalence of self-reported pain and the relationship among pain, crises, and utilization in adults with sickle cell disease. DESIGN: Prospective cohort study. SETTING: Academic and community practices in Virginia. PATIENTS: 232 patients age 16 years or older with sickle cell disease. MEASUREMENTS: Patients completed a daily diary for up to 6 months, recording their maximum pain (on a scale of 0 to 9); whether they were in a crisis (crisis day); and whether they used hospital, emergency, or unscheduled ambulatory care for pain on the previous day (utilization day). Summary measures included both simple proportions and adjusted probabilities (for repeated measures within patients) of pain days, crisis days, and utilization days, as well as mean pain intensity. RESULTS: Pain (with or without crisis or utilization of care) was reported on 54.5% of 31 017 analyzed patient-days (adjusted probability, 56%). Crises without utilization were reported on 12.7% of days and utilization on only 3.5% (unadjusted). In total, 29.3% of patients reported pain in greater than 95% of diary days, whereas only 14.2% reported pain in 5% or fewer diary days (adjusted). The frequency of home opiate use varied and independently predicted pain, crises, and utilization. Mean pain intensity on crisis days, noncrisis pain days, and total pain days increased as the percentage of pain days increased (P < 0.001). Intensity was significantly higher on utilization days (P < 0.001). However, utilization was not an independent predictor of crisis, after controlling for pain intensity. LIMITATIONS: The study was done in a single state. Patients did not always send in their diaries. CONCLUSION: Pain in adults with sickle cell disease is the rule rather than the exception and is far more prevalent and severe than previous large-scale studies have portrayed. It is mostly managed at home; therefore, its prevalence is probably underestimated by health care providers, resulting in misclassification, distorted communication, and undertreatment.

Depression and anxiety in adults with sickle cell disease: the PiSCES project.

OBJECTIVE: Depression and anxiety are common in sickle cell disease (SCD) but relatively little is known about their impact on SCD adults. This study measured prevalence of depression and anxiety in SCD adults, and their effects on crisis and noncrisis pain, quality-of-life, opioid usage, and healthcare utilization. METHODS: The Pain in Sickle Cell Epidemiology Study is a prospective cohort study in 308 SCD adults. Baseline variables included demographics, genotype, laboratory data, health-related quality-of-life, depression, and anxiety. Subjects completed daily diaries for up to 6 months, reporting sickle cell pain intensity, distress, interference, whether they were in a sickle cell crisis, as well as health care and opioid utilization. RESULTS: Two hundred thirty-two subjects who completed at least 1 month of diaries were studied; 27.6% were depressed and 6.5% had any anxiety disorder. Depressed subjects had pain on significantly more days than nondepressed subjects (mean pain days 71.1% versus 49.6%, p < .001). When in pain on noncrisis days, depressed subjects had higher mean pain, distress from pain, and interference from pain. Both depressed and anxious subjects had poorer functioning on all eight SF-36 subscales, even after controlling for demographics, hemoglobin type, and pain. The anxious subjects had more pain, distress from pain, and interference from pain, both on noncrisis pain days and on crisis days, and used opioids more often. CONCLUSIONS: Depression and anxiety predicted more daily pain and poorer physical and mental quality-of-life in adults with SCD, and accounted for more of the variance in all domains of quality-of-life than hemoglobin type.

Recommendations for teaching about racial and ethnic disparities in health and health care.

Racial and ethnic minorities often receive lower-quality health care than white patients, even when socioeconomic status, education, access, and other factors are used as controls. To address these pervasive disparities, health care professionals should learn more about them and the roles they can play in eliminating them, but few curricula are focused on understanding and addressing racial and ethnic health disparities, and well-accepted guidelines on what and how to teach in this complex area are lacking. The Society of General Internal Medicine Health Disparities Task Force used a review and consensus process to develop specific recommendations and guidelines for curricula focusing on health disparities. Learning objectives, content, methods for teaching, and useful resources are provided. Although the guidelines were developed primarily for teaching medical students, residents, and practitioners in primary care, the Task Force's general recommendations can apply to learners in any specialty. The Task Force recommends that a curricula address 3 areas of racial and ethnic health disparities and focus on the following specific learning objectives:1) examining and understanding attitudes, such as mistrust, subconscious bias, and stereotyping, which practitioners and patients may bring to clinical encounters; 2) gaining knowledge of the existence and magnitude of health disparities, including the multifactorial causes of health disparities and the many solutions required to diminish or eliminate them; and 3) acquiring the skills to effectively communicate and negotiate across cultures, languages, and literacy levels, including the use of key tools to improve communication. The broad goal of a curriculum on disparities should be for learners to develop a commitment to eliminating inequities in health care quality by understanding and assuming their professional role in addressing this pressing health care crisis.

Timeliness of immunizations of children in a Medicaid primary care case management managed care program.

OBJECTIVE: This study assessed the timeliness of immunization for children in a Medicaid managed care primary care case management program controlling for patient and provider predictors of immunization status. METHODS: Using administrative data and patient medical records, up-to-date (UTD) and age appropriate immunization (AAI) status were reviewed for 5598 children. The 4:3:1 immunization series (four diphtheria, pertussis, tetanus vaccinations; three polio vaccinations; and one measles, mumps, rubella vaccination) was the standard. RESULTS: Childhood immunization rates were low when assessed using strict adherence to vaccination recommendations. At age 18 months, 28.3% were classified as UTD, and 6.3% were classified as AAI. Compared to children not up-to-date, UTD children were more likely to have public rather than private providers, to have had older mothers, and less likely to have been African American. Among UTD children, AAI children were more likely to reside in urban areas. CONCLUSIONS: Low-income children continue to be under-immunized, even under a managed care initiative. Health care providers and child health advocates need to continue pressure for programs that will increase adherence to nationally recommended guidelines.