Injury-related emergency department use among people with intellectual and developmental disabilities insured by Medicaid from 2010 to 2016.

OBJECTIVES: Data on non-fatal injuries and visits to the emergency department (ED) for injuries are not readily available. The objective of this paper is to describe injury-related ED visits for people with intellectual and developmental disabilities who are covered by the Medicaid insurance programme. METHODS: We aggregated 2010-2016 Medicaid claims data from eight states. Using these data, we identified individuals with intellectual and developmental disabilities and then determined an all-cause ED visit rate, ED visit due to injury rate and admission from ED due to injury rate. Data were stratified by sex and age group. Results were compared with national rates. RESULTS: Medicaid members with intellectual and developmental disabilities visited EDs at approximately 1.8 times the rate of the general population. The ED visit rate due to injury was approximately 1.5 times that observed in the population overall. When ED visits due to injury data were stratified by age and sex, the largest discrepancy was observed in women ages 45-64, who visited EDs due to injury at a rate 2.1 times that of women of the same age in the general population. The admission rate from ED due to injury increased over the study period most notably in the older age groups. CONCLUSIONS: While rates and patterns of ED utilisation among Medicaid members with intellectual and developmental disabilities vary by age and gender, our findings suggest this group visits the ED due to injury at rates well above the general population.

Disability, Hospital Care, and Cost: Utilization of Emergency and Inpatient Care by a Cohort of Children with Intellectual and Developmental Disabilities.

OBJECTIVE: To use medical claims data to determine patterns of healthcare utilization in children with intellectual and developmental disabilities, including frequency of service utilization, conditions that require hospital care, and costs. STUDY DESIGN: Medicaid administrative claims from 4 states (Iowa, Massachusetts, New York, and South Carolina) from years 2008-2013 were analyzed, including 108 789 children (75 417 male; 33 372 female) under age 18 years with intellectual and developmental disabilities. Diagnoses included cerebral palsy, autism, fetal alcohol syndrome, Down syndrome/trisomy/autosomal deletions, other genetic conditions, and intellectual disability. Utilization of emergency department (ED) and inpatient hospital services were analyzed for 2012. RESULTS: Children with intellectual and developmental disabilities used both inpatient and ED care at 1.8 times that of the general population. Epilepsy/convulsions was the most frequent reason for hospitalization at 20 times the relative risk of the general population. Other frequent diagnoses requiring hospitalization were mood disorders, pneumonia, paralysis, and asthma. Annual per capita expenses for hospitalization and ED care were 100% higher for children with intellectual and developmental disabilities, compared with the general population ($153 348 562 and $76 654 361, respectively). CONCLUSIONS: Children with intellectual and developmental disabilities utilize significantly more ED and inpatient care than other children, which results in higher annual costs. Recognizing chronic conditions that increase risk for hospital care can provide guidance for developing outpatient care strategies that anticipate common clinical problems in intellectual and developmental disabilities and ensure responsive management before hospital care is needed.

Mental Health Trainee Facilitation of Sibling Support Groups: Understanding its Influence on Views and Skills of Family-Centered Care.

OBJECTIVES: Prior research suggests family-centered interventions are among the least taught yet most needed skills for practicing psychiatry. In this study, we evaluated whether having mental health trainees lead a sibling support group could serve as a method to promote family-centered care among trainees. METHODS: All trainees in psychiatry, psychology, and social work were invited to participate as sibling support group facilitators. Both facilitator and non-facilitator trainees were then surveyed using a questionnaire inquiring about exposure to family-centered care, comfort level in providing family-centered care, attitudes regarding the importance of family-centered care, and desire to provide family-centered care in the future. A second survey was administered to the facilitator trainees to assess their perceptions of the sibling group leader experience. RESULTS: Facilitator trainees reported increased engagement in family-centered activities during training (p < 0.05), expressed greater confidence in their family-centered care skills (p < 0.05), and reported stronger intentions to practice in a family-centered way (p < 0.05). Facilitator trainees were overwhelmingly positive about their experience with the sibling support program and reported it strengthened their commitment to addressing the needs of siblings as a part of family-centered care. CONCLUSIONS: Facilitating a sibling support group may be an effective way for mental health trainees to gain skills and confidence in delivering family-centered care. Mental health training programs aiming to imbue trainees with the importance of family-centered care may consider creating opportunities for trainees to facilitate sibling support groups.

Patterns of homozygosity in patients with uniparental disomy: detection rate and suggested reporting thresholds for SNP microarrays.

PURPOSE: Single-nucleotide polymorphism (SNP) microarrays can easily identify whole-chromosome isodisomy but are unable to detect whole-chromosome heterodisomy. However, most cases of uniparental disomy (UPD) involve combinations of heterodisomy and isodisomy, visualized on SNP microarrays as long continuous stretches of homozygosity (LCSH). LCSH raise suspicion for, but are not diagnostic of, UPD, and reporting necessitates confirmatory testing. The goal of this study was to define optimal LCSH reporting standards. METHODS: Eighty-nine individuals with known UPD were analyzed using chromosomal microarray. The LCSH patterns were compared with those in a phenotypically normal population to predict the clinical impact of various reporting thresholds. False-positive and -negative rates were calculated at various LCSH thresholds. RESULTS: Twenty-seven of 84 cases with UPD had no significant LCSH on the involved chromosome. Fifty UPD-positive samples had LCSH of varying sizes: the average size of terminal LCSH was 11.0 megabases while the average size of interstitial LCSH was 24.1 megabases. LCSH in the normal population tended to be much smaller (average 4.3 megabases) and almost exclusively interstitial; however, overlap between the populations was noted. CONCLUSION: We hope that this work will aid clinical laboratories in the recognition and reporting of LCSH.

Robust Vaccine Responses in Adult and Pediatric Cord Blood Transplantation Recipients Treated for Hematologic Malignancies.

Because cord blood (CB) lacks memory T and B cells and recent decreases in herd immunity to vaccine-preventable diseases in many developed countries have been documented, vaccine responses in CB transplantation (CBT) survivors are of great interest. We analyzed vaccine responses in double-unit CBT recipients transplanted for hematologic malignancies. In 103 vaccine-eligible patients, graft-versus-host disease (GVHD) most commonly precluded vaccination. Sixty-five patients (63%; engrafting units median HLA-allele match 5/8; range, 2 to 7/8) received protein conjugated vaccines, and 63 patients (median age, 34 years; range, .9 to 64) were evaluated for responses. Median vaccination time was 17 months (range, 7 to 45) post-CBT. GVHD (n = 42) and prior rituximab (n = 13) delayed vaccination. Responses to Prevnar 7 and/or 13 vaccines (serotypes 14, 19F, 23F) were seen in children and adults (60% versus 49%, P = .555). Responses to tetanus, diphtheria, pertussis, Haemophilus influenzae, and polio were observed in children (86% to 100%) and adults (53% to 89%) even if patients had prior GVHD or rituximab. CD4(+)CD45RA(+) and CD19(+) cell recovery significantly influenced tetanus and polio responses. In a smaller cohort responses were seen to measles (65%), mumps (50%), and rubella (100%) vaccines. No vaccine side effects were identified, and all vaccinated patients survived (median follow-up, 57 months). Although GVHD and rituximab can delay vaccination, CBT recipients (including adults and those with prior GVHD) have similar vaccine response rates to adult donor allograft recipients supporting vaccination in CBT recipients.

Mortality of People with Intellectual and Developmental Disabilities from Select US State Disability Service Systems and Medical Claims Data.

BACKGROUND: Monitoring population trends including mortality within subgroups such as people with intellectual and developmental disabilities and between countries provides crucial information about the population's health and insights into underlying health concerns and the need for and effectiveness of public health efforts. METHODS: Data from both US state intellectual and developmental disabilities service system administrative data sets and de-identified state Medicaid claims were used to calculate average age at death and crude mortality rates. RESULTS: Average age at death for people in state intellectual and developmental disabilities systems was 50.4-58.7 years and 61.2-63.0 years in Medicaid data, with a crude adult mortality rate of 15.2 per thousand. CONCLUSIONS: Age at death remains lower and mortality rates higher for people with intellectual and developmental disabilities. Improved case finding (e.g. medical claims) could provide more complete mortality patterns for the population with intellectual and developmental disabilities to inform the range of access and receipt of supportive and health-related interventions and preventive care.

Mortality in People with Intellectual Disabilities.

This paper reviews why an understanding of mortality data in general, and in relation to people with intellectual disabilities in particular, is an important area of concern, and introduces the papers in this Special Edition.

Valosin-containing-protein pathogenic variant p.R487H in Parkinson's disease.

We describe a 66-year-old woman with Parkinson's disease, carrying a known pathogenic missense variant in the Valosin-containing-protein (VCP) gene. She responded excellently to L-dopa, had no cognitive or motoneuronal dysfunction. Laboratory analyses and MRI were unremarkable. Genetic testing revealed a heterozygous variant in VCP(NM_007126.5), chr9 (GRCh3 7):g.35060820C > T, c.1460G > A p.Arg487His (p.R487H).

Sibling Support Program: A Novel Peer Support Intervention for Parents, Caregivers and Siblings of Youth Experiencing Mental Illness.

Caregivers and siblings of youth with mental illness often experience role-related psychological challenges, and it is important to focus on the needs of these family members. Existing literature demonstrates that caregivers and affected children benefit from participation in peer support and family-centered programs. This paper describes the Sibling Support Program: A Family-Centered Mental Health Initiative (SSP), a novel intervention for families of youth with mental illness. The SSP distinguishes itself from existing family-centered programs in that it utilizes a unique combination of peer support, parent mentor guidance, and clinician-led group therapy. The paper details the structure of the treatment model and presents preliminary data from participant surveys. Results show preliminary indications that the program provides both emotional and practical benefits. Along with high satisfaction ratings, family members report decreased feelings of isolation, gains in knowledge, and more positive thinking after program participation. Caregivers report that the SSP helped improve their understanding about the impact of a child's mental illness on family members, and that they learned about effective family management strategies and access to resources. Siblings report learning coping strategies and feeling better after meeting peers with shared experiences.

Respiratory support strategies in the prevention of bronchopulmonary dysplasia: A single center quality improvement initiative.

Background and objectives: Bronchopulmonary dysplasia (BPD) continues to be a significant morbidity affecting very preterm infants, despite multiple advancements in therapies to treat respiratory distress syndrome and prevent BPD. Local quality improvement (QI) efforts have shown promise in reducing unit or system-wide rates of BPD. In preterm infants born between 23- and 32-weeks' gestation, our aim was to decrease the rate of BPD at 36 weeks corrected gestational age from 43% to 28% by January 2019. Methods: Directed by a multidisciplinary respiratory QI team, we gradually implemented the following interventions to reach our aim: (1) early initiation of non-invasive ventilation in the delivery room, (2) initiation of caffeine prior to 24 h of life, (3) administration of early selective surfactant per a well-defined guideline, (4) continuation of non-invasive ventilation until 32 and 0/7 weeks corrected gestational age (CGA), and (5) a revision of the early selective surfactant guideline. Outcome measures included rates of BPD, and process measures included compliance with the above interventions. Results: A total of 509 infants with an average gestational age of 29 1/7 weeks and birth weight of 1,254 (SD±401) grams were included. The rate of BPD in our unit decreased from a baseline of 43% to 19% from the start of the project in October 2016 until the first quarter of 2022 (p < 0.00001). The greatest reductions in BPD rates were seen after the initiation of the guideline to extend non-invasive ventilation until 32 0/7 weeks CGA. The rate of severe BPD decreased from 22% to 9%. Conclusions: In preterm infants born between 23- and 32-weeks' gestation, our local QI interventions to reduce rates of BPD were associated with a reduction in rates by 56%. Increased use of antenatal steroids and higher birth weights post- vs. pre-intervention may have contributed to this successes.

Time trends in emergency department use among adults with intellectual and developmental disabilities.

BACKGROUND: Emergency Department (ED) visits are common among adults with intellectual and developmental disabilities (IDD). However, little is known about how ED use has varied over time in this population, or how it has been affected by recent Medicaid policy changes. OBJECTIVE: To examine temporal trends in ED use among adult Medicaid members with IDD in eight states that ranged in the extent to which they had implemented state-level Medicaid policy changes that might affect ED use. METHODS: We conducted repeated cross-sectional analyses of 2010-2016 Medicaid claims data. Quarterly analyses included adults ages 18-64 years with IDD (identified by diagnosis codes) who were continuously enrolled in Medicaid for the past 12 months. We assessed change in number of ED visits per 1000 member months from 2010 to 2016 overall and interacted with state level policy changes such as Medicaid expansion. RESULTS: States with no Medicaid expansion experienced an increase in ED visits (linear trend coefficient: 1.13, p < 0.01), while states operating expansion via waiver had a much smaller (non-significant) increase, and states with ACA-governed expansion had a decrease in ED visits (linear trend coefficient: 1.17, p < 0.01). Other policy changes had limited or no association with ED visits. CONCLUSIONS: Medicaid expansion was associated with modest reduction or limited increase in ED visits compared to no expansion. We found no consistent decrease in ED visits in association with other Medicaid policy changes.

Health Service Utilization Patterns Among Medicaid-Insured Adults With Intellectual and Developmental Disabilities: Implications for Access Needs in Outpatient Community-Based Medical Services.

Limited existing evidence suggests that adults with intellectual and developmental disabilities (IDD) experience substantial disparities in numerous areas of health care, including quality ambulatory care. A multistate cohort of adults with IDD was analyzed for patterns of inpatient admissions and emergency department utilization. Utilization was higher (inpatient [RR = 3.2], emergency department visits [RR = 2.6]) for adults with IDD, particularly for ambulatory care-sensitive conditions (eg, urinary tract [RR = 6.6] and respiratory infections [RRs = 5.5-24.7]), and psychiatric conditions (RRs = 5.8-15). Findings underscore the importance of access to ambulatory care skilled in IDD-related needs to recognize and treat ambulatory care-sensitive conditions and to manage chronic medical and mental health conditions.

In-hospital mortality among adults with autism spectrum disorder in the United States: A retrospective analysis of US hospital discharge data.

A retrospective data analysis using 2004-2014 Healthcare Cost and Utilization Project Nationwide Inpatient Sample was conducted to examine in-hospital mortality among adults with autism spectrum disorders in the United States compared to individuals in the general population. We modeled logistic regressions to compare inpatient hospital mortality between adults with autism spectrum disorders (n = 34,237) and age-matched and sex-matched controls (n = 102,711) in a 1:3 ratio. Adults with autism spectrum disorders had higher odds for inpatient hospital mortality than controls (odds ratio = 1.44, 95% confidence interval: 1.29-1.61, p < 0.001). This risk remained high even after adjustment for age, sex, race/ethnicity, income, number of comorbidities, epilepsy and psychiatric comorbidities, hospital bed size, hospital region, and hospitalization year (odds ratio = 1.51, 95% confidence interval: 1.33-1.72, p < 0.001). Adults with autism spectrum disorders who experienced in-hospital mortality had a higher risk for having 10 out of 27 observed Elixhauser-based medical comorbidities at the time of death, including psychoses, other neurological disorders, diabetes, hypothyroidism, rheumatoid arthritis collagen vascular disease, obesity, weight loss, fluid and electrolyte disorders, deficiency anemias, and paralysis. The results from the interaction of sex and autism spectrum disorders status suggest that women with autism spectrum disorders have almost two times higher odds for in-hospital mortality (odds ratio = 1.95, p < 0.001) than men with autism spectrum disorders. The results from the stratified analysis also showed that women with autism spectrum disorders had 3.17 times higher odds (95% confidence interval: 2.50-4.01, p < 0.001) of in-hospital mortality compared to women from the non-autism spectrum disorders matched control group; this difference persisted even after adjusting for socioeconomic, clinical, and hospital characteristics (odds ratio = 2.75, 95% confidence interval: 2.09-3.64, p < 0.001). Our findings underscore the need for more research to develop better strategies for healthcare and service delivery to people with autism spectrum disorders.

Disparities in diabetes management among medicaid recipients with intellectual and developmental disabilities (IDD): Evidence from five U.S. states.

BACKGROUND: Diabetes is one of the most common chronic conditions among adults. Little is known about the quality of diabetes care received by adults with intellectual and developmental disabilities (IDD). OBJECTIVE: To determine the extent to which the diabetes care needs are met for a population with both IDD and diabetes who are solely insured by Medicaid in five states (Iowa, Massachusetts, New York, Oregon and South Carolina). METHODS: Medicaid administrative data in 2012 were used to identify Medicaid members (excluding dual eligibles) with diabetes and IDD in five states. Four diabetes care measures were compared between members with and without IDD using bivariate analyses. For those with diabetes and IDD, a logistic regression model was fitted for each state with the following predictors: age, sex, IDD subgroup, and occurrence of a specialist visit in the current or past year. A meta-analysis was then conducted to synthesize cross-state results. RESULTS: Across the five states, 6229 (2%) of the 308,804 non-dual adult Medicaid members 18-64 years old with diabetes in 2012 also had IDD. Comparing those with IDD to their non-IDD peers on receipt of all four diabetes care measures showed differences by state, but state rates of overall adherence were very low, ranging from 16.6% to 28.5% of the population. CONCLUSIONS: Meta-analysis results identified specialist visits as a strong predictor of adults with diabetes and IDD receiving all four components of diabetes care. This important information should be considered in efforts to improve quality care for this population.

Assessing the association between mental health and disability indicators among adults living in the United States.

BACKGROUND: Over 40 million people in the U.S. experience mental illness and/or disabilities. However, there has been limited characterization of the relationship between mental health and disability in national surveys. OBJECTIVE: To examine associations between current indicators for psychological distress, serious mental illness, and disability in the National Health Interview Survey (NHIS). METHODS: Secondary analysis (univariate and multivariate log-binomial estimation of relative risk) of non-specific psychological distress, duration of serious mental illness, disability types, and demographic factors in adults from the NHIS. RESULTS: Strong associations between psychological distress, serious mental illness, and disability types were found. After demographic stratifications and adjustment, associations were strongest for cognitive disabilities, compared to other disability types. Adults reporting serious psychological distress or more than 10 years of serious mental illness were 19.7 (17.8, 21.7) and 11.0 (10.2, 12.0) times more likely to report cognitive disabilities, compared to adults reporting no psychological distress or serious mental illness. After sex and race/ethnicity adjustment, individuals ages 35-54 years reporting serious psychological distress or more than 10 years of serious mental illness were 8.6 (6.9, 10.6) and 3.7 (3.3, 4.2) times more likely to report cognitive disabilities, compared to individuals ages 35-54 years reporting no psychological distress or serious mental illness. CONCLUSIONS: There is a robust association between mental health and cognitive disabilities among adults. Our results suggest health agencies and public policy consider addressing the concurrent nature of mental illness and cognitive disabilities in services and programs for adults with disabilities.

Unlocking the Potential of State Level Data: Opportunities to Monitor Health and Related Outcomes in People With Intellectual and Developmental Disabilities.

No single U.S. health surveillance system adequately describes the health of people with intellectual and developmental disabilities (IDD). Researchers and policy makers have sought to understand the potential of state and local administrative and survey data to produce a local as well as a national picture of the health of the population with IDD. Analyses of these secondary data sources have significant appeal because of the potential to derive new information without the burden and expense of new data collection. The authors examined the potential for data collected by states and territories to inform health surveillance in the population with IDD, including data from the administration of eligibility-based supports, health insurance claims, and surveys administered for monitoring and quality improvement. Although there are opportunities to align and harmonize datasets to enhance the available information, there is no simple path to use state and local data to assess and report on the health of the population with IDD. Recommendations for policy, practice, and research include the development and use of consistent operational definitions in data collection, and research to fill knowledge gaps.

Using Medicaid Data to Characterize Persons With Intellectual and Developmental Disabilities in Five U.S. States.

This project sought to identify Medicaid members with intellectual and developmental disabilities (IDD) in five states (Delaware, Iowa, Massachusetts, New York, and South Carolina) to develop a cohort for subsequent analyses of medical conditions and service utilization. We estimated that over 300,000 Medicaid members in these states had IDD. All members with diagnostic codes for IDD were identified and the three most frequent diagnoses were unspecified intellectual disability, autism or pervasive developmental disorder, and cerebral palsy. The percentage of Medicaid members with IDD ranged from 2.3% in New York to 4.2% in South Carolina. Identifying and characterizing people with IDD is a first step that could guide public health promotion efforts for this population.

Characteristics associated with bone mineral density screening in adults with intellectual disabilities.

BACKGROUND: Certain health characteristics place adults with intellectual disability at increased risk for osteoporosis. However, little data exist to describe how comorbid disease or medications affect screening patterns for these patients. METHODS: We evaluated the relationship between bone density screening and the presence of risk factors using a secondary cross-sectional analysis of 5520 adults aged 19 years and older with the diagnosis of intellectual disability. RESULTS: Of the sample, 22.9% received one or more bone density screenings (34.4% women, 13.3% men). Low screening rates in men prohibited the construction of a valid sex-specific multivariate model of the association between bone density screening and risk factors for osteoporosis. In women, when controlling for age the following factors were significantly associated with ever having bone density screening: use of antiepileptic medication (odds ratio [OR], 1.5) and vitamin D (OR, 3.4); recent receipt of a flu vaccine (OR, 1.4); and living in a 24-hour supported residential setting (OR, 1.3). A diagnosis of Down syndrome (OR, 0.72) was associated with decreased likelihood of screening. CONCLUSIONS: Many known risk factors for osteoporosis affected the likelihood of an adult with intellectual disability receiving screening, yet overall screening rates for adults with intellectual disabilities were lower than screening rates in the general population. Results suggest a need for increased provider awareness about bone density screenings in high-risk adults with intellectual disability, especially men, as well as men and women with Down syndrome.

Evaluating representativeness and cancer screening outcomes in a state department of developmental services database.

Though it is widely recognized that people with intellectual and developmental disabilities (IDD) face significant health disparities, the comprehensive data sets needed for population-level health surveillance of people with IDD are lacking. This paucity of data makes it difficult to track and accurately describe health differences, improvements, and changes in access. Many states maintain administrative health databases that, to date, have not been widely used for research purposes. In order to evaluate the feasibility of using administrative databases for research purposes, the authors attempted to validate Massachusetts' administrative health database by comparing it to a large safety net hospital system's patient data regarding cancer screening, and to the state's service enrollment tables. The authors found variable representativeness overall; the sub-population of adults who live in 24-hr supported residences were better represented than adults who live independently or with family members. They also found a fairly low false negative rate for cancer screening data as compared with the "gold standard" of hospital records. Despite some limitations, these results suggest that state-level administrative databases may represent an exciting new avenue for health research. These results should lend context to efforts to study cancer and health screening variables using administrative databases. The present study methods may also have utility to researchers in other states for critically evaluating other state IDD service databases. This type of evaluation can assist researchers in contextualizing their data, and in tailoring their research questions to the abilities and limitations of this kind of database.