Mental health in paediatric and adult myositis-related diseases: current state of research, interventions, and future steps from the MIHRA Psychological Impact Scientific Working Group.

Psychological and emotional well-being are critical aspects of overall health for individuals with chronic rheumatologic conditions. Mental health-related literature, however, predominantly focuses on systemic lupus erythematosus or rheumatoid arthritis, with limited emphasis on idiopathic inflammatory myopathies (IIMs). High proportions of those with juvenile myositis report psychological distress at levels warranting mental health referral. Adults with dermatomyositis diagnosed with depression or anxiety do not receive adequate mental health care. Mental health symptoms in those with IIMs are associated with worse health-related quality of life, medication adherence, and disease outcomes. Despite demonstrated high rates of mental health burden, access to mental health care remains severely lacking.Data related to mental health burden is limited by small sample size, limited generalisability, variable methods of assessment, and inconsistent diagnosis codes to define mental health conditions. Additional research is needed to validate current screening tools in myositis populations. Other relevant measurable factors include disease severity, non-health- and health-related trauma exposure, loneliness, isolation, loss of control, sleep difficulties, fatigue, pain, self-esteem, body image, sexual health, and health inequities. Studiesare needed investigating the efficacy of therapeutic and pharmacologic interventions among patients with myositis who experience depression and anxiety. Currently, knowledge and resources are limited around mental health burden and potential intervention for those living with IIMs. The Myositis International Health & Research Collaborative Alliance (MIHRA) Psychological Impact Scientific Working Group offers a preliminary road map to characterise and prioritise the work ahead to understand baseline mental health burden and compare avenues for intervention.

Notes from the Field: Results from the Parent Acceptance of Pediatric Integrated Care Survey.

OBJECTIVE: To collect data and gain an understanding of parental satisfaction with and attitudes toward treatment in a pediatric integrated primary care (IPC) model. DATA SOURCES: Primary data were collected across the United States over the course of a few months. STUDY DESIGN: The 35-item, Parent Acceptance of Pediatric Integrated Care Survey (PAPICS) was developed by a panel of IPC experts. The survey was then distributed through the Qualtrics Panels Service with recruited participants (i.e., parents with children under 18-years-old) receiving a $4.00 incentive for their involvement. DATA COLLECTION/EXTRACTION METHODS: A single exploratory factor analysis was performed along with four factor retention tests and clinical judgment to guide factor selection. A 5-factor structure was selected. PRINCIPAL FINDINGS: Parents reported a high level of comfort with an IPC model and a favorable attitude toward child therapy, with some concerns regarding psychological stigma and privacy. Notable variation in parents' beliefs regarding one-on-one psychological service delivery were observed. CONCLUSIONS: Results provided evidence for parental openness to an IPC model. Findings also highlighted potential fundamental misunderstandings regarding evidence-based psychological treatment methods for children and adolescents.

Altered functional network connectivity and working memory dysfunction in adolescents with epilepsy.

Adolescents with epilepsy are at risk for deficits in working memory, which could lead to learning difficulties and poor academic outcomes. We used task-based functional magnetic resonance imaging (fMRI) to examine potential disruption in working memory function in adolescents with epilepsy as compared to healthy controls. We recruited 29 adolescents (13-17 years) with non-lesional epilepsy and 20 healthy controls. All participants performed an N-back fMRI task and neuropsychological testing. A general linear model approach was used to create group activation maps of N-back fMRI for epilepsy and control groups and both groups combined (p < 0.05 corrected for multiple comparisons). Functionally defined regions of interest (ROIs) were identified based on clusters of combined group activation (z > 5). Subsets of these functional ROIs corresponding to cingulo-opercular and fronto-parietal networks were selected and differences in functional connectivity between the epilepsy and control groups were assessed. Adolescents with epilepsy had significantly poorer working memory scores compared to controls. For the N-back fMRI task, there were no significant differences in group activation. Functional connectivity was significantly reduced between the left frontal operculum and anterior cingulate gyrus (cingulo-opercular network) in adolescents with epilepsy compared to controls. This study demonstrates working memory deficits and an altered pattern of connectivity in brain networks supporting working memory in adolescents with epilepsy. Working memory performance was worse in adolescents with epilepsy; though scores were not directly correlated with reduced connectivity. These results suggest the neural substrates of working memory in adolescents with epilepsy may differ from those in typically-developing adolescents, and require further investigation to understand the reasons for poorer working memory performance.

Pilot Executive Functioning Intervention in Epilepsy: Behavioral and Quality of Life Outcomes.

OBJECTIVE: To examine changes in emotional and behavioral functioning and health-related quality of life (HRQOL) following a web-based executive functioning (EF) intervention open pilot trial (e.g., Epilepsy Journey) for adolescents with epilepsy. METHODS: Adolescents with an established diagnosis of epilepsy, EF deficits, and without developmental disorders participated in a single-arm trial of Epilepsy Journey. Epilepsy Journey is a gamified, online learning environment comprised of 10 learning modules targeting EF deficits (e.g., working memory, organization) and tailored to epilepsy with accompanying telehealth problem-solving sessions. Adolescents completed questionnaires assessing emotional and behavioral functioning and HRQOL at baseline, posttreatment, and 2 follow-ups . Longitudinal mixed models and logistic regression analyses were used for these secondary analyses. RESULTS: 39 adolescents were recruited for Epilepsy Journey (Mage=15.3 years; 67% female; 87% White: Non-Hispanic; 39% experienced seizures in the past 3 months). Preliminary data indicate significant improvements in caregiver-reported Externalizing symptoms, Behavioral Symptom Index scores and Adaptive Skills from baseline to 5-month follow-up. Significant improvements were observed for caregiver-reported Mood/Behavior and self-reported Impact, Cognitive Functioning, Executive Functioning, and Sleep subscales of the PedsQL Epilepsy Module. Clinically significant improvements (e.g., clinical/at-risk to normative levels) in behavioral and quality of life domains were also noted. CONCLUSION: Epilepsy Journey appears to contribute to changes in emotional and behavioral functioning and HRQOL in adolescents with epilepsy. Given the proof of concept trial format of this study, an important future direction is to conduct a randomized controlled trial with a larger, generalizable cohort of adolescents with epilepsy.

Epilepsy Journey: A proof of concept trial of a Web-based executive functioning intervention for adolescents with epilepsy.

OBJECTIVE: To examine the preliminary feasibility and acceptability of a Web-based program, Epilepsy Journey, to improve executive function behaviors in adolescents with epilepsy. METHODS: We conducted a proof of concept single-arm pilot trial of Epilepsy Journey with 31 adolescents (average age = 15.3 ± 1.3 years) who had an epilepsy diagnosis and executive function (EF) deficits on the caregiver-report version of the Behavior Rating Inventory of Executive Function (BRIEF). Epilepsy Journey coupled a gamified problem-solving website comprised of 10 learning modules targeting EF deficits (eg, working memory, organization, problem-solving) with Skype sessions with a trained therapist. Outcomes included feasibility (attrition, sessions completed) and acceptability (satisfaction ratings). Exploratory analyses examined changes in caregiver-, self-, and teacher-reported BRIEF scores from baseline to posttreatment and at 2- and 5-month follow-ups. RESULTS: Seventy-nine percent of participants completed the program. Satisfaction was high, with 97% of caregivers and adolescents rating the program as helpful and indicating they would recommend it to others. Caregivers and adolescents reported global improvements on the BRIEF, with caregivers reporting significant improvements on all BRIEF subscales. EF symptoms rebounded slightly between the 2- and 5-month follow-ups for some of the self- and caregiver-reported BRIEF scales. Notably, clinically meaningful improvements (eg, clinical/subclinical to normative levels) were reported for several caregiver-reported BRIEF subscales, including the Global Executive Composite (62% to 33-34%) and Metacognitive Index (74% to 41-42%) from baseline to 2- and 5-month follow-up. SIGNIFICANCE: Findings suggest that a Web-based problem-solving intervention tailored to EF deficits for adolescents with epilepsy is both feasible and acceptable and may contribute to improvements in EF behaviors across domains.

Pattern of executive functioning in adolescents with epilepsy: A multimethod measurement approach.

Youth with epilepsy demonstrate deficits in executive functioning (EF), the skills necessary for goal-directed behavior (e.g., problem-solving, initiating, monitoring, organization, planning, and working memory). Despite 30-50% of youth with epilepsy demonstrating EF deficits, no extant studies have utilized both performance and questionnaire-based measures to examine the pattern of EF deficits in adolescents with epilepsy. Study aims were to 1) identify the pattern of EF deficits in adolescents with epilepsy and 2) identify which assessment tools are most sensitive to EF deficits in this population (adolescents, ages 13-17, with epilepsy). An exploratory aim was to examine group differences on measures of EF by epilepsy type. Standard performance-based neuropsychological measures (Wechsler Intelligence Scale for Children - Version V or Wechsler Adult Intelligence Scale Working Memory Index-Version IV, Delis Kaplan Executive Functioning System, NIH Toolbox, Test of Everyday Attention for Children) and the Behavior Rating Inventory of Executive Functioning (BRIEF) comprised the multimethod assessment battery. Depending on the measure, 30% of adolescents with epilepsy had deficits in working memory, 17% in cognitive flexibility/problem solving, 6% in inhibition, and 18% in planning/organization. Attention was a significant problem for 15% of adolescents with epilepsy. Correlations among the various EF measures were quite poor. Across various EF domains, results indicated that adolescents with localization-related epilepsy demonstrated better EF skills compared to adolescents with unclassified epilepsy. Overall, our findings suggest that executive functioning deficits are selective and different from those observed in other neurological populations (e.g., attention deficit hyperactivity disorder (ADHD), traumatic brain injury) where problems with self-regulation (i.e., inhibition, planning/organization) are more pronounced. These findings support utilizing multiple measures, including both performance-based neuropsychological tests and parent- and self-reports, to assess executive functioning difficulties in adolescents with epilepsy as they are uniquely sensitive to executive functioning domains. Adolescents with unclassified epilepsy also appear to be at higher risk for EF deficits and thus represent an important group to target for intervention.

Predicting Health Care Utilization and Charges Using a Risk Score for Poor Adherence in Pediatric Kidney Transplant Recipients.

Pediatric kidney transplant recipients must follow a complicated regimen of timely adherence to immunosuppressant medication, routine blood work, and medical follow-up visits. Failure to adhere to the recommended regimen can result in medical complications and costly treatment. We developed a novel risk score to identify patients at risk for poor adherence behaviors and evaluated whether it would predict future health care utilization and charges. Our risk stratification score combined three simple pass/fail metrics of adherence derived directly from the electronic health record including standard deviation of immunosuppression drug levels, timely laboratory monitoring, and timely clinic visits as indicated by our clinical protocol. Risk for poor adherence was assessed over a three-month period. Linear regression was used to predict subsequent health care charges and utilization. Greater than 75% of patients had some degree of nonadherence risk during the study period, but there were no significant differences found on any outcomes for the overall score. However, when the individual components of the overall risk score were evaluated independently, patients with tacrolimus drug level standard deviation ≥2 (e.g., a marker of poor adherence) had greater health care utilization (e.g., hospitalizations) and increased total charges. Additionally, patients who did not follow up in clinic at least every 4 months had more ED visits and ED-related charges, but fewer hospitalizations. Regular clinic visits and minimizing drug level variation may deter future costly ED visits and hospitalizations.

Development of a web-based executive functioning intervention for adolescents with epilepsy: The Epilepsy Journey.

INTRODUCTION: Youth with epilepsy exhibit significant deficits in executive functioning (EF), yet there are few interventions to improve EF for adolescents. The aims of the current study were to develop an individually-tailored intervention, called Epilepsy Journey, to improve aspects of EF through an iterative, patient-centered process including focus groups and usability testing. METHODS: Five adolescents and caregivers participated in focus groups. This input was used to develop ten learning modules based on subscales of the Behavioral Rating Inventory of Executive Functions and key issues that may impact EF in adolescents. Six adolescents participated in usability testing and a usability expert conducted a heuristic evaluation. Demographic information, chart reviews and measures of EF were also completed. RESULTS: Focus group participants and their parents reported difficulties with memory, attention, organization, monitoring, initiation, impulsivity, emotional control, sleep, awareness in schools and managing stress. They also identified successful strategies to address memory and organizational difficulties. Usability testing of the resultant Epilepsy Journey modules revealed problems with navigation and identified features that promoted usability, including progress bars and interactive modules. Program modifications were made after each usability trial resulting in a relatively brief, interactive and readily navigable program. Perceived utility was high with all but one participant. Participants rated the content as helpful and indicated they would recommend Epilepsy Journey to others. CONCLUSIONS: Feedback from the focus group and usability testing yielded a feasible, acceptable, relevant and user-friendly web-based intervention for adolescents with epilepsy. The Epilepsy Journey program will be further tested in an open pilot with adolescents with epilepsy and associated EF deficits.

The formative design of Epilepsy Journey: A web-based executive functioning intervention for adolescents with epilepsy.

This case study discusses the design, development, and formative evaluation of Epilepsy Journey, an individually-tailored, web-based intervention designed to address the unique executive functioning (EF) needs of adolescents with epilepsy. This intervention was designed through a three-phase iterative, patient-centered participatory action research process. First, a front-end analysis was completed to identify the unique needs of adolescents with epilepsy and initial design ideas via focus groups. Second, a preliminary design of the intervention was developed from focus group results. Finally, we iteratively incorporated revisions and refinements based on patient-centered feedback collected during usability sessions. Findings based on usage analytics, user reports, and coded qualitative themes from usability sessions suggest that our iterative, patient-centered approach to design, development, and evaluation resulted in a visually appealing interactive and brief intervention that is both engaging and individually tailored to the needs of adolescents with epilepsy.