Perceived Stigma in Patients with Autoimmune Hepatitis.

Perceived stigma (PS) adversely impacts psychosocial and disease outcomes in patients with chronic liver diseases (CLD), and those with autoimmune hepatitis (AIH) may be at risk for PS given inaccurate assumptions about the origin of their diagnosis. The aims of the current study are to describe the frequency of PS in patients with AIH, compare rates of PS in AIH to rates of PS in primary biliary cholangitis (PBC) and CLD, and examine demographic correlates of PS. 262 adults with AIH (95% female, Mage = 51.53 years) completed online questionnaires on demographics, disease information, and PS. 54-68% reported PS with themes of selective disclosure, non-disclosure, or hiding diagnosis. PS was higher in those with AIH compared to those with PBC, but lower than those with various CLD. Age was inversely related to PS. Given the results, provider screening of PS and integration of clinical health psychologists may be helpful for identifying PS in patients with AIH.

A narrative review of the literature on illness uncertainty in hypermobile ehlers-danlos syndrome: implications for research and clinical practice.

BACKGROUND: Hypermobile Ehlers-Danlos syndrome (hEDS) is characterized by joint and skin laxity, and often accompanied by chronic pain, dysautonomia, increased distress and, functional limitations. The journey to accurate diagnosis is often prolonged due to unclear etiology of symptoms. This manuscript is a narrative review of the literature on illness uncertainty (IU) in hEDS, highlighting the unique facets of IU in this population, as compared to the broader chronic pain population (given symptom overlap between these two disease groups), that warrant additional investigation. Additionally, we considered the unique challenges associated with IU in the context of the developmental nuances of pediatric populations. Specifically, we aimed to (1) map the extant literature of the IU experience in chronic pain conditions broadly including the pediatric and adult research to identify key concepts related to IU and incorporate potential developmental considerations in IU; (2) delineate and describe the IU experience specifically in patients with hEDS, with the goal of identifying gaps in the literature based on aspects of presentation in hEDS that do and do not differ from the broader chronic pain population; and (3) elucidate the potential areas of adverse impact of IU in both general chronic pain populations, and those with hEDS specifically, to provide actionable areas for future research and clinical care of individuals with hEDS. Results of this review indicate that IU has been well-studied in chronic pain generally, but inadequately evaluated in hEDS specifically. Specific features of hEDS (complexity of the disorder, involvement of multiple bodily systems, contribution of organic pathology) may uniquely contribute to IU in this population. This review suggests that ambiguities surrounding the diagnosis of hEDS, symptom course, and treatment recommendations, along with misdiagnosis, perceived dismissal of symptoms, or attribution of symptoms to mental health concerns might increase risk for IU and related distress in patients. CONCLUSION: Findings from the present review suggest that distinct features of hEDS yield a set of driving factors for IU that may be somewhat different than those faced by patients with chronic pain or other medical conditions. The development of a validated measure of IU to appropriately assess this construct in patients with hEDS is a research priority. In the clinical setting, providers should be attentive to the potentially aversive diagnostic and treatment experiences reported by patients and attempt to provide clear explanations based on the extant knowledge of hEDS, and implement best-practice recommendations for multidisciplinary treatment.

Psychological interventions for individuals with Ehlers-Danlos syndrome and hypermobility spectrum disorder: a scoping review.

PURPOSE: To identify the nature and extent of the evidence on psychological interventions among individuals with Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD). MATERIALS AND METHODS: Eligible studies reported on psychological interventions for individuals of all ages with EDS and/or HSD. All studies published in English were included, with no restrictions to publication year or status. MEDLINE, CINAHL, EMBASE, and PsycINFO were searched. Two reviewers independently screened studies and abstracted data. RESULTS: This scoping review included 10 studies reporting on EDS, HSD, or both. Only cohort studies and case studies were identified. Four studies investigated Cognitive Behavioural Therapy (CBT), one investigated Dialectical Behavioural Therapy (DBT), two investigated psychoeducation, two investigated Intensive Interdisciplinary Pain Treatment (IIPT), and one investigated Acceptance Commitment Therapy (ACT). Interventions targeted pain management, self-destructive behaviours, and related psychological issues (e.g., depression/anxiety). Sample sizes were small (n < 50) for most studies and interventions were generally poorly described. CONCLUSIONS: There is a critical need for high-quality research surrounding psychological interventions for individuals with EDS/HSD. Psychological interventions for these individuals are understudied and existing studies lack validity. Researchers should investigate psychological interventions for individuals with all types of EDS/HSD with high-quality studies to validate findings from the existing studies.

Median arcuate ligament syndrome: a cost analysis to determine the economic burden of a rarely diagnosed disease.

Background: Chronic abdominal pain (CAP) is a medical condition resulting in enormous economic burden and healthcare utilization costs. One understudied source of CAP is the median arcuate ligament syndrome (MALS). MALS is often not diagnosed and treated for a variety of reasons, including the fact that MALS is highly comorbid with psychological symptoms and psychiatric disorders similar to CAP. To better inform future work on the study of MALS, we undertook a pilot study to estimate the economic impact and public health burden of this condition. We hypothesized that MALS imposes a significant public health burden. Methods: Pediatric and adult patients enrolled in a prospective study undergoing multidisciplinary evaluation and treatment for MALS at a tertiary care facility were invited to participate in a brief self-report survey, the Direct and Indirect Medical Care Impact of MALS Form, to capture health care resources including procedures, surgeries, health care visits, and absenteeism (school and work). To estimate costs from the Direct and Indirect Medical Care Impact of MALS Form, the medical care usage data self-reported by patients were converted to dollar value utilizing FSC-93 billing data and corresponding current procedural terminology (CPT) codes for procedures and provider visits one year prior to surgery and then following surgery. Descriptive analyses were conducted to characterize the sample in terms of demographics and reported absences from school and work. Results: One hundred and nineteen patients (mean age = 30.9 ± 13.0) completed the questionnaires, yielding a 57% response rate. 82.4% (n = 98) of the participants were female and 90.8% (n = 108) were non-Hispanic/Latine white. The mean and median surgical follow-up periods were 5.3 and 5.4 years, respectively. Overall, median cost of provider and ancillary healthcare provider visits for each patient was (US)$19,119 including the pre-operative and post-operative visits. The mean cost for providers alone was (US)$28,908. Wilcoxon signed-ranks tests indicated that the postoperative missed number of days of school were significantly lower than the pre-surgical number of missed school days (Z = -3.36, p = 0.001). Similarly, there were significantly less missed work-days following surgery than before for the entire sample (Z = -2.86, p = 0.004). Conclusion: Median arcuate ligament syndrome imposes a large economic burden on patients and the healthcare system. The current findings, although reflective of a homogenous population, are adding to a growing body of literature suggesting that healthcare disparities play a role in the low rates of diagnosis and treatment of MALS.

Relationships Between Abdominal Pain, Mental Health, and Functional Disability in Youth With Inflammatory Bowel Diseases: Pain Catastrophizing as a Longitudinal Mediator.

OBJECTIVES: Abdominal pain can be a debilitating symptom for youth with inflammatory bowel diseases (IBDs). Across various pediatric conditions, pain predicts adverse physical and mental health outcomes. Understanding mechanisms by which pain impacts outcomes is of critical importance to enhance the well-being of those with IBDs. Pain catastrophizing mediates the aforementioned relationships in other pediatric populations, but little research has examined its role in pediatric IBDs. Attention to the role of pain catastrophizing as a potential mediator in pediatric IBD is the important given unique elements of the pain experience for this population. This study aimed to examine pain catastrophizing as a potential mediator of the relationship between abdominal pain and adverse outcomes in youth with IBDs. METHODS: Seventy six youth (ages 11 to 18; M [SD] age=14.71 [1.80]) with IBD completed the Abdominal Pain Index (T1), Pain Catastrophizing Scale (T2, week 12), Revised Children's Anxiety and Depression Scale (T3, week 20), and Functional Disability Inventory (T3, week 20). RESULTS: Catastrophizing mediated the relationship between abdominal pain and both anxiety symptoms ( b =1.61, Bias-Corrected Accelerated [BCa] CI, 0.25, 4.62) and functional disability ( b =0.77, BCa CI, 0.15, 2.38). A direct effect of abdominal pain on low mood was also noted ( b =1.17, BCa CI, 0.03, 2.50). Post hoc analyses examining mediation via catastrophizing subscales indicated that while magnification and rumination functioned as mediators, helplessness did not. DISCUSSION: Findings are consistent with fear avoidance models and suggest that interventions directed at pain catastrophizing may be worthwhile in pediatric IBD populations, given catastrophizing-mediated relationships between pain and mental and physical health outcomes.

Targeting Coping to Improve Surgical Outcomes in Pediatric Patients With Median Arcuate Ligament Syndrome: Feasibility Study.

Background: Median arcuate ligament syndrome (MALS) is a vascular compression syndrome leading to postprandial epigastric pain, nausea, and weight loss; it can be treated surgically. While most patients report improved quality of life following surgical intervention, 30% continue to experience chronic abdominal pain. Pre-surgical diagnoses of depression and/or anxiety have been found to significantly predict post-surgical: quality of life, highest experience of pain, anxiety, and parent- and self-reported coping strategies. As such, increasing the coping strategies of pediatric patients with MALS may impact their post-surgical outcomes. The purpose of the current study was to: (1) implement a pre-operative cognitive behavioral therapy protocol with a focus on psychoeducation and coping strategies; and (2) determine feasibility of a pre-surgical intervention for this population. Method: Children (<18 years of age) with a diagnosis of MALS who were eligible for surgical intervention were invited to participate in a 7-week in-person or video-based pre-surgical cognitive behavioral therapy intervention. Psychiatric comorbidities were assessed at baseline and post-surgery; patient-reported distress, pain interference and intensity, health-related quality of life, and health status were assessed at four time points (baseline, week 4, week 7, and post-surgery). Descriptive analyses were used to characterize the sample, assess feasibility outcomes (i.e., attrition rates), and explore symptom-based outcomes across time. Results: Twelve pediatric patients (M age = 15.2 ± 1.7; 91.7% female) and their parents (91.7% mothers) participated. Feasibility metrics based on protocol completion were exceeded for engagement at the stages of consent (68.4% vs. goal of ≥50%), treatment initiation (92.3% vs. 85%), and treatment completion (84.6% vs. 75%). Out of the 12 participants, nine (75%) met criteria for at least one comorbid psychiatric diagnosis at baseline and nine (75%) elected to undergo MALS surgery after completing the intervention. Conclusion: The intervention implementation was feasible, despite chronic pain symptoms experienced by the sample, a high prevalence of psychiatric diagnoses, and an international pandemic, suggesting that it would be beneficial to further evaluate the efficacy of the intervention. Future research should include stakeholder input in the design, deployment, and evaluation of a pilot efficacy trial of pre-surgical cognitive behavioral therapy for pediatric patients with MALS.

Caregiver perceptions of importance of COVID-19 preventative health guidelines and difficulty following guidelines are associated with child adherence rates.

OBJECTIVE: To describe child adherence to four preventative-health guidelines during the COVID-19 pandemic and investigate caregiver-level correlates of adherence. METHOD: Two hundred thirty-six caregivers (75% female) of children ages 6-12 years (Mage = 8 years; 53% male) living in the United States rated child adherence to four preventative-health guidelines between 10/16/2020 and 11/14/2020. Caregivers also rated perceived importance of each guideline in limiting virus spread and perceived difficulty in obtaining child compliance. RESULTS: Child adherence was highest for mask-wearing (median [Mdn] = 96%) and hand hygiene (Mdn = 95%). Adherence to social distancing guidelines was lower. Mdn adherence for social distancing with family outside the household was 80%, and Mdn adherence for social distancing with friends was 72%. Furthermore, for each of the four guidelines, fewer than half the sample reported 100% adherence (range = 20%-43%). Adherence was positively associated with caregiver perceptions of importance of a given behavior in limiting virus spread (rs = .38-.62) and negatively associated with perceived difficulty in gaining child compliance (rs = -.37-.25). DISCUSSION: Current results indicated parents perceive child adherence to social distancing more challenging than child adherence to mask wearing or hand hygiene. Lower caregiver perceptions of importance of the behavior and greater perceived difficulty in gaining child compliance were associated with lower adherence to all tasks. Brief targeted interventions (e.g., motivational interviewing, parent behavior management training) may be well suited to enhance caregivers' perceptions of value of the behavior, while also addressing behavioral challenges that interfere with child adherence. (PsycInfo Database Record (c) 2021 APA, all rights reserved).

Emerging Adults' Adherence to Preventative Health Guidelines in Response to COVID-19.

OBJECTIVE: To examine rates of emerging adults' (EA) adherence to preventative health behavior recommendations during early months of the COVID-19 pandemic and to investigate demographic (i.e., gender, years of education, socioeconomic status, school enrollment status, and living situation) and exposure and impact-related correlates of adherence. METHODS: Participants were 273 [M (SD) age = 22 (2.1) years, 55% female, 32% from minoritized groups] EA completed an online survey of adherence to 11 preventative health behaviors recommended by the Centers for Disease Control (CDC) during summer 2020. Participants rated adherence via a visual analog scale. Participants also reported demographic information and completed the COVID-19 Exposure and Family Impact Adolescent and Young Adult Version (CEFIS-AYA). RESULTS: Median levels of adherence to preventative recommendations ranged from 66% to 100%. Highest adherence levels (Mdn > 90%) were reported for quarantining if exposed to COVID-19; covering mouth when sneezing; avoiding the elderly/those at high risk; and avoiding large gatherings. Median adherence was <80% for mask wearing; maintaining a 6-foot distance; avoiding in-person visits with romantic partners or friends; and disinfecting surfaces. Female gender was the only variable significantly associated with overall adherence, and it explained 4% of the variance. CONCLUSIONS: Following guidelines related to social distancing practices may be particularly challenging for EA, possibly because of unique developmental needs of this group, and males may be at greater risk for non-adherence to CDC recommendations. Therefore, public health messaging and adherence intervention development should be designed with males and social distancing practices in mind.

Communication About Medication by Providers-Adolescent and Young Adult Version: Confirmatory Factor Analyses.

OBJECTIVE: To replicate the factor structure of a patient-report measure of provider communication about key medication prescription information, the Communication about Medication by Providers-Adolescent and Young Adult (CAMP-AYA) Version. We evaluated whether the 15-item, two-factor structure identified previously could be replicated via confirmatory factor analysis, and we also examined fit of unidimensional and bifactor models. Associations of CAMP-AYA Total and Factor Scores with provider satisfaction and select patient and medication characteristics were also examined. METHODS: Participants were 739 AYA (ages 18-25) who completed the CAMP-AYA, a provider satisfaction rating, and provided demographic and medication information. RESULTS: The bifactor model was best fitting (χ2 [75] = 689.60, p < .0001; root mean squared error of approximation = 0.11, 90% CI [0.10, 0.11]; Comparative Fit Index = 0.98; Tucker-Lewis Index = 0.98; Standardized Root Mean Square Residual Index = 0.02). Internal consistency reliabilities for Total and Factor Scores were high (αs > .89) and Total and Factor Scores were associated with provider satisfaction (ps < .001). CAMP-AYA scores varied as a function of type of prescription (short vs. long term; new vs. refill), with higher scores reported in the context of long term (>30-day course) or refilled prescriptions (ps < .007) in most cases. CONCLUSIONS: This study provides additional support for the reliability of the CAMP-AYA as a tool to assess AYA perceptions of provider key information coverage about medication prescriptions.

Family Support is Associated with Fewer Adherence Barriers and Greater Intent to Adhere to Oral Medications in Pediatric IBD.

PURPOSE: Medication nonadherence is common among adolescents, and family support can enhance adherence. In a sample of youth with inflammatory bowel disease (IBD), we aimed to describe barriers to oral medication adherence and intent to adhere. Additionally, we examined the role of family support in influencing adherence barriers and intent to adhere, and assessed if age moderated these relationships. DESIGNS AND METHODS: A secondary analysis of baseline data from an adherence-promotion intervention trial was conducted. Youth reported perceptions of family support, adherence barriers, and intent to adhere via validated measures. RESULTS: Among youth (N = 76, ages 11-18), age moderated the impact of family support on total adherence barriers, disease/regimen barriers, social support/self-efficacy barriers, and intent to adhere. At higher levels of family support, younger (Mage = 12.93 years) and mid-age (Mage = 14.68 years) youth reported fewer barriers to adherence and greater intent to adhere. No main effect of family support or evidence of age moderation was found for denial/distrust or peer influences barrier domains. CONCLUSIONS: Younger and mid-aged adolescents benefitted from family support in the context of IBD management. Future research should examine whether interventions to enhance family support may enhance youth adherence. PRACTICAL IMPLICATIONS: Given prior findings highlighting the success of nurse-led interventions in promoting adherence in patients with IBD, present findings suggest additional intervention points for nurses working with pediatric IBD populations.

Pain Symptomatology and Management in Pediatric Ehlers-Danlos Syndrome: A Review.

Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders that manifest with hyperextensibility of joints and skin, and general tissue fragility. While not a major criterion for clinical diagnosis, pain is a frequently endorsed symptom across subtypes of EDS. As such, the present review aims to summarize research to date on pain characteristics and management, and the relationship between such pain symptomatology and quality of life in pediatric EDS. Characteristics of pain, including theorized etiology, relative intensity and extent of pain are described, as well as descriptions of frequently endorsed pain sites (musculoskeletal, and non-musculoskeletal). Interventions related to the management of musculoskeletal (e.g., pharmaceutical intervention, physical therapy) and non-musculoskeletal pain (e.g., pharmaceutical and psychological interventions) are discussed, highlighting the need for additional research related to pediatric pain management in the context of hypermobility syndromes. In addition, the relationship between pain in pediatric EDS and quality of life is described. Finally, limitations of literature to date are described and recommendations for future lines of research are outlined.

Using pupil dilation, eye-blink rate, and the value of mother to investigate reward learning mechanisms in infancy.

The brain is adapted to learn from interactions with the environment that predict or enable the procurement of rewards (Schultz, 2010). For infants, the main caregiver (often the mother) is most associated with primary biological rewards such as food and warmth, as well as the most likely provider of emotional and social rewards such as comfort and responsiveness. In this study we capitalize on the reward value of mother to examine reward learning mechanisms in infancy using multiple eye-tracking measures. Converging lines of research have demonstrated links between reward-related striatal dopamine activity and measurable changes in spontaneous eye-blink rate (EBR) and pupil dilation (Eckstein et al., 2017). We presented 7-month-old infants with video stimuli that parametrically increased in social-emotional value (male stranger, female stranger, mother) or in visual attention value (static image, slowed silent cartoon, dynamic cartoon). After establishing infants' baseline responses to these stimuli, we paired the videos with arbitrary shape cues in an associative learning task. Infants showed superior learning from their own mother's video and a heightened anticipatory arousal response to the mother-associated cue following learning. Both learning measures were predicted by infants' baseline EBR to their mother's video, providing the first evidence of reward learning and transfer in human infants.