Kinesiophobia—an excessive, often debilitating fear of movement or exercise—has emerged as an important moderator of physical activity (PA) and has been linked with quality of life (QoL), anxiety, and depression in adolescents with heart disease (HD). This study explores additional factors that may be related to cardiac-focused kinesiophobia, including self-efficacy, PA engagement, and QoL. Sixty-three adolescents (mean age = 15.5 years; 49

Objectives:This study explored potential resilience factors that facilitate engagement in physical activity (PA) among adolescents with chronic musculoskeletal pain after completing intensive interdisciplinary pain treatment. Methods:Ten adolescents aged 13 to 17 years with a history of chronic musculoskeletal pain from a mid-western pediatric hospital in the United States participated in this qualitative study. Participants provided demographic information and completed semistructured virtual interviews. The interviews focused on their pain experiences, resilience, and engagement in PA. Transcripts were analyzed using primarily directed content analysis based on a pain resilience framework. Results:Participants highlighted several key resilience factors that influenced their PA engagement, including positive affect, motivation, acceptance, and self-efficacy. Positive affect and mood affected their willingness and enjoyment of PA. Motivation was enhanced by focusing on short-term and long-term gains and setting smaller, manageable goals. Acceptance of pain allowed participants to engage in PA even on bad pain days, using psychological flexibility skills. Social support and the ability to pair PA with other valued activities, such as spending time with friends and family, were also crucial in maintaining PA engagement. Conclusion:The study identified resilience factors relevant to PA interventions for pediatric chronic pain. Incorporating strategies that enhance positive affect, motivation, acceptance, and self-efficacy, along with fostering social support, may improve PA engagement and could enhance the effectiveness of PA programs, promoting better outcomes for adolescents with pain. Future research should explore and further validate the importance of these resilience factors in larger, more diverse populations and across different stages of pain treatment.

Emerging work characterizing youth with chronic pain increasingly recognizes a large cohort of youth with co-occurring chronic pain and autism. This development has prompted questions about how to adapt Intensive Interdisciplinary Pediatric Pain Treatment (IIPTs) and the group-based treatments commonly used in these settings to improve accessibility, acceptability, and utility for autistic participants. There is a need for clinically oriented literature that IIPT programs and clinical trialists can use to guide adaptation efforts. Given long-term risks of inadequately treated pediatric pain, we argue it is clinically and ethically important to identify reasonable autism-informed adjustments within existing IIPT frameworks, even as more empirical work unfolds to inform nuance. In this narrative review, we synthesize evidence from pediatric pain psychology and autism intervention literatures to identify overlapping mechanisms and opportunities for adaptation, with a specific focus on group-based CBT/ACT-oriented treatments delivered in IIPTs. We summarize emerging clinical characteristics of autistic adolescents enrolled in IIPTs, bridge autism and pediatric pain group treatment literature, map that literature onto pediatric pain targets and autism-informed IIPT group design considerations and provide practical examples of IIPT group modifications extended from the existing data and the authors’ clinical experience delivering group-based pain psychology services to autistic youth in IIPTs. We also highlight constraints of group formats for autistic youth and emphasize flexible pathways of care.

Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common connective tissue disorder. However, few studies exist on psychiatric and sleep disorders in pediatric patients with hEDS. This study aims to describe psychiatric and sleep disorders and evaluate their impact on health-related quality of life (HRQoL) in pediatric patients with hEDS. As part of a longitudinal study, a convenience sample of 123 pediatric patients with hEDS, were recruited at a hEDS multidisciplinary clinic in sequential order over a seven-month period. Patient-reported outcomes were completed (Patient Reported Outcomes Measurement Information System Pediatric Profile Version 2 [PROMIS], Generalized Anxiety Disorder-7 [GAD-7], Adolescent Sleep Wake Scale [ASWS], and Pediatric Quality of Life Inventory Rheumatology Module [PEDS-QL Rheum]). The mean age was 15.8 years (SD = 2.7), the majority were female (92%) and Caucasian (92%). Most (86%) had at least one psychiatric diagnosis, with anxiety (80%) the most common, followed by depression (42%). Many (42%) also reported poor sleep. Correlations were seen between the GAD-7, PROMIS pain and HRQoL. Worse anxiety and depression were correlated with more sleep problems. Depression, GAD-7, and ASWS were also correlated with worse HRQoL. Performing regression analyses found anxiety and pain to be most predictive of HRQoL. Psychiatric and sleep disorders are prevalent in pediatric patients with hEDS and impact HRQoL negatively. Clinical focus on the anxiety and pain are important due to their impact on HRQoL.

Background/Objectives: Chronic musculoskeletal pain (CMSKP) affects up to 40% of adolescents and leads to substantial disability, reduced quality of life, and long-term health risks. Physical activity is central to treatment, but adherence to moderate-to-vigorous physical activity (MVPA) is inconsistent. We evaluated higher-resilience constructs—self-efficacy, pain acceptance, motivational stage, and affect—and hypothesized that higher resilience would be associated with greater objectively measured physical activity, better daily functioning, and higher quality of life in adolescents with CMSKP. Methods: Forty-three adolescents (13–18 years) with CMSKP completed measures of physical activity-specific self-efficacy, acceptance (AFQ-Y), motivational stage (PSOCQ-A), and affect (PANAS-C). Participants wore activPAL monitors to assess MVPA, light activity, and sedentary time. Physical function endurance was measured by the six-minute walk test (6MWT) and the Functional Disability Inventory (FDI); quality of life by the Pediatric Quality of Life Inventory (PedsQL). Spearman’s correlations assessed associations among resilience variables, physical activity metrics, 6MWT distance, FDI, and PedsQL. Results: MVPA was correlated positively with 6MWT distance (ρ = 0.48, p = 0.002) and negatively with FDI scores (ρ = −0.56, p < 0.001). Self-efficacy related to higher MVPA (ρ = 0.41, p = 0.009), better endurance (ρ = 0.36, p = 0.017), and lower disability (ρ = −0.38, p = 0.013). Acceptance was correlated with PedsQL total (ρ = 0.45, p = 0.004); motivation (specifically maintenance) scores were correlated with higher quality of life (ρ = 0.33, p = 0.027). Light activity and sedentary time were not significantly linked to functional or psychosocial outcomes. In a step-wise regression, only physical activity self-efficacy for ambulation at school predicted MVPA, B = 1.56, p = 0.008. Conclusions: Resilience constructs—including self-efficacy, acceptance, and readiness to change—were meaningfully associated with MVPA, daily functioning, and quality of life, and may have implications for treatment development.

OBJECTIVE:Cognitive-behavioral (CBT) interventions combined with either a physical activity (CBT+PA) or exercise intervention (CBT+Ex) are becoming more common in pediatric populations. Considering the independent effects of PA and exercise on health and psychological outcomes, it is unclear whether CBT alone differs from CBT+PA or CBT+Ex in efficacy. The main objective of this systematic review and meta-analysis of randomized clinical trials (RCTs) was to assess the efficacy of CBT+PA and CBT+Ex interventions in pediatric chronic disease. METHOD:This review included RCTs in children (≤18 years) with a chronic condition, a CBT+Ex or CBT+PA intervention, and an objective measure of PA&Ex. Seven databases were searched using MeSH terms and key terms and included studies published before July 1, 2023. Abstracts were reviewed for inclusion by two independent reviewers, data was extracted by three independent reviewers. Risk of bias (RoB 2) and study quality were coded. Random effect meta-analyses of differences in between-group change in PA&Ex were conducted. RESULTS:Eligible studies (k = 5) reported outcomes for a combined 446 children. A small, nonsignificant overall effect was found (d = 0.10, 95% CI -0.16, 0.35) indicating intervention groups (CBT+PA or CBT+Ex) increased engagement in PA&Ex more than comparator groups (CBT). Additional analyses were inconclusive due to the small number of eligible studies. DISCUSSION:Additional RCTs are needed with integrated PA&Ex interventions targeting pediatric chronic disease. Future trials should report more detailed PA&Ex data. The full protocol for this analysis was prospectively registered in Open Science Framework (project ID: osf.io/m4wtc).

Background Joint hypermobility is common in children and persists in various genetic and connective tissue disorders, including conditions characterized by chronic musculoskeletal pain (i.e. Juvenile Fibromyalgia Syndrome), which involves movement dysfunction. It is unclear if joint hypermobility contributes to this dysfunction. This study investigated whether generalized joint hypermobility is associated with altered landing/jumping biomechanics in adolescents with juvenile fibromyalgia syndrome compared to controls. Methods Adolescents with juvenile fibromyalgia syndrome and hypermobility (n = 17), juvenile fibromyalgia syndrome without hypermobility (n = 17), and non-hypermobile controls (n = 17) performed a landing/jumping task while 3D-motion capture and ground reaction force data were collected. Timewise data were compared using statistical parametric mapping. Findings Both groups with juvenile fibromyalgia syndrome exhibited altered lower extremity biomechanics compared to controls, including increased sagittal hip and ankle kinematics (P < 0.0001), similar to 25 % reduced sagittal knee and ankle kinetics (P <= 0.038) and similar to 2.5x greater knee internal rotation (P < 0.0001) during landing/jumping, as well as similar to 75 % and similar to 20 % reduced ground reaction force during initial landing and jumping (P < 0.0001), respectively. Both groups with juvenile fibromyalgia syndrome, demonstrated 17-26 % reduced landing depth (P < 0.0001;d <= 1.79) and 26 % reduced jump height (P <= 0.01;d <= 0.86), indicating inefficient momentum absorption. Interpretation Altered biomechanics observed in both groups with juvenile fibromyalgia syndrome may reflect an attempt to avoid pain. While hypermobility did not significantly differentiate the groups with juvenile fibromyalgia syndrome overall, it was associated with more inefficiencies. This study highlights the need for hypermobility-specific movement assessments to understand movement-associated pain, strength, and kinesthetics to improve early identification and treatment of youth with hypermobility at risk for chronic pain and functional limitations.

PURPOSE:To assess the validity and reliability of a newly adapted Tampa Scale for KP for adolescents with HD (TSK-Heart-A). METHODS:The TSK-Heart-A survey consists of 17 Likert scale items, producing a summary score (SS) ranging from 17 (low KP) to 68 (high KP). Following content and face validity, adolescents (age 12-18 yr) with arrhythmia disorders or Fontan palliation were recruited. The PROMIS pediatric anxiety and depression short forms, Pediatric Quality of Life Inventory (PedsQL) generic core and cardiac module, and a PA questionnaire for adolescents (PAQ-A) were used for criterion validity. The TSK-Heart-A was completed twice (20.4 ± 6.3 days apart) for assessment of internal and test-retest reliability. Pearson correlations and Cronbach's alpha determined validity and reliability, respectively. RESULTS:Adolescents ( n = 63) were 15.5 ± 1.8 yrs old, 50.8% were female, and 69.8% had arrhythmia disorder. Mean TSK-Heart-A SS was 33.8 ± 7.8 (range, 20 to 56). The TSK-Heart-A SS correlated significantly with PROMIS T-scores (anxiety: 49.7 ± 12.2, r = 0.48; depression: 46.9 ± 12.7, r = 0.41), PedsQL score (generic QoL: 75.2 ± 18.9, r = -0.61; cardiac QoL: 74.7 ± 17.5, r = -0.56), and PAQ-A (score: 2.0 ± 0.7, r = -0.35). Test-retest and internal reliability had a total score of r = 0.77 ( P = 0.89). CONCLUSIONS:The new TSK-Heart-A survey is valid and reliable and suggests that KP is present in some adolescents with HD.

To investigate the experiences of adolescents with chronic pain who participated in an intensive interdisciplinary pain treatment program, this secondary study analyzes the themes that emerged regarding the spontaneous utilization of music in coping strategies for chronic pain. During research interviews focused on coping skills and treatment engagement, participants spontaneously reported using music as an effective coping strategy for managing pain. A deductive thematic analysis revealed key themes related to their usage, including using music as a distractor, motivator and in other ways as coping strategies. Since participants indicated that music is essential to their experiences of coping with pain, incorporating these strategies could improve the effectiveness of treatment protocols. To this end, further investigation is necessary to assess the impact of music on adolescents with chronic pain, focusing on its role in enhancing interdisciplinary treatment.

Youth with heart disease experience comorbidities that impact physical activity (PA), mental health (MH), and quality of life (QoL). PURPOSE:To explore the relationship between PA and MH and QoL in adolescents with cardiac arrhythmia disorders, cardiomyopathies, or single ventricle heart disease. METHODS:Participants completed surveys/questionnaires regarding PA (Physical Activity Questionnaire for Adolescents), anxiety (Patient-Reported Outcomes Measurement Information System [PROMIS]-Anxiety), depressive symptoms (PROMIS-Depressive Symptoms), global QoL (Pediatric Quality of Life Inventory [PedsQL]-generic), and cardiac disease-specific QoL (PedsQL-cardiac). Pearson correlations and regression analysis examined relationships between PA and MH/QoL. RESULTS:The study included 63 participants (males = 32, 50.7%), with an age of 15.46 (1.82) years. The sample was mostly inactive (Physical Activity Questionnaire for Adolescents score: 1.95 [0.65]). Outcomes used T-scores for PROMIS-Anxiety (49.7 [12.2]) and PROMIS-Depressive Symptoms (46.9 [12.7]), and summary scores for PedsQL-generic (75.2 [18.9]) and PedsQL-cardiac (74.7 [17.5]). There was a negative correlation between PA and MH (PROMIS-Anxiety: r = -.338, PROMIS-Depressive Symptoms: r = -.337; P < .05) and positive correlation between PA and QoL (PedsQL-generic: r = .37, PedsQL-cardiac: r = .39; P < .001). Adjusting for key variables using regression analysis, greater participation in PA was independently related to decreased anxiety and depression and greater QoL. CONCLUSIONS:Greater PA engagement was associated with more favorable MH and QoL in adolescents with cardiac arrhythmia disorders, cardiomyopathy, or single ventricle heart disease.

Background Kinesiophobia—an excessive, often debilitating fear of movement or exercise—has emerged as an important moderator of physical activity (PA) and has been linked with quality of life (QoL), anxiety, and depression in adolescents with heart disease (HD). This study explores additional factors that may be related to cardiac-focused kinesiophobia, including self-efficacy, PA engagement, and QoL. Methods Sixty-three adolescents (mean age = 15.5 years; 49% female) with congenital or acquired HD completed the TSK-Heart-A, the Physical Activity Questionnaire for Adolescents (PAQ-A), the Pediatric Quality of Life Inventory (PedsQL™) Generic Core and Cardiac Module, and the Domain-Specific Physical Activity Efficacy Questionnaire (DSPAEQ-A). Data were analyzed using Kendall’s Tau correlations and bootstrapped partial correlations controlling for PA. Results Greater kinesiophobia correlated with lower PA across both PA measures (τ = −.248), lower PA self-efficacy across household, leisure-time, and ambulatory domains (τ = −.293 to − .356), and poorer QoL for both generic and cardiac scales (τ = −.403, − .400). When controlling for PA, kinesiophobia remained significantly related to lower self-efficacy (r = − .314 to − .368) and poorer QoL (r = − .558, − .520) Discussion Cardiac-focused kinesiophobia is a salient psychological factor in pediatric HD. It is tied to lower PA and poorer QoL and is independently associated with reduced PA self-efficacy—a modifiable resilience construct. These findings support the development of interventions that target fear-avoidance mechanisms and build self-efficacy to improve everyday activity and psychosocial functioning in adolescent HD.

Chronic pain in children and adolescents is often associated with functional, physical, and psychosocial challenges. Intensive interdisciplinary pain treatment (IIPT) programs are effective at helping these youth regain functioning, but little is known about their perspectives prior to and during IIPT participation. This study sought to better understand how children and adolescents experience the process from evaluation to completion of the IIPT program. Individual interviews (n = 7) were conducted at three time-points; (1) prior to initial evaluation in a pain clinic, (2) after pain clinic evaluation while considering IIPT, and (3) after completion of an IIPT program. Participants ranged in age from 13–17 years. Across these time points, participants demonstrated changes in thoughts and perspectives. While Time 1 was associated with ambivalence, skepticism, and some hope, Time 2 was characterized by processing information about the program and resolution of some of their ambivalence. At Time 3, participants described their experience as “challenging” and “intense” and reported recognition that they had benefited from the program. Participants also wished to pass along lessons learned to future potential patients. This study provides information to help clinicians better approach adolescents with chronic pain who may be considering IIPT. This study, for the first time, characterizes patient experiences as they navigate entry to, beginning, and completion of an intensive interdisciplinary pain treatment program. It highlights adolescents’ thought processes as they weigh whether to engage in and ultimately complete the program, providing insight on changes in their thinking over time.

Background:: Hypermobile Ehlers-Danlos Syndrome (hEDS) is a connective tissue disorder characterized by joint hypermobility and other systemic manifestations, such as cardiovascular symptoms, musculoskeletal pain, and joint instability. Cardiovascular symptoms, such as lightheadedness and palpitations, and types of dysautonomia, including postural orthostatic tachycardia syndrome (POTS), are frequently reported in adults with hEDS and have been shown to negatively impact quality of life (QoL). Objective:: This brief review will be an overview of co-occurring symptoms in POTS and hEDS to inform potential cardiovascular screening procedures. Results:: While many patients with hEDS report cardiovascular symptoms, few have structural abnormalities, suggesting that dysautonomia is likely responsible for these symptoms. One validated screening measure for dysautonomia symptom burden is the Composite Autonomic Symptom Scale (COMPASS-31). Studies have found that adults with POTS, hEDS, and both POTS and hEDS have higher COMPASS-31 scores than the general population, suggesting a high symptom burden due to dysautonomia, which leads to impaired QoL. Conclusion:: While studies have examined cardiovascular symptoms and the impact of dysautonomia in adults with and without hEDS, there is scant literature on dysautonomia in pediatric patients with hEDS. Therefore, more studies on cardiovascular symptoms and dysautonomia, as they relate to the quality of life in pediatric patients with hEDS, are needed. This brief review summarizes the current literature on dysautonomia and cardiovascular symptoms in pediatric and adult populations with hEDS.

INTRODUCTION:After diagnosis of Ehlers Danlos Syndrome (EDS), it is unclear what information patients and parents need and understand about EDS. The objective of this study is to characterize patient and parent knowledge and concerns about EDS after a diagnosis of EDS is made to determine patient and parent concerns and identify barriers that cause discomfort with the diagnosis.6 METHODS: A convenience sample of patient and parent dyads were recruited after new diagnosis of EDS. Patients and parents completed questionnaires that assessed knowledge, comfort, and barriers of EDS before and after diagnosis, EDS education materials accessed, and additional clinical needs and concerns. RESULTS:Seventy-two dyads completed the survey. CONCLUSION:Many respondents actively seek information on the diagnosis and management of EDS. Parents and patients look for information about EDS differently. Parents have more concerns after diagnosis and both want well-constructed, empirically supported educational materials delivered via multiple modalities, which makes clinical guidelines more essential.

While 10% of the U.S. population are afflicted with a rare disease, patients with a rare disease can have a difficult time finding a provider or useful information. Patients with rare diseases are often frustrated by lack of knowledge from their provider, lack of satisfaction with their provider, and lack of available time with their provider. Social connections and networks can be a useful tool for patients with rare diseases to seek and maintain support and information. For this study, a specific rare disease's (Ehlers-Danlos syndrome) support groups in a moderately sized metropolitan area were surveyed about their social interactions with others around their rare disease. The survey was distributed through online support groups on Facebook and was open over a 3-month period. For the 102 respondents that completed the social networking portion of the survey, there were 448 connections. The main modality of networking was in-person, with discussions about their rare disease occurring daily. Providers can utilize these social networks, after engaging with patients, to disseminate educational information and request feedback from patients. The social networks patients create can help them feel a sense of connectedness they might not otherwise feel due to their rare disease.

Objective: Parents of youth with chronic pain report psychosocial difficulties, yet treatment often focuses on improving their child's functioning and pain. This study evaluated changes in parents' social and emotional functioning and explored predictors of change, as they completed a parent-focused intervention while their child was enrolled in an intensive interdisciplinary pain treatment (IIPT) program.Methods: Parents (n = 69) completed questionnaires at baseline and weekly (average duration of 4 weeks) during their child's participation in IIPT. Parents engaged in 3 groups per week providing education, therapeutic art, and psychotherapy (3 hr/week total).Results: At baseline, 38% of parents reported scores in the clinically elevated range for at least 1 psychosocial variable. Linear mixed modeling for the full sample indicated reduced parent anxiety (t = -2.72, p <.01) and depression (t = -3.59, p <.001), but not increased emotional support (t = 1.86, p >. 05) or reduced social isolation (t = -1.20, p >.05). For parents with at least moderately elevated psychosocial concerns, statistically significant improvements were observed for all 4 outcomes (all p's<.01). Psychological flexibility, cognitive reappraisal, and emotional suppression were found to be related to changes in parent outcomes (anxiety, depression, isolation, and support).Conclusions: Findings support the benefit of parent-focused interventions in addition to child-focused interventions. Many parents of youth participating in IIPT had elevated scores for at least 1 psychosocial concern at baseline. Brief, parent-focused intervention including psychoeducation, therapeutic art, and psychotherapy targeting mindfulness, acceptance, and values had a significant impact on these parents, particularly those with greater struggles at baseline.

Objective:To identify how pediatric patients with hypermobile-type Ehlers-Danlos syndrome (hEDS) present to physical therapy (PT) and better understand their musculoskeletal dysfunction. Design:Patients were included in the study if they had a diagnosis of hEDS and at least 1 completed PT evaluation after diagnosis. A chart review was conducted to assess joint strength and pain characteristics at the initial PT assessment. Physical Function Mobility (PF-Mobility; 4 domains with 1-5 scale with higher score representative of better physical function) as part of the Patient-Reported Outcome Measurement Information System and pain intensity score (visual analog scale, 0-10 scale where a lower number indicates less pain) was completed by participants. Setting:Midwestern tertiary care center. Participants:Sixty-nine participants were included in the study with an average age of 16.4 years (SD 2.8) at the initial PT visit and 96% were female individuals. Intervention:Not applicable. Main Outcome Measures:Joint strength, pain intensity and location, and PF-Mobility. Results:The knee was the most reported location with pain (68%) followed by the shoulder (46%), hip (41%), and back (39%). Average pain intensity was 5.0 (SD 1.8), joint strength scores ranged from 8.2 (SD 0.9) to 9.9 (SD 0.4), and the PF-Mobility mean was 3.3 (SD 0.6). No correlation was noted between joint pain intensity and strength at the joint. Conclusions:Although the participants perceived poor mobility and significant pain, PT-assessed strength did not reveal significant muscle weakness. Additional work is needed to explore this discrepancy, including altered biomechanics, evaluation methods, and nonmusculoskeletal factors such as neuropathic and psychological components.

Patients with joint-hypermobility and joint-hypermobility spectrum disorders (HSD), including hypermobile Ehlers-Danlos Syndromes (EDS) present numerous co-morbid concerns, and multidisciplinary care has been recommended. The complexity of these patient's needs and increased demand for medical services have resulted in long delays for diagnosis and treatment and exhausted extant clinical resources. Strategies must be considered to ensure patient needs are met in a timely fashion. This opinion piece discusses several potential models of care for joint-hypermobility disorders, several ways in which primary providers can be involved, and argues that primary providers should be an essential and integrated part of the management of these patients, in collaboration with multidisciplinary teams and pediatric subspecialists. We review several strategies and educational opportunities that may better incorporate primary providers into the care and management of these patients, and we also discuss some of the limitations and barriers that need to be addressed to improve provision of care. This includes establishing primary care physicians as the medical home, providing initial diagnostic and treatment referrals while connecting patients with specialty care, and collaboration and coordination with multi-disciplinary teams for more complex needs. Several barriers exist that may hamper these efforts, including a lack of available specialty trainings for providers interested in providing care to patients with EDS and HSD, a lack of expertly derived consensus guidelines, and limited time resources in extant primary care practices. Also, primary providers should have an active voice in the future for the further consideration and development of these presented strategies.