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.

Physical performance outcome measures used in exercise interventions for adults with childhood-onset disabilities: A scoping review.

BACKGROUND: People with childhood-onset disabilities face unique physical and social challenges in adulthood. Exercise interventions may improve physical performance in children, but there is a lack of research on adults. OBJECTIVE: To describe studies that investigate exercise interventions and to evaluate the quality of physical performance outcome measures for adults with childhood-onset disabilities. METHODS: Eligible studies reported on exercise interventions for adults (ages 16+) with cerebral palsy, spina bifida, or acquired brain injuries. Only randomized controlled trials published in English from 2008 to 2019 were included. MEDLINE, CINAHL, PEDro, EMBASE, and Cochrane Central Register of Controlled Trials were searched. Two reviewers independently screened studies and abstracted data. RESULTS: This scoping review included 4 trials reporting on cerebral palsy only. Three strength training programs found significant improvements in gait, and one mixed training program found significant improvements in strength and fitness. Only two outcome measures used are valid/reliable for adults (6 Minute Walk Test and Borg-20 Grades). CONCLUSION: Certain interventions may improve physical performance, but there is a lack of research on appropriate exercise interventions and physical performance outcome measures for adults with childhood-onset disabilities. Different exercise interventions should be investigated using larger sample sizes and outcome measures should be standardized.

Transitional Care Interventions for Youth With Disabilities: A Systematic Review.

CONTEXT: Transition from the pediatric to the adult health care system is a complex process that should include medical, psychosocial, educational, recreational, and vocational considerations. OBJECTIVE: In this systematic review, we aim to synthesize the evidence on transitional care interventions (TCIs) to improve the quality of life (QoL) for adolescents and young adults with childhood-onset disabilities, including neurodevelopmental disorders. DATA SOURCES: Four electronic databases (Medline, Embase, PsycINFO, and Cumulative Index to Nursing and Allied Health Literature) were searched. STUDY SELECTION: In the included studies, researchers examined TCIs for adolescents and young adults (12-24 years of age) with childhood-onset disabilities. Studies were experimental, quasi-experimental, and observational studies published in the last 26 years. DATA EXTRACTION: Two reviewers independently completed study screening, data extraction, and risk-of-bias assessment. RESULTS: Fifty-two studies were included. Five studies reported on QoL, but statistically significant improvements were noted in only 1 of these studies. Significant improvements were also found in secondary outcomes including disability-related knowledge and transitional readiness. TCIs targeted patients, families and/or caregivers, and health care providers and exhibited great heterogeneity in their characteristics and components. LIMITATIONS: Inconsistent reporting on interventions between studies hindered synthesis of the relationships between specific intervention characteristics and outcomes. CONCLUSIONS: Although there is limited evidence on the impact of TCIs on the QoL for youth with childhood-onset disabilities, there is indication that they can be effective in improving patient and provider outcomes. The initiation of transition-focused care at an early age may contribute to improved long-term health outcomes in this population.