Janneke E. de Vries

Generalized joint hypermobility in professional dancers: a sign of talent or vulnerability?

OBJECTIVE To study the impact of generalized joint hypermobility (GJH) in professional dancers on physical fitness, musculoskeletal complaints and psychological distress. METHODS Thirty-six professional dancers were recruited and compared with control subjects (mean age 20.1, range 17-27). Height, weight, Beighton score, physical fitness (walking distance, muscle strength, estimated VO2max), musculoskeletal complaints (pain, fatigue) and psychological distress (anxiety, depression) were measured. RESULTS Univariate analysis revealed, in between-group analysis, that dancers (with and without GJH) had higher physical fitness [the six-minute walk test (6MWT): ΔD = +8.4%, P = 0.001; VO2max: ΔD = +12.8%, P = 0.01], fatigue (checklist individual strength: ΔD = +80.3%, P < 0.0001) and greater psychological distress (Hospital Anxiety and Depression Scale: ΔD = +115.0%, P < 0.0001). When comparing dancers and control subjects with GJH to those without GJH, lower levels of physical fitness (muscle strength: ΔD = -11.3%, P < 0.0001; 6MWT: ΔD = -9.9%, P < 0.0001), more fatigue (checklist individual strength: ΔD = +84.4%, P < 0.0001) and greater psychological distress (Hospital Anxiety and Depression Scale: ΔD = +79.6%, P < 0.0001) were observed in subjects with GJH. Multivariate analysis showed that dancers have higher levels of physical fitness (6MWT, P = 0.001; VO2max, P = 0.020); however, when taking GJH into account, this advantage disappeared, indicating lower levels of physical fitness in comparison with control subjects (6MWT, P = 0.001; muscle strength, P < 0.0001; VO2max, P = 0.040). Dancers experienced more fatigue (P = 0.001) and psychological distress (P < 0.0001). This was associated with even more fatigue (P = 0.010) and psychological distress (P = 0.040) when GJH was present. CONCLUSION Dancers with GJH seem more vulnerable to musculoskeletal and psychological complaints. In addition, GJH was also associated with lower physical fitness, despite training. Caregivers for professional dancers should monitor closely the physical capabilities and the amount of psychological strain.

Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type): it is a challenge.

Generalized joint hypermobility (GJH) is highly prevalent among patients diagnosed with chronic pain. When GJH is accompanied by pain in ≥4 joints over a period ≥3 months in the absence of other conditions that cause chronic pain, the hypermobility syndrome (HMS) may be diagnosed. In addition, GJH is also a clinical sign that is frequently present in hereditary diseases of the connective tissue, such as the Marfan syndrome, osteogenesis imperfecta, and the Ehlers–Danlos syndrome. However, within the Ehlers–Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile was identified: Ehlers–Danlos syndrome hypermobility type (EDS-HT). Researchers and clinicians have struggled for decades with the highly diverse clinical presentation within the HMS and EDS-HT phenotypes (Challenge 1) and the lack of understanding of the pathological mechanisms that underlie the development of pain and its persistence (Challenge 2). In addition, within the HMS/EDS-HT phenotype, there is a high prevalence of psychosocial factors, which again presents a difficult issue that needs to be addressed (Challenge 3). Despite recent scientific advances, many obstacles for clinical care and research still remain. To gain further insight into the phenotype of HMS/EDS-HT and its mechanisms, clearer descriptions of these populations should be made available. Future research and clinical care should revise and create consensus on the diagnostic criteria for HMS/EDS-HT (Solution 1), account for clinical heterogeneity by the classification of subtypes within the HMS/EDS-HT spectrum (Solution 2), and create a clinical core set (Solution 3).

The functional consequences of Generalized Joint Hypermobility: a cross-sectional study

BackgroundGeneralized Joint Hypermobility (GJH) has been found to be associated with musculoskeletal complaints and disability. For others GJH is seen as a prerequisite in order to excel in certain sports like dance. However, it remains unclear what the role is of GJH in human performance. Therefore, the purpose of the study was to establish the association between GJH and functional status and to explore the contribution of physical fitness and musculoskeletal complaints to this association.MethodsA total of 72 female participants (mean age (SD; range): 19.6 (2.2; 17-24)) were recruited among students from the Amsterdam School of Health Professions (ASHP) (n = 36) and the Amsterdam School of Arts (ASA), Academy for dance and theater (n = 36) in Amsterdam, The Netherlands. From each participant the following data was collected: Functional status performance (self-reported Physical activity level) and capacity (walking distance and jumping capacity: side hop (SH) and square hop (SQH)), presence of GJH (Beighton score ≥4), muscle strength, musculoskeletal complaints (pain and fatigue) and demographic characteristics (age and BMI).ResultsGJH was negatively associated with all capacity measures of functional status. Subjects with GJH had a reduced walking distance (B(SE):-75.5(10.5), p = <.0001) and jumping capacity (SH: B(SE):-10.10(5.0), p = .048, and SQH: B(SE):-11.2(5.1), p = .024) in comparison to subjects without GJH, when controlling for confounding: age, BMI and musculoskeletal complaints. In participants with GJH, functional status was not associated with performance measures.ConclusionGJH was independently associated with lower walking and jumping capacity, potentially due to the compromised structural integrity of connective tissue. However, pain, fatigue and muscle strength were also important contributors to functional status.

Dutch version of the Fear of Pain Questionnaire for adolescents with chronic pain

Abstract Background: Fear of pain is important in the development and maintenance of chronic pain. The Fear of Pain Questionnaire-Child version has been developed to assess pain related fear in children and adolescents. Objective: Translating the original questionnaire into Dutch, and investigating internal consistency and construct validity to enable use in the Dutch pain rehabilitation setting for treatment and research. Methods: Cross-sectional validation study: After forward and back translation of the FOPQ-C, adolescents (11–22 years old) with chronic musculoskeletal pain completed an assessment containing the Dutch Fear of Pain Questionnaire, and questionnaires about demographics, pain catastrophizing, functional disability, and pain intensity. Internal consistency and construct validity were evaluated through exploratory factor analysis (principal axis factoring with oblique rotation) and hypotheses testing using pain catastrophizing, functional disability, and pain intensity as comparative constructs. Results: Eighty-six adolescents completed the assessment. Exploratory factor analysis resulted in a two-factor structure, explaining 43% of the variance. Internal consistency was strong (Cronbach’s α = 0.92 total scale, α = 0.88 factor 1, and α = .86 factor 2). Five out of 6 hypotheses were confirmed. Conclusions: The Dutch version demonstrated good internal consistency and good construct validity in a population of adolescents with chronic musculoskeletal pain. Implications for rehabilitation The Fear of Pain Questionnaire-Child version was developed to measure fear of pain and avoidance in children and adolescents with chronic pain. Identification of fear of pain and activities that are being avoided are important during screening and assessment of the adolescent for chronic pain rehabilitation treatment. The presence of fear of pain and/or avoidance behavior is important information to shape and target multidisciplinary rehabilitation treatment.

The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers–Danlos syndrome: the impact of proprioception

Abstract Purpose: The patients diagnosed with Ehlers–Danlos Syndrome Hypermobility Type (EDS-HT) are characterized by pain, proprioceptive inacuity, muscle weakness, potentially leading to activity limitations. In EDS-HT, a direct relationship between muscle strength, proprioception and activity limitations has never been studied. The objective of the study was to establish the association between muscle strength and activity limitations and the impact of proprioception on this association in EDS-HT patients. Methods: Twenty-four EDS-HT patients were compared with 24 controls. Activity limitations were quantified by Health Assessment Questionnaire (HAQ), Six-Minute Walk test (6MWT) and 30-s chair-rise test (30CRT). Muscle strength was quantified by handheld dynamometry. Proprioception was quantified by movement detection paradigm. In analyses, the association between muscle strength and activity limitations was controlled for proprioception and confounders. Results: Muscle strength was associated with 30CRT (r = 0.67, p = <0.001), 6MWT (r = 0.58, p = <0.001) and HAQ (r = 0.63, p= <0.001). Proprioception was associated with 30CRT (r = 0.55, p < 0.001), 6MWT (r = 0.40, p = <0.05) and HAQ (r = 0.46, p < 0.05). Muscle strength was found to be associated with activity limitations, however, proprioceptive inacuity confounded this association. Conclusions: Muscle strength is associated with activity limitations in EDS-HT patients. Joint proprioception is of influence on this association and should be considered in the development of new treatment strategies for patients with EDS-HT. Implications for rehabilitation Reducing activity limitations by enhancing muscle strength is frequently applied in the treatment of EDS-HT patients. Although evidence regarding treatment efficacy is scarce, the current paper confirms the rationality that muscle strength is an important factor in the occurrence of activity limitations in EDS-HT patients. Although muscle strength is the most dominant factor that is associated with activity limitations, this association is confounded by proprioception. In contrast to common belief proprioception was not directly associated with activity limitations but confounded this association. Controlling muscle strength on the bases of proprioceptive input may be more important for reducing activity limitations than just enhancing sheer muscle strength.

The Dutch version of the self-report Child Activity and Limitations Interview in adolescents with chronic pain

Abstract Purpose: To assess the factor structure, related constructs and internal consistency of the Child Activity Limitation Interview 21-Child version for use in Dutch-language countries. Methods: Cross-sectional validation study: After forward and back translation of the Dutch version of the Child Activity Limitation Interview 21-Child adolescents (11–21 years old) with chronic musculoskeletal pain completed an assessment. The assessment contained the Dutch Child Activity Limitation Interview, and questionnaires about demographics, pain intensity, functional disability, anxiety and depression. Internal consistency and construct validity were evaluated through exploratory factor analysis (principal axis factoring with oblique rotation) and hypotheses testing using pain intensity, activity limitations, anxiety and depression as comparative constructs. Results: Seventy-four adolescents completed the assessment. Exploratory factor analysis resulted in a two-factor structure, explaining 50% of the variance. Internal consistency was good (Cronbach’s α = 0.91 total scale, α = 0.90 Factor 1, α = 0.80 Factor 2). All nine hypotheses were confirmed. Conclusion: The Dutch version can be used to assess pain-related disability in Dutch-speaking adolescents comparable to the study sample. Scores on both subscales provide insight into the severity of the pain-related disability in both daily routine and more physically vigorous activities. Implications for Rehabilitation Chronic pain is a disabling disorder which not only impacts physically but restricts quality of life. This study provides clinicians a questionnaire to measure pain-related disability and quantify the impact of pain on the daily living of adolescents. The advantage of the Dutch version of the Child Activity and Limitations Interview over other measurements is that it can distinguish limitations in daily activities from more physically vigorous activities.