Interdisciplinary pain rehabilitation for patients with Ehlers-Danlos syndrome and hypermobility spectrum disorders
DOI:
https://doi.org/10.2340/jrm.v56.12431Keywords:
chronic pain, rehabilitation, Ehlers-Danlos syndromeAbstract
Objective: Chronic pain is a common manifestation of Ehlers-Danlos syndrome and hypermobility spectrum disorders; thus it is often suggested that patients undergo generic interdisciplinary pain rehabilitation, despite there being little evidence to support this decision. The aim of this study is to examine the effectiveness of standard rehabilitation programmes for chronic pain on patients with Ehlers-Danlos syndrome and hypermobility spectrum disorders, compared with patients with other chronic pain disorders.
Subjects: Data, collected between 2008 and 2016, were extracted from a Swedish national registry. The patient data comprised of 406 cases with Ehlers-Danlos syndrome or hypermobility spectrum disorders, 784 cases with a whiplash-related diagnosis, 3713 cases with diagnoses relating to spinal pain, and 2880 cases of fibromyalgia.
Methods: The differences between groups on key outcome measures from pre- to 1-year follow-up after interdisciplinary pain rehabilitation were analysed using linear mixed effects models. Sensitivity analysis in the form of pattern-mixture modelling was conducted to discern the impact of missing data.
Results: No significant differences were found in improvements from pre- to 1-year follow-up for patients with Ehlers-Danlos syndrome or hypermobility spectrum disorder compared with other diagnostic groups regarding measures of health-related quality of life, mental health, or fatigue. At follow-up, differences in pain interference (d = –0.34 (95% confidence interval [95% CI] –0.5 to –0.18)), average pain (d = 0.22 (95% CI 0.11–0.62)) and physical functioning (d = 2.19 (95% CI 1.61–2.77)) were detected for the group with spinal-related diagnoses in relation to those with EDS/HSD, largely due to pre-treatment group differences. Sensitivity analysis found little evidence for missing data influencing the results.
Conclusion: This study suggests that patients with Ehlers-Danlos syndrome/hypermobility spectrum disorders may benefit from inclusion in an interdisciplinary pain rehabilitation programme.
Downloads
References
Kinge JM, Knudsen AK, Skirbekk V, Vollset SE. Musculoskeletal disorders in Norway: prevalence of chronicity and use of primary and specialist health care services. BMC Musculoskelet Disord 2015; 16: 75.
https://doi.org/10.1186/s12891-015-0536-z DOI: https://doi.org/10.1186/s12891-015-0536-z
Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers–Danlos syndromes. Am J Med Genet C Semin Med Genet 2017; 175: 8–26.
https://doi.org/10.1002/ajmg.c.31552 DOI: https://doi.org/10.1002/ajmg.c.31552
Syx D, Wandele ID, Rombaut L, Malfait F. Hypermobility, the Ehlers-Danlos syndromes and chronic pain. Clin Exp Rheumatol 2017; 35: 116–122.
Castori M, Tinkle B, Levy H, Grahame R, Malfait F, Hakim A. A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet 2017; 175: 148–157.
https://doi.org/10.1002/ajmg.c.31539 DOI: https://doi.org/10.1002/ajmg.c.31539
Rombaut L, Malfait F, De Paepe A, Rimbaut S, Verbruggen G, De Wandele I, et al. Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: a comparative study with fibromyalgia and rheumatoid arthritis. Arthritis Rheum 2011; 63: 1979–1987.
https://doi.org/10.1002/art.30337 DOI: https://doi.org/10.1002/art.30337
Castori M. Pain in Ehlers-Danlos syndromes: manifestations, therapeutic strategies and future perspectives. Expert Opin Orphan Drugs 2016; 4: 1145–1158.
https://doi.org/10.1080/21678707.2016.1238302 DOI: https://doi.org/10.1080/21678707.2016.1238302
Baets SD, Cruyt E, Calders P, Dewandele I, Malfait F, Vanderstraeten G, et al. Societal participation in ehlers-danlos syndromes and hypermobility spectrum disorder, compared to fibromyalgia and healthy controls. PLOS One 2022; 17: e0269608.
https://doi.org/10.1371/journal.pone.0269608 DOI: https://doi.org/10.1371/journal.pone.0269608
Molander P, Novo M, Hållstam A, Löfgren M, Stålnacke B-M, Gerdle B. Ehlers–Danlos syndrome and hypermobility syndrome compared with other common chronic pain diagnoses – a study from the Swedish Quality Registry for Pain Rehabilitation. J Clin Med 2020; 9: 2143.
https://doi.org/10.3390/jcm9072143 DOI: https://doi.org/10.3390/jcm9072143
Ringqvist Å, Dragioti E, Björk M, Larsson B, Gerdle B. Moderate and stable pain reductions as a result of interdisciplinary pain rehabilitation-a cohort study from the Swedish Quality Registry for Pain Rehabilitation (SQRP). J Clin Med 2019; 8: 905.
https://doi.org/10.3390/jcm8060905 DOI: https://doi.org/10.3390/jcm8060905
Cohen SP, Vase L, Hooten WM. Chronic pain: an update on burden, best practices, and new advances. The Lancet 2021; 397: 2082–2097.
https://doi.org/10.1016/S0140-6736(21)00393-7 DOI: https://doi.org/10.1016/S0140-6736(21)00393-7
Gatchel RJ, Peng YB, Peters ML, Fuchs PN, Turk DC. The biopsychosocial approach to chronic pain: Scientific advances and future directions. Psychol Bull 2007; 133: 581–624.
https://doi.org/10.1037/0033-2909.133.4.581 DOI: https://doi.org/10.1037/0033-2909.133.4.581
Gerdle B, Fischer MR, Ringqvist Å. Interdisciplinary Pain Rehabilitation Programs: Evidence and Clinical Real-World Results. IntechOpen; 2022 [cited 2022 Jun 30].
https://doi.org/10.5772/intechopen.102411 DOI: https://doi.org/10.5772/intechopen.102411
Rivano Fischer M, Schults M-L, Stålnacke B-M, Ekholm J, Persson EB, Löfgren M. Variability in patient characteristics and service provision of interdisciplinary pain rehabilitation: a study using the Swedish national quality registry for pain rehabilitation. J Rehabil Med 2020; 52: jrm00128.
https://doi.org/10.2340/16501977-2765 DOI: https://doi.org/10.2340/16501977-2765
Waterschoot FPC, Dijkstra PU, Hollak N, de Vries HJ, Geertzen JHB, Reneman MF. Dose or content? Effectiveness of pain rehabilitation programs for patients with chronic low back pain: a systematic review. Pain 2014; 155: 179–189.
https://doi.org/10.1016/j.pain.2013.10.006 DOI: https://doi.org/10.1016/j.pain.2013.10.006
Kamper SJ, Apeldoorn AT, Chiarotto A, Smeets RJEM, Ostelo RWJG, Guzman J, et al. Multidisciplinary biopsychosocial rehabilitation for chronic low back pain: Cochrane systematic review and meta-analysis. BMJ 2015; 350: h444.
https://doi.org/10.1136/bmj.h444 DOI: https://doi.org/10.1136/bmj.h444
Swedish Council on Health Technology Assessment. Rehabilitation for chronic pain: a systematic literature review. Stockholm: SBU, Swedish Agency for Health Technology Assessment and Assessment of Social Services; 2010. Report no.: 177–1+2.
Corrado B, Ciardi G. Hypermobile Ehlers-Danlos syndrome and rehabilitation: taking stock of evidence based medicine: a systematic review of the literature. J Phys Ther Sci 2018; 30: 843–847.
https://doi.org/10.1589/jpts.30.847 DOI: https://doi.org/10.1589/jpts.30.847
Palmer S, Davey I, Oliver L, Preece A, Sowerby L, House S. The effectiveness of conservative interventions for the management of syndromic hypermobility: a systematic literature review. Clin Rheumatol 2021; 40: 1113–1129.
https://doi.org/10.1007/s10067-020-05284-0 DOI: https://doi.org/10.1007/s10067-020-05284-0
Baeza-Velasco C, Bulbena A, Polanco-Carrasco R, Jaussaud R. Cognitive, emotional, and behavioral considerations for chronic pain management in the Ehlers-Danlos syndrome hypermobility-type: a narrative review. Disabil Rehabil 2019; 41: 1110–1118.
https://doi.org/10.1080/09638288.2017.1419294 DOI: https://doi.org/10.1080/09638288.2017.1419294
Chopra P, Tinkle B, Hamonet C, Brock I, Gompel A, Bulbena A, et al. Pain management in the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017; 175: 212–219.
https://doi.org/10.1002/ajmg.c.31554 DOI: https://doi.org/10.1002/ajmg.c.31554
Bathen T, Hångmann AB, Hoff M, Øinæs Andersen L, Rand-Hendriksen S. Multidisciplinary treatment of disability in Ehlers-Danlos syndrome hypermobility type/hypermobility syndrome: a pilot study using a combination of physical and cognitive-behavioral therapy on 12 women. Am J Med Genet A 2013; 161A: 3005–3011.
https://doi.org/10.1002/ajmg.a.36060 DOI: https://doi.org/10.1002/ajmg.a.36060
Hakimi A, Bergoin C, Mucci P. Immediate and 6-week after effects of a rehabilitation program for Ehlers–Danlos syndrome hypermobile type patients: a retrospective study. Am J Med Genet A 2020; 182: 2263–2271.
https://doi.org/10.1002/ajmg.a.61772 DOI: https://doi.org/10.1002/ajmg.a.61772
Rahman A, Daniel C, Grahame R. Efficacy of an out-patient pain management programme for people with joint hypermobility syndrome. Clin Rheumatol 2014; 33: 1665–1669.
https://doi.org/10.1007/s10067-014-2539-9 DOI: https://doi.org/10.1007/s10067-014-2539-9
Dworkin RH, Turk DC, Farrar JT, Haythornthwaite JA, Jensen MP, Katz NP, et al. Core outcome measures for chronic pain clinical trials: IMMPACT recommendations: Pain 2005; 113: 9–19.
https://doi.org/10.1016/j.pain.2004.09.012 DOI: https://doi.org/10.1016/j.pain.2004.09.012
Kaiser U, Kopkow C, Deckert S, Neustadt K, Jacobi L, Cameron P, et al. Developing a core outcome domain set to assessing effectiveness of interdisciplinary multimodal pain therapy: the VAPAIN consensus statement on core outcome domains. Pain 2018; 159: 673–683.
https://doi.org/10.1097/j.pain.0000000000001129 DOI: https://doi.org/10.1097/j.pain.0000000000001129
Haefeli M, Elfering A. Pain assessment. Eur Spine J 2006; 15: S17–S24.
https://doi.org/10.1007/s00586-005-1044-x DOI: https://doi.org/10.1007/s00586-005-1044-x
Bergström G, Jensen IB, Bodin L, Linton SJ, Nygren Å L, Carlsson SG. Reliability and factor structure of the multidimensional pain inventory – Swedish language version (MPI-S). Pain 1998; 75: 101–110.
https://doi.org/10.1016/S0304-3959(97)00210-8 DOI: https://doi.org/10.1016/S0304-3959(97)00210-8
Rabin R, Charro F de. EQ-SD: a measure of health status from the EuroQol Group. Ann Med 2001; 33: 337–343.
https://doi.org/10.3109/07853890109002087 DOI: https://doi.org/10.3109/07853890109002087
Sullivan M, Karlsson J, Ware JE. The Swedish SF-36 Health Survey-I. Evaluation of data quality, scaling assumptions, reliability and construct validity across general populations in Sweden. Soc Sci Med 1995; 41: 1349–1358.
https://doi.org/10.1016/0277-9536(95)00125-Q DOI: https://doi.org/10.1016/0277-9536(95)00125-Q
Hedeker D, Gibbons RD. Longitudinal data analysis. Vol. 2006. Wiley; 2006 [cited 2021 Mar 15]. Available from: https://www.wiley.com/en-us/Longitudinal+Data+Analysis-p-9780471420279
Feingold A. Confidence interval estimation for standardized effect sizes in multilevel and latent growth modeling. J Consult Clin Psychol 2015; 83: 157–168.
https://doi.org/10.1037/a0037721 DOI: https://doi.org/10.1037/a0037721
Hedeker D, Gibbons RD. Application of random-effects pattern-mixture models for missing data in longitudinal studies. Psychol Methods 1997; 2: 64–78. DOI: https://doi.org/10.1037//1082-989X.2.1.64
https://doi.org/10.1037/1082-989X.2.1.64 DOI: https://doi.org/10.1037/1082-989X.2.1.64
Bates D, Mächler M, Bolker B, Walker S. Fitting Linear Mixed-Effects Models Using lme4. J Stat Softw 2015; 67: 1–48.
https://doi.org/10.18637/jss.v067.i01 DOI: https://doi.org/10.18637/jss.v067.i01
Treede R-D, Rief W, Barke A, Aziz Q, Bennett MI, Benoliel R, et al. Chronic pain as a symptom or a disease: the IASP Classification of Chronic Pain for the International Classification of Diseases (ICD-11). Pain 2019; 160: 19–27.
https://doi.org/10.1097/j.pain.0000000000001384 DOI: https://doi.org/10.1097/j.pain.0000000000001384
Reychler G, De Backer M, Piraux E, Poncin W, Caty G. Physical therapy treatment of hypermobile Ehlers–Danlos syndrome: a systematic review. Am J Med Genet A 2021; 185: 2986–2994.
https://doi.org/10.1002/ajmg.a.62393 DOI: https://doi.org/10.1002/ajmg.a.62393
Buryk-Iggers S, Mittal N, Santa Mina D, Adams SC, Englesakis M, Rachinsky M, et al. Exercise and rehabilitation in people with Ehlers-Danlos syndrome: a systematic review. Arch Rehabil Res Clin Transl 2022; 4: 100189.
https://doi.org/10.1016/j.arrct.2022.100189 DOI: https://doi.org/10.1016/j.arrct.2022.100189
Aubry-Rozier B, Schwitzguebel A, Valerio F, Tanniger J, Paquier C, Berna C, et al. Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? Rheumatol Int 2021; 41: 1785–1794.
https://doi.org/10.1007/s00296-021-04968-3 DOI: https://doi.org/10.1007/s00296-021-04968-3
Additional Files
Published
How to Cite
License
Copyright (c) 2024 Peter Molander, Mehmed Novo, Åsa Ringqvist, Andrea Hållstam, Hugo Hesser, Monika Löfgren, Britt-Marie Stålnacke, Björn Gerdle
This work is licensed under a Creative Commons Attribution 4.0 International License.
All digitalized JRM contents is available freely online. The Foundation for Rehabilitation Medicine owns the copyright for all material published until volume 40 (2008), as from volume 41 (2009) authors retain copyright to their work and as from volume 49 (2017) the journal has been published Open Access, under CC-BY-NC licences (unless otherwise specified). The CC-BY-NC licenses allow third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material for non-commercial purposes, provided proper attribution to the original work.
From 2024, articles are published under the CC-BY licence. This license permits sharing, adapting, and using the material for any purpose, including commercial use, with the condition of providing full attribution to the original publication.
Funding data
-
Region Östergötland
Grant numbers RÖ-926131