HSD and EDS Research
73 total studies as of April 17, 2022
For navigation, click on the topic you are interested in below:
Introduction to EDS and Subtypes
HYPERMOBILITY TYPE DIAGNOSTIC CRITERIA (2017)
Tinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017). Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 48–69. Portico.
This article reviews the new diagnostic criteria for Hypermobile Ehlers Danlos Syndrome. It shares information about how hEDS affects various body systems, along with some treatment evaluations and suggestions.
Summary by Kimberly Czotter
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THE 2017 DIAGNOSTIC CRITERIA FOR ALL EDS SUBTYPES
Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L., Bowen, J. M., Brady, A. F., Burrows, N. P., Castori, M., Cohen, H., Colombi, M., Demirdas, S., De Backer, J., De Paepe, A., Fournel-Gigleux, S., Frank, M., Ghali, N., … Tinkle, B. (2017). The 2017 international classification of the Ehlers-Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 8–26. Portico.
This article reviews the new diagnostic criteria for the 13 subtypes of Ehlers Danlos Syndrome.
Summary by Kimberly Czotter
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VASCULAR EDS REVIEW
Frank, M., Adham, S., Seigle, S., Legrand, A., Mirault, T., Henneton, P., Albuisson, J., Denarié, N., Mazzella, J.-M., Mousseaux, E., Messas, E., Boutouyrie, P., & Jeunemaitre, X. (2019). Vascular Ehlers-Danlos Syndrome. Journal of the American College of Cardiology, 73(15), 1948–1957.
This article reviews the diagnostic criteria for Vascular Ehlers Danlos Syndrome. It shares information about how vEDS affects various body systems, along with some treatment evaluations and suggestions.
Summary by Kimberly Czotter
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CLASSICAL EDS REVIEW
Bowen, J. M., Sobey, G. J., Burrows, N. P., Colombi, M., Lavallee, M. E., Malfait, F., & Francomano, C. A. (2017). Ehlers-Danlos syndrome, classical type. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 27–39. Portico.
This article reviews the diagnostic criteria for Classic Ehlers Danlos Syndrome. It shares information about how cEDS affects various body systems, along with some treatment evaluations and suggestions.
Summary by Kimberly Czotter
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THE DIAGNOSTIC JOURNEY OF EDS
Halverson, C. M. E., Clayton, E. W., Garcia Sierra, A., & Francomano, C. (2021). Patients with Ehlers–Danlos syndrome on the diagnostic odyssey: Rethinking complexity and difficulty as a hero’s journey. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. Portico. https://doi.org/10.1002/ajmg.c.31935
The authors conducted 22 detailed interviews of hEDS patients' diagnostic journeys, comparing them to the "hero's journey." They expressed difficulties with clinicians, symptoms, and psychosocial consequences.
Summary by Kimberly Czotter
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VASCULAR EDS AND SPONTANEOUS ARTERY DISSECTION
Adham, S., Billon, C., Legrand, A., Domigo, V., Denarié, N., Charpentier, E., Jeunemaitre, X., & Frank, M. (2021). Spontaneous Cervical Artery Dissection in Vascular Ehlers-Danlos Syndrome. Stroke, 52(5), 1628–1635. https://doi.org/10.1161/strokeaha.120.032106
"This retrospective review studied a vEDS patient cohort from 2000 to 2017 - analyzing a total of 144 patients. The authors found that 82 patients had supra-aortic trunk lesions with a total of 183 lesions (132 dissections, 33 aneurysms) in internal carotid and vertebral arteries and transient ischemic attack or stroke in 16 of these 82 patients. vEDS patients frequently experience artery lesions that are mostly asymptomatic.
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Summary by Kimberly Czotter
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COMPARING HSD AND HEDS
Aubry-Rozier, B., Schwitzguebel, A., Valerio, F., Tanniger, J., Paquier, C., Berna, C., Hügle, T., & Benaim, C. (2021). Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? Rheumatology International, 41(10), 1785–1794. https://doi.org/10.1007/s00296-021-04968-3
This study investigated the differences between 97 hEDS (61) and HSD (36) patients in terms of systemic manifestations (bone involvement, neuropathic pain, MCAS) and outcomes following physical therapy at baseline, 6 months, and 1 year. They found that hEDS and HSD patients scored similarly, and had improvements following physical therapy.
Summary by Kimberly Czotter
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ANIMAL MODELS OF EDS TYPES
Vroman, R., Malfait, A.-M., Miller, R. E., Malfait, F., & Syx, D. (2021). Animal Models of Ehlers–Danlos Syndromes: Phenotype, Pathogenesis, and Translational Potential. Frontiers in Genetics, 12.
The authors discuss research in non-human animals demonstrating EDS phenotypes: mice, zebrafish, and domesticated species.They argue that these cross-species models have importantly discerned functions and roles of proteins during collagen biosynthesis and/or fibrillogenesis. These models have shown several clinical similarities with corresponding EDS subtypes: dermatological, similar tissue composition, cardiovascular, musculoskeletal and ocular. They argue that animal models are important to demonstrate pathophysiological aspects of EDS and are critical in EDS research regarding signaling pathways and therapeutic treatments.
Summary by Kimberly Czotter
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VEDS AND ENDOPLASMIC RETICULUM AND COLLAGEN
Ishikawa, S., Kosho, T., Kaminaga, T., Miyamoto, M., Hamasaki, Y., Yoshihara, S., Hayashi, S., & Igawa, K. (2021). Endoplasmic reticulum stress and collagenous formation anomalies in vascular‐type Ehlers–Danlos syndrome via electron microscopy. The Journal of Dermatology, 48(4), 481–485. Portico. https://doi.org/10.1111/1346-8138.15766
The authors evaluated collagen fibers and the endoplasmic reticulum in vEDS patients. They found that the expression of activating transcription factor 6 (ATF6) [a stress marker] was higher in those with vEDS.
Summary by Kimberly Czotter
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HEDS REVIEW
Gensemer, C., Burks, R., Kautz, S., Judge, D. P., Lavallee, M., & Norris, R. A. (2020). Hypermobile Ehlers‐Danlos syndromes: Complex phenotypes, challenging diagnosis, and poorly understood causes. Developmental Dynamics, 250(3), 318–344. Portico.
The authors discuss the current pathogenic, molecular, genetic, and epidemiologic findings related to hEDS.
Summary by Kimberly Czotter
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A MOUSE MODEL FOR mcEDS
Hirose, T. et al. (2020). Systematic investigation of the skin in Chst14−/− mice: A model for skin fragility in musculocontractural Ehlers–Danlos syndrome caused by CHST14 variants (mcEDS-CHST14). Glycobiology, 31(2), 137–150.
The authors discuss a mice model demonstrating the mcEDS phenotype.
Summary by Kimberly Czotter
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CEDS GENETIC PROBOUNDS IN 65 RELATIVES
Colman, M., Syx, D., De Wandele, I., Dhooge, T., Symoens, S., & Malfait, F. (2021). Clinical and molecular characteristics of 168 probands and 65 relatives with a clinical presentation of classical Ehlers–Danlos syndrome. Human Mutation, 42(10), 1294–1306. Portico. https://doi.org/10.1002/humu.24258
This article evaluates both clinical and molecular characteristics of 65 relatives with cEDS. They found the majority of collagen deficits were related to Type V collagen and that 85.6% of molecularly confirmed cEDS patients demonstrated the two major clinical criteria but that there was significant phenotypic variability. They found vascular complications were rare, and that 13.9% of participants had a different genetic defect (COL1A1, PLOD1, TNXB, AEBP1).
Summary by Kimberly Czotter
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hEDS REVIEW (2020)
Gensemer, C., Burks, R., Kautz, S., Judge, D. P., Lavallee, M., & Norris, R. A. (2020). Hypermobile Ehlers‐Danlos syndromes: Complex phenotypes, challenging diagnosis, and poorly understood causes. Developmental Dynamics, 250(3), 318–344. https://doi.org/10.1002/dvdy.220
This review discusses hEDS and its history, epidemiology, diagnostic criteria, genetics, molecular biology (collagen fibers, ECM, gene expression), management, and phenotype (symptoms) of most body systems. The authors also argue there is a lack of knowledge in many of these areas, suggesting future studies must be completed.
Summary by Kimberly Czotter
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Life Quality
Understanding how EDS and hypermobility affect the quality of life of patients.
A QUALITATIVE REVIEW OF LIVING WITH HYPERMOBILE EDS
Sætre, E., & Eik, H. (2019). Flexible bodies—Restricted lives: A qualitative exploratory study of embodiment in living with joint hypermobility syndrome/Ehlers‐Danlos syndrome, hypermobility type. Musculoskeletal Care, 17(3), 241–248. Portico.
This study evaluated the quality of life of patients with hEDS through detailed interviews. They found that all participants noticed their hypermobility from a young age, sharing that others found their bodies "strange." They shared common features of pain and fatigue from daily tasks. Finally, the participants described their bodies and related symptoms in negative terms (frustration, anger, grief, and distress). This study involved only 7 Norwegian participants as a way to demonstrate a need for further research in the quality of life of EDSers.
Summary by Kimberly Czotter
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PREDICTING PAIN AND MOBILITY-RELATED DISABILITY IN HEDS
Kalisch, L., Hamonet, C., Bourdon, C., Montalescot, L., de Cazotte, C., & Baeza-Velasco, C. (2019). Predictors of pain and mobility disability in the hypermobile Ehlers-Danlos syndrome. Disability and Rehabilitation, 42(25), 3679–3686.
This study interviewed 75 Parisian adults with hEDS and quality of life regarding psychological, health (pain, fatigue, diagnostic delay, BMI, mobility disability) and socio-demographics. They found that 43% suffer from severe pain and that 66% have a high level mobility disability. From this, they demonstrate that diagnostic delay, working, and thoughts of helplessness increased the incidence of severe pain. Age, fatigue, and BMI served as predictors of mobility disability. This study confirms research that found a high disease burden in hEDS patients, and the authors suggest multidisciplinary health management: nutritional, psychological, and physical.
Summary by Kimberly Czotter
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CHRONIC FATIGUE IN HEDS
" Hakim, A., De Wandele, I., O’Callaghan, C., Pocinki, A., & Rowe, P. (2017). Chronic fatigue in Ehlers-Danlos syndrome-Hypermobile type. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 175–180. Portico. "
The authors reviewed various studies concerning Hypermobile Ehlers-Danlos Syndrome and chronic fatigue syndrome (CFS). They argue that hEDS and CFS have overlapping symptoms as many hEDS-related problems commonly contribute to fatigue: sleep disorders, chronic pain, deconditioning, cardiovascular autonomic dysfunction, bowel and bladder dysfunction, psychological issues, and nutritional deficiencies. They argue for ongoing medical evaluation and discuss different treatment options such as medication, physical therapy, maintaining function and providing social, physical, and nutritional support
Summary by Kimberly Czotter
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MANAGING PAIN AND FATIGUE IN HEDS
Castori, M., Morlino, S., Celletti, C., Celli, M., Morrone, A., Colombi, M., Camerota, F., & Grammatico, P. (2012). Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome, hypermobility type): Principles and proposal for a multidisciplinary approach. American Journal of Medical Genetics Part A, 158A(8), 2055–2070.
The authors discuss hypermobile Ehlers–Danlos syndrome (hEDS) [diagnosed using outdated criteria] and its related chronic fatigue and pain: articular, chronic, muscular, neuropathic, back, neck, abdominal, pelvic, and headache. The authors suggest that fatigue may stem from muscle weakness, respiratory insufficiency, unrefreshing sleep, dysautonomia, intestinal malabsorption, reactive depression/anxiety, and excessive use of analgesics. They discuss the benefits of various treatments including lifestyle changes, physiotherapy and medication.
Summary by Kimberly Czotter
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CHRONIC PAIN IN HSD AND HEDS
Scheper, M., de Vries, J. E., Verbunt, J., & Engelbert, R. H. H. (2015). Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type): it is a challenge. Journal of Pain Research, 591.
The authors explore chronic pain in relation to hEDS and HSD. They identify three major challenges of research and care in this population: there is a diverse clinical presentation, there is a lack of understanding regarding the development of chronic pain, and that these patients have a high prevalence of psychosocial factors affecting their health. They offer solutions to these challenges. *Uses outdated hEDS diagnostic criteria*
Summary by Kimberly Czotter
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LIFE QUALITY IN EDS
Berglund, B., Pettersson, C., Pigg, M., & Kristiansson, P. (2015). Self-reported quality of life, anxiety and depression in individuals with Ehlers-Danlos syndrome (EDS): a questionnaire study. BMC Musculoskeletal Disorders, 16(1).
This study evaluates the incidence of anxiety and quality of life of individuals with Ehlers Danlos Syndrome living in Sweden through use of a survey. They had 250 responses, and used two validated questionnaires: the Hospital Anxiety and Depression Scale (HADS) and SF-36, They found that participants had high prevalence of anxiety (74.8%) which correlated with age, tiredness and back pain. The EDS group also showed higher rates of depression and a significantly lower quality of health than the general population.
Summary by Kimberly Czotter
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DISABILITY PREVALENCE IN HYPERMOBILITY
Scheper, M. C., Juul-Kristensen, B., Rombaut, L., Rameckers, E. A., Verbunt, J., & Engelbert, R. H. (2016). Disability in Adolescents and Adults Diagnosed With Hypermobility-Related Disorders: A Meta-Analysis. Archives of Physical Medicine and Rehabilitation, 97(12), 2174–2187.
The authors tried to establish the most common symptoms related to disability in Hypermobile Ehlers-Danlos Syndrome (hEDS) by conducting a meta-analysis on 21/714 available publications. They also communicated the effectiveness of treatments for hEDS patients, but could not determine whether treatments were able to effectively reduce disability. *Uses outdated hEDS diagnostic criteria *
Summary by Kimberly Czotter
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EMPLOYMENT AND HEDS
De Baets, S., Calders, P., Verhoost, L., Coussens, M., Dewandele, I., Malfait, F., Vanderstraeten, G., Van Hove, G., & Van de Velde, D. (2019). Patient perspectives on employment participation in the “hypermobile Ehlers–Danlos syndrome.” Disability and Rehabilitation, 43(5), 668–677.
The study explored the employment experiences of nine patients with hEDS through purposive interviews. They found three major themes: (1) elements assisting employment, (2) limitations in employment, and (3) unemployment due to hEDS.They found that hEDS patients worried about work pressure, tasks, and transportation; but that employment also had positive effects on mental health.
Summary by Kimberly Czotter
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SUICIDALITY AND HEDS
Baeza-Velasco, C., Hamonet, C., Montalescot, L., & Courtet, P. (2021). Suicidal Behaviors in Women With the Hypermobile Ehlers–Danlos Syndrome. Archives of Suicide Research, 1–13. https://doi.org/10.1080/13811118.2021.1885538
The authors interviewed 35 women with hEDS regarding their pain, BMI, diagnostic delay, psychosocial variables, and suicidal behaviours. They found that there is a high frequency of suicide-related behaviours in this group - likely related to chronic pain, disability, and psychological stress.
Summary by Kimberly Czotter
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HEDS AND HSD: PSYCHOLOGICAL IMPACTS
Bulbena‐Cabré, A., Baeza‐Velasco, C., Rosado‐Figuerola, S., & Bulbena, A. (2021). Updates on the psychological and psychiatric aspects of the Ehlers–Danlos syndromes and hypermobility spectrum disorders. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. Portico. https://doi.org/10.1002/ajmg.c.31955
This literature review evaluates articles regarding the psychopathological conditions associated with EDS, and EDS symptoms in psychiatric conditions. It confirms that EDS-related psychological processes (ex fear, distress, negative emotions) significantly impact patient outcomes. They found systemic associations between anxiety disorders and EDS and correlations with neurodevelopmental, eating, mood, and sleep disorders.
Summary by Kimberly Czotter
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USING PAIN DRAWING TO DIAGNOSE EDS AND GBS
Wester, L., Mücke, M., Bender, T. T. A., Sellin, J., Klawonn, F., Conrad, R., & Szczypien, N. (2020). Pain drawings as a diagnostic tool for the differentiation between two pain-associated rare diseases (Ehlers-Danlos-Syndrome, Guillain-Barré-Syndrome). Orphanet Journal of Rare Diseases, 15(1). https://doi.org/10.1186/s13023-020-01542-1
This prospective study evaluated 92 patients with EDS (60) or Guillain-Barré Syndrome (32). They aimed to understand whether pain drawings on a map of the human body could help differentiate the diagnosis between EDS and GBS. They found that the patients marked different areas, leading to high diagnostic accuracy in both groups.
Summary by Kimberly Czotter
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PSYCHOLOGICAL IMPACTS OF HSD AND hEDS
Bennett, S. E., Walsh, N., Moss, T., & Palmer, S. (2019). Understanding the psychosocial impact of joint hypermobility syndrome and Ehlers–Danlos syndrome hypermobility type: a qualitative interview study. Disability and Rehabilitation, 43(6), 795–804. https://doi.org/10.1080/09638288.2019.1641848
The authors conducted 17 interviews (14 women, 3 men) to better understand psychosocial factors in HSD and hEDS. They examined lived experiences and effective coping. They found five key themes: a restricted life, healthcare limitations, a lack of awareness among healthcare professionals, social stigma, fear of the unknown, and coping methods. They found that participants had shame, guilt, difficulties with romantic relationships, and difficulty finding reliable information.
Summary by Kimberly Czotter
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CHRONIC PAIN AND HYPERMOBILITY
Scheper, M., de Vries, J. E., Verbunt, J., & Engelbert, R. H. H. (2015). Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type): it is a challenge. Journal of Pain Research, 591. https://doi.org/10.2147/jpr.s64251
EDS diagnoses are often overlooked and often not even considered due to lack of awareness and the absence of objective diagnostic markers leaving patients to search for years within the medical system before a diagnosis is established. This article presents suggestions for creating a standardized diagnostic criteria.
Summary by Jennifer Stacy
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EDS and Contraindications
Treatments, medications, and more that may aggravate EDS.
ANESTHESIA RESISTANCE IN EDS PATIENTS
Schubart, J. R., Schaefer, E., Janicki, P., Adhikary, S. D., Schilling, A., Hakim, A. J., Bascom, R., Francomano, C. A., & Raj, S. R. (2019). Resistance to local anesthesia in people with the Ehlers-Danlos Syndromes presenting for dental surgery. Journal of Dental Anesthesia and Pain Medicine, 19(5), 261. https://doi.org/10.17245/jdapm.2019.19.5.261
Due to anecdotal reports of EDS patients experiencing dental pain due to local anesthetic not working, these researchers gave a survey to 1243 EDS (988) and non-EDS (255) individuals regarding their self-reported rates of local anesthetic efficacy of lidocaine, mepivacaine, bupivacaine, articaine, and procaine. They found 88% of EDS patients did not have adequate pain prevention, compared to 33% of non-EDS respondents.
Summary by Kimberly Czotter
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Treatment Options for EDS and HSD
Broken up into treatments and physical therapy headings.
COMPRESSION GARMENTS FOR hEDS (SHOULDER STABILITY AND MUSCLE STRENGTH)
Chaléat-Valayer, E., Denis, A., Zelmar, A., Pujol, A., Bernadou, A., Bard-Pondarré, R., & Touzet, S. (2020). VETCOSED study: efficacy of compressive garments for patients with hypermobile Ehlers–Danlos syndrome on shoulder stability and muscles strength. Disability and Rehabilitation, 1–8. https://doi.org/10.1080/09638288.2020.1860142
This quasi-experimental study assessed the effects on shoulder stability and rotator muscles by treating hEDS patients with a measured compressive short-sleeve jacket. These 36 patients wore these jackets for a mean of 7.8 hours/day which improved their arthralgia (joint pain), instability, and function and decreased their incidence of dislocation and subluxation.
Summary by Kimberly Czotter
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POSITIVE PSYCHOLOGY AND EDS
Kalisch, L., Boniwell, I., Osin, E., & Baeza-Velasco, C. (2021). Feeling Good Despite EDS: The Effects of a 5-Week Online Positive Psychology Programme for Ehlers–Danlos-Syndromes Patients. Journal of Contemporary Psychotherapy.
This controlled study of 132 EDS patients evaluated their fatigue, pain, disability, and satisfaction following a 5-week positive psychology program. They measured these variables before the start of the program, 6 weeks after, and one month later.
Summary by Kimberly Czotter
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DIETARY INTERVENTION FOR POTS AND HSD
Do, T., Diamond, S., Green, C., & Warren, M. (2021). Nutritional Implications of Patients with Dysautonomia and Hypermobility Syndromes. Current nutrition reports, 10(4), 324–333. https://doi.org/10.1007/s13668-021-00373-1
"This review investigates dietary interventions that have helped reduce symptoms and improve quality of life within patients with POTS, hypermobility, and hEDS. They discuss demographics, symptom overlap, GI symptoms, and nutritional management. They argue that evidence suggests including probiotic and prebiotic-rich foods, fiber supplementation, salt, high fluid intake, and supplement use.
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Summary by Kimberly Czotter
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OCCUPATIONAL THERAPY WITH EDS AND POTS PATIENTS
Levine, D., Work, B., McDonald, S., Harty, N., Mabe, C., Powell, A., & Sanford, G. (2021). Occupational Therapy Interventions for Clients with Ehlers-Danlos Syndrome (EDS) in the Presence of Postural Orthostatic Tachycardia Syndrome (POTS). Occupational Therapy In Health Care, 1–18. https://doi.org/10.1080/07380577.2021.1975200
The authors discuss occupational therapy to treat patients with EDS and PoTS to improve their well-being, symptoms, employability, participation in daily life, and improve overall quality of life. These interventions include environmental modifications, adaptive equipment, orthoses, exercises, pacing/energy conservation, sleep routines, and habit development.
Summary by Kimberly Czotter
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INCREASING TENDON SYNTHESIS RATE IN cEDS WITH GROWTH FACTOR
Nielsen, R. H., Holm, L., Jensen, J. K., Heinemeier, K. M., Remvig, L., & Kjaer, M. (2014). Tendon protein synthesis rate in classic Ehlers-Danlos patients can be stimulated with insulin-like growth factor-I. Journal of Applied Physiology, 117(7), 694–698. https://doi.org/10.1152/japplphysiol.00157.2014
This preliminary controlled study investigated cEDS patients (n=5) and healthy controls (n=10) to see whether they could stimulate the protein synthesis rate in tendons and skin following an injection of insulin-like growth factor-I (IGF-I). At 24 hr and 6 hr prior to sampling, they injected one patellar tendon with 0.1mL of IGF-I and the contralateral tendon with 0.1mL isotonic saline (control). They sound that cEDS and healthy participants had similar baseline protein synthesis rates; but that following injection, the cEDS tendon synthesis rates increased significantly compared to controls (~ 7x the rate).
Summary by Kimberly Czotter
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CURRENT TREATMENT OPTIONS FOR EDS
Song, B. et al. (2020). Ehlers-Danlos Syndrome: An Analysis of the Current Treatment Options. Pain Physician, 4;23(7;4), 429–438. https://doi.org/10.36076/ppj.2020/23/429
The authors explore the currently available treatments and efficacy with a retrospective study at a rehabilitation and physical medicine clinic. They discuss 98 EDS patients seen between Jan 2015-April 2019, finding that the most common treatments included alternative medicine (n=88), although bracing and occupational therapy were most effective (70% improved). The use of neuropathic modulators often led to adverse effects (47% of users), and there were only a few men in the study so it is unclear if there may be effects of hormones. The authors discuss there is a lack of research in this area, especially randomized, controlled studies - making it difficult for patients and providers. *Some EDS patients would not meet the new 2017 diagnostic criteria*
Summary by Kimberly Czotter
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TREATING HYPERMOBILITY AND EDS WITH PROLOTHERAPY
Hauser, R. A. & Phillips, H. J. (2011). Treatment of Joint Hypermobility Syndrome, Including Ehlers-Danlos Syndrome, with Hackett-Hemwall Prolotherapy. Journal of Prolotherapy, 3(2), 612-629.
This article review discusses hypermobility, traditional treatments, and the role of prolotherapy for pain management. Prolotherapy works by initiating inflammation, leading to a cascade of generation for collagen and extracellular matrix to increase connective tissue strength. It has a high safety rating, can treat various joints, is outpatient, cost effective, relieves symptoms quickly, and helps stabilize joints. The authors suggest that this treatment is also helpful in preventing further degeneration that leads to complications like osteoarthritis.
Summary by Kimberly Czotter
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Physical Therapy
PHYSICAL THERAPY FOR hEDS
Reychler, G., De Backer, M., Piraux, E., Poncin, W., & Caty, G. (2021). Physical therapy treatment of hypermobile Ehlers–Danlos syndrome: A systematic review. American Journal of Medical Genetics Part A, 185(10), 2986–2994. Portico. https://doi.org/10.1002/ajmg.a.62393
This systematic review investigated physiotherapy's effects on hEDS. They found 1045 studies, with only 6 of them being randomized controlled trials that were included in the review. The treatment plans were from 4-8 weeks, and demonstrated significant improvements in proprioception, pain and quality of life.
Summary by Kimberly Czotter
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PHYSICAL THERAPY FOR HSD AND hEDS
Engelbert, R. H. H., Juul-Kristensen, B., Pacey, V., de Wandele, I., Smeenk, S., Woinarosky, N., Sabo, S., Scheper, M. C., Russek, L., & Simmonds, J. V. (2017). The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 158–167. Portico.
This physician guide covers diagnostic criteria, prevalence, clinical presentation, and management of hEDS. The authors argue there is limited evidence-based research because of small sample sizes and poor quality and suggest following theoretical treatments. *Diagnostic criteria is outdated*
Summary by Kimberly Czotter
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REHABILITATION AND hEDS
Corrado, B., & Ciardi, G. (2018). Hypermobile Ehlers-Danlos syndrome and rehabilitation: taking stock of evidence based medicine: a systematic review of the literature. Journal of Physical Therapy Science, 30(6), 843–847.
The authors completed a systematic review of hEDS and physiotherapy - including 1 out of 1869 screen papers. This single paper studied physio and CBT but was of poor quality with the authors suggesting there is a lack of evidence based literature on hEDS rehabilitation.
Summary by Kimberly Czotter
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EXERCISE BELIEFS AND BEHAVIOURS IN hEDS
Simmonds, J. V., Herbland, A., Hakim, A., Ninis, N., Lever, W., Aziz, Q., & Cairns, M. (2017). Exercise beliefs and behaviours of individuals with Joint Hypermobility syndrome/Ehlers–Danlos syndrome – hypermobility type. Disability and Rehabilitation, 41(4), 445–455.
This cross-sectional questionnaire explored physiotherapy experiences and exercise beliefs and behaviours of 946 individuals with hEDS/HSD. They found that individuals that saw physiotherapists or had beliefs that exercise is important for management were more likely to exercise weekly. *Uses outdated diagnostic criteria*
Summary by Kimberly Czotter
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Surgical Considerations for EDS
A discussion of anesthetics and appropriate surgery procedures in EDS patients.
ANESTHESIA RESISTANCE IN EDS PATIENTS
Schubart, J. R., Schaefer, E., Janicki, P., Adhikary, S. D., Schilling, A., Hakim, A. J., Bascom, R., Francomano, C. A., & Raj, S. R. (2019). Resistance to local anesthesia in people with the Ehlers-Danlos Syndromes presenting for dental surgery. Journal of Dental Anesthesia and Pain Medicine, 19(5), 261. https://doi.org/10.17245/jdapm.2019.19.5.261
Due to anecdotal reports of EDS patients experiencing dental pain due to local anesthetic not working, these researchers gave a survey to 1243 EDS (988) and non-EDS (255) individuals regarding their self-reported rates of local anesthetic efficacy of lidocaine, mepivacaine, bupivacaine, articaine, and procaine. They found 88% of EDS patients did not have adequate pain prevention, compared to 33% of non-EDS respondents.
Summary by Kimberly Czotter
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EDS PATIENTS AND SURGICAL CARE
Chopra, P., & Bluestein, L. (2020). Perioperative Care in Patients with Ehlers Danlos Syndromes. Open Journal of Anesthesiology, 10(01), 13–29. https://doi.org/10.4236/ojanes.2020.101002
EDS is associated with lax ligaments, weak connective tissue, surgical intervention, and a multitude of co-existing conditions. This review article addresses anesthetic considerations for EDS, and the authors found a lack of articles on this topic. They suggest future research and anesthetic techniques being modified specifically to the EDS and co-morbid conditions. They note that all EDS patients are at higher risk of subluxation of joints, TMJ subluxation, spontaneous pneumothorax, tracheomalacia, obstructive sleep apnea, insensitivity to anesthesia, coagulation disorders, POTS, and MCAS and they suggest methods to reduce surgery risks.
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EDS: ANESTHESIA AND SURGICAL MANAGEMENT
Wiesmann, T., Castori, M., Malfait, F., & Wulf, H. (2014). Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet Journal of Rare Diseases, 9(1). https://doi.org/10.1186/s13023-014-0109-5
The review of anesthesia and EDS found that the majority focus on the vascular subtype, case studies, and that the knowledge is often fragmented and theoretical. The authors summarize different recommendations for regional, general, and obstetrical anesthesia.
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