Hypermobility and EDS

Heard of Hypermobility?

Just because you can do the splits doesn’t mean you’ve got hypermobility, but if you can bend your thumbs back to your wrists, you might surprise yourself!

Hypermobility and Related Disorders (Hypermobility Spectrum Disorder, Ehlers-Danlos Disorder)

What is Joint Hypermobility (JH)?

“Joint hypermobility describes the ability of a joint to move through a larger range of movement than is considered normal, taking into consideration age, gender and ethnicity (1).”

JH can be caused by many factors, both modifiable and non-modifiable, and usually becomes less severe with age (1-2). For some people with JH, it does not lead to injury, pain, or other complaints (1-3). For others, it can cause discomfort and be a part of a disorder or health condition (1). JH can be generalised, meaning it occurs in many joints, or localised to one joint (1). There are several ways to assess JH. For generalised JH, the Brighton score or 5-part Hypermobility Questionnaire is often used (1,4). These are, however, only screening tools and are not diagnostic (1).

From The Ehlers-Danlos Society. (2016). The Ehlers Danlos Society. https://www.ehlers-danlos.com/.

5-year-old to mature age require score >5/9

Under 50 years old require a score of >4/9

Above 50 years old require a score of >3/9

From: (1). Children also confirmed in (5)

What is Hypermobility Spectrum Disorder (HSD)?

This is a term when JH is related to symptoms and does not meet the criteria for hEDS (see below) (1). These symptoms may include pain, subluxations, and dislocations (6).

What is Hypermobile Ehlers-Danlos Syndrome (hEDS)?

The most common subtype out of 13 is named ‘hypermobile subtype of EDS’ (7), which is also a subtype of a group called Heritable Disorders of Connective Tissue (HDCT) (1). Connective tissue is not only found in joints but also in several other systems such the cardiovascular system, dermatological system, and gastrointestinal system (8). hEDS is believed to be the reason for the symptoms affecting multiple systems of the body but the severity of symptoms can vary significantly between individuals (8).

The diagnostic criteria can be found on this checklist.

What is the relationship with injury?

As with many factors that may lead to injury in sport or physical activity, it depends on the individual and the situation. Hypermobility can be a protective factor against injury in some sports while presenting as a risk factor for others (2). A study looked at comparing injury rates between hypermobile and non-hypermobile individuals for various sports and even though the sample size was small, the study demonstrated that overall injury rates were higher for non-hypermobile individuals. Almost unsurprisingly, dislocations were found to be more common in hypermobile individuals (2).

What is the relationship with pain?

Experiencing joint hypermobility is not always associated with pain. In fact, people with generalised joint hypermobility do not experience higher rates of pain (3). However, there does seem to be an association with pain for people diagnosed with HSD and hEDS (4,9). Several theories attempt to explain this relationship, and one such phenomenon known as central sensitisation helps explain pain in HSD and hEDS (4, 10-12). Central sensitisation is when the nervous system becomes hyperalert and can cause pain to occur from a pain-free stimulus or can cause an increase the severity of pain from a minimal-pain stimulus.

Think of an extra-sensitive smoke alarm system. Sometimes it starts to beep with a gust of wind or sets off the building’s sprinkler system when your colleague burns the toast.

It’s important to remember that all pain is personal – it is always influenced by several factors including biophysiological, psychological and social (14).

Is it safe to exercise, play sport, or participate in physical activity?

It is understandable that people with HSD or hEDS, like many others with physical disorders or recovering injuries, are nervous about physical activities. Physical activity can be done safely if some adaptations are made. Having the knowledge and confidence to adapt your movement and continue moving is key. The decision to remain inactive often has far worse consequences (6); don’t let yourself fall into that cycle.

Source van Meulenbroek (12)

What can exercise do to help?

People with hypermobility can benefit from exercise, specifically strength and proprioception exercises (1,15). However, several considerations will need to be made to ensure safety (1). Cardiovascular training is so beneficial for general health, and this is no different for people who are hypermobile.

However, it is imperative that some adaptations are made, especially since POTS (Postural Orthostatic Tachycardia Syndrome which can cause a few symptoms when changing from sitting to standing), post-exercise malaise, and excessive fatigue can be present in people with hypermobility. Health care professionals like your Exercise Physiologist will be able to help you adapt your movement and develop an appropriate and safe program.

What is proprioception?

Proprioception can be explained as the body's ability to sense movement, action, and location. Think of being able to walk through a dark room without losing balance. Exercise has been shown to improve proprioception, including for people that are hypermobile (7). For people with hypermobility, proprioception seems to be lower (7,14), which is theorised to be related to injuries for hypermobile individuals.

What is the role of splinting or braces?

Braces and splinting are a method to limit movement of a joint and can be useful at times for hypermobile joints (1,6,8). After acute injuries, subluxation, or dislocations, these can be useful in allowing recovery (6). However, studies discourage against the use of splints and braces for long-term use or when the joint is not symptomatic (6). Long-term use is theorised to actually decrease the strength of the surrounding muscle and increase dependence (6). These strategies are also thought to play a part in proprioception (8).

Still have questions?

If you still have questions concerning hypermobility, reach out to Exercise Physiologist Sam Bulten at Adapt Movement for a chat!

References

1. Simmonds J. V. (2022). Masterclass: Hypermobility and hypermobility related disorders. Musculoskeletal science & practice, 57, 102465. https://doi.org/10.1016/j.msksp.2021.102465

2. Nathan, J. A., Davies, K., & Swaine, I. (2018). Hypermobility and sports injury. BMJ open sport & exercise medicine, 4(1), e000366. https://doi.org/10.1136/bmjsem-2018-000366

3. Morris, S. L., O'Sullivan, P. B., Murray, K. J., Bear, N., Hands, B., & Smith, A. J. (2017). Hypermobility and Musculoskeletal Pain in Adolescents. The Journal of pediatrics, 181, 213–221.e1. https://doi.org/10.1016/j.jpeds.2016.09.060

4. Kumar, B., & Lenert, P. (2017). Joint Hypermobility Syndrome: Recognizing a Commonly Overlooked Cause of Chronic Pain. The American journal of medicine, 130(6), 640–647. https://doi.org/10.1016/j.amjmed.2017.02.013

5. Tofts, L. J., Simmonds, J., Schwartz, S. B., Richheimer, R. M., O'Connor, C., Elias, E., Engelbert, R., Cleary, K., Tinkle, B. T., Kline, A. D., Hakim, A. J., van Rossum, M. A. J., & Pacey, V. (2023). Pediatric joint hypermobility: a diagnostic framework and narrative review. Orphanet journal of rare diseases, 18(1), 104. https://doi.org/10.1186/s13023-023-02717-2

6. Engelbert, R. and Scheper, M. (2011). Joint hypermobility with and without musculoskeletal complaints: a physiotherapeutic approach. International Musculoskeletal Medicine, 33(4), pp.146–151. doi:https://doi.org/10.1179/175361511x13088377677319

7. Reychler, G., De Backer, M. 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. https://doi.org/10.1002/ajmg.a.62393

8. Russek, L. N., Stott, P., & Simmonds, J. (2019). Recognizing and Effectively Managing Hypermobility-Related Conditions. Physical therapy, 99(9), 1189–1200. https://doi.org/10.1093/ptj/pzz078

9. 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. https://doi.org/10.1016/j.apmr.2016.02.015

10. De Wandele, I., Colman, M., Hermans, L., Van Oosterwijck, J., Meeus, M., Rombaut, L., Brusselmans, G., Syx, D., Calders, P., & Malfait, F. (2022). Exploring pain mechanisms in hypermobile Ehlers-Danlos syndrome: A case-control study. European journal of pain (London, England), 26(6), 1355–1367. https://doi.org/10.1002/ejp.1956

11. Di Stefano, G., Celletti, C., Baron, R., Castori, M., Di Franco, M., La Cesa, S., Leone, C., Pepe, A., Cruccu, G., Truini, A., & Camerota, F. (2016). Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. European journal of pain (London, England), 20(8), 1319–1325. https://doi.org/10.1002/ejp.856

12. van Meulenbroek, T., Huijnen, I. P. J., Simons, L. E., Conijn, A. E. A., Engelbert, R. H. H., & Verbunt, J. A. (2020). Exploring the underlying mechanism of pain-related disability in hypermobile adolescents with chronic musculoskeletal pain. Scandinavian journal of pain, 21(1), 22–31. https://doi.org/10.1515/sjpain-2020-0023

13. Louw, A., Puentedura, E. J., Diener, I., Zimney, K. J., & Cox, T. (2019). Pain neuroscience education: Which pain neuroscience education metaphor worked best?. The South African journal of physiotherapy, 75(1), 1329. https://doi.org/10.4102/sajp.v75i1.1329

14. Baeza-Velasco, C., Bulbena, A., Polanco-Carrasco, R., & Jaussaud, R. (2019). Cognitive, emotional, and behavioral considerations for chronic pain management in the Ehlers-Danlos syndrome hypermobility-type: a narrative review. Disability and rehabilitation, 41(9), 1110–1118. https://doi.org/10.1080/09638288.2017.1419294

15. Buryk-Iggers, S., Mittal, N., Santa Mina, D., Adams, S. C., Englesakis, M., Rachinsky, M., Lopez-Hernandez, L., Hussey, L., McGillis, L., McLean, L., Laflamme, C., Rozenberg, D., & Clarke, H. (2022). Exercise and Rehabilitation in People With Ehlers-Danlos Syndrome: A Systematic Review. Archives of rehabilitation research and clinical translation, 4(2), 100189. https://doi.org/10.1016/j.arrct.2022.100189

This blog was written by Samuel Bulten