#joint hypermobility syndrome

[supernutcollectionuniverse]

One unexpected side effect of physical therapy is that my arms are kind of shredded now?!

If you have hyper mobility issues then I am begging you on my hands and knees to start pt.

[stonebutchooze]

chronic pain is like

INTERNAL SCREAMING

oh no they're gonna think I'm too young to use a walking stick. they'll think I'm faking. oh god

MORE INTERNAL SCREAMING

where did my walking stick velcro go?????

MORE INTERNAL SCREAMING

hmm overtime in a manual job will kill me

so on and so forth

[weirdbrainweirdbody]

Being newly discovered disabled is fun because I'll see a limited resource and be like 'oh, that would be helpful! But I should probably leave it for disabled people who need it'

'oh wait. I am disabled people who need it'

[finned-fish]

My body: Joint hypermobility syndrome :v

My autism: No >:3

My shoulders: guys please stop fighting over me.

[dontdrinkgermx]

the hypermobile experience is having your fingers feel a little stiff, so you pop them and immediately regret it because now there's a sharp pain radiating through your fingers and it won't go away :D

[oh-heck-yeah-doritos]

Walking home in fancy shoes bcs I was at a music concert and FUCK does my ankle hurt

[human-timelord]

Trying very hard to accept that i have chronic pain and might be disabled even though i dont "look" it

[chernobyl]

[supernutcollectionuniverse]

Hate how compression socks help me manage my pain so much when they’re so swagless

[stonebutchooze]

reminder to self: PHYSIO HELPS YOU NOT BE IN PAIN!!!! DO IT EVERYDAY!!! IF YOU'RE IN PAIN BUT FEEL UP TO IT, IT COULD HELP FASTER REDUCE THE PAIN!!!! EVEN IF YOU DONT DO ALL THE SETS!!!!

[random-nerd-queer]

Gotta love when you overexert yourself and end up collapsed on the floor

FROM DOING DISHES!!!!

[obfuscated-abstract]

Title: Hypermobility, the Ehlers-Danlos syndromes and chronic pain

Date: September 2017 Published in: Clinical and Experimental Rheumatology Publicly available? Yes (pdf will download)

Citation: Syx, D., De Wandele, I., Rombaut, L., & Malfait, F. (2017). Hypermobility, the ehlers-danlos syndromes and chronic pain. Clin Exp Rheumatol, 35 (5), 116–122.

Abbreviations:

ECM: extracellular matrix

EDS: Ehlers-Danlos syndrome (lowercase letters in front indicate the subtype; e.g., hypermobile EDS = hEDS)

GJH: generalised joint hypermobility

HSD: hypermobility spectrum disorder

JH: joint hypermobility

JHS: joint hypermobility syndrome

MSK: musculoskeletal

Article Summary

Abstract

Hypermobility, joint hypermobility syndrome, and Ehlers-Danlos syndromes

Joint hypermobility (JH) is a range of movement in a joint that exceeds what is normal (accounting for the age, gender, and ethnicity). JH can be localised or generalised with GJH being defined by a score of at least 5 on the Beighton scale.

JH has a strong genetic basis and is affected by gender, age, and ethnicity but can also be affected by environmental factors including weight, training, trauma, surgery, and medical conditions.

JH does not necessarily lead to symptoms and is not a disease or permanent diagnosis

The history and historical classifications related to GJH are detailed and the overlap with connective tissue disorders including Marfan syndrome, osteogenesis imperfecta, and notably Ehlers-Danlos syndromes (EDS)

EDS is a group of disorders that mainly affect the soft connective tissues but that are varied in their genetic and phenotypic (observable characteristics) presentations. Major characteristics include skin hyperextensibility and fragility; vascular fragility with easy bruisability and a variable bleeding tendency; joint hypermobility (usually generalised) and manifestations of generalised connective tissue fragility. The severity of symptoms can be varied.

There are now 13 EDS subtypes (expanded from the 6 originally identified in 1997) with molecular defects identified in 19 different genes. The effects are briefly discussed and Table III details these defects.

One of the most prevalent subtypes of EDS, hypermobile EDS (hEDS), is still molecularly unexplained. hEDS shows considerable overlap with JHS and it has been considered that they may be the same and/or can be used interchangeably. Hypermobility syndrome and JHS have therefore been combined into the single HSD diagnosis to be used for patients with symptomatic JH who do not meet the new criteria for hEDS or another form of EDS. This review uses JHS instead of HSD as it is a review of old literature (consider them interchangeable).

The natural history of pain in JHS and EDS

The compromised structural integrity of connective tissues, people with EDS and JHS are prone to joint instability, and as a result, joint disclocations (full displacements and subluxations), and articular and soft tissue injuries. This commonly causes acute and reccurent pain which can evolve to chronic widespread musculoskeletal (MSK) pain.

Approximately 3.3% of women and 0.6% of men experience chronic pain but these numbers are likely an underestimation since clinicians are generally unaware of GJH-related pain. More recent studies have found incidences of chronic pain over 90% in hEDS patients -- higher than that in cEDS. Clinicians report that pain is the primary reason fro seeking medical help and the most frequent complaint but more studies are needed.

Most studies focus on hEDS and many do not distinguish between hEDS and JHS.

Pain usually starts in early life and is often triggered by an apparent external factor such as an injury, surgery, psychological distress, or by a comorbidity. The pain is initially felt as an acute and localised symptom or as 'growing pains' related to the trauma. Muscle cramps, pariarticular inflammation, enthesopathies, and nerve entrapment syndromes can add to the localised MSK pain.

The pain later becomes widespread, most commonly presenting as arthralgias and myalgias (joint and muscle pains) in young adult and adult patients. The pain is most frequently localised in the neck, shoulders, forearms, fingers, hips, knees, and feet and may, at first, be localised to a few joints or muscles with a migratory pattern. The pain will gradually become more widespread and additional symptoms including burning sensations, peripheral paresthesias, generalised hyperalgesia, allodynia, and hypersensitivity to various stimuli (e.g., light, sound, and odors) become apparent. Pain in non-articular regions (e.g., headaches, gastro-intestinal, pelvic) as well as fatigue often appear. The underlying cause of the fatigue is unclear.

The pain is generally resistant to the use of analgesics (painkillers), surgery, and physical therapy. This can have a negative effect on physical, social, and emotional wellbeing.

The pathogenesis of pain in hypermobility and EDS

The underlying causes and mechanisms of pain in JHS and EDS are poorly understood.

Pain caused by an injury or damage (nociceptive pain) is often present, especially in early stages and neuropathic pain may also contribute to the pain (and account for parasthesias) and be exacerbated by dislocations and subluxations. Central sensitisation may also contribute to the pain becoming chronic.

It has been proposed that lack of proprioceptive acuity (ability to sense joint position and movement) and muscle weakness play a role in generating chronic pain. The fear of pain is common in people with JHS and hEDS and can cause deconditioning and exercise intolerance which leads to a cycle.

It is hypothesised that abnormalities in the extracellular matrix (ECM) can contribute to the generation and chronification of pain. The ECM has a clear role in the general detection of noxious stimuli (nociception) and painful injuries have been shown to alter the the ECM at acute and chronic time points after the injury. The ECM also has a role in neuroplasticity and connectivity of the central nervous system.

Patients with EDS have defects in different components of the ECM which can lead to a devastating impact on the nervous system. Defective connective tissue can also inhibits the body's ability to react protectively to stretching or pressure of peripheral nerves.

The abnormal ECM may contribute to the central sensitisation that causes the chronification of pain.

The complex nature of pain in JHS and EDS patients, fragmented understanding of the underlying mechanisms, and lack of reported effectiveness of treatments highlight the fact that the management of pain in JHS and EDS patients.

Management requires pharmacological and psychological therapy.

Conclusion

[oh-heck-yeah-doritos]

So there was a cane in an escape room (it was part of it) and I was using it bcs why not?? And it made walking a lot easier and was wonderful and now I’m having a crisis.

… so uh I kinda wanna get one but how the hell would I ask my parents?? Aghhhhhh

Truthfully I’ve wanted a mobility aid for a while but I thought “maybe I don’t need it!! maybe it’d just be a hassle or something” bcs idk I was in symptom denial but now I’m had eXPERIENCE and ah.