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Pain and EDS-H / JHS


Understanding the causes of pain in EDS-H / JHS


Pain found in EDS-H / JHS is unusual in the medical world because it is one of the few conditions causing both chronic and acute pain and because (except after dislocations etc.) it is notably pain without the inflammatory component of other rheumatological diseases. 'Joint Hypermobility Syndrome is the most frequent cause of musculo-skeletal pain in any rheumatological clinic.' (Quote: Dr Jamie F Bravo MD Prof. of Medicine)


EDS-H / JHS pain can be severe and widespread. 'Built with collagen, the fascia is the sort of “Saran Wrap” (cling-film) that holds our bodies together, a connective tissue that surrounds muscles, groups of muscles, blood vessels, and nerves. The result of an overly-stretchable fascia can be literally body-wide because it is wrapped around nearly everything. In addition, every time a joint slips or dislocates, the soft tissue connected to that joint—tendons, ligaments, muscle, fascia, blood vessels—all stretches.'  (Quote: Dr A Hakim  MA FRCP) 


'Because of the structural defect in collagen, all that connected tissue is stretched beyond what normal should be; high-resolution MRIs reveal microtrauma, microscopic tears that start up the inflammation and coagulation cascades. But because each joint can subluxate over and over in just a single day, these microtraumas happen over and over in the same tissue without healing successfully. This was first pointed out as an EDS problem in July 2011 by Dr. Clair Francomano in her comments while presenting at the EDNF Conference: “Microtears are not visible with ordinary MRIs but are experienced by those with EDS, causing pain and instability.' (Dr. Francomano’s, Ehlers-Danlos Syndrome Update 2011: What We Know — And What We Don’t Know)


The onset of chronic pain usually proceeds in a gradual, subtle way, but with harmful effects, it doesn’t generally keep to the expected anatomical parts or relate necessarily a specific injury. Increased sensitivity to pain is often noted, which may be caused by damage to the nociceptors. Nociceptive pain is the pain that happens because of tissue damage and inflammation. It is likely that nociceptive input from over-stretched joints feeds the hypersensitivity to pain in the form of central sensitisation, in response to repeated tissue damaging events. In many, the stimulus may be something that does not normally provoke pain in others.


‘Commonly and intuitively, those with JHS resort to a strategy of movement avoidance as a means of pain avoidance, recognised as kinesiophobia. 45 This has the effect of aggravating and compounding muscle deconditioning — the opposite of what is required in a hypermobile person whose joints are inherently ‘unstable. Not surprisingly, it often plunges them into a vicious downward spiral of declining function, loss of independence, self-esteem and self–efficacy.’ (Beighton P., Grahame, R. & Bird, H)


Add to this chronic pain, the 'acute' pain that is experienced during and following joint dislocations and subluxations, and the pain which may develop from early onset osteoarthritis (due to wear and tear on joints), and it becomes easy to see why EDS-H / JHS can cause so many difficulties.


It may seem counterintuitive, but many hypermobile people also suffer from stiffness and tightness in the their joints and muscles. This is thought to be a result of the extra work muscles have to do, to try and keep a hypermobile person's joints stable. 'Their muscles have to work overtime to stabilise and support the joints, and not in an efficient way, so they may go into a hypertonic state (in a state of abnormally high tension)'. (Rotstein R.). A non-hypermobile person, training at the gym, may experience stiffness after a particularly strenuous work-out the previous day and, in the same way, some hypermobile people find they wake-up feeling like all their muscles and joints are stiff; as fluid gathers in the joint to try to heal the damage caused by overuse the previous day. This may take several hours to dissipate, or sometimes it may last all day.  


While the specific pain can vary from person to person,  some of the kinds of pain that a person with EDS-H / JHS might feel are as follows:


  • Pain in the joints. Any of the joints can hurt, and very often more than one.

  • Dislocation or subluxation (a partial dislocation) of joints

  • Headaches

  • Pain with daily tasks. For instance, people with EDS-H / JHS can have trouble with their finger joints causing difficulty with 'fine' skills such as using pens and pencils (because holding tightly to something so thin becomes painful) chopping vegetables, doing up buttons etc.,  Not infrequently, pain and fatigue in the hand, wrist or lower arm are noted, particularly by teachers, who may also note that unusual or bizarre hand postures are assumed for writing tasks, with the relatively less stable, hypermobile, hand.   Painful joints in the knees, shoulders, spine, hips etc., may cause pain when walking or washing and bathing. 

  • Pain in the  back due to laxity of the spinal ligaments, or scoliosis, or hiperlordosis (deep lumbar area) caused by lumbar disc problems

  • Pain in the abdomen

  • Muscle stiffness and tightness caused by the extra work muscles have to do to try and keep a hypermobile  person's joints stable.

  • Pain all over - patients often state ' I just hurt'.

  • Fibromyalgia

  • Complex Regional Pain Syndrome, (a type of very severe localised pain),  is a co-morbidity of patients with EDS-H / JHS.​​



Howard P Levy MD PhD writes:


  • Chronic pain, distinct from that associated with acute dislocations, is a serious complication of the condition and can be both physically and psychosocially disabling [Sacheti et al 1997, Hagberg et al 2004, Rombaut et al 2010a, Voermans et al 2010a, Rombaut et al 2011a, Rombaut et al 2011b].

  • Pain is variable in age-of-onset (as early as adolescence or as late as the fifth or sixth decade), number of sites, duration, quality, severity, and response to therapy.

  • Severity is typically greater than expected based on physical and radiologic examinations.

  • Severity sometimes correlates with degree of joint instability and with sleep impairment [Voermans et al 2010a].

  • Fatigue and sleep disturbance are frequently associated [Rombaut et al 2010b, Voermans et al 2010a, Voermans et al 2010b, Rombaut et al 2011a, Rombaut et al 2011b, Rombaut et al 2012b]. Affected individuals are often diagnosed with chronic fatigue syndrome, fibromyalgia, depression, hypochondriasis, and/or malingering prior to recognition of joint laxity and establishment of the correct underlying diagnosis.

  • Headaches, especially migraine, are common [Rombaut et al 2010b, Rombaut et al 2011a, Rombaut et al 2011b]. Cervical muscle tension, temporomandibular dysfunction and stress are some of the likely contributing factors.


Several recognisable pain syndromes are likely:


Muscular or myofascial pain: localised around or between joints, often described as aching, throbbing, or stiff in quality, may be attributable to myofascial spasm, and palpable spasm with tender points (consistent with fibromyalgia) is often demonstrable, especially in the paravertebral musculature (muscles which run beside the spine)  [Castori et al 2010a, Rombaut et al 2010b, Rombaut et al 2011a, Rombaut et al 2011b]. Myofascial spasm possibly occurs in response to chronic joint instability, but this has not been systematically studied. Myofascial release often provides temporary relief.


Neuropathic pain: variably described as electric, burning, shooting, numb, tingling, or hot or cold discomfort, may occur in a radicular or peripheral nerve distribution or may appear to localise to an area surrounding one or more joints [Camerota et al 2010]. Nerve conduction studies are usually non-diagnostic. Skin biopsy may reveal reduction or absence of small nerve fibers, but may be normal. One hypothesis is that neuropathic pain may result from direct nerve impingement (e.g., by subluxed vertebrae, herniated discs, vertebral osteoarthritis, or peripheral joint subluxations). In addition, there may be mild-to-moderate nerve compression within areas of myofascial spasm. Neither of these possibilities has been evaluated clinically.


Osteoarthritic pain: occurs later in life (but earlier than in the general population) and typically presents as aching pain in the joints, frequently associated with stiffness. It is often exacerbated by stasis (motionlessness) and by resistance and/or highly repetitive activity. 


Howard P Levy, MD, PhD, Dept of Medicine, Division of General Internal Medicine



Although there is no evidence to support the claim that weather makes conditions such as

EDS-H / JHS or Arthritis worse, many people find that their joints often tend to feel worse when the atmospheric pressure is falling, for example, just before it rains.


'The body learns pain and increases pathways for pain, setting up a cycle that remodels the nervous system. “A cardinal feature of EDS is pain. At first there are just small pains; but acute pains may accumulate, then become continual and chronic. There may be a major dislocation of injury to start the cycle; but without adequate treatment, persistent pain can change the nervous system in a process that is difficult to reverse. By lowering the threshold for pain signals, chronic pain becomes harder to treat.' (Quote: EDNF’s Pain Management Medical Resource Guide) 


'In addition to their proneness to injury, dislocation and osteoarthritis, (people with EDS-H / JHS) may also have a fault in the way their pain signals are picked up for onward transmission to the brain, where they reach consciousness. Research work is in progress to try to sort out this enigma. Much more needs to be done'. Quote: Prof. R Grahame CBE,  MD, FRCP, FACP)



Fighting Pain


There are many treatment options that can be considered when trying to reduce pain, indeed it is usually necessary to use a combination of treatments to keep pain under control. The key is to use a multi-targeted approach, be flexible, and not to give up.


The importance of using multiple strategies, even if each one only makes a tiny difference, should not be underestimated. The difference made when they all are added together is significant.


For example, pain management clinics, specialising in pain caused by EDS-H / JHS, can help you learn to 'break the pain cycle'.  A specialist physiotherapist or gait assessment centre can help with pain problems caused by biomechanics (posture, weakness or misalignment), and your GP or consultant may decide to try appropriate pain medications (If one medication doesn't work, another drug, or a combination of others may be the right solution that can improve your quality of life).



Kenneth Goldschneider MD, FAAP, Director of Pain Management at Cincinnati Children's Hospital writes:


'We have found several effective treatments for pain relief, but physiotherapy  (PT) is the most important intervention. The right kind of PT emphasises “proprioception,” which is the sense of where one’s joints are in space. Patients with EDS lose some of the sense of where their joints should be, which causes misalignment.  And this is a large factor in their pain. Aquatic therapy can be another great way to go, if PT on land is too hard to start with.


Here are other ways we have found to relieve pain associated with Ehlers-Danlos Syndrome:


  • Work with a pain psychologist. He or she can provide excellent interventions for chronic pain. The idea is to harness the brain to help control the pain and to figure out ways to have a good life in spite of pain. This is extremely important.

  • Medications can help, but cannot change the joints, only lessen the symptoms.  No medication can take the place of proper PT, OT, and psychological therapies. A fast acting, short-lasting pain medication can be helpful during a painful dislocation.  A muscle relaxant to help reduce muscle spasm after a dislocation can help get the joint back into position. Some anti-seizure and antidepressants can help with widespread pain, or nerve-related pain.'

  • Acupuncture can be helpful for some people.

  • Meditation and gentler martial arts such as Tai Chi can sometimes combine the strength of the mind and the body to bring some relief.

  • Yoga is often used as a good mind-body therapy. But be cautious. For people with EDS, speak with the instructor and watch a class or two to see if the positions used are okay for your joints.'

It is important for practitioners to realise however,  that although physiotherapy is the mainstay in management of the condition, some symptoms/pain, suffered by those with EDS-H/JHS, are more complex, such as problems with the gastrointestinal system, or dysautonomia, and these need investigation and approriate treatment.  (For more on this topic, please see 'Physiotherapy and EDS-H' in the 'Additional Information' section).


To address pain, people with EDS-H / JHS often have to use a variety of medicinal and non-medicinal strategies. This requires becoming a ‘collector of methods of pain relief’ and

keeping  a ‘tool box’ of options to manage your pain.


Here Dr C Francomono describes her 'Tool Box Plan' for Pain Relief:

‘Take, for instance that your medications take care of 20% of your pain relief. Then you use your TENS unit for another 10% reduction. Some Tiger Balm on your painful joints brings another 5% of pain relief. Maybe you could pull out your heating pad too for another 5% of pain relief. Then you decide upon a warm soak with Epsom Salts for another 10% pain relief. Deep breathing and some PT exercises combine for another 10% of relief. And then you watch a great movie to distract you for another 15% of pain relief. So now you have 75% of your pain managed effectively.’


When a child or young adolescent is involved, it may be useful to create a 'COMFORT TOOL BOX' This may be a real box full of things or a virtual box that have been created between the young person and their family.:


A Comfort Tool Box is useful for the child and family to develop their own collection of activities and interventions that can be used to reduce the pain. These can be items that facilitate distraction (e.g. DVDs, toys, music or spoken word stories) as well as positive coping statements, relaxation scripts, aromatherapy oils, helpful exercises, things that bring back memories of wonderful days out, and comforting objects such as favourite photos, postcards, treasures and keepsakes or pictures they have drawn. 





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