She was twenty years old – a competitive skier – complaining of muscle and joint pains, fatigue, gut problems, difficulty sleeping, cognitive problems, difficulty concentrating and tingling sensations. When she tried to exercise the pain in her shoulders and limbs would get worse forcing her to stop. She had difficulty urinating but no infections. When she chewed her mouth tended to ache.
Her blood work was normal. She had no sign of an autoimmune disease and her physical examination – with one exception – was normal. Her muscle tone was good and her reflexes fine. Her history was intriguing.
She’d experienced sprains and tendinitis in her wrists and ankles since childhood. When she was fourteen she was diagnosed with Raynaud’s phenomenon, a disorder of sympathetic nervous system hyperactivation which causes reduced blood flows to the fingers. At fifteen she caught SARS and then infectious mononucleosis keep her weak and in bed for months.
She’d tried to resume training at sixteen only to be felled again and again by muscoskeletal injuries which responded poorly to treatment. One ski fall which altered the curvature of her neck worsened her fatigue and arthritis-like pain. When she started experiencing paresthesias or tingling sensations in her lower legs she was suspected of having a neurological or muscle disease but extensive testing (brain and spinal MRI’s, four-limbs EMG, body bone scan, skull and jaws X-ray) revealed nothing. At this point she was diagnosed with fibromyalgia.
Over the next two years attempts to run and swim caused her so much pain that she was forced to stop. When she saw the doctor who wrote up this case report she was depressed, had trouble sleeping, thinking and had gained weight to boot. The doctor had her do one test which changed her diagnosis completely. Her main problem wasn’t fibromyalgia or chronic fatigue syndrome or some mysterious muscle disease; he felt she had primary joint hypermobility syndrome (JHS).
Joint Hypermobility Syndrome
The main feature of joint hypermobility syndrome or JHS is the ability to flex your joints beyond the normal range.
Despite being present in approximately 5% of the population JHS was only first described in the medical literature in in the 1990’s. Many people with JHS don’t experience symptoms but those that can experience a range of muscoskeletal problems including tendonitis, bursitis, osteoarthritis, subluxations, prolapsed back discs, whiplash, TMJ and carpal tunnel syndrome. They also tend to experience severe fatigue (ME/CFS) and a generalized pain hypersensitivity (fibromyalgia) that extends beyond the joints that are effected. One study found that over 75% of people with Ehlers Danlos Syndrome – the most common kind of JHS – experience severe fatigue.
Autonomic nervous system problems are also common and can result in orthostatic intolerance including postural orthostatic tachycardia syndrome (POTS). People with EDS can experience fatigue, visual changes, poor concentration, discomfort in the head or neck, throbbing of the head, weakness and fainting when they stand.
Diagnosing Hypermobility Syndrome
“If you can’t connect the issues, think connective tissues”
Like ME/CFS and FM – two other diseases that can generate an enormous number of symptoms, JHS and EDS are often misdiagnosed.
Physicians who know of the Beighton score – a test you can take at home – use it to help them diagnose the condition. It should be noted that a high Beighton score by itself does not mean that an individual has a hypermobility syndrome. Other symptoms and signs need to also be present.
It should also be noted that a low Beighton score may not be accurate because the questionnaire doesn’t ask about some areas in the body (jaw joint (the ‘TMJ’), neck (cervical spine), shoulders, mid (thoracic) spine, hips, ankles and feet) which can exhibit joint loosening.
It’s also possible to have JHS without having overt joint hypermobility. Because of changes in the connective tissue as we age some people who exhibit strong joint hypermobility as children no longer do so as adults. They may still, however, still experience the pain associated with JHS/EDS.
With those proviso’s out of the way…..
The Beighton Score
- One point if while standing forward bending you can place palms on the ground with legs straight
- One point for each elbow that bends backwards
- One point for each knee that bends backwards
- One point for each thumb that touches the forearm when bent backwards
- One point for each little finger that bends backwards beyond 90 degrees.
Another quick tool to use is the hypermobility questionnaire. An answer of ‘Yes’ to two or more of the questions suggests hypermobility is likely present. Again, like the Beighton score, this does not mean that the person has a Hypermobility Syndrome. (Why some people are hypermobile and in pain while others are hypermobile and healthy is not clear.)
- Can you now (or could you ever) place your hands flat on the floor without bending your knees?
- Can you now (or could you ever) bend your thumb to touch your forearm?
- As a child did you amuse your friends by contorting your body into strange shapes OR could you do the splits?
- As a child or teenager did your shoulder or kneecap dislocate on more than one occasion?
- Do you consider yourself double-jointed?
The Overlap Between JHS/EDS and ME/CFS/FM
Thus, a person with ANS dysfunction wastes tons of energy regulating body processes that should be automatic. Dr. Alan Pocinki on EDS
Dr. Pochinki is an ME/CFS/FM doctor who has found such increased rates of EDS in ME/CFS that in a 2011 Solve ME/CFS Initiative talk he called it a “characteristic factor” of the disease. After rattling off a long list of similar symptoms (including post-exertional malaise) he stated that sleep studies and autonomic nervous system (ANS) findings in the two diseases are identical.
Pocinki’s talk on the ANS and EDS (“The Pseudo-scientific symptoms in EDS”) could have been taken right out of an ME/CFS/FM seminar. He believes that low epinephrine (adrenalin) reserves found in EDS cause a paradoxical response: the more depleted the ANS is the more exaggerated its stress response becomes.
He Pochinki described an ANS that just cannot find relief; Staci Stevens has described the ANS in ME/CFS in a similar way and many people with ME/CFS can probably relate.
He used the metaphor of a car. A person with ANS dysfunction is constantly hitting the breaks and gas attempting to get his/her body where it needs to be. Instead, this person is getting surges of too much gas/slam on breaks/too much gas/slam on breaks/too much gas….and on it goes. Thus, a person with ANS dysfunction wastes tons of energy regulating body processes that should be automatic.
If you have ME/CFS or FM consider the possibility that you might have JHS/EDS as well. Pochinki’s basic treatment plan for helping to restore autonomic nervous system balance in EDS is an interesting blend of treatments used in FM and/or ME/CFS.
- better sleep – reducing arousals and increasing deep sleep;
- adequate – really – pain control, including appropriate exercise,
- physical therapy;
- adequate salt and fluid;
- minimize emotional stresses; and
- conserve energy, rest when needed and don’t “push through” fatigue.
He says that while not curative these simple measures help the majority of his ME/CFS patients.
To learn more about EDS check out the EDS media section of Health Rising
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