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Proprioception and EDS-H / JHS
Proprioception is the body’s ability to transmit a sense of position, analyse that information and react (consciously or unconsciously) to the stimulation with the proper movement (Houglum 2001). In its most basic form, proprioception allows you to scratch your head without looking in the mirror or walk up a flight of stairs without having to peer at each stair. If your proprioceptive system is dysfunctional your responses to stimulation might be diminished or absent.
On a deeper scale, this sensory proprioceptive feedback is used by the body’s central nervous system to understand the position and movement of the body and limbs, allowing it to process information from special receptors in the skin, joints, muscles and tendons to provide knowledge about the body's position in space (Houglum 2001). These impulses transmit vital information, such as the amount of tension in a given muscle during a given movement. For example, In day-to-day activities such as writing, a person with poor proprioception may hold a pen too tightly or not tightly enough, which may cause joint pain in the fingers, wrist, arm or shoulder. Poor proprioception can also involve poor motor planning; you may feel that 'you're always bumping into door-frames', you might be thought of as 'clumsy' and be prone to tripping, falling, or not being 'graceful' in your routine movements.
Scientists have shown that people with the EDS-H / JHS are often not quite as good as other people in knowing exactly where their joints are in space, particularly in the case of the lower limbs including the proprioceptive performance of the knee joint. When a limb moves beyond the normal range of motion and your proprioception is poor it is easy for your brain to subconciously or momentarily lose a sense of where the limb is in relation to the body, leading to a further increase in the risk of injury (e.g. dislocation, subluxation or increased instability).
This reduced sensory feedback may also lead to biomechanically unsound limb positions being adopted. Poor posture may become habbit, and over pronation of feet and ankles is often found; some muscles become overactive and others become underactive, which can cause pain and excessive strain on joints. The potential for the development of overuse injuries and muscle strains increases dramatically. The shift in joint loading and stresses exceed the ability of lower-limb structures to withstand those forces, and microtrauma and pathology ensue (also see 'Pain and EDS-H / JHS' in the 'Useful Information' section of the main menu).
‘Hypermobile individuals frequently have difficulty focusing movement in a particular area, probably due to defective proprioception. Therefore movement re-education involving improving postural control and alignment, joint position sense, balance, and proprioception may be required.’
(Quote: Dr Jamie F. Bravo)
Joint proprioceptive deficit is already well documented in a variety of musculoskeletal conditions including osteoarthritis and Ehlers-Danlos Syndrome, but what about Joint Hypermobility Syndrome? Recently, a systematic review was carried out to assess joint proprioception in those classed as having Joint Hypermobility Syndrome (published June 2013). The review concluded that, although it remains unclear whether proprioception is reduced in the upper limbs, joint proprioception in the lower limbs is reduced in those with Joint Hypermobility Syndrome compared to non-Hypermobility Syndrome cohorts. This further validates the advice that: 'the management, and advice are exactly the same for both JHS and EDS-H' (Dr A Hakim MA FRCP).
A defective proprioceptive mechanism, of the sort found in Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome, allows acceleration of degenerative joint conditions (such as Osteoarthritis) and may account for the increased prevalence of such conditions seen with EDS-H / JHS subjects.
Proprioception garments aim to increase sensory and proprioceptive feedback as well as musculoskeletal support. They provide constant, consistent compression, stretch, support and sensory information for the time that the garment is worn. The garments can be specially made lycra medical garments, prescribed by an occupational therapist, or, if that option is not available, sports compression garments or compression support tights can also be very effective. Trials are being held at one of the Country's leading hospitals where patients (with conditions which are complicated by poor proprioception including Ehlers-Danlos Syndrome Hypermobile-Type and Cerebral Palsy) are trying out tailor-made proprioception garments.
It should be noted that use of proprioception garments can be a double edged sword. If worn all the time, they can cause muscles to become weak in the same way that constant use of braces/splints can. Used properly however, (i.e. only worn at the most vulnerable times and by a person who is committed to carrying out specific muscle strengthening exercises every day), they can be a very effective tool in increasing proprioceptive awareness and reducing multiple dislocations and pain. The danger is that (as with braces and splinting), the wearer becomes reliant on the garment, wearing them all of the time instead of only at the most vulnerable times and muscle strength, therefore, declines.