Hypermobility and Joint Hypermobility Syndrome? Is it good to be bendy??

Last month’s blog was all about stretching. However there are some people who don’t even have to try to be flexible. People with hypermobility have joints which easily stretch beyond what would normally be expected. They are sometimes referred to colloquially as being ‘double jointed’ a definition which brings to mind images of contortionists squeezing their bodies into ridiculous positions, often self dislocating joints in order to do so. However, extreme examples aside, joint hypermobility is actually incredibly common and is often a factor behind musculoskeletal injuries. This blog will discuss the problems associated with hypermobility and why despite the advantages in terms of flexibility it is so often linked to injury.
What causes Hypermobility?
Specific hypermobility can be acquired after an injury to the stabilising structures of a joint. For example the ankle joint can become hypermobile in certain directions if the ligaments are ruptured. However general hypermobility is more commonly present without injury. It seems that complex genetic factors related to the type of collagen in connective tissues are largely important in determining hypermobility. The collagen in the connective tissues of a hypermobile joint is thought to be ‘weaker’ than normal, enabling the joint to stretch further than average increasing the risk of damage and overstrain.
Research is still establishing the exact genetic prototypes of collagen in Joint Hypermobility Syndrome (JHS). This has lead to the syndrome being linked to a group of genetic connective tissue disorders, which include the Ehlers Danlos Syndromes, Marfan Syndrome and Osteogensis Imperfecta. These once distinct conditions are now thought to have some overlap in terms of symptoms. JHS was sometimes referred to as Benign Hypermobility Syndrome because this group of patients didn’t appear to suffer from the more serious symptoms of the other genetic connective tissue conditions mentioned (such as aortic dilatation, bone fragility, skin hyperextensibility and delayed wound healing). However more recent research has suggested that features of these conditions may exist in much milder form even in the so-called ‘benign’ JHS. Therefore JHS is now often considered synonymous with Ehlers Danlos Syndrome type III (Hypermobility type)(Tinkle et al 2009).

Diagnosing Joint Hypermobility Syndrome
A scale of hypermobility called the Beighton scale is commonly used in the clinic to assess for general hypermobility. This is quick and easy to use and your physiotherapist may check through these tests if she suspects hypermobility. For example, when examining someone’s lumbar spine movement, if they can easily touch both hands to the floor, (first test below) it is always a good idea to check quickly through the other tests to determine if there is some generic hypermobility and to rule out Joint Hypermobility Syndrome (JHS) as being an underlying cause of the current problem.

Beighton Scale
1. One point if while standing forward bending you can place palms on the ground with legs straight
2. One point for each elbow that bends backwards
3. One point for each knee that bends backwards
4. One point for each thumb that touches the forearm when bent backwards
5. One point for each little finger that bends backwards beyond 90 degrees.
Whilst the maximum score is 9, a score of 4 or more on the above scale indicates that you have some joint hypermobility. However this must be put into context and related to a person’s signs and symptoms. It is possible to have some hypermobile joints and be asymptomatic. In this case you should not be diagnosed with JHS.
To help establish a diagnosis of JHS some clinicians use the Brighton Criteria.
Major Brighton Criteria
• a Beighton score of four or more – either now or in the past
• joint pain for longer than three months in four or more joints

Minor Brighton criteria
• a Beighton score of one to three, or a Beighton score of zero to three if you are over 50 years of age
• joint pain for longer than three months up to three joints, back pain for longer than three months, or spondylosis (spinal arthritis) or spondylolisthesis (where a bone from the lower spine slips out of position)
• dislocation or partial dislocation of more than one joint, or the same joint more than once
• three or more injuries to your soft tissues – such as tenosynovitis or bursitis
• particular physical characteristics called Marfanoid habitus – this includes being tall and slim and having long, slim fingers
• abnormal skin – such as thin and stretchy skin
• eye-related symptoms – such as droopy eyelids or short-sightedness
• swollen and enlarged veins (varicose veins), a hernia or – in women – pelvic organ prolapse.
According to the Brighton criteria, JHS may be diagnosed if you have:
• two major criteria
• one major criteria and two minor criteria
• four minor criteria
• two minor criteria and a close relative, who has been diagnosed with JHS .

Managing Hypermobility
Hypermobility is particularly linked to overuse injuries. If a joint has limited restraint from passive structures then the active structures, tendons in particular, crossing the joint are placed under more load. This makes a hypermobile joint more prone to tendinopathies as well as sprains and strains. Examples include knee and hip tendon problems in the lower limbs and repetitive strain injuries to the wrist, elbow and shoulder
Treatment aims to manage the condition by reducing the load on the joints themselves and improving the active muscular control at affected joints. The following may form part of this management:
• Strengthening exercises
These might be specific for an individual joint. For example a hypermobile knee joint will benefit from strengthening of the quadriceps and hamstrings, which help to control the movement of the joint. Increased strength of these muscle groups reduces shock absorption and control movement in the joint during weight bearing activities and can therefore minimise joint damage and pain. For those with global hypermobility, an exercise programme will need to look at strengthening across multiple joints whilst minimising overload. Hydrotherapy exercises and Pilates can be very useful in these cases.
• Core stability exercises
Strengthening the deep abdominal, pelvic floor and back muscles in particular helps to stabilise hypermobile sections of the lumbar spine. These segments are often associated with more advanced disc degeneration due to overuse.
• Posture re-education
Due to the impaired joint position sense associated with the condition, hypermobile people will often stand in damaging postures with their joints right at the end of range; for example a sway back position of the lumbar spine, a hyperextended knee position or an overly extended head on neck position. These postures overload the joint structures over time often leading to pain. Re-education can help by stimulating proprioceptors, joint receptors in the connective tissues, to improve awareness of where the ‘neutral’ position is for a joint.
• Balance retraining.
This can further improve the proprioceptive awareness of joint position by challenging the ability to maintain a neutral position whilst experiencing perturbation of equilibrium.
• Ergonomics
Adaptations can be made to ergonomic situations to reduce the risk of straining hypermobile joints. For example your occupational health department at work may be able to provide you with alternative mouse options or keyboards as well as wrist supports for using these items. It is particularly important if you have hypermobility to have an optimum work station set up which promotes a good neutral posture with minimal strain on your joints.
• Podiatry
If you have hypermobility in your feet then orthotics can be very useful to promote an optimum foot position during weight bearing. Their main function is thought to be neuromuscular in that they help to stimulate appropriate muscle activity in the lower legs which would act to reduce joint and tendon overload. Taping and kinesiotaping also have a role in controlling muscle activation patterns across joints through similar pathways.
Finally-Important Points to note!
• Being hypermobile does not mean you have Joint Hypermobility Syndrome (JHS). In other words, you can have hypermobile joints without being in pain. A combination of the Beighton scale and the Brighton criteria can help to distinguish this diagnosis.
• Whilst hypermobility can be seen as an advantage initially in sports, where flexibility is important, such as gymnastics and ballet, it can lead to problems further down the track in terms of sprains and strains as joints are overloaded.
• Interestingly whilst the prevalence of hypermobility in children in ballet schools is high, the incidence of JHS, as identified by the Brighton Criteria, is rare in professional dancers (McCormack 2004). It may seem that many hypermobile dancers do not make it professionally because they succumb more easily to injury by overloading their passively weaker joints. However it is also possible that through intense dance training motor control and proprioception of hypermobile joints improves, enabling the successful dancers to avoid the pain associated with hypermobility, which is the defining factor of JHS.
• Untreated JHS is linked to chronic pain conditions such as fibromyalgia and is often associated with an early onset of Osteoarthritis.
• Pilates can be a very useful exercise for hypermobile clients and is usually recommended over Yoga for this group, who need to focus more on control of their joints rather than stretching.

By Nikki Richards MPHTY(Sports) BSc(Hons)Physio MCSP

References
http://www.nhs.uk/Conditions/Joint-hypermobility/Pages/Treatment.aspx
Grahame R. (1999). Joint hypermobility and genetic collagen disorders: are they related? Arch Dis Child. Feb; 80(2): 188–191.
Malfait,F, Hakim AJ, De Paepe A and. Grahame, R . (2006). The genetic basis of the joint hypermobility syndromes. Rheumatology ,45 (5): 502-507.

McCormack, M (2004). Teaching the Hypermobile Dancer. The Journal of Rheumatology.
Grahame, R (2001). Time to take Hypermobility Seriously (in adults and children). Rheumatology ,40 (5): 485-487.
Grahame R, Bird HA, Child A et al (2000). The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol;27(7):1777-9
Tinkle BT, Bird HA, Grahame R, Lavallee M, Levy HP, Sillence D (2009). The lack of clinical distinction between hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome. Am J Med Denet A. 149A(11):2368-70
Beighton PH, Solomon L, Soskolne CL (1973). Articular mobility in an African population. Ann Rheum Dis; 32: 413-17
Kyndall L. Boyle, Philip Witt, and Cheryl Riegger-Krugh (2003). Intrarater and Interrater Reliability of the Beighton and Horan Joint Mobility Index. J Athl Train.; 38(4): 281–285.
Palmer, S, Bailey, S, Barker, L, Barney, L, Elliot, A (2014). The effectiveness of therapeutic exercise for joint hypermobility syndrome: a systematic review. Physiotherapy Volume 100, Issue 3, September 2014, Pages 220–227

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