Joint Hypermobility Syndrome
Hypermobility implies that a patient has joints with a greater range of movement than would normally be expected. For some this is completely asymptomatic and can be a blessing – think of the benefits to gymnasts and dancers an extended range of movement can have. For others, it causes pain and discomfort in the joints with a range of other symptoms. Additional problems alongside an extended range of movement around a number of joints is referred to as Joint Hypermobility Spectrum Disorder or Hypermobility Ehlers-Danlos Syndrome (hEDS).
What is hEDS?
Symptomatic joint hypermobility is a disorder of the connective tissue. Collagen is present in ligaments, tendon, skin and bone. Abnormal production, structure or processing of the collagen results in a reduction in strength of the tissue and makes the structures more elastic and less stable. This allows excessive movements of the body's joints and a lack of control around the capsules which in turn produces pain.
If collagen is lax in the joint it can also be lax in other places in the body. Some types of JHS are more severe than others and there is a crossover with other medical conditions including Ehlers-Danlos Syndrome (EDS), Marfan Syndrome and Osteogenesis Imperfecta. EDS Type III and JHS are now classed as the same condition. The diagnosis of hEDS is made when there are other problems associated with hypermobility.
Because joint hypermobility presentations vary so greatly they are now measured on a spectrum which aims to cover the broad range of symptoms that we see. It ranges from the benign, completely asymptomatic to the debilitating chronic pain and life altering issues.
Diagnostic criteria for Joint Hypermobility Disorder
The Beighton score and the Brighton criteria are used for a formal diagnosis.
Beighton score
One point for each you can do, up to a maximum of 9 points:
* Can you put your hands flat on the floor with your knees straight? (1 point)
* Can you bend your elbow backwards? (left 1 point, right 1 point)
* Can you bend your knee backwards? (left 1 point, right 1 point)
* Can you bend your thumb back on to the front of your forearm? (left 1 point, right 1 point)
* Can you bend your little finger up at 90º (right angles) to the back of your hand? (left 1 point, right 1 point)
Other symptoms need to be present so a high Beighton score alone doesn't necessarily mean an individual has hypermobility syndrome. Likewise, a low score should be read with caution as it does not take a number of body sites into consideration where there could be widespread pain e.g; jaw, cervical spine, shoulders, hips, thoracic spine, ankles and feet.
Brighton criteria
Major criteria
* Beighton score of >4
* Arthralgia for longer than 3 months in four or more joints
Minor criteria
* Beighton score of 1, 2, or 3
* Joint pain (>3 month duration) in one to three joints or back pain (>3 month duration) or spondylosis, spondyloysis/spondylolisthesis
* Dislocation or subluxation in more than one joint, or in one joint on more than one occasion
* Three or more soft tissue lesions (eg, epicondylitis, tenosynovitis, bursitis)
* Tall, slim, span greater than height (>1.03 ratio), upper segment less than lower segment (<0.89 ratio) (marfanoid habitus), long and slender toes and fingers (arachnodactyly), being able to overlap your thumb and fifth finger when wrapped around the wrist (positive Steinberg test)
* Skin striae, hyperextensibility, thin skin, or abnormal scarring
* Drooping eyelids, myopia, downward slanting eyes
* Varicose veins, hernia, or uterine or rectal prolapse
* Mitral valve prolapse
The Brighton criteria differentiate someone who is hypermobile from one who has the syndrome as it includes not just range of movement measures but signs of faulty collagen and positive responses to joint pain. Formal diagnosis consists of any one of the following:
Two major criteria
One major plus two minor criteria
Four minor criteria
Two minor criteria and an unequivocally affected first degree relative in family history
Additional symptoms
While pain and fatigue constitute a large part of the condition, symptomatic hypermobile people suffer from various combinations of additional symptoms, some of which are listed in the Brighton Criteria and most of which are listed below.
Primary
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Pain – JHS causes both chronic and acute pain without the inflammatory component of other rheumatological diseases. Normal NSAID protocols may not be effective.
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Muscle stiffness, tightness and fatigue – muscles need to work harder to stabilise the joints and counteract muscle imbalances.
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Popping or clicking of joints with movement
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Frequent dislocations or subluxations
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Reduced proprioception
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Hyperextended joints
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Recurring musculoskeletal problems – such as tendinitis, bursitis and repeat sport injuries in a number of sites.
Secondary symptoms and comorbidities
These are generally (but not all) related to the mild autonomic neuropathy that is often present and lack of integrity in the collagen in connective tissue in body sites not related to the joints, such as the gastrointestinal and circulatory systems.
- Irritable bowel syndrome
- Easy bruising, stretch marks, slow wound healing
- Severe Raynaud’s phenomenon – for most this is usually triggered in cold temperatures, or by anxiety or stress. With JHS it can also be present at other times
- Hypotension
- Asthma
- Anxiety
- Insomnia
- Headaches – due to lack of stability in the neck muscles, shoulder stabilisers and thoracic spine
- Menorrhagia and menstrual cramps
- Local anaesthetics – insufficient pain relief from anaesthetics and painkillers
- Lower back pain
- Crowded teeth
- Stress incontinence
Additional cardiovascular issues can be present in severe cases of other types of EDS. Because of the individual presentations, formal diagnosis is often missed and no link made between the symptoms.
So what can be done?
Because hypermobile ehlers-danlos syndrome is a hereditary condition and passed on through a gene mutation there is currently no way to prevent it and no cure. Management is the best option and this can be achieved through exercise, which is where we come in.
Strengthening the muscles around the joints increases stabilisation and minimises the risk of injury. The ideal scenario is close collaboration between physician, physiotherapist, osteopath and specialist strength and conditioning trainers.
A full static and movement assessment identifies possible areas of dysfunction. From the information gathered we can then devise a progressive exercise programme based on strength and stabilisation which promotes healthy, functional movement patterns creating balance, stability, efficiency, co-ordination and greater body awareness.
As with our approach to all clients at Hillcliff, it’s vital we look at the body as a whole unit and not particular joints or problem areas in isolation.
Core stability and postural correction go a long way to managing joint hypermobility disorders but this is only the start. Corrective exercise prescription can alter movement patterns of dysfunction commonly seen with joint hypermobility such as:
* Poor scapula control, winging and instability. Strengthening the rotator cuff muscles, serratus anterior and rhomboids and lengthening through the anterior chain can provide greater stability reducing the risk of bursitis, impingement and overuse injuries.
* Poor control of the hip, pelvic and lumbar regions. Exercises can address lower cross syndrome by mobilising tight muscles and strengthening their weak antagonists.
* The knee is a slave to the hip and the ankle (including problems associated with both flat feet and over pronation) and so problems originating above or below in the kinetic chain can present at the knee. Hyperextension also causes fatigue and instability.
* Reduced movement in the thoracic spine.
* Weak neck stabilisers and overworked upper trapezius that cause pain and headaches.
* Poor balance, co-ordination and proprioception can also be addressed through exercise reducing the risk of falls and sprains and of course improving performance in sport.
Our clients say. . .
"I suffered with pelvic girdle pain throughout my pregnancy. Six months after having my son, I was still in pain but my osteopath was unable to put a finger on the exact cause of the problem. My osteopath referred me to Claudine as she felt I needed to build my core strength to see an improvement to the injury.
I've worked with Claudine for the past 18 months (now 5 years) and have seen a huge improvement with the pelvic injury as well as my overall fitness. Her sessions are fun and challenging and have gradually rebuilt my strength after my pregnancy and c-section. Claudine is hugely knowledgeable on women's health, post natal issues and hypermobility and so has been able to give me a lot of advice and create an exercise programme tailored to my needs."
R Walsh