How normal is hypermobility (double-jointed) and what pain can it cause?

For most people, joint hypermobility is a normal phenomenon. For some, it can lead to debilitating conditions. Dr Anthony Hammond, a consultant rheumatologist and specialist in interventional pain management, explains what hypermobility is, when it goes beyond a normal physical occurrence and if the consequences of hypermobility can be treated.

What is hypermobility? Is it similar to flexibility or being double-jointed?

The first thing to say is that, for most people, joint hypermobility is normal. This is also called being “double-jointed”, which is simply the layman’s term to describe the tricks or contortions of joints that the more hypermobile people can readily do. Flexibility is a general characteristic and has a range from stiff to very bendy. The people with the bendiest joints are said to be hypermobile and most children and many young women, particularly of Southern or Eastern Asian origin, are in the most mobile or hypermobile range.

This isn’t necessarily bad and may provide an athletic advantage - I would guess all gymnasts are hypermobile and anybody who can do “that” pose in the yoga class will be too. Furthermore, being flexible probably contributes positively to healthy ageing. If you answer these questions positively, you are probably hypermobile:

Can you now (or could you ever):

• Place your hands flat on the floor without bending your knees?
• Bend your thumb to touch your forearm?
• Consider yourself double-jointed?
• Do tricks to contort your body or do the splits?
• Dislocate your shoulder or kneecaps more than once?

What are the symptoms of hypermobility?

Joint hypermobility can cause musculoskeletal problems which are, effectively, an over-expression of the kind of things that happen to us all, such as:

• Repeated and severe joint sprains
• Recurrent or persistent tendon injury e.g. epicondylitis (tennis elbow) or foot problems like plantar fasciitis, minor dislocation (patella/knee cap) or subluxations (slipping) of shoulder etc.

Anyone, regardless of their level of flexibility, can have these issues but the excessively hypermobile patients will accumulate repeated problems and may eventually have multiple persisting issues. Episodes of exercise-related injury merge into chronic painful problems and simple joint hypermobility begins to become joint hypermobility syndrome (JHS), which is hypermobility plus associated problems.

These are frequently readily understandable in terms of weakness and vulnerability of tendons and joints, but there are all also an extended range of symptoms which are less directly explained but clearly associated:

• POTS (postural orthostatic tachycardia syndrome), which involves fainting (syncope) and palpations
• Hypotension (low blood pressure)
• Altered temperature control
• Hyperhidrosis (excessive sweating)
• Irritable bowel syndrome (IBS) and “dumping’ syndrome

Most debilitatingly, there is a tendency to develop chronic fatigue and central pain amplification (fibromyalgia syndrome).

When these occur together and develop, fully normal hypermobility can turn into a very debilitating and painful syndrome of associated features. A good proportion of these patients, especially younger women with chronic fatigue, enter this condition through a hypermobility associated path, though this is frequently not recognised.

What are the causes/risk factors of hypermobility?

The “causes” are constitutional and genetic. Joint hypermobility syndrome (JHS) can be thought of as part of a range of hereditary disorders of connective tissue along with conditions such as Ehlers-Danlos syndromes (EDS) and Marfan syndrome. The genetic problems in these syndromes are well understood to be caused by deletions in genes. These lead to defective protein synthesis within the extracellular matrix of collagen, elastin, proteoglycans and glycoproteins - the structural tissues of the skeleton and body that hold us together and give us shape and substance.

However, there are no identifiable genetic abnormalities in JHS and there is a form of EDS where genes can be identified, but the syndrome is exactly the same as the non-genetic JHS form.

In practical terms, EDS and Marfan syndrome have hypermobility only as part of a more complex condition, and often with readily recognisable bodily features, while many  hypermobile patients may show some features of the genetic conditions such as skin stretchability, unusual stretch marks, papery scars, easy bruising, varicose veins  etc. and may have the hypermobile (only) form of EDS. The distinction is not of practical importance since medical management does not change if one could identify given genes, and tests are not routinely carried out.

Does it affect some parts of the body more than others?

The joints and tendons are the most frequently affected. Individual joints often have recognisable patterns of problems such as:

• Rotator cuff injury
• Hip impingement
• Snapping hip (lateral band syndrome)
• Patellar dislocation

There can be functional issues of internal organs with abnormalities of heart rate and bowel (called autonomic dysfunction), lung leak and lung collapse (pneumothorax). In the rare extreme forms of EDS, there can be significant alterations in larger arteries and heart valves which can be dangerous and less so in Marfan syndrome.

Can it be cured? How is it treated?

As flexibility is a constitutional feature (i.e. it’s how you are made), it cannot be “cured” in any sense. However, individual problems can be managed to the patient’s benefit and it can be very helpful to have a clear statement of why all these curious and unrelated things are happening in one person – this is the dignity of a diagnosis.

• Muscle and tendon problems are managed by physiotherapists who will also look after training and deconditioning.
• POTS and autonomic issues are managed by physicians, often cardiologists, and may involve complex medical therapies.
• As an arthritis and spinal pain expert, I am often involved in the management of the general pain state, fibromyalgia and specific spinal issues which are common. These require special consideration and different interventions in the JHS patient.
• Chronic tendon injuries can be encouraged to mend with regenerative injections.
• Psychological support in the form of cognitive behavioural therapy (CBT) and counselling can be of great help.

Importantly, self-help is possible and I would point all patients to the HMSA (Hypermobility Syndromes Association) website.

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