Joint hypermobility syndrome

Hypermobility means joints that move beyond their usual range. On its own it is not an illness: a sizeable share of people have unusually loose joints, particularly children, teenagers and women, and most of them are none the worse for it, while gymnasts, dancers and musicians may even gain from it. The word syndrome is used when the flexibility comes with complaints — pain, fatigue, joints that give way. The distinction matters, because flexibility is not something to be treated, whereas the complaints can be influenced a great deal, though usually not in the way people expect medicine to work.

Being bendy is not the same as having the syndrome

Joint range is assessed with a short set of manoeuvres known as the Beighton score. The examiner looks at whether the little finger bends back past a right angle, whether the thumb reaches the forearm, whether the elbows and knees straighten out beyond the line, and whether you can bend forward with straight legs and put your palms flat on the floor. Each sign is counted on both sides and the total gives a score.

The score is not a diagnosis by itself, and the cut-off depends on age and sex: children's joints are looser and range falls off with the years, so the same figure means different things in a child and in an older adult. The scale also looks only at hands, elbows, knees and spine, so if your shoulders, jaw or feet are the flexible ones while those joints are ordinary, the score comes out low despite obvious hypermobility. That is why a doctor always reads the manoeuvres alongside the symptoms rather than following a single number.

What people actually complain of

The picture is not made of one symptom, and half of it is usually not put down to the joints at all:

• aching joints and muscles by the evening and the day after exertion, most often knees, fingers, shoulders and the lower back; in children it shows up as legs that hurt at night;
• tiredness that rest does not fix: holding a loose joint in place makes the muscles work harder than usual, and that comes at a cost;
• frequent sprains, turned ankles, partial and full dislocations, sometimes over nothing — a shoulder that pops out while reaching for something on a shelf;
• clumsiness and a poor sense of where the body is: loose joints send the brain a blurred signal, hence the unsteadiness on uneven ground and in the dark;
• thin, soft, stretchy skin, easy bruising, stretch marks, thin scars after cuts;
• flat feet and ankles that roll inwards, sore soles after a long walk;
• in some people, dizziness and palpitations on standing up quickly, a tendency to constipation, bloating and reflux, leaking urine when coughing or laughing;
• anxiety, which is commoner among hypermobile people than average and makes pain considerably worse.

None of this is obligatory and it does not all appear at once. Plenty of people with obvious hypermobility have no symptoms whatever and find out about it by chance.

Where the looseness comes from

The strength of ligaments and of the joint capsule comes from collagen, the protein that connective tissue is built from. Differences in how it is put together and how it assembles into fibres make the tissue a little more yielding, so the joint is held less firmly. The trait is inborn and inherited, which is why hypermobile families usually have several members with it: a child with rubbery fingers almost always turns out to have a parent the same way.

The names have shifted over the years, which is worth knowing so that old and new documents do not confuse you. What used to be called joint hypermobility syndrome or benign joint hypermobility syndrome is now treated as a spectrum: hypermobility without symptoms, hypermobility spectrum disorders, and hypermobile Ehlers-Danlos syndrome. The boundaries between them are a matter of convention and the management is broadly the same, so a change of label changes nothing about your life.

When something more serious sits behind the flexibility

There are rare inherited disorders of connective tissue in which hypermobility is only the visible part, while what matters is happening in the blood vessels, the heart and the eyes. These must not be missed, because here surveillance saves lives. Show these signs to a doctor without waiting for something to start hurting.

• Marfan syndrome — tall build with disproportionately long arms, legs and fingers, a sunken or jutting breastbone, marked scoliosis, severe short sight or a displaced lens. The main danger is widening of the aorta, which is monitored by echocardiography.
• Vascular Ehlers-Danlos syndrome — very thin, translucent skin with visible veins, bruising at the slightest knock, fine facial features. It can lead to rupture of arteries and hollow organs, so certain investigations are avoided in these patients and contact sports are ruled out.
• A family history that should raise concern — sudden death of a young relative, a torn aorta, rupture of a blood vessel or of the bowel, several relatives who have had a heart valve operated on.

Separately: if a joint comes out and will not go back, or the limb goes numb, pale or cold, that is an emergency and needs attention within the hour rather than tomorrow.

What happens at the appointment

There is no single test for hypermobility. The doctor asks about pain, dislocations and relatives, checks joint range and looks at the skin, the feet and the posture. From there the job is not to confirm the hypermobility but to be sure the symptoms are not coming from something else: inflammatory arthritis, rheumatic disease, an underactive thyroid and various deficiencies cause similar pain and fatigue and are treated quite differently. Blood tests including markers of inflammation may be needed for that. X-rays in hypermobility usually show nothing and belong after an injury rather than in the diagnosis.

If the picture suggests Marfan or the vascular type, the doctor refers on to a specialist in inherited connective tissue disorders and arranges echocardiography and an eye assessment. The main work from then on, though, is not done in the consulting room but by a physiotherapist, and that is the first referral that matters.

What genuinely helps

There is no way to make ligaments stronger, so the whole point of treatment is to replace the support the ligaments do not provide with muscle. It works slowly, but it works, and nothing else comes close.

• Strength matters more than stretching. Hypermobile people are naturally good at exactly what they do not need, which is stretching; what they need is strength work for the muscles around the joint and control of position.
• Start with activity free of impact and jerking: swimming, an exercise bike, walking, bodyweight work through a small range. Build up gradually, because jumping a month ahead ends in a flare of pain.
• Balance and body position sense are trained separately; it takes weeks, but that is precisely what cuts down the number of turned ankles and giving-way episodes.
• Complete rest makes things worse: without activity the muscles weaken faster than the pain settles.
• Avoid party tricks with hyperextension and sitting in positions where the joint hangs on its ligaments. The habit of cracking joints does no favours either.
• Spread work and study over time: alternating tasks with short breaks is tolerated far better than several hours of the same movement.
• Comfortable, supportive footwear, and insoles if a podiatrist advises them. Splints and braces are useful selectively, during exertion or after an injury, rather than around the clock: wearing them constantly weakens the muscle.

Heat helps with pain — a warm bath, a hot water bottle. As for medicines, paracetamol and anti-inflammatory painkillers make sense, including gels applied over a particular joint, taken in short courses rather than continuously. Strong opioid painkillers are ineffective in this kind of long-term pain and do more harm than good. If pain has gone on for months and is getting in the way of life, the sensible step is not a higher dose but a referral to a service that deals with chronic pain.

Children, teenagers and everyday life

Hypermobility is commoner in children, and for most people the joints stiffen with age, so some of the complaints go away on their own. But "they will grow out of it" is no reason to do nothing: the child who refuses long walks, is exhausted by PE and gives up writing quickly because the hand tires is usually not being lazy but genuinely cannot keep going. What helps is not excusing them from effort but choosing it well: swimming instead of contact games, breaks while writing, a comfortable pen, a second bag instead of a heavy rucksack slung from one shoulder. Anyone dancing or doing gymnastics seriously should talk to their coach and doctor about the balance between stretching and strength work.

Adults do well to look at their workstation: desk and chair height, support for the forearms, a change of position at least once an hour. Keeping weight within sensible limits helps, since extra kilos load the knees and feet. Hypermobility rules out neither sport nor pregnancy nor travel; it simply asks that effort be prepared for in advance rather than pushed through on willpower.

Online consultation

In an online consultation the doctor will work out what is actually going on: whether this is flexibility alone or already a set of complaints worth addressing. They will ask about pain, dislocations and fatigue, go through the family history and say the important part — whether other conditions need ruling out and which tests do it. They will look separately at the signs that call for genetics, an echocardiogram or an eye specialist, and explain which specialist comes first. If the diagnosis is already known, an appointment is a good place to go over the exercise plan, work out why it is not paying off, adjust pain relief and decide whether insoles or splints are worth it. Feeling a joint and measuring its range cannot be done online, but avoiding several years of going round in circles and finding the right specialist certainly can.

This material is for information only and does not replace medical advice.