Benign joint hypermobility syndrome is mainly a condition of children and young adolescents with extra-flexible (hypermobile) joints who develop aching and pain when they exercise. Hypermobile joints are very common in young people and it isn't known why some experience pain whilst most don't.
The condition does not usually cause long-term harm but it can affect fitness and education at a crucial time because those affected tend to reduce their exercise levels. This makes the problem worse and treatment is aimed at breaking this negative cycle. In the longer term, recovery is usually good and most benign joint hypermobility syndrome does not continue into adulthood.
What is benign joint hypermobility syndrome (BJHS)?
BJHS is a condition where the joints are more than usually flexible (sometimes called 'double-jointed') and this is linked to exercise-related joint and muscle pain. It is most often seen in children and young people.
Is benign joint hypermobility syndrome (BJHS) harmful?
Having flexible joints can be an advantage and many successful sportsmen and dancers have hypermobile joints. It's likely that almost all ballet dancers and gymnasts have them. The flexibility makes them extra mobile and agile.
Unfortunately there are some possible problems from stretchy joints. Firstly, they may be more prone to injury as they are less tightly supported. Secondly, in some patients, they seem to cause muscle and joint pains. These pains tend to be worse after exercise - both immediately afterwards and the next day when muscles can be stiff and tender. As a result, affected young people start to avoid exercise. This makes their muscles and bodies less fit. As the muscles and the body become less fit young people develop worse symptoms. In particular, they have worse pains and much reduced ability to exercise and take part in sport. They also have more marked tiredness and worse sleep patterns.
Whilst the muscle symptoms are most commonly in the legs, also affected are the arms, backs and shoulders. Handwriting can become a problem, with the hand and wrist starting to hurt. Children can become fidgety and tired and their concentration may fade fast.
All of these things can affect school performance and learning. They can also affect joining in, and the things children learn from sport, such as leadership, teamwork and perserverance. BJHS can therefore affect what a child gains from their education and important learning opportunities may be lost. It does not generally have any other long-term consequences and it usually resolves as the child grows up.
How common is benign joint hypermobility syndrome (BJHS)?
Double-jointedness is very common in children of school age. Up to 4 in 10 of young teenage girls and about 1 in 10 of young teenage boys have hyperflexible joints. However, only about 1 in 10 children experience BJHS, and most experience it fairly mildly. It is not clear why some children with hyperextensible joints develop pain and others do not.
What are the symptoms of benign joint hypermobility syndrome (BJHS)?
The main symptoms are joint hypermobility with muscle and joint pains and tiredness. There is enormous variation in how severe this is and the impact it has.
Pain is most common in the legs and knees and is usually after activity and at night. Younger children seem to have more pain. Muscles are often stiff and sore for a few days after increased exercise and children may quickly be put off from exercising. Back pain and headaches are common, mainly because posture gets worse as muscles become weaker.
As children become less fit they become more tired and their muscles tire more easily with activity. This becomes a vicious cycle as they become less and less active. Unused muscles become restless and fidgety.The muscles of the hand and wrist are also affected. Therefore, handwriting may be uncomfortable and writing may become harder to do and harder to read.
This affects everything - sleep, tiredness levels, concentration and general activity. As muscle tone becomes worse, posture worsens and headaches and back pain become common. Balance is also affected as the core muscles become less supportive. This means skills at sports like hockey and football will also reduce.
Children typically bruise easily - although this is harmless. They get 'clicky' joints, which they may click repeatedly (this is usually harmless, although rarely joints can dislocate). Tummy (abdominal) pain and constipation are common, probably because of lack of abdominal muscle fitness helping the bowel to work.
Rarely, children can develop hernias, fainting and other physical signs of muscle weakness, although this (like dislocation) is more likely to be seen in Ehlers-Danlos syndrome than in BJHS.
How is benign joint hypermobility syndrome (BJHS) diagnosed?
BJHS is diagnosed using the Brighton diagnostic criteria. 'Criteria' is a medical term for 'features to look for.' in the case of BJHS these are a combination of the Beighton hypermobility score and the child or young person's symptoms. The Beighton hypermobility score is a 9-point scoring system based on little fingers, thumbs, elbows, knees and trunk. A score above 4 suggests the joints are hypermobile.
Diagnosis of BJHS requires two major criteria, or one major and two minor. Two minor criteria are considered enough for diagnosis if there is a first-degree relative with BJHS.
The major criteria are: Beighton score 4 or more and pain in 4 or more joints for over three months
There are several minor criteria. They include:
- Beighton score 1-3.
- Joint pain (arthralgia) in 1-3 joints for over three months.
- Back pain for over three months.
- A back condition seen in teenagers (spondylosis/spondylolysis/spondylolisthesis).
- Slipping 'out'/dislocation of more than one joint or of one joint more than once.
- More than three 'inflammation' conditions (for example, tenosynovitis).
- Tall, slim body with long fingers and toes.
- Thin stretchy skin with paper-like scars.
- Drooping eyelids.
- Varicose veins, hernia.
What other diagnoses are similar to benign joint hypermobility syndrome (BJHS)?
BJHS is very similar to Ehlers-Danlos Syndrome type 3 (hypermobility). The two conditions are very similar and may be different degrees of the same thing. Ehlers-Danlos syndrome is usually the diagnosis where there is a strong family history, or where conditions like hernia and dislocations occur. BJHS is usually the diagnosis where the main symptoms are pain and joint hypermobility.
Other conditions which need to be ruled out if your child has joint and muscle pains are:
- Marfan's syndrome
- Juvenile idiopathic arthritis
- Rheumatoid arthritis
- Ankylosing spondylitis
How is benign joint hypermobility syndrome (BJHS) assessed?
Children with possible BJHS are usually seen by a specialist. They will examine the child and talk to them about their activities, including hobbies, physical activity and sleep. They will also talk to them about the effect their symptoms are having at school and at home.
The doctor will look at your child's joints and will use the Brighton diagnostic criteria to look for the diagnosis. He or she will assess your child's muscle strength by pulling and pushing at various muscles, particularly in the legs and arms. They may be asked to walk or run.
There are no other tests for BJHS; the diagnosis is based on these findings. However, if your doctor still needs to rule out other conditions like arthritis then blood tests or X-rays may be needed.
How is benign joint hypermobility syndrome (BJHS) treated?
Treatment is aimed at strengthening the muscles that need to support the hypermobile joints. This will improve posture, strength and fitness.
It can be difficult to persuade young patients of the need to do this, since exercise hurts and increasing exercise hurts more. Successfully returning to normal fitness needs education of the affected child so that they believe in the treatment and will persevere. This needs support from parents. It involves a graduated supervised exercise programme. This can initially benefit from help from physiotherapy, and a visit to a podiatrist can be valuable. The goal is full participation in all activities.
Will the NHS treat my child?
Many professionals can help with BJHS, as detailed below. However, whether some or all of these are needed depends on the severity of the condition.
It is important for children to be assessed (for diagnosis and advice, and to make sure their pain doesn't have some other cause). After this, if BJHS is the diagnosis, most children will recover with parental and school support, without the need for other professional help. The NHS has very limited ability to offer ongoing help to patients who are able to get better without it. There is great pressure on physiotherapy services, for example. However in severe cases, or where families are struggling, there are a number of therapists whose help and intervention can be extremely useful.
The aim is for young people to understand the need to exercise regularly and work through the discomfort which this will bring but that this may take time.
The most important thing to understand is that the pain in BJHS is not a sign of muscle damage and does not mean that exercise is harmful. Instead it means that the muscles are not able to do their job properly and need strengthening. Similarly, tiredness shows that the body needs to increase fitness. Gradually increasing activity levels is the best treatment. If you are tired during the day, a snack and a walk are a better solution than a rest.
Pain can affect concentration, memory, mood and sleeping. Reassurance is needed that the pain is not harmful. It is also important to help your child find ways of dealing with pain. In general, painkillers are not helpful. Other things will help more, such as:
- Distraction (for example, music, other activities, talking).
- Talking and encouragement from parents and friends.
- Relaxation treatments like massage and aromatherapy.
- Gentle exercises.
- Warm baths.
- Encouragement with understanding.
Physiotherapy uses specific exercises to work on the muscles that most need help. It also helps support your child to help them add regular exercise into their daily life. Children with BJHS will at first have a lot of pain and fatigue the day after physiotherapy. It can also be hard for them to repeat the exercises accurately at home alone.
Typically, these days, it is difficult to access physiotherapy for young people, particularly on an ongoing basis. Frequent review appointments in physiotherapy would be helpful, but because this condition usually causes no lasting harm the NHS cannot offer long term physio. However, parents can play an important role in monitoring and checking the exercises, and your child's school PE teacher may be able to help.
This type of therapy aims to help with activities of daily living. An occupational therapist (OT) may look at the type of chair your child sits in, at their mattress, at a pen that makes writing less painful. An OT assessment in school may be helpful, particularly in more severely affected children.
A podiatrist is a person who is qualified to diagnose and treat foot disorders. A podiatrist may therefore be suggested to offer corrective insoles to correct the posture of the feet. If these are tiled 'out of line' this can markedly add to leg pain. Again, many successful sportsmen, including top tennis players, use these. A single appointment may be all that's needed.
Choosing your sport
Some sports may need to be returned to later than others, particularly those that strain the joints, such as trampolining and using bouncy castles.
It is important for your child to maintain a healthy weight - or to try to return to one. Being underweight will make muscles weaker, whilst being overweight puts more strain on the joints. Both extremes will tend to increase symptoms.
If your child has a low mood because of tiredness and pain then they may need some help with this from a psychologist or doctor. Advice about returning to a good pattern of sleep can also help with this.
No special equipment should be required. Wheelchairs and crutches are very unhelpful in BJHS, as they will tend to make the situation worse. Anything that makes your child feel or behave like a physically restricted person is unhelpful.
The Hypermobility Syndrome Association (see below) is a source for support and information for families, patients and schools.
What can schools do to help?
Schools should encourage affected children to take part in sport. However, they should make allowances (particularly at first) for the child's reduced stamina. Supportive seating will help with backache, fidgeting and tiredness.
Children struggling with handwriting may benefit from keyboard use or from extra time in tests and examinations. Extra time may be needed to move between lessons and the school should try to avoid expecting children to carry all of their books around with them all day.
What is the long-term outlook for benign joint hypermobility syndrome (BJHS)?
BJHS generally improves as your child's muscle size and strength increase. This reduces joint looseness. A few children do continue to have problems into adulthood, usually because their joint laxity results in easy injury.
BJHS in children rarely causes long-term problems like arthritis. However, problems with kneecap pain may develop in those in whom the kneecap has recurrently dislocated. Adults who continue to have BJHS are at slightly greater risk of developing osteoarthritis of affected joints.
Further reading and references
Adib N, Davies K, Grahame R, et al; Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology (Oxford). 2005 Jun44(6):744-50. Epub 2005 Feb 22.
Guidelines for Management of Joint Hypermobility Syndrome in Children and Young People. A Guide for professionals managing young people with this condition; The British Society for Paediatric and Adolescent Rheumatology (2012)
Hakim A; Is there a difference between Joint Hypermobility Syndrome (JHS) and Ehlers Danlos – Hypermobility Type (EDS-HM)? The consensus view of the HMSA Medical Advisors, Hypermobility Syndromes Association, 2013
My age is 28 and i have joints snapping and poping problem in all my body joints.some time i feel my joints broke😭😭. Also little bit pain if i work.Muscul is like elastic.. very stretchedPlease helpalam74544
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