Flexible Management for Flexible Children
It is well recognised that the management of children with Joint Hypermobility Syndrome (JHS) nowadays is very different to the advice and treatment provided in the past. All treatment and advice has the goal of ensuring the children are able to lead full and independent lives; including attending full time school, participating in sport and activities as well as having reduced experiences of pain and fatigue.
Present day understanding of JHS in Children
- Not all children who are hypermobile have the syndrome. Between 10-50% of all children are hypermobile but far fewer have the associated symptoms related to the syndrome.
- The most common symptoms are fatigue and mainly in joints and muscles but also abdominal pains and headaches.
- The musculoskeletal symptoms are related to the strength and fitness of the muscles and not the amount of extra movement the joint has. The less the strength and fitness is the greater the pain experienced. The pain is often most prominent after activity, at the end of the day and at night and this indicates that the muscles are not strong enough or fit enough for the whole day and all the activities. More rest will only increase the symptoms.
- Children with JHS will benefit from planning careers that do not involve manual labour; therefore attending school full time and getting a good education is vital.
- Too much rest and inactivity and the use of crutches and wheelchairs is detrimental to the musculoskeletal system of children. In general the muscles of children with JHS get weaker quicker than average and take longer to regain strength and so any activity that reduces mobility and activity will in fact make the symptoms ultimately worse.
- Hypermobility should be a biomechanical advantage for all young people, and this can only be achieved if excellent muscle strength and fitness is achieved. All professional sports people/dancers/ gymnasts are a prime example of this.
- In childhood and adolescence the body undergoes many changes both physically and mentally and the normal challenges of this change can be magnified in young people with JHS. The body’s response to pain changes as the body grows and for adolescents with JHS pain can become out of control and may dominate everything. This requires a combined physical and psychological approach, as pain medication does not reduce this type of pain. Many adolescents experience episodes of dizziness and ‘funny feelings’ and these symptoms can be increased in JHS. The most effective management involves increasing overall strength and fitness; drinking lots of water, marinating salt content of the body and reducing the speed of changing positions – such as taking slightly longer to get up from sitting etc. Constipation is common throughout childhood; however the incidence of it is higher in children/adolescents with JHS and will require input such as discussing the use of regular laxatives with the GP, drinking lots of water, and modifying the position sitting on the toilet. Being physically active is also very important for maintain healthy gut movement. Sleeping patterns naturally change from childhood through adolescence into adulthood and the normal fatigue of the teenager can be exaggerated in young people with JHS. Careful management involving maintaining regular sleep patterns, avoiding afternoon naps and increasing physical activity are all useful is counteracting this fatigue.
- The NHS has limited resources and so should be used in order to be able to allow you and your child to develop a self-management programme and then to be used for treatment following specific injuries etc.
- Dislocations in children are very rare and therefore should not be of concern to most with JHS.
- Children with JHS usually have joints that click a lot. This is in fact the body’s solution to pain and therefore should not be restricted. It will not cause any damage to the joint and is a very effective treatment for specific pain, though clicking should never be forced.
- Children who have JHS are often fidgety and need to move their joints frequently, again this should be encouraged.
Effective management of the symptoms of JHS is very possible nowadays. The evidence shows that pain medication is not effective in managing the pain and fatigue and can in fact make other symptoms, such as abdominal pain, constipation and head ache worse.
The most effective management for this condition is a combination of physical and psychological techniques.
Physical Management – physiotherapy, occupational therapy, physical activity.
Research completed at a major centre for managing JHS in children showed that a progressive resisted specific exercise programme (PRSEP) had significant response with a reduction of the symptoms of pain and fatigue.
In 80 children treated as outpatients with a home based programme progressing from 5 repetitions per specific exercise to 30 reps with 2.5kg weight; 100% of the children who completed the programme had a significant increase in strength and fitness back to full strength and fitness. They all reported a reduction in their pain: the average pain score was 5-6/10 in severity at the start, dropped to 3/10 after the initial course of treatment, and to less than 1/10 over time.
In another study looking at 32 children who attended a residential physiotherapy-led intensive rehabilitation programme very positive outcomes were demonstrated. The average increase in muscle strength after the 2 weeks was 80% with the majority reporting a 50% reduction in pain – reported by both parent and child.
80% of these children maintained their home exercise programme and their strength and energy levels continued to improve. Of these 80% full time school was achieved by all of them and they returned to sport and physical activity. In the children who maintained their strength and fitness and increased their activities 95% of them continued to see an improvement in their pain and fatigue.
PRSEP: The progressive resisted specific exercise programme consisted of exercises being prescribed targeting the muscles that were found to be weak on a detailed physical assessment. These muscles are then strengthened individually starting with low reps (5) but with constant and gradual increase to 30 reps and progressed further with the use of specific weights.
This approach is designed to ensure that each muscle is strengthened so that the joints are protected and the hypermobile range used effectively in order to reduce pain and fatigue and to ensure the body is fit enough so that complex activities such as sport and physical activities can be completed safely.
Occupational therapy has also progressed with its approach. As it is recognised that immobility is detrimental to hypermobile joints, splinting is virtually never indicated. The only exception would be for an extremely mobile thumb joint during writing. OT in put will be effectively focused upon handwriting skills, pacing skills and advice for schools.
All sport is possible for children with JHS, the most important aspect is that the child is strong enough for the activity and this is where the PRSEP is vital. Many people who do sport also complete a specific strengthening programme so that they are fit and strong – and the principal is the same in JHS.
Discussion with your physiotherapist regarding a specific programme is important so that you can be confident that your child has strong enough muscles in order to do the sport they want.
Psychological Pain Management Skills
The pain for young people with JHS does not respond to pain medication and so other pain management techniques should be used. Techniques such as relaxation, distraction and imagery have been shown to be very useful. However these are skills that have to be learned and practiced when the pain is not too severe so in times when the pain increases (such as the end of the day and bedtime) these techniques can be used effectively. Other senses can also be used to reduce the pain, such a smells which your child relates to a relaxed environment, and sounds, such as music, are also effective.
It is important not to focus on the pain as this immediately increases its intensity. It is also important to remember that the pain is telling you that your child’s muscles are not strong enough or fit enough but it is not a sign of damage or disease. Therefore it is very important for you to remain calm and positive as this will also give your child very positive reassurance too.
JHS in children is often complicated and has many symptoms that are associated with it; however now it is recognised and understood much more comprehensively and more importantly there are many treatments available that have very positive effects on the associated symptoms. With the correct approach this condition can be well managed and young people are able to participate fully in school and other activities with relatively well-controlled pain and fatigue.
Clinical Specialist Physiotherapist in Paediatric Rheumatology
Great Ormond Street Hospital
Updated June 2013 adapted from HMSA Newsletter Sept 2012. Date Planned for Review June 2015
For more information on helping children and adolescents, and advice for schools go to ‘Advice and Help’ and take a look at other articles including the HMSA booklets.
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Fatoye F, Palmer S, Macmillan F, Rowe P, van der Linden M. Proprioception and muscle torque deficits in children with hypermobility syndrome. Rheumatology. 2009 Feb;48(2):152-7
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