Understanding pain and fatigue in joint hypermobility and EDS

Children with joint hypermobility often experience pain in their joints and muscles. This is expected after injury to a joint but sometimes the pain occurs after even mildly strenuous exercise or without any apparent cause.

Pain following injury to a joint

Hypermobile joints are less stable than normal and as result are more prone to injury especially when there is associated muscle weakness and poor coordination.

Joint sprains and subluxations are the fairly common injuries in JHS and result from overstretching the lax joint ligaments and capsule. The overstretching leads to damage to the joint connective tissue which leads to inflammation, swelling and pain. Over time the inflammation and swelling decrease and the pain should become less. 

The RICE routine (Rest, Ice, Compression and Elevation) helps to minimize the amount of inflammation, swelling and pain following an injury. Rest does not mean keeping the joint quite still. It  important to start with gentle movements early on. This not only helps to reduce the swelling but also restores normal movement and importantly helps to overcome the fear of movement that can develop after an injury. 

Once the inflammation has subsided and the swelling starts to go down, a program of exercises to restore full range of movement, strength and coordination is very important, not only to ensure full recovery of function but also to help prevent injury in the future.  

After an injury movement will still cause some pain as the injured structures are stretches and start to move. However, it is possible to exercise in a way that provokes very little pain and restores full function very quickly.  The trick is to overcome the fear of movement and do lots of small movements each time going just a little further. and to start moving against some resistance early on.  

Studies have shown that strengthening muscles and improving coordination can help to prevent subluxation of joints. 

The link between injury and pain

Generally speaking the amount of pain experienced following an injury is related more or less to the degree of damage.  It alerts the individual to the injury, helps in promoting the early physiological response to the injury and by stopping movement may help to prevent further injury in the short term. In other words pain following an injury serves a purpose. 

If  full range of movement  and good muscle strength, coordination and balance are not restored after an injury the joint capsule, ligaments and muscles may continue to be a source of pain, with fear of movement and pain leading to reduced activity levels which in turn leads to sensitisation in the pain pathways.  What seems to happen is that the pain receptors in the joint  become oversensitive and start to respond in an abnormal fashion and this in turn leads to over activity in the sensory areas of the brain so that the link between injury and pain breaks down.  (See below.)

Muscle pain from trigger points  

Trigger points (TrP's) are small bands of thickening within a muscle which are very sensitive to compression and stretching and are an important source of muscle pain in joint hypermobility. 

Trigger points develop in weak and tight muscles. They may be silent and only painful when compressed. But sometimes they become sensitized and start firing whenever the muscle contracts or is stretched, and they may even  start to fire spontaneously leading to pain even at rest.  

Pain from TrP's may be a dull nagging ache or a burning pain.  It can be localised or spread over a wide area. Sometimes the pain is felt in the area of the TrP, but often it is experienced at a distance from the tight band of muscle. The term for this is referred pain.  

Pain from a TrP in a muscle often leads to spasm and or weakness in the affected muscles. This in turn leads to abnormal patterns of movement at the joints (referred to as muscle imbalance) overuse of muscles and formation of Tr P's in surrounding muscles.

The formation of painful trigger points may be triggered by inactivity,  anxiety and sleep apnea.  When an individual develops widespread and persistent pain associated with many tender TrP's along with fatigue and other symptoms a diagnosis of fibromyalgia may be given. (See below.)

How to reduce pain from trigger points 

Pain from muscle TrP's  can often be lessened with gentle stretching of the affected muscles. The difficulty lies in applying a stretch that targets the tight muscles and does not overstretch lax joint structures.  (You will find a few ideas for stretching the leg muscles on the  Leg  and night pain in joint hypermobility.)

Gentle stretching is great for relieving pain but to really be effective the tight and weak muscles containing the TrP's need to be strengthened in order to provide sustained relieve of pain.  Implementing a program of strengthening and coordination exercises , overcoming the fear of movement and increasing activity levels is the only way forward for chidlren with JHS/EDS. 

Active stretching 

Active stretching is an adaptation of the well established stretching technique known as hold-relax.  The muscle is put on the stretch and then the stretched muscle is made to contract.   

Try the following

Sit erect, tuck your chin in and bend your head forwards. Notice the stretch in the muscles at the base of the skull. There may even be some pain or discomfort. 

Now press your fingertips against the back of your head. At the same time push your head back against your fingers and maintain this pushing action for  5 seconds. Relax and repeat the push and hold  2 more times. Lift up your head.

If you now bend your head forwards you will find that you can bend the head further forward with less stretching discomfort. 

(Sign up as a SfA member to get updates on new pages with safe targeted stretching exercises for children.)

subscribe to participate.png

Pain following exercise 

Everyone experiences muscle pain after a bout of  unaccustomed or strenuous exercise. This pain often occurs  24 hours after the exercise bout and lasts about 48 hours. 

However children with sensitized trigger points in muscles may experience quite severe pain after even mild to moderate exercise.  The pain may occur immediately after exercise or later at night when the child settles down to sleep.  

In some cases this pain arises from joint which are being overstretched and where the pain receptors have become oversensitive and start to fire at the smallest provocation. 

How to prevent and reduce pain following exercise

Keep moving, get stronger and deal with tight muscles 

  1. Improve general fitness to improve heart and muscle endurance. This improves the flow of oxygen to the muscles and allows them to work aerobically. 
  2. Strengthen muscles to provide added protection to the joints.
  3. Identify tight muscles and do some gentle stretching to restore full mobility and desensitize TrP's  

Creating an exercise program to meet your child's needs

Your best option always is a full physical therapy assessment  and exercise program tailored to your child's specific needs with a therapist who has a special interest in joint hypermobility. 

This is not always possible.  In order to provide parents with an alternative SfA offers an online physical therapy advice service which includes an assessment of the child's particular needs and a suggested exercise program.  For more information please email pam@skillsforaction.com

Chronic pain 

For the most part acute pain is related to signals coming from injured tissue. However when the pain receptors and pain pathways become sensitized they take on a life of their own and start to fire spontaneously or in response to sensory stimuli that would not normally cause pain.

This abnormal activity is referred to as sensitization, wind-up or kindling.  It is not clear why such sensitization happens in some children, and why children with JHS develop chronic pain. When this happens the term chronic pain is used.

The American Pain Society defines chronic pain as follows: 

"While acute pain that follows bodily injury is generally self-limited, in some patients the pain persists beyond the expected healing time (arbitrarily defined as >3–6 months) and develops into a chronic persistent or recurrent pain syndrome.

The important thing to know about chronic pain is that it sets of a chain of  physiological, emotional and social responses including sleep disturbances, feat of moving, fatigue, anxiety and depression which all interact to sustain pain and fatigue and interfere with the child's ability to participate fully in everyday and school life. 

Chronic pain is also made worse by stress, fatigue, being hungry and tired, inactivity. sleep disturbances  and so on.

Chronic fatigue 

Many individuals with JHS who have persistent and recurring episodes of pain also suffer from chronic fatigue.  Like chronic pain, chronic fatigue is related to poor fitness levels, muscle weakness, lack of activity as well as disrupted sleep, anxiety. 

There seems to be some evidence that chronic fatigues is also related to sensitization and over activity in the areas of the brain that register the sensations of effort that arise when muscles are working hard. 

Disrupted sleep due to pain or obstructed breathing  is an important underlying cause of daytime fatigue in children.  In obstructive sleep apnea breathing stops for 10 s or more several time an hour.  This leads to decreased oxegen to the brain for brief periods. It also stops the child from achieving periods of deep sleep necessary for brain the brain to recuperate and lay down memories. 

Sleep apnea also leads to daytime fatigue, irritability,  as well as anxiety, poor attention and cognitive skills. 

More about sleep apnoea

"Usually, each time we breathe in, air passes through the nose, where it is filtered, warmed and moistened. It then travels down the upper airway to the lungs. The oxygen contained in the air is absorbed into the bloodstream in the lungs and is transported to where it is needed in the body.

When we are asleep, the muscles in the body naturally relax and become floppy. In some people this can cause the upper airway to fall in on itself, making breathing difficult. This can lead to bigger and bigger efforts to breathe, which can narrow the airway further. At this stage, the body senses the airway problem and the person wakes up briefly.

This returns the airway to normal again, allowing them to breathe, and the person goes back to sleep. This process of periods of struggling to breathe, followed by waking briefly, may happen many times during the night.

Generally the person recalls very few, if any, of these events in the morning. This condition is called obstructive sleep apnoea."   Great Ormond Street Hospital for Children  Read more

Children with obstructive sleep apnea  tend to snore a lot, may be very restless and sleep in funny positions. They complain of being tired when they wake up. 

Help for children with chronic pain and fatigue

Because of complex interactions between biological (related to the body structures), emotional and social aspects of chronic pain interdisciplinary chronic pain clinics or teams usually provide the most effective way to help a child with chronic pain.

What is included in a good chronic pain program

A medical assessment and medication 

A full medical assessment by a physician with a special interest in joint hypermobility and chronic pain is very valuable. This will include looking for and ruling out any underlying disorders that may be contributing to pain and disability. 

Checking for sleep disorders is also very important. Poor sleep not only increases pain but also affects attention and daytime fatigue. 

The physician also works with the child and the family to find the best way to use medication for dealing with anxiety, damping the overactive and sensitized pain  pathways and for managing day to day pain experience. 

Education about the complex nature of chronic pain.

It is really important that the child and family understand that in chronic pain, the pain does not signal ongoing or new damage to tissues.

Pain that occurs with movement provokes a fear that the movement is causing more tissue damage and it is very important to understand that this is not the case.

Understanding how chronic pain impacts on family life and function

Working with the child and family to understand how the child’s chronic pain impacts on family life and on the child’s function and participation at home and at school. It is also important for the health care provider, the child and the family start to look at how behavior patterns may help to sustain pain behaviors. 

Dealing with pain behaviors, anxiety and attitudes 

Cognitive therapy and mindfulness training can help the child to respond to the experience of pain in a different way.   Decreasing anxiety and fear responses and learning to shift the focus of attention helps to moderate the pain experience and allows the child to start participating in school and family life despite the pain.

Fitness training for strength, flexibility, participation and activity levels

Fitness training helps to build muscle strength, improve coordination and allows the child to use exercise as a way to manage stress and participate with their peers in the playground. 

Fitness training needs to be approached carefully and in a stepwise fashion so that it does not provoke an increase in pain but rather increases the amount of exercise a child can do before pain is experienced.

Some tips for designing a fitness program for children who experience chronic pain.

First a few basic rules

1  Fitness training should be enjoyable – even fun.  

So to start with choose activities that your child enjoys.  Playing  Wii Fit games can be a good entry to getting more active.

2  Start with activities that do not cause any pain and are fun.

If your child has pain in the legs, you can start with ball games sitting on a chair. If your child likes being in the swimming pool, doing exercises in water can be a good place to start,

The important thing is to help your child get past the fear of movement and to get the heart racing a little, and the “lets go for it”  juices flowing

Be ready with the pain meds, a nice warm bath or shower, or hot packs if they are needed after a bout of exercise.  

3  Next you need to move in on the painful joints and muscles.

In this phase you start with pain free movements of the trunk or affected limb. This helps to overcome the fear of moving and starts to get the muscles working and provides the sensory pathways with a more balanced input which in turn helps to moderate the activity in the pain pathways. 

Here are some examples.

If the knee is affected: sit on the edge of a table with the legs dangling down and move the lower leg backwards and forwards as far as is comfortable. Do 10-20 reps.

Or you can run a deep bath and practice bending and straightening the leg with the support of the water.  

The hip joint can be moved standing up and lifting the leg forwards and backwards and sideways. This action works the muscles of both the standing and the moving leg.

4  Lastly start strengthening the muscles in functional activities.

This is called task based training. It is based on the principle that muscles need to be strengthened to work effectively as part of a  team of muscles working in a coordinated fashion to achieve a task. 

Tasks can be adapted to make them less strenuous and then slowly changed to increase the level of difficulty.

An example of a graded approach to task based strengthening:  

Standing up and sitting down to strengthen the knees.

Start by sitting on a high stool so that the hips and knees are only bent a little way and it is easy to stand up.

Practice standing up and sitting down 10 times fairly slowly and with good control. This builds confidence and control. 

Next speed up the movements. How many reps can be completed in 10s. 

Once standing up from a high stool is mastered, the next step is to lower the height of the stool so that it takes more work to stand up and sit dowm. 

Practice standing up and sitting down 8-10 times in a controlled manner. 

Move on to doing the exercise faster.

Before starting an exercise program - check with your child's physician. 

Although it is usually safe to start an exercise program with your child, especially if it is carefully paced to meet a child's abilities, it is best first to talk to your child's doctor about possible precautions. This is particularly important in children with EDS who may have autonomic symptomes such as POTS, respiratory or heart involvement that needs to be considered. 

Useful Resources 

Guidelines for Management of Joint Hypermobility Syndrome in Children and Young People.

These guidelines have been compiled by the Allied Health Professionals Group of the British Society for Paediatric and Adolescent Rheumatology (BSPAR, 2012).

Bibliography and references

3 Comments

Thank you for this article!Jen (not verified)

Comment: 

My childhood doctors couldn't make sense of my pain, couldn't get their heads round the fact that swelling would come and go, the marked chronic pain and fatigue after activity. One particular pediatrician insisted it was somatic, that if my mum really wanted to help me she should accept a referral to child psychiatry. We've both been mentally scarred by it. Now I have CFS/ME, fibromyalgia, POTS symptoms, frequent subluxations. I will be seeing a rheumatologist soon, just happens to be the same one I saw as a kid! Lot's of people online have suggested I have EDS. I think it's all clicking into place finally. Or out of place as the case may be!
joint pain and mobilityBarbara Marshall (not verified)

Comment: 

This is me as a child, it explains a lot and being on the spectrum probably made it worse. A physio used to tell my mum to do those feet exercises and mum used to do them. I also had foot orthotics for knocked knees and flat feet. Thankyou for showing me this again.

Yours sincerely

Barbara Marshall from Australia

Please help to improve the information on this page pam

Comment: 

Hello 

I have worked hard to ensure that the information on this page is accurate and reflects the best available evidence. 

Pain is a complex subject and it is tricky business condensing ideas into non-technical language without oversimplifying and misrepresenting information. If I have done this, please post a comment so that I can sort it out. 

I would also welcome suggestion from parents, children and young people with regard to living and dealing with JHS and chronic pain. 

Pam 

Receive weekly updates in your in-box

subscribe to participate.png

Add new comment

Users

  • No HTML tags allowed.
  • Lines and paragraphs break automatically.

Plain text

  • Allowed HTML tags: <a> <em> <strong> <cite> <blockquote> <code> <ul> <ol> <li> <dl> <dt> <dd>
  • No HTML tags allowed.
By submitting this form, you accept the Mollom privacy policy.