Physiotherapy and EDS-H / JHS
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When EDS-H / JHS is properly diagnosed, physiotherapy can play an important role in its management, with the goal of reducing pain and muscle spasm, and increasing the muscle tone around joints (as primary muscular hypotonia often occurs in EDS-H / JHS). 'However, if the condition is not diagnosed correctly a patient is likely to do less and less in response to the pain and discomfort. A downward spiral is initiated: symptoms worsen, stamina further decreases and chronic pain and fatigue develop.' (Julie Barber STATNews 2012)
In an article for The Rheumatologist, specialist physiotherapist, Rosemary Keer writes: 'Rehabilitation focuses on restoring a range of motion that is normal for each patient, helping the patient gain control of hypermobile areas, and improving joint proprioception. Exercises begin with establishing trunk stability and teaching appropriate recruitment of pelvic floor and transverse abdominus muscles. Exercises progress from static to weight bearing, and functional activities are encouraged as patients improve'.
Physiotherapists, specialising in hypermobility, will often begin by guiding patients towards exercises that concentrate on strengthening their internal core and correcting their posture to improve stability. 'Stability always precedes mobility in coordinated action... it is extremely important to ensure stability before directing a person outwards into mobility' (Julie Baber 2012).
Adopting Good Posture:
Good posture is often over looked in the battle to treat pain in EDS-H /JHS, but it is actually crucial. Left untreated, poor postures can cause significant muscle pain and contribute to injury. Take a look at the image below and try to identify which one most closely describes you. If necessary, ask a friend or family member to help.
As an illustration of the effects of poor posture, we will look at the ‘forward head pose’. Tight hamstrings are often an indication that someone has a ‘forward head posture’. Tight hamstrings can be the reason why some hypermobile people, who are flexible in so many ways, find that (despite what they have seen in clinical diagnosis leaflets - showing poses that can often be performed by those with EDS-H / JHS), they cannot bend forward with their legs straight and place their hands flat on the floor. It is indeed a common misconception that all hypermobile people can do this. In fact, many hypermobile people have extremely tight hamstrings (tendons that run up either side of the back of the leg behind the knee and up into the posterior), caused by years of poor posture.
'Forward head posture will lead to neck pain and headaches from tight muscles and restricted nerves from the neck and upper back. You may also have a forward tilt of the pelvis caused by tight hip flexors. This compresses the lumbar spine resulting in low back pain and stretches the hamstrings and sciatic nerves. This then forces the thigh into internal rotation twisting the knee and placing stress on the lateral thigh (outside). These areas become shortened and are usually implicated in cases of lateral knee pain. This in turn twists the lower leg and is associated with over-pronation of the foot (flat feet).’ (Quote: Fix Your Posture, Triradar.com)
It is often enlightening for a person with EDS-H /JHS to take a really good look at their posture. Standing sideways in front of a wall mounted, full length mirror; try to make your shoulders, back, bottom and legs form a straight line down one edge of the mirror frame (as if you’re standing against a wall). If you can see gaps between you and the mirror edge, try tightening (pulling up) the muscle in your bottom and the muscles in the front of you thighs, whilst pulling in the area of your stomach that runs along the bikini line; tuck your chin and neck back slightly and relax your shoulders. Take the time to feel your feet on the floor; are you putting too much weight through your heels; are all your toes touching the floor; is your weight evenly dispersed through your feet; do your arms hang in front of your body or at your sides?
Changing posture is not something that can be fixed overnight, after all it will have taken many years to develop, but through a combination of specifically targeted exercises, determined conscious effort and sustained practice, great improvement can be made. Proper assessment and exercises designed to strengthen under-used muscles, prescribed by a podiatrist, gait analyst or physiotherapist with a real understanding of EDS-H / JHS is very important. Patients with EDS-H / JHS may need more help than most in learning correct posture, as many also struggle with poor proprioception (the body’s ability to transmit a sense of position, analyse that information and react, consciously or unconsciously, to the stimulation with the proper movement (Houglum 2001)). They will need to learn to recognise and constantly correct posture throughout the day and to carry out prescribed exercises to strengthen targeted areas of weakness. They may also need to wear made-to-measure orthotic insoles in supportive footwear .
Some important factors should be taken into consideration when putting together a physiotherpay treatment plan for a person with a connective tissue disorder such as EDS-H / JHS
In many ways, the physiotherapy approach required is the opposite of traditional physiotherapy. Instead of aggressive, short-term building of muscle mass through rapidly increasing weight or resistance, physiotherapy for EDS-H / JHS has to be focused on long-term, gentle building of muscle tone.
Special attention has to be paid to ensure that physiotherapy does not injure the person with EDS-H / JHS further and that joints are not over extended or slipping out of place.
While some degree of muscle ache is part of building muscle, phyisotherapists must be aware that, for those with a connective tissue disorder, such as EDS-H / JHS, some types of pain during physiotherapy may also be signaling joint instability and subsequent tissue damage.
Use low resistance exercise to gradually increase muscle tone and to help stabilise loose joints; minimise joint trauma by avoiding joint hyperextension and any high resistance or impact activities.
Low resistance, high repetition activities
Low-impact aerobics and/or water aerobics
'Low resistance muscle toning exercise can improve joint stability and reduce future subluxations, dislocations, and pain. ...Improved joint stability may be achieved by low-resistance exercise to increase muscle tone (subconscious resting muscle contraction, as opposed to voluntarily recruited muscle strength). Examples include walking, Bycycling, low-impact aerobics, swimming or water exercise, and simple range-of-motion exercise without added resistance.
Resistance exercise can exacerbate joint instability and pain. Elastic resistance bands should be used with caution, if at all. In general, it is preferable to increase the number of repetitions of exercise rather than to increase the resistance. Isometric exercise can also be problematic if too much force (resistance) is applied.
Progress should be made by increasing repetitions, frequency, or duration, not resistance. It may take months or years for significant progress to be recognized.'
(Quotes: [Levy] at Manual Techniques)
(NB/ A slightly different approach, to that given by Dr. Levy (above), is discussed later on this page)
The goal is to improve static and dynamic muscle tone to promote increased joint stability during weightbearing and functional activities.
Remember that, just as it often takes years for joint laxity to cause significant pain and instability, it can take at least months and usually years to gradually reverse the process via muscle toning exercise.
Consideration as to the impact of certain exercises on problematic hypermobile joints should be considered before embarking on them. Take cycling as an example: -
Cycling is an excellent all-over work out for the body, and often recommended as a way of strengthening the knees and legs in those with EDS-H / JHS. However, those who are prone to subluxation or dislocation of the patella should take into consideration that during the 'push' phase of pedaling, cyclists seldom complete the last 35˚ of knee extension; a movement which is largely under the control of the vastus medialis oblique (VMO) muscle. This means that the muscles down the outside of the knee/thigh become much stronger and tighter than the less-used medial muscles such as the VMO, which are found on the inside of the knee/thigh.
The patella can be pulled subtly off-kilter (towards the outside of the knee), which can lead to increased subluxations, dislocations and pain. Advice from a physiotherapist, experienced in EDS-H / JHS, before deciding on a form of exercise can be invaluable. They can often suggest compensatory exercises, such as those that will strengthen the VMO muscle and inner medial areas of the knee, which can be carried out after each session of cycling, in order to balance out the stabilising forces of the knee.
Stabilising forces on the knee
Vastus Muscles and Rectus Femoris – the medical names for separate parts of the quadriceps (front of thigh) muscles. Iliotibial Band (ITB) – a thick, ﬁbrous strap of tissue running all the way down the outside of the thigh. Can get very tight, pulling strongly on the kneecap.
Lateral retinaculum – short, tough ligaments tethering the kneecap to the ITB. Can shorten over time, compounding problems of a tight ITB.
Medial Retinaculum – similar to lateral retinaculum but less ﬁbrous, exerting less of a pull on the kneecap.
Patellar Ligament – a continuation of the tendon coming from the quads muscles. It's how they attach to the shin bone.
No 'protocol' of physiotherapy interventions exists to address the impairments and functional limitations associated with EDS-H / JHS. This is due, in part, to the varied presentation of the condition in each affected individual. Therefore, each physiotherapy plan of care must be specially created for the patient depending upon the subtype of EDS and the signs and symptoms of that patient.
In general, physiotherapy intervention focuses on increasing joint stability through a low resistance, high repetition training program, preventing excessive loading through weightbearing joints, avoiding excessive use of involved joints for heavy lifting and using adaptive equipment to accomplish activities of daily living without accentuation of symptoms, and pain relief/pain management.
The information above reflects the prevailing view, that those with EDS-H / JHS should avoid using their additional range of movement when carrying out physiotherapy, and should increase the number of repetitions of exercise, rather than increasing the resistance, but research and expertise are evolving all the time and some therapists take a slightly different approach. The following paragraphs provide an example of one such approach, called PRSEP, which has been pioneered by a 'centre for the management of JHS in children'. It is hoped that this new approach will prevent today's children from going on to develop some of the debilitating complaints often seen in today's adult symptomatic-hypermobile population.
Patients whose joints hyperextend (extend beyond what would be considered normal in
non-hypermobile people), are often encouraged by their physiotherapist to learn what is considered ‘normal range’. For instance, a physiotherapist may place their hand behind your knee, acting as a stopping point to mark the maximum range you should extend into. Patients are often told that they should learn not to extend into their hypermobile range because they risk injury to the joint.
In day-to-day activities, it is indeed prudent not to over-extend weak/loose joints, but in order to strengthen and protect those joints from future injury, physiotherapists at Great Ormond Street’s Rheumatology Unit (GOSH), which specialises in joint hypermobility and fatigue in children, have had success using a different approach. In severe cases, GOSH will admit a child for a two-week ‘in patient’ intensive physiotherapy rehabilitation programme. The children take part in a progressive resisted specific exercise (PRSEP) where children are encouraged to exercise fully into their maximum range in a controlled manner, gradually building up use of resistance weights from 1lb to 8lb (for example, the child may be discharged at the end of the fortnight having achieved the ability to carry out the prescribed excercises using a weight of 3lb and will then be set the goal to reach 8lb by continuing the exercises at home over the following 6 months) . The belief is that for a hypermobile person the extra range given to them is ‘their normal’. If this extra range is not strengthened it will remain unprotected and then, when they accidentally slip back into this extra range whilst carrying out an activity one day, the extra range, which has been left weak and unprotected, will inevitably suffer injury.
Research, completed at a major centre for managing JHS in children, showed that the those who undertook the progressive resisted specific exercise programme (PRSEP) showed 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.
This specialist programme may be particularly effective where children are suffering from weak joints and/or deconditioning related fatigue and need an intensive programme to strengthen all the muscles in their body. A conservative approach to treatment, through physiotherapy and pain management, is promoted and surgical intervention strongly discouraged. However, children who are suffering from repeated dislocations may wish to consider undergoing an MRI scan prior to commencing the programme, in order to establish, from the outset, whether the underlying cause is purely inadequate muscle strength, or whether a combination of weak, damaged or ruptured ligaments is further complicated by skeletal abnormalities (such as severe trochlear dysplasia and patella alta in the knees), whereby surgical intervention may still need to be considered. This programme is available via consultant's referral only.
(For more information on treatment of hypermobility in children please go to: and search for 'Flexible Kids').
Only time will tell if this different line of thinking will prove beneficial. It is hoped that this relatively new approach to physiotherapy treatment for symptomatic-hypermobility may prevent today’s children from suffering some of the debilitating symptoms often reported by today's symptomatic adults.
A recent study into, what appears to be, a similar programme in Australia concluded that: significant improvements in child-reported maximal knee pain were found following treatment, regardless of whether the children were allocated to the group allowed to exercise into their maximum range, or the group who were not. Significant differences between treatment groups were noted for parent-reported overall psychosocial health, specifically self-esteem, mental health and behaviour, in favour of exercising into the hypermobile range compared to neutral range only. Conversely, parent-reported overall physical health significantly favoured exercising only to neutral. No other differences were found between groups and no adverse events occurred.
The study established that, regardless of which group the children were allocated, physiotherapist prescribed, supervised, individualised and progressed exercise programmes can effectively reduce knee pain in children.
So, physiotherapy is considered a mainstay in the management of EDS-H / JHS, but is physiotherapy alone enough?
Appropriate exercise, increasing muscle strength and endurance , undoubtedly, plays an extremely important roll in the treatment of EDS-H / JHS. At times, however, it can seem that this message is promoted within the medical community as the ‘only’ treatment required for EDS-H / JHS, or even as a cure.
It is important to realise that although physiotherapy is the mainstay in management of the condition, it can only do so much. Physiotherapy can significantly improve a patient’s musculaskeletal symptoms; it is unlikely, however, to have much effect on the areas of the body’s internal organs, which are so frequently prone to issues caused by defective collagen in those with EDS-H / JHS. Promoting the concept that physiotherapy is the ‘only’ treatment that is required for patients with EDS-H / JHS, is misleading and incomplete advice. Of course medical practitioners need to address musculoskeletal symptoms, but they also need give due consideration to any overlying symptoms, which the patients are experiencing, and to really listen to the impact these issues have on a patients life. Many have debilitating problems relating to dysautonomia, delayed gastric emptying (gastroparaisis), compliant (floppy/sluggish) bowels (caused by increased elasticity of the gut), and many other symptoms, which cannot be treated with physiotherapy. These symptoms need to be properly investigated and treated appropriately.
Treatment of the patient ‘as a whole’, needs to be considered, taking into consideration all of their symptoms and, where necessary, the appropriate referrals must be made. When all of the patient's symptoms have been taken into consideration, a lifelong commitment to appropriate exercise, which is, most certainly, the cornerstone in the management of this condition, needs to be taught to, and undertaken by, patients. Promoted from a young age, using specific exercises and fun activities that, wherever possible, can be incorporated into daily life, exercise can be a significant tool in EDS-H / JHS managment and, most importantly, can be enjoyed!
References: For references and information sources used within this site, please see 'References' page, under 'Resources and Links' on the main menu.