PART FIVE: TREATMENT AND MANAGEMENT

11.0 Treatment and Management

“Emotional support and behavioral and psychological therapy may help in developing acceptance and coping skills.” (Malfait et al., 2018).

Early diagnosis is extremely important for an individual’s present and future health. In general, symptoms will be treated as they arise. Care is largely preventative – being to support and manage the sub-type with the aim of preventing any damage or keeping any damage to a minimum.

Depending on the EDS sub-type and symptoms, treatment may include:

  • Physical therapy;
  • Medication;
  • Braces and cushions;
  • Compression wear/garments;
  • Psychological/psychiatric;
  • Dental treatment;
  • Surgery; and/or
  • Exercise.

It cannot be understated the importance of working with professionals who have the skills, knowledge, and experience of EDS, including:

  • Medical professionals (e.g. general practitioners, rheumatologists, and orthopaedists);
  • Physical therapists (e.g. general physiotherapists, sports physiotherapists, and occupational therapists); and
  • Exercise professionals (e.g. fitness instructors and personal trainers).

The skills, knowledge and experience of the above will vary. Most will unlikely have encountered the condition and there are no specific qualifications, especially for exercise professionals, aimed at EDS or hypermobility.

11.1 Physical Therapy

In general, physical therapy intervention focuses on decreasing the patient’s disability from a multidirectional approach. This includes activities of daily living (ADL’s), ambulation, sports activities, and quality of life. However, there is not set protocol (as of 2019) to address the impairments and functional limitations associated with EDS due, in part, to the varied presentations of the condition. Consequently, each individual has a plan of care that is specially developed for them depending on the sub-type of EDS and the symptoms displayed.

“Physical therapy is considered a foundational treatment approach for a number of the different types of EDS. Unfortunately the outcomes associated with physical therapy management are not always positive.” (Laferrier et al., 2018, p.383).

There have been reports that physical therapy has exacerbated symptoms or individuals have received treatment that has only focussed on one joint, rather all joints affected (Laferrier et al., 2018). At least one study suggests that those who received exercise advice from a physiotherapist were 1.75 times more likely to report high volumes of weekly exercise (Simmonds et al., 2017) than those with no advice. For those where physical therapy is positive, it is important to address the following:

  • Pain;
  • Proprioception;
  • Balance and stability;
  • Muscle tone; and
  • Overall physical fitness.

This can be achieved through a four-step model:

  1. Manage movement dysfunction such as hypermobility, pain, and decreased and impaired movement patterns.
  2. Enhance functional abilities such as challenges with daily, social, and recreational activities.
  3. Restore, maintain, and promote optimal function, optimum wellness, and optimal quality of life.
  4. Prevent the progression of functional limitations and disabilities.

Myofascial release (any physical therapy modality that reduces spasm) provides short-term relief of pain, lasting hours to days. While the duration of this benefit may be short, and needs to be repeated frequently, this pain relief may be critical to facilitate participation in toning exercise for stabilisation of the joints. Modalities must be tailored to the individual and includes (but not limited to):

  • Myofascial release may be short-term relief, but can be critical in facilitating participation in toning exercises for stabilisation of joints.
  • Heat/Cold therapy.
  • Massage therapy.
  • Biofeedback/conscious relaxation.
  • Low-resistance muscle toning exercise programme.
  • Ultrasound.
  • Electrical stimulation.
  • Acupuncture.
  • Acupressure.

Low-resistance muscle toning exercise can improve joint stability and reduce future subluxations, dislocations, and pain. Transvaginal pelvic physical therapy and myofascial release (in which massage or ultrasound is applied to the pelvic musculature via a transvaginal approach) may improve dyspareunia, abdominal pain, back pain, and sometimes radicular lower-extremity pain. However, this should only be performed by a physical therapist with training and expertise in pelvic floor physical therapy and with patient consent.

In 2018, Laferrier and colleagues (2018) published a paper which outlined the possible benefits of a novel exercise protocol for an individual with classical EDS, and was based on a book (published in 2015) written by one of the authors, Kevin Muldowney. Although only based on one patient, and therefore requiring careful interpretation, the results certainly showed promise. At the time of publication, the authors believed it to be the only EDS specific exercise protocol being used clinically. However, a book published in 2013 by Dr Alan Hakim and colleagues (2013) suggests this was not quite accurate.

It is important to note that not all physiotherapists have the knowledge and experience to deal effectively with EDS patients. This means it is important to ‘shop around’ for a physiotherapist who can help you manage and treat the symptoms of your EDS effectively, as it could have a significant impact on your quality of life.

11.2 Medication

Medication should be tailored to the individual’s subjective symptoms and objective measures of pain, not to physical examination or radiologic findings.

  • Mild to moderate pain: May get sufficient relief from as-needed use.
  • Significant pain: May require higher doses and combinations of multiple medications.

Prevention or control of pain with regularly scheduled dosing is often more successful than acute treatment with as-needed dosing.

Medication that may be beneficial (depending on sub-type and symptoms) to those with EDS include (Levy, 2018):

  • Acetaminophen:
    • Will not completely alleviate pain but is a useful and well-tolerated adjunct in combination with other agents.
  • Non-steroidal anti-inflammatory drugs (NSAID’s):
    • These include ibuprofen, naproxen, meloxicam, and nabumetone.
    • Particularly useful for arthralgia, myalgia, and secondary inflammatory conditions (e.g. bursitis, tendinitis, costochodritis, or post-dislocation pain).
    • Bruising is not a contraindication to NSAID therapy, but occasionally requires dose reduction or change to a Cox-2 inhibitor.
  • Cox-2 Inhibitors:
    • Cyclooxygenase (COX) is the enzyme which mediates the bioconversion of arachidonic acid to inflammatory prostaglandins.
    • These include rofecoxib, celecoixb, and valdecoixb.
    • May be better tolerated than NSAID’s and thus more effective.
  • Topical lidocaine:
    • As a cream or patch is sometimes useful for localised areas of pain.
    • Topical capsaicin is of questionable utility, but is safe.
  • Skeletal muscle relaxants:
    • Can be useful in combination with all of the above to treat myofascial spasm.
    • They are also sometimes helpful in treating neuropathic pain.
    • Metaxalone may be the least sedating, but all are potentially limited by sedation.
    • There is theoretical risk that skeletal muscle relaxants can increase joint instability by reducing muscle tone.
    • This is not a contraindication to their use, but does justify monitoring for potential complications.
  • Magnesium:
    • Either topically as Epsom salt baths or orally in any form, may also reduce muscle spasm and pain.
    • No specific formulation or dosage has been established as superior to any other.
    • The most common adverse effects – sedation, nausea, abdominal pain, and diarrhoea – are more likely to occur with oral rather than topical supplementation.
    • Also potassium.
  • Tricyclic antidepressants:
    • Often effective for neuropathic pain, with additional benefits of mild sedation (for those with sleep disturbance) and a little mood elevation.
    • Constipation, a common side effect, can be managed with fluids, fibre, stool softeners, and laxatives.
    • For those with diarrhoea-predominant irritable bowel syndrome, the constipating effect may be therapeutic.
  • Serotonin/norepinephrine receptor inhibitors (SNRIs):
    • These include venlafaxine, desvenlafaxine, duloxetine, and milnacipran.
    • They also offer combined benefit for depression and neuropathic pain.
    • Venlaxafine may raise the blood pressure a few points, which potentially could be helpful for individuals with neurally mediated hypotension.
  • Anti-seizure medications:
    • These include gabapentin and pregabalin (both associated with weight gain) (Ericson Jr & Wolman, 2017), topiramate, and lamotrigine.
    • Some are also effective for neuropathic pain and can be used in addition to tricyclic and/or SNRI antidepressants.
    • All require gradual titration before reaching therapeutic levels.
  • Steroids:
    • Short courses of steroids can be very effective for controlling acute flares of pain associated with secondary inflammation.
    • However, as EDS is not an intrinsically inflammatory condition, and there is no role for chronic steroid use.
  • Glucosamine and chondroitin:
    • May help to prevent or treat osteoarthritis in the general population.
    • They have not been studied specifically in EDS, but are not contraindicated.
  • Cannabinoids:
    • Dronabinol and (where legal) marijuana may be helpful for several different types of pain.
    • Their benefits should be weighed against the potential for dependency and/or psychoactive effects.
    • Cannabidiol (CBD) was isolated in marijuana in 1940 and its chemical structure was characterised in 1963. It does not have euphoric effects and is increasingly being used in oils, edibles and other forms to treat medical conditions such as pain.
    • The human body has two types of receptors CB1 (found in the brain) and CB2 (found in the rest of the body, immune cells and glia cells in the central nervous system). Chemicals that cause inflammation in the peripheral parts of the body are modulated by cannabinoids and, when applied topically, may be helpful.
    • CBD works on CB2 and is responsible for pain relief and helps autoimmune dysfunction.
    • With regards to EDS, the research on the efficacy and effectiveness is divided (Chriss, 2018), with some showing positive results and others no difference, especially when measuring pain.
    • On 01 November 2018, the UK government changed the law so that doctors can now prescribe cannabis-based medicines, however this did not extend to chronic pain or hypermobility-related conditions (The Ehlers-Danlos Support UK, 2018).
    • There are also medications that potentially interact with CBD, which are frequently used by people with health problems, including:
      • Amitriptyline which is used to treat depression, pain and migraines;
      • Ibuprofen which is used to treat pain; and
      • Meloxicam which is used to treat arthritis.
  • Tramadol:
    • Can be added to acetaminophen plus an NSAID or Cox-2 inhibitor before resorting to opioids.
    • Nausea is the most common side effect.
  • Opioids:
    • Are effective for both myofascial pain and neuropathic pain, but should be reserved for use after failing the above medications.
    • They should be administered in conjunction with all of the above, except tramadol, in order to minimise total opioid requirements.
    • Aince they are typically used chronically (or at least several months), the primary formulation should be long acting (e.g., sustained-release oxycodone or morphine or topical fentanyl patch) with short-acting forms of the same drug used as needed for breakthrough pain.
    • Routine use of two or more daily doses of a short-acting form should prompt an increase in the long-acting dose or another adjustment to the pain regimen.
  • Benzodiazepines:
    • May offer some short-term reduction in muscle spasm, but are poor choices for long-term muscle relaxation and carry high risk of tolerance, dependency, and addiction.
    • Routine use of benzodiazepines is not recommended.
  • Naltrexone has been used off-label for chronic pain with some success (Ericson Jr & Wolman, 2017).

Medication to avoid for those with EDS include:

  • “…acetylsalicylate (aspirin)”, especially for those with Classical EDS (Malfait et al., 2018).

Medication precautions include (Levy, 2018):

  • Total daily dose of acetaminophen should not exceed 4,000 mg; evaluate all potential sources.
  • Abrupt cessation of anti-seizure medications can precipitate a seizure therefore, when discontinuing, they should be tapered gradually.
  • Chronic opioid use can result in tolerance and dependency with escalating dose requirements and diminishing effect
  • Narcotic bowel syndrome can also develop, and may be confused with irritable bowel syndrome.
  • Serotonin syndrome:
    • Can occur when combining multiple serotonergic medications, such as tramadol, tricyclic antidepressants, SNRI antidepressants, anti-seizure medications, and opioids.
    • Symptoms and signs may include agitation, restlessness, tremor, hyperreflexia, ataxia, confusion, irritability, nausea, diarrhoea, hyperthermia, tachycardia, hyper/hypotension, and/or diaphoresis.
    • Severity can range from mild to life threatening.
    • Many of these manifestations overlap those associated with EDS, making diagnosis difficult.
  • o Some individuals find mild-to-moderate serotonergic symptoms acceptable in order to achieve adequate pain control.

11.3 Braces, Splints and Cushions

Braces, splints and cushions, also termed assistive devices – in combination with orthopaedists, rheumatologists, and physical therapists – can be used to promote increased joint stability and decrease likelihood of joint subluxation/dislocation, especially in upper extremity joints and vertebral joints. These assistive devices can be used to provide loading relief to joints during ambulation and weight bearing activities. Points to consider include (Levy, 2018):

  • Orthopaedists, rheumatologists, and physical therapists can assist in recommending appropriate devices for commonly problematic joints such as knees and ankles.
  • Shoulders and hips present more of a challenge for external bracing.
  • Occupational therapists may be consulted for ring splints (to stabilise interphalangeal joints) and wrist or wrist/thumb braces in affected individuals with small joint instability.
  • A soft neck collar, if tolerated, may help with neck pain and headaches.
  • In some cases, a wheelchair or scooter may be necessary to offload stress on lower-extremity joints.
  • Special wheelchair customisations such as lightweight and/or motorised chairs, seat pads, and specialised wheels and wheel grasps may be necessary to accommodate pelvic and upper-extremity issues.
  • A waterbed, adjustable air mattress, or viscoelastic foam mattress (and/or pillow) may provide increased support with improved sleep quality and less pain.
  • Compression wear/garments can also provide support as they can help “…the brain understand the position of the body parts, muscles.” (Chopra, 2017, p.16).
  • Neuromuscular taping or Kinesio™ taping can help to simulate the sensors in the skin as we move, thereby (Chopra, 2017):
    • Improving proprioception;
    • Reducing swelling and pain;
    • Relaxing muscles;
    • Stabilising joints; and
    • Supporting weak joints.
  • Splints can be useful for specific types of joint instability, particularly as part of a co-ordinated treatment programme.
  • Although splints limit joint motion and can limit pain, they may not result in increased stability and, if used consistently, can make muscles weaker through disuse (Ericson Jr & Wolman, 2017).

11.4 Psychological/Psychiatric

“Validation of the affected individual’s symptoms can be immensely helpful, as many with hEDS have been accused of malingering or diagnosed with primary psychiatric disorders by previous physicians.” (Levy, 2018).

Points to consider include (Baeza-Velasco et al., 2011; Branson et al., 2011; Levy, 2018):

  • Establishing trust, rapport, and a supportive relationship between patient and provider is important.
  • Emphasis should be placed on chronic, rather than acute, pain management.
  • Distraction and hypnosis are often helpful.
  • Depression is a common result of the chronic pain, disability, and other complications.
  • Psychological and/or pain-oriented counselling can improve adaptation to and acceptance of these issues and the necessary physical limitations.
  • Cognitive behavioural therapy can be particularly beneficial, but requires active patient participation.
  • Antidepressants are also of great benefit.
  • Many individuals initially resist a diagnosis of or therapy for depression because of concern that their problems are being written off as purely psychiatric.
  • Consumer support groups are available and can be very beneficial.

11.5 Dental Treatment

Points to consider regarding dental treatments include (Levy, 2018):

  • Orthodontic and palatal corrections may tend to relapse, requiring prolonged use of a retainer.
  • Periodontal disease should be identified and treated.
  • Temporomandibular joint laxity and dysfunction are difficult to treat.
  • There are no specific interventions of proven benefit.
  • Intra-oral devices are sometimes helpful.
  • Oral rest (minimisation of chewing and talking), local myofascial release, and muscle relaxant medications may be beneficial for acute flares.
  • Surgical intervention is often disappointing and should be considered only as a last resort.

11.6 Surgical/Orthopaedic Management

Generally, non-surgical options for treatment of joint pain should be exhausted prior to recommending surgery.

“Surgical stabilization of joints may lead to disappointing, or only temporary, improvement.” (Malfait et al., 2018).

EDS can cause early damage to joints and joint instability (Section 4.2). Therefore, individuals will benefit from working with physical therapists and orthopaedic surgeons with EDS experience.

Individuals may have undergone one or several orthopaedic procedures prior to diagnosis, which can include joint debridement, tendon relocations, capsulorraphy, and arthroplasty.

The degree of stabilisation and pain reduction, overall patient satisfaction, and duration of improvement are variable. In general, orthopaedic surgery should be delayed in favour of physical therapy and, for example, bracing.

Unlike classic and vascular EDS, hypermobile EDS is not associated with increased risk for perioperative skin and soft tissue complications.

Other procedures to consider include (Levy, 2018):

  • Prolotherapy:
    • Saline and/or other irritants are injected in tendons or around joints to induce scar formation and increase stability, has not been objectively studied (Levy, 2018).
  • Anaesthetic/corticosteroid injections:
    • For localised areas of pain and acute inflammation are often helpful, but cannot be repeated indefinitely.
    • “Dry needling” without injection of any material sometimes provides similar benefit.
  • Anaesthetic nerve blocks:
    • Can provide temporary relief of neuropathic pain.
    • These are sometimes followed by surgical nerve root destruction and/or implantable stimulators (sensory or motor), with variable results.
  • Constant intrathecal delivery of anaesthetic and/or opioid medication may reduce the need for oral/systemic medications, but should only be considered as a last resort.

For those with vascular EDS treatment may include:

  • Blood pressure maintenance in normal or low range; and
  • Surgical interventions to, for example, replace valves.

11.7 Exercise: The Benefits

Before starting any exercise or training programme it is important to consult your medical professional.

Those with EDS can have decreased tolerance to exercise, in part because exercise frequently causes increased pain. They may, therefore, be less active and, as a result, have decreased strength and aerobic fitness.

“Physical deconditioning and poor aerobic fitness are common findings in patients chronically unwell with hEDS. A history of onset of symptoms of cardiovascular dysfunction following a prolonged period of reduced physical activity is not uncommon.” (Hakim et al., 2017a, p.170).

Although exercise is a very good method of treatment for those with EDS, an otherwise untreatable condition, exercising with EDS is not straight forward, for a variety of reasons:

  • They have to be very careful when performing exercise, especially the exercises/activities they will participate in, in order to avoid injury; and
  • They may experience pain during their workouts.

However, there are a number of benefits to be derived from starting or continuing exercise for individuals with EDS, including:

  • Cardiac and musculoskeletal:
    • Poor response to change in position:
      • Retrain response to postural changes.
      • Perform exercises to improve blood flow.
    • Poor cardiac strength/endurance:
      • Graded challenge to the cardiovascular system to improve strength/endurance.
    • Poor venous return:
      • Use of compression garments.
      • Better function of muscular component.
  • Gastrointestinal and musculoskeletal:
    • Heightened sympathetic and suppressed parasympathetic:
      • Poor sleep habits.
      • High anxiety.
    • Poor food digestion and nutrient absorption:
      • Nausea.
      • Extreme reactions to food.
    • Decreased muscle growth and healing time:
      • Sustained inflammation.
      • Delayed response to exercise routine.
  • Psychosomatic and Musculoskeletal:
    • Build trusting and collaborative relationships.
    • Rescue exercises.
    • Relaxation exercise.
    • Anti-stress activities.
  • Cognitive and musculoskeletal:
    • Improves:
      • Blood flow.
      • Endorphins.
      • Control.
    • Decreases:
      • Brain fog.
      • Confusion.
      • Difficulty with focus.
  • Sleep and musculoskeletal:
    • Relieves anxiety allowing a quieter mind for sleep.
    • Fatigues the system inducing sleep more easily.
    • Can help reduce pain, making sleep more comfortable.
  • Increased physical fitness may counteract orthostatic intolerance (Fu et al., 2011).
  • Research has shown that after a 3-month training programme, moderate gradual endurance and strength training can (George et al., 2016):
    • Decrease upright heart rate;
    • Improve baroreflex sensitivity and heart rate variability; and
    • Improve quality of life.
  • Non-weight-bearing muscular exercise, such as swimming, is useful to promote muscular development and co-ordination (Malfait et al., 2018).

Quality of life can be improved through a well-planned and progressive maintenance programme including:

  • Movement:
    • Encourage the individual to move, no couch potatoes!
    • Pool exercises.
    • Recumbent bike.
    • Walking.
    • Thinks to consider: start slow and easy; monitor heart rate; and train endurance and avoid variable heart rate.
  • Closed chain exercises:
    • These are exercises or movements where the distal aspect of the extremity is fixed to an object that is stationary.
    • In other words, the hand (for arm movement) or foot (for leg movement) is fixed in space and cannot move.
    • Also known as closed kinetic chain (CKC).
    • These include: supine exercises; sidelying exercises; hands and knees; wall exercises; tall kneeling exercises; and balance exercises.
  • Integrative exercises:
    • Think walking, climbing stairs, doing housework, or gardening.
    • Pat your head and rubbing your stomach.
    • Cross body patterns.
    • Combination arm movements.
    • Combination leg movements.
  • Rescue exercises:
    • Chronic pain can often trigger what is known as neurogenic pain, meaning our bodies have become sensitive to things that never used to cause us pain, but now do.
    • In simple terms, pain tolerance has dropped significantly because the neurological system is always active and ready to ‘brace for impact’.
    • Rescue exercises can offset this – being movements and positions one would try out on babies that will not stop crying.
    • For example, try lying on your back, feet on the floor, knees facing up, and extend arms so they are flat on the floor. Roll from side to side slowly, moving from your hips, dropping your knees as you go.
    • Small, slow movements (similar to restorative yoga postures) are useful.

11.8 Exercise: Guidelines for Exercise

“Patients with POTS and comorbid hypermobility spectrum disorders should be instructed in exercise approaches that emphasize joint stabilization and core control. Excessive stretching and hyperextension of joints should be avoided.” (Miranda et al., 2018, p.168).

Some guidelines to keep in mind when exercising (Levy, 2018):

  • DO:
    • Make exercise a priority!
    • Be consistent with your exercise.
    • Exercise regularly, i.e. five days per week.
    • Progress exercises gradually.
    • If possible, train with a partner.
    • Exercise when you are tired.
    • Exercise when you have minimal to moderate pain. You are encouraged to ‘push through’ some pain as strengthening your muscles will reduce chronic pain in the long run.
    • Keep a diary of your training and symptoms.
    • Have a 1:1 session with an (appropriately experienced) exercise professional for a full assessment and a tailored programme before starting exercise.
    • Have an initial assessment of posture and movement. For example, standing on one leg can highlight compensation, as hypermobile clients may have trouble balancing, their hips will move, or they will tuck. Other signs include difficulty holding neutral pelvis and lack of pelvic-floor control.
    • Follow a structured exercise programme.
    • Use foam rollers to massage out ‘knots’.
    • Avoid high impact sports and exercises such as running (especially on hard surfaces and long distances). High-impact activity increases the risk for acute subluxation/dislocation, chronic pain, and osteoarthritis. Some sports, such as tackle football, are therefore contraindicated. However, most sports and activities are acceptable with appropriate precautions.
    • Perform low-impact weight bearing exercise, which is strongly encouraged, to maximise bone density.
    • Perform low-resistance exercise to increase muscle tone (subconscious resting muscle contraction) and improve joint stability, as opposed to muscle strength (voluntary force exerted at will) (Levy, 2018).
    • Place emphasis should be placed on both core and extremity muscle tone. Examples include walking, bicycling, low-impact aerobics, swimming or water exercise, and simple range-of-motion exercise without added resistance.
    • Perform core toning activities, such as balance exercises and repetitive motions focusing on the abdominal, lumbar, and interscapular muscles, as these are also important.
    • Cross train.
    • Use dumbbells over barbells, as greater stability.
    • Use caution with dynamic movements:
      • None with weights, use medicine balls or bodyweight.
      • No push press, overhead (OH) squats, Olympic lifting (oly lifting), kipping movements, or handstands.
    • Avoid distance running.
    • Avoid increasing the resistance as resistance exercise can exacerbate joint instability and pain. In general, it is preferable to increase the number of repetitions of exercise rather than to increase the resistance.
    • Perform simple weight-bearing exercise, such as walking or use of an elliptical trainer. This should not be overlooked as a means to help maintain bone density as well as improve resting muscle tone.
    • Be cautious with chiropractic adjustment. Not strictly contraindicated but must be performed cautiously to avoid iatrogenic subluxations or dislocations.
    • Avoid end ranges (at least initially). For example, maintaining a micro bend in the elbows at the top of a press-up rather than fully extending the elbows.
    • Understand that everything is connected. For example, if you lack core strength, you may find your ability to reach overhead is limited. Lack of core strength may lead to poor posture, which would affect your shoulder mobility.
    • Incorporate isometric exercises (where you hold a particular movement for a specific amount of time). Focus on exercises that isolate/target the individual muscles and activate them without actually moving the joint. This is can be a safe way to work those muscles that need strengthening (i.e. finding a neutral position and gently activating them).
    • Focus on stabilisation and strengthening:
      • Stabilise muscles as they help to stabilise joints, which is important for those with EDS.
      • Primary stabilisers: E.g. rotator cuff, multifidi, and deep hip stabilisers, usually via subtle, isolated, contracted movements. These are deep, core muscles. Neck stability is particularly important for EDS patients, many of whom suffer from POTS and dysautonomia. Shoulder stability is also vital for EDS patients because of how mobile and therefore vulnerable the rotator cuff is.
      • Secondary stabilisers: E.g. pectorals, biceps, gluts, and quads. These are superficial muscles, i.e. one’s we can see, and help us to move.
    • Take supplements to help prevent secondary complications (Levy, 2018). This includes calcium (500-600 mg/2x/day) and vitamin D (400 or more units daily).
  • DO NOT:
    • Take off more than one day at a time from exercise.
    • Perform open chain exercises, i.e. exercises that are performed where the hand or foot is free to move.
    • Progress too quickly.
    • Perform large plyometric exercises.
    • Stretch, or at least avoid static and/or excessive stretching techniques as you may end up stretching ligaments instead of muscles!
    • Push too fast or hard on a ‘good’ day.
    • Participate in sports heavy joint strain such as contact/collision sports, fighting sports, football, or distance running.
    • Train to fatigue. The more fatigued the muscles get, the less they are able to stabilise at a particular joint.
    • Perform Olympic lifts or participate in CrossFit-style training.

When developing a training programme (Section 11.11 above), for any individual, you will/may have to mitigate for the following:

  • The exercise(s) or programme is too complex. Demonstration videos via, for example, YouTube or own website.
  • The exercise(s) or programme is too much. Advice on how to integrate exercises into daily life.
  • The exercise(s) or programme is too boring. Renewing exercises, making changes and/or giving individualised advice.
  • Unmotivated. Spreadsheet via email stating what they have done, for example, can motivate and give accountability to the individual.
  • Uneducated: may need more explanation of the exercises and their objectives.

11.9 Exercise and Orthostatic Intolerance

“Avoid orthostatic stress during exercise until patient build up exercise tolerance, can take MONTHS/YEARS.” (Stiles, 2017, p.16).

Hakim et al. (2017a, p.172) provide some advice for those with orthostatic intolerance and whom wish to perform exercise:

  • Exercise modality:
    • In general, aerobic activities with a local resistive component for the lower limbs are preferred, such as (reclined/recumbent) cycling and swimming.
  • Type of exercise:
    • Dynamic exercise may be better tolerated than isometric exercise.
    • The latter is more prone to provoke valsalva manoeuvres, which decrease blood pressure.
  • Prevention of peripheral blood pooling:
    • “Start slow and low.” (Stiles, 2017, p.16). Exercising in the supine position is better tolerated than the upright position.
    • Exercising in water may also help decrease peripheral blood pooling, because of the greater pressure exerted by the water on the lower limbs.
    • However, an over-heated pool may cause venous dilation and be poorly tolerated.
  • Intensity and frequency:
    • Training at a target heart rate of 75% of the estimated maximal heart rate for about 30 min per session, 2–3 times per week is advised (Fu et al., 2011; George et al., 2016).
    • This should be adapted according to level of disability.
  • Increase of fluid intake, preferably with added sodium, and if helpful, the use of pressure garments during and after exercise.
  • Avoidance of meals 1 hour prior to an exercise session, because vasodilatation in the gastrointestinal tract lowers the capacity of the circulatory system.
  • Prevention of sudden drop in blood pressure after a training session by engaging in low-intensity cooling down activity.

“OI is an important determinant of fatigue in EDS-HT.” (De Wandele et al., 2016).

11.10 Exercise: What to Expect

Some things to keep in mind when you start to exercise, if not already exercising:

  • Being hypermobile means you can probably get into positions that require a fair amount of flexibility, without any pain.
    • However, because you have a lot of flexibility that does not necessarily mean you have a lot of strength or control!
    • You need to be strong throughout the range of flexibility you have, aka range of motion (ROM).
    • Your strength should come from your muscles not your ligaments.
  • There will be a slow progression of impact on symptoms and muscle growth.
  • There may be symptom provocation due to the addition of new/different exercises.
  • Progress should be made by gradually increasing repetitions, frequency, or duration, not resistance.
  • It often takes months or years for significant progress to be recognised or to notice any significant change.

11.11 Exercise: Some Practical Ideas

Individuals should receive an initial assessment (usually referred and guided by a physiotherapist) to determine the most appropriate training programme for them by an appropriately qualified exercise professional who can monitor and adapt the exercises accordingly.

It is not the purpose of this article to provide an exhaustive list of exercises suitable for everybody, as each will have their own needs and physical issues. With that in mind, there are some generic principles applicable to everyone that can be followed when it comes to exercising.

Prior to performing any exercise one must prepare both psychologically and physically, and this is via a warm-up. Due to the issues associated with EDS, for example hypermobility, one must be careful when warming-up. Some warm-up ideas include:

  • Bridging > activate posterior chain.
  • Banded rowing: horizontal pulling before vertical to activate scapular stabilisers.
  • Slo-mo air squats: hold at the bottom, show control.
  • Bird dogs: coordinate core control with dynamic movement.
  • Mobility is dependent on individual deficits, for example:
    • Lateral lunge for adductors.
    • Front lunge with palms on ground for hip flexors.

In the same manner as those without EDS, those with EDS should follow a graduated, structured training programme which should include:

  • Resistance training to avoid recurrent joint subluxations/dislocations due to increased muscle tone and to counteract presence of excessive joint, ligament, tendon, and muscle laxity (low resistance, high repetition activities).
  • Proprioception training such as CKC, balance, and (possibly later on) plyometric training.
  • Aerobic training to promote increased static and dynamic muscle tone to prevent acute joint subluxations/dislocations by minor trauma or stimulus. Examples include walking, cycling, low-impact aerobics, water aerobics, and swimming.
  • Core strength and stability training.

The following is a general example of such a programme:

  1. Stage 1, Initial Training:
    a. Initial-stage training may include posture, lumbar control, PNF, taping, mirror cueing, and be fairly simple!
    b. Use of low-impact exercises until increase in strength/stability.
    c. Importance of posture and optimal positioning.
    d. Exercises to improve stability should incorporate the neck, shoulders, and hips.
    e. Incorporate core stabilisation exercises during early rehabilitation and progress throughout.
    f. Activity modification.
    g. Avoidance of end ranges.
    h. Strength/resistance training does not just mean dumbbells!
    i. Decrease fear avoidance.
    j. Teach the hip hinge.
  2. Stage 2, Mid-Range General Strengthening:
    a. May include CKC circuit, pain-free range, gradual increasing of repetitions, and endurance.
    b. Goal is to increase training volume without increasing stress to the joint.
    c. Encourage rowing and cycling for aerobic exercise.
    d. Look at ability to complete 10-15 reps pain free.
    e. Use the 30 seconds chair rise test once it is felt the individual can properly perform a hip hinge with limited cueing.
    f. Refer to Table 6 below for example exercises.
    g. Progress exercises to next stage if there is no increase in pain with these exercises AND improved quality.
  3. Stage 3, Stability & End Range (Testing):
    a. Core stability.
    b. Bottoms up kettlebell rhythmic stabilisation for time (kettlebell is commonly abbreviate to KB).
    c. Single leg squat progress.
    i. Take subjective report of pain into account.
    ii. Focus on quality.
    d. Refer to Tables 7a and 7b below for example exercises.
    e. Progress exercises to next stage if there is no increase in pain with these exercises AND shows adequate control.
  4. Stage 4, Isolated Strength, Endurance, and Power:
    a. Check progress towards goals.
    b. Refer to Tables 8a and 8b below for example exercises.
    c. This late-stage training may include sport-specific, running, low-dynamic plyometrics, and racquet activities.

Individuals will want and desire differentiation in their training programme and Table 9 provides a range of exercises to facilitate this.

For those with EDS who are determined to run, they should consider the following:

  • Return to running should be phased. A walk to run programme should initially be more walking than running transitioning to more running than walking, with emphasis on time rather than distance.
  • Running assessment (aimed at exercise professionals/physiotherapists). Consider:
    • Foot contact;
    • Knee flexion angle at IC;
    • Vertical displacement;
    • Stride length;
    • Cadence; and
    • Torso angle.

11.12 Isometric Exercises

An isometric exercise is one where you hold a particular movement for a specific amount of time. For example, holding halfway down in a press-up position for 10-15 seconds, 1-3 times, instead of performing a set of 10 regular press-ups. Doing this will make a big difference in how your body handles being hypermobile through developing strength and stability where it needs it most – near the joints.

Examples of isometric training on YouTube:

  • 23 Isometric Core Exercises: https://www.youtube.com/watch?v=R6n7tHiSlzY.
  • Isometric Exercises for Legs. Vs. Lifting Weights: https://www.youtube.com/watch?v=snD1UaR56ys.
  • Shoulder Isometric Exercises: https://www.youtube.com/watch?v=qQjjc3UlKCY.

However, note that for those with orthostatic intolerance (Section 11.9), Hakim et al. (2017a, p.172) suggest that “dynamic exercise may be better tolerated than isometric exercise. The latter is more prone to provoke valsalva manoeuvres, which decrease blood pressure.”

11.13 Exercise: Other Methods

The following is a list (not exhaustive) of other methods to aid the health and fitness of those with EDS.

  • Pilates:
    • This a non-impact exercise which is great for joints and controlled movements (Di Bon, 2017).
    • Described as “…an excellent supplement for any treatment from your medical practitioner.” (Di Bon, 20170, p.6).
    • Di Bon (2017) developed the Integral Movement Method which is Pilates-based but with a rehabilitation and pain management focus. Includes six elements: breathing; relaxation; proprioception; joint stability; balance; and posture.
  • Yoga:
    • EDS and yoga have a complicated relationship, and there are many articles devoted specifically to the subject.
    • Yoga is possible with EDS, and can be deeply beneficial, but do your research and find a yoga teacher with appropriate skills and experience.
  • Alexander Technique (AT):
    • “The Alexander Technique is a skill for self-development teaching you to change long-standing habits that cause unnecessary tension in everything you do.” (STAT, 2019).
    • Research suggests the Alexander Technique may aid dynamic modulation of postural tone (Cacciatore et al., 2006).
  • Aqua Training (or Water Training):
    • Some consider this the “Best form of exercise in EDS.” (Chopra, 2017, p.23).
    • Swimming, water-resistance training, and aqua therapy can be used as an alternative to, for example, running since they can give a full-body workout without stressing your joints.
    • Examples of muscle and core stabilisation exercises that can be performed include (but not limited to) (Hydro Worx, 2019): knee extensions; flutter kicks; shoulder flexion and extensions; horizontal abduction and adduction; windmills; punches; triceps press; push pulls with a kickboard; reverse abdominal curls; prone hip abduction and adduction; prone flutter kick; high knee walking with fins/flipper; walking; and jogging.
  • Elliptical Trainers:
    • Elliptical running is another great way to get the benefits of a cardiovascular without potentially damaging your joints.
    • Incline treadmills, stair-steppers, and stationary (typically recumbent) bikes are also good ways to get low-impact exercise.
  • Free Weights:
    • Working out with free weights strengthens stabiliser muscles in a way that machines, which often only have your muscles move on one axis, do not.
    • The downside of free weights is that their lack of rules gives you more room to make mistakes.
    • It is best to start light and to work with someone who understands your condition to do this safely.
  • Tai Chi:
    • For core strength, flexibility, and balance (Lavallee, 2015).
    • It is comprised of slow, controlled movements.
    • Chi Gung is also advocated.
  • Ballroom dance:
    • For core strength, flexibility, and balance (Lavallee, 2015).
  • Feldenkrais Method:
    • This is an educational system that uses movement to teach self-awareness and improve function.
    • It is based on principles of physics, biomechanics and an understanding of learning and human development.
    • “This method of exercise is excellent for improving proprioception in Ehlers Danlos Syndrome.” (Chopra, 2017, p.155).
    • The Feldenkrais Method was developed by Dr Moshe Feldenkrais (1904-1984).

12.0 What is the Prognosis or Outlook?

The prognosis for EDS depends on the:

  • Sub-type; and
  • Individual.

Life expectancy:

  • Can be shortened for those with the vascular EDS due to the possibility of organ and vessel rupture.
    • Median age 49 for males and 53 for females, but can range from 10 to 80 years of age.
    • Arterial rupture is the common cause of sudden death.
  • Is usually not affected in the other sub-types.

There can be a wide or narrow range of severity within a family, but each family member’s case of EDS will be unique.

While there is no cure for EDS, there is treatment for symptoms, and there are preventative measures that are helpful for most people.

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