Management of EDS and HSD

Chronic pain in the Ehlers–Danlos syndromes, especially in hypermobile EDS, is very common and may be severe. It may be widespread or it may be limited to one area of the body such as a limb. Headaches and gastrointestinal discomfort can occur as well as joint, muscle and nerve pain. Management strategies include physiotherapy, medication, and sensible use of medical equipment such as splints and braces.

We have a whole page in the Information Hub on managing pain Managing pain

One of the first things many people with chronic illnesses hear is, “You need to learn how to pace yourself.”

Pacing is about managing your energy in a way that helps you do the things that matter to you without making your symptoms worse.

It’s not always easy, but learning to pace can make a huge difference.

Pacing is about finding a balance between exercise and day-to-day activities (including but not limited to education, socialising, self care tasks, showering and even brushing your teeth) to try and minimise the amount/extent of a flare which you may have, pacing is also a part of knowing what to do if a flare up happens.

It is a skill that can also be used to gradually increase the amount you can do of certain things.

Pacing simply put is about taking things at your own pace and learning what this is, finding patterns and learning how and what other parts of your pain/self-management toolkit to implement at certain points

On a good day, we feel better and try to catch up on everything we’ve missed.

Then the next day – or sometimes even later that same day – we crash.

Symptoms flare up, fatigue increases, and we may need days to recover.

Pacing helps break that cycle by encouraging us to spread our energy more evenly over time.

The Spoon Theory

One of the most popular ways of explaining energy levels in chronic illness is called Spoon Theory.

The theory was created by Christine Miserandino to help explain what living with a chronic illness feels like.

Imagine you start each day with a certain number of spoons.

Each spoon represents a unit of energy.

For someone without a chronic illness, they might have lots of spoons and not need to think much about how they use them.

For someone with a chronic illness, the number of spoons may be much smaller—and they may use more spoons doing everyday activities.

For example:

• Getting dressed might cost one spoon.
• Going to work might cost three spoons.
• Walking around town with friends might cost two spoons.
• Doing housework might cost another spoon.
• Attending a medical appointment could cost several more.

The challenge is that once your spoons are gone, they’re gone.

Pacing sounds simple, but in reality it can be really hard.

Nobody wants to say no to seeing friends.

Nobody wants to stop halfway through an activity they’re enjoying.

And when you’re feeling well, it can be tempting to pretend you’re not ill at all.

Sometimes we worry that people won’t understand.

Sometimes we feel guilty for needing breaks.

Sometimes we’re simply tired of having to think about our health all the time.

All of these feelings are completely normal.

Tips for Better Pacing

Learn Your Warning Signs

Your body often gives clues before symptoms become overwhelming.

You might notice:

• Increased pain
• Brain fog
• Feeling shaky or dizzy
• Difficulty concentrating
• Increased fatigue

Recognising these signs early can help you take a break before you completely run out of energy.

Asking questions such as:

• What does my body need right now?
• I’m going to check in with my body now, what is it asking me for?
• Am I ok to continue?
• Do I need to adapt what I’m doing to be able to continue or should I stop this activity and return later or stop it completely for today?

Asking  these types of questions are all a really good way of building an understanding of your body and pacing skills. They help you to listen to your body and identify its needs.

Some find an activity diary helpful to notice and track trends in their pain/energy and to help work out what may be leading to flares and to work out how to better pace themselves.

It can be helpful to use a colour scheme many opt for a traffic light system such as red pencil, orange pencil and green pencil shading in the boxes with the relevant colours based on how the activity affects your pain &/or energy levels.

Take Breaks Before You Need Them

One of the biggest pacing lessons is not waiting until you’re exhausted.

Short, regular breaks can often help prevent bigger crashes later.

Think of it like charging your phone before it reaches 1%.

Prioritise What Matters Most

You don’t have to do everything.

Some days you may need to choose between activities.

Ask yourself:

“What is most important to me today?”

Using your energy on things that matter most can help you feel more in control.

Plan Ahead

If you know you have a busy event coming up, such as a party, trip, or family gathering, consider saving some energy beforehand and allowing extra recovery time afterwards.

Many people call this “energy budgeting.”

Remember That Rest Is Productive

Rest isn’t being lazy.

Rest is a tool.

Taking time to recover can help you participate in more activities over the long term.

Finding Your Own Pace

The most important thing to remember is that pacing looks different for everyone.

What works for one person might not work for another.

Some days you’ll get it right. Some days you’ll use too many spoons and pay for it later. That’s part of learning.

Living with a chronic illness often means becoming an expert in listening to your body. Pacing is one of the ways we can work with our bodies instead of constantly fighting against them.

It may not always feel fair that we have fewer spoons to start with, but learning how to use them wisely can help us spend more time doing the things we love and less time recovering from doing too much.

Chronic widespread and regional pain

Chronic pain is a very common symptom in the general population. The terms ‘acute’ and ‘chronic’ are used to describe the time that pain has been present, as opposed to the more common description of severity. ‘Acute’ means sudden and short-lived, whereas ‘chronic’ is persistent and lasting for more than three months. Both acute and chronic pain can have any degree of severity ranging from mild to excruciatingly disabling.

The distribution of pain is defined as ‘widespread’ if it occurs at sites on both sides of the body AND both above and below the waist AND in the spinal region. The term ‘regional’ defines pain confined to specific areas, e.g. one arm, or one arm and one leg on the same side of the body.

Together the above terms describe ‘chronic widespread pain’ (CWP) and ‘chronic regional pain’ (CRP). From large surveys, we know that CWP is found in approximately 11% and CRP 25% of the population. It is thought that most CWP arises from soft tissues, such as muscles and tendons, that otherwise appear to be normal. Far from normal, however, is the experience of poor health and reduced quality of life among sufferers of chronic pain.

When a particular pattern to the distribution and nature of the pain is recognised the diagnosis of fibromyalgia is made. Fibromyalgia (FM) is probably the most common diagnosis given to people with CWP. However Ehlers-Danlos syndrome (EDS) (particularly hypermobile Ehlers-Danlos syndrome (hEDS) and the related hypermobility spectrum disorders (HSD)) can also present with a similar picture. Equally, hEDS/HSD can present with recurring acute or persistent chronic injury to a few hypermobile joints giving rise to CRP.

Both FM and hEDS/HSD are common conditions. It is not surprising therefore that they may occur together by chance in an individual. However, several small studies and commentaries also suggest that they may be associated with each other by potentially sharing similar mechanisms that lead to pain. Whether by chance or whether associated, it is important that your doctor does not ignore hEDS/HSD just because FM is present. Aside from the fact that hEDS/HSD has other problems not seen in FM that could be missed, the physical therapies used to help FM might cause harm if not adapted for the hypermobile individual.

Diagnosing fibromyalgia

The definition of FM was formed in the late 1980s by the American College of Rheumatology. Prior to this the symptoms were recognized as ‘fibrositis’. The term ‘fibromyalgia’ is more descriptive of the fibrous tissue (fibro) and muscle (myo) pain (algia). FM is usually explained as a condition in which the muscles become tense, tender and tight due to lack of relaxation. Inflammation (itis) is not a feature.

The 1990 American College of Rheumatology criteria for making a diagnosis of FM are:

• CWP – chronic and widespread pain as described above
• At least 11 tender points out of 18 sites as defined on a body map

The sites of tender points are shown in the figure below:

However, recently it was proposed that FM might be diagnosed using a symptom checklist without undertaking an examination for the tender points. This proposal has been controversial, not least because the symptoms can be found in other conditions such as hEDS/HSD, and it should always be the case that someone with pain and fatigue should be examined carefully and not assumed to have FM.

The cause of FM is not known. There are no abnormal blood tests, scans, biopsy tests or other investigations to support the diagnosis, although muscle pain may be associated with a low vitamin D level, which should be corrected. Like hEDS/HSD, FM is associated with headache, fatigue, poor sleep pattern, restlessness, bowel disturbance (e.g. irritable bowel syndrome) and autonomic disturbances of heart rhythm and blood pressure.

Treating fibromyalgia

There is no cure for FM. Given the various symptoms aside from pain, it is likely also that no one treatment is sufficient. Treatment is aimed at reducing symptoms as much as possible. A number of treatments have been described with varied success; not all treatments help in all cases and often people present with very different circumstances and symptoms requiring an individual approach, not unlike the principles applied to hEDS/HSD. In 2007 a team of experts looked at the research evidence for the effectiveness of different treatments. Their conclusions were published in a guideline called the ‘EULAR Evidence-Based Recommendations for the Management of Fibromyalgia Syndrome’.

Exercise

Aerobic activities such as walking, cycling and swimming are thought to be the best form of exercise to improve symptoms. Gradual build-up over a period of several months, leading to exercising 4 to 5 times a week for at least 20 to 30 minutes at a time is recommended.

Physiotherapists can support you with exercise advice, adjustments and assessment of effectiveness. This should include advice on lifestyle, relaxation and ‘pacing’. When you are hypermobile your therapy assessment may need to include:

• Core stabilising exercises
• Joint stabilising exercises, including reducing risk of dislocations
• A programme to improve proprioception (joint position sense)
• Encouragement of full hypermobile range of movement whilst avoiding undue stress on joints and long periods in potentially damaging positions

Exercising for 20-30 minutes in any form of heated pool (hydrotherapy) – whether it be a specific therapy unit or local heated swimming pool – may help some people.

Cognitive behavioural therapy (CBT)

CBT is one type of psychotherapy (‘talking treatment’) undertaken by clinical psychologists and can be of value in managing FM. CBT tends to deal with the way current thoughts and behaviours are affecting a person. It is a problem-focused and practical therapy aimed at taking control of and easing pain, fatigue, mood and anxieties that interfere with day-to-day living. It is a valuable addition to exercise, other clinical psychology, physiotherapy, and drug treatments.

Complementary therapies

Some people find therapies such as massage, aromatherapy and acupuncture effective. There is little research evidence upon which to base advice for the use of these treatments and they might work in part by helping relaxation.

Medication

It should be noted that whilst clinical trials of the following painkillers have been undertaken in FM, they have not been undertaken in hEDS/HSD. The most commonly-used painkillers include antidepressants (for both pain control (e.g. milnacipram and low-dose amitriptyline) and low mood (e.g. duloxetine, fluoxetine, sertraline and venlafaxine)) and anticonvulsant drugs (e.g. pregabalin and gabapentin). Tramadol in combination with paracetamol appears to have similar benefit to the antidepressants in treating FM. It is unwise to take strong opiate drugs such as morphine long-term because of the risk of dependency and side-effects. Also, opioids often do not work other than in relieving episodes of acute pain.

For very short periods of time, for example a week, a doctor may recommend a muscle relaxant such as a benzodiazepine (e.g. temazepam or diazepam) if spasm is severe. These may also reduce anxiety. Their use as sedatives to help correct poor sleep pattern may work for a short period but the long-term effect on FM overall is very disappointing. Therefore sedatives are not recommended, furthermore there is the same concern of dependency as with opiate painkillers.

Fatigue

Fatigue is a common and disabling finding in many musculoskeletal conditions including FM, hEDS and HSD. It is important to exclude other causes of fatigue such as anaemia, endocrine abnormalities like an underactive thyroid (hypothyroidism), chronic infections and deteriorating heart, lung, liver or kidney function. In the vast majority of cases of FM and hEDS/HSD however, fatigue is simply a feature of the pain syndrome.

It is more than the typical tiredness one associates with exercise or a busy day. It is often an overwhelming lack of energy after even minimal activity. In children and adolescents poor concentration may lead to problems with schooling, especially if the issue goes unrecognized or unsupported. The inability to control fatigue may come across to others as lazy, antisocial, or perhaps even depressed.

Many individuals report intolerance of the cold, dizziness, a fear of blackouts, poor concentration and note that they cannot stand for too long without aggravating their fatigue. This may be due to autonomic disturbances of the heart and blood pressure (see EDS UK’s article ‘Autonomic Dysfunction’).

Managing fatigue

There is very little evidence to support the use of the various forms of therapy available for fatigue. In FM and hEDS/HSD, analgesics and antidepressants used to control pain may help relieve fatigue indirectly. In the same way, control of autonomic disturbances of the heart and blood pressure may help. Lifestyle changes including pacing, changing sleep pattern and exercise may be helpful too, as may behavioural therapy.

While there is limited evidence supporting the use of nutritional supplements, the commonly recommended supplements in the management of chronic fatigue syndrome (and therefore potentially of fatigue in FM and hEDS/HSD) include carnitine, co-enzyme Q10 and 5-HTP. Such agents are considered to be effective in boosting the immune system, raising energy levels, and improving cognitive functioning.

With all these supplements, it is important to remember the potential for interactions with medication which may not be known, that there is often limited or no data on use in pregnancy and lactation, and that there are no specific dosage recommendations for FM or hEDS/HSD.

Dr Alan Hakim and Professor Rodney Grahame, Consultant Rheumatologists, The Hypermobility Unit, Hospital of St John and St Elizabeth, London

In 2021 the British Medical Journal published the first ever case study of medical cannabis as a novel treatment for chronic pain. The patient whose story broke new ground in the move towards wider acceptance of cannabis as a medicine, lives with Ehlers-Danlos syndrome (EDS). Lucy Stafford, 21, who was diagnosed with hypermobile EDS aged 17, after years of living with unexplained symptoms, has seen her health transformed with medical cannabis. Previously, Lucy was prescribed numerous treatments, including strong opioid medication and underwent surgery for a dislocated jaw. At the age of 15, her condition was so bad she was forced to leave school and a few years later, she needed the use of a wheelchair and a feeding tube.

Lucy began using cannabis medicinally at the age of 18. According to the BMJ report, within days of self-medicating her pain levels “considerably subsided” and within three months she was able to come off all her opioid medications. But more than just managing her pain levels, Lucy found cannabis helped with a range of symptoms brought on by the condition, from gastrointestinal issues to the anxiety and depression that comes hand in hand with living with a chronic illness.

“This is the first BMJ case report that attempts to quantify the multifaceted benefits given by cannabinoid medication to a multisystem condition,” says author of the report, Sabeera Dar, of University College London Medical School. “The use of medical cannabis rapidly alleviated long standing chronic pain and dramatically reduced side effects related to opioid medication as these became less necessary to take.”

Lucy is now one of over 10,000 patients who has a private prescription for medical cannabis, which was legalised for medicinal use in the UK in November 2018. She is studying biochemistry at university, and when she’s not studying is out roller skating, or campaigning for NHS access in her role as advocacy director at PLEA (Patient-Led Engagement for Access).

The BMJ paper is the first to quantify the multifaceted benefits given by cannabinoid medication to a multisystem condition. But Lucy is not alone. Many other EDS patients have seen considerable improvements in their symptoms with cannabis. A survey of 500 patients with EDS in the United States found that 37 percent used cannabis therapeutically. In the UK, after chronic pain and anxiety, EDS is one of the commonly prescribed for conditions, according to data from Drug Science’s Project Twenty21, an observational study which subsidises the cost of prescriptions in order to build the UK’s largest database for the safety and efficacy of medical cannabis.

While more research is needed to help us understand exactly why EDS patients are reporting success with cannabis, Dr Dani Gordon, a Canadian British doctor now practising in the UK and a leading expert in prescribing cannabinoids, believes that the mechanism by which cannabis medicines work in the body makes them effective in a way that conventional treatments are often not.

“In general, I find medical cannabis is one of the best quality of life medications for EDS because it affects multiple systems and symptoms across multiple domains,” says Dr Gordon “EDS is not just about being hypermobile, like many of us are taught in medical school, patients also suffer from a number of symptoms that can be difficult to treat and are often misdiagnosed. When you have this correlation of chronic pain, fatigue and poor sleep, which is often what EDS patients experience, medical cannabis can help with all these symptoms. Although it’s not a cure, in my experience, it often works better, with less side effects than the medications they are currently taking.”

This is partly due to how cannabinoids interact with the endocannabinoid system – a system in the human body responsible for restoring balance and regulating functions such as temperature and fluid levels. Dr Gordon continues: “The endocannabinoid system is a homeostatic system that works to bring balance to the brain and the body in many different ways. Just like many other chronic conditions with symptoms that cross multiple systems in the body, EDS is one of those diseases where medical cannabis is particularly beneficial, because there are cannabinoid receptors in most of our tissues throughout the brain and body, and the endocannabinoid system has so many diverse functions. Because of this we tend to see relief from symptoms that don’t necessarily appear to be connected.”

But there may be a more direct reason cannabis helps. Alongside the role of the endocannabinoid system, early evidence suggests it may play a part in reducing mitochondrial dysfunction thought to be associated with EDS.

“There’s an early theory that conditions like EDS may have a component of mitochondrial dysfunction, which contributes to symptoms such as fatigue and poor sleep, as the mitochondria are the powerhouses that make the cellular energy in our brain and our body,” adds Dr Gordon. “Early evidence suggests that cannabinoids help support the mitochondria and may even help reverse or at least, ameliorate mitochondrial dysfunction by multiple mechanisms. That may be another reason why patients respond so well to medicinal cannabis, almost universally, when you find the right mix of cannabinoid therapy for them.”

Dr Gordon cautioned that as EDS patients can be particularly sensitive to medication, it’s important to “start low and go slow” when introducing cannabinoids such as CBD and THC, using what she calls an “ultra slow titration schedule”.

Sarah Sinclair is managing editor of Cannabis Health, the UK’s leading magazine covering the use of cannabis for health and wellbeing purposes. Find out more at http://www.cannabishealthnews.co.uk and contact PLEA via www.pleacommunity.org.uk

Further research

Since this article was first published, further peer-reviewed research has explored the use of cannabis-based medicinal products in people with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorder (HSD). The links below are provided as suggested further reading and are not intended to represent a comprehensive review of the evidence.

• Dar S. (2021). Treating pain related to Ehlers-Danlos syndrome with medical cannabis. BMJ Case Reports.
  The original case report referred to in this article, describing the use of cannabinoid-based treatment for chronic pain in a person with hypermobile EDS.
  Read the BMJ case report
• Dickinson M, et al. (2025). UK Medical Cannabis Registry: An Analysis of Outcomes of Medical Cannabis Therapy for Hypermobility-Associated Chronic Pain. ACR Open Rheumatology.
  A UK case series of 161 people with hEDS or HSD and chronic pain, reporting improvements in patient-reported measures of pain, sleep, anxiety and quality of life following treatment with cannabis-based medicinal products. The authors highlight the need for randomised controlled trials.
  Read the 2025 study
• Stafford LV, Lynskey MT, Schlag AK, Nutt DJ, Eccles J. (2025). Medicinal cannabis in the treatment of hypermobile Ehlers-Danlos syndrome & hypermobility spectrum disorder: Real-world evidence from Project Twenty21.
  Research using data from Project Twenty21 examining real-world outcomes of medicinal cannabis treatment in people with hEDS and HSD.
  Read the Project Twenty21 study
• Alemi M, et al. (2026). UK Medical Cannabis Registry: an updated analysis of clinical outcomes of medicinal cannabis therapy for hypermobility-associated chronic pain. Clinical Rheumatology.
  An updated UK registry study involving 240 people and follow-up of up to 24 months. Cannabis-based medicinal products were associated with sustained improvements in pain, sleep, anxiety and quality-of-life measures, although the observational design means the study cannot establish that treatment caused these improvements.
  Read the 2026 study

In common with people who have other long-term conditions, many people with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorder (HSD) experience chronic fatigue. The reasons for this are far from clear. While there is no easy fix for chronic fatigue there are various coping mechanisms which can help. These are similar to coping mechanisms for ME/chronic fatigue syndrome, although chronic fatigue with hEDS/HSD and chronic illness is distinct from ME/chronic fatigue syndrome.

Stay hydrated

Staying hydrated is vitally important when trying to fight fatigue as it helps to ensure a good flow of blood to your brain. This is especially important if you have a sister condition such as postural tachycardia syndrome (PoTS) or orthostatic intolerance, as these conditions may also reduce the blood flow to your brain. As well as water, electrolyte-rich drinks such as coconut water or sugar-free sports drinks can help you to stay hydrated. Urine should be pale yellow.

Have a healthy diet

Many deficiencies can lead to fatigue, such as anaemia (iron/haemoglobin deficiency), vitamin B12 deficiency and vitamin D deficiency. Low vitamin D can be more of a problem during winter, as you have less exposure to sunlight which helps your body to produce vitamin D. Having a healthy and balanced diet can improve energy levels and prevent deficiencies. Taking a multi-vitamin may also help. If you have gastrointestinal issues causing malabsorption or you take medication which inhibits the absorption of nutrients (e.g. proton pump inhibitors, gabapentin, pregalabin) you could request that your GP tests your nutrient levels. If you have a severe deficiency your doctor may prescribe a higher-dose vitamin. Some people find that the supplements carnitine, co-enzyme Q10 and 5-HTP also help them in fighting fatigue, although the evidence is anecdotal. You should always discuss taking supplements with your doctor, especially if you are on other medication.

Avoid caffeine and sugar

Whilst caffeine and sugar might make you feel less tired in the short term, they can make fatigue worse in the long run. Reducing or stopping your sugar and caffeine intake can reduce energy fluctuations and make you feel more stable and less fatigued.

Exercise

Although you probably won’t feel like it, exercise can really help when you’re suffering from fatigue as it triggers your body to produce adrenaline and other hormones that can make you feel more energised. Starting gently and slowly, gradually build up the amount of exercise you can do because it is important to avoid overexertion at first. Swimming, walking and specialist physiotherapy are all good forms of exercise for someone with hEDS/HSD.

Pace yourself 

Make sure you don’t do too much and get enough rest between activities, otherwise you might pay for it the next day. A good explanation of ‘pacing’ is the spoon theory which can be found here:

http://www.butyoudontlooksick.com/wpress/articles/written-by-christine/the-spoon-theory/

Good sleep hygiene

Many people with hEDS/HSD find that they have a hard time getting a good night’s sleep. There are several things you can try yourself to improve things. Good sleep hygiene is really important. This means getting into a good night-time routine. Go to bed at the same time every night (shift work can cause further problems), and don’t use a computer or phone for several hours before bed (the bright light from screens inhibits the release of a hormone called melatonin which helps you sleep; the light from a TV shouldn’t affect it so much as you are sitting further away but it may help to cut out TV for several hours before bed as well). Keeping your phone/computer away from the bed can also help you to avoid the distraction (“log off before you nod off”) and make sure there are no other distractions in the room. Having a warm bath or a warm (non-caffeinated) drink, reading or doing something to relax in the few hours before bed can really help as well. It is also a good idea to cut out caffeine for at least 4-6 hours before sleep, as well as any other stimulants.

Pain is one of the factors that can cause sleep problems (‘painsomnia’). Ensuring that pain is kept under control is important. Using heat pads on sore joints at bed time can help. If you believe pain is one of the main things preventing you from getting a good night’s sleep, it might be worth talking to your doctor about pain management. It may be possible to get a prolonged release pain medication that works throughout the whole night. Sleeping tablets may also be prescribed, but these are generally not a good long-term solution as the body can become resistant to their effects.  Getting too much sleep can also worsen fatigue. Try to sleep for the same amount of time each night as this can lessen tiredness and help you to get into a good routine. Most need around eight hours sleep but people with hEDS/HSD or chronic fatigue may find that they need more than this.

If you find that none of these things help with sleeping, it may be worth getting a referral to a sleep clinic where they will be able to assess you and attempt to find the cause of your sleep problems. There are several other conditions which influence sleep and which may be more common in hEDS/HSD. These may include sleep apnoea and restless legs syndrome.

Therapy

Certain types of therapy may be effective in treating chronic fatigue, especially Cognitive Behavioural Therapy (CBT). This does not mean that the fatigue is all in your head, but CBT can help you to come to terms with the problem. It can also be helpful in learning to live with hEDS/HSD and chronic pain, and many pain management clinics will offer CBT as part of your treatment.

Brain fog

Many people with hEDS/HSD also suffer with brain fog. They may find themselves feeling confused, being unable to process information, or forgetting things for no apparent reason. Whilst the reason that hEDS/HSD causes this is not confirmed, it is thought to have similar causes as fatigue. Managing fatigue should therefore also help with episodes of brain fog.

In hEDS/HSD, it is thought that brain fog may be related to lack of blood flow to the brain due to blood pooling in the legs because of stretchy veins. There is further research needed to confirm this suspicion. Brain fog also appears to be more common in those with PoTS secondary to their hEDS/HSD, suggesting that there may be a link there.

There are several other things that may cause or worsen brain fog, and making some small changes in your life may help to improve things.

Hormones can influence fatigue and brain fog. Many women find that brain fog worsens at certain times during the menstrual cycle and/or during pregnancy. Some women also find that symptoms worsen when they are on certain birth control or hormonal treatments.

Vitamin and mineral deficiencies can cause or worsen fatigue and brain fog. Making sure you eat a healthy balanced diet is important and, if your symptoms are severe, your doctor may wish to arrange a blood test to find out if you have any specific deficiencies. These include anaemia (iron deficiency), B vitamins and vitamin D deficiencies, and potassium deficiency. Taking supplements if you have a deficiency can help with the symptoms, although it is important to check with your doctor before you take any medications or supplements, especially if you are taking prescribed medication.

Some medication can cause fatigue and brain fog. These include some antihistamines, cough medications, painkillers and antidepressants. If you are worried that these may be causing you problems you should discuss the situation with your doctor. It may be possible for your doctor to change the medication to one which does not cause those side effects.

Other conditions can also contribute to fatigue and brain fog. These include diabetes, heart disease, hypothyroidism, alcohol and/or substance abuse, emphysema, and obesity. Managing these conditions effectively can help with symptoms.

Please note: The following text cannot and should not replace advice from the patient’s healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.

The practicalities of physiotherapy

It is widely accepted among the medical community that physiotherapy forms one of the mainstays of managing the conditions hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD). You may possibly have been newly diagnosed and referred to physiotherapy by your medical consultant, unsure of what to expect. Others of you may have received physiotherapy in the past but without particular focus on your condition. In either event, this article aims to explain what to expect from physiotherapy and how it can help with you.

 

Subjective assessment

Whether you are referred to physiotherapy via the NHS or privately, your initial appointment usually starts with what is known as a subjective assessment. This is basically a conversation, or an information-gathering exercise if you prefer, during which your physiotherapist (physio) aims to collect as much information as possible about you and your problems, and then uses this information to determine what physical and functional difficulties you have. This should also guide them as to what to choose to look at when they go on to physically assess you.

The subjective assessment often goes a little like this: “I have a referral from Dr so-and-so stating that you have a new diagnosis of hypermobile EDS/hypermobile spectrum disorder. So tell me a bit about yourself. What are your main problems?” At the same time the physio is usually also clutching an empty body chart in their hand – a picture of a generic blank human body staring at you from the front and back. They will then often ask you to helpfully “Please shade in on the body chart the bits of you that correspond with where you feel your pain or have your issues”.

Now at this point I’ll let you into a little secret. When we train as physios we practise filling in loads of these body charts in class and get handed hundreds of little example ones, each usually covering a different problem. Sometimes it might be a painful knee that’s shaded in, other times a dodgy hip. Commonly it’s a shaded-in back with a jaggedy line running down one of the legs. Physios know where they stand with body charts like this.

And then suddenly, there’s you lot. You take hold of that nice empty body chart and set to work. Shading in here, scribbling in there, colouring in areas and dotting other bits. Commonly, by the time you hand it back to the physio the whole body is covered in dots, splats, areas of shading, pain, points of dislocations/subluxations, aches, headaches and gastric squiggles. Trust me, this can be scary for your regular physio who may well be unfamiliar with what unfortunately still remains a lesser-recognised condition. That’s no good for you, staring non-confidently across at the colour draining from your physio’s face. So what do we do at this point? What should happen?

First of all, stay calm. We’ve established that you may well have a ton of issues going on: pain, subluxations, postural tachycardia syndrome (PoTS), gastric issues and goodness knows what else. Well, it’s not going to get solved all in one go. We’re probably not even going to understand it all properly in one go. So let’s not try to. We’ll get through it if we take our time and do it methodically.

Let’s go back to the subjective history. As mentioned, this is an information-gathering process and the more effectively your physio undertakes it, the better they should be able to help you further down the line. In my opinion, there is a series of key areas that your physio needs to know about:

• Pain: where you have it, nature and severity.
• Dislocations/subluxations (clicking, popping, etc): where, how often, how do you get it back in place?
• Fatigue, dizziness, digestive symptoms: how often and for how long? Do you faint? Do you have any allergies?
• Current order of most problematic areas: really helps to prioritise what to deal with first.
• Previous interventions: what’s worked well before, what hasn’t?
• Past medical history: need to know about other medical stuff going on.
• Medications: what are you taking, does it help, are there side effects?
• Social history/lifestyle: home set-up, do you work, socialise, have friends/family?
• Functional difficulties: how do you cope with everyday tasks?
• Issues of anxiety, mood and confidence

Now they’re beginning to build a picture of you – about what actually matters to you. This is about you and your life: you’re not just a diagram on a piece of paper or a drawing on a body chart!

 

Objective assessment

Once the subjective assessment has been undertaken we move on to the ‘objective (physical) assessment’. Physios (and patients) can often get a little bit panicky about this bit, i.e. physically moving you about. Understandably they don’t want to hurt you and certainly don’t want you to dislocate on them, and the same goes for you. Unfortunately this part can sometimes be a bit uncomfortable but it does serve a really important purpose. If we don’t start to move and assess you then we’ll never get an idea about what’s strong, weak, short, tight, imbalanced, how you move, what slips out, what doesn’t and why. If your physio is going to help improve your situation then they need to know about these things. So don’t panic, stay calm and take your time to do the hopefully gentle movements that your physio asks you to do so that they can start to get a sense of your physical status.

With trust hopefully established between the pair of you, a good basic physical assessment should feature evaluation of the following:

 

POSTURE

• Observed from front, back and sides
• Look for hyperextended joints (knees, elbows, etc)
• Check for flat feet
• Watch for ‘hanging’ on hips (passively leaning to one side when standing, using your ligaments to hold you up)
• Assess sitting and standing postures, for instance do you have a wide base of support when standing?

 

RANGE OF MOVEMENT

• Observation of quality and range of movement around each joint, including the spine
• Assess for areas of stiffness (alongside hypermobile joints)
• Compensatory movements
• Fear of movement secondary to pain or dislocation
• Control of movement
• Reproduction of symptoms
• Passive movements

 

STRENGTH

• Muscle strength around joints
• Any obvious weakness
• Muscle patterning – overactivity, underactivity and inappropriate activity!

 

STABILITY / PROPRIOCEPTION

• Joint position sense (proprioception)
• Clumsiness
• Swaying
• Poor balance

 

GAIT / MOBILITY

• Assess sitting to standing
• Walking pattern (gait – with and/or without aids)
• Compensations to ‘normal’ pattern
• Stability
• Quality of movement

 

More often than not that’s more than enough for one session. In fact, if there’s a long history then there may not have been time to cover even that much, but if that’s the case then no panic. Don’t expect a rushed treatment programme to be thrust at you. This is not a race – we’re not dealing with a hamstring strain that you can whack through in one session, we’re talking about a long-term condition that takes time to assess and manage properly. There’s a lot to consider with hEDS/HSD and usually a lot to cover, so we get through what we can in the time available and if we panic and try to do too much in one go, that’s where things sometimes go wrong – key information gets missed and patients get ‘yanked about’ and end up in pain. So things need to be taken a little steady, but equally we haven’t got all day so we try to get through what we can during a session in a timely manner.

Having completed the subjective and objective assessments this is where the reality check kicks in…

No physio can treat everything in one go. They can’t clear you of your hEDS/HSD or instantly rid you of your pain. They don’t have a magic wand. There’s only so much that they can do in any given session, but don’t despair because if you work together with time, effort and application from both sides, and the right strategies in place, you can achieve an awful lot more than you may imagine – but you have to stay realistic! There’s no sense in turning up and setting yourself a target of running a marathon or scrambling up Mount Everest. Be sensible. You have to be mindful that you have a condition that you need to manage.

Therefore, please remember:

1. Your physio cannot treat everything in one go
2. Things don’t change overnight
3. The hEDS/HSD is not going away
4. Pain may not necessarily change (hopefully, it might and it can) but strength, stability, fitness, function and outlook on life certainly can.

 

So what are the practicalities of physiotherapy?  

What can be done to help influence and improve your life? The accepted key principles of physiotherapy for managing hEDS/HSD are:

• Self-management – putting you in charge of your condition, not your condition in charge of you
• Restoring function – working towards realistic goals
• Addressing weakness – if it’s weak, try to strengthen it
• Addressing tightness – if it’s short/tight, try to lengthen it
• Addressing compensatory movement patterns
• Addressing poor proprioception
• Improving fitness
• Addressing fatigue
• Acute care management – dislocations/subluxations
• Education – anatomy, healing, non-pharmacological PoTS management advice and PACING!

 

“There are no recipes” 

The physiotherapist has to treat what they find. Yes, you all have hEDS/HSD, which often comes with many common similarities and presentations, but you are all different. You each have different strengths, weaknesses and deficiencies and as such need to be assessed and treated individually, and any physio worth his/her salt should look for the issues specific to you. That said, there are still many common treatment methods used in physiotherapy for addressing some classic hEDS/HSD presentations which will apply to almost every one of you. Although not all of these approaches have been tested with research in hEDS/HSD populations, expert clinicians have recommended their use when appropriate:

 

STRENGTHENING

• Use some form of load (resistance bands, dumbells, body weight, you can even use gravity)
• Baseline number of repetitions (start low) – use a paced approach
• Movements done with control
• Make sure that the correct muscles are working!

 

PROPRIOCEPTION (JOINT POSITION SENSE)

• Use taping for feedback
• Gym ball exercises
• Single leg stands (if able), wobble boards, balance boards
• Pressure garments can provide proprioceptive feedback
• Exercising with mirrors can give great visual feedback for proprioception

 

POSTURE

• People massively underestimate the role posture plays in a pain condition. If you hold yourself in a poor posture for hours on end, don’t then be surprised if there’s a painful consequence to that.
• Work with your physio to identify what correct posture to hold yourself in (and why). Do stretches to address your tight bits.
• Work that core. Postural re-education also needs core, core and core!
• Use mirrors to help set your posture.
• Use prompts to help remind you about your posture (e.g. every time you get a text message think about how your posture is, etc).

 

HYDROTHERAPY

• Water can be a really nice medium in which to exercise. It can be kind on the joints and offer the opportunity to do some gentle strengthening exercises in a supportive way.
• Water exercises can also be a useful way to build cardiovascular fitness and improve proprioception.

 

STRETCHING

• Yes stretches! Just because you are hypermobile doesn’t mean to say that parts of you don’t get stiff too. Stiffness can be a common complaint
• Global muscles can often overwork and get tired, causing ache and muscle spasm
• Stiffness can occur through pain and disuse
• But please make sure you don’t overstretch… “Just because it goes there doesn’t mean you should take it there!”

 

ACUTE MANAGEMENT (DISLOCATIONS AND SUBLUXATIONS)

• Advice and education regarding the management of such episodes. This is covered in a further EDS UK article ‘Managing dislocations and subluxations in hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders’.

 

EDUCATION

• Knowledge is power
• Education helps dispel unhelpful beliefs about what might be going on inside of you
• Education can help de-catastrophise
• Education can offer advice regarding the non-pharmaceutical management of PoTS symptoms.
• Education helps you understand pacing, flare-up management and pain management principles. And PACING is one of the most important principles that one can learn for managing hEDS/HSD.

 

What about working with my local physios? 

The principles are all the same. Your local physio may not always be quite as familiar with hEDS/HSD, but they’re all trained and qualified and as long as they follow the subjective and objective assessment plan as outlined above, then they should have a pretty good shout at helping you to get on track.

 

NHS physiotherapy considerations

Due to NHS pressures some local physios come under constraints that can make their job that little bit more difficult when dealing with hEDS/HSD patients.

 

Number of sessions

Some local physio services are constrained by the number of sessions they are allowed to offer patients (often only up to six treatment sessions). That’s not really a lot when dealing with hEDS/HSD patients with a lot to cover, but sadly the physios’ hands are often tied.

 

Time constraints

Some departments are under pressure concerning the length of a treatment session. Sometimes that can be 45 minutes, sometimes 30 or even 20 minutes. That means your poor local physio has to do a lot in a very short time. Again, not their fault, so please don’t take it out on them. They’re doing their best.

 

Triaging

Hospitals are under pressure to make sure that patients referred to physiotherapy do not have to wait too long for an initial appointment. What does this actually mean for you? On occasion you may get triaged to see a physiotherapist who happens to have space in their diary but who may not necessarily have the most experience with hEDS/HSD, and you may then end up getting frustrated with them during your appointment. Again, please be kind, it’s not their fault and they are doing their best. All physios are qualified and will often discuss your case with a senior physio afterwards if they need any extra advice.

 

Private physiotherapy considerations 

For those of you who choose to seek private physiotherapy to help manage your hEDS/HSD, please remember that paying for it does not mean that they can get you ‘better quicker’. It also does not mean that they are necessarily any better than an NHS physio. All physios are always trying to do their best to help you as much as possible whether they be private or NHS.

 

What’s the bottom line with regards to the practicalities of physiotherapy and hEDS/HSD? 

Go with realistic expectations and make sure that you set realistic goals.

Help guide your treatment – provide a clear, concise history and a problem list. This gives both you and your physio something to work towards.

Follow the advice your physio gives you, then give feedback at the next session as to whether it is helping. Don’t decide by yourself to start changing and altering things that your physio has given you to do between sessions (unless it’s causing you raging pain) otherwise they won’t know whether what they’re doing is working or not.

Stay calm and don’t get frustrated.

HEDS and HSD are lifelong conditions, but with good careful management and the application of key principles the impact on your life can be greatly reduced. Best of luck!

Peer reviewed by: Dr Jane Simmonds, Senior Teaching Fellow, Great Ormond Street Institute of Child Health, UCLH and Specialist Physiotherapist, The Hypermobility Unit, Hospital of St John and St Elizabeth, London

Jason Parry, Extended Scope Physiotherapist / Clinical Specialist Physiotherapist, University College London Hospital and The Hypermobility Unit, Hospital of St John and St Elizabeth, London
Please note: The following text cannot and should not replace advice from the patient’s healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.

People are always talking about the importance of exercise. Hardly a week goes by without some expert or other popping up on the news telling us what’s best for our lifestyle and to “get more exercise”. Every time you go to see your GP, I bet they keep mentioning exercise as well; and if you ever go and see a physiotherapist you can be certain that they will be advocating the virtues of exercise. All of this preaching of exercise and a healthy lifestyle is typically aimed at the entire population, but sometimes reserved in particular for certain sections of society where the benefits are said to be greatest.

Unsurprisingly, the evidence advocating the benefits of exercise and movement is positively overwhelming. In fact, if exercise could be wrapped up in a tablet, it would be the most widely prescribed drug in the world! So what are some of these benefits? Prevention and management of the following conditions for a start:

Diabetes
High blood pressure
High cholesterol
Heart disease
Obesity
Osteoporosis
Arthritis
Depression and anxiety
These are some of the most common health conditions affecting society and account for a huge proportion of GP/hospital visits and even deaths each year, and just because you happen to have hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorder (HSD) it does not preclude you from developing any of the above conditions as well. You can’t prevent yourself from having hEDS/HSD, but you can help prevent yourself developing those conditions listed above, so already you’re winning with exercise.

And there are other benefits to exercise too! For instance:

Feeling more confident about moving
Improving the flexibility of stiff parts of the body
Allowing you to resume activities
Improving stamina
Improving the body’s muscle endurance
Stress and tension release
Losing weight and increasing muscle tone
Improving fitness levels
Improving self-esteem
So we’ve established that exercise is beneficial, but how can you exercise with hEDS/HSD? This is one of the most common questions among hypermobile patients. They report various difficulties related to exercise – unstable joints, chronic pain, postural tachycardia syndrome (PoTS) getting in the way, limiting gastric symptoms and of course fatigue. Well, exercise (and movement in general) actually forms one of the mainstays of managing hEDS/HSD and can offer you the capacity to hopefully improve your functional ability, allowing you to achieve more goals in life.

It isn’t always easy and there is a whole spectrum of ability when it comes to hEDS/HSD and physical status ranging from mild to very severe, but everybody, yes everybody, can benefit from exercise and movement. You just need to know what type is suitable for you and how to go about it, which leads us to the next most common questions that get asked – “What exercise is safe for me to do?”, “Are there any exercises that I should avoid?”, “How can I avoid deconditioning?”, “Should I do stretches?”, “How much exercise should I do?” and “Can I exercise at home without having to go to the gym?” All valid questions and ones that will hopefully be addressed within this article. But before we go on to do so, there are some important things to mention.

Firstly, you should always ensure that you are initially cleared to be able to exercise by any medical practitioner whose care you may be under (cardiologist, gastroenterologist, rheumatologist, GP, etc) prior to undertaking any form of exercise programme. Secondly, everyone is an individual and hEDS/HSD affects everyone in different ways. There is no substitute, therefore, for an individual assessment in order to determine the most appropriate exercise regime for you to follow. This should ideally be done by a fully-trained healthcare professional (chartered physiotherapist) who will then also be able to monitor and adapt the exercises accordingly. It would be impossible to provide an exhaustive list of exercises suitable for everybody in an article like this as each of you will have different 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.

To begin with, let us look in turn at the different types of exercise components that exist. There are four key types:

Aerobic
Strength
Flexibility
Proprioception/balance

Aerobic exercise
Otherwise known as cardiorespiratory or cardiovascular exercise. This is the exercise that gets the heart and lungs pumping and transfers oxygen to the muscles. We generally achieve this form of exercise by means of activities such as walking, use of treadmills, use of exercise bikes, using cross-trainers and swimming to name but a few. The recommended amount in the ‘normal’ population is 150 minutes of moderate intensity per week, broken down into five 30 minute sessions across the week. Although this may sound a lot, each 30 minutes can be broken down again into three 10 minute chunks per day, or even six 5 minute chunks if you so wish. What is moderate intensity? As a guide, there is something called the ‘Talk Test’ whereby if you are doing moderate intensity activity, you should be able to talk but not sing during the activity.

This may be achievable for some of you, in which case go for it! However many of you may well find the above difficult to achieve as a starting point. Pushing too hard too early may result in a flare up. How can it be made easier, or done at home? Well, as stated above, if able then walking is a great way to achieve aerobic exercise. Going up and down stairs if you can is another way. What if you find walking difficult? Swimming may be an option. You can either swim or simply walk around in the pool. No access to a local pool? Try buying a small set of free-standing pedals and ‘cycle’ at home, this may be very useful if you have PoTS or are very deconditioned. No need for a full exercise bike. If you find pedalling difficult, then you can place the pedals on a table and use them as a hand-bike. Even household activities such as cleaning and gardening can be a form of cardiovascular exercise. Don’t worry as well if you can’t achieve 30 minutes per day. All exercise should be paced and you should always start with a small manageable ‘baseline’ amount that you are able to do without really flaring-up your symptoms. Start with low-intensity exercise for 5-10 minutes two to three times per week. That’s fine. Do what you can manage. It can always be built up, but doing something is better than doing nothing. Ironically cardio exercise can be a great way of helping to beat fatigue, as although it may seem like it expends all of your energy, over time your energy capacity should increase as you become fitter.

Strength exercise
This form of exercise enhances muscles, ligaments, tendons and bones. Being stronger reduces the risk of injuries and provides additional support to your joints. Having strength offers you greater capacity to undertake physical and functional tasks. Strengthening can be achieved at home (or in the gym) in the following way:

Use some form of load (resistance bands, dumbells, body weight – you can even use gravity). Some examples might include strengthening your legs simply by doing sit-to-stands from a chair, or loop a resistance band around the back leg of a chair that you are sitting on, pull it forward and place your foot in it, then straighten out your knee. Strengthen arms by doing bicep curls holding a tin of beans or a carrier bag with stuff in it. Strengthen shoulders by holding a small dumbbell in your hands and raising your arms out to the side (be careful if you are prone to subluxations/dislocations).
As mentioned earlier, begin by working out a baseline number of repetitions (reps) per exercise that you feel you can manage without flaring-up your pain (start low) – and use a paced approach (though your pain may be there all the time, we want to avoid anything that really ramps it up and makes it even worse).
Pilates – basic level mat-work exercises modified for a clinical population can be excellent for building core strength, for proprioception and body awareness, and is also thought to help with motor control. Avoid higher level traditional Pilates exercises that can be excessively difficult and can put undue stresses on the body. If doing a Pilates class, then inform the instructor about your condition first. A good instructor should then be able to modify the exercises so that they are appropriate for you.
Movements must be done with control.
Make sure that the correct muscles are working (this is where the advice of a physiotherapist can be especially useful).

Flexibility
Despite being hypermobile, parts of you can still get stiff. Stiffness can be a common complaint. Global muscles can often overwork and get tired, causing ache and muscle spasm. Stiffness can occur through pain and disuse, so maintaining flexibility can be beneficial. Parts of our body can often stiffen up if we maintain static (and poor) postures for long periods of the day. Think how long you sit, stand or lie in one position throughout the day. This is why it is important to change position regularly. Doing gentle stretches (or ‘mindful movement’ – a form of gentle controlled movements) can also help address the stiffness that can develop. Yoga can be useful too, but find a good teacher and please take care not to overstretch into hyperextended positions. Try to remember that “just because it goes there doesn’t mean you should take it there”! Commonly, it can be difficult to know where “there” is. It can be difficult to know at which point to stop the stretch. That’s where the next bit comes in – proprioception and balance.

Proprioception / balance
Proprioception is the body’s ability to sense position and movement within joints. It enables us to know where our limbs are ‘in space’ without us looking. It relates to coordination. Impaired joint position sense can cause joints to slip out of place. In general the better your proprioception, the better your stability. How can we train this at home? There are a number of different ways, but again without an official assessment what you choose to do depends very much on your own perceived ability to manage the exercise. Most importantly you should choose an appropriate level for you and ensure a safe environment when doing it. That said, some ideas could include:

T’ai Chi – an excellent form of exercise, comprised of slow, controlled movements. Good for balance and stability. Chi Gung is also advocated.
Standing balance exercises – these can range from standing with feet together, trying this with eyes closed, single leg standing or standing on a wobble board. You could try throwing and catching a ball whilst standing on a wobble board or even try mini squats if you’re really brave.
Wii Fit – if you have one of these, they can be a fun way of training balance and stability. There are a variety of different games that can be incorporated into using the Wii Fit board.
Gym ball – a brilliant and versatile piece of home exercise equipment. Try sitting on the ball in a good posture. If able, try this with your eyes closed. Progress to sitting with your feet close together. If able, try straightening out one knee and lifting your foot off the floor, maybe try that with your eyes closed if you’re feeling really brave.
As already mentioned, when doing any form of balance/proprioception type of exercise you MUST ensure a safe environment (exercise on a mat, place chairs either side of you or stand close to a wall), as well as pick an exercise that is at a suitable level for you, i.e. challenging but not impossibly difficult.

Exercise doesn’t have to be a chore, it can be fun. Prepare yourself a nice environment in which to exercise. Play some music or spray the room with nice smells (avoiding the smell of hard exercise!) Exercise with friends or family for added motivation. You don’t have to be a gym member to get the benefits of exercise and movement. As outlined above, there are lots of exercises you can do at home, standing or sitting. Just as an additional note though, it is generally advisable to avoid high impact and/or contact sports (for the more adventurous of you). Focus instead on low to moderate impact aerobic activity and low load strengthening and proprioceptive training.

Although the best way to develop and follow an exercise programme is under the supervision of a physiotherapist who will carefully assess your specific needs and be able to monitor and adjust your progress, it is still possible, and to be honest important, to maintain some level of movement and simple exercises yourself at home.

We can see, therefore, the importance of movement and gradual exercise for so many reasons in the hypermobile population. It can help with the pain from stiffness and deconditioning. Exercise can also give you more energy to help with fatigue. It can help improve your overall stability, reduce the risk of injury and reduce the frequency of dislocations. For those of you that may suffer from PoTS, exercise can also help reduce its symptoms (just make sure you replace lost salt afterwards through electrolyte drinks or salt tablets with water). Exercise can help release endorphins and encephalins, the feel-good hormones within us, aiding mood management; and ‘mindful movement’ can help take the fear out of moving and using our bodies. Exercise enhances general health and wellbeing. Try not to think about doing exercise for exercise’s sake, think of it as a means to you being able to achieve more meaningful things in your life. Exercise really is the best medicine.

Peer reviewed by: Dr Jane Simmonds, Senior Teaching Fellow, Great Ormond Street Institute of Child Health, UCLH and Specialist Physiotherapist, The Hypermobility Unit, Hospital of St John and St Elizabeth, London

Date of last review: 01/12/2017