Building your own EDS and HSD toolkit

Tips from support groups around the UK
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.

This article contains information provided by patients and it should be taken as their own opinion not that of medical professionals. Self management is an important part of managing EDS and HSD but we always recommend discussing self management techniques with a medical professional first. This article was published in the Summer 2020 edition of Fragile Links.

We know that having an accurate diagnosis is important for many reasons. But, once diagnosed, people are often left to manage their symptoms themselves. Diagnosis for quite a few of our members has been ‘by chance’, for example when they have consulted a specialist about a problem that seemed unrelated. We’ve heard from many of you that once you know what you’re dealing with, you can do your own research and start to assemble your own ‘toolkit’ to help manage symptoms. Here, we’ve pulled together some useful tips shared my members at our support groups around the UK.

‘Friendly’ specialists and therapists

Local support groups and our volunteer Area Coordinators are often a great source of information about sympathetic or knowledgeable local healthcare professionals and therapists. EDS UK is always keen to hear about positive experiences. We then make contact with the professional and ask for their permission to hold their details on our ‘friendly specialists’ list. This means the information can then be shared with those who contact our adviceline.

Several members said that occupational therapy (OT) input had been be very helpful for them. Some people find even minor adaptions to their homes can make a big difference – for example a different type of shower; a tipping kettle. Occupational therapy (OT) advice can also be key to helping young people overcome challenges at school or college. We recommend talking to your GP about accessing NHS occupational therapists. In some areas it is possible to self-refer to OT. Private appointments are also available.

Managing pain

How to manage long-term (chronic) pain is a regular discussion topic in our support groups. What works for one person doesn’t work for others and, like a lot of aspects of EDS and HSD, some trial and error is needed. We hear that most people have to find solutions other than prescribed medication for pain as it’s often not enough or stops working after using it for a long time.

Many people find heat is useful. Long, wrap-around microwavable heat packs (such as Hotties) which can be used around the waist and hips are particularly popular. Hand-held massagers such as the DrGraeme massagers are also highly rated by members to ease muscle pain.

TENS (transcutaneous electrical nerve stimulation) machines can be helpful for some joints. People report them being useful while they are on but don’t find them a long-term solution. Some osteopath clinics may loan you a TENS machine to try out if you are thinking of buying one.

If you are comfortable applying products to your skin, magnesium spray, cream or gel helps some people, especially with leg cramps or restless legs at night. Capsaicin cream (which contains the substance from chilies which makes them hot) has also be found to be helpful.

Kinesiotherapy tape (KT Tape) can be useful to support joints and aid recovery. There are many different brands available from Amazon, Physio Room, Rock Tape, and other retailers, however the tape can irritate sensitive skin, produce allergic response, or damage very thin skin. Some members of our community advise wetting the tape to remove it or using Pepto-Bismol or specific ointments underneath as recommended by their physios to help with this.

Distraction and relaxation techniques can help too – listening to audiobooks and podcasts; crocheting (which unlike sewing and knitting, can be done lying down), mindfulness and guided meditations of which there are many on YouTube (also see the By Members, For members section in Fragile Links). Many of you also get comfort and distraction from your pets.

Pacing

Pacing is an important part of the self-management of EDS and HSD and can help with fatigue and pain. Working with a traffic light system can help to manage this well.

Dividing activities into three categories depending on the level of energy they take creates a ‘traffic light system’. Green is for easier tasks, such as answering a couple of emails or making a bowl of cereal. Amber are tasks which take a bit more energy, for example preparing a simple meal or taking a shower. Red tasks take a lot of energy: going for a dog walk or attending a medical appointment. These tasks are very personal to each individual and can be adjusted as such. There should only be one red task a day. If you find you have a day with two or more red tasks planned, look at adjusting this to enable you to pace and avoid a boom and bust cycle. Stickman Communications has a fab fridge magnet set to help you organise your traffic light system: https://stickmancommunications.co.uk/product/pacing-fridge-magnet-set/

Mobility aids

Members shared their experiences of overcoming the psychological barriers to using mobility aids. People talked about having their ‘worlds opened up’ by being able to get out and about and participate more fully once they had an aid that worked for them. A good OT can help to explain to other healthcare professionals or funders how something like a wheelchair can be beneficial, even if it is not needed all the time. Aids such as Smartcrutches™ and collapsible canes were also seen as very helpful to increase mobility. Smartcrutches™ have helped people who have wrist problems and have struggled to use traditional crutches and canes.

Exercise

We’ve heard some concerning stories about exercise advice given to people with EDS and HSD, sometimes by professionals. The right type of exercise is central to managing a range of EDS and HSD symptoms. It can take a long time to increase the stability in your joints through exercise so it’s important to find exercise that works for you. You’re likely to need to do something every day. What works for one person might make things worse for someone else. Get advice from an exercise professional who has experience with hypermobility, preferably someone who has been specifically trained in managing hypermobility.

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. However, there are some generic principles applicable to everyone that can be followed when it comes to exercising. Jason Parry provides a comprehensive overview of these under the exercise row in the dropdown here.

One example of a simple exercise from our support groups is a stretching exercise which can be done in bed or sitting in a chair – stretch your leg out, point your toes and make shapes of the alphabet with your feet. Do each foot separately and repeat daily. This helps with ankle swelling and stability.

There were mixed views about the benefits of swimming. Some people found it very soothing, as the water supports your joints while they are moving, minimising pain. Other people found that warmer pools triggered PoTS symptoms or that they struggled to get in and out of the pool. In these cases, it was recommended to look for accessible swimming pools. Some have dedicated sessions for people who are less able.

Some special needs schools have hydrotherapy pools where you may be able to arrange access for a lower fee than through a private provider. Hydrotherapy is also offered as part of hypermobility programmes at some hospitals in the UK.

Some hospital physiotherapy departments run Pilates sessions. These are often adapted for people with specific joint problems or conditions.

Diet

Some of our members have found some relief for their symptoms using the low FODMAP diet. This diet is clinically recommended to help manage irritable bowel syndrome (IBS) and is based on reducing or eliminating foods which are high in fermentable oligosaccharides, disaccharides, monosaccharides and polyols (FODMAP).

The diet is complex and can be difficult to follow, especially long-term. It is certainly not for everyone and research is still needed to test its usefulness in conditions other than IBS. As with all significant changes to your diet, it is important to make sure you are still getting all the nutrients your body needs. It is recommended to talk to a FODMAP-trained dietician before starting the diet. Your GP practice should be able to refer you. If you want to see a dietician privately, make sure they are registered with the British Dietetic Association (BDA).

If you do decide to give it a try, there is lots of information and hints and tips online and in cookery books specifically to help people who are trying to follow it. There are also products available in supermarkets and health food shops to substitute some of the foods to be avoided on the diet, for example asafoetida (or Hing) powder to replace onion and garlic. Please check the allergen information carefully though!

There are two excellent webinars on the low FODMAP diet, by NHS dieticians, at: https://patientwebinars.co.uk/condition/ibs/webinars/

Working with healthcare professionals

A key part of your toolkit will be working positively with healthcare professionals, either those you are already seeing, or those you get referred to. As EDS and HSD symptoms affect the whole body, most people won’t have one doctor or therapist who can deal with everything but GPs play a key role in helping you, coordinating your care and of course, involving specialists, when this is needed. Working at building up a good relationship with your GP will be beneficial in the longer term. EDS and HSD are complex, life-long conditions and your GP’s involvement is unlikely to be short-term!

Understand your doctor

Your GP may well feel at a disadvantage. They may have barely heard of EDS or HSD, let alone know anything about them. The range of symptoms and diverse ways the different types of the conditions affect people is also a huge challenge for medical professionals faced with it for the first time. Reassure your doctor that you are not expecting miracles but would appreciate his/her help and support to make your day-to-day living as acceptable as possible.

The Royal College of GPs’ EDS toolkit, developed in partnership with EDS UK, is aimed at helping GPs to spot hypermobile EDS and hypermobility spectrum disorder. It guides GPs through how they can support their patients in managing the most common symptoms.

GPs are specialists in people not just diseases. They are intended to be the patient’s advocate and can help to interpret information and advice from specialists and to work with you to apply medical advice to your specific circumstances. This is particularly important where information from different specialists may be conflicting or unclear.

Preparing for appointments

Consulting a doctor can be a daunting prospect. You are looking for help, you do not want to be classed as a time waster and you may rehearse what you are planning to say over and over again. The consultation is a two-way process. The doctor can help you best if you can explain what you are hoping for as simply and as concisely as possible. Remember, GP appointments are usually allocated in 10 minute slots. Be realistic about what can be achieved in this time, especially if it’s the first appointment to discuss something. Sometimes you may be able to book a double appointment if your issues are more complex.

There will not be time to go through your full medical and family history in a single appointment. If you have multiple symptoms or problems you would like help with, choose the one which is causing you the most difficulties or you are most concerned about. It is worth keeping a symptom diary if you can. Some GPs find these useful to see the pattern of symptoms over time. It can also help you to remember what has happened in between appointments.

It can be helpful to the GP to come prepared to talk about your:

Ideas (what you think might be wrong or going on in your body)
Concerns (any serious condition you would like ruled out)
Expectations (what you are hoping the doctor will do for you today)

However, these are likely to result in a discussion and may not necessarily lead to the outcome you were expecting or wanted.

Consider using an e-consultation (electronic consultation or online consultation) with your GP, if this option is available to you. With these systems, you are usually taken through a series of questions to answer online. Your responses are looked at by a GP, who can then choose a number of different options. They may advise you, either online or via telephone, arrange tests or a prescription. If you progress to a face-to-face consultation after using the online service, this is often more constructive as the GP has been able to go through and think about the information you have provided in advance.

Referrals

We are sometimes asked what to do if a GP won’t refer you to a specialist. Only one in 20 GP consultations result in a referral to a specialist and this can be for a variety of reasons. GPs can only refer to specialists where they exist – for EDS/HSD, as we know, there are very few doctors who have in-depth knowledge about all aspects of the conditions.

If you have asked your GP to refer you to a specialist and they have not done this, listen to their reasons and ask what they plan to do instead. Then, go home and consider this carefully. Remember that it may be months before your appointment comes through and your GP may be able to try other management strategies or arrange certain investigations before referring you. Sometimes this may resolve your problem more quickly, and on other occasions it will mean that you get more benefit from any subsequent specialist appointment. We recommend you try the management your GP has suggested and, if it doesn’t help, go back. Keep doing this if your problem doesn’t improve. If you think your GP might be wrong, go back and explain clearly and calmly what you hope to achieve from a referral.

If you are referred, we suggest making a list of the issues you’ve been having from when you were younger until now to give the doctor an overview. This also means that you don’t forget anything when you’re in your appointment.

Make notes and ask questions

It can be valuable to have a friend or relative come into a consultation with you especially if you are anxious or depressed. Your companion can give you moral support and maybe make a note of what the doctor is advising you. It can sometimes be hard to remember all that was said once you leave the appointment. Do ask questions if you are unsure what the doctor is saying or if you want him/her to explain further. It is important you are aware of all the facts and fully understand what he/she is saying.

Working with a physiotherapist

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 aims to collect as much information as possible about you and your problems, and then uses this 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 key areas it is helpful for your physiotherapist to know about during the subjective assessment are:

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

The subjective assessment is usually followed by a physical assessment. As the name suggests, this involves moving various bits of your body and can be a bit uncomfortable but it serves a really important purpose. So, try to trust your physio and take your time. A good, basic physical assessment will usually look at your:

Posture
Range of movement
Strength
Stability/proprioception
Gait/mobility

It is only after these two assessments that a treatment plan can be put in place. If there is a lot to discuss in your subjective assessment, your physical assessment may not be finished in one appointment. It is only after both of these assessments have been completed that a treatment plan can be agreed. No physiotherapist can treat everything in one go. We recommend going with realistic expectations and setting 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.

Medical allies from other disciplines

Some people have found a medical ‘ally’ from perhaps unusual sources, for example a gastroenterologist, a cardiologist, a dermatologist or pain psychologist. In some cases, they have been key to getting a diagnosis or a change in management plan by writing letters to other medical colleagues, giving their views.

Community pharmacists can be excellent sources of advice and information and usually have more time than GPs. Consider building up a relationship and helping them to develop an interest in your condition, if they don’t have one already.

Awareness of EDS and HSD is improving in the medical community but these are complex, life-long conditions which are difficult to spot and challenging to manage. While the healthcare professionals you come across may not know very much about your condition, if they open-minded and willing to listen, try to build up a good relationship and work with them to help them learn. Remember to let us know about positive experiences too!

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.

https://www.paintoolkit.org/

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:

Body Chart

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.

Useful contacts

Fibromyalgia Action UK

Studio 3006, Mile End Mill, 12 Seedhill Road, Paisley PA1 1JS

Tel: 0300 999 3333

http://www.fmauk.org/

Peer reviewed by: Dr Hanadi Kazkaz, Consultant Rheumatologist, University College London Hospital

Last year 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

Jenny Morrison, Neurogenetics Research Nurse, University College London Hospital

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.

Peer reviewed by: Angela Hunter, SLT (retired) and trustee, EDS UK

Jason Parry, Extended Scope Physiotherapist / Clinical Specialist Physiotherapist, University College London Hospital and The Hypermobility Unit, Hospital of St John and St Elizabeth, London

Joint dislocations and subluxations can commonly occur in people with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD). Such events can obviously prove very distressing so it can be valuable to learn how to manage and cope when such situations arise. A good place to begin is to understand the difference between a dislocation and a subluxation:

A ‘dislocation’ is defined as “displacement of a bone from its natural position in the joint”. This is where the two bones that form a joint fully separate from each other. If we consider a shoulder for example, which is a ball and socket joint, this can be illustrated whereby the ball (which forms the top of the arm bone) slips entirely out of the socket it should sit in within the shoulder. This can happen in any direction, but the main point is that the two bones completely separate. Very painful!

A ‘subluxation’ is basically defined as “a partial dislocation”. It can be no less painful than a full dislocation but the two bones that form the joint are still partially in contact with each other. So once again if we consider the shoulder joint as an example, the ball which completely came out of the socket in the dislocation example above would still be partially sitting in the socket if it was a subluxation.

These events can happen in almost any joint but some are more common than others. Shoulders, knees, thumbs and ankles seem to be some of the most prevalent. Both dislocations and subluxations can be painful, irritating, infuriating and occasionally debilitating problems. However, they don’t need to ruin your life and can with patience, effort, trust or time be managed. Not necessarily completely eliminated, but managed.

Why does this happen?

The main reason is abnormal collagen composition. Collagen – primarily Type I collagen – is the main structural protein of the various connective tissues in the body. It is found in ligaments, tendons and joint capsules and makes ligaments and tendons strong like little ‘guy ropes’. What do ligaments and tendons do? Ligaments connect bone to bone, tendons connect muscles to bone, and joint capsules are like envelopes of tissue that surround a moveable (synovial) joint. So we can see that these ligaments, tendons and joint capsules play an important role in giving a joint stability.

So let us now consider hypermobile EDS (hEDS). This is a heritable disorder of connective tissue (HDCT) caused by a defect in the structure, production or processing of collagen, which makes the collagen in ligaments and tendons stretchier (more lax). This means that joints are potentially less stable – hence greater propensity for subluxations and dislocations.

There are other reasons for dislocations and subluxations:

Altered muscle tone – this can often account for dislocations. Inappropriate muscle patterning, whereby certain muscles around a joint switch on when they shouldn’t necessarily and then inappropriately work way too hard, can often ‘pull’ a joint out of place. The joint then also becomes easier to slip out, of course, if it is more lax in the first place. Muscle fatigue, spasms and stress can all play a part in this too.

Impaired proprioception – proprioception is the body’s ability to sense position and movement within joints and 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.

Repeated overstretching – otherwise known as too many ‘party tricks’. Some hypermobile patients have the capacity to ‘amaze’ people with their ability to wrap themselves into weird and wonderful positions, putting their arms and legs into positions that the rest of us gawp at, such as choosing to pop shoulders in and out of joint or folding legs over the head. As ‘amazing’ as this may be, it’s time to stop! Repeated overstretching to that degree will only exacerbate the laxity and the chances of the joints slipping out of place. It is perfectly acceptable for hypermobile people to undertake regular stretches within ‘normal’ joint ranges, but don’t stretch your joints way beyond this ‘normal’ range and give up the party tricks. Just because the joint ‘goes there’ doesn’t mean you should take it there.

The shape of your joint surfaces – some of you may be born with shallow shaped joint sockets or other bony shaped ‘anomalies’ that predispose a joint to possibly slipping out of position more easily. Unfortunately, that just may happen to be the shape of your skeleton.

Traumatic incident – one of the other reasons for a joint to come out of place (and the most common one for those without EDS). Traumatic incidents can happen to anyone, but extra joint laxity may actually work a little in your favour with this one, whereby it may prevent you damaging some of your ligaments/tissues in the way that a non-hypermobile person who suffered a traumatic dislocation probably would.

How frequent are these events?

The answer to that is different for different people. Some people can get them maybe just once or twice a year, others once a month. Some people have them once a week and others once a day. Some people get them happening repeatedly several times throughout the day and in certain people they never seem to stop. In some, when it happens the joint just finds its own way back in to place, which is a relief, but even then we need to try to reduce the frequency if we can. But in others, once the joint slips out it won’t go back in again, and the natural reaction then is to panic! This is perfectly understandable. The joint is out of place, the pain kicks in (often in a big way) and the panic starts. At this point, some people understandably pick up the phone, call for an ambulance and head off to A&E.

DON’T PANIC! STOP FOR A SECOND, BREATHE NORMALLY AND STAY CALM

Panic causes more stress. Panic causes more muscle spasms. Stress and muscle spasms cause more pain and then there is less chance of resolving the dislocation. In order to start managing this situation and taking control you are going to have to start learning not panic. Because what happens at A&E? They will often give you pain relief of some sort (perhaps Entonox) or they may go the whole hog and give you a general anaesthetic. Then they’ll yank your joint back into place. However frequently, and we could be talking within minutes, the joint will often pull itself back out of place again because of the muscles still spasming around the joint, and you’re back to where you started.

In these situations, some A&E doctors might then stick you in a plaster cast to ‘hold’ the joint in place. Imagine, then, the battle going on under that cast, with your joint often trying to pull itself back out of position again while being forcibly held in place by the cast. It’s often very painful and then when do you take the cast off? This is not a viable management solution or a good way of life for you.

So what should you do if your joint comes out?

Six key principles of management

Here are the six key principles that I suggest you need to start utilising in order to begin to get a grip on managing this situation as opposed to this situation managing you. The main aims are to stay calm, keep on top of the pain and allow the muscles to relax. It takes lots of practice and patience, but it can be done.

Breathe

Use slow deep, relaxed breaths. Try using some relaxation techniques, there are lots of different ones out there. As painful as it is, and as difficult as it may sound, you need to start to try to take control of this situation. So start to learn how to breathe through it.

Use painkillers

Take some appropriate painkillers (analgesia) if you have some. However, note the word “appropriate”. You should only ever take analgesia according to the dosage indicated by your prescriber. Never take more than the suggested dose. You might feel like it may not be enough at the time, but if it can take some of the edge off, then that’s a great start. Please don’t ever overdose. What about Entonox (commonly known as gas and air) as pain relief? Some people have access to Entonox at home, or use it at A&E. There can be a role for it, but this must be used with caution. Prolonged use can lead to vitamin B12 deficiency and can interfere with DNA synthesis, not to mention cultivating a dependency, all of which are big issues.

Support the joint

You need to try to make yourself as comfortable as possible (I know it’s not easy). Use pillows or a sling if you have one. Find a comfortable resting position as much as possible. This allows the muscles to relax and stop spasming.

Try heat

Hot water bottles, wheat bags and a warm bath can all help to relax spasming and overactive muscles.

Distraction

Try to take your focus away from the pain and the situation. Listen to music, watch a film if you can, talk to friends/family, try a relaxation CD/MP3. This can be helpful as a short-term pain relieving strategy. Again it can help muscles relax.

Gentle massage

Sometimes gentle massage around the joint can help relax the muscles enough to be able to gently relocate the joint or for the joint to just slip back into place by itself.

What if it doesn’t go back?

Don’t expect the joint to go straight back in. It is often not unusual for joints to remain out of place for hours or even days. But once it’s out, it’s out. It’s not going out even more, so try not to panic.

Is it dangerous? Am I damaging my joint if it comes out?

It is highly unlikely. Your joint laxity allows for your ligaments and capsules to stretch. It is mostly just distressing as opposed to damaging.

When should you go to hospital or get help?

If the limb starts to change colour due to a lack of blood supply.
If your limb goes completely numb.
If you have tried strategies 1-6 above, have waited a reasonable amount of time and are still desperately struggling.

But as mentioned earlier, it is not unusual for A&E to relocate your joint only for it to pop straight out again or when the anaesthetic wears off. Therefore you need to learn to stay calm and to start to self-manage.

Reflect and learn lessons

One of the most valuable things you can do after a subluxation/dislocation is to reflect on the event (once you have had a chance to calm down). Were you moving in a way that normally causes the joint to dislocate? Did you move without thinking? What was your posture like? Were you tired or overdoing it? Were you stressed about something? It is so valuable to reflect on these types of questions to look for triggers as to why the event may have happened. It may have been none of these reasons, but if it was then you can hopefully learn to avoid repeating them in the future.

Finally, prevention is better than cure. It is obviously better if we can prevent these situations occurring in the first place as opposed to having to deal with them. So to that end, the following can hopefully help to reduce the frequency of such occurrences:

Physiotherapy to learn to control the muscles around joints and to use the right ones.
Rehabilitation to improve proprioception.
Possible use of supports/braces if required.
Try to manage stress and anxieties.

But ultimately, stay calm! The more you stay calm when these events happen and manage it yourself, the easier it should get each time.

Peer reviewed by: Dr Helen Cohen, Consultant in Rheumatology and Pain Management, Royal National Orthopaedic Hospital, Stanmore

Jason Parry, Extended Scope Physiotherapist / Clinical Specialist Physiotherapist, University College London Hospital and The Hypermobility Unit, Hospital of St John and St Elizabeth, London

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:

Your physio cannot treat everything in one go
Things don’t change overnight
The hEDS/HSD is not going away
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.

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.

Date of last review: 01/12/2017

Building your own EDS and HSD toolkit

‘Friendly’ specialists and therapists

Managing pain

Pacing

Mobility aids

Exercise

Diet

Working with healthcare professionals

Understand your doctor

Preparing for appointments

Referrals

Make notes and ask questions

Working with a physiotherapist

Medical allies from other disciplines

Managing pain .cls-1 { fill: #1f1e23; }

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Managing fatigue, sleeping problems and brain fog .cls-1 { fill: #1f1e23; }

Jenny Morrison, Neurogenetics Research Nurse, University College London Hospital

Stay hydrated

Have a healthy diet

Avoid caffeine and sugar

Exercise

Pace yourself

Good sleep hygiene

Therapy

Brain fog

Managing dislocations and subluxations in hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders .cls-1 { fill: #1f1e23; }

Jason Parry, Extended Scope Physiotherapist / Clinical Specialist Physiotherapist, University College London Hospital and The Hypermobility Unit, Hospital of St John and St Elizabeth, London

Why does this happen?

How frequent are these events?

Six key principles of management

What if it doesn’t go back?

Is it dangerous? Am I damaging my joint if it comes out?

When should you go to hospital or get help?

Reflect and learn lessons

Physiotherapy for adults with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders .cls-1 { fill: #1f1e23; }

Jason Parry, Extended Scope Physiotherapist / Clinical Specialist Physiotherapist, University College London Hospital and The Hypermobility Unit, Hospital of St John and St Elizabeth, London

The practicalities of physiotherapy

Subjective assessment

Objective assessment

So what are the practicalities of physiotherapy?

“There are no recipes”

What about working with my local physios?

NHS physiotherapy considerations

Private physiotherapy considerations

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

Exercise and movement for adults with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders .cls-1 { fill: #1f1e23; }