Category Archives: Joint Hypermobility Syndrome

24Oct/16

Women in pain

Women in painRecently I gave a talk to a group of female health professionals at the Inspiring Women in Medicine meeting entitled ‘Women in pain’. I spoke about the significant societal problem of women suffering persistent pain, which is one of the issues that comes under the banner of women’s health. Society needs women to be healthy and hence the problem of women in pain must be addressed. Fundamentally at present, society does not understand pain sufficiently to address this enormous public health matter effectively, which is where I believe we must begin: understanding pain.

If society understood pain….

  • individuals would know what to do and think in order to orientate themselves towards getting better
  • it would not be feared; instead the focus would be on overcoming pain
  • healthcare would deliver the right messages early on so that the right actions are informed by correct beliefs about pain
  • the right treatment appraoches would be employed from the outset
  • there would not be the same level of suffering — the figures say: 100 milliion Americans suffer persistent pain; 20% of the population; 1:5 children

Chronic pain is a huge global health burden that costs both individuals and society enormously in terms of finances and suffering. Of course, this pervades out into family and social networks and hence those around the individual can also be suffering through their on-going provision of care. Pain is a strain on society, literally. If it were understood, this can change.

Women in painWomen are reported to suffer more pain and visit their doctor more often about pain than men. Females are more likely to suffer functional pain syndromes. There are still many people, including healthcare professionals, who do not know what functional pain syndromes are or have insight into the basic biology that emerges as a range of painful problems that are very common. They include irritable bowel syndrome (IBS), migraine and headache, back pain, fibromyalgia, pelvic pain (e.g. vulvodynia, painful bladder syndrome, dysmennorhoea) and temporomadibular dysfunction. Other regular features include anxiety, depression, a history of early life events (and later in life when a challenging situation brings about pain and suffering), perfectionism, a person who is very hard on themselves and hypermobility.

This being the case, one would expect that research into how females experience pain and why they feel more pain would be stacking up. Unfortunately this is not the case with most research done in males and male rats. Clearly that has to change alongside the overall attitudes to women in pain.

Women in painRecently the press ran with stories about how women in pain receive different care and approaches to men. Women waited longer for treatment, were less likely to receive opiates for pain (opiates are effective for acute pain — there are big issues with the use of opiates for chronic pain) and were deemed to be more emotional and hence somehow their pain was different in the sense of how it should be treated. Of course this is wrong on every level. Each person has a unique pain experience that is flavoured by a perception of threat within a certain context and enviornment, based on prior experience and beliefs of that person. Therefore, each person needs to be addressed as such and treated according to this principle, man or woman.

There arWomen in paine some ideas as to why men and women should experience pain differently. The most obvious is that of gender biology based primarily around hormones and the menstrual cycle. In particular there maybe an important time at the onset of menarche when sensitisation could emerge in some individuals, thereby priming them for future events such as injuries, viruses and illnesses when the systems that protect us (immune, nervous, sensorimotor, autonomic, endocrine — they work together as opposed to being in isolation) are active in the face of a perceived threat and increasingly vigorously. What the person lives are the symptoms of thee systems working including fever, pain, altered perceptions of the world, altered thinking and emotions. It can sound like these are all separate ‘reactions’ when in fact they are part of an on-going cyclical process: we think, perceive and act as a unified lived experience.

Another observation relates to empathy and how women maybe more empathetic for the purposes of caring for their children. A truly empathetic person is a caring person yet they must be careful and skilled so as not to embody their own versions of observed others’ suffering. As an example, it is not uncommon for me to feel a pain in the same place that a patient is describing their pain to me. Understanding the mechanism, I can rationalise the feeling and it will pass as I actvely change my perception — this is likely the same mechanism that underpins the change from being in pain to not being in pain in all people. I know that others I have spoken to also have this experience, which one could argue is deeply helpful as a healthcare practitioner as we seek to understand the causes of the other person’s suffering.

A described emotion that often appears within conversations about pain, particularly women in pain, is that of guilt. The reasons for expressing guilt are based around the conflict between work, home, partner and children — trying to please all but rarely pleasing or looking after oneself. Being kind to self is important in the sense that being hard on oneself can be the cause of great suffering. This is common and will almost certainly be taking the woman closer to her biological protect line, the point at which threat is perceived and enacted as a pain experience. Learning how to foster the existing compassion towards oneself then, is a typical part of a comprehensive programme for getting better. With many whom I see displaying and admitting perfectionist traits, it is not a surprise that harsh inner dialogue results in repeated negative emotions. Strung together frequently, this forms the basis for chronic stress, which in turn is the means for a pro-inflammatory state, which emerges as aches and pains, troubled tummies, headaches, mood changes, sleep issues, fertility problems and more. The reason is simply that in the pro-inflammatory state, the body is in survive mode that is great when there is a real threat. However, most of the time there is no threat, it is just something we are thinking about that triggers the same response via a prediction taht one exists.

Now, there is nothing wrong in experiencing negative emotions. We need them as much as the others. It is really about the apporpiateness of the emotions: when we feel them, how long we feel them for, how often etc etc. If we consistently think that something bad will happen or ruminate on things that have happened rather than seeing things for what they really are in this moment, then this basic survival biology will keep going. This is where mindful practice is so beneficial, cultivating awareness of existing habits that allows for a reappraisal, a space to see things for what they are and gain insight into the causes of your own suffering and others, from which you can choose a new and healthy way onward. Clearly there is much more to say about mindfulness and its benefits, in particular in the face of mcuh exciting data from studies across the world.

Whilst this blog scratches the surface, it hopefully provides some food for thought. This is a significant public health issue that we can tackle by understanding pain and applying simple and sensible compassion-driven care, which will make a huge difference. Coaching the individual woman to coach herself in a direction that is toward her desired outcome is out role as we empower individuals and allow them to realise their sense of agency in getting better. There are simple measures such as movement, exercise and mindfulness that work in synergy to create a meaningful life to be engaging and enjoyed so that when challenges arrive, they are overcome and used as learning experiences. Science, compassion and sense are at the heart of the Pain Coach approach, one that we can all adopt to change for the better. Ourselves and our patients.

RS

The Pain Coach 1:1 Mentoring programme is for busy clinicians who wish to develop their working knowledge and to be effective in coaching people suffering chronic pain to lead meaningful and fulfilled lives. Contact us on the form below or call Jo for further information t. 07518 445493

 

 

 

20Jun/15

The problem of migraine

migraine by r. nial bradshaw (2012)

migraine by r. nial bradshaw (2012)

The problem of migraine is bigger than most people realise. In fact, the problem of chronic pain is bigger than most people realise, this being apparent as I purposely ask people I know and meet if they know what is the number one global health burden. It is chronic pain by the way, and migraine and headache sit in the top 10 along with back pain, neck pain and osteoarthritis. Depression is at number 2.

Migraine is sometimes referred to as a functional pain syndrome. Not everyone likes this term, myself included, yet it’s use does mean that we can consider migraine as one of a number of conditions that hurt and cause great suffering. These conditions have a common biology known as central sensitisation, meaning that the individual’s systems that protect are more likely to do so, resulting in persisting pain in many cases.

The other well known functional pain syndromes include irritable bowel syndrome (IBS), temporomandibular disorder (or jaw pain, clicky jaw etc), pelvic pain, dysmennorhoea, vulvodynia, interstitial cystitis, chronic back pain and fibromyalgia. These are often co-morbid with anxiety, depression and hypermobility. As individuals, it is common to find perfectionist or obsessive traits that may be useful in certain arenas such as work, helping to achieve great success, yet in other areas of life cause problems. More women than men report these problems, although I am seeing increasing numbers of men who often describe groin pain as a starter but then we explore the history and discover one or more of the aforementioned list. A further frequent finding is difficulty conceiving, this primarily due to the body systems that protect being persistently fired up (by normal living and exposures as well as stressors), and whilst that person is in such a mode, having children is not on the body’s agenda whereas survival is.

As with most of the functional pains, the story highlights certain vulnerabilities that can increase the likelihood of persisting pain including genetics, epigenetics, early life stressors and prior infections/injuries. These factors sculpt the systems that protect as they learn how to respond as well as becoming increasingly vigilant. The combination therewith creates an individual who is more likely to respond to actual or potential threat with vigorous and prolonged action and behaviours. With anxiety in the mix, this person is then likely to over-worry, which in effect further raises the threat value and heighten the responses even more. And so it goes on.

Rarely are the conditions explained adequately to patients, and certainly knowledge of the link between the seemingly different problems has never been volunteered to me by a patient. Therein lies a problem that the individual is suffering one or more pains and other symptoms (e.g./ tiredness, poor concentration, disrupted sleep, lethargy, flu-like symptoms, brain fog), yet they have no understanding as to why, or how it comes on, or what they can and must do to change the situation and move forward. Explaining the condition(s), the links, what the patient needs to do and what we can do to help and support them over a period of time that we can estimate is a key start point.

Further to the common biology, we can observe in the clinic the posturing, movements, guarding, poor body sense, altered sensorimotor function and the overall manifestation of how that person is feeling through body language and the words they use. We can gather far more information about the person, the whole person, by talking to them, listening to them and their concerns. What is their lived experience? The structured interview does not allow for this conversation. Yes we need some specific questions, but creating an open environment gives the person a chance to talk, feel heard and validated. This sets the scene for specific training, techniques and strategies that need to be used throughout the day and the development of understanding, all of which are the knowledge and skill base that the patient needs to overcome their pain.

No matter how long you have had pain, it can and does change. We are designed to change, and this is happening all the time. We are on a continuum, and we can have a say in where we go. It is a challenge and requires dedication, motivation, resilience and practice, but with the right thinking, action and support, great things can be achieved. I am honoured to see this happen in the clinic every week as people overcome their pain and resume being who they think they should be.

If you are suffering or think that you could be suffering with functional pain syndromes, call me for a chat and we can decide what you need to do to start overcoming your pain: 07518 445493

Clinics in Harley Street, Chelsea and New Malden Diagnostic Centre

22Oct/14

Girls, stress and pain

I have seen a number of teenage girls over the past year who are affected by chronic pain. They are often referred because of recurring headaches or migraines but we discover that there is widespread sensitive at play. How does this happen? Why does it happen?

Headaches and migraines can be functional pains. When these pains are part of a picture of sensitivity, often accompanied by anxiety, there are often other problems such as irritable bowel syndrome, pelvic pain and jaw pain. Whilst these problems all appear to be different, they have a common biology. Typically I work with women aged between 30 and 55 who suffer these aches and pains, but increasingly this is an issue of the younger female. Having said that, when I explore the story of an adult, we often find reasons for sensitivity that begin in childhood. This priming sets the scene for later events.

As adults we face many challenges. We have body systems that are trigged by these challenges, especially if we think they are threatening to us. In particular the autonomic nervous system (ANS) is quite brilliant at preparing us to fight or run away, which is very useful…..if you are facing a wild animal. On a day to day basis, it is in fact useful for the ANS to kick in and create some feelings in the body that alert us to danger — the caveat being, nothing is dangerous until it is interpreted as so, and hence we need a construct of ‘danger’ and of the thing that is perceived to be dangerous. For example, a baby may not have the construct of a lion and hence sees this big, cuddly, moving….thingy…like my teddy (may not have a construct for any of these either!), and essentially detects no threat. As the baby detects no threat, he or she behaves in a way that may not threaten the lion and hence the lion may feel safe. Both feeling safe, they become friends. Perhaps — these things have happened apparently. Please do not try this at home, but hopefully you get the idea. Back to day to day….

In the modern world we often feel anxious. This is the body warning us that something is threatening. In many cases that I see, there is a strong reaction to banal events and non-threatening cues. Or if the cue is worthy of attention, the response is well out of proportion — e.g. utter panic and defensive thinking-behaviours. To what do we respond most frequently? Definitely not lions. Muggers? Gunmen? Earthquakes? Tidal waves? These are all inherently dangerous situations, that we simply do not often face. Sadly some people do have such encounters but the majority of us do not. The answer is our own thinking. The thoughts that are evoked — seemingly appearing form nowhere at times — are not the actual problem but instead the interpretation of the thought (metacognotion; our thinking about our thinking). The meaning that we give to a thought, often automatically, will determine the body response as our thoughts are embodied. And just to complicate things further in relation to thinking, there’s a world of difference between the experiencing-self and the memory-self. The former refers to what is happening right now, the latter to what we remember, or think we remember. In terms of pain, if our memory of a painful event concludes with a high level of pain, this will flavour the memory-self and we will report as such. The story, which is a snapshot within our lives, and how it turns out has a huge impact upon the subsequent memory of what happened.

The adult within an environment that becomes threatening, the workplace for example, can become very responsive to different cues that once were innocuous. Now they pose a potential danger and each time that happens and we respond with protective thinking and behaviours, the relationship becomes stronger — conditioning. There is no reason any this cannot be the same for younger people who are consistently within an environment and context that begins to pose a threat; a demanding school environment with high expectations plus the child’s own expectations and perfectionist traits. Place this context within a changing period of life and minimal time for rest and there is the risk of burn out or development of problems that involve many body systems. We cannot, no matter what age we are, continue to work at a level that is all about survival.

I focus on girls and women because females outnumber the males coming to the clinic. Many are perfectionist, many are hypermobile, many are anxious, many are in pain and many are suffering. This is a situation that needs addressing worldwide, and starts with understanding what is happening, why it is happens and how it happens. Over the past 10 years this understanding has evolved enormously, providing tangible ways forward. This does not mean that we need to change perfectionism, but rather recognise it and use it wisely; this does not mean that anxiety is abnormal, but rather recognise it as a normal emotion that motivates learning and action; this does not mean that feeling pain is a problem to fear, but rather know it can change when we take the right action; and it does not mean that we will not suffer, but rather accept that part of living involves suffering that we can overcome and move on.

We have created an incredible, fast moving world. The body does not work at such a pace. It needs time to refresh and renew so that we can think with clarity and perform to a high level, achieve and be successful. We are humans. We are a whole-person with no division between body and mind; instead one thinking, feeling, sensing, creating, moving and living entity responding to the experience of the now and to memory of what we think happened. Gaining control over this with understanding and awareness provides a route forward to wellbeing, no matter where the start point.

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If you are suffering with persisting pains — body pain, joint pain, irritable bowel syndrome (IBS), headache, migraine, pelvic pain, jaw pain + feeling anxious, unwell, tired — call now and start moving forward 07518 445493 | Clinics in Harley Street, Chelsea and New Malden

04Mar/14

Ehlers-Danlos Syndrome (EDS) – a personal experience

Huge thanks to Kayleigh (Follow Kayleigh on twitter @KsEDSchallenge) for sharing her experience of Ehlers-Danlos Syndrome (EDS). The way that clinicians and the public truly understand conditions is through the personal stories that are described first hand. The narrative is key.

I was diagnosed with EDS in late 2013 (aged 25), having displayed symptoms my whole life – more noticeably from the age of 19 when my right knee became unstable and I began to notice symptoms such as mennorhagia, recurrent urinary infections and shortness of breath. Then from the age of around 23 when seemingly overnight lots of my symptoms became pronounced, leading to an eventual diagnosis.

I have seen 3 different physiotherapists during that 4 year period, and every one of them commented on my flexibility and pointed out things such as my tight hamstrings, stiff upper back and neck muscles, which I now understand are the result of my muscles trying desperately to stabilise my joints.

It’s reassuring that the physiotherapists picked up on this hypermobility, but at this stage it would have been so helpful if they had suggested even in passing that I might want to visit my GP if I had ever had any other symptoms such as the ones I had experienced, and had visited GPs for, but which had never been linked to each other. This could have lead to EDS being diagnosed 6 years before it was, and 3 years before I suddenly worsened. I would encourage physiotherapists to suggest this where they feel it could be appropriate. I have read several statistics suggesting that anywhere from 70 – 80% of people with EDS (who do not know of any family predisposition from a young age) do not get diagnosed until they are in their mid to late 20s, by which point little can be done to reverse the damage already sustained.

Now that I have been diagnosed, my physiotherapist plays a central part in the ongoing management of my condition as I choose to manage my condition without the use of NSAIDs, muscle relaxants or analgesics as much as possible.

There are several reasons that I choose to engage more intensely in physical therapies than drug based relief. The overriding reason is the damage drugs will do to my organs. I already suffer with kidney infections, GERD, costochondritis and digestive issues. I’m quite sure none of these will be improved by long term usage.

I also feel that I cope very well with my day to day pain levels. Before I was even aware that I had EDS, necessity had forced me to develop coping strategies for day to day life. I didn’t even realise until I was diagnosed that the majority of things I do, I do in some adapted way to mitigate against pain. If I were to use painkillers for a prolonged period, and then stop, I’m sure the ‘background’ pain I don’t even notice would become debilitatingly noticeable.

An equally important reason that I choose to engage heavily in physical based therapies is prevention. We all have to accept that there is no cure for EDS. But I fully intend to work until I reach retirement age, to have an active lifestyle and to have a family. To give myself the best possible chance of enjoying all of these things, I know I must strengthen my body and learn safe habits.

I have realistic expectations of my physiotherapist and what they will be able to achieve. But physiotherapists do not always have realistic expectations of me. I am not, for example, going to perform a single leg squat.

If you ask me to perform that quad stretch where you hold your foot against your backside.. remember that you need to consider – is my straight knee hyperextended, is the ankle I am holding in my hand bent at an unusual angle, is the elbow of the arm holding that foot straight and hyperextended, am I touching the wall with my fingertips for balance and bending the joints backwards… you can probably also assume that my elbow and wrist subluxed with the weight of my foot/leg.

What may seem like a simple stretch, for me is a whole host of things that could be going wrong. My concept of ‘normal’ is non existent. You will need to point these little possible dangers out to me, or I will be going home and doing damage to myself without realising it.

By pointing those things out to me, and by obviously knowing your stuff when it comes to EDS, you will gain my trust.

I would say the key to successful physiotherapy with EDS is to work with your patient to improve proprioception. I do not receive correct messages back from my body to my brain, so if you tell me to lay with my spine perfectly aligned I will probably think that I am doing it when really one hip has rolled forward or my shoulder has rolled backward. I will often perform exercises incorrectly once at home as a result.

I am currently working with a therapist to improve my proprioception in a number of ways. One of those ways is that I am learning to write with my left hand (being right handed), this will open new neural pathways and should help with my overall balance and co-ordination. It will also serve the dual purpose of allowing me to swap between hands when taking notes and using a computer mouse at work, to relieve the stress on my right arm. These kinds of techniques are great as they show measurable progress, where often with physiotherapy exercises I wonder if I am even doing anything to my body – I can’t feel the stretch in the stretches, so I just have to take it on faith that they are doing any good!

What I really appreciate is a physiotherapist who will go the extra mile. There are several things you can do to show that you understand EDS, that you are taking us seriously, and that you care. These are three things that we have come to expect not to receive from people, which we find a source of frustration and upset. We have been labelled as clumsy, melodramatic, hypochondriacs our whole lives. You may be trying to reassure us by downplaying an issue, but we will just think you are doing the same thing  our families, friends and doctors have in the past and will become defensive and distrustful.

Little things mean a lot. EDS sufferers are ALWAYS cold – so ask if the temperature is OK for us if we are covered in goosebumps. A lot of us have PoTS so don’t ask us to ‘jump’ off the table, advise us to get up slowly and be nearby ready to catch us if we go dizzy or a knee collapses as we stand. Learn about EDS and think about what you can do to demonstrate that you are not ‘just another medical professional’ and we are not ‘just another syndrome’.

These things I hope will help to build what is the most crucial part of managing any chronic condition – a positive, trusting relationship between practitioner and patient. That is all any of us, on both sides of the table, are asking for!

If you would like to share your experience, please contact me [email protected]

Ehlers-Danlos Support UK

09Jan/14

PoTS — Postural tachycardia syndrome

Postural Tachycardia Syndrome

The autonomic dysfunction that manifests as PoTS is poorly understood yet can cause alarming and distressing symptoms that hugely impact upon an individual’s quality of life. The experience of PoTS includes dizziness, palpitations and syncope, triggered by a range of stimuli such as heat, exertion, food ingestion and others. The variable nature and the plethora of symptoms that can be subtle on occasion, extending to the more overt collapse, can mean that day to day living becomes extremely challenging. The shift in the sense of self with PoTS as with other conditions, defines the suffering that the individual bears.

PoTS is often associated with hypermobility, joint hypermobility syndrome (JHS) and Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT). Many patients who visit the clinic with chronic pain and functional pain syndromes will demonstrate hypermobility on moving — lumbar spine, knees, elbows, thumbs, little fingers. It makes sense then, to be vigilant for symptoms of PoTS and dysautonomia.

For further information about PoTS, visit the PoTS UK website

There are several very good reviews of PoTS:

Postural tachycardia syndrome–current experience and concepts –Mathias CJ, Low DA, Iodice V, Owens AP, Kirbis M, Grahame R.

Abstract
Postural tachycardia syndrome (PoTS) is a poorly understood but important cause of orthostatic intolerance resulting from cardiovascular autonomic dysfunction. PoTS is distinct from the syndromes of autonomic failure usually associated with orthostatic hypotension, such as pure autonomic failure and multiple system atrophy. Individuals affected by PoTS are mainly young (aged between 15 years and 40 years) and predominantly female. The symptoms–palpitations, dizziness and occasionally syncope–mainly occur when the patient is standing upright, and are often relieved by sitting or lying flat. Common stimuli in daily life, such as modest exertion, food ingestion and heat, are now recognized to be capable of exacerbating the symptoms. Onset of the syndrome can be linked to infection, trauma, surgery or stress. PoTS can be associated with various other disorders; in particular, joint hypermobility syndrome (also known as Ehlers-Danlos syndrome hypermobility type, formerly termed Ehlers-Danlos syndrome type III). This Review describes the characteristics and neuroepidemiology of PoTS, and outlines possible pathophysiological mechanisms of this syndrome, as well as current and investigational treatments.

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Postural tachycardia syndrome: a heterogeneous and multifactorial disorder — full article free here
Benarroch EE.

Abstract
Postural tachycardia syndrome (POTS) is defined by a heart rate increment of 30 beats/min or more within 10 minutes of standing or head-up tilt in the absence of orthostatic hypotension; the standing heart rate is often 120 beats/min or higher. POTS manifests with symptoms of cerebral hypoperfusion and excessive sympathoexcitation. The pathophysiology of POTS is heterogeneous and includes impaired sympathetically mediated vasoconstriction, excessive sympathetic drive, volume dysregulation, and deconditioning. POTS is frequently included in the differential diagnosis of chronic unexplained symptoms, such as inappropriate sinus tachycardia, chronic fatigue, chronic dizziness, or unexplained spells in otherwise healthy young individuals. Many patients with POTS also report symptoms not attributable to orthostatic intolerance, including those of functional gastrointestinal or bladder disorders, chronic headache, fibromyalgia, and sleep disturbances. In many of these cases, cognitive and behavioral factors, somatic hypervigilance associated with anxiety, depression, and behavioral amplification contribute to symptom chronicity. The aims of evaluation in patients with POTS are to exclude cardiac causes of inappropriate tachycardia; elucidate, if possible, the most likely pathophysiologic basis of postural intolerance; assess for the presence of treatable autonomic neuropathies; exclude endocrine causes of a hyperadrenergic state; evaluate for cardiovascular deconditioning; and determine the contribution of emotional and behavioral factors to the patient’s symptoms. Management of POTS includes avoidance of precipitating factors, volume expansion, physical countermaneuvers, exercise training, pharmacotherapy (fludrocortisone, midodrine, β-blockers, and/or pyridostigmine), and behavioral-cognitive therapy. A literature search of PubMed for articles published from January 1, 1990, to June 15, 2012, was performed using the following terms (or combination of terms): POTS; postural tachycardia syndrome, orthostatic; orthostatic; syncope; sympathetic; baroreceptors; vestibulosympathetic; hypovolemia; visceral pain; chronic fatigue; deconditioning; headache; Chiari malformation; Ehlers-Danlos; emotion; amygdala; insula; anterior cingulate; periaqueductal gray; fludrocortisone; midodrine; propranolol; β-adrenergic; and pyridostigmine. Studies were limited to those published in English. Other articles were identified from bibliographies of the retrieved articles.

Visit our Hypermobility Clinic page here or call 07932 689081 to book an appointment

08Jan/14

We must think more widely on hypermobility and joint hypermobility syndrome

Commonly when a patient presents with hypermobility or joint hypermobility syndrome (JHS) there are a range of other problems that are painful or at least involve some sensitivity. This can include headaches, migraines, pelvic pain, chronic and widespread musculoskeletal pain, irritable bowel syndrome (IBS), fibromyalgia and fatigue. The underpinning sensitisation is the thread that runs through all of these conditions and can be targeted with a range of strategies. These functional pain syndromes are increasingly a problem, affecting large numbers of people, more women than men and at the seat of a great deal of suffering. This does not need to be the case.

Comprehensively looking at an individual’s situation, allowing them to tell their story, reveals much of what we need to know and understand. The narrative is key, providing all the clues that can be confirmed in the main by a simple examination. Of course in some cases further investigations are required, such as an MRI, blood tests or an x-ray.

Once the problems have been identified by listening and observing — much like good detective work! — then a programme can be designed to target the pain sources (mechanisms) and the influences upon these sources such as anxiety, stress, fatigue, negative thought processes to name but a few.

This recent paper seeks to raise awareness of hypermobility – Ehlers-Danlos Syndrome (EDS) –  Joint hypermobility Syndrome (JHS), much as I do via these writings and talking to others in the profession. There are the more notable features of these conditions but then there are the factors that impact, the suffering caused, the limitations, the expectations and beliefs of the patient and the range of other painful presentations that are in fact part of the same picture, and must be addressed as such. The mission continues…

See our hypermobility clinic page here — or call us to start your treatment and training programme: 07932 689081 — we are always delighted to chat.

Am J Med Genet A. 2013 Dec;161(12):2989-3004. 

Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

Castori M, Morlino S, Celletti C, Ghibellini G, Bruschini M, Grammatico P, Blundo C, Camerota F.

Abstract
Joint hypermobility syndrome (JHS) and Ehlers-Danlos syndrome, hypermobility type (EDS-HT) are two clinically overlapping connective tissue disorders characterized by chronic/recurrent pain, joint instability complications, and minor skin changes. Fatigue and headache are also common, although are not yet considered diagnostic criteria. JHS/EDS-HT is a unexpectedly common condition that remains underdiagnosed by most clinicians and pain specialists. This results in interventions limited to symptomatic and non-satisfactory treatments, lacking reasonable pathophysiologic rationale. In this manuscript the fragmented knowledge on pain, fatigue, and headache in JHS/EDS is presented with review of the available published information and a description of the clinical course by symptoms, on the basis of authors’ experience. Pathogenic mechanisms are suggested through comparisons with other functional somatic syndromes (e.g., chronic fatigue syndrome, fibromyalgia, and functional gastrointestinal disorders). The re-writing of the natural history of JHS/EDS-HT is aimed to raise awareness among clinical geneticists and specialists treating chronic pain conditions about pain and other complications of JHS/EDS-HT. Symptoms’ clustering by disease stage is proposed to investigate both the molecular causes and the symptoms management of JHS/EDS-HT in future studies.

07Jan/14

Lesser known problems associated with Ehlers-Danlos Syndrome and Loeys-Dietz Syndrome

Here is a selection of reports and studies that discuss lesser known features of Ehlers-Danlos Syndrome (EDS):

Unexpected association between joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type and obsessive-compulsive personality disorder.

Rheumatol Int. 2013 Nov 23. — Pasquini M, Celletti C, Berardelli I, Roselli V, Mastroeni S, Castori M, Biondi M, Camerota F.

Joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT) is a largely unrecognized, heritable connective tissue disorder, mainly characterized by joint instability complications, widespread musculoskeletal pain, and minor skin features. In a case-control study, 47 consecutive JHS/EDS-HT patients were investigated for the prevalence of psychiatric disorders and compared to 45 healthy controls in a single center. The psychiatric evaluation consisted of structured clinical interview for DSM-IV criteria by using the SCID-I and the SCID-II. Symptom severity was assessed using the Hamilton Anxiety Rating Scale (HAM-A), the Hamilton Depression Rating Scale (HAM-D), and the Brief Psychiatric Rating Scale (BPRS). The Global Assessment of Functioning Scale (GAF) was used to assess the overall severity of psychological, social, and occupational functions. JHS/EDS-HT patients had significantly higher mean scores for all questionnaires: HAM-A (6.7 vs. 3.8), HAM-D (6.4 vs. 2.7), GAF (75.0 vs. 86.1), and BPRS (27.5 vs. 25.6). The JHS/EDS-HT group had a 4.3 higher risk of being affected by any psychiatric disorder, and in particular, a 5.8 higher risk of having a personality disorder. In particular, 5 JHS/EDS-HT suffered from obsessive-compulsive personality disorder with an observed prevalence rate of 10.6 % (3.6-23.1). Psychiatric assessment of JHS/EDS-HT patients showed an extremely high prevalence of personality disorders (21 %), and of Axis-I disorders (38 %), mostly depressive. This study did not confirm the previously reported increased rate of panic disorders in JHS/EDS-HT.

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Ocular features in joint hypermobility syndrome/ehlers-danlos syndrome hypermobility type: a clinical and in vivo confocal microscopy study.

Am J Ophthalmol. 2012 Sep;154(3):593-600.e1. — Gharbiya M, Moramarco A, Castori M, Parisi F, Celletti C, Marenco M, Mariani I, Grammatico P, Camerota F.

To investigate ocular anomalies in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT).
DESIGN: Prospective, cross-sectional study.
METHODS: Forty-four eyes of 22 consecutive patients with an established diagnosis of JHS/EDS-HT and 44 eyes of 22 age- and gender-matched control subjects. Administration of a standardized questionnaire (Ocular Surface Disease Index) and a complete ophthalmologic examination, including assessment of best-corrected visual acuity, slit-lamp biomicroscopy, intraocular pressure measurement, indirect ophthalmoscopy, tear-film break-up time, Schirmer I testing, axial length and anterior chamber depth measurement, corneal topography, corneal pachymetry, and confocal microscopy. Main outcome measures included comparing ocular anomalies in JHS/EDS-HT and control eyes.
RESULTS: JHS/EDS-HT patients reported dry eye symptoms more commonly than controls (P < .0001). Scores of tear-film break-up time and Schirmer I test were significantly lower in JHS/EDS-HT eyes (P < .0001). Minor lens opacities were significantly more common in the JHS/EDS-HT group (13.6%; P < .05). Pathologic myopia with abnormal vitreous was found in 7 JHS/EDS-HT eyes (15.9%) and 0 controls (P = .01). Corneas were significantly steeper and the best-fit sphere index was significantly higher in JHS/EDS-HT group (P < .01). By confocal microscopy, the JHS/EDS-HT group showed lower density of cells in the superficial epithelium (P < .001) and higher density of stromal keratocytes in anterior and posterior stroma (P < .0001).
CONCLUSIONS: The most consistent association of eye anomalies in the JHS/EDS-HT group included xerophthalmia, steeper corneas, pathologic myopia, and vitreous abnormalities, as well as a higher rate of minor lens opacities. These findings indicate the need for ophthalmologic survey in the assessment and management of patients with JHS/EDS-HT.

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The cornea in classic type ehlers-danlos syndrome: macro- and microstructural changes.

Invest Ophthalmol Vis Sci. 2013 Dec 11;54(13):8062-8. — Villani E, Garoli E, Bassotti A, Magnani F, Tresoldi L, Nucci P, Ratiglia R.

To analyze in vivo corneal morphology and ultrastructural features in patients with classic Ehlers-Danlos syndrome (EDS).
METHODS: Fifty patients with classic EDS and 50 age- and sex-matched control subjects were studied. A clinical evaluation was made with the Ocular Surface Disease Index (OSDI) questionnaire and a complete ophthalmic examination, including assessment of the best-corrected visual acuity and refraction, slit-lamp biomicroscopy, tear break-up time, intraocular pressure, Schirmer test without topical anesthesia, and corneal diameter. Scheimpflug camera topography and in vivo confocal microscopy (IVCM) were used to investigate corneal morphology and corneal ultrastructural features respectively.
RESULTS: Classic EDS patients, compared to controls, had thinner and steeper corneas (P < 0.001 and P < 0.05, respectively; independent samples t-test). IVCM showed thinner stromas, lower keratocyte densities (P < 0.001), increased applanation-related stromal folds (P < 0.001; Mann-Whitney U test), and increased endothelial hyperreflective dots (P < 0.05) in these patients. The study group also had increased symptoms (OSDI score: P < 0.01, independent samples t-test) and signs (tear break-up time and Schirmer test: P < 0.001 and P < 0.05, respectively) of tear film dysfunction.
CONCLUSIONS: Patients with classic EDS had macro- and microstructural changes of the cornea, which is a target tissue of the disease. These findings should be considered to optimize clinical management of these patients and to evaluate the opportunity of adding ocular findings to the classic EDS diagnostic criteria.

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Neurologic manifestations of inherited disorders of connective tissue.

Handb Clin Neurol. 2014;119:565-76. — Debette S, Germain DP.

Inherited disorders of connective tissue are single gene disorders affecting structure or function of the connective tissue. Neurological manifestations are classic and potentially severe complications of many such disorders. The most common neurological manifestations are cerebrovascular. Ischemic stroke is a classic complication of vascular Ehlers-Danlos syndrome (type IV), homocystinuria, and arterial tortuosity syndrome, and may occasionally be seen in Marfan syndrome and pseudoxanthoma elasticum with distinct underlying mechanisms for each disease. Vascular Ehlers-Danlos syndrome can also lead to cervical artery dissection (with or without ischemic stroke), carotid-cavernous fistula, intracranial dissections and aneurysms potentially causing subarachnoid or intracerebral hemorrhage, and arterial rupture. Other neurological manifestations include nerve root compression and intracranial hypotension due to dural ectasia in Marfan and Loeys-Dietz syndrome, spinal cord compression in osteogenesis imperfecta, and mucopolysaccharidosis type I and VI, carpal tunnel syndrome in mucopolysaccharidosis type I, II, and VI. Impaired mental development can be observed in homocystinuria, mucopolysaccharidosis type II, and the severe form of mucopolysaccharidosis type I. For the neurologist, being aware of these complications and of the diagnostic criteria for inherited connective tissue disorders is important since neurological complications can be the first manifestation of the disease and because caution may be warranted for the management of these patients.

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Pediatr Radiol. 2011 Dec;41(12):1495-504;

Loeys-Dietz syndrome: cardiovascular, neuroradiological and musculoskeletal imaging findings.

Kalra VB, Gilbert JW, Malhotra A.

Loeys-Dietz syndrome (LDS) is an increasingly recognized autosomal-dominant connective tissue disorder with distinctive radiological manifestations, including arterial tortuosity/aneurysms, craniofacial malformations and skeletal abnormalities. LDS exhibits a more aggressive course than similar disorders, such as Marfan or the vascular subtype of Ehlers-Danlos syndrome, with morbidity and mortality typically resulting from complications of aortic/arterial dissections. Early diagnosis, short-interval follow-up imaging and prophylactic surgical intervention are essential in preventing catastrophic cardiovascular complications. This review focuses on the cardiovascular, neuroradiological and musculoskeletal imaging findings in this disorder and recommendations for follow-up imaging.

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J Bone Joint Surg Am. 2010 Aug 4;92(9):1876-83.

Musculoskeletal findings of Loeys-Dietz syndrome.

Erkula G, Sponseller PD, Paulsen LC, Oswald GL, Loeys BL, Dietz HC.

BACKGROUND: Loeys-Dietz syndrome is a recently recognized multisystemic disorder caused by mutations in the genes encoding the transforming growth factor-beta receptor. It is characterized by aggressive aneurysm formation and vascular tortuosity. We report the musculoskeletal demographic, clinical, and imaging findings of this syndrome to aid in its diagnosis and treatment.
METHODS: We retrospectively analyzed the demographic, clinical, and imaging data of sixty-five patients with Loeys-Dietz syndrome seen at one institution from May 2007 through December 2008.
RESULTS: The patients had a mean age of twenty-one years, and thirty-six of the sixty-five patients were less than eighteen years old. Previous diagnoses for these patients included Marfan syndrome (sixteen patients) and Ehlers-Danlos syndrome (two patients). Spinal and foot abnormalities were the most clinically important skeletal findings. Eleven patients had talipes equinovarus, and nineteen patients had cervical anomalies and instability. Thirty patients had scoliosis (mean Cobb angle [and standard deviation], 30 degrees +/- 18 degrees ). Two patients had spondylolisthesis, and twenty-two of thirty-three who had computed tomography scans had dural ectasia. Thirty-five patients had pectus excavatum, and eight had pectus carinatum. Combined thumb and wrist signs were present in approximately one-fourth of the patients. Acetabular protrusion was present in approximately one-third of the patients and was usually mild. Fourteen patients had previous orthopaedic procedures, including scoliosis surgery, cervical stabilization, clubfoot correction, and hip arthroplasty. Features of Loeys-Dietz syndrome that are important clues to aid in making this diagnosis include bifid broad uvulas, hypertelorism, substantial joint laxity, and translucent skin.
CONCLUSIONS: Patients with Loeys-Dietz syndrome commonly present to the orthopaedic surgeon with cervical malformations, spinal and foot deformities, and findings in the craniofacial and cutaneous systems.

07Jan/14

Hypermobility – Ehlers Danlos Syndrome and gastrointestinal problems

Hypermobility is common and is certainly a feature that we often see in patients at the clinics. Some patients have been diagnosed with hypermobility but do not know what it really means and need clarification, some are suffering aches and pains that are limiting and troublesome and still others visit with chronic pain and hypermobility is seen at the assessment.

See hypermobility blogs — blog 1 — blog 2 — blog 3

I always begin with an explanation that includes pointing out that many top athletes are hypermobile and hence there can be advantages. Per se, hypermobility is not necessarily a problem and in fact many who come for advice do not have any significant issues. They may need a programme that includes spatial awareness training, balance and proprioceptive exercises, but in essence, they can continue as normal.

Those patients who suffer pain and on-going pain, often widespread, require a different approach that considers the pain source and the influences upon pain. The training will include proprioception and spatial awareness exercises, but the baseline start point will be different. Before this even, there is often a need to tackle the sensitivity in several ways, termed top-down and bottom-up. Top-down refers to how we can target the brain including education, strategies to deal with thoughts that create anxiety and adaptations to the body maps that change our body sense and experience. Bottom-up is the use of the body tissues to change sensory processing and hence pain and sensitivity. There are many ways of doing this, and altering the combinations of the top-down and bottom-up  strategies creates potent ways of tackling pain.

Frequently, those who suffer persisting musculoskeletal pain will also bare pain through other body systems, especially the gut. See this recent review:

Functional digestive symptoms and quality of life in patients with ehlers-danlos syndromes: results of a national cohort study on 134 patients. Zeitoun JD et al.

Abstract

BACKGROUND AND OBJECTIVES:

Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders. Gastrointestinal manifestations in EDS have been described but their frequency, nature and impact are poorly known. We aimed to assess digestive features in a national cohort of EDS patients.

METHODS:

A questionnaire has been sent to 212 EDS patients through the French patient support group, all of which had been formally diagnosed according to the Villefranche criteria. The questionnaire included questions about digestive functional symptoms, the GIQLI (Gastrointestinal Quality of Life Index), KESS scoring system and the Rome III criteria.

RESULTS:

Overall, 135 patients (64% response rate) completed the questionnaire and 134 were analyzable (123 women; 91%). Mean age and Body Mass Index were respectively 35±14.7 years and 24.3±6.1 kg/m(2). The most common EDS subtype was hypermobility form (n=108; 80.6%). GIQLI and KESS median values were respectively 63.5 (27-117) and 19 [13.5-22]. Eighty four percent of patients had functional bowel disorders (FBD) according to the Rome III criteria. An irritable bowel syndrome according to the same criteria was observed in 64 patients (48%) and 48 patients (36%) reported functional constipation. A gastro-esophageal reflux disease (GERD) was reported in 90 patients (68.7%), significantly associated with a poorer GIQLI (60.5±16.8 versus 75.9±20.3; p<0.0001). GIQLI was also negatively impacted by the presence of an irritable bowel syndrome or functional constipation (p=0.007). There was a significant correlation between FBD and GERD.

CONCLUSIONS:

Natural frequency of gastrointestinal manifestations in EDS seems higher than previously assessed. FBD and GERD are very common in our study population, the largest ever published until now. Their impact is herein shown to be important. A systematic clinical assessment of digestive features should be recommended in EDS

It is routine in our clinic to ask about other body systems as this tells us a great deal about the level and type of sensitivity, which in turn guides the comprehensive treatment and training programme.

Increasingly, patients are being referred for irritable bowel syndrome and other functional pains (e.g./ migraine, headache, chronic back pain, chronic joint pain, pelvic pain, vulvodynia). Due to the underpinning sensitivity residing within the central nervous system — this is not a disease but rather an adaptation; neuroplasticity at play — we can target these mechanisms with a range of effective strategies to re-learn or re-programme the way in which the neuroimmune system is expressing itself. These systems are fundamentally designed to change, learn and grow. They simply need the right conditions to do so, and all too often there is a belief that a situation cannot change and hence all the choices and behaviours prevent any form of forward movement. This is just not true and through our understanding of the body systems and their adaptability, we are creating increasingly effective and diverse ways of tackling pain and suffering.

If you have been diagnosed with hypermobility or are suffering with chronic pain, call us now to discover how you can change your experience and move on: 07518 445493 — Specialist Pain Physio Clinics in London: Hypermobility Clinics

10Jul/13

Women and Pain Clinic @ 132 Harley Street

The Women and Pain clinic is dedicated to providing contemporary treatment, training and coaching for females who suffer persisting pain.

Common examples of on-going painful problems include:

  • pelvic pain: including pain from endometriosis, bladder problems, muscular spasm & guarding of the pelvis and abdominal area
  • back pain
  • joint pain (often multiple)
  • abdominal pain (irritable bowel syndrome or similar sensitivities)
  • migraine & headache
  • jaw pain & dysfunction
  • fibromyalgia

It is not uncommon for there to be several painful areas that are seemingly unrelated. However, with the advancing understanding of the neurobiology of pain, we know that there is a common thread that ties these problems together. This is termed central sensitisation and refers to adaptations within the nervous system that both amplify pain and reduce our natural ability to dampen sensitivity. The body areas that hurt can expand and involve a range of body systems, hence why the pain can manifest in different regions and organs. The pain is an expression of this underlying sensitivity that needs to be targeted at a nervous system-immune system-endocrine system level as well as addressing the health of the body tissues. We use a contemporary and neuroscience-based programme of treatment, training and coaching to tackle the problem of pain, focusing upon the inter-related dimensions of pain: physical-cognitive-emotional.

Working closely with leading gynaecologists and gastroenterologists in Harley Street, you will have a detailed assessment that includes diagnostics as indicated, a full explanation of the nature of the pain and symptoms (pain education) and a comprehensive treatment programme designed for you. 

How do we treat these problems?

A pathological or structural basis for pain only explains part of the problem or in some cases not at all. It is the adaptations within body systems that create the pain experience to which we respond in thought and action. Whilst acute pain serves a vital survival purpose, drawing our attention to a body region that needs protecting for healing, a persisting pain becomes increasingly about the neuroimmune system and endocrine system responses. Pain certainly emerges from the body yet there is an underpinning correlate of activity within a vast network of brain cells that actually drives the experience. This network monitors the activity in the body systems and responds according to need. The response can be protective when the brain perceives the body to be in potential danger and includes pain, changes in movement and a range of other actions. In the early stages of a condition this is useful and adaptive, however if these responses continue beyond a useful time they themselves must be targeted alongside body nourishing strategies.

How can we target these systems? In an integrated manner, these systems can be re-trained with a range of sensorimotor techniques, specific exercises designed to restore a normal sense of the body and movement, strategies to deal with stress and anxiety that both affect the body systems, techniques for the progression of day to day living (work, home, sports), and general activity and exercise with confidence. Interlaced with these strategies, pain education (reduce the threat by developing your understanding of pain and the body’s ability to change), mindfulness-based stress reduction, focused attention training, resilience, coping and motivational skills, are used to optimise outcomes that are based upon improving your quality of life, sense of wellness and performance.

For further information, to book an appointment or to refer a patient please call us on 07932 689081

06Jun/13

Hypermobility & anxiety

Hypermobility is a common obsevation in our clinics and is often associated with persisting pain, stress and anxiety. Increasingly neuroscience studies are linking body, brain and mind to highlight the fact that we must address all these dimensions within a contemporary treatment and training programme. In particular, we know that there is a strong association between those who are hypermobile (see here for the Brighton classification) and panic disorder – see here.

‘the most significant and important association between joint hypermobility syndrome (JHS) and any other disorder from a clinical point of view is with panic disorder’ Garcia-Campayo et al. (2011) – see here

Last year a study by Eccles et al. (2012) described hypermobility as a ‘multisystem phenotype’:

Br J Psychiatry. 2012 Jun;200(6):508-9.

Brain structure and joint hypermobility: relevance to the expression of psychiatric symptoms.

Eccles JA, Beacher FD, Gray MA, Jones CL, Minati L, Harrison NA, Critchley HD.

Abstract

Joint hypermobility is overrepresented among people with anxiety and can be associated with abnormal autonomic reactivity. We tested for associations between regional cerebral grey matter and hypermobility in 72 healthy volunteers using voxel-based morphometry of structural brain scans. Strikingly, bilateral amygdala volume distinguished those with from those without hypermobility. The hypermobility group scored higher for interoceptive sensitivity yet were not significantly more anxious. Our findings specifically link hypermobility to the structural integrity of a brain centre implicated in normal and abnormal emotions and physiological responses. Our observations endorse hypermobility as a multisystem phenotype and suggest potential mechanisms mediating clinical vulnerability to neuropsychiatric symptoms.

Tying together specialties with science is a vital step forward and one that we embrace at Specialist Pain Physio Clinics. In particular, joining the dots from neurology to rheumatology to psychiatry to gastroenterology to gynaecology is one of our main aims as there are underpinning changes in the neuroimmune system that present as a range of functional pains that are seemingly different complaints yet all feature central sensitisation. Therefore we can target the sources for more effective outcomes, pointing the individual’s compass towards wellness and life satisfaction.

Learn to deal with your anxiety with mindfulness based stress reduction, movement, exercise & positive emotion – Specialist Pain Physio treatment, training & coaching

People who are hypermobile who also decsribe a range of funtional pains (e.g. IBS, fibromyalgia, TMJ pain, migraine, pelvic pain) along with anxiety, panic and depression (not necessarily all of these problems) certainly require a comprehensive approach: for example, strategies to deal with the pain and symptoms, training to improve body awareness and normal movement, techniques to tackle negative thinking, anxiety and emotions as well as those that develop positive emotions and accomplishment, ways to enhance performance and ultimately to improve quality of life.

Contact us to make an appointment and see how you can move forward: 07932 689081