31 Dec 2016

How we can use the power of thought to recover from the body blows of 2016

2016 may not have actually seen more celebrity deaths but the point is, it felt like it. Those who died were largely beloved figures. By New Year's Eve, I was wondering whose turn it was today, December had slapped us around so much.
It's as if some malevolent force had picked off the people most likely to invoke public grief so a vast swathe of human emotional vibrations would resonate negatively across the world. And as well as the deaths, there was Brexit and then Trump, two of the most unexpected of events that if, two years ago you said would happen, no one would believe you.

The outrage felt by the respective losing sides was, and continues to be, so palpable that they almost have a physical form of their own. If the reverse results had come about, there would not have been such an outpouring of shock and indignation. Again, a wave of negative vibrations all around us.
If you think vibrations caused by human emotion isn't a 'thing', just remind yourself of the last time you walked into a room and 'felt' an atmosphere, be it exuberant, somber or hostile. The saying 'cut the air with a knife' isn't literal, but it's still an expression we all understand at a visceral level.

There were moments of joy, mainly sporting, such as Andy Murray's Wimbledon win (yay!) and his Olympic gold (double yay!) but even the Olympics were marred by Russian cheating and that's the impression that overshadows the memory of athletic glory.

Can we change things by feeling differently about them?

So how can we change things? There is a saying in the so-called New Age community that 'thoughts become things'. In fact, far from being airy-fairy nonsense, is absolutely accurate, in my opinion. To become a human-led event or a thing, first it must have been a thought in someone's head. That thought is then acted on whether positive or negative. This is the beginning of everything that we create. Then, if the thought takes hold in more people, the effect is amplified. Hence that 'atmosphere'.
The 'thoughts become things' mantra is both more complex and more simple than this explanation. It is that if we visualise a truly-held, resistance-free desire, it will come about. I'm no expert in either explaining or achieving this, but I would recommend the late Wayne Dyer, Abraham Hicks (the inspiration for and source of The Secret) and myriad others who can express it far better than I. You may want to start here, if you are interested and open to learning more.

So how can we change this feeling when all around us is pretty grim? It's a tough one, isn't it? Of course we're going to react to something happening, we're only human after all. Even an ancient tree's branches bend in a fierce wind. How can we withstand such extreme external forces and still be positive?
The answer is not an easy one, but it's something I have been learning as a parent of children with SEND, as a person with chronic pain, as someone who grew up feeling constantly misunderstood only to be recently diagnosed with autism. I learned that if you are determined enough, you can withstand any storm and come out more resilient and even smiling. I have learned that being angry at others, whoever they are, does not help if it uses all your energy and consumes you. The object of your ire is not hurt your anger; it is you who is further damaged by the real, physical and mental effects of stress. If you can, channel your anger into positive action to change what you don't like, but (dear Brexiteers) it's a good idea to actually have a some thoughts beyond just 'change' first!

The power of emotions

We all need to be more consciously and constantly self-aware of how our feelings affect us and those around us. Emotions are a powerful force and the growing understanding of this is at the root of why the pursuit of Mindfulness has become popular. It is the desire to no longer be a ship cast adrift and rudderless on the high seas, but to have self-management skills to get that vessel- yourself - under your own control and steer it to safety. Without adequate self-control and the ability to be in touch with ourselves, we cannot easily help anyone else, not least our children.

Emotions are also infectious. Who doesn't smile when they see a child laughing and full of joy? We are also able to feel the agony of a friend or loved one when they are in pain, as if the feeling is our own.
I'm not suggesting we smile the next time a celebrity- or anyone else- dies or that we should not react to personal tragedy or loss. Neither am I suggesting that we should be automatons and not feel anything at all. It is our ability to feel complex emotions that sets up apart from the rest of Earth's inhabitants (as far as we know)
But we should remember that we are not the branches of a tree, we are the trunk and we can stand firm and spread our roots deep so that not even the strongest hurricane can tear us down. We are the captain of our ship and we can set our own course regardless of where the masses are heading.
Because who benefits from us being paralysed by fear and sadness or transfixed by major events whether a disaster, terrorist attack or something as trivial as a TV show? Not us, that's for sure but those who have a vested interest in the status quo are quite happy when we are confused or distracted.

Plow your own furrow

I have been involved in the media industry all my adult life, so it may seem counter-intuitive of me to advise you not to let the media decide how you should feel about something. Actually, as someone who knows how the news is made, it should make perfect sense. If you're in any doubt, read this.
If you're not sure of the facts of a story, try Full Fact or, in the US, Snopes or the Center for Public Integrity.  You could trust your gut, but that depends on how partisan your gut is...

Do something to make a positive difference. You can't stop any war by yourself, but you can welcome a refugee into your community or make a donation in time or money to a disaster relief or child poverty or exploitation charity. You can't bring George Michael back, but you can celebrate his life and learn from the kindnesses we have now discovered he showed to others and do something similar ourselves, however small. We are not rudderless ships in a storm.

Every day, ask how our actions and emotions are affecting those around us. Ask if you've made a positive difference today, even a smile or a kind word. Be on the look out for opportunities until it becomes second nature. Even in the depths of despair we can do this. The world needs all of us to make a positive difference, however small you may think it is; it still counts. Most of all, we feel better in ourselves for having done it. Our thoughts brighten, our backs straighten despite our burden and we breathe a little easier.

As we move into 2017, battered from the year gone by, we must ask ourselves what kind of a world we want to live in. Then we can use those powerful, positive thoughts and vibrations of ours that we have created by thinking good thoughts and doing small, kind things, to foster a more collaborative community that cares about each other as if we were all one family. Because, in the end, if we are all to prosper, this is exactly how we must consider ourselves - as part of each other.

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23 Dec 2016

Building a website for your group or charity on a shoestring budget

I recently took part in a webinar aimed at helping rare disease patients who want to start a website for their patient group or small charity.
Almost half of the world’s population is online, with the number of users growing daily. People are increasingly turning to the Internet to answer all of their questions – and this includes health.
A 2013 study from the Pew Research Centre found that 72% of internet users have looked online for health information in the past year. For rare disease patients, dispersed around the world, the Internet can be their first port of call following a diagnosis.
It is crucial that patient groups are able to reach out to these patients, to provide them with accurate information and engage them in the group’s community. A simple way to do this is through a website.
It can be a confusing process when you're trying to choose where to host your site, how to structure it, construct it and what to put on it. Added to this, you're certain to want something that costs next to nothing to create and maintain.
Building a website for your group or charity on a shoestring budget

Tips can be used by anyone wanting to start a website

This webinar, held by Findacure Foundation, starts with a talk from Rob Stobo, a web copywriter about thinking about your content.
My presentation starts at 33" and the webinar concludes with questions from the participants. I talk about the options for starting a website with little knowledge, how much it may cost and tips for designing, building and publicising it.
Although it's aimed at patient groups, it's important to note that the tips are usable by anyone who wants to start their own low cost website. Do please share with anyone who might be interested.

To see my presentation by itself (no commentary), click here
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15 Nov 2016

Ehlers Danlos syndrome. Sometimes hard to see, always hard to live with

Son2 and I were both diagnosed with Ehlers Danlos syndrome by leading world expert in the condition, Professor Rodney Grahame. As well as being about as knowledgeable about the condition as it is possible to be, he's also kind, genial and still passionate about his field, despite being past the age at which many would be thinking of retirement.

Prof. Grahame, however, is still at the top of his field and has just published a new medical paper on EDS Hypermobility type, along with two other authors, Dr Yael Gazit, Dr Giris Jacob. It's entitled Ehlers–Danlos Syndrome—Hypermobility Type: A Much Neglected Multisystemic Disorder.

It's an important paper for EDS, I believe as it's just the kind of easy to understand information that can be presented to medical professionals to help them understand how the condition can affect so many different parts of the body at the same time. It's not "catastrophising" (don't you just hate that word?), it's not imagined, it's not a coincidence. If it contains collagen, as most things in the body do, it is likely to be affected to a collagen disorder. I'm not quite sure why this is a surprise to anyone.

Ehlers Danlos syndrome. Sometimes hard to see, always hard to love with

It's also the CQC's Invisible Conditions Week, so I thought it would be a perfect subject for a post here, my irregular counterpart to Special Needs Jungle which is for children and young people with disabilities.

Many types of Ehlers Danlos are extremely rare and can be fatal. EDS-HT, however is much less so, but because it is a connective tissue disorder, found all over the body, it is pervasive and can be extremely disabling, as I have found. While EDS can be invisible if you're able to walk about easily, many need to use crutches, splints or wheelchairs or perhaps rely on naso-gastric tubes for nutrition. Almost everyone with EDS is affected by fatigue.

Living with EDS is, at best, a bit crap. Work is left undone because I have to sleep. Days are lost to pain, bone-dragging, walking through-treacle fatigue or recovery from activity. Pain meds must never be forgotten or you get hit twice as hard. Attending events has to be carefully planned so I can rest before and after. It's a pain, living with chronic pain. 

The article describes EDS HT as a "complex hereditary disorder which is multi-systemic, probably due to the prevalence of connective tissue in all body systems. Its gene defect has yet to be found and might be of multi-genetic nature".  This basically means it is likely to be caused by more than one gene and so any cure is likely to be a loooong way off. Increasingly, suspicion is growing that it is also connected to autism because of the increasing number of people diagnosed with both conditions, including my boys and me.

Even more perplexing, the level of hypermobility a person has can have no correlation to the amount of pain they experience. As a child, I was bendy, but not double-jointed. I did, however, suffer unexplained, severe bouts of pain in different parts of my body that would come and go. Back pain has been an almost constant companion, even at one point, a slipped-disc that sparked back spasms, something I would say was actually worse than labour pain. I actually used to think living like this was normal because to me, it was. I have also always had regular dizzy spells, neck pain, jaw pain, intestinal pain and digestive difficulties, palpitations, was extremely short-sighted before I had Lasik, have life-long depression and anxiety, dysmenorrhea so severe it made me throw up and excessive adhesions as a result of abdominal surgeries. These are all manifestations of EDS that the paper cites as common to the condition. I never once wondered if they were connected. I just thought I was a general weakling. 

It's hard to believe it took until I was 45 to be diagnosed. Well, not really that surprising considering how many people out there are also late being diagnosed after similar experiences to mine. I had a successful career despite all those ailments. I just "got on with it"; what else was I supposed to do? My ambition was far superior to anything else at the time, anyway. 

I got married, had kids, though childbirth was extremely difficult, ending in caesarians. It wasn't until, after a particularly stressful, exhausting time when I'd gone back to working, pushing myself hard, that the regular aches exploded into an all-over conflagration of agony in 2012, that has never gone away.

invisible conditions

To look at me today, sitting in a conference or on a train, you wouldn't guess there was anything amiss. I actually look quite well. It's only when I stand and then perhaps sit down again when my blood drops to my feet and my heart starts to race, or you see the stick I use (for stopping, not for walking), that you notice anything different. 

The stick is a handy prop, really. It turns the invisible into the visible. The stick makes my life easier not just because it helps me stay upright when there is no person to hold onto, but because it signals to others that I have a disability. And it works, as I am usually offered seats, helped with heavy bags, offered directions to the lift instead of stairs and so on. The stick is like a magic wand that spurs kindness and humanity in strangers. The wheelchair, when I need it, even more so, but that also gives me a ring-side seat on how disability-unfriendly the built environment is. Something to write about another day.

Treatment for EDS is hard to come by. The paper recommends: 
...multidisciplinary co-operation and consulting with a cardiologist with echocardiogram monitoring every 2–5 years, orthopaedic surgeon with a follow-up once a year, oral and maxillofacial surgeon for temporomandibular joint involvement, gastroenterologist when gastrointestinal manifestations are present, ophthalmologist to rule out other connective tissue diseases and when ocular manifestations are present, urologist and urogynecologist when urologic manifestations are suspected, neurologist and neurosurgeon when prolonged headache is present to rule out Chiari 1, and psychiatry when anxiety and/or depression are suspected. Allergologic consultation may also be needed when there are multiple drug reactions and/or food allergies. An autonomic nervous system specialist should be consulted when signs and symptoms of POTS or other autonomic nervous system manifestations are present. Management includes physiotherapy and hydrotherapy aimed at symmetric and generalized muscle strengthening and proprioception acuity improvement, including deep connective tissue manipulations after each session, occupational therapy when wrists and fingers are involved, and cognitive behavioral therapy for proper adjustment to the chronic nature of the condition. Nutrition has an important role in treating EDS-HT, and nutritional deficiencies should be sought out and treated.

Wowzer, well that would be nice, wouldn't it? But the point of highlighting this paper is that people can print it out and take it along to their doctor. It's great that the Prof recommends this as a treatment standard, but it's another story when you're trying to persuade your local doctors to give it to you.

It also recommends hydrotherapy, which is one of the NHS's best kept secrets and when you find out about it, it only comes in blocks of six, which is when it's only just starting to show benefit for someone with a long-term condition. This really needs to be rethought, or at least the facilities could be rented out after hours, perhaps. 

This new papers' authors make an important recommendation in conclusion that until a gene or genes for EDS-HT have been identified, doctors must think about the possibility of the condition in every chronic pain patient and look for joint hypermobility, as well as the other multi-systemic manifestations the paper mentions. Do you think they will? Sadly, I doubt it. It's much easier  - and quicker - to send people (mainly women) having been treated for each symptom, as indeed I was for much of my life, than to consider looking at the patient as a whole. 

This is why charities such as The Ehlers Danlos Society exist, so that information about the different types of EDS can be publicised, patients empowered by knowing the symptoms and where to go for help and how to ensure they get it. And, most importantly, so that doctors can be educated in how to recognise, diagnose and treat it and be reminded that zebras can sometimes also be horses.  
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28 Aug 2016

Study indicates deconditioning doesn't cause PoTS, but has a cardiac trigger

Study indicates deconditioning doesn't cause PoTS, but has a cardiac trigger

A very small, yet enlightening medical commentary about Postural Orthostatic Tachycardia syndrome (PoTS) has recently popped up.

PoTS is often -erroneously- put down to the result of deconditioning - i.e, not doing enough exercise.
It's one of the things that people with the condition (including me) find most upsetting, the thought - sometimes spoken, more often just reflected in someone's face, that it's really all our own fault.

While PoTS can be as the result of many things, for example, a chronic pain condition such as Ehlers Danlos syndrome, its effects can be utterly disabling. It ends up as a vicious circle of not being able to exercise or even stand up through the symptoms of dizziness, fainting, chest pain or nausea (among others) and that makes it even harder to get up because you're weaker through lack of moving about. This is called 'exercise intolerance'.

This explanation may be given to patients in a dismissive way - sort of, 'You've let yourself down by being lazy so just try harder, get up and you'll get better'. But how do you do that when you can't stand up in the first place? And what was the trigger for those people whose PoTS came on suddenly, at the same time as the other illness (as in my case) or just by itself out of the blue? Deconditioning does not explain this.

However two doctors have written to the journal, Pulmonary Circulation, as a follow up to another study that sparked their attention. Doctors, Svetlana Blitshteyn, MD of Buffalo's Dysautonomia clinic and David Fries, MD of the Sands-Constellation Heart Institute in Rochester, both in New York State, analysed this study on low ventricular filling pressures as a cause of exercise intolerance and dyspnea. They believe it sheds some light on the causes of PoTS saying:

Previously, a low stroke volume and decreased cardiac mass in patients with POTS have been attributed hypothetically to deconditioning. This study provides the first objective evidence that low ventricular filling pressures in patients with POTS are contrary to what would be expected in deconditioned patients—high filling pressures.

The doctors commented that the study (although small) could be applicable to the majority of PoTs patients in that, "exercise intolerance in POTS is not caused by a lack of maximum effort from the patient but that low ventricular pressures occur despite the maximum effort."

If they're lucky, patients with an autonomic dysfunction such as PoTS have non-invasive (i.e, not surgical) tilt-table tests or other cardiac stress tests, ECGs and perhaps blood tests to diagnose them. Unless you can pay privately, in the UK these tests have incredibly long waiting lists on the NHS, during which time patients are becoming more ill. Many such as myself often need to use a wheelchair to even leave the house.

My Automonic Testing

My own story illustrates how difficult life with this condition can be and I do, in fact, consider myself lucky that I am not a young woman, just setting out on life. It also shows why compassion and understanding is far more required than telling someone they just need to pull their finger out and get on the exercise bike. I'm not saying that doing nothing is the right thing either, but a huge amount of support is needed, especially if you are young with little experience of life.

For me, the wait was 18 months before I got an initial appointment. Then, a further three months before I got the tilt-table tests. Then, incredibly, TEN months before I got the results of the tests. All during this time, life was very difficult. I could only walk short distances without having to stop and sit down or, if there was nowhere to sit, to bend down to get my head as low as possible.

Of course family life continued around me, with me feeling like I was less relevant, less able to participate and altogether more useless by the day. It was probably my own fault for not trying hard enough, of course. I tried dying, but I didn't even manage to try hard enough at that. What a slacker.

When I went back, I was given a trial of Ivabradine, which had a very positive effect. My GP however, didn't want to dispense it as it was off-label and the specialist letter hadn't yet arrived (and didn't for some time). After I become quite upset, my GP relented and made out the prescription. 

Although I was told by the Autonomic clinic I would get a follow up appointment in three months, I didn't get any letters until I received one discharging me, as I had apparently failed to attend! Like I would fail to attend after all that waiting! The letter had simply not arrived.

Despite explaining this to them and speaking to my GP and to my EDS specialist, they have not yet sent me another appointment and we are now more than a year after the results appointment. In the meantime, I have taken it on myself to double the dose as it appears no one else gives a stuff whether it's working or not, so I'll just do it my way.

The Ivabradine certainly isn't perfect - not by a long shot. But it does give me more ability to move around and I have worked to increase my strength. Of course there is always the knock-on of fatigue but I'm doing as much as I can, paying for one-to-one Pilates to try to recover some strength. It's not easy, especially as I am no 20 or even 30-something, (barely even a 40-something now) and it often feels like you get through one door only to have it hit you in the back and knock you flat.

Deconditioning a result, not a cause

In their letter about the study, the doctors reiterated how people's responses to patients with this condition can often make it worse.

"Patients often feel frustrated and blamed for their illness and a lack of improvement or recovery when they are labeled as deconditioned or told that they are not putting their maximum effort, regardless of whether they are exercising routinely as part of their therapeutic regimen or have not been sick long enough to become deconditioned.
"Deconditioning can occur secondary to prolonged bed rest and chronic inactivity in patients with POTS, but appears to be not a primary underlying mechanism. Larger studies comparing the invasive cardiopulmonary exercise tests in a cohort of POTS patients vs. sedentary healthy individuals are needed to further delineate the pathophysiology and possible therapy for POTS, both in terms of tolerated exercise programs and pharmacotherapy."

This last bit means they need to do more research so they can shore up the findings and come up with improved treatment of both medication and suitable exercise programs.

Getting upright again can be done, but it is a slow progression and patients need lots of support and the funding to pay for it. For example, hydrotherapy is great, but in the NHS it usually comes in blocks as it's designed for injuries, not chronic conditions and is in short supply. I had it and it was indeed helpful but I needed someone to take me and bring me back and a day to recover as it was so exhausting. 

In my own case, and that of many others, this study's findings seem to be self-evident. In EDS, the veins and arteries don't snap shut quickly enough in standing, so blood starts to pool in the lower extremities and the heart must work harder with less blood to keep the body going. Ergo, low blood volume.

A new role

I'm delighted to say that I have taken on a new, volunteer role as UK and Europe Advisor to the new Ehlers Danlos Society, which has been formed from the US-based EDNF. Lara Bloom, formerly CEO of Ehlers Danlos UK, is jointly leading the new charity and the aim is to expand globally providing trusted information of use to patients wherever they live. I'm happy to be involved.
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11 Jun 2016

The Hydrafacial: an easy way to beautiful skin

When you have a chronic illness, everyday life can seem like walking head-on into a hurricane. Putting one foot in front of the other, both physically and metaphorically, can seem like you are up against an unseen but powerful opposing force that, with one unwise step, will whip you off your feet into a maelstrom with an unknown ending. It's an uncertain and exhausting existence.

That's why having nice things happen can mean far more than you might ordinarily expect. It lifts your spirits and reminds you that the sun's warmth can still reach you. You remember that you are still a woman first, not just a patient or a carer or a parent.

That's especially true when it's something as delicious as a facial. And not just any facial, but the treatment that's sought after by A-list celebrities: The Hydrafacial.

When Miss Sherina Balaratnam of the state-of-the-art S-Thetics clinic in Beaconsfield, asked me if I'd like to try out the Hydrafacial for myself, it was just the thing to help prepare me for a challenging three-day conference that was coming up. In fact, just being in the clinic, with its all-white decor and hi-tec clinical equipment, makes you feel like you're about to experience something really special as Sherina and her team make you feel so welcome and at ease.

I've been lucky enough to test several other treatments at S-Thetics, the Fire and Ice treatment, Juvéderm fillers and my absolute favourite, the EndyMed 3DEEP, none of which I would have been able to do without Sherina's generosity in exchange for an honest review. And from the description of the Hydrafacial as a six-step, personalised, pore-deep treatment, I knew my tired-feeling skin was in for a treat.

Sherina even sent me on my way with a divine bottle of Copper Mist firming mist, to keep my skin happy and hydrated.

Sherina's Copper Firming Mist gift from iS Clinic

The Hydrafacial

The Hydrafacial can be tailored to match whatever needs your skin may have, and this can be analysed first with S-Thetics' Visia which analyses the state of your skin deep into the dermis. I already knew from a previous Visia that I have sun damage from being fair-skinned and the signs of ageing you would expect. Sherina selected the Hydrafacial Skin Health for Life treatment which, which detoxes, exfoliates, brightens, extracts impurities and then treats the skin with pure botanicals to rejuvenate, plump and firm.
You can buy the same serums that the Hydrafacial uses separately

The Hydrafacial uses a multi-functional wand, attached to a machine that pumps through the right serum for each stage of treatment. The wand has different heads according to the job it's doing, from exfoliating dead skin cells, to extracting deep down dirt from the pores to delivering the soothing and firming serums, hyaluronic acid and peptides to leave you looking your best.

The Hydrafacial kind of reminds me of when I first got a Dyson vacuum cleaner. I thought my carpet was clean until I used the Dyson. It sucked out deep down fine silt that other cleaners hadn't touched. The Hydrafacial is a similar premise: you might think your skin is clean, but wait until you see what clean really looks like. It means your face looks fresh, more youthful (if that's what you need) and with any skin troubles greatly improved.

Rather than just tell you about the treatment, I made a short video with everything you need to know. And I just know you'll want to try it out yourself.

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7 May 2016

The enigma of Ehlers Danlos syndrome and why it's vital to make the #InvisibleVisible

Ah, it's Ehlers Danlos Awareness Month again, hurrah, zebras unite and shake your stripes! Gently, though, we don't want any awareness-raising injuries.

EDS is a very enigmatic condition. It's rare, yet it might not be particularly rare. It's often in the papers with one case or another, yet no one has heard of it. It's a condition that makes collagen very stretchy, so it affects pretty much all the body and yet medical professionals continually fail to consider it as a possible diagnosis or to make an appropriate referral.

Let's look at these conundrums in more detail, [and make sure you read the end for a chance to donate a £1 to EDS UK and buy buying something stripy and cool for yourself!]

The enigma of Ehlers Danlos syndrome and why it's vital to make the #InvisibleVisible

Is it rare, or just rarely diagnosed?

Certainly most types of EDS are rare, several extremely so, but what about Type Three, Hypermobility type?

Hypermobility in itself is not rare. Most dancers and gymnasts will be hypermobile- just ask Isobel Knight, a dancer with EDS, turned author and Bowen Practitioner. And, when you are hypermobile, you will be more prone to joint injuries.
But EDS III or Joint Hypermobility Syndrome isn't just about being a bit bendy or doing a few stomach-churning tricks with your double-jointedness. In fact, many people with EDS III aren't even especially bendy, particularly if they are older.

The thing about EDS is that it's a spectrum condition, which means that patients will all be different. Most will have one or more of a range of co-morbid conditions that stem from having dodgy collagen, any or all of which can be disabling just by themselves. These can include orthostatic tachycardia (PoTS), gastric issues like reflux or more severely, gastroparesis. Some will dislocate or part dislocate (subluxate) their joints regularly or have such instability that they need bracing or life-saving surgery. Depression and anxiety are very common and several physicians and practitioners familiar with EDS have noted to me the growing numbers of patients with both EDS and various degrees of Autism Spectrum Condition.

What EDS patients do have in common is chronic, intractable pain that can bear no resemblance to their level of flexibility. In fact, it is not uncommon for someone with EDS to be very stiff as their body attempts to stabilise itself and because chronic pain can lead to lack of activity.

It's often in the papers

Why is it that the media seem to love stories about people with EDS? See them here, in a wheelchair talking about "popping out" all over the place or there looking brave, with a naso-gastric tube. Why, even Coronation Street has an actress with EDS, Cherylee Houston. Sadly, the mainstream media often get a bit confused, cause consternation and then have to apologise. This does put EDS in the spotlight though, giving advocates a chance to set the record straight.

Added to this, if you move in certain social media circles it can almost seem that every single EDSer in the world under 30 has a Tumblr or other blog, a YouTube channel, a Facebook page or an Instagram, all of which post selfies, memes and videos about living with the condition.

I think this is as much a sign of the times as an indication that the condition is more common than thought. Why? Because EDS seems to affect mainly women. And teenage girls and young women like connecting pro-actively through social media, for what else is there to do when you're stuck at home ill than take to the internet and do your bit to raise awareness? With the number of EDSers who are on social media, it's amazing that there is anyone in cyberspace who hasn't heard of it. It's certainly a shame that more doctors don't seem to be active social media users - then it would be job done, no need for any more awareness days for EDS!

Pity us "more mature" ladies who suffered in cyber-silence and diagnostic ignorance because social media wasn't invented for most of our lives. Most of this age group will probably have been diagnosed following their child's diagnosis, have been told they have Fibromyalgia or just written off as being a "hypochondriac".

For our children, however, these dismissive reasons do not wash. We have sought answers for our sons and daughters so they receive better care and a more timely diagnosis. Hopefully.

Because of this I think it's painting a skewed picture of the prevalence of the condition, but the question remains: Despite all this media coverage, why isn't the medical profession moving more quickly to a greater understanding of the condition?

So why aren't medical professionals finding a cure?

Because of its multi-systemic nature, it's easy to mistake EDS for so many other conditions. Speaking for myself, I have experienced most of the stated symptoms of EDS III during my lifetime, just not all at once. So when you show up at the doctor's surgery with a slipped disc or depression or dizzy spells or intestinal difficulties etc, that is what you are treated for and why would anyone look any further?

It's only when they come together all at once that there is any reason to hypothesise. If you just get a lot of one particular symptom, you're quite possibly going to be misdiagnosed or just be filed in the 'medical mystery' drawer.

There is no single drug or operation that will cure Ehlers Danlos syndrome, nor is there likely to be any time soon. The main reason for this is, as I said above, it's a spectrum condition so no one solution will do.

It's also genetic, so the 'cure' is most probably in gene therapy or CRISPR - gene editing. These are controversial, highly regulated and expensive areas, so a rare condition that doesn't instantly kill the patient isn't going to be at the top of the list.

The third reason is a mixture of the above. Because of the rare and genetic nature of the EDS rainbow, no one drug will make it better so just the symptoms are tackled. Pharmaceutically speaking, this involves drugs for pain, heart regulation and gastric distress, among others. Most of these are cheaper off-patent drugs such as opiates, beta-blockers and so on. There are some newer drugs for heart regulation such as Ivabradine and Midodrine, but they were not developed for this purpose, they only suit certain kinds of PoTS and are mainly prescribed by a specialist - and you have to find one first. So, for these reasons, big pharma have little interest in driving EDS-specific drug research forward as there is little in it for them financially. There is more scope, however for drug repurposing.

Keep on raising awareness

So all these reasons above are why it is so necessary to continue to raise awareness of Ehlers Danlos and related conditions and why it is vitally important to educate medical professionals, education providers and the general public about the condition.

In particular, it is important to educate social workers that an often ill or bruised child is not always an abused child; that before they swoop in and fracture a family by removing children from their parents, they should seek professional, expert guidance as to whether the child may have an undiagnosed condition such as EDS (or other similar conditions).

So why not help illuminate, educate and make the invisible visible? Join or start a conversation on the international EDS Forum at RareConnect (anytime!) I'm there, my username is Tanushka
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21 Mar 2016

Disabling effects of PoTS shown in largest ever survey and how educating health professionals can help


Postural orthostatic Tachycardia syndrome or PoTS, is a complete bastard of a condition. It doesn't kill, thankfully, but it can quite often make you feel like you have no life in you.
It can be a condition on its own or a comorbidity of another condition, such as mine, Ehlers Danlos syndrome. 
In its extreme, life-altering state, it's pretty rare and extremely misunderstood. The charity PoTS UK, has teamed up with researchers and conducted the biggest ever survey of people - mainly women - living with the condition. The research, A profile of patients with postural tachycardia syndrome and their experience of healthcare in the UK is published in the British Journal of Cardiology.

It covered causes, symptoms and treatment options. You can find the whole survey here. 
Below are a few excerpts: 

Postural tachycardia syndrome (PoTS) is a recently recognised condition that usually affects younger women, who develop symptoms of orthostatic intolerance and a persistent tachycardia on standing upright. Healthcare professionals, patients and the national patient support group (PoTS UK) together created a survey, and the responses of 779 UK PoTS patients were analysed. The most common symptoms of PoTS at presentation were the triad of fatigue, lightheadedness and palpitations. Mobility, ability to work or attend education, and quality of life were significantly restricted. Cardiologists, followed by patients, were most likely to be the first to suggest the diagnosis of PoTS. Patients waited a mean of almost four years from presentation to obtain their diagnosis and, meantime, psychiatric mislabeling was common. Advice given to patients regarding lifestyle changes was variable, and those referred to specialist practitioners for help, found practitioners had limited knowledge about management of PoTS. Increased education of healthcare professionals and improved services for patients are recommended. 

And this is key. I find a little knowledge is a dangerous thing. If a health professional has heard of PoTS, their knowledge is usually incomplete and often based on faulty assumptions, such as, "Well your blood pressure didn't change quickly enough when you stood up so it can't be PoTs," or, "We did a 24 hour ECG and you were fine," despite numerous spikes in heartbeat of more than 30bpm when just standing or walking slowly up stairs (or half a staircase).

Treatments vary depending on if your symptoms are blood pressure or cardiac arrhythmia driven. For me, I have little blood pressure response, but my EDS means my blood vessels in my lower extremities are too stretchy to constrict quickly enough when I stand. This means blood begins to pool in my extremities, my brain tells my heart it isn't getting enough oxygenated blood and so the heart races to restore the flow. I feel dizzy, light-headed, pressure in my chest from a racing heart and nausea. It can continue for a while after I sit or sometimes have to lie down after the after-effects can linger for hours. On occasion, that's not enough and I have to put my legs in the air as well - tricky if you're out! If no chair is around, you'll see me bending completely over to try to even things out. So much for 'invisible illness'. 

The report says of PoTS:  

Diagnosis is usually made by active stand test or tilt-table testing. Few treatments for PoTS have been tested in randomised-controlled trials. Patients are initially managed with increased fluid and salt intake to increase blood volume, avoidance of symptom triggers (heat, prolonged standing, alcohol, drugs that induce tachycardia or hypotension), small frequent meals (low in refined carbohydrates), graded exercise (initially in a horizontal position) and compression garments to reduce venous pooling. Drug treatment can be aimed at reducing tachycardia (low-dose beta blockers, ivabradine, pyridostigmine), vasoconstriction (midodrine), boosting blood volume (fludrocortisone, desmopressin) or for their sympatholytic effects in hyperadrenergic PoTS (clonidine, methyl dopa).

showing heart rate of 151
My heart rate from 69 sitting to 151bpm 15 seconds later walking slowly half way up the stairs

Interestingly, 92% of the survey respondents were female and half had an additional diagnosis, like myself, of EDS. Many reported fatigue as one of the most debilitating symptoms.

Physicians call conditions like this 'benign' - i.e, you don't die from it. But believe me it feels anything but benign; it can be utterly life-altering and not in a good way.

As for diagnosis, many were wrongly told their condition was psychological or psychiatric- "It's all in you head, dear girl." Just writing that makes me extremely cross. And the least aware and the most important to educate? Your first port of call, the family GP. The report says: 

"In only 7% of cases did the GP suggest PoTS as a diagnosis. This may be due to lack of awareness in primary care about PoTS or the non-specific and multiple symptoms that are associated with PoTS. However, it is possible to undertake a 10-minute stand test in a consulting room, and perhaps this should be considered in patients with relevant medically unexplained symptoms or chronic fatigue, especially if there is a postural link to symptoms."

The impact of PoTS is considerable, with 23% becoming wheelchair users (including me, part-time for long distances) with 37% are unable to work. I am no longer able to be 'employed' but my time is used as a freelance writer, voluntarily with Special Needs Jungle and as a Trustee of Genetic Alliance UK. I am a 'real' Patient Advocate - I live it, and I work to help others. In other words, I don't do it for a living and I can't walk away into a better job when I fancy it. 

Tania in wheelchair with dog
One of my first times out in a wheelchair in 2013

The most important recommendations in the survey report are education of health professionals and the need to improve accessible and relevant rehabilitation services. It's no good saying go away and do some gentle exercise when often even getting out of bed is impossible. 

I pay for a one-to-one Pilates instructor, who completely understands the conditions I have because she is similarly affected. Her career choice has kept her mobile (most of the time!). I'm aiming for the same thing, though it is often two steps forward and one step back; sometimes two back. I have been awarded a Patient Fellowship to attend the three-day European Conference on Rare Diseases in Edinburgh in May - that's a big goal to increase my fitness for!

But even now, three years on from the onset of chronic illness and pain, depression can flip on easily, from someone's unkind or careless word from someone - another battle to guard against. As a mum of older teens with both Asperger syndrome and milder EDS, I can't just think about my own needs. If it wasn't for my husband, I wouldn't be able to manage. 

Congratulations to Leslie Kavi of PoTS UK, named as a co-author of the report. Great work and hopefully this will be printed out by as many medical practitioners as patients. If you're a patient or carer, print the report out and pass it to your GP, physiotherapist, workplace if you have one or child's school or college. They're most probably too busy to find it, so help them out with their understanding. 

The survey has many more detailed figures and much more information. Once again, find the report here: http://bjcardio.co.uk/2016/03/a-profile-of-patients-with-postural-tachycardia-syndrome-and-their-experience-of-healthcare-in-the-uk/
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