Getting stronger with EMSCULPT, the no-pain, muscle-building, fat-loss medical device*

*In collaboration with S-Thetics, Beaconsfield.

My husband is super-fit, works out most days with weights and the odd run. But although I have a chronic pain condition, I've recently done far more sit-ups in half an hour than he - or anyone - could ever dream of. Twenty-thousand to be precise.

While I let that sink in, I’ll admit, it wasn’t under my own steam. I've used medical technology for my abdominal muscles to benefit from the physical effects of the equivalent of 20,000 sit-ups and without actually moving myself.

Intrigued? Who wouldn't be?

Lack of body strength

In recent years I’ve found myself with a bit of a problem. I have a structural weakness in my back thanks to Ehlers Danlos syndrome, but repeated abdominal surgeries have decimated my stomach muscles, leaving me with a dangerously weak upper body.

The result has been regular episodes of my back giving way, with my weak abs leaving me crawling on the floor, unable to hold myself up. One recent, memorable incident meant my husband and son had to roll me, shrieking, onto a large drawing board to carry me to bed.

Something had to change - but my EDS and PoTS preclude me from doing sufficient - or really any - exercise. Even standing up still for long can be a challenge.

Then I came across something that changed my life.

Magical muscle machine

The answer was EMSCULPT. This miracle medical device makes you not only stronger but also slimmer in just four sessions of half-an-hour each. I was introduced to it by Miss Sherina Balaratnam at her multi-award-winning medical aesthetics clinic, S-Thetics, in Beaconsfield, Buckinghamshire.

I'm a huge fan of S-Thetics and have had a number of beneficial and life-enhancing treatments there previously.

This video I've put together explains what it’s done for me - and could do for you.

*I received this particular session of EMSCULPT courtesy of S-Thetics Clinic.

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EDS Awareness: Coming to terms with chronic pain

As ever, My EDS Awareness month post sneaks in on the last day of May. It's been a big month, including a short, but amazing, trip to Barcelona to celebrate my and my husband's 50th birthdays (wtf?!). His was in April and mine is on 6th June (666... really rather fitting.)
I wasn't sure how I would manage it, given the uncertainties of my Ehlers-Danlos syndrome, but we had booked assistance at the airport and the train station and I took my own wheelchair.
The weather was perfect for me, warm, but not too hot for walking/wheeling around the very accessible city, visiting the wonderful architectural sights of Gaudi, including the incomparable Sagrada Famiglia, the stunning mosaic quirkiness of Park Güell and the stirring originality of La Pedrera and Casa Battló. It made my heart sing and I felt so alive and joyful to be there.

At Gaudi's Park Güell

Because of the limitations that Ehlers-Danlos syndrome places upon me, I had carefully planned the trip to include rest times, using taxis to get around so as not to use precious energy on getting between destinations. Slow walking with a stick, lots of breaks to watch the people go by and an afternoon rest while my husband visited the Picasso Museum were all factored in. Still, by the last day, I was exhausted and had to take to my wheelchair with the planned visit to Montjuïc being left for a future visit.
It's not how I used to go on holiday pre-illness but, after four years, I feel I have come to terms with structuring my life around it.

ACCOMMODATING EHLERS-DANLOS

It took more than two years to come to the realisation that this is how my life now is, restricted, stilted by fatigue and, if I forget to take my pain meds, in agonising pain. I had to decide if I was going to be angry that this had happened, or to accommodate it as an unwelcome guest, planning your life around their convenience.
At the same time, the years were inexorably moving forward, my children would soon both be adults and no longer so reliant on me. And, as I began staring down the barrel of 50, I have had to decide what kind of life I wanted to have, given what I was physically able to do.
I have seen any number of YouTube and Instagram videos of young women with EDS talking or complaining about their conditions; they're hard to watch and I'm grateful that my own illness didn't blow up until much later in life. Although I was always plagued by various bodily pains, digestive problems, dizziness and so on, I had no diagnosis and I wasn't a regular joint dislocator. I just got on with it. I was lucky.

But coming on, as it did, just after I was getting back into working after caring full time for my autistic sons was a huge blow. Realising it was here to stay plunged me into depression, always something that has clung to my event horizon, never quite able to escape completely.

A VICIOUS CIRCLE

One of the problems with EDS is that it can become a vicious circle of debilitation. You are tired and faint and in pain so you don't - can't - move about much. As a result, your body becomes weaker and "deconditioned". It is the cruel partner of any chronic illness.
At my age, this was even more of a danger that had become my reality. Going anywhere took extreme effort and the payback was several days in bed just to get back to the usual poor state.
It was going on the RNOH Stanmore Hypermobility Rehab course that gave me the confidence to start to try to push myself a little bit more. Armed with skills for pacing (that came in very useful in Barcelona) I began to look at my work schedule to try to ensure that I had energy for both the things I wanted to do and the things, like caring for my boys, that I needed to do.

Serendipitously, I found a fitness expert who now also has a diagnosis of EDS. She puts me through weekly (or so) gentle one2one Pilates using a Reformer and, over time, my strength has built up to a level where I can go to a meeting in London and take a bus from the train station, if there is a stop within a few metres of my destination. It's a big money saver from always needing a taxi, although it does exact a greater physical toll. Of course private Pilates is a financial investment that not everyone can afford. Once again, I'm lucky.
Taking up mindful meditation, stopping eating meat and developing a keener understanding of myself, via my own autism diagnosis, have all helped. It's important to do my best to ensure that I, and not Ehlers-Danlos, am in control of my future.

FIFTY PLUS

Although my caring duties are far from over, I have reached a point where they are not as all-consuming as they once were. In theory, I have more time to find or create work and earn money.
Luckily, again, over these years, I have developed my special needs website, worked as a volunteer and honed my skills as a social media specialist for social good. Mostly from my bed. But is this what I want to do for the next 20 years?
50 is nothing to be afraid of; it is a time, for many, of new freedom from parental duties if you've had your children by your early 30s.
But of course, Ehlers-Danlos doesn't like to release its grip quite so easily. Just because I've reached a turning point in age and mental strength doesn't mean it's going to pack its bags and bugger off. I may have learned to accommodate its threatening ever-presence, but I am mindful that it is always a precarious balancing act. One missed or too-late dose of pain meds, too little movement because I'm not feeling well, eating something my EDS digestive system doesn't like or just doing too much (what is too much varies daily), can destroy the delicate equilibrium I have acquired. Even emotional upset or stress can tip the scales into a flare up.
But I never forget that despite all these challenges, I am lucky to be where I am right now. Ehlers-Danlos syndrome is a greedy bastard. It knows that no matter how hard you work to regain strength, one slip and it will have you back in its painful embrace.

READ MY OTHER POSTS ON EHLERS-DANLOS SYNDROME

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

My youngest and I were both diagnosed with Ehlers Danlos syndrome by leading world expert in the condition, Professor Rodney Grahame (Update: Prof Grahame sadly passed away in early 2026). 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.

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. hEDS, 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 nasogastric 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 hEDS 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.

A useful prop

 

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.

 

Hydrotherapy 

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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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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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!]

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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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).

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. 

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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Can you help #careforLaura crowdfund fund life-saving surgery for Laura's Ehlers Danlos syndrome?

As you may know, since 2013 have been disabled by the rare disease (or rarely diagnosed disease), Ehlers Danlos syndrome. I've been at rock bottom and have, with the support of my husband, managed to drag myself to a somewhat precarious place where I can work a few hours a day.

Days out or meetings are always a knock back but I build the down-time in. I've know that I'm lucky to have got to my mid-forties before this genetic condition hit me with its full force.

Recently, I was put in touch with another woman who lives close to me with the same condition. Laura Sylvester and I met for a coffee in Farnham, where she told me her story. 

Laura is just 23 years old, beautiful, smart and determined. A lover of sports, Laura was studying for a Masters degree at Imperial College, London, when EDS took hold. She became so unwell she had to pause her studies and return home to be cared for by her parents.

Laura's condition has continued to deteriorate and her vertebrae are now so unstable, it's put her life at risk. She needs vital neurosurgery that is only possible from a particular surgeon. He happens to be in the US and so this is going to cost a lot of money. A lot of money that Laura and her family don't have. 

However, I mentioned that she is smart and determined, and so Laura has launched a crowdfunding campaign #careforLaura, to ask for people to help her reach her funding goal so she can have the surgery early next year. This will mean she will be able to resume her studies and, although she will still have EDS, she will have a shot of achieving her goal of becoming a geologist.

Here, Laura tells her own story:

Can you help #careforlaura?On 7th August 2014, I was diagnosed with a rare genetic disease called Ehlers Danlos Syndrome (EDS) with PoTS (Postural Tachycardia Syndrome). EDS causes the connective tissue throughout my body to be extremely stretchy and easily breakable - there is no cure for it. Over time, my muscles in my neck have become very unstable, which has further led to dislocation/subluxation of my vertebrae, disruption of my spinal cord ligaments, and compression on my cerebellar tonsils and brain stem.

Suddenly my life was flipped upside down and this degenerative muscular condition caused me to deteriorate quickly, forcing me to postpone my Master’s degree at Imperial College, return home, and be cared for by my parents.

Once an extremely sporty, sociable, positive and driven young woman, I have become tremendously vulnerable. I am in extreme pain, I wear a neck brace all day, and I am at constant risk of further damaging my brain stem, that would consequently cause me to stop breathing.

I am in desperate need of specialised EDS neurosurgery which will be done on 13th January 2016 in Washington D.C., USA. There are few experts in the world who can do this. The total of the medical expenses, travel and recovery is very high. I hope and NEED to raise at least £75,000 ($110,000) to fund this.

The surgery will create stability in my neck and stop the compression on the brain stem and cerebellar tonsils, essentially saving my life. This will enable me to carry on living my life as a normal 23-year-old, hopefully mostly pain-free and without the worry of causing further damage, which could otherwise prove fatal. 

Even though the surgery will offer me the stability that I need, unfortunately it comes at a cost of losing complete movement of my head and neck. Nevertheless, I am determined to adapt to this and begin fulfilling my dreams once again and resuming my Master's degree.

Any amount of donations and support for this specialist neurosurgery will be so tremendously appreciated, not only by myself, but also my family and friends. Please do consider contributing to the first major stepping stone that I need to live my life without fear.

22nd December 2015:

Laura is now within £5000 of her target - an INCREDIBLE achievement in just two weeks. Can you help push her over the finish line?

Click here to make a contribution, however small, it will help make Laura's Christmas fantastic and give her the chance of a New Year appointment with her surgeon!

Listen to Laura talking about her appeal and her EDS on Eagle Radio 96.4

Read their story here

For more information about EDS, visit EDS UK
For new stories about EDS, check out my Tumblr news site A Rare Diagnosis

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Invisible Disabilities Week: Our experience with treatment for Ehlers Danlos syndrome

This week it's the Care Quality Commission's #InvisibleDisabilities Week and today, they're highlighting Ehlers Danlos syndrome which as you know is the delightful multi-systemic condition with which my sons and I are diagnosed.

I managed to get through to my forties before I became very ill with EDS in December 2012. It followed a return to paid work which I threw myself into as well as managing Special Needs Jungle which had come into its own the same year. I might have got away with it if it wasn't for the fact I was also falling into the swirling vortex of an unexpected early menopause, again due to consequences of EDS.

Looking back, I began to suffer the racing heart symptoms of PoTS before I'd started the job that March 2012, but I had shrugged it off, the same way I had just carried on my whole life up until then.

At various stages I'd suffered with weak ankles, broken limbs, strange sharp pains that would come and go, dizzy spells, an inability to stand for long without back pain, pleurisy, gastro problems, fatigue, mysterious abdominal pains, a constantly stiff neck, frozen shoulder and even a slipped disc. On top of this was an intractable depression that started at 16 and has plagued me on and off ever since as well as other mental health difficulties.

These are all symptoms of Ehlers Danlos syndrome, but dots had no chance of being connected - no one had ever heard of it. It was far more likely that you'd be diagnosed with a huge case of being a chronic hypochondriac.

Because it was the 70s and I grew up in a single parent, working-class family, you just got on with things and I was out of the house more than I was in it. Maladies were dealt with matter-of-factly and a constant forward motion was encouraged. The standard refrain was, "You'll be alright when you get going." It serves to make you resilient but even the resilient have their limits for coping.

Whizz forward a few decades and I twigged that my youngest was suffering from symptoms matching EDS, that I had discovered during a search of the Genetic Alliance UK website for Rare Disease Day. Of course I realised I had the same, but I was alright when I got going, as I had been taught so long ago.

My GP had never heard of the condition, so I was able to give her some information from the support charity, EDS UK. I had also researched the options for consultants and discovered there were not only very few, but the wait was ginormous.

We opted to pay for a private consultation with the pre-eminent specialist in EDS, Professor Rodney Grahame at The London Hypermobility Unit where he was diagnosed. Several months later, being alright when I got going just wasn't working and my own body metaphorically fell off a cliff from too much work, stress and too few hormones. I was also diagnosed privately by the Prof.

The CQC-led Invisible Disabilities Week want to hear tales of our treatment experiences. For my son and I, they are vastly different.

My youngest was referred back into the NHS at our request, and Prof Grahame recommended Dr Nelly Ninis, an extraordinary Paediatrician at St Mary's, London.  Dr Ninis sees many children with EDS and sent youngest for a 24-hour ECG and an echocardiogram. This is standard in EDS because of the possibility of mitral valve prolapse and abdominal aortic rupture if you have EDS IV.

Because of suspected Postural Orthostatic Tachycardia (PoTS), youngest was given a Tilt-Table test and found to definitely have this condition too, which is very often seen as a part of EDS.  Stretchy collagen means the blood vessels in the extremities do not respond quickly enough on a postural change, e.g. standing, and so blood drops to the feet. The heart then responds by racing to try to get blood back up to the head which makes you feel very dizzy or you can pass out completely.

The ECG and echo were done locally and it was apparent that the doctor we saw had little real understanding of EDS beyond the 'headline'. He also once kept us waiting for 45 minutes, by which time youngest had become very 'Potsy' and thrown up and I was exhausted. I was not best pleased, as you can imagine.

Dr Ninis recommended Hydrotherapy,  but the local physio team were very nervous of possible fainting with the heat of the water. It went fine for most of the sessions and was beneficial. However, the problem is, hydrotherapy is designed for short-term use with just six sessions, which just isn't sufficient for a long-term condition. It's also under-resourced and hard to find. This is mad - hydro is an ideal gentle exercise that can help to transform the lives of people living with chronic pain, if only they could get it or even knew about it.

We also tried with local dry-side physio, but again encountered the problem of a lack of understanding of EDS beyond what they might have read in a medical reference book. Youngest is also autistic, very sensitive to personalities and took an instant dislike to the bossy physio's insistence of being given a "grading" with a counter in a slot rated from Excellent down to Poor. Youngest rightly pointed out that with her standing right there, only full marks would be appropriate, so declined to take part. We didn't go again.

Dr Ninis, who is kind and compassionate and most of all understanding, sees youngest every three to six months, although it's an arduous journey to St Mary's from our home in Surrey. It means my husband needs to take the day off as my own EDS means the drive is impossible for me. As a contractor, this means the loss of a day's pay.

How was it for me?

My own experience has been much worse in many ways. After diagnosis, I too had to go back into the NHS as we can't afford to keep paying for something we already pay for through taxation. It was seven months before I saw the EDS consultant, Dr Hanna KazKaz at UCL. She referred me for the Stanmore hypermobility rehab course. 

The wait for a first visit for Stanmore assessment was over a year and then it was another eight months before I managed to get on the course. Shortly after I was accepted for the rehab, the list was temporarily closed due to the waiting times already being experienced. It's the only one in the country I believe, so it's hardly surprising they are overwhelmed.

The wait for PoTs diagnosis was even longer. Again we paid for an initial appointment to see the eminent Professor Chris Mathias in May 2013 but the tests would cost £1800 so we waited to see his former NHS team, as he had retired from the NHS himself. The wait thereafter was interminable; a year for a consultation, another few months before the Tilt Table test and a further 10 months for the results of the tests. In retrospect, perhaps we should have paid for the whole lot privately, but hindsight is 20:20.

Where's the care?

For both youngest and myself, every aspect of this has been driven by me, from the realisation of what the problem was to finding the consultants, to going back to the GP time and again to ask for other referrals.

There is no joined up care, there is no creative thinking, there is little thought of the patient experience. Certainly not when the available doctors for the conditions are so few and far between and the waiting lists are so very long.

Neither is there local coordinated long-term care. I understand GPs are generalists and very busy, but I would have hoped that when one family's members in the practice are diagnosed with the same complex, rare condition and the children also have autism, a more proactive approach might be taken. At least to read up on it a bit.

It's a long-term condition, but we have been left to find our own solutions and there is no monitoring. Our experience is very far from the ones described on this CQC page

 It's no wonder so many cases of Ehlers Danlos go undiagnosed.

Being resilient with my long-suffering husband

Lucky for me that I'd had that Gen X "just get on with it" upbringing, because the pressure to cope with offspring with autism, EDS and PoTS as well as my own chronic pain is immense. Indeed, in the past I have cracked under the strain when that one thing comes along and causes overload. I have had to seek psychological help. but we won't even go into the debacle that the Mental Health service is, and continues to be.

This may seem like a critical post, but it is only critical of the resources and the structures that prevent medical professionals giving the care patients need, particularly in the field of rare diseases. Long term health conditions have a psychological as well as a physical impact, even when there is no specific mental health problem. Feeling cared for and that your experience as a patient actually matters, is a huge part of that. It's all part of that joined-up experience that starts and finishes in the GP surgery as what should be the coordinating service.

However, with a shortage of GPs and constant pressure to give more for less put on them by politicians, it is hard to see how this will improve. Perhaps the increase in patient participation boards may make this difference, as a reminder that a 'patient' is also a human being.

The question, "Would this be good enough for your child or parent?" is the mantra everyone employed in caring services should recite every day.

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EDS Awareness: My experience with Ehlers Danlos syndrome and Pregnancy

It’s EDS Awareness Month in May and I’ve decided to write about something that may be on the minds of many young women with Ehlers Danlos- how will having EDS affect me when I want to have a baby?

Ehlers Danlos syndrome is a multi-faceted condition that, much like autism, has a number of different types and within those types, each patient is affected in a different way. The most serious is Type 4, or Vascular EDS. If you are female and diagnosed with this type, you will no doubt already understand that pregnancy is very risky and potentially life-threatening as it can increase the possibility of a catastrophic arterial or organ rupture.

Vascular EDS and pregnancy: statistics

A study published in 2014 found that pregnancy-related deaths in women with Vascular EDS occurred in 30 of 565 deliveries (5.3%). Interviews with 39 women indicated that 46% had uncomplicated pregnancies, while the most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and preterm delivery (19%).

Life-threatening complications occurred in 14.5% of deliveries and included arterial dissection/rupture (9.2%), uterine rupture (2.6%), and surgical complications (2.6%). This is just one study however, anyone with this diagnosis should always seek their own personalised medical advice before attempting to become pregnant.
I've also added some free to access resources at the end of this post regarding pregnancy with Ehlers Danlos syndrome.

Just before youngest was born

My Experience

While I am not a medical professional, I can talk about my own experience, as someone with EDS Type III, who has been through two pregnancies. Please feel free to leave your own experiences in the comments as well as they will be helpful to other couples.

At the time of my pregnancies, I did not have a diagnosis of EDS, nor was I seriously affected. In fact, my two children were born 15 years before I fell life-changingly ill with chronic pain.

I had always experienced symptoms at one time or another but as they were transient, by the time I'd decided to visit the doctor they had either disappeared or the doctor wasn't particularly interested. These include unexplained pains that would disappear as quickly as they came, joint pains, neck and back pain, dizzy spells, weird stabbing pains in different parts of my body, cold extremities, weak ankles, low energy, poor sleep and so on.  They just didn't happen, as they do now, all at once and so no one ever put them together into one whole, and even if they had, they probably wouldn't have heard of Ehlers-Danlos syndrome anyway.

If I had realised, I would perhaps have been able to get specialist advice but at the time, EDS was even less known than it is today. Plus, I was working as a television journalist with a new contract with a top agent and I was more concerned with how I was going to manage work and being a mother after the baby arrived.

Hypermobility means that the joints can be loose or unstable and the collagen, which makes up most of the body, is more stretchy than normal. Pregnancy hormones themselves will also increase the elasticity of every expectant mother's body so for someone with EDS, this can mean joints, muscles and ligaments are much more likely to be over-stretched and injured.

Eldest comes home

Early to mid-pregnancy with EDS

Within weeks of being pregnant for the first time, I was suffering with back pain severe enough to send me to A&E. It wasn't regular back pain, but shooting pains and aching in the lower half of my back. As I had previously suffered with back pain, no one was too bothered and the baby was growing well. Very well. I suffered morning/all day nausea and sickness for a while, enough to miss a few days off work, but not too bad.

But the fatigue! It seemed, at times, that the baby was sucking every single ounce of energy out of me. I was working near Tottenham Court Road in London and would stand at the bottom of the steep flight of steps at one of the exits and just look up, wondering how the hell I was going to get up to the top.

In the end, I had to stop work at 6 months pregnant. A 12-10pm shift reading pan-European TV news bulletins may seem like a cushy number, but not when you're hefting around an unceasingly active growing human inside you.
As soon as I knew I was pregnant, I began to use lots of moisturising lotion on my abdomen to stave off the dreaded prospect of stretch-marks. Little did I know at the time, but having stretchy skin comes in rather handy when you're pregnant. I had always thought that making sure I remained oilier than a tanker spill was the reason I didn't end up with a single stretch mark after two babies. However, when EDS finally engulfed me in my forties and I consulted the eminent Prof. Rodney Grahame, he asked if I had developed stretch marks in pregnancy. When I said no, he laughed excitedly, “Ha ha! He said, why do you think that was? Because of EDS!” A small comfort, I suppose, but a comfort nonetheless. However I have heard of some women whose body became criss-crossed with stretchmarks, so as with many “spectrum” type conditions – when you've met one person with EDS- you've met one person with EDS. In other words, you can't generalise. Those women may in fact have a type other than hEDS. 

 Of more concern to me at the time, was that we had a "double" blood test and we were called in because the test indicated an elevated risk of Down's syndrome. Did we want an amniocentesis? My partner and I discussed it and decided that on comparing the risk of miscarriage with the chance of DS, we would not go ahead with the test. If our child had Down's, so be it, he would still be ours.

Youngest with his splint for unstable hips worn for 10 weeks

Late pregnancy with EDS

Not long after I stopped working at six months pregnant, moving about at all became very difficult. The baby was growing rapidly and his weight was pushing down on my pelvis. I became unable to walk far without a lot of pain. Getting in or out of the car required my husband helping me in or dashing around to the passenger side to haul me out, laughing, "I'm a comin' Lambsy.." as he scooted round the car.

The problem was Pubis Symphysis Dysfunction, which is when the pelvis over-softens from progesterone and causes pain and instability from the weight of the baby. It usually resolves after the birth but it was one more easily missed sign.

Sheepdog, with his Lambsy

Meanwhile two months before the birth was our wedding day! You might think we'd left it a bit late, but this baby wasn't expected - we had only been together for a short time - but I really wanted to be married before he arrived. The day was long but wonderful and of course, we couldn't go on a big honeymoon. We promised ourselves one at a later date. 18 years on, we still haven't been because raising two sons with autism is pretty all-consuming, especially when you get tired easily (even though I didn't know why at the time)

Here he comes. I said here he comes! Come on!

By my due date on 22nd November, I was desperate for this baby to come out. He, however, was quite happy in my comfy, stretchy body, turning from side to side and sticking a foot out now and again so hard that you could see the outline of his toes. It felt like having an alien inside me.
The date came and went. And then some more days, and some more. I began to feel embarrassed at this failure to appear. I made my husband do three rings on the phone, hang up and then call again so I knew it was him before I would answer it.

I was admitted for an induction on 1st of December. The baby had missed my Dad's birthday, today was my late Grandad's birthday and the 2nd would be my sister, Fiona's birthday. Would it be today? I hoped not actually, as I had shared my birthday, 6th June, with my uncle and it had felt like I had never had a special day all to myself when I was growing up.

The induction medication is given as a pessary, but for me, nothing happened. My husband visited and went home, still nothing but some regular early pains that weren't going anywhere. They gave me an injection of pethidine - it did nothing to make me more comfortable but gave me a huge, painful bruise from the injection - again neither of which is unusual in someone with EDS - if I'd known I had it. I spent the night kept half-awake by the wailing of women whose babies knew that womb time was up and were preparing to emerge into the big bad world. Unlike mine.

Late morning the next day, I was already exhausted from a poor night's sleep. Just as they were preparing to administer a second pessary, my waters broke.

Labour came on so hard I thought I was going to die – no, I was sure of it. My whole body was engulfed in agony and I was moved to a side room as soon as my husband arrived. An epidural was arranged while I enjoyed some gas and air. It didn't help the pain, possibly again, related to the EDS resistance to anaesthetic - but I just didn't care as much as they wheeled me down to the delivery suites giggling and hurting at the same time.

Unfortunately, even though it was a walking epidural, it seemed to slow the labour down and more than 24 hours after full labour had started, the baby was still nowhere near ready to emerge.


In order for a baby to be delivered, the cervix has to dilate (open) and efface (thin) and whether this was as a result of EDS or not, I don’t know, but in me, this process had only occurred fully on one side, not leaving enough room for the baby to exit.

I was exhausted and so was my husband, who was cramming for his accountancy finals in the hospital room in between walking around the corridors with me to try to move things along. First eleven days overdue and now this? Are you kidding?

Finally, it got too much. When I eventually got a midwife who would understand what I was saying - most of the staff were from overseas - a c-section was arranged. My reasoning was that as I was already exhausted, I would not have the energy to push and even now I wasn't sufficiently dilated. I did NOT want anyone using forceps on my baby's head to drag him out, thanks very much. 

At 10:40pm, 32 hours after labour started in earnest, my 8lb 3oz boy was pulled out, kicking and screaming so loudly, even the doctors looked shocked.

I briefly held him, distracted only by the voice of a doctor stitching me up saying, “We’ve got a bleeder here…” What?

I was barely able to believe that this large baby had grown inside of tiny me. I then quickly had to give him to my husband so I could throw up from the effects of the anaesthetic.

Youngest at five months old, first Christmas

Caesarean a mistake?

I have since wondered if it was a mistake to opt for a c-section, given what was to ensue, but after a day and a half of fruitless labour, I felt there was little choice to avoid the dreaded forceps. I would rather be cut open than have that happen to him. 

So why a mistake, when there was really no choice?

My second baby, 19 months later, was also a c-section. We did try labour but the same failure to efface and dilate was happening. The doctor later noted that I had the formation of some adhesions in the abdominal cavity from the first section which had made the second c-section more tricky.

My second child was born with an unstable hip and needed to be in a splint for 10 weeks. Still no one thought of any connections. Both children now have an EDS diagnosis.

Within a year, I was plagued with almost continual abdominal pain and an investigation revealed that the adhesions had spread and were sticking all my internal organs together. Adhesions, or scar tissue, are very common in EDS (which I still didn't know I had).

Youngest had suffered from Reflex Anoxic Seizures from the age of 13 months so I didn't have a lot of time to think of myself and why this pain was happening. On top of this, my older son who had made his embarrassingly late arrival, was making up for it by walking at nine and a half months old, and now dashed everywhere head on, resulting in numerous trips to the hospital to be stitched up again.

No more babies...

To try to resolve the pain, the only option seemed to be more surgery. This time, a hysterectomy, aged just 35, to remove the spidery adhesions that had welded my inner stomach wall, my bowel, bladder, intestines, uterus and ovaries together. No wonder I was in pain!

It did improve things for a while, but I do wonder if I had had an earlier diagnosis, my maternity care could have been better prepared, potential difficulties might have been foreseen so that more pro-active options for a better labour could have been tried.

At the time of my first delivery, being so big and uncomfortable I just wanted the baby to come out. If I'd had more confidence, I would have avoided going in for an induction and just waited at home for labour to start naturally so I could have been more rested.

It was the fatigue, which plagues my life again today, coupled with poor maternal care in the central London hospital ward, that led to the chain of events ending in my first c-section.
Knowledge is a powerful thing; for people like myself it can mean being able to make informed decisions for our own care and being able to advocate effectively for ourselves (and for our families). I hope that by writing this it may help other women with EDS or who have similar symptoms to know what certain decisions may lead to before they are faced with making them.

Some free to access medical articles about EDS and pregnancy:

• Gynecologic and obstetric impact of the ehlers-danlos syndrome : Clues from scrutinizing dermal ultrastructural alterations
• Ehlers-Danlos Syndrome Type III and Pregnancy - A Case Report
• Pregnancy in JHS and EDS-HT
• Pregnancy and Delivery in Ehlers-Danlos Syndrome (Hypermobility Type): Review of the Literature
• Pregnancy-related deaths and complications in women with vascular Ehlers–Danlos syndrome
• Gynecologic and Obstetric Implications of the Joint Hypermobility Syndrome (a.k.a. Ehlers–Danlos Syndrome Hypermobility Type) in 82 Italian Patients
• Gynecologic and obstetric impact of the ehlers-danlos syndrome : clues from scrutinizing dermal ultrastructural alterations

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