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.

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 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.

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 EDS UK's Thunderclap  (before 20 May 2016) or start a conversation on the international EDS Forum at RareConnect (anytime!) I'm there, my username is Tanushka

Buy to donate - EDS Awareness Month offer from Cool Blue Zebra

If you're in the UK or buy from the Amazon UK store, my husband's little design outfit, Cool Blue Zebra, is donating £1 from each of its zebra-themed cool bags and zipped pouches sold in May and June 2016 to EDS UK.

So if for example, you buy both cool bag and pouch, we make a £2 donation in total. The pouch can take an iPad Mini or Kindle or be used as a make-up bag, pencil case or as a perfectly-fitting insert inside the cool bag to keep items separate. Use this code: EDSUK16 when you check out so he knows to allocate £1 of the sale proceeds to EDS UK.


cool blue zebra cool bag and pouch
£1 from the sale of the cool bag and £1 from the sale of a pouch You MUST use code EDSUK16 in the voucher code field at checkout at Amazon UK only.
If you have problems with the code, let me know [via email] and we can match your purchase details to a donation

3 Apr 2016

Family Faces of #autism - Autism Awareness 2016

It's Autism Awareness Month 2016. I've made a little video slideshow of faces through the years of me and my boys, all of us with a diagnosis of autism. 

It also includes my mother, who once said she recognised the condition in herself and if you'd known my mum, that was something of a revelation of self-awareness. 

family faces of autism

The genetics of autism is being more researched every day and in my opinion, it is genetics, whether inherited or environmentally altered, where we will find the key to this condition.

Environmental triggers of many kinds are known to spark mutations in genes and potentially cause autism behaviours and other disabilities and this is something that must be investigated fully. We must look at how we live, the chemicals we use and invest instead in natural alternatives and clean technologies that inflict no damage to us or to the planet. This is the smart and the only way forward.

However, our civilisation would also be so much poorer without those people among us whose genetic make-up allows them to think in pictures, in 3D or to make seemingly random connections that lead to positive advances in our society.

For these people - and for anyone who is neurodiverse or disabled - it is our duty to change society so that everyone in included in the way they wish to be.

We must nurture a society of individuals whose needs are met holistically and compassionately. A society of people, whose individual talents - whatever they may be - are recognised, developed and supported for the good of humanity and to ensure we deserve this beautiful planet that we inhabit.

And now the bit where you go awww...

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/

22 Dec 2015

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?

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

2 Dec 2015

How to rejuvenate your skin in two easy steps

You may have seen my previous post about visiting the S-Thetics medical aesthetic clinic in Beaconsfield, Buckinghamshire when I sampled the delights of Allergan Juvéderm fillers. If not, take a look at the video I made demystifying and informing you about the procedure, which really made a difference not only to my 48 year old face but also to my confidence.

endymed image slate

*Scroll to the end to watch the short video

Surgeon, Miss Sherina Balaratnam, who owns the clinic, invited me to return to try out her Signature facial treatment, which consists of a session with a revolutionary non-invasive EndyMed™3DEEP® radiofrequency treatment.  That's followed by a luxurious Fire and Ice facial tratment that is described thus:

"Pioneered in Beverly Hills and coveted by A listers globally, this renowned intensive treatment is clinically formulated with the purest pharmaceutical grade ingredients and designed to offer the ultimate results driven facial treatment.

The Fire & Ice treatment gives the skin a deep cleanse, leaving it with a beautiful glow. Your skin will feel plumper, smoother and more hydrated, leaving you ready to face the day! This treatment is ideal as a one-off skin rejuvenation treat, but for more intensive and long-lasting results, we recommend using once per week over a six week period."

How does the EndyMed™3DEEP® work?

The EndyMed™3DEEP® is a non-invasive radiofrequency treatment, designed to stimulate collagen, combat loss of elasticity, wrinkles and impaired texture that, let's face it, are for most of us, part of life's little expectations when we are lucky enough to have made it thus far. But it doesn't have to be that way.

"Using radio frequency (RF) energy, EndyMed™3DEEP® safely heats the collagen in the skin making it contract, providing an immediate change in skin appearance.  The treatment also triggers a second process of new collagen production, stimulating your body’s natural collagen renewal process leading to skin tightening and a reduction in the appearance of wrinkles."

You see, this is why those celebrities look so amazing for their age. It's not just good genes, it's great maintenance and luckily, it's not so expensive that it's completely out of reach if you are not famous.

Back at the S-Thetics clinic in Beaconsfield, Bucks, Sherina shows me the Endymed machine, which does indeed look very medical. Reassuringly so. The other factor that takes away any concern is the fact that Sherina is a highly experienced medical doctor and surgeon (hence the 'Miss' and not Dr).

Why did I go for it?

I had noticed in recent months, the skin of my lower jaw feeling a little thinner, less taut and more fragile. Almost certainly no one else would have noticed it, but it was just that the 'quality' of it I could feel had changed. So when Sherina offered me the chance to try out the treatment, I didn't hesitate, especially knowing I was in such good hands.

In any case, I'm not interested in pleasing anyone else - my husband loves me as I am - but I'm just not ready to see in the mirror a face that doesn't reflect how I feel inside. Living with a chronic pain condition can make you feel battered and old before your time, so for me, looking the best that I can helps keep my spirits up.

Back at S-Thetics

In the quiet sanctuary of the S-Thetics clinic, I lay on the treatment bed while Sherina began the EndyMed Facial Tightening treatment. The hand-held part of the EndyMed device reminded me a bit of an ultrasound and as Sherina applies it to the area being treated, my lower face, I could feel the heat penetrating through some contact gel into my skin. It wasn't unpleasant and it didn't hurt. The temperature is monitored and when I indicated, as instructed, that it was approaching the limit of comfort, Sherina knew that it had done its job.

The treatment was very quick, painless and you could have gone straight back to work if that's what you wanted to do. Of course I was just testing out one session, but a course of six is recommended for long lasting effects.

On to the Fire and Ice

The second part of the 'Signature treatment' is the Fire and Ice facial treatment. Most of us have had facials, but this one is a multi-step procedure that peels and resurfaces the skin with 18% unbuffered glycolic acid, citric acid, lactic acid, malic acid, vitamin B3, retinol and potent antioxidants, including green tea extracts. That's the 'Fire' part but it didn't hurt in any way. I have very sensitive, allergy-prone skin and I was a bit nervous that I might have a reaction, but everything is based on natural, botanical ingredients and I was perfectly fine.

The Fire part is an intensive clinical treatment designed to reduce fine lines and wrinkles and provide deep cleansing of blocked pores. 

The second 'Ice' stage is another masque application that is cool, soothing and intensively hydrating. It contains hyaluronic acid, often described as 'the fountain of youth',  Japanese green tea extracts, aloe vera gel, liquorice extracts, rosemary extracts and grape seed extracts. This second masque cools the initial resurfacing and is left on for about 5 minutes. 

After a final cleanse, a moisturising serum and a strong sunscreen are applied to the face because the treatment will leave your face susceptible to the sun for a short while afterwards. 

The Verdict

I'm often sceptical of facial treatments because I rarely feel any different afterwards apart from a bit lighter in the wallet area. But the S-Thetics Signature treatment honestly made a big difference to my middle-aged skin.
It immediately felt plumper and firmer and to me, it looked much brighter and less tired. 
But it was the EndyMed radiofrequency treatment that has made the lasting difference. The fragility I was feeling in my neck and lower jaw has disappeared along with any slight loss of elasticity I had noticed. Now it's back to what it was at least a decade ago. 
My neck especially has shown the benefits. The treatment has tightened all the collagen in my skin so that it's all back where it used to be and I am delighted! 

Ideally, I would have the full course of six treatments but even with just one, the effects have already lasted several months. I'm rather late with this review but in this case, it means I have had a chance to see how the treatment has lasted - and it really has nipped that first sign of sagging skin in the bud and I would recommend it to anyone my age or even younger (depending on how your skin is faring). 

It works just as well for older men and women too - possibly it would have even better results - and its benefits are not restricted to the face. It can treat sagging body areas as well including the abdomen, arms, breasts and bottom. It really is an amazing treatment and if I sound as if I'm raving, it's because it really is worthwhile having, to give you a confidence boost. 

It's true that for many mums, this kind of thing is a luxury we can't often afford, but when I work out how much I've saved by giving up alcohol for example, that would easily cover a treatment every so often. 

And of course, it's nearly Christmas, so if you're looking for a no-down-time treatment to give you that skip in your step for the festive season, look no further than S-Thetics Signature EndyMed™3DEEP® and a Fire and Ice facial.

Thank you once again to Miss Sherina Balaratnam of S-Thetics in Beaconsfield for her generosity (and her patience!) I cannot recommend her enough.

10 Nov 2015

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 Son2 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.

Son2 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 Son2 for a 24-hour ECG and an echocardiogram on his heart. This is standard in EDS because of the possibility of mitral valve prolapse and abdominal aortic rupture if you have EDS IV.

tilt table

Because he also had suspected Postural Orthostatic Tachycardia (PoTS), he was given a Tilt-Table test where he was 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.

Son2 had his ECG and echo 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 Son2 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 his possibly fainting with the heat of the water. He was fine for most of the sessions and did benefit. 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.

hydrotherapy sign

Son2 also tried with local dry-side physio, but again we encountered the problem of a lack of understanding of EDS beyond what they might have read in a medical reference book. As Son2 also has Asperger's, he is also very sensitive to personalities and took an instant dislike to the bossy physio and her insistence that he give her a "grading" with a counter in a slot rated from Excellent down to Poor. He rightly pointed out that he felt he had to give her full marks as she was standing right next to him and so he declined to take part. We didn't go again.

Dr Ninis, who is kind and compassionate and most of all understanding, sees him 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 Son2 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.

Tania being resilient
Being resilient with my long-suffering husband

Lucky for me that I'd had that "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.

9 Sep 2015

Not ready for wrinkles: My facial rejuvenation with Juvéderm CosmeticFillers

The years zip by so quickly, especially when you have children. You're naturally more focused on them especially, as in my case, when they have special needs.

Throughout my 30s, it was just head down, full-on searching for the right help and the right school as well as dealing with their difficulties.

Then, in my mid-forties, just as I was starting to get more time to start working part-time (besides founding Special Needs Jungle), I was knocked down by a huge and lasting flare up of Ehlers Danlos syndrome. It was prompted by hormonal changes and regrettably throwing myself into a new work challenge on top of everything else. My body ended up in a state of collapse. Since then, I've lived in pretty much constant pain, with limited mobility and that definitely takes its toll.

I remember catching a glimpse of myself in the mirror one day; it took a second before I realised it was me, worn out with dark shadows under my eyes. I looked again, a little closer, noticing the lines on the forehead, around the eyes and from nose to mouth that seemed to have appeared or become more pronounced in a short space of time. I tried to shrug it off, after all, I was getting older and lucky to be so, having lost dear friends to cancer in recent years.

But still...I didn't feel ready for wrinkles.
Sherina talking about the visia
Sherina explaining my skin analysis

Soon after this, almost as if the universe was watching, the BritMums Blogging team put a call out for someone prepared to have a consultation about Juvéderm® cosmetic fillers and in exchange, to write about it. Well,  I didn't need asking twice - this might just be the pick-me-up I was looking for!

As we grow older, our skin loses moisture and elasticity and begins to show the signs of ageing. You've probably heard of hyaluronic acid; it is often called the "fountain of youth" and can be found in moisturisers, serums and is also used medically to improve joint disorders, so it's a safe substance.

Juvéderm® by Allergan, make injectable fillers using hyaluronic acid and say it's the only way to replenish the hyaluronic acid deep within skin. Juvéderm® says it was developed to be smoother than most hyaluronic acid dermal-filler treatments. This makes it easy to inject and gives the face a very natural look and feel once it’s in the skin.

I've had facials before of course, even had a facial with acupuncture, but I didn't know much about cosmetic fillers. However, thanks to Britmums, Allergan and Miss Sherina Balaratnam at S-Thetics in Beaconsfield, Buckinghamshire, I decided to take the opportunity to find out, up close and personal...

It's fair to say I'm pretty thrilled with the results. Several people have said how well I'm looking and one medic I saw at an appointment had to do a double take at my age on the form when I saw her. Now that's a result, I'd say!

If my video has started you thinking that perhaps it might be something you would consider, then these links will be of use:

20 Aug 2015

Why "good enough" may never ultimately, be good enough.

My sons are now both sixth-formers and thinking about (hopefully) where life might take them. As they have Asperger's, that path is unlikely to be traditional or smooth.
Thinking back to myself at a similar age, I was struggling to see the value in anything. I didn't even know who I was or what I might be capable of achieving

It's exam results season, AS last week for us and today, GCSE. Son2 has literally not slept all night. I have been searching for inspiration for them and this morning I have found some on Quora, the question and expert answer site.

I love Quora, I've said this before.

This particular answer to the question, "Why did Google create Alphabet" (Google's new holding company for innovative product creation) is so in line with my own beliefs of what I hope every young person, including my own sons, should realise before it's too late, that I wanted to share it.

It has thousands of Quora Upvotes, and so it should. It's long, but it's worth reading if you want to be inspired and truly understand why being "good enough" is never truly "good enough".

As a special needs mum, and someone living with chronic illness, I have often had to accept that 'good enough' really is the best I can do at that moment, which in fact, makes it also the best I could have hoped for. No point in feeling guilty about it. 

Even so, we should always strive to do our best- whatever that is - to help others and to wake up, "uncomfortably excited" about our plans. Go beyond your comfort zone; no one ever did anything memorable doing the same old thing they did yesterday, or the day before.

You don't have to be great, or become a billionaire (though that would be nice!). You DO have to think laterally, seek better solutions to every problem, innovate and think creatively. Never settle for easy. Wake up hungry for better, go to sleep knowing you did something towards that.

Some days, if you're like me, that might just be getting out of bed. I might not have been able to yesterday. I am, if nothing else, an optimist. 

But while you're striving, remember: Don't Be Evil. Don't exploit or take advantage of those weaker than you. Live your life for the service of others because ultimately, helping humanity into a better future is the best we can hope for.
Page quote

Here's the answer from Quora: 
Read James Altucher's answer to Why did Google create Alphabet? on Quora

27 May 2015

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 Son2 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 sons 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.

Son1 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 crisscrossed 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 Type 3. 

 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.

Son2 with his splint for unstable hips that he wore 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.

Son2 at five months old, his 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 son was born with an unstable hip and needed to be in a splint for 10 weeks. Still no one thought of any connections. He also now has 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).

Son2 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: