EDS and the shame of our health service

 

MILLIONS of people world-wide are let down by health services continuing to dispute well-grounded international scientific research into the serious genetic disorder,*Ehlers Danlos Syndrome (EDS), and Hypermobility Spectrum Disorders (HSD).

*EDS refers to a group of inherited connective tissue disorders that affect various organ systems.

                                                 The zebra symbolizes the rarity and uniqueness of EDS and HSD,

                                and it serves as a reminder to consider less common diagnoses 

                                                                 when evaluating symptoms. 

“No other condition in the history of modern medicine has been neglected in such a way as Ehlers Danlos Syndrome” – Professor Rodney Grahame, Rheumatologist.

Professor Grahame advocates for a holistic approach to managing EDS. This includes addressing not only physical symptoms but also considering the impact on mental health, quality of life, and overall well-being.

He encourages doctors to listen to their patients, validate their experiences, and provide comprehensive care.

I’ve written about this condition in this column before – concerning our daughter who is afflicted by EDS and associated chronic illnesses including POTS (Postural Orthostatic Tachycardia Syndrome) as well as with Autism.

This time I am prompted to do so during EDS Awareness Month, which opened with a major gathering of researchers attending the recent HEDGE (Hypermobile Ehlers-Danlos Genetic Evaluation) Study Update held in April in New York, USA.

The  concern is that most doctors continue to 1: misdiagnose; 2:dismiss the science, telling patients it’s all in their head or; 3: and, this is perhaps worst of all, know something about the condition and yet won’t get off their backsides to learn more and so help those afflicted.  This is leading to serious mental health consequences.

It is an acute problem in Britain where one of the leading advisors is Jeanie Le Bon, an internationally recognised Movement Therapist specialising in Hypermobility, Ehlers-Danlos Syndrome and Chronic Pain.   (jeanniedibon.com)

She, too, is a chronic pain sufferer who has a lifelong lung condition called bronchiectasis, as a result of three episodes of pneumonia.

She also has EDS and Mast Cell Activation Syndrome (MCAS), *Postural Orthostatic Tachycardia Syndrome (POTS) and Chronic Fatigue Syndrome (CFS).

*POTS: when slightest movement causes adrenalin rush sending heart rate sky high while blood pressure drops alarmingly low, leaving you feint and giddy.

 

 

Le Bon highlights the work of Professor Rodney Grahame in Britain, who is

contributing much to research, clinical practice and education.  Professor Grahame is a distinguished rheumatologist with extensive expertise in hypermobility disorders who is striving for greater understanding in the wider NHS.

What does it mean to those who come face to face with doctors who are unable or simply refuse those to help those ill with these horrible conditions.

Here is an extract from Charlotte Twinley’s story on t

; the diversity and ability blog.

https://diversityandability.com/blog

“I was told it was in my head”: Life with Ehlers Danlos Syndrome

by Charlotte Twinley

 “It can take over 10 years to be diagnosed with Ehlers Danlos Syndrome (EDS).

I was diagnosed after about 5 years by pure luck.

When I was 10 years old, I started to have the odd stomach pain. After various doctors’ appointments and tests, nothing was found. It was suggested I tried going gluten and dairy free on odd occasions, which helped – sort of.

At 12, I started to injure myself a lot more whilst playing netball or hockey at school. Everyone just assumed I was ‘getting too into it’ (which I was, but that’s beside the point!).

Two years later, the stomach pains became worse. More appointments, referrals and tests still showed nothing. I had an endoscopy (where a tube with a small camera on the end is inserted down the throat to look at the inside of my stomach), which came back clear.

A couple of days after an intense hockey training session, I woke up and couldn’t move my legs. My dad carried me to different doctors and I had an MRI which, again, showed nothing. The doctors had no idea. They gave me a pair of crutches to drag myself around with, hoping that it might improve as I could still feel my legs. A week later, I managed to wiggle my toes and gradually – literally step by step – I started to walk again.”

 

Eventually, a consultant found out she was hypermobile (extra bendy) and often injured playing sports at school. He then referred her to a specialist in London.

Many more examinations followed and eventually they led to a diagnosis.

However, there is no cure for EDS and management of the condition is essential – and yet physiotherapists with the right skills are few.

 

In my family’s case, our local surgery was asked by the Royal Orthopaedic Hospital to source a specialist physio for our daughter. This was because they judged her too weak to take part in the Hospital’s rehabilitation program, until she had improved.  Our local medical team failed to respond.

That was 12 years ago.

 Similarly, the consultant at Kings College Hospital who diagnosed her with POTS and also requested our local doctors keep an eye on her. They have failed to do so…unless specifically requested to make a house visit for a “normal” illness, perhaps requiring antibiotics.

To cap the neglect, Surrey mental health services have also denied her psychiatric support because they don’t do Autism!!!!

 

Some facts for you.

EDS leaves you with weak connective tissue which can affect every organ in the body – causing bendy limbs which cannot support you. Or dislocation in some cases.

There are variations:

Classical EDS includes a skin condition – 1 in 20,000 people.

Hypermobile EDS: weak connective tissue, resulting in bendy limbs, and risk of dislocation in some cases. 10 to 30 per cent of the population.

Vascular EDS, the most serious, leads to weakened blood vessels and is estimated to effect 1 in 90,000.

 

 

 

 

Labour to nationalise rail network

 

ENCOURAGING to see that Labour “pledges to nationalise rail network within five years”, taking back the system  from private ownership created by the Conservatives  nearly three decades ago promising cheaper tickets!  Instead, it  led to spiraling ticket prices.

Of course this depends upon Labour winning the upcoming General Election, which everyone expects them to do.  

This is a distinct possibility after Labour's huge victory in Local Elections, which for the Conservatives should be re-named the Bye Bye Elections.

And we must hope that the outgoing Conservatives do not throw all their toys out of the pram and completely trash the economy as a going away present.

We really do need for these deadbeats to be shunted into the sidings, thence into the breakers yard.

If Labour succeeds, can we hope they will do what successive governments have failed to do, and provide the investment necessary to make the railways fit for purpose?

Labour's challenge is to create a successful 
economic model for the future of rail

The system has always lacked decent government investment.  It never recovered from being run down during the Second World War, after railways played a vital role shifting soldiers and supplies.

The system was further damaged by the infamous Beeching Report in the 1960s, which resulted in the controversial decision to shut thousands of miles of routes and stations on the basis they were unprofitable.

There were those who thought Beeching’s report was used to “injure” the railways, knock them back, in favour of encouraging the expansion of the road transport industry!

This was a controversial period if ever there was one with one key player at the centre of it.

This was tax dodger Ernest Marples – although we wouldn’t know about that until he had left his job as Minister of Transport.  Marples later did a runner, left the country in a rush, to avoid prosecution for tax fraud.

As Minister of Transport he oversaw major road construction and also the closure of a huge tract of the rail network given the death sentence by Beeching.

He clearly had conflict of interest, for he had been managing director of construction company Marples Ridgway and even when he gave up that post it was rumoured he still enjoyed a nice not-so-little earner.

Shortly after he was elevated to the peerage, the golden boy of the Tory party scarpered to avoid the police at his door. 

He rushed for  a night train for the Continent – fortunately for him it was one of the lines not closed under Beeching.

In the meantime, the Beeching story fed the British public was that a new slimmed down rail network would enable them to concentrate on improving the main trunk rail routes. Except this ignored the role many of the closed lines played in feeding people and goods to the main lines.

The cuts had gone too far. And over the following three decades the railways continued to suffer from lack of proper investment.

France, as I understand it took a different view of their railways worth.  The railways role was to enable the country to function, contributing to the wider economy by transporting people and goods. And therefore, while it must remain accountable, it would never be denied the investment to do that.

In Britain, meanwhile, the drama took  another twist. Enter, stage right, the Conservatives who made the ill-fated decision to privatise in 1997. It was probably just another opportunity for their mates in business, to make a buck or several.

Basically, the move failed the litmus test because the government’s promise that privatisation would take the cost of running the railways out of the public purse was wildly over optimistic. 

For at the point of sale there was not enough revenue in the rail system to meet the new operator’s costs, capital investment and claims of shareholders.

Low and behold, they were soon subsidised to the tune of several £bn of public money and a tidy sum of that would be go into shareholders pockets! More public money than ever was being poured into our rail system. But this time, we, the tax payers, were also funding shareholders and rich businessmen!

Bizarre.

I’m no expert, but here are a few of the pros and cons of that privatisation, gleaned from articles covering the subject in depth.

The Conservatives promised that privatisation would lead to better and cheaper services and also reduce the public subsidy.

Well, passenger numbers did increase as more services were provided.

But on the down side punctuality suffered and fares sky rocketed by 24 per cent, making rail travel in Britain the most expensive in Europe, if not the world. So, failure.

Transport journalist Christian Wolmar, in one of his many stories about this sorry episode, said it was clear that the new management would attempt to boost profits by restricting spending.

And therein lies the single biggest flaw in the privatisation of public utilities, as we can see with the current Thames Water crisis. The company has been pouring so much raw sewage into our rivers fish are dying and human health is threatened.  They are accused of prioritising paying shareholder dividends over investment and they are now £15bn in debt!

Or the country is, because the tax payer may pick up the tab.

Same story with the railways.

The new rail management under privatisation got rid of experienced engineers and as the numbers of trains increased, track maintenance suffered and there was an increase in broken rails, which led to the terrible crash at Hatfield.

Costs soared across the network and there was no longer the expertise to identify which other tracks were at risk of catastrophic failure.

After Hatfield, Railtrack, the company formed to maintain the rail infrastructure, was accused of not being up to the task and responsibility was transferred to quasi-public corporation – Network Rail.

One reason why the whole shambolic exercise failed was because privatisation put shareholders’ interests over that of rail passengers.

One a lighter note, the one thing the privately run railway did excel at -  for besotted train spotters at least – was the introduction of so many different colour schemes representing the different rail companies.

So if your train was cancelled and you waited in hope for the next one, you could at least gaze in wonder at jazzed up diesel and electric locos as they rumbled through.  Some of them became works of art.