Physical Wellbeing - What is Joint Hypermobility Ehlers Danlos Syndrome (hEDS)

Always Trust Your Instincts when it comes to Your Health

This blog documents my scary, lengthy journey to getting a diagnosis for what turned out to be Joint Hypermobility Ehlers Danlos Syndrome. I’d never heard of it, you may not have either, and I had to fight hard every step of the way to be taken seriously! This was a gruelling journey into fighting for my physical wellbeing regularly to the cost of my mental wellbeing!

Friends in the NHS, I know you work tirelessly for peanuts, which is ridiculous given how important your role is in our collective survival?! However, I do need to share my  experiences in order to purge some of the frustrations and perhaps save someone else...

The start of my diagnosis journey - April 2013

I managed to birth my 2nd child naturally, with only a couple of paracetamol. A goal of mine, given the insane 50 hour bells and whistle-stop tour of hell I endured with the first birth.

Unfortunately, the same cannot be said of the placenta! The wretched thing had attached itself to a load of scar tissue and despite a number of different medical staff burying half their arms - very scary - deep inside me to try to pull it out, it wasn't for shifting.

If you've ever had that pleasure, you will know what an unnervingly violent experience that was. One of the lucky dippers, chose their moment to enlighten me that my womb was so scared it would never hold another pregnancy and I would probably need a hysterectomy. Lovely news shortly after you've just given birth!

December 2013 something ain’t right

In the subsequent months after the birth, I developed severe crippling pains in my abdomen. I went to the Doctors and organised a scan, which showed scarring and abnormally thick walls in my uterus.

January 2014

It was decided to give me another scan a few months on to see if this had settled down; the next scan apparently didn't reveal very much but the pain hadn't eased up. I couldn't leave it there though, because it was making parenting 2 small children feel like a punishment and not a joy. My Doctor actually said she thought it was just a case of severe Irritable Bowel Syndrome (IBS). Having had that diagnosed since my early 20's, I knew it was definitely not!

May 2014

I managed to persuade them to do some further investigations and was given a Hysteroscopy and Laparoscopy. They found nothing abnormal about my womb?! But noted that my colon was "loaded" and decided that was probably causing the pain. 

December 2014

Under duress, I got my GP to refer me to the Heads' of Urogynaecology and Gastroenterology at the main local  hospital. After, waiting months to see these 'experts', both of these men again diagnosed IBS.  They patronisingly, told me to face the facts, I'd had 2 children, wasn't getting any younger,  this level of pain was to be accepted and to put up and shut up!

January 2015

When I refused to take that diagnosis, it was agreed to give me a Proctogram. During which it was discovered I had a rectocele and a pronounced descent of the pelvic floor, basically a prolapse! But everything was apparently so mild no one wanted to operate. When asking what else could be done, it was suggested nothing for me there but I could try the University of College London Hospital. 

February 2015

Of course, I couldn't live with it because it was a daily living hell! I was too unwell to work, I'd lost the patience I needed to mother the way I wanted too and had no desire to live on strong painkillers for the rest of my days - I wasn't even 40yrs old!

On a practical note, I live rurally so am dependent on driving, which doesn't mix well with strong painkillers! So, I returned to my Doctor thoroughly exasperated and depressed and asked to get a referral to University College Hospital London (UCLH).

April 2015

Out of a combination of desperation and pressure from my parents, I went for a private consultation. This doctor recommended the pill for my heavy and unbelievably painful periods; it didn't work and sent me loopy.

May 2015

The doctors and consultants at UCLH  were wonderful, they took me seriously and proceeded to run a series of tests. I underwent tests from then until January 2016, some of the most degrading, humiliating experiences of my life to date.

I kid you not, one involved me sitting on a pretend toilet in a room with 3 medical staff having a poo! But bless them, it wasn't their fault, I tell you this to show how committed I was to finding out what was wrong. Each test showed there was something wrong but not necessarily enough to act upon surgically. I decided I’d had enough needed a hysterectomy to remove this pain.                  

April 2016  - Eureka moment                               

2 1/2 years in, whilst temping to see if I could manage to hold down a job, I caught up with an old friend/client.  After updating her on why I'd been in hiding for the last few years, she said it sounded like a condition she'd just been diagnosed with, Ehlers Danlos Syndrome. She gave me some information, which for once I actually remembered to look into - perhaps because I was desperate for answers.

Unbelievable coincidence

Quelle surprise,  it looked like I did have this genetic condition, which would explain not only all the existing painful problems but a life's worths of seemingly isolated issues. I contacted the private hospital she recommended and discovered that I'd have to pay, at vast expense, to repeat all the many tests I'd had done at UCLH in order to get a diagnosis. As most of them were so vile and humiliating that this wasn't a desirable option. When I questioned the receptionist about finding someone on the NHS she said there was a consultant who was an expert on this, it turned out to be my consultant at UCLH?!!!

June 2016

I immediately contacted my consultant and said I believed I might have this condition, which could explain all this pain etc, and asked her what she thought. She agreed it did sound plausible, she hadn't been aware of all my other symptoms. She referred me to the Head of Rheumatology.

I had lost faith in my consultant and UCLH now and the cost of attending all these appointments in London was astronomical, in terms of finance and time. They had also caused no end of stress, having to juggle childcare, school hours and work. I therefore asked to be transferred back to Haywards Heath's Princess Royal. 

September 2016

The diagnosis was confirmed, I had Joint Hypermobility Syndrome, Type 3 Ehlers Danlos, a connective tissue disorder. Which, basically means that once things stretch they don't pingback due to poor collagen, my skin is very fragile and I scar very easily. 

2017

I'd love this to be the end of my story, but it certainly isn't...

With my diagnosis, I suddenly found my pain being taken seriously at UCLH and my minor issues being seen for what they were - a cluster fuck! A hysterectomy, quickly became a very real option to treat a number of the problems but unfortunately my condition stopped it from being straight forward.

Additionally, having navigated once through the complexities of having an operation 2 hours from home with little childcare cover I wanted the operation to take place closer to home, so I also started the process more locally at the Princess Royal.

Hysterectomy finally

Fast forward to the 31st October - 'the day of the dead' and I'm actually having this much-needed operation, though only after out of desperation, I'd put Myself on the cancellation list. 

If you only take one thing from this Blog take this..... Always keep checking the cancellation lists if you really need an operation - no one will tell to you that.

Be your own doctor

During the months it took to organise the damn thing, I was given contradictory information about the type of hysterectomy I should have by each different consultant I saw - I never saw the same one twice.

Full filleting - aka full hysterectomy

Finally, through all the confusion and personal research, I made up my mind to have the full filleting, I wanted nothing reproductive left in my body. After 4 1/2 years of waiting to solve my pain puzzle, I just wanted everything shut down and gone.

Last minute u-turn

However, 30 minutes before this major operation, the Consultant performing it, whom I'd never met before, told me he wasn't going to remove my ovaries. Why? because he felt that I was having enough "done" and a oophorectomy with my condition would fast track me to osteoporosis!

He asked whom had recommended all this could be done at once, and then took the opportunity to enlighten me that the surgeon I was on the waiting list for, wasn't currently  operating on anyone for the time being (nice to be told - would I still be waiting?) due to something that didn't sound too wonderful - lucky escape?!

Googled decision

But back to the story: Key question: Why had no one had thought to tell me about the high risk of osteoporosis in ALL the pre-operative appointments??

I asked my husband to quickly Google this to confirm it - ridiculous... but if it's good enough for my GP! After a 5 minute read of Google, my husband said yes perhaps I shouldn't have this done at this point in time, so I agreed...I confirmed this just before being put to sleep for my major operations!

Always trust your instincts

I woke in recovery in unbearable pain, the solution to which being bucket loads of morphine which just weren't cutting it. I knew something was wrong but was told what I was experiencing was normal. I looked so unwell and was groaning from pain so loudly that upon returning to the ward I was put into a private side room. Here, I was given the usual food and drink but couldn't face eating because I felt so rough. The combined effect from all the morphine and dehydration, courtesy of the prerequisite empty stomach, which was now about 36 hours in, was making me feel extremely sick.

They did discover, during the operation that despite all their previous investigations that I had a major rectocele, not a minor one and a full womb prolapse! None of which had really shown up in all the scans and my word wasn’t enough evidence!

Self healthcare

I knew enough to know that I needed to rehydrate to recover any appetite, and so asked the visiting doctor for a saline drip, she responded by asking me why I felt I needed one?! I pointed out that I was clearly dehydrated from enforced and prolonged abstinence (I'd been in recovery for hours because they didn't know what to do with me) and that coupled with the heap of morphine I'd had on an empty stomach was making me feel dreadfully nauseous. She agreed it was a sensible idea and organised the rigging up of a drip - which did make me feel improved enough to eat a bit and drink.

Bed shortage nightmare

The part of this torrid tale, comes in the form of a bossy middle-aged nurse, who having just come on shift seemed determined to oust everyone from their beds and home as soon as possible. It was less than 24 hours since I'd had my operation, and despite my telling her I was still in awful pain and that I knew something wasn't right, she whipped out my catheter and told me to go to the loo.

No spare capacity to deal with rudeness

She was so irritating and brusque I just wanted to get away from her, so I did as I was bid. I couldn't sit down to do my business properly, but that was apparently good enough and I was discharged. I did complain to her superior about her on my way down to be picked up!

During my wait for my husband to pick me up, which of course was alone and not in a wheelchair, which I should have been,  I ran into my kids' swimming teacher - great! She asked me what on earth was wrong because I looked so stunningly awful. I kid you not my entire body was blanched of all colour and stayed like that for the following 2 weeks until.....

Fighting for my life

After ever mounting pain levels, and the advent of total incontinence, I landed up in A&E in floods of tears begging to be catheterised - not something that was on my bucket list believe me. I also asked for a scan, which showed that I had a large hematoma that was blocking everything.

It pays to know what you’re talking about

I was immediately re-admitted for observation, but over night my haemoglobin levels dropped to a dangerous level and had to be  'ambulanced' off to a more major hospital. I knew I needed this hematoma drained, but the 'normal' approach was to wait and see if it went down on its own.  After a lot more tears, begging and some very strong language 36 hours later I was finally operated on.

Surprise surprise

When I awoke I immediately felt better and how I'd expected to feel the first time around. But life had another surprise for me, my original chosen surgeon appeared, as if by magic, and explained that he'd just performed the latest op.

Apparently, it was the right call because the hematoma had created its own force field and could never have drained away naturally.

The honesty of morphine

In a stupefied haze, courtesy of yet more morphine, I responded by saying 'but it's you? I thought you couldn't perform surgery anymore because you did something wrong?'.

The poor man's smile was immediately wiped from his face, and he clearly then felt he had to explain his personal history. Still high as a kite and so pleased I was back to my normal pain-free self, I didn't care about his mistakenly accused wrongdoings - he was my saviour - which I told him. But, I did feel mildly uncomfortable having put him in this situation given the help he'd given me and like a nasty shit stirrer.

How to keep your hospital bed

Again, all was well until another one of these highly annoying middle-aged bossy nurses came on shift and ousted me again within 24 hours of my operation; the pressures on bed spaces is very poorly placed.

There was a couple of older women in the beds opposite me who knew how the play the game. I could tell they'd been in the ward for a long while, they both had personal items galore upon the furniture around them and knew everyone by first name despite the shift changes. They simply refused to play ball, wouldn't get up and do their recovery exercises so couldn't have their catheters removed and so couldn't go home but remained politely obstinate during all the bullying and cajoling that was going on.

Will this hysterectomy nightmare ever end

Again, I'd love to say "the end" but AGAIN 2 weeks later I was experiencing bad pains and was forced to do another crack of dawn flit to A&E.  I was now being recognised by the staff!  

And a scan revealed another hematoma, and the additional bonus prize of a hemorrhagic cyst. Apparently, both common outcomes post hysterectomy for the unlucky - Me!

Oophorectomy in the end - August 2018

So, I did have to have my ovaries removed and fell fast into choppy boiling waters of menopause. This oophorectomy did take another year to organise, despite being told by the original surgeon that if I did change my mind it would only take a matter of months to have them removed!!!!

Interstitial cystitis diagnosis

And, to really add insult to injury, they discovered in the oophorectomy operation that all my bladder pain was down to having interstitial cystitis, a bladder disease?!

Ketamine abuse?

This time I came around to the surgeon asking me if I had a drug problem? Seriously, I was again in another morphine induced haze and couldn’t believe he was asking me if I’d been abusing ketamine. Something I’ve never touched in my life. I had to ask him about it again when I met him three months later in my first follow up appointment, when I was actually sober.

Still in pain

Unsurprisingly, because it's me, the pain actually didn't really go after the two years its taken to get through the menopause. Given all the antics I went through to get to this point, I put off going to the doctor as long as my mother would let me. I am now on a 12 month waiting list, again. 

Histamine intolerance diagnosed Sept 2018

A clever Herbal Doctor, Alice Bettany from Sacred Seeds, diagnosed my intolerance to histamine rich food, which was the cause of all of my life long bladder issues! Read my blog on Physical Wellbeing - What is histamine intolerance and what damage can it do?

The real culprit

So, once again we bit the bullet and went private, to discover that the culprit is largely my colon. Which is an overly sensitive, slow transiting bitch, created by my Joint Hyper-mobility Syndrome.

This doctor, couldn't reconcile the connection between my diet and my symptoms?! He poo pooed my histamine intolerance, despite medical evidence that the only drug that's ever had a positive effect on my bladder was a histamine receptor blocker! 

Caveat

I know and truly appreciate the pressure the NHS is under and that if I was in the USA I wouldn't have been able to afford all the care I have received, especially as I've been so unwell I haven't been able to work properly and thus earn. 

I also firmly believe that we as a nation need to recognise that our health is one of the most important things we have got. And, that in order to protect and retain it, it needs to be funded properly. 

Final word

Our health system needs to evolve, to take the best of the Western approach and blend it with the ancient wisdom of the East. Mind, body and spirit of equal in terms of achieving true wellbeing. This was some of my wounded healer journey, there are lots of other earlier experiences but these are the most recent. From this, and because I can’t pull off working for someone else because of my limiting health conditions, I started formalising my wellbeing wisdom with courses. I am now qualified in aromatherapy, medical astrology, vitamin therapy and herbs, all of which I use in my holistic wellbeing practice.

Next step

If you like my style and have a similar health clusterfuck going on, book yourself a 360 Bespoke Wellbeing Consultation and I’ll help you to work out what on earth is going on and how to achieve natural physical, mental and spiritual wellbeing.