Friday, 24 March 2017

Gastroparesis, EDS, & the final piece of the jigsaw #Part 2

Click here for #Part 1

Most of you will already know through my blog, I'm not keen on surgeons (to put it mildly) so that key moment when I first meet a new one always feels like a very tense and anxious exchange. Years of plodding through the various health systems has unfortunately made me very cynical so it takes a lot to win me over these days.

... However,  Dr G had a very open and friendly manner about him which was instantly reassuring, we felt like equals from the offset so any tension we had felt beforehand quickly disappeared. He managed to hit my top two priorities on the head in pretty much the first sentence! He explained that he had been very interested in my unusual presentation and was keen to understand more, which is always difficult through email conversation. (not exactly a demanding list but surprisingly hard to find, especially among the surgical fraternity!)

Prior to the appointment I had submitted a series of questionnaires including; a CCI questionnaire, Oswestry ScoreKanofsky Scale , a basic analog pain scale (1-10 you know the drill) and a comprehensive medical and surgical history.

One of the questions was, "list your top 5 complaints in decreasing order of importance" now obviously, for me the number one slot goes to the Gastroparesis, followed closely by the headaches/migraine. This had really surprised him,  he explained that he had never been approached by anyone with such severe gastric issues, especially as a primary concern over the headaches. I tried to explain as best I could that I have learnt to live with pain, pain is no longer a priority because I know it will always be there. I've had severe headaches for years but I've been told for so long that there's nothing wrong and no serious cause, that I stopped worrying about the severity and accepted them as part of my life (this is the short version obviously....more about this in part 3).

However, I strongly believe that ironically, the one thing that's keeping me alive will actually be what finishes me off and that's the TPN. I told him about my sepsis history and he instantly understood why that would be a priority to me.

So, having ironed out the small stuff we moved on to the scans. I had included my original radiology report with the scans so he could see what information I already had. Bearing this in mind, he went on to explain that he had re-calculated the measurements himself using the correct criteria (I will explain more about this in later blog) and the results were drastically different. He pulled up one of my images and said, "I'm sorry to have to show you this but you can see better than I can explain, yes?"

Flexion Clivo-axial Angle 132.1º (Normal Range: 150º-180º)

Neutral Clivo-axial angle 132.8 º (Normal Range:150º-180º)

I don't think either of us expected to be able to see anything significant without a detailed explanation, but as he circled the area with his pen (where the angle is marked on this image) we both saw exactly what was going on. The room fell silent for a while, I don't think either of us knew what this really meant but we knew it wasn't good. I studied the Dr's face for a moment as I tried to grasp what I was seeing and saw the seriousness wash over his face. That expression you never want to see.

He explained that the dark area between the bone and where the angle is marked is a pannus formation (layer of fibrovascular/granulation tissue) which is a direct result of excessive movement of my Skull, C1 & C2 (the top two vertebrae). Effectively, instead of pivoting on the spine my head is sliding back and forth.

He went on to say that when he first saw the images, even without going over my symptoms he presumed that surgical fixation would be necessary due to the level of brainstem compression. He then explained that your head should not "slide" at all but there is a small tolerance of 1mm which they would deem abnormal but tolerable...anything more than that is a big problem. He then proceeded to demonstrate a calculation of my results (see table of measurements below) to determine precisely how much my head is sliding. I have to admit to not fully understanding this bit at the time but it wasn't 1mm... it was 4.3mm. These might sound like small numbers but even I can understand that 4 x greater than acceptable... is NOT acceptable!

Here is a list of my measurements for those who are interested:

Cervical spine flexion: 21º (Normal 12º)
Extension: 71º (Normal 32º)
Range of motion: >90º 

Neutral CXA:132º 
Flexion CXA:132º
Extension CXA:146º
CXA (Clivo-Axial Angle) Normal Range: 150º-180º

Neutral BDI: 11.6mm
Extension BDI: 12.1mm
Flexion BDI: 11.5mm
BDI (Basion Dens Interval) Normal: Less than 12mm 

Neutral BAI: 8.6mm
Extension BAI: 3.6mm
Flexion BAI: 7.9mm
BAI (Basion Axial Interval) Normal: Less than 12mm 

Translational BAI: 4.3mm (The "sliding" motion) Normal: 0-1mm

Neutral Grabb Oak: 9.6 mm (values greater than 9mm indicate ventral brainstem compression)

Grabb Oak Measurement

He then confirmed that my head is also over rotating when I look left and right which is causing subluxation (partial dislocation) of the facet joints. 

Excessive rotation of the neck can be very dangerous in itself as the main vertebral artery runs down through C1 & C2, this image explains better than I can....

The vertebral artery is the main blood supply to the upper spinal chord, brainstem, cerebellum and posterior part of the brain so I'm sure I don't have to spell it out that damaging this could be catastrophic. Thankfully there is no impingement of this artery, but the excessive rotation is definitely playing its part in my overall symptoms, causing irritation and on it's own, carries significant risk. 

Obviously all this information was way too much for us to fully understand at the time but neither of us was left in any doubt, we both fully understood what it meant underneath, regardless of the science.

If you're still reading... WELL DONE! I'm sorry to bore you with such detail but there was no other way to write this blog...believe me it's taken a few attempts! I think the crash course in neurology is enough for now so I will continue in Part #3 with the final outcome. 

Friday, 10 March 2017

Gastroparesis, EDS, & the final piece of the jigsaw #Part 1.

As most of you here know, I've been struggling with gastroparesis for the last 7 years. It's a very long story but this led to the diagnosis of Ehlers-Danlos syndrome. For those new to my blog... Eds is a genetic condition which makes the collagen in my body weaker and stretch more than it should.

I was told as soon as I got the diagnosis, that Eds was the probable cause of my gastroparesis. No one explained why, mainly because the link is not fully understood, even in today's world where we can grow tissue in labs and print 3D parts from a computer!

I've spent so many of those years in between, trying to find that elusive link and believe me when I say...I've read about every weird and wonderful theory known to man, every useless trial, and every obscure symptom. I've spoken to others with the same issues and heard their stories of how it all began. From testing new drugs, surgery and some rather suspicious looking electrical devices, believe me when I say I've tried it all! Anyone would get down hearted when you've been through all that and still have no answers and still no cure.

BUT... In finding out those things that didn't work, and didn't go to plan we eliminated so many possibilities and forged new thought processes from the myriad of "unexpected" symptoms.  I can say that we are closer than ever before to understanding the actual connection!

Most of you know I've had to dip in and out of private services to get the answers to ALL the big questions and yet again I came to the point where the only way to put that last piece in place was to seek private help outside the uk! I took the plunge several months ago and had some specialist MRI scans done. I got a written report which highlighted that my top two vertebra over rotate and sublux (partially dislocate) when I turn my head to the left and right. Alongside a huge range of movement beyond what is "normal" when bending my head forward and backwards. This leads to continual damage to the nerves that control just about EVERYTHING that we do without thinking!

Due to the complex nature of this condition alongside Eds complications there is no hope of getting help through the NHS at the present time and very little knowledge within the uk as a whole... including accurate diagnosis! So yet again I was left with a dilemma, Knowing what could be causing all my issues but no specialist with enough knowledge to tell me what to do about it. Through talking to others online I joined a group and found all the information I needed. I did some research and sent my MRI images to a leading neuro-spinal surgeon in Barcelona who has worked with EDS patients... a very rare find! He responded to my email promptly requesting that I make an appointment to see him in person because he had seen more in my scans than what I had initially been told.

So, in mid December hubby and I embarked on a journey to see the man himself. A 24hr marathon appointment dash across Europe right before xmas! At the point of booking, I sat there wondering if I was doing the right thing, but I wasn't getting any clear advice here about what I should be doing or indeed NOT doing to help the situation. From what I had read, my scan report seemed fairly minor and I was struggling to understand how they could be so different so I honestly thought that the trip was maybe a little excessive but by this point I just needed to know!

The journey out was pretty tough going, I've only flown once since getting sick so it was a first for hubby and I on our own.  Thankfully we have now mastered the skills of travelling with TPN so that wasn't so daunting this time round! We got to experience the joys of the freezing cold airport translift (a box van on stilts for those who can't manage the aircraft stairs!), a first for both of us. Then we proceeded to get stuck on the plane after landing at Barcelona because someone had sent the translift to the other side of the airport, holding up the flight back for everyone waiting! Followed by the most insane taxi ride known to man...queue crazy taxi driver...120mph...while texting!!!!!! I've never been so pleased to reach a destination in all my life!

Vilana Hotel

Freezing cold and ready for a well earned rest I laid down and slept....for roughly 40 minutes before I was awoken by a dream where I had overslept and had missed the appointment...after that, it was a very very long night!

Thankfully the hotel was just up the street from the hospital so we had no trouble finding where we needed to really can't miss the place!

Hospital Teknon, Barcelona

It looked more like a palace than a hospital. We arrived in plenty of time so had a fairly long wait, everyone knows what that's like....ten minutes can feel like an hour in a Drs waiting room! However, finally, in what seemed like an eternity we were called through.

To Be Continued....

Click here for #Part 2

Thursday, 27 August 2015

The Sick Role

I know I've rather slowed down with the writing. I always have plenty to say but sometimes I need to keep things to myself a bit. However I do realise that not writing means that you guys miss out on all my wonderfully happy stories! So I thought I would top you up with the joys of my life and all the little challenges it brings. Today I'm merely ranting out loud but I promise to post a more personal blog soon....

I've been thinking a lot lately about other peoples perception of what my life entails. Obviously not those who actually take the time to find out, those who send me messages because they've not heard from me in a guys are fine! No, I'm talking about those who choose to judge from a thousand paces purely on the fact that you have put a little make-up on!

Just want to get this out there... YOU GUYS SUCK! Big time!

What is it with this obsession that sick people must be:

a) miserable   (sick people obviously NEVER smile)
b) pale   (anything warmer than casper the ghost and your obviously fit and healthy)
c) unable to walk (omg...your not chained to the house?)
d) Totally useless (what, you can still do that...really?)

Just a few of my personal favourites but you get the idea. Well, maybe when you have flu, you behave like that for a couple of weeks. Take a day or so off work and hide under the duvet and it works for you, right? Yeah...

Now relate that to my life...

a) Do I want to spend the rest of my LIFE being miserable?
b) Do I want everyone I meet to tell me I look like Morticia Adams?
c) Do I want to live my life, or rot my brain on day time TV?
d) Everyone needs a purpose!

I don't want to spend my life hiding away in a duvet fort, looking like shit, crying all day feeling sorry for myself because I'm so useless. Maybe you like to do that when your sick for a day or two but if that was forever...would that still be your strategy?

Of course not!

So why judge me negatively for trying to do as much as I'm able, smile as much as I can and try even though I might fail. It's my choice to fight back, whether you like it or not. Simply because I choose not to let it beat me does not mean that I am well. I merely choose not to share my pain with people who will never's too short!

Saturday, 21 February 2015



Fuming doesn't even come close to how I feel right now. Life has been SO hard lately and getting all this down on "paper" in a coherent way might be a bit of a challenge but here goes!

Rewind back to November (2014) my jej tube started to misbehave. It started with a niggle and what we thought was granulation tissue around the stoma. So I told my GI and went to the gp for some silver nitrate to burn off any troublesome tissue (nice huh). The granulation disappeared but the pain was getting worse and now it was weeping all the time too (infection was ruled out straight away).

So, step two was to ring my team and get someone with experience to look at the tube and find out what was going on. December, I get an "urgent" appointment to see the surgeon who initially placed the tube for me, by which time I was having to take the dreaded oramorph to be able to get to the appointments!

My mum and I attended the appointment together like we always do to make sure everything is covered. We arrived at the clinic to be greeted by a registrar instead. No problem, he's a surgeon too right? NO…. just NO! He was about as much use as a chocolate tea pot and really didn't know what to do other than order and ultrasound to check for fluid collections. Fair enough, that's fine, just do it quickly PLEASE!

It's nearly christmas.

A week goes by and I'm getting worse not better. no sign of a scan and the response from the tube nurse is, "it's christmas, there's nothing available." So she arranged for me to go and see the surgeon (who we should have seen in the first place) the next day.

As usual, my mum got the job of taxi service! We arrived a little early but when we got there it appeared that he hadn't been informed of our appointment because he hadn't checked his email. Great start!

When he did finally arrive and check things out he decided it had to come out. The tube is eroding on the inside and the skin is mottling around the site. It feels like someone is pulling it from the inside, causing my abdominal muscles to spasm right down to my pelvis. It REALLY needs to come out!

I go home knowing that I would have to wait until after christmas, which was obviously not what I wanted to hear but I get it, winter is a strain blah blah blah.

Well, on xmas eve, life decided I really didn't have enough to deal with so it handed me a whopping dose of the flu…..yeah…..thanks! So I spend xmas day wrapped in an electric blanket in the corner with Freddie krugger having a pop every time I needed to cough, move or shiver. The oramorph had completely knackered the rest of my gut so I had to stop taking that as well. (MERRY CHRISTMAS!)

Fast forward to today (feb 21st) What's been done?

Erm, well?

I was ringing the tube nurse every fortnight until last week to find out if they had a date. Not unreasonable, not pushy I just need to know when because Im really not coping with this.
In our last conversation (after the usual flannel… bed shortages blah blah fucking blahhhhhh) she told me that she couldn't tell me when it will be, neither could anyone else and she didn't want to be piggy in the middle anymore and that I should try and find out myself through the secretaries!

Oh my, I really can't take much more of this telephone ping pong!

I rang the secretary straight away and explained what had been going on. Horrified by the situation she went off to find out what was happening. GREAT finally! She rang back the following day to say that she still couldn't tell me when it would be but I had an outpatient appointment coming up (today) and I should raise all my concerns when I see him.

I thought to myself, why do I have an appointment with him again when he has already decided what he needs to do? But in my naivety I thought it was because he wanted to see the stoma again since its been 3 months. Plus, the tube has started leaking so I've been tying a rubber glove over the end of it to stop the (sorry for the graphic explanation) "shit" from leaking all over my clothes!!!

Yeah that was dumb…. dumb dumb dumb!

Today my husband and I went to that appointment. We walked in, sat down and asked… when are you going to sort this out? Erm…. I can't tell you I don't know!

(Is it me?)

Why have we come to this appointment then? Erm… I don't know, someone must have booked it by mistake!

(Really is it me?)

I asked him why he couldn't tell me and he explained that I'm complicated and they want the right people there etc etc blah blah blahhhhhhhhh.

So I politely reminded him that IF I didn't have a problem with local anaesthetics it would be a quick procedure.

He agreed. Yes you could have had it done straight away as a quick in and out if you could have local anaesthetic (they don't work on me).

So that being said, what exactly is the complex part of this?

He said, " I'm not an anaesthetist so I can't tell you. All I know is my part of the job but given your allergies etc this makes the anaesthetic a higher risk."

What fucking allergies? I'm only allergic to cyclizine, nothing else…zippo zip!

We were about to leave when I asked him if he was even going to look at the tube or not. If I hadn't asked he wouldn't have even thought to look.

The only solution he gave was for the leaking, so they replaced my  practical waterproof glove with….gauze?… so it can soak into that, and THEN my clothes! EXCELLENT IDEA…ugh…aaaand the rubber glove is back. I look so good right now!

All in all I've dragged my family to Leeds 3 times now and I'm no further forward than I was when I started!

If you read all that and followed it your better than me ; ) Sorry for ranting but Im so so sick of having to fight for every single thing. It's just wrong and I know it's not just me, it's everyone. Is it so unreasonable to ask your practitioners to keep you comfortable in the absence of a cure????

P.S. Oh and the scan… the urgent one….ordered in december… yeah I had that at the beginning of February!