Posted on October 1, 2017
So it's been a while since I updated, please forgive me for this as I frequently update the www.facebook.com/savingsamantha page because of the ease and large following, however, updating here means I must log into a laptop or such.
Anyhow, much has happened since my previous update when I was told of the need for further surgery, and this will be a long post so that I can back date and keep some clarity. I will copy and paste extracts from my Facebook blogs and date them below for your convenience.
AUG 12th 2017
Surgery is now booked for Saturday 19th. This gives a more reasonable amount of time with regards to booking pre-surgical tests and anaesthetist etc... but it also gives me a week of waiting and knowing from experience what I'll have to endure.... eek.
This next surgery is further up my spine. It's nearer to my brain stem and I'm scared of the risks involved, I have deep fears around the lack of pain relief but mostly I'm concerned about the vague nature and uncertainty of the whole situation.
EDS is misunderstood by so many in the healthcare systems world wide.
It is a connective tissue disorder;
The human body is composed of just four basic kinds of tissue. Connective tissue is the most abundant, widely distributed, and varied type. It includes fibrous tissues, fat, cartilage, ligaments, bone, bone marrow, and blood.
As the name implies, connective tissues often bind other organs together, hold organs in place, cushion them, and fill space....
Knowing this, it's glaringly obvious that ALL specialists in ALL sectors of the healthcare system should have a wealth of understanding about how faulty connective tissues can wreak havoc in their specialist area.
So we (The EDS community), are left fighting for someone, anyone, with an ounce of interest to help us... not just in one part of our body but in every last area.... and this means travelling the world for that rare gem with healthcare training, who has had their eyes opened to the cruelties of the condition... and wants to learn and to help... it SHOULD be mandatory in healthcare training- it isn't.
It's not good enough.
Even the experts don't know enough to be genuine experts.
It's still early days in understanding the full complexities of EDS so in many ways it's a guessing game, trial meds, trial surgeries and hopeful doctors and patients....
So when my neuro-surgeon tells me that he's doing his very best to help me, but that in many ways he has no answers...
That he's just hopeful that this next op will be the one to work for me....
I die a little bit inside... my hope falters... and I'm only enthused again by the sheer knowledge that at least one of my babies has this condition and there is NO WAY on earth I will allow them to walk this path as blindly as us before them.
We absolutely MUST change the system at the highest level. There are far more people with EDS than are diagnosed.
The vast majority of us suffer without answers, or with many misdiagnosis' (fibromyalgia, chronic fatigue syndrome, anxiety disorder, pain disorder, arthritis, auto immune conditions, IBS, palpitations, hyper-mobility ....).
In my case, I knew I had EDS for years before my doctors ... I've known about 5 years . My doctors refused to send me to a geneticist.
I have the letters by my side from august last year when I refused to leave my rheumatologists office with more meds and no answers...
I was struggling as a single mum to keep my business and my life functioning any longer.
I stated that I would not leave until she referred me to a geneticist .... she begrudgingly accepted.
The referral letter that followed read like this....
"Samantha has convinced herself that she has Ehlers Danlos Syndrome. I have tried all avenues for treating her symptoms with no success.
I have told her on numerous occasions that she has hypermobility, chronic fatigue and fibromyalgia; she refuses to accept these diagnoses.
Please see Samantha for an assessment with the genetics service to settle her mind.
I will no longer need to see Samantha as I have exhausted all treatment options"
"Specialist in this area"
And just like that I'm left with no specialist.
BUT- I got my appointment December 23rd 2016, and the geneticist told me I'm one of the most obvious cases of EDS she's ever seen, that there's no cure and maybe I could consider saving money and going privately to London where there's a small team able to advise... The geneticist had more empathy than this - she even followed up a few month later with a call to see if she could help me and how I was getting on... but that's the general jist.
(I made it to London in Jan 2017 and was then referred for testing, again privately, to confirm this CCI / AAI and a need for surgery).
The exact same day that I received a letter from the few that understand EDS clearly stating that I have a 'Grossly abnormal cervical spine', I received the results from an NHS MRI on my head and neck...."results normal. No concerns"....
This is what we are contending with.
It took 30 years to get to the answer -and that's a typical story leading to diagnosis in the U.K. having spoken with others.
Had I had the knowledge and care that my body needed we maybe could have avoided this stage.
Something has to change. Watch this space.
Aug 15th 2017
Local press release (click here)
Aug 16th 2017
My operation date has now been brought forward to this Friday 18th August....
At present my c1-2 and possibly my Occiput are 90% dislocated ... this traps my vertebral artery and other veins and arteries supplying blood & oxygen to the brain, keeping me at risk of internal decapitation and is believed to cause many of the symptoms which have left me bedridden in recent weeks.
During this operation, the surgeon will stabilise the C1 & C2, hard fuse them and decompress my spinal cord.
You may also be able to see the connection between the C1-2 spine and my Jaw on the image below; My Jaw has been locking and dislocating many times a day since the last surgery and this will hopefully be addressed within the next two weeks.
We are still unclear on the issues surrounding my spinal cord thickening and my brain blood flow.
We are hopeful that this operation may address many of my issues for the time being and that this could allow for a return home and a few months rest and rehabilitation (& fundraising!) around loved ones.
It is this operation that has been the catalyst in the increased fundraise and deadline.
Thanks... Sam x
Aug 19th 2017
Update from Samantha's Dad
Today was stage 2 of surgery to fuse C1-C2
Over the past 2 or 3 days leading up to today we have noticed Samantha appears to be coping better. Not entirely pain free but at more manageable levels, so it seems somewhat a shame to now have to go back under the knife to inflict yet more pain.
We have had this discussion but the alternative is out of the question if Samantha wants some form of normality. So fingers crossed here goes...
Today's surgery was early for all those who know Samantha getting up at 4am is a tough ordeal but to our surprise she was raring to go. Surgery was at 7:30, the nursing staff put her entirely at ease, the contrast from the previous hospital could not have been greater.
Surgery was 31/2 hrs long. Dr Henderson spoke to us after and stated all went well. She was in quite a lot of pain but was quite responsive. He has also said that her Occipital nerve was crushed and he has had to remove it, this could explain why she had been in so much pain.
Having since spoken to Sam she definitely seems more positive about the future still in tremendous pain and suffering from muscle spasms but says although early days it is different this time around.
Here is hoping for a speedy recovery...
Many thanks for the continual support
Aug 24th 2017
Hi all, short one because I'm really not in the frame of mind to message.... I wish I could share news of learned lessons and successful analgesics .... I can't.
Dominic, Myself and my family worked hard to put a list together for care after this op, recognising what would & wouldn't work from last month.... unfortunately the hospital had other plans.
Yesterday, I had an experience I'm not proud of and I'm not emotionally strong enough to share.
My gosh I was frantic with fear .
Today I spent the day in excruciating pain from 4am through to 6pm when DrH finally managed to organise IV medications (more trial and error pain management.
I can not and will not ever try to fathom the pain I was under.... I still am only now slightly relieved as the edge is taken off a little.
I'm not scared; I'm petrified. And I'm internally beating myself up for writing this yet again... god, I wish I could share positivity.
I am just so so thankful for my family, Dom and friends for keeping me going.... one small millisecond at a time.
Ps. the bruise is from my head clamp in surgery. I have blood filled ones around my skull. Nice 👍🏻
Pps. My heads been shaved 🤣
Love love love you all. 🙏🏼💝
Aug 25th 2017
Latest update from Samantha's dad:
I don't enjoy writing these updates especially the ones which are not upbeat and full of promise, but in order to continually raise awareness of this cruel disease and maintain the fantastic support of you all then I have to do it.
As most of you are aware Samantha had to under go further surgery last Friday, and if you read my last update, you will know that surgery went well and for the first 2 days Samantha responded brilliantly, unfortunately this positive response was not too continue, the last few days have been the most harrowing.
Emotionally as a family and carers we are both mentally and physically drained. To watch my daughter going through this torture, where every second of suffering seems like an hour and every hour seems like a day is very hard to endure. Samantha has been and is still going through an immense amount of pain she has begged us to get her some form of pain relief and all we can do is to try and comfort her until it is time for the next medication. I have mentioned the word torture on a few occasions but there is no other way to describe it both for Samantha and her family. There has to be a better way than this.. the nursing staff although caring have no understanding of EDS and the continual strain it puts upon the body, they can't comprehend that oral medication which would knock out each and everyone of us have no effect on Samantha she may as well have taken smarties.
For the sake of Samantha and everyone else who suffers from EDS we need to keep this story going we need to reach a wider audience and push for changes. Although we the carers do not suffer the actual pain we feel torn apart by our helplessness. I only wish I could see an end to this and I keep telling Samantha everything will soon be ok but I now wonder who I am trying to kid?
*Plus Aug 25th local news report Click here to view
Aug 28th 2017
I've spent two nights discharged from hospital and at the hotel with family around. This means that whilst I had the rotational company of my dad and brother in hospital I now get to wake up around any one of the family (mum, dad. Brother, children and sister) and it should mean dad and Mike get a break from the night watch of my heart monitor....
Unfortunately, whilst I feel they find strength in number, being home doesn't mean I'm any better; in fact the night before they discharged me; I was told resuscitation had been on the cards numerous times due to my seriously low blood pressure and heart rate. That's one massive heap of responsibility at my families feet.
The nurses had been extremely concerned about the seemingly minimal pain benefits being achieved by extreme doses of pain medication when I was in hospital. Many voiced concerns at overdose; paranoia, hallucinations, lucid dreams, fears....
Perhaps, most concerning of all was that my right upper cervical vertebrae are severely dislocating still. The surgery has fused this area together, however in two sections it was soft fused; this means that it was fused without the metal in two segments to enable some mobility, independence and to reduce the strain on unfused areas of my spine. IT is likely the areas without metal where I am dislocating as I can not imagine it possible in the areas where the metal is used.
I told my surgeon about this and he suggested that I was feeling metal grinding. I do feel metal grinding, however, this is different. Dislocations and instability are so very rare after such extensive surgeries that he felt it highly unlikely. I was prescribed a long list of oral medications to be taken every hour.
By now we know many oral tablets are metabolised to the point of no use, but in my desperation, I would have taken a hammer to the head for some relief from the pain. I still would.
Some of my new meds are known to damage the progress of bone fusion. Maybe it's a game of weighing up the lesser of two evils. Let's roll with the experts.
I could now be discharged from the hospitals care.... The next issue we hit (and still haven't managed to resolve) is that the one medication that many pharmacies didn't have in stock is the one that I must take in the very easy accidental overdose of the many hourly medications prescribed.....
I feel dismay....desperation....
I feel heart broken.
I wonder whether my life is still at risk or whether it is stabilised enough to keep me safe in spite of these dislocations.
I feel like I am lay on a throbbing sharp axe ploughed into the back of my head.
I wonder what my future holds.....
To YOU, I am so damn thankful that you read my story and that you choose to partake in my journey in anyway that you wish to....
To my family here, I have no words. I owe you my life. You amaze me continuously. I am sorry. To my babies... you are my very reason every day. Simply you.
To my family and friends at home; I crave you.
To my old life- normality, school run, simple smiles.... oh god, I pray you come back to us soon.
Sept 3rd 2017
In short, my recent surgery had some benefits, however, we have discovered that I have further cervical instability.
How this is possible with all of the fusion and hardware, no-one seems to know at the moment, but it has been suggested that there is a hormonal imbalance which is a large catalyst towards the extent of my instabilities. This makes sense to me as my condition didn't become apparent until my pregnancies 10 years ago and they fluctuate throughout the month.
It's too soon & dangerous to operate. My body must rest and I can not fund staying in the US for testing any longer, therefore, we will return home with family in two weeks time.
We will rest, re-gather ourselves, allow the children to have some normality and get ourselves stuck back into fundraising so that we gather answers and surgery as soon as is safe and financially possible.
I am coming home very poorly and I feel I should warn people in advance that I am not arriving back all fixed up.
I'm devastated. Again. And I am working hard on my mindset, rehab and getting fundraising moving so that we can continue our plight to #SaveSamantha. I can not wait to see my loved ones back home.
Sept 14th 2017
We're getting ready to fly home tomorrow and it's bringing it to the forefront of my mind all that we've been through this last 3 months here.... I am heartbroken to be heading home with so much pain and a need to continue fundraising....
A year has passed since my symptoms took over my life.... A whole year wasted.
My thirtieth year.
My son will be 9 soon and my daughter 8 not long after; with anniversaries to measure time by, I begin to wonder, how much more of their childhood will I lose to my health? Will I ever be the mummy I was again?
What about the life I imagined. The relationships, marriage, adventures....
I feel suffocated by frustration and the ticking of time.
This isn't how our journey to the US was supposed to end. I didn't go through this agony and fear and torment just to go home in the situation I left the UK in.
The fundraising isn't over as we had all hoped it would be and I'm coming home to continue with it- frustratingly, I am still physically unable to do much myself, therefore, draining the resources of those willing to set up events around me.
I'm so sorry that I have to ask you to continue to fundraise for my future. I understand this wasn't the plan. I hope you do too.
I've no idea how I'll manage back in 'normality' whilst lives continue around me.... all I know is this is not how it was supposed to be....and the only way through it is forward.