May Is EDS Awareness Month – 2017

Yes, it’s that time of year once again folks, May is EDS Awareness Month and this year, I update my diagnosis story as it just grows in volume and substance every year since all this started in 2011!! So here I go again, it’s 2017 and my story is in need of a clean up and update, so let’s jump right into it!

So firstly, What is EDS?

Put simply, EDS (Ehlers Danlos Syndrome or ‘The Ehlers Danlos Syndromes’, as it is now known since March 2017) is a group of connective tissue disorders. Here is a better explanation according to the EDS Wiki:

” Ehlers–Danlos syndrome (EDS) is a group of genetic connective tissue disorders. Symptoms may include loose joints, stretchy skin, and abnormal scar formation.^[1] These are typically noticed at birth or in early childhood.^[2] Complications may include aortic dissection, joint dislocations, scoliosis, chronic pain, or early osteoarthritis.^[3][1]

EDS is due to a mutation in one of more than a dozen different genes. The specific gene affected determines the type of EDS. Some cases result from a new mutation occurring during early development while others are inherited in an autosomal dominant or recessive manner. This results in defects in the structure or processing of collagen.^[1] The diagnosis may be confirmed with genetic testing or a skin biopsy. People may be misdiagnosed with hypochondriasis, depression, or chronic fatigue syndrome.^[3]

There is no known cure.^[4] Treatment is supportive in nature.^[3]Physical therapy and bracing may help strengthen muscles and support joints.^[3] While some types have a normal life expectancy, those that affect blood vessels generally have a shorter life expectancy.^[4]

EDS affects about 1 in 5,000 people globally.^[1] The prognosis depends on the specific type.^[3] Excess mobility was first described byHippocrates in 400 BC.^[5] The syndrome is named after two physicians, Edvard Ehlers from Denmark and Henri-Alexandre Danlos fromFrance, who described it at the turn of the 20th century.^{[6]” –}

^{Ehlers Danlos Syndrome Wiki}

If you would like to know more about EDS types, symptoms and a host of further information then I highly recommend The Ehlers Danlos Society Website for more info.

My Personal Diagnosis Story.

Though I have had many medical issues throughout my life, my Chronic Illness journey only began properly in 2011.

One Tuesday morning in March, I woke to not feeling very well and as I was exiting the bathroom I called out to my husband who just happened to come and catch me as I passed out in his arms.

I continued to come to and then pass out again every time I straightened my legs. My Heart rate was racing and my Blood Pressure was dropping really low. My husband called the doctor for advice who told him to immediately call an ambulance or take me to A&E. He decided to drive rather than waste more time waiting for an ambulance which would take at least 30 minutes to get to where we lived.

On the way to A&E my husband had to keep shouting at me in the car to try and keep me alert as I kept needing to pass out. We finally arrived at the hospital, I was rushed inside and long story short, many tests and doctors later I was kept in for nearly a month where many further tests were performed, one of which was a Tilt Table Test with which I was diagnosed with Postural Orthostatic Tachycardia Syndrome (Pots) and Vasovagal Syncope (VVS) or Neurocardiogenic Syncope (NCS) – they are both the same thing, basically neurological fainting!

 

So where does EDS come into it?

In the 2 years following the diagnosis of Pots, I was hospitalized many times because of the fainting, low blood pressure and other complications. In the process of trying to figure out what caused the Pots, Hypermobility was mentioned a few times by a couple of doctors and physios, however, whenever I mentioned it to my Pots doctor (he is a geriatrician but he is the specialist who looks after me for my Pots), he didn’t seem to think it was anything to be worried about, even though I did have chronic pain and I did feel it was affecting me at the time.

As time went on, the pain became worse and I felt a formal diagnosis of Hypermobility would benefit me, though there are no EDS or Hypermobility specialists anywhere in Ireland, I still felt a diagnosis would help me.

I had heard about a Rheumatologist in Cork who knew about EDS and Hypermobility so I decided to pay him a visit just to see what he thought. Down I went to see him and within a few minutes of him seeing me he had me diagnosed with a ‘classic case of Hypermobility EDS’, with possible Classical EDS overlaps. I was surprised and kinda happy that I had finally confirmed my inkling that I had it.

However, this diagnosis from the Cork Rheumatologist wouldn’t be worth the paper it was written on… with my pots doctor anyway. He never accepted the diagnosis and just ignored it outright so I just had to live with the fact that my Pots was probably caused by the EDS but there was nothing I could do about it.

To help, my Pots doctor did organize for me to see a Rheumatologist in Croom hospital who confirmed my possible hypermobility and organized for me to do Hydrotherapy and physio at their facilities in Croom Hospital. Even that physiotherapist confirmed I had possible hypermobility but she never believed I had EDS. I found the HSE as a whole were fine to say hypermobility but would never confirm EDS (for fear they would have to treat me for it if they confirmed the diagnosis! I have always been paranoid that was the reason anyway… maybe not!)

You Said You Always Had Some Medical Issues?

Yup! I was even breached for a while before birth but thankfully righted myself before being popped out! When I was born then, I was born with a Fissure and a broken Tail Bone and throughout my life I always had gut issues, travel sickness and dysmotility and I was never without a cast, sling, crutch or some other bandage or plaster thanks to stupid injuries and broken bones which, even though broken bones are not symptoms of EDS I put a lot of my past injuries and ailments down to my EDS as you’re born with it, it’s with you from the start and I seemed to have a lot of various symptoms.

I am currently severely deaf and wear Bi Lateral Hearing Aids, I have and have had since the age of 7, Bi Lateral Sensory Neural hearing loss and Otitis Media with under developed eustachian tubes and auditory canals. My younger life was plagued by ear infections and severe ear pain.

When I hit puberty and teenage years the fainting, feeling really weak, Nausea, Dysmotility and chronic pain got bad with a vengeance and again all the way through school there are photos of me in slings and on crutches, it was crazy! I did do Karate though from the age of 13 and I was constantly breaking bones from it! Baaaaad idea with EDS but sure I never knew and the A&E at the time only ever treated the individual injuries and never looked at everything as a whole!

My teeth and gums gave me problems too. From the age of 13 to 16, I was with an Orthodontist and had braces for the full 3 years. As well as always having gum disease for as long as I can remember, They could never successfully freeze my gums, they had to do lots of injections and finally had to bring in a heavy chrome looking contraption thing to freeze the gums, either way lots of freezing needed. I also had receding gum and bone and every time the braces were removed my teeth would start quickly moving back to where they had been! I now know all these teeth things are problems of EDS.

Things Improved and I Returned To Work

After a little while things slowly began to improve, life from 2011 had been turbulent but in 2013 I decided the time was right for me to return to work. I had been working as self employed while I was running my Media Production Company from 2010 after I had finished my Masters of Science in College and it went very well while I had the energy for it and obviously while I was sick I became unable to deal with 12 hour days traveling all over the country for day long photo and video shoots so I decided something slower paced would suit me better and I found the perfect job working from home for Apple Computers. I absolutely loved the job and because it was from home it was sedate enough for me to deal with some symptoms and still be able to work but just as things had started to go well, about 4 months in I tripped over my Mums dog we were minding and wrecked my hip. Symptoms seemed to crack up from here!

Things Then Got Worse and I Went Into A Wheelchair and Had To Stop Work

Things got worse and worse from here, my hip pain was daily and excruciating, I had to go into hospital for investigations where I was advised to stop work because of my illness and to start using a wheelchair to help my mobility. Of Course, I didn’t want this at all and resisted it at all costs but had to give in, in the end as I simply just needed it and now I am glad I have decided to use a chair as it has given me much freedom in this restricted state.  Work however has stopped and has not restarted since stopping in 2013. It doesn’t look likely that I will ever go back to work as this is a progressive disease.

The symptoms continued to get worse and worse, I started having seizures, ended up in the ICU at one stage for a couple of days and I decided that going to a specialist in the UK was the right thing to do. I was hospitalized so many times where the doctors didn’t know what to do with me and didn’t accept my EDS diagnosis from the Rheumatologist in Cork and I was left with very little help or treatment.

Even my hydrotherapy and physio had stopped early in 2014 as I had fainted in the pool because of my Pots in the hot water and the physiotherapist didn’t want to see me back at the pool or gym until such time as I stop fainting… which is never! So unfortunately I havent been able to get back to that either since it stopped!

Prof. Rodney Grahame, EDS Extraordinaire in London, was the next port of call.

The Hospital of St. Johns and St. Elizabeth in London

I started a Go Fund Me and started fundraising to go to London. So many people generously helped out, Thank you to all, everyone was amazing, even a quiz night was organized and everything, I was blown away! I finally had enough to go and so I did, You can read all about the trip HERE.

Long story short, we got to London and I got a confirmed clinical diagnosis of EDS Hypermobility Type with secondary GI Issues and possible Classical EDS overlaps, from the Professor himself. He did up a great long letter and treatment plan to send to my doctors that couldn’t be ignored and I have found has helped me hugely since getting it. He referred me to Harold’s Cross which I did in Feb 2016 and you can read all about that time HERE and I find all doctors and nurses take the diagnosis far more seriously than the one from Cork. I have had no more trouble from anyone on believing or disbelieving the diagnosis. He wanted me to return to see Prof. Aziz a Neurogastroenterologist for further tests and treatment and I had hoped to return sooner than I am able to. I will be returning in 10days time (2.5years later) to see him and I am excited!

Why Didn’t You Return To London Before Now?

Simply put, I was too ill to fly. The past 2.5 years have been by far the worst in terms of my symptoms. My Nausea is daily and intractable, my dysmotility causing so much pain and trouble that I have been hospitalized loads of times because of it and what I have been diagnosed with called Sphincter of Oddi dysfunction has been causing severe pain and again I have been hospitalized because of this and other chronic pain. I have also been diagnosed with Neurogenic Bladder Dysfunction and have had complications because of that also.

My Neck has been giving me huge trouble. I am receiving Occipital and SI Joint Nerve Blocks for severe pain. My pain specialist thinks I have instability in my neck but that cant really be checked without an upright MRI, which I may also need to get, but again there is none in Ireland so this will have to be done in London if it is needed. At the moment he is treating me as though I have instability in my neck with the Nerve blocks and opiate pain meds until such time as I can get it checked properly. He has me in for a lie down regular MRI for which I am waiting to be called but he doubts it will show anything.

My Gastro issues are probably my worst problem. They are what I am hospitalized for the most and they never seem to go away. This is why I decided to visit another specialist in Cork, this time a Gastroenterologist who has studied with Prof. Aziz (the Dr. I am seeing in London for my EDS Gut related issues) I went to see him in Cork and he immediately identified all my problems and symptoms, took note of all the medications I am on and booked me in for a number of tests that may be asked for in London anyway.
In Feb this year I had a Barium Swallow test that showed up all clear which is great. Next Monday I have a Gastric Emptying test to do which is happening just before I go to London to see Prof Aziz. I will be flying out the morning of Sat May 13th. The reason I decided to go to this doctor in Cork was that he understood EDS, I heard great things back about him and the fact that he studied under Prof Aziz all meant that he could possibly help me out and so far I feel he has. He was the one who wrote the referral letter to Prof. Aziz for me and he said he would work with whatever Prof. Aziz says in his treatment plan.

So, When Are You Off?

All going well with my health (it has been very up and down lately, mostly down) we hope to fly out Sat Morning the 13th May at about 07:30am and landing in London around 09:05am. From here we may grab a bite to eat before heading to the appointment with Prof. Aziz at 12:30pm at The Princess Grace Hospital, where afterward we return to the hotel to rest.

That’s the plan anyway, lets hope that’s how it works out! I tried a dry run of trying to stay up for 11 hours (sounds easy for some but for me I find it difficult to stay up past 4 hours before needing rest) as 11 hours is the time it takes from being up from about 05:00am that morning until about 4pm which is roughly the time we will make it to the hotel at, thats 11 hours up. I tried that the other day and actually failed at 9 hours and needed to crash so bad! so I am worried I may not be able for this trip but I will persevere and hope for the best. My husband will be with me so at least I wont be alone when I go to London and of course I will update you all when I return. I really hope the good professor can help! 🙂

Lette (Fainting Goat!) xxx

Ambulance Time Once Again!

On August tenth we had to call an ambulance yet again!
I had been in bed for weeks at that stage nursing a savage pain on the right side of my abdomen. This wasn’t the first time it has happened, and probably wont be the last! It got to a stage where I was trying to manage the pain myself at home, trying to avoid going into hospital, but I had to face facts and call it as it got too bad to manage on our own this time.

The Ambulance arrived promptly and began to tend to me. They were a crew of two, Male and female, and were excellent. Friendly, Professional, funny easing the mood when needed and seemed to take a genuine interest in what both Keith and I had to say and contribute.

Unlike the last few ambulance calls, this visit from them wasn’t rushed. It was calm, sedate and gave us time to properly prepare to go into A&E. While they took their time trying to locate a vein on my foot, Keith ran upstairs to the computer, got ready and printed off an A4 sheet with simple information on it about me and my condition, highlighting the main issues I present with, medication I am on and what would be needed when first admitted like fluids, Pain control and Catheterisation, etc.

The reason for this sheet was because through previous experience we have found to be constantly repeating ourselves while giving out my information to different people and yet only parts of the overall story get heard by all different people and nobody has the full story! This time we  said we would try getting this sheet in as a common denominator of information in my files so that everyone will be literally be on the same page regarding my care and see what happens. It was worth a try at least.

After getting a vein in my foot and administering some morphine for pain relief, we were eventually ready to go to the hospital and the ambulance crew were brilliant with everything as well as taking on the information sheet, which they said they thought it was a fantastic idea and wished more people were that organised!

We quickly made it to the hospital, after getting more Morphine on the Ambulance, I was brought straight into the A&E main area, Thankfully I wasn’t rushed into Resus at any stage this time, it was calm and sedate and a far better visit this time round.

I was delighted to hear back from the Female paramedic that she passed on the sheet Keith had given her, to the triage nurse and that the nurse also commented on what a good idea it was to have all the main things highlighted on one page where everyone can see it. She said she would put it in my file so that all the doctors would see it too. So far so good for our little A4 sheet!

While waiting around for a short while, waiting on the triage nurse to find a place to put me as it was busy in A&E that day, The Paramedics were chatting to me and told me they knew some student and new paramedics who were doing papers based on me for some exam that was due the next week! Morto! I found it funny that they would pick me based on my rare or under diagnosed EDS condition or as I like to call it, ‘my awkwardness!’ :p

I once again had terrible trouble getting veins in me, doctor after nurse after doctor tried and failed and it started getting really sore. The vein the Ambulance crew had gotten was already failing just a few hours after it was put in, my foot swelled up  and they were worried as they really wanted to administer fluids and iv meds so they wanted a vein soon. Throughout the following week while in the hospital, this continued. a vein would be gotten and it would fail almost immediately until in the end the anaesthetics team had to be called and they had no trouble getting a vein and thankfully now a note has been put in my file to say that only anaesthetics are to place a cannula on me and no one else, so hopefully all the prodding and poking will come to an end now.

After spending no more 24hours in A&E this time around, I was admitted to the surgical ward. While in the A&E I had all the usual tests, xrays, scopes, exams, the usual trouble of about 50 different doctors trying to get a vein after a brief moment of being cocky and saying something like, “Oh I’m good at this, don’t you worry”, or,”Oh really? Bad veins, huh? well, I like a challenge!” and then just end up being unsuccessful anyway! That always provides me with a giggle especially towards the cocky ones who think I wont post a problem, cha! Say that to me after 20 mins of trying hopelessly! :p Being admitted after 24hours was quick though, considering that I have often had to wait over 46hours before.

I always like being admitted to the surgical ward. Not only does everything seem to be more up to date and cleaner, but Compared to the medical wards, The mood on the ward is generally brighter both from the staff and the patients. The Nurses seem to genuinely care, are not as dismissive as others I have encountered on the medical wards. They get upset when you get upset, they don’t like to see you in pain or discomfort and as much as they are able, they won’t keep you in pain for long and will treat you asap when they are asked to, a lot of the time, they don’t even need to be asked, they will notice themselves and will get you what you need to help ease your discomfort. Also, the electric beds are well cool! :p

I saw My Surgical team the next morning bright and early and they took the situation serious when they saw I had lost over 3.5 stone with the nausea, vomiting and general pain and discomfort I have been having with my gut issues.

As well  as my pain team to cover my Occipital and Sacroilliac joint Nerve Blocks which were due once again, They called in a dietician, a tissue viability nurse to talk about any difficulties I may be having with the chair, thankfully theres nothing serious going on there but she wanted to refer me to a Dermatologist for a small bit of irritation caused by the chair that was easily sorted with some long term antibiotics (starting with an 8 week course and if it needs to continue after that it could go up to 16 weeks), will just have to wait and see how it works. Then they wanted to get a serious OT assessment for me as my own local OT never properly assessed me for manual chair or a bed, given my situation, these are the two basic things my surgical team wanted to be covered while I was admitted.

The Dietician was concerned for my weight loss compared to my height and prescribed a high fiber, high calorie diet as well as Fortisip Calorie drinks to take twice a day along with my usual daily food intake to help at least maintain my current weight if not try to increase it. Sshe also prescribed a new tummy med to take with the other ones I am on to try and help with the nausea and vomiting as well as to try and increase appetite. So I have now started these and I was thinking of doing quick video reviews of the different drink flavours on offer, if you are interested that is! Let me know and I will do it if you like 🙂

Throughout there was non stop testing to find out what was causing the pain in the right side. Xrays, Ultrasounds, CAT and MRI scans, Scopes, blood tests, you name it, it was done.

In the Ultrasound Room!

One outcome is that they have ruled out any Gallbladder issues. There definitely seems to be Sphincter of Oddi, Severe Gut Dysmotility and Neurogenic Bladder Dysfunction problems, as well as a large and painful Lymph node on the inside of my right hip which is causing a lot of pain too and is currently under investigation, all these are contributing to the pain as well as the Ehlers Danlos Pain itself, but they have ruled out Gallbladder and Appendix issues, which is good!

I had 2 major cardiac events while I was in, though I had been battling low Bp all week, these events were the opposite, they came out of nowhere in the middle of the night, they were raging high bp and very fast heartrate, the ecg showed some abnormalities but thankfully they didn’t stay long (about 2 or 3 hours) and after they administered some medication to lower my bp and hr, I seemed to return to my regular low bp self, they were scary though as the pain in my chest when it happened was frightening and I was soaked through with a tempreture. The nurses seemed concerned as Long story  events came out of nowhere. Thankfully though after a few hours I returned to normal and could get some sleep.

Long story short, I got to see everyone that was called for me except OT. The final team was the pain team with the good professor who loves to give injections and this time was no different! He administered my usual Occipital and Sacroiliac joint nerve blocks under ultrasound which he did at my ward bed, he had a little portable ultrasound machine or at least that’s what it looked like and he went for it there and then. The pain team in conjunction with the anestetic team said that I may have chronic piritonitis ( information or even tearing of the abdominal lining that holds your organs) this is still being investigated and will continue as an outpatient appointment along with all the other teams I had to meet while I was in.

The surgical team try everything they can and they are so good to want to learn but they still are not sure exactly what is causing the severe pain and weight loss. I had mentioned to them about a Gastroenterologist specialist in Cork university hospital who is very well up on EDS and speaks regularly as well as trained under Prof Aziz over in London whom I hope to see as soon as i am able to travel. They said go to see him, just to see if he can help and my surgical team said they would be delighted to consult with both him and Aziz going forward as they are of the mind that more on the overall team to help me then the better!

In the meantime my team wanted to bring my case up at an EGM (emergency general meeting) where the head consultants and specialists of a department get together and discus special cases at a count table meeting so that other people’s views and suggestions can be taken on board, then once they have news they  will call me for a new outpatient appointment or send me for further tests if required.

 Unfortunately the OT never turned up and there were questions as to wether she would turn up anyway as she usually only sees Stroke and Neurology patients so my surgical team along with the head nurse on the ward rang her boss as well as had to write a letter in order to argue the case as to why they felt I was a special enough case for her to make an exception and come and see me, alas I was left waiting all over the weekend just to see her and she never turned up so my team decided to send me home at this stage with some new meds and a ton of outpatient appointments and they would follow up with her to get me a much needed appointment as my local OT isn’t really doing what she is supposed to do. 

In the meantime I have made a new appointment to see that Gastro specialist in Cork, his name is Akbar and I have heard great things so I really hope he can help. That appointment is on the 20th of this month (September) and of course I’ll fill you in on how that and any subsequent appointments go.

For now, I’ll just chill and try to recover 🙂

Thank you as always for taking the time to read.

Lette ( the fainting goat!)

Made The Longlist of The Irish Blog Awards 2016!

 

WOO!! So not only did the Blog make the long list finalists for Best Health and Lifestyle Blog in The Irish Blog Awards 2016, but also one of my blog posts, ‘The Good, The Bad and the Emergency‘, made it onto the the long list for Best Blog Post!!

I am delighted and I have you all to thank for adding your entries for the blog! I cannot tell you how much I appreciate it 🙂 From here on, as far as I know, The blogs will now be judged by a panel and then those who make it onto the shortlist may need to get votes for the remainder of the judging so if I ever even make it that far, I may be back to ask for some votes! :p

For now, Thank You all once again and I will keep you posted on how everything goes 🙂 ❤

Lette (Fainting Goat)

The Blog Awards Ireland 2016 – Nominations Now Open

Hi all,

I am delighted to say that Irish Dysautonomia Awareness has been entered into this years “Health & Well Being” – Personal Blog – Category in the blog awards nominations and hopefully will make the long list at least, that’s where it got last year so to make that again would be really nice! 🙂

If YOU would (Please) like to Nominate this blog Please click HERE (Give it a minute to load, it can be slow!) or click the image below and follow the instructions. I would greatly appreciate your input, THANK YOU! You need to register with the site but you can do so quickly and easily  by signing in with your Facebook and you can control what information you give them.

Please enter The Title of the blog: Irish Dysautonomia Awareness,
Also pop in the URL of this Blog which is: https://irishdysautonomia.wordpress.com
also please be sure to enter it into the ‘Health & Well Being’ – Personal Blog – Category.
Thank you ever so much!

Please click here and enter the info to Nominate this blog! – THANK YOU!

This year there is also an entry to nominate your favorite Blog Post from the blog here. In the last number of months the post that has raised most interest seems to be this one:
‘The Good, The Bad & The Emergency – Part 1’

I would also greatly appreciate if you have the time, to maybe nominate that blog post Please and thank you most kindly! as far as I can figure, there’s no harm in trying! 🙂

It is the same process as the first, please click Here:
Hit ‘Nominate a Blog’ – Enter the ‘Blog Post’ Title as: The Good, The Bad & The Emergency – Part 1

The Blog Post link is : https://irishdysautonomia.wordpress.com/2016/04/05/the-good-the-bad-and-the-emergency-part-1/

The Category is ‘Best Blog Post’ – Personal Blog

Please Click Here and Enter ‘Blog Post’ Details (As I have laid out above) for Nomination – Thank You

Thank you so very much for you time in doing this each and every year, I may be back to you for more support if we make it to the ‘public vote’ part but even if it doesn’t get anywhere, a few more people will view the blog as a result of just entering and further our much needed awareness, even just a teeny bit.

Thank you once again,

Lette – Fainting Goat!

Admitted after the ERCP – Possible Bowel Pacemaker!

Things never just go straight forward, do they? Nope! and of course, this time was no exception!

In I went to the UHL on Tuesday the 21st at midday, all prepared for my ERCP procedure to be done and so I could be kicked back home that evening to recover, however, little did we realise just how long it would actually take!

They popped me on a trolly as soon as I arrived as I had been dizzy all morning and they didn’t want a risk of me fainting and deemed it best that I wait in as comfortable a position as possible as I could be waiting around for a few hours.

I said my goodbyes to Keith and the nurses told us to expect to be in until at least after 5pm. Keith went home and I lay on the trolly waiting for things to start happening. There wasn’t even signal in this particular part of the hospital for my phone so I had no internet, texting or anything to distract myself!

All was well until they needed to take some blood from me and wanted to insert an IV line. This is when all the fun started!

3 nurses tried and failed, 2 lads from the Cath Lab tried on not only my hands and arms but also on my feet (Very uncomfortable to say the least!) and failed, One of the theater consultants came out from doing an operation to take a look at my veins, checked them over and said, “Nope! I will hand it over to Dr. Moloney!” (referring to the consultant who would be doing my procedure in theater)

Finally at about 6pm, Dr. Moloney comes out of theater to take a look at my veins and decides, “We don’t need bloods anyway, not to worry about it and I will pop an IV in, in theater before the procedure”
So that ended the long wait around waiting for someone to be able to find a vein, now it was time to actually get this done! The nurse helped me into a very sexy blue paper theater outfit and pushed me into theater where there were 2 nurses, the first tall doctor who had earlier come out to check my veins and said ‘Nope!” and the good doctor himself who actually successfully found IV access after a few attempts, Excellent, time for sedation… am, about that…!

I have a sensitivity to Benzodiazepines, having ended up in ICU last year after being treated with them for seizures I started having in May 2014. It turned out the first seizure was caused by a complete once off event of a lack of oxygen to the brain after a Vasovagal Syncope where I had stopped breathing. The Ambulance staff immediately treated me with Benzos, the standard treatment to stop seizures, not realizing that the more Benzos they treated me with the worse my seizures got. I was treated for these seizures over the course of about 3 months with these drugs until such time as I ended up in the ICU before realising that it was the Benzos causing all the trouble.

Thing is, Benzos are used as sedatives in minor surgeries and procedures too, which was meant for this ERCP, so the Dr. decided to sedate me through the IV with something else they had, that would make me feel a little drunk, but I was still wide awake and feeling everything!

They sprayed the back of my throat with something nasty tasting to numb it and popped a round yellow thing in my mouth, between my teeth to keep my mouth open so that the endoscope could easily pass through. My eyes watered as the scope went down my throat, into the stomach and pushed through the duodenum.

I kicked out and squirmed in intense pain as soon as this happened. I gave the doctors and nurses a good fright too as they were under the impression that I was sedated!

The Dr. looks down at me,
“You’re still with me, yeah?”

I look directly at him while gripping my right side in pain and I nod quickly!

He looks at the nurses and said
“Ok, no cutting today, lets just do the Botox Injection to numb the site, make it quick!”

He tells the other doctor to give me extra pain relief and sedative as he quickly tries to inject the site. I feel the sharp pinches of the needle, they are completely bearable compared to the pressure and pain I feel because I presume of wherever the scope is after leaning.

The procedure is done quickly, I am given more pain relief and feel the earlier sedative continue to tingle the back of my skull but the pain is still there as I am wheeled into the recovery area to be monitored.

The Doctor comes out to check on me and says that if the pain continues I will have to be admitted. I am watched for a couple of hours where I begin to spike a temperature, a reg doctor is called for me, I am put on IV fluids and painkillers and am admitted to a ward by 8:30pm.

In the confusion of trying to get me settled into the ward, make sure that I am properly assessed and medicated, the transfer of my regular meds list (Daily meds for Pots and EDS pain, some of which I cant just suddenly stop) to the nurses on the ward went a little askew and would come back to haunt me in the next few days, but ill get to that in a minute!

After many uncomfortable, sweaty, loud and painful hours they finally found a concoction of meds and opiates that got on top of my pain enough for me to want to rest for the night without feeling the need to moan loudly and annoy others in the room. I didn’t sleep, even with a sleeping tablet and much morphine but I really needed the rest.

I ended up staying in hospital over the course of a full week, in lots of pain and having major trouble with my Gastroparesis because of the opiates I was on. They wanted to keep an eye on my bloods and me to make sure nothing dangerous happened during the ERCP, incase I may have an infection or something as I was spiking temperatures on some days but thankfully this was not the case, I was just in a lot of pain.

On one day, I requested pain meds in plenty of time as I felt pain coming on only for the nurses to have been busy and left me without for a couple of hours. I ended up fainting after returning to my bed from the bathroom and I frightened the other ladies on the ward!

It turned out that when they initially admitted me to the ward my regular meds list got taken down wrong and they hadn’t been giving me my full regimen of meds that I normally take so my bp was all over the place and on the day I was due to be released my HR went up to 163bpm but settled again later with rest.

Prior to letting me go my Gastro surgeon came to talk with me and went through my meds and everything I would need going forward to help with pain and the Gastroparesis difficulties.

It looks as though things are getting worse in that department. I am losing weight, having difficulty eating anything without severe pain, bloating, nausea and vomiting and he mentioned the possible need for a Pacemaker in the bowel in the not too distant future to help with the ‘slow’ and sometimes ‘non existent’ transit in my gut, of which he wants to discuss further on my next appointment with him which is the 15th of Sept.

I was let go home a week after being admitted on raised pain meds and antiemetics with orders to take it slow and steady until they see me next month. I have been very sore and very sick. Pretty much bed bound nearly every day since being released and have even had a small seizure for the first time in over a year but I am able to keep the bright side out. Even lying in bed there are things to watch and I am eternally grateful for my wonderful husband who provides all of my supports.

So for now I am recovering slowly, I will see the specialist again next month and until then, I will keep you all updated with things as much as I am physically able 🙂 Ideally I need to get back over to London where they are specialised in those with EDS, to meet a neurogastroenterologist however, I need to get better before being able to travel once again, can you believe it has been a whole year this month since I first went to London for treatment? that story can be read here 🙂

Please remember, I am most easily able to keep the Facebook and Twitter pages up to date most frequently so be sure to check us out over there too for almost daily updates and thank you for following this humble bloggy! 🙂

Admitted To Hospital, Possible New Diagnosis

This was me getting ready to go home so I look a lot happier than when I was admitted first!

Oh dear! it happened again!

On Monday the 30th March, I was taken to hospital by ambulance after being bed bound and in severe pain with my gallbladder all over the weekend. I held out as long as I could at home because I knew nothing would be done over the weekend if I went into A&E.
On the Monday, things just got a little too much for me and we had to call for me to be taken in.

I am very disappointed with my GP in all of this. I had gone to him a couple of weeks before this happened, with a renewal script for some very strong pain killers that were prescribed to me when I was last admitted to hospital for the same thing in December. My surgeon had prescribed these opiate based drugs for the pain that helped and were needed. When I went to my GP to renew, he said only Cancer patients get these drugs and that he wouldn’t renew my script even though a higher authority than him prescribed me what I needed!

Then, when my Gallbladder pain started up again last week, we called him about getting referred into the Acute Surgical Unit (ASU) in the University Hospital Limerick, as this was said to me the last time I was in, that if I had more pain, not to go through A&E but to be directly referred in. He didn’t come through on this either. When we rang he gave some excuse about not having my files with him in the surgery he was at (His 3 surgeries computer systems are linked, he should have had all my information in front of him!) and when he called the ASU he couldn’t organise a bed for me so when everything got too much, we just called an Ambulance anyway and I had to go through the A&E system like everyone else. Which is fine but it was unfortunate the GP couldn’t come through. He has been very angry lately when we visit him. He is constantly giving out and very bitter about the HSE (Health Service Executive) and he complains to his already sick patients, This is not just coming from me but from other patients of his that I have spoken to. To be honest I am getting sick of his attitude and shortly I will be transferring to a lovely lady doctor who has come highly recommended!

My hand reacted strangely to the morphine, the IV line was hardly hanging in my vein too so it didn’t last long!

So I was in the A&E about 10 hours, They did a chest XRAY first, then I was being pumped full of morphine through an IV line that was barely hanging on to one of my tiny veins. The doctor in A&E had tried about 5 times to find a vein and by the time I got to the ward the Line had already failed and they had to call another doctor up to fit a new one. He had some trouble and tried about 8 times before he got one and again it was just about in the vein and he said it may only last a few hours. My veins are useless and over the next week, I had three more doctors try about 18 more times to get veins and each line would fail or they just couldn’t get access, eventually during the week they give up on me and give me oral antibiotics when I am able to take them.

Just as I was in my ward Bed and getting ready to sleep, it was about 2am and they called me for an abdominal XRAY. It was over before I knew it, I was back to bed, Injected with something that helped the pain, popped on a drip and I tried to get comfortable enough to sleep.

Not a single wink was had!

It was quiet and all on the ward but I was sore, the surroundings were different, there were beeps and talking in the background and just everything that home wasn’t!

I was uncomfortable and twisted and turned through the night and into the next morning.

The following morning the surgical team came to see me and discuss my case, The same doctor and team that had me in December. He mentioned that he was happy to see me again but not under these circumstances and he felt by looking at me that I had lost weight. They ordered an ultrasound of the gallbladder so that they could compare it to the one from December when I was in then.

At that time my gallbladder was distended and had fluid around it showing infection. It didn’t respond to the fat test and showed it had dysmotility as well as gut dysmotility.

This time the ultrasound came back normal, no sign of infection in it or in my bloods. They mentioned about the possibility of taking out the gallbladder but as it looked healthy with the worry that my EDS may slow or aggravate healing, they were reluctant, as was I!

The head doctor said to give him some time as he wanted to speak with a colleague, the Dean of the Medical School at University Hospital Limerick, who knew surgeons in the UK who deal with rare cases like me. So now the waiting game begins!

I have to say he and his team are excellent. They have a real interest and go to great lengths to diagnose and be careful about removing anything they shouldn’t unless they absolutely have to! They also have an interest in learning more about EDS which is great to see. Unlike some of my other doctors who just don’t seem to care at all.

Gadgety Bed!

Most of the time I was trying to sleep on the very comfy gadgety bed that I could move into all sorts of comfy positions or stoned out of it so all I could do was lie there in a sweaty trance trying to ignore pain and wishing sleep would come to me!
My BP was very low as I hadn’t been eating for many days due to horrible nausea and vomiting so I had missed my regular meds for those days. Over the week, once the nausea was controlled I was taken off the fast and put on a light diet, I could hardly eat anyway but getting a tiny bit of nutrition really helped. I was also able to take my regular meds which helped normalise my BP and I began to feel a little human again.

Mostly pain meds, an antibiotic and meds to raise my BP

On Friday, as it was coming up to the long weekend I was eager to get home, though still in pain and I had no clue as to what they wanted to do with me, I asked what was the possibility of me going home for the weekend? and they said no problem as long as I was prescribed everything I needed! YEAY!!! I was delighted then everything came together! I was visited by some doctors and got some information, finally!

I was seen by a pain specialist, she was going through my pain meds and what I needed to be comfortable at home until they call me into them next week where they can review everything and make a pain management plan going forward.

Later on, I was visited by a UK Surgeon, The Dean of the Medical school. He told me he trained and studied under Prof. Rodney Grahame (The Prof. in London who officially diagnosed my EDS) and highly respected his opinion. I was delighted to speak with him. He was a gentleman and explained to me what he thought was going on.

They as a surgical team discussed my case and came to the conclusion that the gallbladder may have been masking the true pain that could be coming from a thing called the ‘Sphincter of Oddi‘

I know right, WHAT? Never heard of that before, but long story short, there is a little valve thingy under your gallbladder that allows bile though it to add to the pancreatic juices that feed into the digestive system to help you break down and digest your food. This sphincter opens and closes but if you have this dysfunction, it remains clamped shut and cramped and causes a back up of bile and severe abdominal pain. It is most common in people who have already had their Gallbladder taken out, I still have mine so it is a strange one!

There is a test to check for it called ERCP, endoscopic retrograde cholangio-pancreatography, a procedure that uses an endoscope and looks directly deep inside the duct system while taking pictures and measuring pressure. You have to be sedated as it is way deeper into your system they have to look than a regular esophageal, stomach endoscope or similar.

While they do this, they inject some Botox into the tiny sphincter itself which relaxes it and allows it to open again. If after about 2 weeks your pain is gone, this is a sign you have the dysfunction.

The cure for it? a series of ops where again I will be put to sleep and they have to cut the sphincter to weaken it to the point where it can’t clamp up any more. This should take a few turns because if they do it all at once and cut straight through it, there is serious risk of heavy bleeding with this so they do it in small stages over time to make it safer.

This procedure however, has never been done in the University Hospital Limerick and it is not licensed here. But they are seeing what they can do for me, if it can be done, I will be the first person they will have done this op on! If they cant license it here, I may have to go to The Matter Hospital in Dublin or even as far as the UK if needs be, however because I am being treated publicly, these procedures if done in the UK will be fully covered by the HSE. This is a huge relief!

So in the mean time, He and the surgical team looking after me are going to sit down with some other doctors in a Multi Disciplinary Meeting where they will discuss my case and how to proceed with it and once they have a plan in place they will call me in for the initial test. I feel kinda special! but I can see why this meeting needs to be done. If I do have this thing, those procedures need to be licensed and I am sure that includes some amount of paperwork!

Going Home!

Happy Camper waiting for the discharge letters!

So for now, I am happy to be home with the furry pup and himself, I am comfortable (enough) on the pain medication and I await my appointment for next week to be called back in to the Pain clinic and then the appointment to be followed up by the surgical team.

For now, I will try to relax and recover, it is hard on this medication as it has some nasty side effects, but if it helps the pain I cant complain! HA! could put that on a T-Shirt!

Anyway, that is all for now, I will update again soon 🙂

Lette (Fainting Goat)

Hopeful News for my POTS!