Updates and Everything Falls In March!

I have been so unwell over the last few months that I have missed and had to reschedule a lot of hospital appointments and now they all seem to have come in March!

I was last admitted to hospital in November last but never got around to writing about it, I was in for a week with another bout of agonizing pain in my right side and gut, all stemming from the Sphincter of Oddi Dysfunction/ Gallbladder pain I had been having all along and that already hospitalised me over 4 times in the one year previously. They took real note of my weight loss this time and started me on new calorie drinks, some new meds, as well as doing some further tests, ultrasounds and abdominal X-Rays all leading up to a second ERCP on the 28th of March, of which I will write about below!

Invacare Action 3 NG – Ocean Blue

Yesterday I had a manual wheelchair assessment with my Occupational Therapist, I have now been measured and fitted for a new manual chair as my current one is loose, rickety and near impossible for himself and myself to push, not to mention that it is far too big for me at a size 18 where I need a size 14 or 15. Yesterday, I was fitted for the new Invacare Action 3NG  (In Ocean Blue!) and have been put on the funding list for it, which means if approved, I will get the chair through the HSE but if not I simply wont get it at all, especially since I was already approved for a new Motorised Wheelchair (Invacare Spectra XTR2 Pictures Below) a few months ago (But is only good for outdoor local use and we do not have a modified car to transport it so I need a manual one to use in the home and transport) There is no guarantee that I will get approval for the manual one, We will just have to wait and see.

Invacare Spectra XTR2

March kicks off with my birthday on the 5th, I have nothing planned only to celebrate with hospital appointments which start on Monday the 6th with a follow up appointment with my doctors who look after my Pots care in UHL. This usually involves looking at the current meds I am on, seeing if they need an update, they ask about my symptoms to date and see if I require another Tilt Table Test or some other tests etc. This will have been my first ‘Pots’ appointment in over a year now so it will be good to catch up and see if any changes are needed.

 

Wednesday of that same week, the 8th, follows with a Urology appointment to check my kidney and bladder function. This Urodynamics Test needs to be done every 6 months or so now that I rely on catheters for painful urinary retention, it can leave you more prone to kidney and bladder infections and kidney dysfunction so that needs to be checked frequently to try and catch infections and Kidney dysfunction as early as possible to prevent any damage.

Image of a Type 1 Arnold-Chiari Malformation. The cerebellum has descended 7mm and there are herniated cerebellar tonsils into the foramen magnum. – Wikipedia

The next day, Thursday the 9th, I have an MRI of my neck and spine, as ordered by my Pain Specialist, to try and rule out Chiari Malformation or other complications because of the severe pain and headaches I am getting coming from around the base of my skull/ top of my neck area, I already get ‘Occipital Nerve Blocks’, for that and ‘Sacroiliac Joint Nerve Blocks’, for the pain I get in my lower back and hips, but I find these nerve blocks are only helpful for a very short period of time (sometimes, if at all!) and are not advised for long term treatment. I have been getting them on and off for over 3 years now altogether as well as continuously taking two types of Opiates (Fast and slow release), meds for neuropathic pain, anti-inflammatories and also muscle relaxants, daily, even with all of these and the injections there has been no proper ‘cure’, especially for the severe neck and head pain, if anything that has gotten worse, so he wanted to investigate that further. We spoke and he explained that he understands that EDSers usually don’t show any evidence of Chiari during a lying down MRI, it is preferred that an upright MRI is performed for a more honest view, however there is no upright MRI in Ireland, one of the closest being in London. I may not need an upright one at all, we will see what the lie down one shows first but he said he would refer me to London if needs be. I will also be returning to London, (privately as none of this is covered by the HSE!) to see another GI specialist that specialises in EDS very soon and I will also see what that specialist suggests I do because the pain and headaches have me bedbound most days now and have worsened my quality of life, where it was very low to begin with! 😦

The following Wednesday the 15th I am back down to Cork University Hospital to see the new private GI specialist I started seeing in November just gone. I won’t mention Names here but, he was recommended to me by a good few people on the Irish EDS related Facebook Pages stating that he is an excellent GI specialist who is very well read up on EDS (Also having studied and worked with the private GI Consultant who specialises in EDS, in London, that I had already heard about (and spoke about in the previous paragraph) and had planned to go and see.) It was recommended that I see him before going straight to London as he can do (in Cork) a lot of the tests they do in London, so I said I would give him a go, and I am so glad I did.

Sphincter of Oddi Placement

I have to say first that My Limerick GI Team in UHL are fantastic in that they have tried almost every test they could to see what is causing all my gut trouble. Since starting all the GI investigations in December 2014, up to now, they found out that I have ‘Gut Dysmotility‘ and ‘Sphincter of Oddi Dysfunction‘,  but beyond medicating and treating me for those, they do not know what is causing my Nausea, Vomiting, Trouble Swallowing and Severe Gut Pain that has hospalised me more than 4 times in the last year, nor do they know why I have lost over 3.5 stone and continue to lose weight even though I am keeping my calorific intake as high as possible on as many days of the week that I physically can. The team have read up on EDS where they have needed to and continue to educate themselves as we continue to investigate (The tests in Limerick are ongoing, even with another ERCP Operation with Bile Duct Sphincterotomy (where they cut the muscle) at the end of this month) but I feel we are still coming to a dead end, Especially when the symptoms have eased only a little and ultimately continue to cause daily trouble and the head GI specialist of the Limerick Team came to me already and said that it would probably be better if I went to London to see what ‘The EDS Experts’ have to say. So on hearing about the GI doctor in CUH who knew his EDS, I made an appointment and went down to see him and get his advice before I decided to go straight to London.

 

The first Cork appointment came in November and I was pleasantly surprised when I met him. He was very well read with regards to EDS, listened to my full medical history, symptoms, complaints, procedures, tests and operations that I already had. Went through the medications I was on and went through some with me that I had never heard of before but he wanted to do a few tests and X-Rays before changing my medications. When he heard I was interested in going to the GI specialist who he trained under over in London, he was delighted to refer me over and suggested that it was a great idea to get his opinion as he would have the most expertise when it came to treating GI trouble in EDS patients and he thinks this London specialist should be able to help me.

He immediately wrote up a referral letter as I was there as well as booking me in for some new tests in Cork hospital that are not available in Limerick. He said these test appointments would be sent to me in the coming weeks after the appointment and sure enough, they did, they came through very fast, a lot faster than I have experienced in Limerick. I had a Barium Swallow X-Ray done in January and still waiting on the results of that which will probably be given during the clinic appointment I have on Wednesday the 15th of March and I am currently waiting on a Gastric Emptying Test appointment which should arrive, I am told, in the next few weeks. Either way I am very impressed with this Cork based GI Specialist who knows his EDS, I will certainly stick with him, as well as the Limerick team, for now while I wait for my London appointment to come through.

Not finished yet! I have a Gynae appointment in the Maternity Hospital on Monday the 20th. Then that Friday the 24th I have an EEG back at the UHL and finally, The Big One! I have another ERCP operation thingy with a Bile Duct Sphincterotomy the following and last Tuesday of the Month, the 28th.

Sphincterotomy – Cutting The Muscle 

 

The ERCP and Sphincterotomy is to treat the severe pain, nausea and vomiting I am having because of what the GI doctors in Limerick think is Sphincter of Oddi Dysfunction. I have written and You can read about SOD and the ERCP procedures HERE.

I have already had the first ERCP procedure where they injected Botox into the SOD and you can read all about how (Kinda bad, though it inevitably worked!) that went HERE.

and that’s it!! That covers all appointments for March only! Every month there is usually something and it feels like, I only get out for doctors appointments these days! but what can you do?! 🙂

Lette xxx

The Sphincter of Oddi and Other Stories!

Say that in the voice of the narrator from He-Man and you have a pretty formidable title there!

The last time I was admitted to hospital at the end of March, early April the surgical team came to the conclusion that I could possibly have something called Sphincter of Oddi Dysfunction (SOD) that was being masked by what they thought was Gallbladder pain.

In the last blog post I outlined what this was and that there is a test to check for it called an 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 or under general anesthetic as it is way deeper into your system that 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. Sometimes if the pressure in the bile ducts is measured as too high or if the surgeon deems it necessary at the time they will cut the sphincter muscle first time.

Cutting the sphincter of Oddi muscle surgically during an ERCP is called a sphincterotomy. It’s generally effective and relieves symptoms of SOD about 70% of the time.

The long term cure for it? a series of ERCP’s 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 and Pancreatitis with this so they do it in small stages over time to make it safer.

A video of the procedure can be seen here, if you have the stomach for it! 😉

Since coming out of hospital in early April, I have had a number of follow up appointments. I’v been seen by a gastroenterologist in Nenagh General Hospital. He reviewed my case and changed my pain meds saying that the morphine I was on was a spasmodic drug and wasn’t helping the pain in my side so told me to ween off them and start other anti-spasmodic drugs which he prescribed. He also gave me new antiemetic meds for my stomach and gut as I am constantly having trouble with it. He said getting back over to London to a GI specialist over there when I am well enough is greatly advised, but to wait until I am well enough.
He also prescribed me with a spray for under your tongue, usually used for people with angina but said as it’s job is to open the blood vessels and relax muscles that it may help the pain in my side, and it really does, although drops my blood pressure and gives me a cracking headache but I would rather have that than the pain in my side!

I have been to the Pain specialist in Croom hospital for more injections into my Sacroiliac joint and hips which usually takes about 3 days to a week to kick in, but of course I got cocky and tried to hobble out of the bathroom the day after getting them, without any chair, crutches or support, thinking I could do it and SMACK! I slapped off the ground hurting my hip, but thankfully not seriously so it has healed again and the injections have slowly begun to work to ease the pain in my lower back and hips. Unfortunately though they only ever last about a month so I am coming to the end of that relief already.

I was back into the University Hospital Limerick for a follow up from when I was admitted last and to see when my ERCP will be. I was told I should be called sometime this month so I am currently still waiting on the appointment and hopefully I will be called soon as it is getting very sore and uncomfortable.

and finally… My powered wheelchair finally arrived! I had to chase it up through the local OT manager because I was getting nowhere emailling my OT about it. The manager got things moving immediately once she knew I had been waiting nearly 2 years and the replies to my emails were just one excuse after another, it finally arrived a couple of weeks ago, Miles too big for me, refurbished and not new but mine, great fun and gives me great freedom! The powered chair deserves a blog post of it’s own so I will do that as the next post. It’s name is Marvin and I’ll explain where that came from too! 🙂

For now, that’s all my current news! 🙂

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)