May – EDS Awareness Month

May is EDS and HSD Awareness Month!

For many people, the journey to a diagnosis of an Ehlers-Danlos syndrome (EDS) or hypermobility spectrum disorder (HSD) has been a long and challenging road. Poor awareness of the conditions amongst medical professionals means they can go undiagnosed for many years, with people often being misdiagnosed with other conditions, being given incorrect or unnecessary treatment, or no treatment at all. Early diagnosis, and recognition of EDS and HSD, is key to improving quality of life.

A better understanding of EDS and HSD in primary care could make a huge difference to the day-to-day lives of many people struggling to manage their symptoms. Most GPs will not have had any specific training on EDS or other hypermobility-related disorders and therefore their awareness is not high. The EDS toolkit (one of many on the RCGP website) provides GPs with the best sources of reliable, up-to-date information to support you better.

Understanding of the complexities and multi-systemic nature of EDS and HSD from medical professionals in all aspects of care, including rheumatology, physiotherapy and nursing, can ensure a quicker diagnosis, and more accurate, and beneficial care.

Societal awareness and understanding of these conditions, their comorbidities, and chronic illness can also vastly improve the lives of our community. From education to employment, from relationships to friendships, and in understanding disability and often-invisible illness: understanding the impact these conditions have on our lives, and their fluctuating nature, goes so far in improving wellbeing.

May is EDS and HSD Awareness Month – let’s join to raise awareness together. We may not be connected physically, but there is still so much we can do virtually! Keep reading to find out how you can get involved this May. (Shares from Ehlers-Danlos Support UK)

#edsawareness #ehlersdanlossyndrome #hypermobilityspectrumdisorder #hsdawareness #zebrastrong #letsmaketheinvisiblevisible 🦓

Thank you to @EhlersDanlos UK for providing this information for me to share

Look out this month on the blog as there should be more awareness information added

Stay safe and stay home ❤️🦓❤️🦓

Different Types of Stripes

Ehlers Danlos Syndrome now comes in thirteen subtypes, when I was diagnosed there were only seven, the images you See below explain the variances between each type.  There are overlaps of symptoms between the subtypes and this can be why it is difficult for us to be diagnosed, or it can take longer.

Lets keep raising awareness, so we can bring down the diagnostic times and ensure people don’t suffer

#edsawarenessmonth

#ehlersdanlossyndrome

#zebrastrong

 

EDS Awareness Month

Hi guys,

It’s been an awful winter, one of the hardest I can remember. Pain wise I have had to lock myself away, put the heating up high to mimic the Caribbean and wrap myself in my duvet and just alternate between sofa and bed, then bed and sofa. When I have made it out of the house it’s been to those lovely hospital appointments, that us zebras really look forward to. So two weeks ago, the sun came out, I threw off my winter coat, got my lighter weight summer jacket and the excitement was just a little too much as a week later, winter was back with a vengeance along with May!

So as we start to shout from the rooftops about Ehlers Danlos and it’s co-morbidities, make everyone understand how it feels to live with the pain, the ignorance of people not understanding the “invisible” illness aspect, fighting with the medical profession to get the correct treatments and diagnosis’

Our battles are hourly, daily, weekly, but we will always fight ….. we just ask that with your tweets and retweets on our awareness campaigns we will get there in the end 👊🏻

Oral Surgery at Guys

After having a tooth removed at Guys under sedation back in May I have been struggling with multiple chronic sinus infections.  Then in July I was referred down to the Oral Surgery team to find out what had gone wrong, as I could definitely suck air through the cavity but worse than that was this constant smell of what I could only describe as “death”. Now anyone in the uniformed services who has attended a sudden death which has been there a while will know exactly what that smell is like but to carry that round in your head since May is pretty horrific.  Worse still it kept bringing back PTSD flashbacks, from my time as a Special Constable, so the sooner this ended the better.

After a great consultation on Floor 23 it was agreed that I had an OAF, Oral Antral Fistula, had developed following the extraction, so the only way to resolve this would be surgery and due to the invasive level of this it would need to be under general anesthetic.  So once all the discussions had taken place about my complex health conditions and what they would be doing, consent was signed and I was added to the waiting list.  That was in the middle of August, so six weeks later I was finally heading up to the hospital for the dreaded surgery.

A week prior to the appointment I had started some antibiotics but it also seemed to make the sinusitis much worse, then Friday night I went to bed seemingly ok, Saturday morning I couldn’t move.  My vertigo was off the scale if I moved I fell, so Andrew tried to perform a technique called the Epley Manoeuvre, this involves four head movements to move the crystal fragments that cause vertigo.  Sadly no chance it just made me violently nauseous, so I had to lay very still.  I considered that the sinus infection was making the vertigo worse or maybe my neck?  With EDS it could he anything, so it was a case of bedrest and fluids, I didn’t want to go to hospital, I didn’t have the energy to deal with Drs and nurses to be honest.

So I slept and just lay in a darkened room, hoping it would settle, two days later and it didn’t, Monday and I woke feeling woozy but I could sit up,  that was a positive I rang the hospital and explained and they agreed I could come in.


I got to hospital and I was doing ok, fresh air was helping, not moving my head also a positive, at check in, I explained it all and they checked me over, I had no infection, blood pressure, pulse etc all ok, the anesthetist came to chat and despite all the health issue was happy to proceed.  

After a while it was time to say goodbye to Andrew and head off to theatre, this is where my nerves kicked in but the staff were fabulous.  I had already met the anesthetist, her nurse introduced herself and was adding monitoring pads, chattering away trying to distract me.  With the drip in, the fentanyl was soon added and wow that felt great like five G&T’s in quick succession, with a oxygen mask on, it was soon time for the knock out juice and the next thing I knew I couldn’t breathe and it was post op!!

That panic was awful, I think they must have bought me round quickly I was really tachycardic and I was struggling to breathe, so they nebulized me but that didn’t help, so they got a second one and I just thought I’m panicking, I need to slow down. So I started to try and breathe in through my nose and out through my mouth to get the salbutamol into my lungs, I tried to explain I wasn’t going to panic.  They reassured me I was doing well, I was in control, and needed to deal with this as best I could, but my HR was all over the place, they hung more fluid but I could barely speak from the sore throat post intubation and the lack of oxygen in my lungs.  Eventually I was stable enough to take to recovery, I hadn’t realized I was still on theatre at that point, so they pushed me through and got me hooked back up to the O2, and monitors.  My HR was still to high and blood pressure very low, basically my pots had kicked off, so the extra fluids should help.  They started offering sips of squash but that just burnt my painful throat so I agreed to water.

After nearly an hour, I seemed to have stabilized enough to get into my wheelchair and be reunited with Andrew ready for discharge.  I could tell he would be worried once again a 45 minute wait had turned into over two hours, so I was thrilled to see him.  After a handover of paperwork and my cannula being removed it was agreed I could go home.  After all we would be hitting rush hour traffic and all I really wanted was my own bed, so we thanked the team, who had been amazing and headed home.


Follow up is in four weeks time, fingers crossed this is the end of my terrible sinus issues caused since the tooth extraction 🤞🏻

S xx

My First Nephrology Check Up

Being told you have Stage 3 Kidney Disease is a bit of a kick in the teeth, especially on top of the catalogue of illnesses I already have.  Andrew often asks if I am playing Chronic Illness Bingo and he’s sure I am nearly at a full house because surely there can’t be many more space left on my card left to dab?

After seeing my GP and her insisting that it was a matter of urgency that I be seen by a consultant as I didn’t fit any of the criteria for Kidney Disease, I haven’t got high blood pressure, grossly obese or diabetes, so why had I been struck down?  My initial appointment was postponed, and the rescheduled was for the middle of September and first thing in the morning, which is always a good thing, clinics rarely run late, or so we thought !!

By the time I got into the consultants office he was already an hour late, this was at 9am, he was a very kind man and in discussion, he explained that my creatinine levels had been incorrect for two years, he believed the medication I was taking for my EDS and PoTs was causing the deterioration but I  needed both to keep me better, so it was a case of maintaining my kidney function now.  However he needed two medications needed adjusting my stomach protectors and my anti-inflammatory tablets these were badly damaging my renal function.  If I could get my doctor to adjust these we would be able to slow the kidney issues down and keep me in Stage 3 and not progress the illness.

He ordered a ultrasound on my kidneys to check them and blood work, he wanted to see me again in a couple of months.  He explained that the clinics were overbooked, that morning alone 26 patients in a clinic for 18, hence he was already running late, but he didn’t rush my appointment, he was kind, empathetic and supportive, I liked him, I felt listened to and not rushed out the door.

So hopefully we can keep the Stage 3 exactly at that, I do not want to progress further, as I have enough issues on my plate.

S xx

Pharmacy Follow Up

This week I popped into my pharmacy to collect my monthly Nomad packs, I still need my medications dispensed for me as my brain fog is so severe there is a risk I would overdose my meds.

 

My pharmacist is probably one of the most supportive I have ever met, I arrived and explained that next week I would be running out of my Fentanyl patches and would she mind organising more for me, without issue and a few clicks they were ordered.

Next I asked her about the new stomach protection medication tablets that needed to be changed over, basically the Lansoprazole might be causing bigger kidney issues, so the nephrologist had suggested a switch over, but I forgot the name of the drug.  She grabbed the Mimms medicine book and took me to the consultation room.  We sat and went through all the medicines and she told me all that type of drug caused renal issues so she wasn’t sure want that consultant was talking about.  Her advice was to call his secretary and find our from my notes what the name was and to get it switched over as a matter of urgency, especially as my levels had changed so rapidly in four weeks.

We then decided to do a quick annual review and she was concerned that so many consultants were trying to interfere with my medication, if a cardiologist had advised one medicine then a gastro doctor couldn’t then mess the prescription around, etc etc  She was worried that I was getting lots of mixed messages from various consultants, I told her how difficult it was being stuck in the middle, especially when you have two or more hospital or doctors appointments a week.

She was very kind and told me I was doing my best under the circumstances, which were not easy but she would support me as well as she could, we signed off the review paperwork and I thanked her and her team for all their help.

They have always helped me, ensure my medication is there, at short notice, when prescriptions are written incorrectly and just been very supportive, just what you need when you are chronically ill.  Thank you Krupa at Boots in Burnham !

Continuing with Botox

I’ve had at least two if not three treatments during my blogging absence.

Friday 17th August was my most recent appointment, it was straight after my GP visit and although I don’t like needles, there is something quite pleasing about going to get this done.  You know that despite the pain, very soon that constant headache/migrainous nagging pain will diminish and life will become tolerable at least neurologically.

I’ve a lot to thank Dr W for had it not been for him I would never have been diagnosed with Postural Tachycardia and ultimately Ehlers Danlos Syndrome.  He was the only specialist who noticed the despite being on beta-blockers my heart rate was unusually fast.  He conducted what I now know to be a poor mans Tilt Table Test in his office at the Chiltern Hospital, and decided I would need a proper TTT for a full diagnosis.  That was in 2013!

During the procedure, Dr W always chats to me to keep my mind off all those needles and what I admired about him today is that he is attending a seminar about PoTs to learn as much as possible about this sodding illness, he also said he would feed back any info he could to me.  Two of my consultants are going to be speaking on the day Dr Gall, my cardiologist and Professor Aziz, my Gastroenterologist.  It sounds like a fascinating day in which they will not only discuss the illness based on findings in the UK but also findings around Europe, that  interests me, are they any further advanced in their care for potsies than we are in the UK?  

After wincing and yelps my injections were over, chattering about his seminar really had worked it’s magic.  A few needles sites were more painful than usual, indicative that the Botox really had worn off but hey it’s in now, he let me sit and rest for a few moments as I felt really woozy and sick, he could just tell.  I like that he never rushes me out the door, despite knowing there were people outside waiting.  A couple of minutes and I’m sure the colour was back in my cheeks.

We said our goodbyes and I would see him in four months for more Botox.  That’s one appointment I don’t mind attending even if it does involve lots of needles 

S xx

Going to Guys

Since January this year, every month I have been a patient of Guys Hospital. 

(this is the amazing view from the 26th floor)

Due to the severe periodontal damage and gum disease that Ehlers Danlos can cause, my teeth had ended up in a terrible state, plus I have a real phobia of dentists and dental procedures.  If I am honest the phobia stems from pre-diagnosis days, basically anaesthetia doesn’t work well on EDSer type patients.  So from my childhood years to the age of 40 I have never known why going to the dentist, being injected and it still hurting was possible.  Dentists would say that was not possible and I had managed to find a local clinic that would knock me out for “heavy duty” work but not for general care.  

In January I broke a tooth, in fact it happened a while before that but by Jan I was in agony and knew I couldn’t hold off any longer, so agreed to see a dentist. Que call to my old dental surgery and yes I was still on their books, they kindly fitted me in as a matter of urgency and Andrew took me straight down.  My dentist there was pretty fab, he obviously was unaware of how sick I had become and we chatted about the EDS, he had heard of it in dental school, I was his first ever real patient.  So he was quite thrilled, he gently examined me and promised he wouldn’t hurt, Andrew held my hand and good to his word I didn’t yelp once.  Decision made, a temp plug would be put in the gap but an urgent referral up to Guys was needed, he felt I needed their EDS specialist knowledge.  So for the last six months we have been day tripping up and down to London for treatments, I’ve had fillings, tooth extractions, major cleanups, tooth shaving and now we are at the stage where they are looking and feeling good.

BUT

When my tooth was removed in May, I developed a Fistula, it’s a tiny hole between the gum and the nasal cavity, I’ve had three severe bouts of sinusitis, with a constant infection lingering.  I’m ow on my fourth set of antibiotics and I have nose drops too.  I went last week to see the Oral Surgery Team, twenty third floor, and it’s been decided that they need to operate.  After hearing about what’s got to be done, I’ve opted for a quick GA, I don’t think sedation will be deep enough for the amount of work they need to do.  Then I am hoping my oral issues will be resolved for now.  Op date is set for early next month, so I’m just waiting to speak to the surgeon next week to confirm.


(The view from the waiting room on the 26th floor, London Bridge station below)

After this it will be back to annual check ups hopefully at Guys with just a clean up, while I am asleep, that way I may keep my teeth for a few years 🤞🏻

S xx

Chronic Kidney Disease Stage 3

Imagine logging on to your online doctors booking system and finding that diagnosis.  Yes that is what happened to me a couple of weeks ago!


I had an initial blood test, for a silly health check and my creatine level was off, so one of the GPs called me and discussed the results.  She asked me to drink a little more, asked about other symptoms, I told her about my previous urinary retention problems and that I was experiencing the odd “retention issue” now and again but patience prevailed, it was just a case of sitting and waiting.  Plus I had a backache type pain near my kidneys but just kept dismissing it.  She said I should get myself checked out but first get the repeat bloods done.  So Monday 7th, was the day of blood test, all done and as I went to leave the nurses office, I was told not to worry and the results would be available on Friday.  The next day I logged onto the online system to book the Drs appointment as agreed with the Dr during our previous call and there in black and white is my diagnosis 😳


I was so shocked I got a fit of the giggles, Andrew just looked at me like I had lost the plot and I just said I refused to call the surgery the next day, as it was my birthday I planned on having a normal day with nothing medically related just for a change.  So I left it until the 10th when I called to speak to someone, but once again they couldn’t help me, as only the Dr who had ordered the blood test could speak to me, and she was now off sick.  So an appointment was booked for the 17th Aug.  Fast forward a week and my phone finally rung at lunchtime that day and this is how the call started.  

Dr “Hello Sarah, how can I help you”

Me “Your calling me about the blood test results”

Dr “What blood test”

Me “Well I’ve already logged onto the online system so I know what it says I have so I need to know what to do next”

Dr “ok let me log in and see what’s going on”

FFS you would have thought a bit of prep before making the call?? So in the end she agreed I needed to be seen, however she couldn’t see me as she had no appointments, so could I call back and speak to reception *facepalm*

I sat and thought for a while on what should I do and decided to go into the surgery and ask nicely for an appointment, take it from there.  I headed in and, the lovely receptionist explained there were no prebookable appointments, call back on Monday, this was Thursday!!

So I asked to see the Practice Manager, enough was enough, I wasn’t willing to lose the plot with this lady she was just doing her job, I took a seat and waited for the big chief after all that’s what he is paid the big bucks for.  The lady called me back to the desk, the manager has agreed to release me an appointment for the next morning, whilst grateful and yes I did take the appointment I still wanted to see the Manager, he wasn’t getting out of it that lightly.

So off she went to explain that Mrs Webb was on the war path, she came back and showed me to his office.  I took a seat and calmly and clearly explained my disappointment in the surgery over the last two weeks.  I explained prior to this I had been a huge advocate for BHC and the Drs and Nurses but the way I had been treated was less than acceptable.  He couldn’t understand why the results were on the screen, why nobody hadn’t called me to discuss them first and that was only my first issue.  I said in the act of compassion just shoving results on a PC when you have no idea what’s going on in people’s lives is tactless.  A call was the least I deserved, he offered me an apology, I know sorry is only a word but an apology is an apology I suppose.

As for the Kidney Disease, he then went an found a Dr and we had a quick discussion, I was firm with them both in that room, explaining that with all the other health issues I have right now Stage 3 is the highest level of Kidney Disease I actually want, we need to stop this in its tracks, I don’t want to get a year down the line and find I’m at Stage 4 due to bad condition management.  

My diagnosis’ so far EDS, PoTs, Gastroparesis with Slow Gut Motility, Chronic Daily Headaches/Migraines, Depression with PTSD, Chronic Fatigue, Chronic Pain, Marfanoid Habitus, Fibromyalgia, Osteopenia, Chronic Anaemia , BPPV, Mast Cell Intolerances, and now Chronic Kidney Disease – Stage 3 it’s just a little too much, like the straw that broke the camels back  😢 

I thanked them both for their time and headed home, also thanking the receptionist as I left the building.

What I had forgotten as I explained all this to Andrew was at 10:30, the very next morning we needed to be in London at the Charing Cross Hospital having my next round of Botox for my headaches.  My stomach just sank, why does my brain do this to me?  He told me not to panic just send my neurologist a message that I may be 15 minutes late and would that he ok?  I pinged the message across and got the thumbs up that it would be fine, I heaved a sigh of relief.  

I had a restless night, uptight about the GPs appointment and I’m not a fan of needles so the 30+ in my head and neck are not a joy, but they mean life can return to some kind of normality in the neurological sense, especially when the pain levels half.

We got to the doctors early but she was running ten minutes late, I got worried we would then hit traffic, I shouldn’t have.  I have never met this GP before, I explained quickly why I was there and she said I needed the referral to Nephrology straight away, I didn’t fall into any of the normal categories for Kidney Disease, so I needed to be seen asap.  She tapped away on her PC and booked me an appointment for the 31st August at my local hospital.  I was so impressed with her, I was not expecting that.  We left the doctors and still made it to Charing Cross in time for my appointment!!

Friday was a good day all in all 

S xx

Battling the DWP


In 2014 I started my first ever claim for PIP that’s Personal Independence Payment for those that don’t know the benefit lingo, it’s a disability payment that has two levels to it, standard and enhanced.  In 2014 I attended the Reading assessment centre and met with an amazing rheumatologist who knew all about Ehlers Danlos Syndrome.   He was empathetic and as I left the centre although it had been an awfully stressful experience, it hadn’t been as bad as others had made out.  My award arrived and I was given an enhanced decision on both mobility and care, which when you look at the questions was fair, the aids, the care Andrew had to provide behind closed doors and the constant escorts as I am no longer able to travel on new journeys alone, meant the gentleman had listened with care and written a detailed report for the DWP to base their decision.

Move forwarded to the end of 2016, my reassessment paperwork arrived and most of it was tick boxes, how had my condition improved, better, worse, the same.  I answered the questions accordingly and sent the form back, we hoped the process would be a fast one, we were desperate for a new car and wanted to get a Motability car but with less than 12 months left on my award they couldn’t help.  As Christmas got close, no sign of an assessment, we got through and into the new year, the old car just played up to the point of falling to pieces, it had reached its death bed.  So that had to be replaced.  We had to get finance for a newer car, but on the plus side the newer car was similar to a Motability car, it was new, light weight, easy to drive and might mean on good days when I was well enough to leave the house I would be able to use it.

It was another two months until I got the letter to explain I would be having a home assessment on the 3rd April.  I was shocked that I had been offered an assessment at home with no fuss but maybe being on enhanced rate, this was standard procedure.  My appointment was at 10:30am so the night before we headed to bed fairly early hoping for a decent nights sleep.

As you can imagine Mrs Stressy Pants, yes me, got next to no rest, I was worried sick how this would pan out, the press had been rife with stories of benefits being cut and poor assessment decisions, so I just felt on the back foot and the assessor hadn’t even arrived.

There was a knock at the door at 9:30am and I panicked, it couldn’t be now, this was too early!! The appointment was for 10:30am, I was at this stage still resting in bed due to pain and fatigue, so hubby ran down and yes of course it was an hour early assessor.  He set her into our front room and then came to get me out of bed, she would sadly just have to wait.  So after helping get me up, dressed in PJs and dressing gown, trying to tie my messy hair up and cleaning my teeth, hubby then got me downstairs, the assessor missed that bit, me bum shuffling down our stairs, I’ve been doing it for years now, I can’t deal with having a stair lift fitted!  We got my crutches and I met her, she was very young, she mentioned her name and job, but I couldn’t tell you who she was.  We sat and started our meeting.  I can’t tell you much of what happened, it’s a blur now, but we went through my form.  All I do know is at the beginning she admitted that she had googled EDS that night to check up on the symptoms etc before coming out to me.  She didn’t ask me about my memory, the last assessor did number games and alphabet checks, nothing like that this time.  She didn’t ask me about any depression and to be fair I forgot, I have terrible “brain fog” I can’t remember anything, so if you don’t ask me a direct question I won’t remember to tell you something.

In fairness I should have just written, pages and pages of lists, but this would have kept her at the house for weeks, however it would have meant I got everything done and thoroughly.  She asked to physically check me which I could do, of sorts but it hurt, but I hide my winces, the pain,  from everyone it’s habit.

After an hour she felt she had done everything she needs and thanked us both, Andrew had sat through it as my witness but I’m not sure he will remember much, he can’t remember what he had for lunch yesterday 😳

A few weeks later the dreaded brown envelope dropped through the letterbox, I sort of knew before I opened it, I had been downgraded from Enhanced to Standard, in their opinion I “have got better”. My chronic debilitating illness has miraculously improved??

Two things that stand out from the reports are, my medications, I state that I require aids not only to remember to take them but also I have them all dispensed via the Chemist as I was getting confused with my tablets, so they are sent to me in Nomad packs, the DWP awarded me zero point saying no aids used or required.  Secondly the DWP claim I am able to plan a new journey to a route I’ve not travelled before.  That is impossible, I can’t even travel 30 miles alone.  My furthest is about two miles up and down our hill and I explained that, I can get to Sainsbury’s/Tesco but no further, again zero points awarded as I can plan a new journey according to them.  The whole report is full of inaccuracies and non truths, it’s hard to read something so wrong.  

So the first step was Mandatory Reconsideration, which we started back in April and the DWP stood by their decision.  The next step is appeal, this means I have to take them to court and fight for my rights, which I am terrified to do, but I have to, they are wrong, if I could travel alone I would do so much more with my life, if I could remember things, like my meds, I would be employable, this is more than just a benefit, this is about the bigger picture.  So we are still waiting for our day in court, despite the DWP’s wish that it was overturned, I just have to go in and try and have a clear less foggy day.  Maybe write lots and lots of notes to help me get through those stressful few hours 

Wish me luck x

S xx