Eds The not so Beautiful Life-London Update 

Two weeks ago I finally got to see the Cardiologist. 

Its about time its not like my heart hasn’t been on a nonstop rave waiting for this ‘urgent’ appointment referal that went in on August 4th.

I’ve been having none stop issues with tachycardia again since about May again.Tachycardia is a racing heart beat over 120 beats per minute when it should be general between 60-80 beats per minute (bpm) 

Oh the Tachy is getting me down

But it had been calmed , the thing is it was being lowered by a concotion of medications but they are not managing it anymore.
I’m struggling badly with all the symptoms this brings and I know what it is when it gets to its worse and in the back of my mind its a constant worry. Stress is no good right now in the position I am so I try not to stress and think about it but its hard not to when you’ve a constant reminder thumping in your chest every time you take a few steps.

My upright mri of my brain and neck showed, in medical jargon that I’ve a reduced clivoaxial angle of 133° , and a retroflexed odontoid peg. Basically thats the upmost tip of my spine that’s kinking into my brainstem. Apart from it causing me major pain and symptoms along with the chiari I have there and the instability I have in my cervical spine at the base of my skull.

 This is also the are that controls your heart rate and my NeuroVascular Consulant said it is going to be very hard to get my heart rate under control with that kink there. I was so pleasantly surprised that the cardiologist read about my issue and scans also and was in agreement. 

Images from my mri scan where the RO kinking back into brain stem can clearly be seen in my upright mri. Reduced CAA measurements under 135° is pathological and requires intervention
Notes on my report from upright mri only available in London

So what can be done about that. Well usually its surgery but this is a highly complex surgery in eds patients with only a few surgeons in the world that operate everyday on our complex cases. These specialists are abroad not even in the Uk and costs tens of thousands including months of being away. It seems impossible. It was off the table after I had been refered to one if the world’s top specialist surgeons in the States since last year when I had my cardiac crisis.I was too underweight and my heart was too weak to survive any surgery.

I’m left in limbo.

The Cardiologist was so nice. He was so apologetic that there is not much they can do for my condition , to which I told him , I fully accept as I’ve heard that before.

When I asked him had he dealt with someone with my condition before , he said although rare he had, but he had dealt with alot of people especially with pots but never anyone with the complexities I have.  I told him I heard that before too 😁

My main issue is symptom relief asap, I accept that there is nothing they can do for my heart. I’ve postural orthostatic tachychardia, ventricular tachycardia and full autonomic dysfunction.

I’ve to increse one of my heart meds up to try lower my heart rate which had jumped up to 160bpm the day I was at the cardiologist and while on the ECG. I also have to introduce a new mediction of symptoms don’t improve

My heart rate got so high on the day of the ecg just from the effort of moving out of my chair. It was recorded at 160 on ECG with lots of interference.

So far there’s been no changes wince increase of meds. Im still struggling especially with breathlessness when moving .
My NeuroVascular Consultant referred me on in August to a Geneticist to rule out any other connective tissue disorders or other eds types I might have in case I have to have surgery as this will effect the outcome of the decision. I will have these tests which are essential for my case at a big cost in London on the 15th November.

 I am also seeing my Neurogist at Queens Square and I will repeat the 40 cranial injections again and hopefully get some relief from the pain that’s becoming unbearable again.

This never gets any easier no matter how many times.

Thank you to everyone who supports my treatment fund and allows me to access all of my care, this testing and my injections which give me so much relief for a few weeks.

I was hoping to have my full upright MRI done again on this trip too but it will be too much to fit it all in with traveling, injections and tests so I will have it done in January, again another massive cost and something that cannot be done in Ireland.

This time of year I always seem to be off. My Facebook memories show me that this October is one of the first October since 2011 I haven’t been in hospital. And not for lack of health issues, oh I needed to be in hospital a few weeks ago. I actually had a day where I said I think I’ll go. Me!! willingly going to hospital. That never happens. I have to be taken unconscious 😂

But I stayed stubborn and I’ve huge supplies at home and I willed it out. The last week has been pretty horendeous though.

I actually hate my bedroom now it’s a hospital room to me

Christmas is such an important time of year especially with kids in the house and I’ve been Ill for the last few but after spending most of one in hospital over Christmas and new years I’m just glad to be at home enjoying the day.

Last year i had hospital appointments up until Christmas Eve. 

Winter time with chronic illness is tough my whole focus is staying well enough to get to Christmas and that preparation starts already. I really have to hope for no hiccups until after then.

I will get through this trip to London, I’ve hospital appointments at home up until December and then back to London in January where it will be head on with plans to meet with more specialists and Surgeons hopefully for my Chiari and Atlantoaxial Instability/ CranioCervical Instability.

I try to offput some things for as long as possible. Sometimes its easier to avoid the harsh reality, but there is only so long you can do that for.

I have days I wish there were no doctors , no appointments , no hospitals , no medications, no tests, no injections. That I could just turn it all off and ignore it all. It would be so much easier to not have to think about what you have to face in to everyday and trying to plan ahead and organising the medical side. It just gets exhausting.

But then you tell yourself I’ve done it for so long and I’ve done one hell of a job at it so I can keep going . I have to keep on going.

I’ve met some fantastic people suffering from the same conditions as me and we all will each other on and give and take strength from each other. Everyone has been through so much.

We will all get through it. No matter how hard it gets we will get there. 

Without that hope there’s nothing .

Thank you to everyone who allows me to continue to have that hope even in the most worrying of times. 

Z x x 


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