Neuro-vascular autonomic consultant in London

Okay, so April has been manic. Flare after flare, simply owing to the amount of appointments I had to get through. One of these was the long awaited autonomic consultant appointment at St Mary’s neuro-vascular department in London.

Prior to the consultant appointment I had to go through a battery of tests. This took two days (during last November) and I was a wreck by the end of it, but I was really pleased that I did it!

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Day one:   Get wired up with a portable monitor of blood pressure and heart-rate.

I was to wear this for 24 hours.

Every 20 mins it would beep at me and the arm cuff would start inflating to take a reading. If I was doing a physio’ walk I had to stop and stand still as it did this, but most of the time (of course) I was sitting or lying down anyway. Every time it did this I had to scribble down my symptoms at that moment i.e. extent of dizziness, nausea etc. So I had paperwork and a pen to hand at all times and after a few hours of this it was getting pretty tiring. My brain fogs up and needs to be left alone to recover. Asking me to get thinky and writey every 20 minutes is a crazy request. But I’m a stubborn ole whatsit so I did it, of course! (And paid the price, but hey, that’s what happens at appointments, eh?)!

I also had to do one test where my blood pressure and heart rate were monitored whilst I was lying down but cycling with my legs (trying to keep an rpm dial in a steady position). More on this later.

We went straight to the hotel room after the appointment, (we even got minty chocolates on our pillows, ooooh)!

And this was one of the cheaper hotels.  London, baby!

And this was one of the cheaper hotels.
London, baby!

In addition to the every-20-minutes lark there were also specific tests I had to run myself. They were set for certain times of day. Things like lying down – take blood pressure (bp) and heart rate (hr) – then sit up – take bp and hr – then stand up – take bp and hr. Readings after eating. Readings after walking. Readings after climbing a flight of stairs. Readings before sleep, Readings after waking. Readings. Readings. Readings.

Magic Dude was on hand to get me everything I needed to get through the evening. Hot water bottle and painkillers were also invaluable!

Magic Dude was on hand to get me everything I needed to get through the evening. Hot water bottle and painkillers were also invaluable!

I cannot describe adequately how exhausting this is for someone already trying to cope with problematic chronic conditions as well as the aftermath of a long ride in the car as a passenger and an appointment involving walking/sitting/talking. But while I was up doing one of these tests I got Magic Dude to take a piccy of the tech. Some kindly soul had knitted a monitor bag which matched the colour of the arm cuff, awwwww.

ECG monitor, with matching bag and arm cuff!

So getting out of the hotel room is a toughie. We made it round the corner to a local Italian. The manager of which was a real poppet when I explained why I was using a second chair to put my legs up. Bless his cottons, “You can rest as long as you need to” he said, even after we’d finished eating and the tables around us were beginning to get occupied. What a darling.

London, baby!

London, baby!

Back to the hotel room, painy legs so fun has to be found. The bathroom mirror was surrounded with many white bulbs as if I was to be slapping on stage make-up in preparation for a great stage entrance. (Ta-daaaah, the beeping patient enters stage left!) And, as I really don’t get out much to find out about the existence of hotel paraphernalia, the room slippers were a novelty!

Hilarious one size 'fits' all hotel slippers. The toenails are orange for CRPS awareness month last November :-)

Hilarious one size ‘fits’ all hotel slippers

(The toenails are orange for CRPS awareness month in November). 🙂

The pre-inflation warning beep actually stops at 11pm. Supposedly to let us get some sleep. But the monitor makes growly noises when it’s working. So I put in some earplugs (you can get ones that stick over the ear-hole rather than push in which reduces pain that some of us patients get from earplugs), I also put the growling monitor in the drawer of the bedside cabinet with just enough room for the air hose to fit through the opening. But I have no idea how anyone with chronic pain is supposed to sleep through having their arm crushed hourly between midnight and 6am, and every 20 minutes the rest of the time.

A gold star to anyone who can guess what kind of state I was in the next day! (Here’s a clue.. 5am: adjusting pillows under legs because the CRPS pain went off the scale). (Here’s another clue: sleep deprived zombie mode)!

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Day two:   Return portable monitor along with my tome of related notes. Then undergo multiple tests.

The last of 6 pages (3 of exercises with readings, and 3 with notes every 20 minutes)

The last of 6 pages (3 of exercises with readings, and 3 with notes every 20 minutes)

My last words on this sheet read, “…really foggy and groggy. Really tired of this now.” (On a side note, I suspect the poor consultant felt the same way on trying to read my writing. My writing was described by my school biology teacher as “characteristic”, and by my beloved school physics teacher as looking “like drunken spiders” had walked across the page)!

So…. the tests…

They are designed to see what’s going on in the autonomic nervous system (ANS).

The ANS is made of of two parts that balance each other: the sympathetic nervous system (SNS) and the parasympathetic nervous system (PSNS). They work in tandem, and when they’re working together the ANS is a smooth-running super machine. With the sympathetic kicking in to do things like raise heart-rate, increase oxygen supply etc when required (including the fight or flight system, hence my feeling of being stalked by Bertram the bear), and the parasympathetic doing all of the calming down and making sure that mundane aspects, like digestion, keep on happening. Together they work beautifully, but if they are not balanced, we have problems.

Pinned to the wall units in the testing room were examples of hr and bp outputs for each of several autonomic conditions (e.g. PAF, POTS, NCS), and each had a distinct pattern, which was intriguing to look at as I lay there between tests, but I can’t remember anything else about them as I was shot away by the end of all the tests!

These tests are designed to see what the separate behaviours and responses of the SNS and PSNS are, to figure out what’s going on in my body and how exactly I am ending up with all these symptoms, particularly the near-fainting, nausea, brain fog and gastro hassles (which I flagged up as the priority issues on my looong list of symptoms).

Of course, standing and sitting mean being upright and this can affect the blood flow. So to remove the effects of gravity in that way – all tests were conducted with me lying down. (The tilt table doubles up as a bed)!

So here are the tests I experienced, in no particular order (because I cannot remember the order!). For every single test my bp and hr was being constantly measured.

Test Bicycle: (which I did on Day 1) Lying down whilst cycling my legs (on an actual bicycle-like apparatus with changeable resistance levels etc) and trying to keep the rpm dial at the point indicated by the specialist nurse.

Test Squish: Holding a rubber cylinder filled with air in one hand – squish the cylinder to get the attached dial to stay in the same place (as indicated to me by the spec’ nurse). The catch is that the air is escaping, so to keep the dial in the same place you have to squish harder and harder to keep the pressure the same.

Test Exhale: Breathe out into a tube to keep the pressure dial on the same readout. One baseline reading taken when lying down and then another reading taken with the tilt table at about 20 degrees.

Test Argh: (the only one I didn’t do) holding a block of ice. It would have been covered with something, but as icing adversely affects nearly all subtypes of CRPS so would have been daft for me to agree to this. Especially as since then I have found that even getting vegetables out of the fridge hurts my hands. In true Gollum stylee…it buuurrrnses! So I didn’t do this test. But they had enough to go on, so they didn’t mind me missing one test, (apparently a lot of patients will miss one out).

Test Stress: Maths is often used as a stressor in tests. 😉 I was asked to start at 400 and keep subtracting 7, saying each number out loud. If I was wrong the nurse would tell me, and I would try the incorrect answer again until I got it right, then I would continue. (This varies for each patient as the numbers can be changed to make sure we find it difficult enough for them to be able to monitor a stress-ish response. Under brain fog circumstances it doesn’t take much for it to be hard enough, though)!

Tilt Table Test: Monitoring bp and hr whilst lying flat, then raising the table to about a 70 degree angle (so not entirely upright) and continuing to monitor along with noting down any comments from the patient about what symptoms are being experienced when. Also barefoot to check for colour change in the feet (which can indicate blood pooling).

The Tilt Table Test

The Tilt Table Test

Test Food: Two little plastic cups worth of nutrition drink made with cold whole milk  drunk through a straw as quickly as possible whilst lying down flat on the tilt table. Left lying down under a blanket for 45 minutes whilst the food is pottering through our system. Then another (much briefer) tilt table test.

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By the end of all those tests I was a shot away, off the planet, foggy brained, mumbling, bumbling, vague creature who needed guidance down the corridor, bounced slowly off the walls but nonetheless had a rather sneakily good idea of filling up the hot water bottle from the coffee machine in the hospital canteen before the long ride home.

November journey home

On the motorway, and oh sooooo grateful for Magic Dude’s wonderful care assistant and chauffeuring skills, x

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I had been forewarned that I might not get to see the consultant for 2-3 months, but in the end it was more than 5 months later.

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The consultant appointment:   15 minutes of brief exchange, followed by thinking of lots of questions that I wish I’d asked at the time.

Consultant appointments always seem to be so short. 10-15 minutes seems to be the norm’ in my own small experience, though my primary care doctors over the years all seem to be under the impression that we’re supposed to get an hour or so. I have no idea why there is this discrepancy, and there’s nothing I can do about it anyway. 15 minutes is what I got.

The tests were to assess the level of SNS and PSNS activity to figure out exactly what is going on in the autonomic nervous system. It is important that they are carried out by a top specialist so that everything is taken into account by someone who really knows their stuff, (the tilt table test is renowned for producing both false positives and false negatives, so a specialist assessor is far far better).

So first of all… the consultant said that the tests do not show any actual damage to my autonomic nervous system. For other patients this bit of the appointment could also be to advise that they have a certain type of autonomic dysfunction. Either way it means we learn more about what is going on inside our bodies and it gives us more information to move forward with choosing appropriate ways to try and manage our symptoms.

So, for me, he didn’t want to put me on more medications unnecessarily (which fits with my personal preferences, too), and instead had other suggestions.

We know that I have autonomic dysfunction though, because a) it all adds up, and b) the meds my primary care doctor and I decided to try out actually helped. The meds I tried were a type of SSRI (Selective Serotonin Re-uptake Inhibitor) which are more commonly known for their use as anti-depressants, but they are also prescribed for pain management, as well as to stabilise the ANS. It stabilises the ANS by flooding the receptors in the brain with serotonin. This helps because our receptors get really sensitive to serotonin levels which makes our neural reactions to the levels more extreme than they need to be. That is, they over-react and we experience the consequences! By flooding the receptors with serotonin the levels become more consistent, so the receptors have much less to react to, which improves our symptoms. SSRIs are not the only medications that have a stabilising effect, there are similar meds which do the same for our adrenaline (norepinephrine) levels (SNRIs), a bit of both (SSNRIs), and probably some others that I’m not aware of, too. I started taking a basic SSRI a year ago and (after the first few days of hiiiiideous side effects) I noticed improvements on not just the near-fainting (which is what I was most desperate to address) but also many other ANS based symptoms, too. (I came off these meds for the days of tests, by the way).

The tests demonstrated that:
– my autonomic nervous system is not actually damaged (‘just’ wonky ANS signals, then!)
– my blood pressure is always too low
– they fiiinally have recorded proof of palpitations in my heart-rate variations
– they also have evidence of the blood pooling in my feet

So…. his recommendations are:
– prescribed salt tablets (I’ve previously increased my sodium intake, but it’s nowhere near enough)
– to continue with the ANS stabiliser as there have been improvements (I totally forgot to mention the side effects of the jaw pain getting worse and soft tissue getting affected as well as the joint)
– prescribed grade 2 compression tights in the winter (my feet burn so I need toeless ones, and my left hip is badly affected by pain when wearing tights so…. toeless compression stockings?!)
– that I  get stress ball to squeeze to help blood pressure increase when it is low
– to drink eeeeven moooore fluids (at least 2.5 litres per day, I think it was 2.5 anyway, who can ever be sure through brain fog?!)

He had me walk up and down, walk with one foot placed directly in front of the other, hold my hands out and splay my fingers (afterwards I wondered if he was looking for tremors, but he didn’t say so, so as I wasn’t suffering with them at the time I assume he thinks I don’t get them. Doh!). Then he asked me to touch my toes! I’m hypermobile,…. dooooown I went!

So, the other outcome is…. that he is referring me across to hypermobility specialists (also based in London as there aren’t many specialists of that level in hypermobility, either). Now, hypermobility isn’t just about being bendy. I know I’m bendy. I didn’t realise when I was younger as most kids are bendy. The weird thing was when other people stopped getting bendy as they got older. I just didn’t realise that most people over 21 cannot touch their toes (according to my lovely knee specialist a few years ago). Hypermobility also affects the autonomic nervous system. So all of my autonomic symptoms are also listed under aspects of hypermobility as well.

Which effectively means another avenue to explore – another angle I can take in trying to better manage and understand my symptoms. So it is a gooood thing.

x

Temporary cyborg and wearer of an invisible (purple) cape

I must be turning into my very own special version of old-young-person…. coz there I was a few weeks ago, sitting in my third cardiology appointment complete with thin hospital fleece, and whilst I was aware of my body temperature yo-yo-ing between shivering cold and a half-hearted attempt at self-combustion, my brain was otherwise occupied and volunteered a suggestion….. ‘oooh, this fleece would be really handy for covering the winter veg’’!

<facepalm>

Gardening thoughts? In a cardio’ appointment? Ah well, I do use distraction as a handy tool for coping with my conditions and their symptoms. I guess it must’ve become a seriously ingrained habit!

So yes, … I finally got to see a cardiologist, and my near-fainting issues all made the same sense to him as they did to me. Huzzah!

He even knew what CRPS was, and how it can sometimes result in general autonomic dysfunction. It’s such a relief to not have to explain my medical condition for once. That’s the trouble with having a comparatively rare condition, you usually end up training the doc’s, and they’re not always too happy about the knowledge switcheroo!

Yes, the near-fainting persists. As does the generally increased, but crazily variable heart-rate. This coupled with my existing autonomic dysfunction and my long list of resulting varied symptoms, suggests that it’s ‘just’ my ANS being spectacularly inappropriate! As usual!

I say ‘just’. Obviously it’s actually quite bad as there’s not much in the way of treatment. But, as I see it, it’s nice to hear normal things about my body these days! Normal size and shape of heart, for example. Always nice to find something that’s being what it should be. Even if its behaviour is wrong, courtesy of the weird instructions being sent because I have a neurological skew towards biological mayhem!

So I started to write this whilst wearing another ECG. My fourth home-ECG in the last few months. There were wires sprouting out all over the place. I probably looked like some kind of cyborg. And I probably should have practiced some robotic beepy noises for the occasion.  😉

(To be read in your best Dalek voice….) “Exteeerminaaaate!”?

The cardiologist’s plan was that the three-day ECG would (hopefully) show a degree of heart-rate variability which would back-up the theory that these symptoms are also because of my autonomic dysfunction. It would be easier to prove if we could record an arrhythmic heart moment, but they are random and I seem to be in a quiet phase on that front at the moment.

During those three days, though, my heart was being quite calm. Continuous light-headedness as usual, of course, but not any heart-rate variability that was obvious to me. Perhaps sitting at the laptop or having a calming Tai Chi lesson would not be that helpful in trying to demonstrate symptoms of heart-rate variability! I made sure I listened to Jared Leto’s dulcet tones on the drive home from Tai Chi, though, so surely listening to that voice would have raised the heart-rate a wee bit?! 😉

Oh?… Jared looks a bit surprised to be in a blog about dysautonomia!
(Image: free-extras.com)

It’s such a good feeling when I get to see a doctor who already knows about my condition. It’s a whole conversation that I just don’t have to have. When I visit my general physician, he doesn’t say much. He just sits and looks at me. He listens to what I say, but he doesn’t usually enlighten me about what he’s thinking at all.

It’s tricky not having a two-way conversation with your medical first point of contact. You never know where you are or what is really going on. My doc’ doesn’t know much about CRPS or dysautonomia simply because he’s not specialist trained.

Poor confused Doc’!

Neither am I of course, not officially. But after more than 12 years with CRPS I wasn’t surprised to be told by the specialist pain-management doc’ a few months ago that she was adding “expert patient” to my notes. It doesn’t seem to have made any difference, but it was a nice compliment! 🙂

As I write this now, I am no longer part-cyborg. And the ECG results came through as not showing enough heart-rate variability to officially pin the symptoms on the dys’. Not enough Jared Leto, I should think.

When I went to see the cardio’ again, (because I’m now getting near-faint symptoms after just ten minutes of activity and in fact all the symptoms are worsening at a very scary rate), I was sooo nervous! Not about the appointment, but about the likely unhelpful outcome.

I’ve been advised that there are no specialists in autonomic dysfunction, and that I’ll have to go and see other specialists about each symptom, starting with the most problematic. So… gastro-doc’ next then, as the continuous near-fainting and permanent nausea are the ones I’m fighting hardest to get something done about. The neurologist will have to be a close third because the varying loss of vocabulary and basic lack of comprehension generally is causing major problems at times. (Yes, I know I probably sound fine on here but I write this when I happen to be more able, and I re-read for errors about a zillion times before posting)!

By the way, he also advised that the array of other specialists that I should see, will also not know how to treat me! I ask you….what is the point? Send me to a specialist who does know, then. Surely that would be the next logical step? Bit of a waste of everyone’s time otherwise.

Strangely, he later added that there are no autonomic dysfunction specialists in this part of the country. So they do exist then? I am confused! If I lived somewhere else would I get treated?

I found out some more info’ about my ECG results. It turns out that there actually was heart-rate variability demonstrated. A normal heart-rate for an adult is 60-100 beats per minute, some online info’ refers to an average of about 72 beats per minute which probably varies with age. My average was consistently 70 across all three days, cool, eh? Um, not exactly! It may be a good average, but my heart-rates throughout each day were actually ranging wildly. It was pretty much the same each day, so as an example: on one day I ranged all over the shop from 34 to 154bpm! That’s not normal! Especially during quiet, chill-out days.

And this is where my glee at finding a specialist that knows about my condition ended. It turns out that there are people that know of, and people that actually know how! He knows of CRPS and some dys’ conditions like POTS. But He doesn’t have any experience in treating them. He does not know exactly why I am near-fainting all the time. I get the low heart-rate (vaso-vagal) near-fainting, but also the high heart-rate near-fainting that suggests something like the blood vessels not constricting in my legs, but he doesn’t really know. And he doesn’t appear to know how to find out, either. Which leaves me in a bit of a pickle, because you can’t treat something when you don’t know what it is you’re treating.

He’s asked for a fainting test, though. Although he says he doesn’t know if ‘they’ will consent to run the test on me as I haven’t actually passed out yet!

You don’t have to actually pass out to be diagnosed with POTS. I am puzzled as to why he thinks fainting is important in dysautonomia, but that near-fainting is not.

We already know that I have dysautonomia, albeit only through verbal discussion. I have no official diagnosis letter to wave at doctors. And a key diagnostic tool for dysautonomic stuff is this fainting test he’s referred me for. It’s known as the ‘tilt table test’. It basically entails monitoring the heart-rate while you’re at rest lying on a table, to which you are gently affixed(!), then the table is raised so that you’re nearly upright and the heart-rate continues to be monitored at intervals for a fairly long period, (I’ve been told that the appointments last about an hour).

The Tilt Table Test

Fair enough. A positive outcome from a tilt test would go on my notes and give future doc’s a medical starting point instead of going through the usual time-wasting rigmarole of doubt and confusion! So it could be helpful. Although bearing in mind that even 70% of POTS patients come up with a negative tilt table test, a negative outcome could actually be more detrimental when in the hands of the untrained and uninformed.

What I was not expecting was to be told that the reason for requesting a tilt test was to see whether I should have a pacemaker fitted……..*gulp*. Pacemaker???? At my age?? Egads!

This is his reasoning….he says he only knows of two treatments:

1)      Heart medications. Which affect the heart-rate, but as my heart rate varies so much… it can’t be medicated for. As soon as a med kicks in to solve one heart-rate problem, my heart will hippity-hop to a different pattern of behaviour, thus requiring different meds before the other ones have even worn off. So meds like beta-blockers and such like are not viable for me.

2)      Pacemaker! He says my ONLY option is therefore a pacemaker. It would be set to kick in if my heart-rate drops too low. But it would not solve any of the other problems and so would not be of much use overall. As a CRPS patient I need to avoid all invasive procedures unless absolutely necessary. And this would mean multiple surgeries as each pacemaker wears out over the years.

So that’s the choice that I’ve been given. No meds. Just…pacemaker…or continuous and worsening near-fainting.

He expected to wave me off to live my life despite continuously nearly fainting all day every day. Why did he not suspect that this may not be very appealing to me? Mysterious minds of specialists. ‘Can’t fix it, so it won’t get fixed, that’s just the way it is….byeeeee, have a nice life’. Really?? Yikes! I know of some meds that could usually be discussed at that point, but he doesn’t usually treat people like me so he just doesn’t know. In his view it’s simply pacemaker or nothing.

So is that it, then? There is no help?

I know that there isn’t much that can be done, but after doing lots of research and watching the Dysautonomia Information Network’s fabulously informative video about POTS, (a primary form of dysautonomia which has overlaps with my own dys’ symptoms), I was under the apparent misapprehension that some meds could at least be trialled with each patient to have a go at helping to reduce symptoms. Things like increasing sodium uptake to improve blood vessel constriction in the legs, or increasing serotonin in the brain to even out heart-rate, and so on. But the cardiologist is only specialist in his own area. So unless I can find someone out there by my own random and untrained searching of the health service, then I will only get to see specialist doctors who are not trained in the dys’ stuff.

Suffice to say that it was not a jolly experience that day. I was basically told that my worsening symptoms, that are preventing me from being on the planet and from doing-stuff/making-sense-of-stuff moooore and moooore of the time, are actually…. the New Me. 😦

My view of the world is rather like this a vast percentage of the time. My eyes don’t necessarily see it like this, it gets experienced like this.

So yes, I felt a few tears trickle down my face. Sometimes even the strongest and most determined cannot avoid a chink appearing in their armour once in a while! I kept talking, though. It’s not often I get the chance to see someone who has any clue at all about my condition, so I wasn’t going away without asking my questions first and being gracious and appreciative for his time. Despite the emotions wibbling on the inside!

Then I left the building circuitously, (via the disabled loo), and blew my nose as if I had a severe addiction to tissue and there was an international snot shortage that I was hastening to amend. There, sorted. No more snot shortage in the modern world! Job done.

Right. Deep breath. I had to drive home safely and get on with my day, and my life. Stuff to do, life to be lived. Check the bathroom mirror – how bad do I look after the torrents have passed?

Well, it had resulted in a comedy pink face, but my eyes looked all twinkly so maybe it’s a new look to consider?!

So with my imaginary (purple) cape swirling around me, I unlocked the door and stepped back out into the world to continue to be a version of me.

Okay, so the cardiologist wasn’t so helpful after all. So before I ask about a gastro-doc, and a neuro-dude, I’d better rethink the vascular issues. Hmmm…time to start searching for an Electrophysiologist, apparently!

Gnomes Bane. Daaahn-da-da-daaaaahn! My Clark Kent version of me was having a wobbly day. But that was that, no more crying. The answer was a nice cuppa. Isn’t it always? And maybe some commiseration chocolate. Oh, and this post, of course.

x

Newer post on autonomic tests (including TTT) here.