My Hero, Zero

The pacemaker technician stared at the print out. She showed it to her supervisor.
“Rachel, you’ve had zero episodes in the last six months!” the supervisor grinned. I tried to understand what she was saying.  My pacemaker is set up to pace me up to 100 beats per minute if my heart rate drops rapidly. This is to prevent asystolic episodes like the one they caught on my Tilt Table Test when I first got sick. It’s been my security, my safest and most effective medical intervention over the last seven years. It even got to the stage where the wiggly worm feeling in my chest when I have a pacing episode was comforting. But come to think of it, I haven’t felt that for some time…

“Should we check to see if it is working?” I wondered aloud.  They nodded, one of them had never seen a zero reading before and it was important to make sure it wasn’t because something had gone wrong with the pacing program, the battery, or something else. So they looked at the data, ran the tests, paced me a few times to make sure it was behaving according to the parameters. They commented that all my stats looked great, like a young woman.

And a happy bubbling thing broke the surface of my brain.

ZERO!

A picture of me making the hand symbol for zero

When I started immune suppression at the beginning of this year, I couldn’t even imagine a result like this one.  But there it is. Concrete evidence that things in my body have changed.  I don’t understand it. But I love it. And I have a new favourite number.

Zero is my hero.

 

Pacemaker Progress

Yesterday, I had my pacemaker checkup.  Usually, these happen now every six months, but this one was a little shorter in time because they recently interrogated my little device when I had my second Tilt Table Test, right before starting my infusions.

We’ve been looking for some objective data to measure the improvement since the immuno-suppression began in January.  I’ve been feeling so amazing compared to before, and doing more all the time.  There have been crashes when I have pushed too hard, but the trend from my point of view is upwards!  So it was really cool yesterday to find that my heart is needing so much less help from the pacemaker.  In the time since my new treatment, I have only had 62 rate drop episodes, most of them during the night (it’s normal for the heart rate to drop during deep sleep, but my pacemaker still paces me, even then.  These episodes are much more benign than the ones when I am up and about).  62!  Most of which aren’t a worry at all! So happy.  I don’t remember ever having a number across such a long stretch of time that was only two digits.  Real progress!

yep, that's right_

And because ridiculous hopefulness is part of my way of being at present, I enquired whether or not it meant that one day I might be able to have my pacemaker removed. Lisa, the lovely nurse specialist (who was in theatre with me the day it was inserted) said it would be more likely they would just upgrade me when it was time, to the new MRI compatible pacemaker.  She thought having one on board would be sensible, even if my wellness continues because, well, you know, old age.  Maybe she is doubtful this good run will continue. But I am hopeful.

For me, the MRI compatible pacemakers seemed frustratingly pointless, because my understanding was that my leads were not MRI compatible.  When they change out your pacemaker, they don’t swap the leads.  That’s because over the last six years, my leads have been growing into my veins.  Removing them would be too dangerous, there isn’t even a surgeon in NZ who does such a surgery. But Lisa told me yesterday that my leads are MRI friendly!!  So happy about that!  It means that down the track, once this old model is replaced, if I ever need further testing about anything, I’ll be able to have it.

So two pieces of great news!

Did you ever wonder how they interrogate a pacemaker?  The hardest part about it is finding a park and getting to the pacemaker clinic on time. Otherwise, it’s as easy as 1, 2, 3!

STEP ONE_

STEP ONE_(1)

STEP ONE_(2)
And that is it!  Done!
At Auckland City Hospital, there is an awesome barrista on Level 5.  So if there was ever a step four, it might be a quick detour to pick up an excellent coffee!

Do you have a pacemaker, too?  They’re such clever little devices aren’t they?  Are your pacemaker check-ups like this one?  Or maybe you are going to get a pacemaker… got any questions?  Or maybe you are just curious… my pacemaker lump has always invited curiosity and tentative ‘feels’ from people I know.  I’m all about the information, so if you have any questions at all, I’m your girl.

 

A Medical Menagerie

 

This may be of interest to you if you also have Dysautonomia, Pandysautonomia or Seronegative Autoimmune Autonomic Ganglionopathy, or if you know someone who does.  If neither of those apply, please don’t feel obliged to read through.
I record below all of my medical weirdnesses because I know that people will find recognition here. I took a lot of comfort from learning that there were others like me when I first heard my diagnosis.  I have recorded all my symptoms because sometimes, something small can be a clue.  I am looking for answers and you might be too.

nb_-i-keep-this-list-here-for-others-looking-into-their-own-condition-i-am-now-well-but-what-you-see-below-is-how-things-were-for-me-before-i-went-into-remission

 

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Symptoms From Top to Toe:

Screen Shot 2014-05-26 at 4.32.54 pm‘Cognitive Fog’; memory issues, variable ability to concentrate, dizziness.
 These challenge my very identity.  I was smart and now thinking is much harder for me. Sometimes I sound like I’ve lost my marbles, my words get jumbled and my memory scrambled.  It’s distressing.  If I sound weird when I talk to you, it’s not that I am stupid.  I am just struggling.

Screen Shot 2014-05-26 at 4.32.54 pmI have trouble with vision sometimes, my eyes go into focal spasm.  My pupils are not always equal in size and sometimes they don’t adjust to the light properly.  My eyes are often dry.  Other times they stream and people think I am crying.  But it’s just my Dysautonomia (as moved as I am by whatever it was you just said)!

Screen Shot 2014-05-26 at 4.32.54 pmDizziness when I have a postural change has been my reality since I was eight. Sometimes I am dizzy even lying in bed and it feels like someone is slowly tilting my bed backwards in a full circle. It feels a bit like being in a raft on the ocean and I feel like I am going to fall off. Or I have a kind of up-and-down dizziness when I am just standing (like I’m bouncing internally), or presyncope when I bend over, or stand from squatting. My dizziness makes me feel unsteady on my feet and I bang into walls and doorframes. I always have to steady myself against something if I have to stand. I use a ‘shooting stick’ cane that has a seat so that if I need to sit quickly, I can. I can also use it to prop myself up.  Descending stairs is terrifying. Dizziness is never something you get used to. You learn to breathe through it, but your body always feels stressed during a dizzy episode.

Screen Shot 2014-05-26 at 4.32.54 pmThe inside of my mouth feels like after you’ve taken a big mouthful of hot soup.  That burnt tongue feeling? It is sometimes tender, dry and my gums are red and sore.

Screen Shot 2014-05-26 at 4.32.54 pmHeart issues are part of my scenery, managed mostly by my wonderful pacemaker.  I don’t take cardiac medicines anymore.  My heart rate ranges from the early thirties to the mid two hundreds. If my heart rate drops rapidly, my pacemaker kicks in.  I have had thousands of rate drop episodes since my pacemaker was inserted. I am grateful for it every day. My blood pressure can be very low, or very high, or somewhere in between.

Screen Shot 2014-05-26 at 4.32.54 pmMy stomach, digestive tract and bowel can miss important nerve messages that make them work.  This can result in a ‘pseudo obstruction’.  First, I get ‘post prandial bloating’; my tummy distends like a ten-month pregnancy.  Then, all tummy services are suspended  (so much for never having to be pregnant again)!  These days I wear forgiving clothes and I frequently get asked if I am expecting. When it is extremely bad, I make the trudge back in to hospital. It’s hideous; but my new normal is a bowel that doesn’t work without extreme inducement. I take ten various doses of laxative a day and also self administer enemas.  I hate this symptom most of all.

Screen Shot 2014-05-26 at 4.32.54 pmI am nauseous on and off all day and especially at night.  This is a side-effect of the medications which  produce peristalsis (intestinal squeezing).

Screen Shot 2014-05-26 at 4.32.54 pmMy bladder either retains urine and have to self-catheterise, or I am incontinent.  I have detrusor dysfunction, so emptying my bladder fully is tricky. Sometimes for brief and lovely periods, the pendulum passes through the middle of the arc and my bladder behaves like it used to.

Screen Shot 2014-05-26 at 4.32.54 pmMy body doesn’t regulate temperature properly.  Some parts of my body can’t sweat anymore, so other parts of my body overcompensate.  I struggle greatly with heat.  I don’t know how to dress for the conditions because my experience of them is not accurate.  In the past, I often over-dressed or under-dressed my children.  Now they are old enough to let me know if they need thermals and a jacket.  For me, it is always layers so I can try to manually adjust things from the outside in.

Screen Shot 2014-05-26 at 4.32.54 pmI get fasiculations in my muscles, in random places all over my body, sometimes these go on for a long time, other times it is just for minutes.  Sometimes, they are not there at all.

Screen Shot 2014-05-26 at 4.32.54 pmMy leg muscles are weak, so standing up from a seated position is hard.  I struggle going up and down stairs or uphill.  My left leg often burns.  I’ve been told this is nerve pain.  Sometimes I have a strange gait, it is tricky at these times to lift my feet properly.

Screen Shot 2014-05-26 at 4.32.54 pmI have pins and needles and burning sensations in my feet and hands, known as peripheral neuropathy.  We found an excellent medication for this, but now I only take it when it is extreme because the medication made my brain fuzzier.

Screen Shot 2014-05-26 at 4.32.54 pmI live with fatigue. It stands to reason that my body is tired.

 

Medications and Treatment (pre-Immune Suppression)

The general approach with Dysautonomias, is to treat the symptoms. So for everything you see above, there is something I take or do to try and help it.

 

Screen Shot 2014-05-26 at 3.40.06 pm

  1. I take Mestinon, Domperidone and Cyclizine 4x day.
  2. I take Solificenate Succinate, Gabapentin and (a list too long to be bothered recording) of laxatives on an as-needs basis.
  3. I use intermittent self-catheterisation and enemas as necessary.
  4. I also have, and highly rate, osteopathy (thank you Glyn, you are a genius!)
  5. I have iron infusions when my iron gets too low from malabsorption (these are also genius).
  6. I am currently seeing some immunologists who think it is probable that I have sero-negative Auto-immune Autonomic Ganglionopathy.  Pulse IV steroids point to an auto-immune aetiology, so moving forward I may trial other immune modulation therapies.

I rely on my husband, my kids and my friends for help, encouragement and support.  I don’t like to ask, I don’t like to receive, but I am learning how.

Still with me?  You are amazing!  Thanks for sticking around!