Yesterday I went to see my neuro-immunologist. I was really thrilled to be able to show him what has happened to me since I started my new treatment. He, in turn, was enthusiastic about recommending IVIG or Rituximab to try to address the unknown antibodies that have been wreaking havoc in my autonomic nervous system for so long.
What a foreign place I find myself in, here in the land of the well. Yet, utterly changed from my years in the wilderness.
I hear small whispers in my ear. “Be careful”, “Don’t overdo it,” “Choose your activities carefully,” “Do everything while you can,” “It’s now or never”.
I am more hopeful than I have been before, because we have found strong indicators that my problems are auto-immune. But there are equal parts fear and doubt. I guess you can’t be sick for as long as I have without being afraid that it will all come crashing in again. You can’t take feeling better, for granted. And because I don’t fully understand the science, I worry that I might be duping myself about it all. Maybe, somehow, I’ve convinced myself I am getting better. And that willpower will only carry me so far? It is a ridiculous thought, but there nonetheless. I have thoroughly examined my sanity, I have canvassed my doctors and my nearest and dearest. Apparently, I am sane. I just need to find a way to rest in this current state of relative wellness.
And then; there are the others. As each new day arrives and I wake, swinging into an upright position and moving to the bathroom with ease, I remember. How waking used to feel like I was drowning. I remember that for so many of you, it still does. And the burden of your illness weighs heavy on my heart. How can it not? I know your suffering. I know that for you, it carries on. And I try to fill my day with every little thing, for you. For me. For the unfairness of it all.
I am in this place of plenty, but I have forgotten the language of ease.
I can’t make it through a grocery shop without gushing to the checkout attendant. “How are you?” she asks, because it is part of the script. “Oh, I am GREAT! So good, like, really really wonderful!” I grin. And she eyes me with suspicion.
I can’t help out at my kids’ school without giddily and sincerely responding to thanks. I yelp, “My pleasure!” because it really is. Maybe it raises some eyebrows. This happy girl I am. Maybe it causes discomfort. Maybe it seems over-the-top. But it is not.
This life, this ease of moving, this chance to do things and be part of things.
If only you could feel how I feel.
I asked my specialist yesterday if he thought it was reasonable to expect this good run to continue. “It’s early days” he said. And I thought, in my stubborn way, that early days are always followed by late days and all of those days together make up more days of good than I was having before. And I shushed the whispers in my ear and skipped out to the car. The hubster commented that the last time we were parked in this carpark he’d had to help me to the car and get me into the seat. I swung my legs into the footwell and grinned at him. We drove home, my hand on his leg.
My heart in my mouth.
Please, please let this continue. I promise I won’t waste it. Not one second.
A week ago, I had my first Methylprednisolone infusion. It’s been an interesting week!
It’s as if, last Monday, someone beamed me up, out of the foggy, high gravity atmosphere of planet Pandysautonomia and re-homed me somewhere different. Somewhere the air is clear and the sun is shining. I must actually be a native of this new planet, because I feel so good here. Like all of nature was built for my body’s needs. The air, the water, the food, the beauty. It feels like I’ve been here before, it feels right. Have I slain the beast? Is it possible that this could be the beginning of the end?
I was so hopeful I would respond to steroids, because that bodes well for my panDysautonomia to have an auto-immune origin. I was also a little fearful that my high hopes would make me want to be well so bad that I would somatise wellness (is that possible?). But there is no doubt in my mind that what I am experiencing is real. I feel better. SO much better. I have been DOING things! Supermarket shopping without having to stop and sit! Walking my son to swimming, and back again, without my cane! Strolling around Bunnings with my family! Taking the kids to the beach, without my hubster! Helping out with household tasks! I know that is a lot of exclamation marks, but if you were me, you’d be full of superlative expression too!
Every now and then, I catch my hubster’s eye and the tears well up, for both of us. There is no joy like being able to help, to be part of things, to be effective. I am marvelling at my body. Wiggling my hips and jiggling to the beat. To dance! It is sublime. And, just now, I mounted our stairs. This is something that I have done with difficulty every single time since we moved in six years ago. The bannister has been my help; I heave myself up with my arms and my legs, pausing to rest along the way. But just now? I skipped up the inside of the stairwell, no bannister for support, no pausing. I made it the whole way, body upright, under my own steam!
Feeling like this, is frabjous news!
Feeling like this makes me realise anew, just how incredibly awful I felt before.
And it horrifies me that there are people feeling like I have felt for the past six years. Millions of them. Feeling like that and facing life regardless. Feeling like they are swimming through concrete as they fight for wakefulness each morning. Like their heads are fashioned not of blood and bone and brain, but stone. Like their will is too weak to force their legs to stand, to force their faces to smile, to force themselves again and again simply to do the smallest of tasks. It terrifies me that I might feel that way again myself. I try not to dwell on that. If this is temporary, then I am making the most of it! If it is a sign of better things to come; wahoooo!
One of the big changes for me is that my pelvic and hip pain has disappeared. I’d had x-rays just before Christmas, in case the pain I was experiencing was due to a structural problem. They were all clear. So our conclusion was that the old nerve pain from my previous surgery was back. It crept in a year ago, stealing a spot along my synapses and shouting out orders. Radiating down my leg and so insistent that I often couldn’t get to sleep. I carefully limited how often I took painkillers because I hate to rely on them. I knew I was in pain, but I didn’t realise how debilitating the pain was. It subversively stole my objectivity and didn’t stop until it had a good portion of my mobility, too. Last Monday, when the immunologist asked me for a state of play before my infusion, she wanted to know where I would rate that pain on a scale of ten. Ten being worst. I rated it at 4. Now that it is gone I can see how very much higher I should have rated it. That pain dictated so much about my sense of wellbeing, but I’ve been so long without the perspective of being pain-free, I had no idea how bad it was. In hindsight, I’d call that pain a 7. At it’s worst, a 9. And without it the smiles keep stretching slowly across my face. How much easier it is to be happy when you are pain free.
Other things I have noticed that make me happy:
less dizziness (YES! six years of being dizzy every time you move wears you down)
better temperature regulation
better perfusion in my feet and hands, less numbness and prickly pins and needles.
Easier digestion (the post prandial bloating still happens, but it is resolving itself faster)
more sweating at the right times (like when I am hot) and in places that haven’t sweated for a while.
less dry eyes and mouth
no nausea (win!)
I can walk for more than ten minutes
I am craving sweet foods less (I think this is because I have more energy)
Less of the regular sore throats
On the down side I have noticed
higher heart rates
higher blood pressure
problems with my eyesight
no significant improvement with my bladder or bowel
I tire quickly and need to take naps still
my medications might be a bit much for me now
When I was a little girl, one of my favourite poems was The Jabberwocky, by Lewis Carroll. I loved it so much that I memorised it. The nonsense words made so much sense to me and ever since, I have found ‘frabjous’ to be the perfect way to describe a great day. It’s so good to be able to write that I have had a great day. It’s so strange to be able to tell you that this great day has followed others! Here’s to more frabjosity. Goodness knows we all need more of that.
And if you are reading this and you are still stuck on that planet that sucks the life force from your bones, take heart. If a frabjous day can happen for me, there is no reason it can’t happen for you too. Hang in there, keep pushing, keep looking, keep trying. Sometimes, it is a long time the manxome foe we fight. Take hold of your vorpal sword and advance!
(from Through the Looking-Glass and What Alice Found There, 1872)
`Twas brillig, and the slithy toves
Did gyre and gimble in the wabe:
All mimsy were the borogoves,
And the mome raths outgrabe.
“Beware the Jabberwock, my son!
The jaws that bite, the claws that catch!
Beware the Jubjub bird, and shun
The frumious Bandersnatch!”
He took his vorpal sword in hand:
Long time the manxome foe he sought —
So rested he by the Tumtum tree,
And stood awhile in thought.
And, as in uffish thought he stood,
The Jabberwock, with eyes of flame,
Came whiffling through the tulgey wood,
And burbled as it came!
One, two! One, two! And through and through
The vorpal blade went snicker-snack!
He left it dead, and with its head
He went galumphing back.
“And, has thou slain the Jabberwock?
Come to my arms, my beamish boy!
O frabjous day! Callooh! Callay!’
He chortled in his joy.
`Twas brillig, and the slithy toves
Did gyre and gimble in the wabe;
All mimsy were the borogroves;
And the mome raths outgrabe.
There is a lot of frustration happening in the world of Dysautonomia, in fact, in the worlds of all chronically ill patients.
For some, diagnosis is fast and a cause is identified, treatment begins. Sometimes treatment is successful.
For others, for so many… diagnosis takes years. People are often mis-diagnosed first with anxiety, depression, hysteria and all manner of strange conditions. Until someone starts to put the puzzle together, piece by agonising piece. Someone recognising that the pattern of symptoms is autonomic is usually the start of that process. Sometimes, that someone is the patient themselves, forced into a position of applying their non-medical brain to medical journals. It is a scary and difficult job, sifting through medical literature, looking for words you recognise, learning as you go. But you have to. Who else will take the time? To whom else does your life matter enough that the research is worthwhile?
I don’t know why some doctors are excellent and others are not. But I know what the experience of a good doctor is from a patient’s point of view. A good doctor is prepared to listen. They will ask you good, probing questions. They are thorough, respectful and give you time to respond. That’s important when your brain is foggy and your words don’t always step out in the correct order. It requires patience toward the patient. I guess that is hard when the schedule is busy and they’ve been listening all day. But a good doctor, even after years of practise, can still say “What if?” and “I don’t know, let me see what I can find out…”. A good doctor is prepared to engage in the conversation with an open mind.
I’m sad today. Sad because since I’ve been working on this blog, I have a much more personal realisation about the affect of Dysautonomia on the lives of people who have reached out to me. Even here in New Zealand. I have had messages from people, more than ever before. Can you help me? My doctor won’t help me. I need information. I need recognition. I need support. They are asking: How can I make my doctors care about me? Take notice? I am declining. My life matters. Why don’t they care?
Like them, I too, am locked in a frustrating dance with a doctor who doesn’t seem to care. He is my main specialist and has taken over my case from someone who cared a lot. The contrast is painful. When I am in his consulting room I can see his attention drift off, his thoughts elsewhere. He often begins by saying things like “I see you have had an admission, but you are quite well now?” The question mark is an afterthought. I am supposed to say, ‘yes’ and go through the motions of the rest of the consultation so he can tick his boxes. But I am not ‘quite well’. My daily life difficulties are increasing all the time. We are accommodating the changes the best way we can. Navigating the path of progressive illness without guidance, lit only by the flame of our own frustration. He has decided the best approach with me, is to “observe the progression“. And because he has decided this, I must oblige. I am powerless to suggest, enquire, wonder, report, relay, present, offer… anything further. Because the doctor has decided.
But, Doctor. This life I have. I love it, I need it. Even broken and interrupted by illness, I want to be here.
It is valuable to me, Doctor, in your armchair.
I choose to fight for my family, for the love that pulses out past my illness and into every corner of their lives. I choose to fight for the autonomic nerves that have not yet been damaged by this disease. The longer you leave it, merely observing my progression, treating the symptoms, waiting, watching; the more my ganglia are picked off, one by one.
And there it goes. Another day. No other dollar. I am not working, I am not teaching. My work to do was worthy, wonderful work. I had much to contribute, I like to believe I still will.
And there. Another day. My children. Growing older, stepping out beyond yesterday and into a tomorrow I need to be part of.
Another day; my husband. Carrying burdens too heavy even for his broad shoulders. Pushing on. I want to help him more, be there for him, too.
This life is valuable, doctor. Do you see me? I am your impatient patient. The one who would rather not be a patient at all.
Should you come into my home and observe my life, not just my disease progression, you might try to take some action. You might begin to listen. You might think us worthy of that much. You’d be welcome. Doctor, get up from that chair.Get down from that high horse.
Another day came around. Another day with no more answers, no direction, no help.
And another day came around,
and another patient wrote to me. Can you help me? Can you help me find a pathway through? Can you make them see, Me?
Another day and I am counting More ganglionic death within my frame. Less function, more frustration. And you, Doctor, barely know my name.
Another day, but are you counting? do you record the way I do? do you read the publications scour the boards to read the news?
In another country,
I might be getting something done.
I might be making a kind of progress,
I might be a …’lucky’ one.
In another percentage band,
my life might take different course.
In another doctors hands,
my life might matter as much as
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.
Symptoms From Top to Toe:
‘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.
I 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)!
Dizziness 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.
The 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.
Heart 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.
My 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.
I am nauseous on and off all day and especially at night. This is a side-effect of the medications which produce peristalsis (intestinal squeezing).
My 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.
My 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.
I 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.
My 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.
I 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.
I 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.
I take Mestinon, Domperidone and Cyclizine 4x day.
I take Solificenate Succinate, Gabapentin and (a list too long to be bothered recording) of laxatives on an as-needs basis.
I use intermittent self-catheterisation and enemas as necessary.
I also have, and highly rate, osteopathy (thank you Glyn, you are a genius!)
I have iron infusions when my iron gets too low from malabsorption (these are also genius).
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!