I don’t think that there is anything that more profoundly impacts our state of ataxia than the stress related to anxiety. It encompasses every aspect of our being. We feel anxious about the disease itself. We feel anxious about how the disease affects our performance and lifestyle. We feel anxious about how we are going to cope with the changes coming our
way.
Personally, I find it curious that helping us to voice, understand, and manage our anxieties is not something discussed in the doctor’s office very much. Anxiety is uniquely different for each of us. Thus, it becomes our job to learn about it and manage it. And while that is a very good thing, it is no easy task.
In this three part series, I want to explore some of how anxiety is created and how it affects Ataxians. In part one I want to provide us with a framework for discussing anxiety and its connection to ataxia. Looking at some of the triggers and issues surrounding ataxia that fuel anxiety with be the subject of part two. One of the most subtle but powerful sources of anxiety is our own sense of shame which desires its own discussion. Over the next couple of months I hope you will join me in this journey and I invite you to leave a comment here or on one of the FaceBook pages for Ataxians.
It’s Wednesday morning and for five days straight my body has been extremely kinetic — twitching, tremors, visual blurring, and lots of tipping, toppling, but no tumbling, thank goodness. (see …). I describe this as my new normal now and I actually get anxious when I am not vibrating like a freezing Chihuahua. I wobble through my PT regime and strength training at the gym, come home and melt into what I refer to as a crash. You know it. That feeling like your eyes just want to close, your legs are jelly, nausea sets in, your eyes are darting all over the place, and your head feels like it is stuffed with feathers. (These are all precise medical descriptions, right ‽) But I have a meeting in two hours and our on-line support group later. I do what most of us would do. I sit and regroup. Where the hour went while I was regrouping, I have no idea, but it passed and I felt somewhat better. I figure I had better get ready for the meeting and I stand up.
The shaking and trembling start up immediately. My balance is off and I can feel my coordination is clumsy as I step into the kitchen. The weakness is not as bad as it was an hour before, but it is definitely there. I am worried about the meeting, how I will manage, and how I will be perceived. At that moment I decide to take my trusty wheelchair, “Smirf” — its blue and awkward — to the meeting and my mind immediately goes to thinking about how accessible is the meeting place? My body kinetics dial up more.
Looking back at this episode I saw how anxiety related stress was fuelling my condition. My crash was not my sense of normality — something is wrong. I have things to accomplish today — regardless of how I feel. I need to use my wheelchair, which is socially symbolic of being “disabled” and I needed to figure out how to navigate the accessibility of meeting location
.
Gosh, it is pretty easy to just give into it, crawl onto the couch and turn on Netflix. And if that is how you respond, that is OK — you know you! But you also know my mantra: Ataxia - Live Into It. It is my personal battle cry of not letting the disease control my life any earlier than it absolutely has too. Three years ago my muscle memory (a bonus feature of the cerebellum) would have glided me effortlessly out of the house, into the car, and onward to the meeting. Today, I struggled to keep balanced, to coordinate my uncoordinated limbs, and focus my vision, feeling that each movement was new and foreign to me.
So, why did I swing from being wildly kinetic, to rested, to comfortably active, to crash and back to kinetic all in a space of a few hours? It tends to be a mystery to which Dr. Kamran Khodakhah* from Einstein College of Medicine has shed some light on. His very technical report presents an explanation as to why stress triggers ataxia symptoms. I am going to make an attempt to explain it.
Before we get into that, I want to share with you a working definition as to what “anxiety” is. I use Brené Brown’s pithy definition: anxiety is concern about the future. It is the feeling we get when we sense we are not in control of what is happening or will happen. Given that Ataxians by default, have a sense of lost control over their coordination, balance, gait, vision, and motor skills, we have a built in anxiety generator! Brown also clarifies the relationship between anxiety and stress. Stress, she offers is what we feel when the events we are experiencing demand more of us than we anticipated. Again, for Ataxians, this can be a pretty low threshold and I think you can see how anxiety can feed stress. So let’s assume that there is connection between stress and anxiety and look at Kodakhah’s research regarding stress and ataxia.
As with most medical treatments and theories, Khodakhah used mice to simulate the condition of stress and ataxia, so his results are not proven in human subjects. But, this is the typical starting point for neurological research. He and is researcher found that if they induced stress into mice by activating the alpha-1 adrenergic receptor on the Purkinje cells (let’s just call these ‘unique neurons’) in the cerebellum, that these neurons released a neurotransmitter called GABA (ready for it? - Gamma-amino butyric-acid) and this stuff regulates and coordinates our muscle/motor movements. Let me say it like this as it helped me to get my brain around it: In the cerebellum there are these unique neurons. They look like fine tree branches that are reaching out toward the surface of the cerebellum. These unique neurons produce GABA juice which controls our motor movements, like moving our legs.
So, if everything is normal and we have a healthy cerebellum and typical life stress, then everything is cool. But that is not us. We have a disease that is actually degenerating those unique neurons. Plus, we have multi-source life stresses, arising from that degenerative condition also triggering those juices. I am pretty sure Khodakhah (Ko-dah-ka) would give me an F- for that interpretation.
Dr. Khodakhah found that when they activited stress in mice with ataxia, those unique neurons, fired erratically causing abnormal muscle movement and coordination. Rather than the cerebellum creating fluid movement through synchronized neuron firing, the ataxia brain fires like a warehouse full of fireworks.
“Disruptions or damage to the cerebellum often result in discoordination and dysmetria of body and mind, as observed in patients with cerebellar ataxia” (Bergland, 2021)
In our case, we already have a weakened unique neuron structure in the cerebellum that by itself produces abnormal muscle movement and poor coordination. When we add a neurologically based stressor, like anxiety, into this mix, we end up with magnified abnormal movement and coordination problems. At that point, I would posit that our survival instinct jumps into action and encourages us to stop doing what is causing us to be anxious, which leads us to want to cocoon onto the safety of our chair or couch. And if we do that too much we begin to withdraw from our life, our family and friends, and society.
The seeking refuge scenario may sound familiar to you. It does to me, and it is what I try to focus my energy on fighting. I want to still feel alive and enjoy my friends and family. I want to get out into society and participate for as long as I can. For me, that means understanding and wrestling with my anxieties. And it means willingly accepting and adapting to this evolving life.
In the next part I will look how other Ataxians are triggered by stress and identify at least four categories of anxiety stressors that seem to impact us and our symptoms.
You can read Khodokhah’s research article on-line at https://www.science.org/doi/10.1126/sciadv.abh2675 . I warn you. It is very technical.
I highly recommend any of Brené Brown’s books. Here I referred to Atlas of the Heart. Brown, B. (2021). Atlas of the heart: mapping meaningful connection and the language of human experience. First edition. New York, Random House.
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