Part I: Recognizable Progressions
These past three weeks have been emotionally more challenging for me. No doubt, my own sense of systems decline has caused me anxiety. I carry my anxiety like a backpack right in the middle of my back. And it aches, which in turn plays nasty tricks on my breathing, which is already waiting for some excuse to join the autonomic failure gang. But of course, I don’t let it.
I also admit that some of my anxiety has come from reading two peer-reviewed medical articles. They had to do with examining the progression of MSA over time. You might ask, ‘how the heck did I get on that topic?’ Easy.
I read some of the postings on FaceBook and people were asking each other what comes first? when does this happen? and how long?
As I read those questions I realized I too had these questions, but I had no reasonable answer, only ambiguity. I began to feel this chilling sense of vacantness and an absence of knowledge. It threw me back to when I was a kid, laying in my bed, alone and in the dark, and on the edge of fear. I knew - we all knew - that to vanquish that fear all that was needed was to turn on the light. I hoped to turn some light onto our shared questions. I think I did, but it wasn’t as illuminating or enlightening in the way that I had hoped.
The two research papers I looked at appeared to have gained credibility within the research community as they have both been cited repeatedly by others. Both studies involved a large enough sample size of patients to allow for reliable statistical data analysis and thus, generalizations. So, let’s see if some of our questions can be responded to through research.
First, both papers found, along with their own examination of related literature, that it is not possible to resolutely describe specific steps, stages of the disease, or time-frames that could be regarded as typical or universal. Such research findings underscore the often ambiguous nature of neurodegenerative diseases which in turn contributes to both the patient’s and medical practitioner’s frustration. It’s not like when we describe baby teeth coming before adult teeth. The unique, multi-faceted nature of the disease interacting with unique humans at different stages of life development creates too many possible scenarios. I think this is important for me (and maybe you too) to understand. Each of us is on a very unique journey and while we may recognize similar symptoms in each other, much like similar trees in a forest, our paths meander in unique ways. As rare as the disease is, so too is our journey.
That journey is pretty lonely and for that reason, I also believe that we need to reach out to each other on spaces like the patients’ MSA COALITION FaceBook pages. Sharing experiences and creating a conversation about them helps us feel understanding, connection, and empathy.
The research did offer some generalizations which we and our medical teams can use as discussion points. First, since symptom occurrences don’t follow reliable patterns, an alternative way to frame the stages is by describing the mobility of patients. This is logical because it is observable evidence. Researchers have used these terms to describe the advancing mobility dysfunction stages.
Level | Disease Stage | Mobility Stage |
Independent | Prodromal* / Premotor | Unaided mobility |
Aided Independent | Possible or Probable MSA | Cane, Walker |
Limited Independent | Probable MSA | Wheelchair |
Immobile | Probable MSA | Bedridden |
While researchers have recognized that some symptoms have a greater incidence of occurring in one of these stages, there does not appear to be a hard-fast rule. And there clearly is no defined precise time frame. At best, research offers a generalization based on reasoned inferences. It explains why when we ask these kinds of questions of our doctors they can’t provide precise answers. It’s not that they wouldn’t want to. It is just the nature of this beast. It does remain a good discussion point for you and your medical team.
In the second part of this posting, Trends and Relationships in the Progression of MSA, I want to share five patient/disease characteristics researchers have identified and how they relate to influencing progress.
*Prodromal: denotes the period between the appearance of initial symptoms and onset.
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Fanciulli, A., & Wenning, G. K. (2015). Multiple-system atrophy. The New England journal of medicine, 372(3), 249–263. https://doi.org/10.1056/NEJMra1311488 Foubert-Samier, A., Pavy-Le Traon, A., Guillet, F., Le-Goff, M., Helmer, C., Tison, F., Rascol, O., Proust-Lima, C., & Meissner, W. G. (2020). Disease progression and prognostic factors in multiple system atrophy: A prospective cohort study. Neurobiology of disease, 139, 104813. https://doi.org/10.1016/j.nbd.2020.104813
Watanabe, H., Saito, Y., Terao, S., Ando, T., Kachi, T., Mukai, E., Aiba, I., Abe, Y., Tamakoshi, A., Doyu, M., Hirayama, M., & Sobue, G. (2002). Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain: a journal of neurology, 125(Pt 5), 1070–1083. https://doi.org/10.1093/brain/awf117
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