Filed under: Uncategorized | Tags: AIA, arthritis, ataxia, balance, blogging, Covid-19, economics, fasciculation, genetics, health, introvert, jim downey, Lake of the Ozarks, Machado-Joseph Disease, medicine, MJD, neurology, neuromuscular disease, pain, pandemic, peripheral neuropathy, restless leg syndrome, RLS, SCA3, science, self care, sleep, society, spinocerebellar ataxia type 3, vertigo, Wikipedia
Yesterday I spent the most I’ve ever spent on a glass of water. And I couldn’t be happier about it.
See, my wife had a business function at a conference center at the Lake of the Ozarks. She’s retired, but still somewhat active with the AIA in a volunteer/mentor capacity. We drove down, checked into the hotel, dropped off our bags, and changed to go to a reception we thought also included dinner, awards presentations, and a full evening. I had a glass of water to take my routine evening meds, and we went off in search of the reception.
All went fine, but it quickly became clear that our expectations for the evening were somewhat at odds with what was actually planned. This was the first time this particular event was being held post-Covid, and contrary to the pre-pandemic routine (we’ve attended a number of these events), there was just a reception and brief awards ceremony. It was good that we were there, since my wife is a previous recipient of the same big award, and she was happy to support the new recipients. It was also important for her to be recognized for her past work on behalf of the profession.
But it was all over by 7:00 PM.
Now, since it was just an informal reception, we’d all be standing around chatting (and then listening to the presentations) for a couple of hours. A couple of hours during which some of my MJD symptoms made it abundantly clear just how much had changed for me since the last time I’d attended such a function (pre-Covid). I wasn’t miserable, but I was painfully aware of just how fragile I was feeling (as noted recently). Just being around people was work — even moreso than my usual introvert reaction to such events.
So, as we walked back to the room from the reception, we talked about just checking out and heading home. In just 90 minutes we’d be back in our refuge, I could sleep in my own bed and get back to my usual routine. It meant forfeiting the room cost, though.
I decided it was worth it. One of the lessons I’ve learned in my life is that sometimes you just have to write off the sunk costs of a decision, and get on with things.
So we changed again in the room, gathered up our things, and left. Got home safe & sound, played with the cats, crashed. I slept well (which I never do at a hotel), and this morning was able to get in my usual sunrise walk and exercise routine.
This wasn’t entirely due to MJD, of course. Partially it was due to the weird almost-over-but-not-really pandemic reality we’re still adjusting to. My ‘extrovert batteries’ aren’t what they once were, due to lack of use. And there was a breakdown in communications as to what was happening, so our expectations were at odds with what actually happened. Had we known that the event was going to be over so quickly, we would just have planned all along to return home after it was over.
But I do have to say that the changes I have experienced due to MJD were a major factor. None of my symptoms were noticeable to anyone else, and we didn’t discuss it with anyone. Yet I felt it, and it took a toll.
Lesson learned, and adjustments made.
Jim Downey
Filed under: Uncategorized | Tags: arthritis, ataxia, balance, blogging, Celeste Zink, cerebellum, Dr. Audrey DeClue, dystonia, genetics, health, jim downey, Machado-Joseph Disease, medicine, MJD, neurology, neuromuscular disease, pain, peripheral neuropathy, restless leg syndrome, SCA3, science, self care, Shivers Horses, Show Horse, spinocerebellar ataxia type 3, testing, The Horse First podcast, vertigo, Veterinary medicine, Wikipedia
My feet hurt. My hands hurt.
Actually, let me be a little more specific: the backs of my hands and wrists hurt. The tops of my feet and the fronts of my ankles hurt. If I were on all fours, you could see the symmetry of the places that hurt. It feels like all four joints were hyper-extended, and now hurt from it.
I put it into these terms because there’s value in understanding something about MJD: it affects the cerebellum, that part of the brain that controls movement and coordination, as well as having a role in pain awareness and some emotional/intellectual controls. This is an ‘old’ part of the brain that we share (in evolutionary terms) with most vertebrates.
Like horses.
Why do I mention horses, specifically?
Because of this:
Episode 51: Shivers Horses and Its Association to the Cerebellum
In this episode, Dr. Audrey DeClue focuses specifically on the association of the cerebellum to shivers horses. She shines a light on the existing, published research on shivers and the trouble with conclusions based on a small sample size. She also includes an interview with longtime friend, Celeste, to provide a powerful story of what it is like to live with a degenerative cerebellar lesion.
The ‘Celeste’ there is my sister. Who first experienced the onset of MJD symptoms about 20 years ago, and has been living with the disease since.
The whole podcast is well worth listening to, but the discussion with my sister starts at about the 28 minute mark, and lasts for about 20 minutes. In the interview Celeste is forthright about the challenges and pain associated with the disease, and how her symptoms have evolved over the last two decades. In listening to it, I was struck not only by her astute awareness of her body and how it has changed over the years (she is very smart and observant, no surprise) but also in just how much her early experiences echo what I have been experiencing the last couple of years (though my symptoms are mild compared to hers). That includes not only symptoms, but the frustrations of trying to get doctors to actually listen to her describe her subtle symptoms early on.
It’s an excellent interview, and Dr DeClue is a sensitive and talented interviewer. I encourage you to give it a listen if you are interested in this rare disease, and what it is like to live with it.
Jim Downey
Filed under: Book Conservation, Connections, General Musings, Health, Machado-Joseph, Predictions, Preparedness, Science, Society, Uncategorized | Tags: arthritis, ataxia, blogging, book conservation, bookbinding, dystonia, health, jim downey, Legacy Bookbindery, Machado-Joseph Disease, medicine, MJD, neurology, pain, restless leg syndrome, RLS, SCA, SCA3, science, spinocerebellar ataxia type 3
[I’ve decided to be public about my realization that I have the onset of MJD, the diagnosis process, and then living with the disease. Given the rarity of this disease, my hope is that this series of blog posts will help educate others, and perhaps provide some insight into it and related conditions. This is the third post in the series, written about a week after the second, as I started reframe what this would mean for me going forward.]
Been an interesting week, as I have been adjusting to the new normal of having MJD (as noted in my previous post, this is now where my head is at, though I won’t have an official diagnosis for about two months yet). It’s mostly been a process of re-calibrating my body awareness: noting that I have these muscle spasms in hands, feet, and legs, that the pain levels are there, and so forth, and then just moving on with my day. Living with chronic pain from a torn intercostal muscle that’s never healed properly, along with the random aches & pains of various abuses I’ve subjected my body to over the years of living life fully, means that to a certain extent the new aches & pains associated with MJD are just more of the same.
That’s not to discount the disease, nor the pain that comes with it. That’s real. And it has an impact. More pain, even if it isn’t worse pain, takes a toll. It wears me down faster. It’s more distracting, so it makes it harder to focus on any given task, especially creative ones. For the most part, though, I can just acknowledge the pain, listen to what it is telling me, account for it, and then get on with whatever I’m trying to do. So the pain isn’t the problem.
What is the problem is the degree to which this interferes with my bookbinding/conservation work. I’d been attributing the aches and pains in my hands to arthritis for years, and in truth I actually do have arthritis in some of the joints in my hands. That’s the result of the trauma inflicted by martial arts (primarily SCA combat) for about a 15 year period when I was a young man.
But worse than the aches and pains is the stiffness, spasming, and occasional loss of control in my hands. So far, the latter has only happened after I have been working using my hands (specifically while putting up the stamped copper ceiling in the kitchen this past winter). But I know it is just a taste of things to come. Dystonia is a classic symptom of MJD, and the spasms and pain in my hands that I’ve noticed the last few years is only likely to get worse and become more of a limitation. I had already started to scale back the conservation work I do, but now I need to be thinking in terms of finishing up pending obligations, handing off clients to other conservators, and becoming officially ‘retired’ as a book conservator. That’ll be a hard adjustment to make, since so much of my self-identity is tied up with it.
* * *
(a few days later… 4/7)
Something I’ve been thinking more about is the difference in the types of pain I’ve been feeling. This is distinctly muscle pain, as opposed to the kind of pain one has from arthritic damage to a joint, or tendon damage, or a broken/bruised bone. This is the kind of thing pretty much everyone has experienced at one time or another, from over-using a muscle (or a group of muscles). It feels almost exactly like what you experience the day after a really hard physical exertion, except it also feels a bit like what you experience shortly after exhausting the muscle in exercise. Think how your calves/feet feel after a five mile hike in street shoes: tired, twitching, and sore. Then add in how your calves would feel the next day, in terms of stiffness and that deep ache. It’s unusual in my experience to have both of those at the same time, but that’s pretty much what my hands and feet/legs feel when I’m having an episode of the MJD effects.
And that’s another thing: this isn’t constant. Not yet, anyway, for me. It’s episodic, lasting for a few hours, at most a day or so. Then it fades for a while. I haven’t yet been able to identify a pattern to the episodes, unlike my intercostal tear (which usually acts up in response to a change in barometric pressure or certain kinds of exertion). It’s possible that there isn’t one, and no way to predict what might trigger an episode. I suspect that may be the case, given that the episodes typically become longer and more frequent over time, until the pain is pretty much constant.
I’m sure I’ll find out. *sigh*
* * *
4/8
Bad episode today. Spasms, twitches, RLS (Restless Leg Syndrome), and a fair amount of muscle pain. Particularly in my hands. Possibly a connection to the cold front (we’ve had spitting snow and wind today, with a hard freeze forecast for tonight)? Whatever, been a rather annoying day. Particularly so since I had been planning on getting some binding work done, and my hands are just in no shape for it, even with having had them in the microwaveable mitts I use to loosen things up.
As I noted above, this is a psychological blow, as much as anything. I’ve never really defined myself in terms of my job, but it has always been one of the interesting things about me. Conservators are so rare that it’s always a talking point when I introduce myself to someone; they always ask about what sorts of things I work on, what’s the oldest/rarest/most valuable item, et cetera. Even surgeons, who seldom suffer from a self-esteem deficit, will pause and with a note of respect ask how I got into such a profession.
I’ll miss that. And I’m honest enough to admit it.
Jim Downey
Filed under: Alzheimer's, Amazon, Book Conservation, Connections, Gardening, General Musings, Genetic Testing, Health, Machado-Joseph, Predictions, Preparedness, Science, Uncategorized | Tags: arthritis, ataxia, blogging, book conservation, bookbinding, health, jim downey, Legacy Bookbindery, Machado-Joseph Disease, medicine, MJD, neurology, pain, restless leg syndrome, RLS, SCA, SCA3, science, spinocerebellar ataxia type 3
[I’ve decided to be public about my realization that I have the onset of MJD, the diagnosis process, and then living with the disease. Given the rarity of this disease, my hope is that this series of blog posts will help educate others, and perhaps provide some insight into it and related conditions. This is the first post in the series, as I started to come to terms with the realization.]
3/13
I’ve known this disease for almost 50 years. From well before my family even had a name for it. I’ve seen it kill family members by millimeters, and do its best to ruin the life of my sister over the last 15 years.
So, why did it take me so long to recognize that I have it?
* * *
Denial, perhaps. This is not the sort of future one particularly wants to face.
Or perhaps a sense of entitlement? That I have had enough other pain and suffering in my life, that I deserved to be missed by this curse?
Or maybe a lingering perception of invulnerability? It’s said that none of us can really envision our own mortality until it steps out of the shadows and confronts us face to face.
All of the above.
What I know is that I first experienced tremors & cramping in my hands several years ago. The stiffness and ache I had felt I attributed to osteoarthritis — I had abused my hands considerably during my years of jujitsu and SCA combat, after all. At first, I just figured that the tremors and cramping were due to the same.
Except arthritis doesn’t cause tremors and cramping. Some part of me knew this, but discarded the information.
The same was true of the foot/leg cramping, the RLS (restless leg syndrome). I figured it was due to walking. Or climbing ladders. Or digging through turf. Or just ‘one of those things’.
All plausible explanations.
Because some part of me didn’t want to acknowledge the possibility of MJD/SCA3 (spinocerebellar ataxia type 3). As noted, this is not the sort of future one particularly wants to face. Depending on the age of onset and some other factors, MJD can mean anything from almost total debilitation and an early death to prolonged suffering and loss of bodily control. And we’ve seen the full range in my family. Not the sort of thing one wants to particularly acknowledge, since there is no cure and precious little in the way of medical treatment for the symptoms.
What was harder for me to ignore were the moments when I lost my sense of balance. My formerly exceptional sense of balance; graceful, fluid motions on the field of combat or the practice mats of a dojo. Gone now. Oh, routine walking is still perfectly fine. But when I turn my head quickly, or close my eyes while still moving, things will spin/sway/shift for just a moment. That never happened before. Well, unless I got drunk. Because that’s pretty much exactly what it feels like.
And then recently, I started just dropping things. Particularly tools, when I was tired from using them after a couple of hours. My hands would just stop working for a moment, and the item would slip from my grasp.
Now, I can be as clumsy as the next person in just routine day-to-day stuff. But when I’m working with tools, my focus, my control, is exquisite. It has to be, in order to be a book conservator, as I have been for thirty years. I don’t just start losing control of my tools. Let alone dropping them.
But I have.
* * *
Just before New Years we got together with my sister and her family. We’d had visits during the pandemic, but they had been brief, sometimes outside, more superficial. For whatever reason, as we were talking about how she was doing in her struggle with MJD, we got a little deeper into the details than usual. And as we talked about her symptoms, it was like a checklist formed in the back of my mind.
A checklist of symptoms. A checklist with each box ticked.
I realized that it was possible that I had been experiencing the onset of MJD. I scheduled an appointment to see my GP, far enough out in the future that I figured that the Omicron surge would be past, and it would be relatively safe to talk with her.
I didn’t put it out of my mind — how the hell could I? — but I was able to convince myself that it made the most sense to just wait and see my doc, and likely then see a neurologist after. Because MJD is rare enough (like 0.005% of the population rare) that my GP had zero knowledge of it, as I knew from previous discussions with her about family medical history. But I did start paying closer attention to that checklist. And I did some more reading on the latest research and medical information about MJD. It turned out that there had been a LOT of additional research and publications, and the disease was now much better understood than the last time I had dived into the literature some five or six years ago.
After digesting that, I was reasonably certain that yes, I had the onset of the disease. I shared those thoughts with my wife and a couple of very close friends I knew I could trust to not freak out or over-react. I still wanted to wait and see my doc, then a neurologist, and get the results of the genetic test that would confirm whether I had the disease, and to what likely degree, before I said anything to my family or other friends.
* * *
Last Tuesday (the beginning of March, 2022) I saw my doctor. It went as I expected, with me explaining my suspicions and how they matched the symptoms of the disease. What I didn’t expect, as we discussed it, was that her questions about the disease sharpened and confirmed those suspicions. She agreed with my analysis completely, and I left the appointment convinced that I have MJD, and that I had likely experienced the first onset three or four years ago.
I’m still waiting to get an appointment with a local neurologist (through a referral from my GP’s office for insurance purposes), though that will probably happen in the next week or so. Everything above is what’s been playing out in my head as rehearsed dialogue for that meeting. Time and time again.
So I thought I would write it down. Like being a care-giver, it helps me to process and understand what I am going through. Perhaps I will make it public. Perhaps it will grow into something like Her Final Year, as a way for me to share my perspective and help others who experience ataxia or have someone in their life who does. We’ll see.
For now, I will keep these thoughts to myself. But will update as seems useful.
* * *
3/16
Today it was a week since I was told by my GP’s office that they had sent over a referral to the local university Neurology Clinic, where they actually have at least one person on staff who is familiar with ataxias. (I went and looked at the public profile info about the staff on their website).
Since I wasn’t quite sure how the University referral/scheduling system actually works, I called the Neuro Clinic, and chatted with the nurse who answered the phone. Yes, I can be funny and charming if necessary. Looks like my case is already in discussion with the docs there, and I should hear from someone soonish about actually getting in to get an appointment/evaluation. The nurse I spoke with said that the process usually takes a month or longer, and that it has happened in just a week means that it’s on a fast track. Looks like I’m special. Woo-hoo — lucky me!
* * *
3/18
Haven’t heard from the Neuro Clinic yet — hadn’t really expected to, but still …
I’m used to chronic pain. From the joints I’ve abused, from the intercostal tear in my side that I’ve had for a decade+, from the ache of bones broken in a full and vigorous life.
So the extra pain in my hands and feet isn’t really a change. And it’s not like the pain is any more intense or distracting. But it is a change. Like using a larger hose to fill a pool; the volume of pain has increased. It fills me up faster, reduces the energy/attention that I have for other things.
It’s like being out of shape, and discovering that you just can’t run as far or as fast as you used to be able to. It catches you a bit by surprise, and there’s a part of you that just wants to deny that something so fundamental has changed.
I told a friend (one of the very few who knows about this) that I’m currently uncertain whether or not I’ll put a garden in this year. It’ll depend on what I find from connecting with the doctors. Oh, I’m sure that I *could* put in a garden, but how much would that take away from other things I want to accomplish through the summer & fall? I’m being a little more cautious about how I commit myself, not knowing what resources I’ll have available.
So I hope the scheduling nurse from the Neuro Clinic calls next week.
Jim Downey
Filed under: Art, Book Conservation, Uncategorized | Tags: Anglo-Saxon, art, Beowulf, blogging, book art, book conservation, book design, bookbinding, bookbinding techniques, calligraphy, chained bindings, Cheryl Jacobsen, Dragon, Early Medieval England, Glowforge, goatskin, Grendel, jim downey, laser, leather, Legacy Bookbindery, Longship, vellum, Viking, Wikipedia
For many years, whenever I’ve given lectures, or taught classes about the history of the book, I would discuss the incredible value of books before the advent of the printing press (1454). I’d tell people that there was a reason such books were carefully guarded, even chained to a library shelf: they were about as valuable as a new car would be today, and you didn’t want them walking off.
Well, I was partially correct. Now, having done my part in creating a completely hand-made, hand-calligraphed edition of Beowulf, I can say that the value of such a book is AT LEAST that of a new car. An expensive one. Maybe two. I don’t actually know how much this book is worth. But I know that I put over 60 hours of labor into it. And I have a good idea of the cost of that much calligraphic-quality vellum. And I’m sure that Cheryl Jacobsen, who did the beautiful calligraphic work, must have hundreds or even thousands of hours of labor in the project.
What follows is documentation and explanation of my contribution to this incredible work of art. It’s photograph-heavy, so I’m going to put the bulk of it after a break, but here’s a glimpse of the finished product, to entice you:
Filed under: Uncategorized
[For some reason, Facebook is having problems with my ballistics blog being considered “spam”. Until I get it resolved, I’m going to post partial info about new blog posts over there, here, so people can link it off FB. Please just ignore if ballistics isn’t of interest.]
Ever hear of a 4 Bore?
Here’s the first line from the Wikipedia entry:
Four bore or 4 bore is an almost obsolete black powder caliber of the 19th century, used for the hunting of large and potentially dangerous game animals.
The term “4 Bore” indicated that it would fire a sphere of lead weighing 4 ounces, or one-quarter of a pound of lead. This was an old measurement system from which we also get our shotgun gauge measurements: a 12 gauge shoots a sphere of 1/12th a pound of lead, etc. So, a 4 Bore shoots a sphere of lead that is three times the weight of what a 12 gauge would shoot. As in a ball 1.052″ diameter that weighs 4 ounces, or 1,750gr. Compare that to a typical 12 gauge slug, which weighs from one to 1.125 ounces. The 4 Bore ball is more than three times the weight.
And shooting one feels like it.
[The entire post can be found here.]
Filed under: Uncategorized | Tags: .45 ACP, .45 Super, .460 Rowland, ballistics, BBTI, Glock, Glock 21, lasers, Lone Wolf, Timberwolf, Trijicon
[For some reason, Facebook is having problems with my ballistics blog being considered “spam”. So while I would usually just post about this on that blog, I’m also posting it here. While it is about a handgun, it’s also about some of the laser design stuff that I’m doing these days. Just ignore it if guns aren’t of interest. Thanks.]
Some years back I got a Timberwolf frame for my Glock 17 from Lone Wolf Distributors. It was shortly after they were introduced, and I liked the idea of the 1911-style ergonomics. I was very happy with it, and only wished that they had one for the larger caliber Glocks.
But as such things go, I never got around to following up and getting one when I heard that they had developed a larger frame to accommodate 10mm and .45 caliber Glocks. But I had recently introduced a shooting buddy to the 9mm Timberwolf, and that reminded me to look into getting one for my G21 I have set up to handle .45 Super. This one:
And I was pleasantly surprised when I found out that one option when ordering the Timberwolf frame is to get it without any texture. The idea is that Lone Wolf offers some different laser-texture designs as upgrades. But since I have my own laser …
… I decided to do my own custom design. Not just for the Grip, but as something of a theme for the gun. I could have completely personalized it, but decided that I wanted to stick with something which might be of interest to someone else, should I ever decide to sell the gun. So I came up with a geometric motif I liked which I thought would give sufficient grip texture to handle the fairly powerful .45 Super loadings. So what follows are pics of the process:
I have already had a chance to shoot it with this configuration, and was really happy with the way it felt in my hand. The texture was fine, and I felt like it wouldn’t slip around even if my hands were wet with sweat, etc.
And I’m very happy with the new Timberwolf frame’s ergonomics and how it points more naturally for me. Glocks are good guns, but they have always felt a little awkward in my hands, requiring more attention for me to shoot consistently well. This has solved that issue.
Jim Downey
PS: No, I’m not available to do custom laser work. But you’re welcome to see the full range of designs I do have available here: http://enlightened-art.com/index.html
Filed under: Uncategorized
[For some reason, Facebook is having problems with my ballistics blog being considered “spam”. Until I get it resolved, I’m going to post partial info about new blog posts over there, here, so people can link it off FB. Please just ignore if ballistics isn’t of interest.]
This past weekend I got to try the new(ish) Tavor TS12 semi-auto shotgun, made by IWI.
This gun got a LOT of attention when it was announced at SHOT 2018, and generated a fair amount of interest later when the commercial version was finally released not quite a year ago. And for very good reason: it’s a hell of a package.
[The entire post can be found here.]
Filed under: Ballistics, Uncategorized | Tags: ballistics, BBTI, data, testing
[For some reason, Facebook is having problems with my ballistics blog being considered “spam”. Until I get it resolved, I’m going to post partial info about new blog posts over there, here, so people can link it off FB. Please just ignore if ballistics isn’t of interest.]
So, I have some important news to share.
After months of discussion, and soliciting the opinions and suggestions from a number of people involved in the firearms/shooting community, we’ve made some decisions about BBTI going forward.
[The entire post can be found here.]
[For some reason, Facebook is having problems with my ballistics blog being considered “spam”. Until I get it resolved, I’m going to post partial info about new blog posts over there, here, so people can link it off FB. Please just ignore if ballistics isn’t of interest.]
Yesterday I got a box of cartridges. Now, even with the shortages these days, that isn’t that unusual.
But take a look at the contents:
OK, for scale: that’s a full-sized .44 magnum cartridge on the right, outside the box.
[The entire post can be found here.]
Communion Of Dreams 