Communion Of Dreams


Machado-Joseph Disease: Adjustment.

[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



Machado-Joseph Disease: Realization

[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



Binding Beowulf

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:

Continue reading

Shooting big stuff.
May 19, 2021, 4:58 pm
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.]



Customized Timberwolf G21
December 11, 2020, 4:48 pm
Filed under: Uncategorized | Tags: , , , , , , , , , ,

[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:

Here it is with the .460 Rowland barrel and compensator. I seldom shoot it in this configuration now, and the pics below show it with the .45 Super barrel and compensator.

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:

Bare frame.
First, I did the grip sides with this “3-D Cube” motif.
Then I added diamonds along the side of the front of the grip. This was slightly problematic, since my laser has limited focal range, and I couldn’t just stand the frame up to work on the front of the grip directly.
The backstrap was easy to do with isolated 3-D Cubes, since it is a detachable piece.
I like having some tape on the front of the slide, so came up with this simple design based on the 3-D Cubes.
Finished product, view 1.
Finished product, view 2.
Finished product, view 3.
Finished product, view 4.

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



IWI Tavor TS12 review
August 25, 2020, 2:51 pm
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.]



The Future of BBTI
August 24, 2020, 3:52 pm
Filed under: Ballistics, Uncategorized | Tags: , , ,

[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.]



So, you think .44 magnum is powerful?
August 2, 2020, 3:41 pm
Filed under: Ballistics, 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.]

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:

Box

OK, for scale: that’s a full-sized .44 magnum cartridge on the right, outside the box.

[The entire post can be found here.]



With apologies to Ursula K. Le Guin

So, since I haven’t been blogging here much in the last couple of years, I haven’t said anything about just how surreal it was working to finish my novel about a global pandemic … while an actual global pandemic was unfolding around us.

Yeah. Seriously. Real Lathe of Heaven stuff, making me wonder about just how much my envisioning a given reality was bleeding into this reality.

To a certain extent this had been an ongoing problem with writing St Cybi’s Well, as I had mentioned previously. I had to keep going back and making the ‘dystopia’ of SCW worse as our own world took a turn for the worse with the election of Trump, elements of Christian fascism seemed to be in ascendancy, et cetera.

But this year, after I had gotten a solid re-start on finishing St Cybi’s Well, watching the Covid-19 virus start to spread, was just … bizarre. And as you’ll see when you read the book, how the virus spread and the efforts that various governments tried to curtail it was pretty much exactly as what happened in real life. Fortunately, of course, C-19 hasn’t proven to be nearly as deadly as the Fire-Flu.

Well, at least not yet.

< shiver >

Jim Downey



“… telling you a tale that just *might* be real.”

So, almost two months ago I ‘officially’ launched the publication of St Cybi’s Well.

No, I didn’t forget to mention it here. Since I have allowed this blog to go quiet, I didn’t see it as an important venue to announce it, and figured that it would make a little more sense to just let the book exist in the wild for a little while, then write about the reactions to it.

Currently, there are 14 reviews on Amazon, with an overall rating of 4.9 stars. Some are from friends. Some are from acquaintances. Some are from complete strangers. Among the reviews I have my favorites, and not necessarily ones which say good things. At this point, after struggling with the book for so long, I have very mixed feelings about it.

But my strongest emotion about the book, and something that keeps coming up in the reviews of it, is just how surreal it is to have finished the book during the middle of a real pandemic, and having our reality seeming to follow the path I had laid out in the book. Here are some excerpts as examples of what I mean.

The first review, by someone who backed my Kickstarter and had an advance copy of St Cybi’s:

With some recent political developments and COVID-19, I found this unsettlingly realistic.

And from other readers:

That he wrote this well before our current pandemic was even a thing is a testament to his spooky prescience

And:

The images are vivid and remain. No one took epidemic plagues too seriously anymore, Polio was long ago. But since Covid and Ebola, there is a realization that the 4 Horsemen of the Apocolypse are alive and kicking.

And:

What I found most compelling is the almost prescient storyline of the Fire Flu and its attendant effects on society. I can’t imagine a more difficult proposition than trying to finish your novel about an apocalyptic disease while having to do so with one currently taking over the news. There are some eerie moments in the book where it feels as though it’s a ‘ripped from the headlines’ story.

And:

Set in 2012, the overlap with current events in 2020 is uncanny.

And:

the story is kind of terrifying considering its striking similarity to current events

Of course, I’m not prescient. I had no real idea that the coronavirus pandemic was coming, though I had long known that we were about due for another pandemic and were likely unprepared for it. And what I put into the book about how the FireFlu virus spread, and how people reacted to it, was just based on history. What we’re seeing now … all the good and bad of it … was entirely predictable, because it is the sort of reaction that human societies have always had to pandemics.

Which, of course, doesn’t give me any comfort. As is said in one of the reviews:

I ended up feeling that the story is part of what science fiction does best – telling you a tale that just *might* be real.

Stay safe. Stay healthy. Download my book, or order a paper copy. If money is a little tight, wait until the first of the month, and download it for free. And please, if you do read it, leave a review.

Thanks.

Jim Downey