Filed under: General Musings, Genetic Testing, Health, Machado-Joseph, Science, Survival, tech | Tags: arthritis, ataxia, blogging, health, jim downey, Machado-Joseph Disease, medicine, MJD, neurology, pain, restless leg syndrome, RLS, SCA3, science
[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 second post in the series, written a few days after the first, as I started to come to terms with the realization.]
According to the “stages of grief” I should probably be still somewhere around either denial or anger, in reaction to the realization that I am experiencing the onset of MJD. But I’m not.
I’m not some exceptionally well-adjusted person or anything. (Well, OK, I am, but it’s taken me 60 years and working through a lot of personal trauma to get to this point.) Rather, I think that’s mostly due to the fact that I’m not really ‘grieving’ the loss of my good health or anything. In the short term, this is mostly one additional annoyance of aging that I’ll deal with. I’ve already been living with chronic pain for more than a decade, and going through the cardiac catheterization six years ago was educational in terms of forcing me to re-adjust my perception of myself as eternally young. Yeah, that whole thing actually turned out to be a great benefit for me, correcting a previously unknown heart defect, but it was still a moment when I thought that I had a serious heart condition that would end my life sooner rather than later.
So I’ve been through the experience of reframing my expectation of ‘good health’. And I’ve found it relatively easy to accept that there’s about a 99% likelihood that I have MJD.
I realized this when I was talking with the scheduling nurse from the Neurology Clinic, setting up an appointment for my initial assessment with one of the attending physicians who has an expertise in neuromuscular disorders and ataxia. She said that when the staff saw my family history of the disease (from my medical referral) it was obvious who I needed to see and why. I don’t want it to sound like she shocked me, or let the cat out of the bag — it was I who initiated that aspect of the discussion. She just confirmed it. At that point I went from being reasonably sure what my symptoms meant to being all but certain.
And I found that I was at peace with that.
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: Amazon, Art, Book Conservation, Connections, General Musings, Kindle, Marketing, Promotion, Publishing, Science Fiction, tech | Tags: Amazon, book art, book conservation, book design, bookbinding, bookbinding techniques, Communion of Dreams, Kickstarter, Legacy Bookbindery, Science Fiction, St Cybi's Well, writing
Last May, I wrote about the process of designing and executing the artistic leather bindings of Communion of Dreams. Now that all of my Kickstarter backers have made their choices, I thought I’d give a preview of the process of designing and executing the artistic leather bindings of St Cybi’s Well.
I had a piece of Preseli Bluestone from the quarry at Craig Rhosyfelin (which is the source for the Stonehenge Bluestones in the inner ring). This site appears in a scene in chapter 8. Well, I had the stone cut into 14 slices (two times the magical number 7). Which I then used to construct a “well” as the cover design. The center of the well has thin blue leather to represent the water in the well. Like this:
Each of the 14 leather-bound copies will have one actual slice of the stone mounted on top of the leather, and thirteen ‘stones’ of bookboard under the leather for bas relief. In this way, all fourteen copies of the leather-bound edition will be connected into one “well”. Here are two examples:
As with the titling for Communion of Dreams, the letterforms are etched using my Glowforge laser, then infilled with real gold leaf.
The other major design decision was what to do for the endpapers. Communion of Dreams had marbled endpapers. For St Cybi’s Well I wanted something different. Thinking through the various visuals in the book, one recurrent image I used was of a Celtic spiral. A symbol of whirlpools and infinity, but also of the transition between realms of reality. Combine that with the ‘healing energy’ in the novel characterized as being a luminous blue. So this is what I came up with: a thin sparkly blue spiral, cut with the laser from commercial glitterpaper stock. It will be mounted onto black endpapers, one each on the paste-down sheets front and rear. Here’s an example:
I do have all the text blocks sewn up and ready to use. I’ve ordered the leather, and soon will be completing these bindings. There are five text blocks and five stones (numbers 1, 4, 5, 6, and 11) still available. If you’re interested in one, you can still choose your color of leather. Details here. Once I finish the nine books for my Kickstarter backers, I’ll just finish the remaining five in leather of my choosing (and raise the price).
I’ll post pics when I have the first batch finished.
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: Amazon, Art, Book Conservation, Connections, Failure, General Musings, Kindle, Marketing, Promotion, Publishing, Science Fiction, tech | Tags: Amazon, book art, book conservation, book design, bookbinding, bookbinding techniques, Communion of Dreams, Kickstarter, Legacy Bookbindery, Science Fiction, St Cybi's Well, writing
It’s been five years since I last wrote about my efforts to come up with a satisfactory cover design for the premium leather edition of Communion of Dreams. Well, needless to say, a lot has happened since then. Not the least of which was getting, and learning to use, my Glowforge laser.
And now I’ve finally resolved the many different design and execution issues to my satisfaction, to the point where I’m completing the promised leather-backed copies for my Kickstarter supporters. Here it is:
This is going to be a bit about this binding, and how it differs from the hardcover cloth binding.
First thing, the sewing is different. Rather than just being sewn onto linen tapes, the books are sewn onto heavy linen cords:
Why the weird arrangement? So that those cords provide additional texture to the spine of the finished book, along the location of where the tree branches are (see the first pic above). Once the sewing was done, the text blocks were glued up and rounded slightly. All of that was very straight-forward.
However, as noted in that blog post in 2016, the problem I had was trying to achieve the raised texture of the tree for the rest of the cover. I played around with a bunch of different solutions, until I settled on using the laser to cut out a slightly abstracted version of the Burr Oak image:
That’s in the bed of the laser. The material is archival 50pt board. Trying to cut out such an image by hand would take me hours, probably. The laser does it in about four minutes. (Though I did spend some considerable amount of time coding the design so the laser would do it.)
Here’s the image free of the surrounding board:
That is then pasted onto a sheet of paper, and the book cover boards are mounted on the back in the appropriate location. Then it is time to mount the leather, and impress it such that the tree is in relief, with this result:
(Actually, that was a practice piece, not the final version pictured above. But I forgot to take an image of the final version at this stage.)
The edges of the leather are then turned-in, and the corners formed. This gives you a finished case (what bookbinders call the cover).
Next, need to do the titling. And this is where the laser once again comes in very handy, though it took me a while to get just the right technique worked out. After the design for the title is done, the leather is masked and then engraved with the laser to an appropriate depth:
Once that is done, the engraved areas are cleaned of residual charred leather, and gilding size applied:
Once that cures, then it’s time to apply the gold leaf:
Now, that’s real gold, in multiple layers, about $25 worth. This process is different than traditional gilding done by bookbinders, so I had to work up a whole different process to do it (based on my experience with traditional gilding). The result is very satisfactory, though, since I have a much greater range of options for the final design.
Once the titling work was done, it was time to prepare to mount the text block to the case. First, I tear the outer page of the outer signature, and trim the cords to the appropriate length:
This combination, with the two liner tabs, will make for a *very* secure mounting to insure the cover and text block stay together. Then, you fray out the linen cords, so that they will not present excess bulk inside the cover:
Then the whole thing is pasted out and mounted inside the case, similar to how the hardcover cloth bindings were done. Once everything is dry and secure, I added endpapers of hand-marbled paper I made:
Giving the finished product:
I tried a lot of different color combinations, and have decided that this is the one I think works the best (and echoes the original cover nicely). My Kickstarter backers have the option of choosing a different color, but henceforth this will be the only color option available for other collectors.
Next, after finishing these bindings: designing the premium leather binding for St Cybi’s Well.
Jim Downey
Filed under: Ballistics | Tags: 12 gauge, bullpup, Crimson Trace, CT-1000, IWI, reviews, shotgun, Tavor TS12, technology, 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.]
Last August, I got to shoot a new IWI Tavor TS12 shotgun. You can find the full review here, but here was my conclusion:
Final thoughts: it’s a hell of a package. I’m not sure I’d use it for “sport shooting”, but for fun at the range or as a home defense gun, yeah, it’d be fantastic, though a little pricey.
Well, after thinking about it for a few months, I decided to go ahead and get one. Here it is:
[The entire post can be found here.]
Filed under: Amazon, Art, Book Conservation, Failure, General Musings, Kindle, Marketing, Promotion, Publishing, Science Fiction, Wales, Writing stuff | Tags: Amazon, book art, book conservation, book design, bookbinding, Communion of Dreams, Kickstarter, Legacy Bookbindery, Science Fiction, St. Cybi's Well, writing
Last week I went through the mechanics of turning words into books. Today I’d like to talk a little bit about the economics of that, as well as being an author.
When I set up the Kickstarter for St Cybi’s Well lo these many years ago, a lot of my estimates in deciding on what goals to shoot for were based on … blind faith, to be perfectly honest.
I’d published Communion of Dreams, and had some sense of the possible income that could generate, as well as the amount of work that went into writing/editing/formatting it. But I really had no idea what the costs would be for printing and hand-binding copies of that book or the yet unwritten St Cybi’s Well. I had a pretty good handle on how much time it would take me to do the hand binding, based on my book conservation work. And likewise, what the cost of materials would be, other than the printing. Now that I’ve gone through all of that with both books, I have a much better idea of how costs break down, so I thought I would share all of that.
Based on 55 copies (edition of 53, plus two author/artist proof copies), the actual out-of-pocket expense runs about $50 per copy for the two different books. That covers the cost of the oversize archival paper (so the grain/drape of the pages is correct for hand binding), the printing, and going to collect the printed pages. It also covers the cost of archival bookboard and cover cloth. (The leather copies are two or three times as much, depending on the actual leather used.) Were I to bind a single copy, it would take me about 2 hours of labor. If I bind multiple copies at a time, that drops to about 1.5 hours of labor. (Leather is about 2x the labor.)
My Kickstarter goal was $17,000. Which succeeded. After deducting the fees paid to Kickstarter, and the costs of the different “rewards/premiums” for the backers, I wound up with about $12,000. Which, truthfully, isn’t a bad advance for a relatively unknown author.
It took me seven years of writing work to finish St Cybi’s Well, rather than the one or two years I originally expected. That was both embarrassing and stressful, since I made promises to people I didn’t fulfill. But it’s over, and everyone seems happy with the end product, so let’s just talk about the amount of labor that went into it. I’d conservatively guesstimate that I have something on the order of 2,000 hours of labor in writing, rewriting, editing, and then composing (the last is mostly thanks to my Good Lady Wife) the book. That *might* be as much as twice as long as it took me to do the same with Communion of Dreams, though spread out over a longer period of time. So, do the math, and I earned about $6 per hour.
That’s just the Kickstarter, of course. Now that the book is done, I’ll continue to earn money on purchases of the downloads, printed paperbacks, and special order hand-bound copies. How much is hard to say. I have had about 40,000 downloads of Communion of Dreams, though a big chunk of those are free downloads. Still, with Amazon’s system, 2/3 of every sale goes to the author (as opposed to like 5-7% for conventional royalties with a publisher), and that adds up. To date, I’ve only had about a thousand downloads of St Cybi’s Well, and most of those have been free downloads. Which has been a little disappointing, but we’ll see how things go over time.
As for the printed paperback copies, there I earn a bit more from Amazon than I would for the downloads, but not a lot. And they don’t amount to very many sales. The signed copies I sell directly do a little better still, but again, that’s just a handful of books per year.
As for the hand–bound books, there I do make a lot more, about $150 per copy. But my conservation labor is billed at $200 per hour, so at 1.5 hours of labor, I’m making half of what I would professionally. The return on the leather bound copies is even worse.
So, why do it? Why even offer those books on the websites?
Because I enjoy it. I enjoy knowing that the books that I create will be enjoyed, perhaps cherished. Read, and passed down to children and grandchildren. Like most artists, I’m willing to trade some financial reward for that satisfaction, and I can afford to do so. Maybe it shouldn’t be the case, and it wouldn’t be the case ideally, but we do what we can under the circumstances.
Anyway, my books will be available for free download tomorrow (the First of the month, as always). Give ’em a try. If you like ’em, you’re always welcome to either purchase a download or one of the physical copies later, if you can afford to do so. But don’t feel guilty if you can’t; as noted I can afford to give them away, and take joy in knowing people read them.
Jim Downey
Filed under: Amazon, Art, Book Conservation, Pandemic, Plague, Publishing, Science Fiction, Society, Wales, Writing stuff | Tags: Amazon, book conservation, bookbinding, Communion of Dreams, Covid 19, Covid-19, Kickstarter, Koob, Legacy Bookbindery, life, pandemic, St. Cybi's Well, writing
As I noted a couple of months ago, I found a printer to produce the pages for a hand-bound copy of St Cybi’s Well. This is a photo-essay of the process of turning those pages into finished books. This is not intended to be actual instruction on how to bind books.
If you would like your own hand-bound copy of St Cybi’s Well or Communion of Dreams, click the links. Each edition is limited to just 53 numbered copies, plus two Artist’s/Author’s proof copies. At either link you can also order one of the remaining copies of the Amazing Koob, as well as signed paperback copies. And of course, you can always download the books from Amazon (remember, they’re free on the first of each month).
That completes the next-to-last phases of my Kickstarter. The final phase will be the design and completion of the leather bindings.
Jim Downey
Filed under: Art, Connections, Health, Pandemic, Society, Survival | Tags: art, coronavirus, Covid 19, health, pandemic, survival
So, I have an idea …
Yeah, usually that should be read with some trepidation.
This is an idea for an art installation, which would be intended for people to walk through to experience. One about what we’ve just lived through, and what we still don’t know when will end. For now, it’s a thought exercise, though I am giving serious consideration to how it could be practically accomplished. This is the general outline for the idea.
Picture a WW2 era naval mine. Like we’ve seen in countless submarine movies. Like this:
Remind you of anything?
How about this:
The installation would be set in a very long, narrow hall with low light. The floor would have dates marked on it, starting in January 2020, and progressing month by month until some time in 2021.
Tethered to the floor would be helium-filled balloons* at different heights. The balloons would look like the coronavirus particle. At first, there would be just a few, well scattered apart. But as you progressed through the hall, the number of balloons in a given ‘month’ would rise and fall, according to the waves of the coronavirus pandemic in terms of cases/deaths.
And as someone touched one of the balloons, they’d pop. Or flash. Or turn a different color. Or something like that to indicate that they had been touched. This would indicate to the viewer that they had just become a statistic of the pandemic.
The installation could be site-specific, reflecting the experience of a given country, or state, or city with the pandemic in terms of the numbers and timing of the waves.
That’s the idea. If you have a venue, and would be interested in creating such a work, contact me.
Jim Downey
*it wouldn’t need to be balloons, but could be solid plastic spheres or something, either on a free-standing stalk or positioned on a wire anchored between floor and ceiling.
Edited to expand: The more I think about this, the more practical I see it. I would set aside the idea of balloons, instead go to touch-sensitive LED lamp technology, and put that (probably with a sound element) inside semi-translucent plastic cast virus particles, positioned on a wire.
Communion Of Dreams 