Filed under: Art, Connections, General Musings, Wales | Tags: Archaeology, art, blogging, book art, bookbinding, Glowforge, Helen Geake, Ireland, Jeremy England, jim downey, laser, Patreon, Phil Harding, Scotland, St. Cybi's Well, Tim Taylor, Time Team, Wales
My wife and I have been big fans of the UK TV show “Time Team” for a long time, and a couple of years ago when producer Tim Taylor decided to try and revive the series using a Patreon model, we were happy to sign on and tell a number of our friends about it. Unsurprisingly, we discovered that the series was also popular in our friends group, especially those who share our interest in English/Welsh/Scottish/Irish history.
Well, about a year ago, one of our friends entered a competition hosted by Time Team about the series, and, well, I’ll let her tell the rest:
Maybe you remember back in October, Time Team asked people to tell them “What are you most excited about Time Team returning, and why?” Like many people I fangirled a bit to show my appreciation, then forgot about it.
So I was very surprised when Time Team contacted me around Thanksgiving to tell me they liked my comment so much they are sending a gift. I asked if they would send it to the friends who had introduced me to Time Team, and given Customs and all they thought it would be less confusing if it came to me.
And she sent it to us: Custom Time Team Trowel #400.
Well, we were thrilled. It’s really cool to hold that thing in your hand, feel a connection to the show we loved.
But we decided that after we’d kept it for a time, that it would be best to share it. To pass it on to someone else who shared our love for the show. For it to become a ‘traveling trophy’.
I spent some time thinking about it over this past busy (and eventful) year, and finally decided to make a simple journal for recipients of the “Traveling Trowel” to record some thoughts and dreams inspired by the show. And of course, there needed to be a box to hold the trowel and journal. I wanted everything to feel informal, yet professional. Sorta like Time Team.
This was the result:
In order to set the stage for the journal, I asked my wife to write the following introduction:
This trowel is magical.
Take it in your hand. Feel the weight of it. The balance. How it fits into your palm. How your thumb and fingers curl around it.
Then close your eyes. Let your mind roam. To somewhere you love, even if you’ve never been there in person. Picture the place. Then channel your inner Phil Harding. Or Helen Geake. Or whichever member of the Time Team, past or present, comes to mind. See yourself exploring the past of the site you’ve chosen, trowel in hand.
Then, if you’re willing, share here where you’ve been. Where the Traveling Trowel has taken you.
And, as a record documenting the history of the Trowel, I mounted my friend’s letter next, followed by my own entry. For that entry I naturally pictured St Cybi’s Well …
Ffynnon Gybi
There, in the valley below the hamlet of Llangybi on the Llŷn Peninsula, the old stone structures beckon. But behind them, the Holy Well itself springs forth.
It is here that the trowel leads me. Without disturbing the ground, I can visualize scraping away the upper layers of dirt. Removing the modern soil. Down past the traces of the 18th and 19th century structures that now dominate the site. Deeper, to the remaining imprint of the medieval surface where pilgrims knelt beside the water, seeking healing for body and soul.
Then deeper still, seeking the foundations of myth of the man who would become Saint Cybi. Perhaps his simple hovel where he meditated, seeking a connection with his God. Perhaps some small artifact which has survived the centuries, uncovered, pristine in my mind’s eye, a holy relic …
The Traveling Trowel will move on. I hope that each recipient will add their own bit to the lore of it. But I also hope that each recipient will give the next person some small memento related to their time with it. Here’s what I made for my friend, us, and the person that I have passed it on to:
Thanks for visiting. And if you haven’t yet, check out the Time Team Patreon page.
Jim Downey
Just a note: none of this is for sale. And yes, I took some minor liberties with the official Time Team design to better fit my personal aesthetic and the capabilities of my laser. No criticism of the original design is intended.
Filed under: Art, Book Conservation, Brave New World, Connections, General Musings, Genetic Testing, Health, Humor, Machado-Joseph, New Horizons, Predictions, Preparedness, Science, Society | Tags: art, arthritis, ataxia, balance, blogging, book conservation, bookbinding, Communion of Dreams, fasciculation, genetics, guns, health, jim downey, Legacy Bookbindery, Machado-Joseph Disease, medicine, MJD, MMJ, neurology, neuromuscular disease, pain, peripheral neuropathy, SCA3, Science Fiction, spinocerebellar ataxia type 3, St. Cybi's Well, travel, vertigo, Wikipedia
For about 30 years, I’ve been a conservator of rare books & documents.
That came to an end yesterday when I met with my last institutional client and explained that I need to retire officially as a practicing conservator. Since they follow my blog posts and social media presence, this did not come as a surprise.
As I was driving home from the meeting, I was working through a fairly predictable mix of emotions. Guilt, because I know that this means that important works in their collection won’t get the treatment they need anytime soon. Relief, because now I won’t worry about accidentally damaging some important/valuable book or document. Loss, because my career was now over. Pride, because I know that I have done good work over the years, and made a real difference. And disorientation, because for some 30 years a big part of my identity was being a Book Conservator in private practice — something almost as rare as many of the items I have worked on over the last four decades.
I think anyone who reaches retirement age probably has some mixed emotions about actually retiring. But for most people, they’re ready to retire — to live life on their own terms, to travel, to just get out of the office, to get away from annoying co-workers.
The problem is, I wasn’t ready to retire. And I had already arranged my life so that I lived it largely on my own terms. I’ve traveled. I didn’t have an office I had to go to. I don’t have co-workers who annoy me. I had honestly expected that I would continue to do conservation work until old age claimed me, since the work is typically not strenuous.
MJD had other ideas, as I’ve noted.
So, officially, I am no longer a Book Conservator.
Yes, I am still many other things. An artist. A writer. A cool, handsome guy who is just 64 and certain that he’s still a babe magnet.
OK, maybe not that last one.
But the point remains that there are still many facets of my identity that remain, even though I have of necessity set aside the title “Book Conservator”.
I suppose “Retired Book Conservator” still sounds pretty cool.
Jim Downey
Filed under: 2nd Amendment, Art, Book Conservation, Brave New World, Connections, Feedback, General Musings, Genetic Testing, Guns, Health, Machado-Joseph, Predictions, Preparedness, RKBA, Science Fiction, Travel, Writing stuff, YouTube | Tags: art, arthritis, ataxia, balance, black powder, blogging, book conservation, bookbinding, Communion of Dreams, fasciculation, genetics, guns, health, jim downey, Legacy Bookbindery, Liberal Gun Club, Machado-Joseph Disease, medicine, MJD, MMJ, neurology, neuromuscular disease, pain, peripheral neuropathy, SCA3, Science Fiction, spinocerebellar ataxia type 3, St. Cybi's Well, travel, Utah, vertigo, wabi-sabi, Wikipedia, www youtube
Recently I drove out to Las Vegas, for the first post-Covid national meeting of the Liberal Gun Club (I can be seen at several points in the video, wearing a red & white flannel shirt). It’s a drive I’ve made previously in two days (about 12 hours each day), but I decided that it would be wise for me to split it up into three days each way, to better reduce my stress and better accommodate the unpredictable episodes of MJD symptoms (since I didn’t want to take any of the painkillers or MMJ stuff that help me manage those episodes while I was driving).
It was a good decision. When I had an flair-up of symptoms, I knew that I could take a break without feeling a lot of time-pressure.
And it gave me more time to think.
To think about this past year, and where I’ve found myself. To think about the LGC event (particularly after it was over, and I could reflect upon what happened there). To think about the near-term future.
As I noted yesterday (and in this series of MJD-related blog posts generally), it’s been a challenging year. And there’s nothing like going naked (in the sense of not taking any meds) for a prolonged period of time to show you, honestly and clearly, what your real condition actually is.
Mine isn’t bad. But it is perhaps a lot worse than I had realized, in my day-to-day life. That’s because being able to take things that help manage it means that I can largely ignore the symptoms. Without those meds, though, the truth tends to be a little sharper edged (as is the pain). While teaching a black powder workshop I had hand spasms that were so bad I couldn’t hold onto the gun I was using at the time, let alone manage to load it. So much for the idea that being focused on a given task (which I was) would be enough to set aside that symptom. I verbally walked my students through the process, and we got on the other side of it fine. But it was a sobering moment.
A moment that drove home the idea that it was time for me to make some changes. Specifically, that it is time for me to pretty much completely retire from conservation work. As I noted in this blog post last May:
As a conservator, I can’t afford to celebrate my mistakes. There will of necessity come a time when I need to stop doing conservation work, out of respect for the items entrusted to my care. That time is rapidly approaching; indeed, it may already be here.
I think I crossed that line sometime this summer. So the time has come for me to (mostly) stop doing conservation work altogether, at least in terms of being hands-on.
That’s a big change for me. I’ve largely defined myself as being a book conservator for 30 years.
* * *
Western Utah is stunning. But also bleak. And more than a little alien to my Midwestern eye.
I think those vistas, and the mental space I was in on my drive home, helped me realize something else.
That I’m ready to start writing a sequel to Communion of Dreams.
I hesitate even mentioning this, since I had so many people after me about the long delays in writing St Cybi’s Well. But I decided to share it to help offset the seemingly ‘bad’ news that I need to retire as a conservator.
So here’s the deal: don’t ask how it is going, or when I expect it to be done. I’m at the very beginning of the whole process, and it is likely to take years. I may occasionally mention things about it. Or not. But asking me about it is not going to get any additional information beyond what I volunteer, and will just annoy me. You can wish me well with the writing, but leave it at that, OK?
Thanks.
Jim Downey
Filed under: Book Conservation, Connections, Failure, Feedback, General Musings, Genetic Testing, Health, Science, Society, Survival | Tags: arthritis, ataxia, balance, Big Medicine, blogging, bookbinding, dystonia, fasciculation, health, jim downey, Machado-Joseph Disease, medicine, MJD, neurology, neuromuscular disease, pain, peripheral neuropathy, restless leg syndrome, RLS, SCA3, science, sleep, spinocerebellar ataxia type 3, Tramadol, vertigo, Wikipedia
This is going to be a hard post to write. It might be a hard post to read. In part because I’m probably going to come across as a pompous ass to at least some extent. And in part because it’s not yet resolved, so I don’t know where the story goes from here.
But when I made the decision to start writing about this experience, I told myself that I would be honest about it, the same way I was honest about the care-giving experience, however painful or embarrassing it might be. I know that honesty has helped other care-givers; I hope this honesty helps people who may be facing a diagnosis of ataxia or some similar condition, or who have struggled to get the medical care they need.
Yesterday I had my long-awaited neurological assessment at the local large-institution university hospital which shall remain nameless. I’ve mostly avoided medical care within this institution in the 30 years I’ve lived here. Oh, they have a solid reputation, and do a great deal of good both for the community and for medical science. But I had worked for five years at the large-institution university hospital where I went to grad school, and knew all too well what “Big Medicine” is like. That experience taught me that whenever possible, I should stick with independent doctors/medical groups, where there was less chance that I would be treated as a medical file and more chance that I would be treated as a person with a medical concern.
However, with something as rare as Machado-Joseph, I wanted to tap into the best pool of talent/knowledge I could. And that meant at least starting with the local large-institution university hospital system.
The assessment started out well enough, though I felt poorly from lack of sleep the previous couple of nights. The Intern Doctor came in, introduced himself, went over my file info with me, confirmed that I had been referred by my primary care doctor for an assessment for MJD. He then asked me why I thought I was experiencing the onset of the disease. I started by saying that I was a conservator of rare books and documents, so tended to be hyper-aware of how my hands functioned. This didn’t seem to register as anything different than if I told him I mowed lawns or something for a living.
About five weeks ago I wrote this:
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.
So … well, I was surprised at his lack of reaction. I then told him that I had been a highly accomplished martial artist and athlete in my 20s & 30, with exceptional reflexes, sense of balance, and eye-hand coordination. Again, he took this in stride, as though I’d just told him I played Little League Softball. I explained that I’d always had a heightened awareness of my body, and invariably knew when there was something wrong with it. As an example I told him about my experience with detecting a subtle problem with my heart, finding out that I had a congenital defect, and having the stents put in … when almost no one else would have noticed a problem (and, in fact, nothing has shown up in routine physical exams). Again, he nodded, as though I told him I’d once diagnosed a hangnail. Then he shuffled his papers and said, “Well, let’s do some tests, shall we?’
He ran me through a bunch of tests, checking balance, reflexes, body sense perception, eye tracking, hearing perception, hand movements, and a variety of other things I was unfamiliar with. I was shocked at how poorly I did at a number of these, even being aware that I had been having problems with some of them for months. When we finished, we sat down again, he looked over his notes and then back at me and said, “well, almost all of your tests are within normal parameters, and the ones that aren’t aren’t *that* bad. Are you sure you’re having a problem?”
I must’ve looked like an idiot. Lord knows I felt like one, sitting there, mouth agape. When I finally shook off the shock, I said “well, yeah. I have these pains, frequent urination, these tremors, hand spasms, etc etc etc …” and I ran through the list. Again.
He frowned, looked over my information again. “Well, I see you drink a lot*. That can cause problems. I think we should run some labs, maybe do an MRI. We can also do the genetic test for MJD, if insurance approves that. But I don’t think you have a big problem. Let me go consult with my Attending Physician, see what he says.”
Time passed. I was … bewildered. I honestly had not expected things to go like this. What was so OBVIOUS to me in terms of my changing abilities (and which my wife has likewise noticed), seemed … normal? I felt a little stunned. Well, more than a little, to be honest. I felt completely adrift.
A tap on the door, then the Attending Physician entered, followed by the Intern. It was NOT the Attending Physician I had been expecting. Evidently, something had come up, so this other person was handling cases today. He introduced himself. He was polite, and going off what the intern had told him, he started out the same way, asking why I thought there was a problem. I said that I knew there was a problem with how my hands were functioning because I’d been a conservator for 30 years, and losing control of my tools suddenly was not normal. That got his attention. I also explained that with my family history of MJD, both my sister and uncle had experienced very similar onset symptoms, etc etc.
He said that he’d had experience with MJD patients at a hospital back East where there was a large Portuguese population, and asked if I knew there was a Portuguese connection in my family. (Machado-Joseph is also known as Azorean Disease due to the high frequency in that population … but it is well known to occur in unrelated populations around the world.) I told him not to my knowledge. He then said that I “didn’t have the look” of someone with MJD. Meaning, I suppose, that I didn’t have the narrow face and protuberant dark eyes that many people (including my aunt and cousin) have. But neither my sister nor my uncle have/had those characteristics.
But he said that they’d put in for the genetic test, and that they’d get me a prescription for a beta-blocker to help with the hand tremors. Oh, and he chided me for drinking 2-3 scotches each night. Told me to cut back to just one. With that, he was out the door.
The Intern sat down, started making notes on the computer. He explained that they wanted me to have my B-12 levels checked with a blood test, just to be on the safe side, and instructed me where to go in the hospital complex to get that done. He confirmed which pharmacy I wanted to use for the beta-blocker. And he told me that he was leaving at the end of the month (next week), but that someone else would be in touch if they saw a problem with my labs or needed info for the genetic test. Otherwise, I’d probably be sent info from the hospital about how to have the genetic test done, where, and when.
Then, politely, he showed us out.
We went over and got the blood draw done. My mind seemed to slowly be coming back online as we walked, parts and pieces of the whole session coming back to me and starting to integrate. I was discussing it with my wife, who confirmed my recollections and understanding of what we’d just been through. But I felt completely bewildered and full of self-doubt when we got home. I wrote my sister and a couple of close friends, explained briefly what had just transpired.
* * *
Last night I took extra pain meds, crashed early, and got a decent night’s sleep. This morning I woke to an email response from my sister. We’re close, and she is fiercely loyal & loving. The email was furious that I’d had the experience I’d had, at least in part because she had almost the exact same thing happen to her some fifteen years ago when she first started experiencing the onset of MJD.
After thinking it all through again this morning, and in writing this, I’ve set aside the self-doubt. I know what I’ve experienced. I may or may not have MJD, that will likely only be determined by the genetic test. But I know that my balance has been compromised, that I have been experiencing a wide range of symptoms that point at MJD onset. Perhaps it is a mild case (I think this is most likely) and hopefully will progress slowly. But even in the last six months since I first noticed the symptoms, things have gotten worse.
And this is why I decided to write about this at such length. Because if I, a very privileged, highly educated, white, middle class professional man can be subject to such dismissal of a medical complaint, then I can only imagine how others without such advantages must fight for proper care.
This will not come as news to many people who are less privileged, or who exist at the margins of our society. Actually, it wasn’t news to me, either. But I thought it might prompt others to perhaps give it another thought.
Jim Downey
I’ve also noticed an uptick in the amount of alcohol I’m drinking. Self-medicating, in other words. Again, this does tend to cycle, with some times of the year it being a little higher (2-3 double Scotches in the evening) and other times lower (just 1 double, occasionally 2). Years ago I stopped worrying about it, after discussing it with my doctor, because she observed that it was probably healthier for me than increasing my use of even mild opioids (the Tramadol and codeine), so long as I didn’t develop an alcohol problem.
Filed under: Art, Book Conservation, Brave New World, Connections, Failure, General Musings, Health, Machado-Joseph | Tags: art, arthritis, ataxia, blogging, book art, book conservation, bookbinding, bookbinding techniques, fasciculation, health, Japanese, jim downey, leather, Legacy Bookbindery, Machado-Joseph Disease, MJD, neurology, neuromuscular disease, pain, peripheral neuropathy, SCA3, spinocerebellar ataxia type 3, St. Cybi's Well, wabi-sabi, Wikipedia, Zen
“Jim Downey was a noted book artist, conservator, and author who, at the height of his skills, was betrayed by his body with the onset of Machado-Joseph Disease in his early 60s …”
That could be the opening line of my obituary some (hopefully many!) years hence. Or perhaps a change to my Wikipedia entry.
But the thing is, I don’t feel betrayed by my body. Not in the least.
Yes, I likely have MJD. And yes, it has started to cause me physical difficulties in accomplishing things, as well as additional pain, as I have been outlining here on the blog.
But we all live with limitations. Of strength. Of endurance. Of skill. Of intellect.
I can’t fly like a bird. Should I say that I am therefore ‘betrayed’ by my body? Of course not.
I can’t think as rapidly or as clearly as I could when I was, say, 40. Again, that’s not a betrayal. That’s just change that comes with being a normal human. Of living a normal life.
There’s a Japanese concept of wabi-sabi that informs a traditional aesthetic common in the culture. It has roots in Zen Buddhism, which I studied and tried to embrace as a young man. It can be a difficult concept to explain, but concerns an appreciation for that which is imperfect and/or impermanent in nature and beauty, as all life is imperfect and impermanent. A classic example of wabi-sabi is an elegant teacup which has a flaw (perhaps broken accidentally), but made more beautiful by the application of a gold filling to repair the cup and make it functional again. It is an understanding that all things can only be fully appreciated by respecting their limitations, and that experiencing the thing in the moment, as it is, with all the flaws it has.
As I noted a couple of months ago, I’ve been working to finish the leather-bound edition of St Cybi’s Well. Though this has been complicated by the onset of MJD, I’m almost finished with the edition of 14 books. As I was working to “turn in” (the process of folding the leather around the edges of the bookboard for the cover) the covering leather on one of the first of these books, I made a mistake. My hand slipped. And the tool I was using, my favorite thin bone folder, marred the cover.
Dammit.
I took a deep breath, finished what I was doing, and set the cover aside to think about it later. When these things happen, the >worst< thing you can do is panic and over-react. A lot of times if you just leave it be and revisit it later, you can usually mitigate the damage with a little careful pressure, or extra moisture, or one of several other techniques.
I turned my attention to the next cover.
And as I was finishing that one, almost the exact same thing happened. Not in the same place, but a similar, though worse, tool mark. Like this:
Gawddammit.
We all make mistakes. “It’s inherent in hand process”, as is commonly said by artisans. But making two very similar such errors?
That was likely thanks to MJD symptoms. Which I had been working through.
I quit for the day.
And as I thought about what it meant, I had to consider how I thought about myself, and my art. I am now an artist/artisan who has this additional limitation, this new part of who and what I am. Allowing that to be reflected in my art — indeed, embracing it — was the only honest thing I could do.
It was time for a little wabi-sabi.
So I did this:
A little gold leaf, to embrace the imperfection.
In fact, I added a wabi-sabi element to each of the 14 books. Because I made more mistakes as I finished the edition. Not all of them had tool marring, but many did (and, curiously, all along the top edge of the covers). I decided that for the edition to be complete, for it to reflect this particular moment in time, each needed to have a similar flaw/enhancement, though each one is unique.
Here they all are:
As a conservator, I can’t afford to celebrate my mistakes. There will of necessity come a time when I need to stop doing conservation work, out of respect for the items entrusted to my care. That time is rapidly approaching; indeed, it may already be here. I’ll know more after my neurological assessment tomorrow.
As an artist, I’d be a fool deny my mistakes. Because denying them would be to deny myself, and what has brought me to this point in time. This particular, wonderful, moment.
Jim Downey
Filed under: Book Conservation, Connections, Feedback, General Musings, Health, Humor, Machado-Joseph, Science | Tags: arthritis, ataxia, balance, blogging, bookbinding, dystonia, fasciculation, health, jim downey, Machado-Joseph Disease, medicine, MJD, neurology, neuromuscular disease, pain, peripheral neuropathy, Reddit, restless leg syndrome, RLS, SCA3, science, sleep, spinocerebellar ataxia type 3, Tramadol, vertigo, Wikipedia
OK, this is going to sound like I’m complaining. And I am, to the extent that if this wasn’t bothering me I wouldn’t write about it. But I’m mostly writing about it as documentation: documentation for when I get the neurological assessment in a week, documentation for how things are now as a baseline to compare in the future, and documentation for anyone who wonders what this weird and rare disease is like. The truth is, presently all these elements are mostly just annoying rather than being really painful or debilitating. I’ve been so sick that I can’t get out of bed, and this ain’t it. I’ve been in significant pain where I can’t think of anything other than hurting and trying to make it stop; this ain’t that, either.
A lot of people have experienced Restless Leg Syndrome, a fairly common minor neurological disorder that isn’t very well understood. For me, it comes with a kind of itch or burning sensation, mostly on the surface of my lower legs and feet, that just makes me want to move them to avoid an unseen irritant. Now, this is one of the earliest symptoms I can point to, and it goes back at least six or seven years. Was it an indication of MJD onset, or just due to something else? Who knows. I will say that it has become more noticeable in the last few months, and now happens every day or two.
It’s also, weirdly, spread to my arms. Yeah. Exact same kinds of sensations, mostly confined to my forearms and the area around my elbows. I’ve never heard of this before, but one of the sites I checked about RLS mentions it happening to some people. I guess I qualify.
Something that is kinda like RLS, but is more intense, is a sharp, spike-like pain. It really does feel like I just stepped on a nail protruding from a plank. A nice, rough & rusty one like the one I remember as a kid, going through some dilapidated old house, that went through the sole of my tennis shoe, through my foot, and then out the top. Graphic memory, eh? Yup. And that was the exact same kind of pain I get with these spikes. These are usually a one-off, can happen to either foot, ankle or calf, or in my hands. I’ve joked with my wife that it’s just memories of my crucifixion as a rebellious slave. These are kinda rare, occurring a couple of times a week.
More common are unexpected cramps in the foot, leg, or hands. These are classic “Charlie Horse” type, and just about anyone who has over-exerted themselves at some point has experienced them. One of these happen every couple of days, and can be so intense that it leaves my affected muscle aching the next day or two. I’ve got a couple of sore feet right now due to this (one the bottom of the foot from last night, one the side of the ankle from a couple of days ago). This can also be triggered by using my hands in a repeated motion, like I do when doing book conservation. Or typing.
Related, but not as intense, is a “tightening” of the muscles/ligaments on the back of my hand or top of my feet. Makes it feel like it’s pulling my hands back towards my forearm or my feet towards my shins. Usually happens to both hands or both feet at the same time. Not really painful, just weird.
Then there are the twitches. Like a tic, or a spasm. These tend to come in clusters, lasting for a few minutes at a time, and usually just hit one hand or the other. Again, not particularly painful, but an annoying reminder that my body is not entirely under my conscious control.
I’d mentioned recently the problems with balance. Random vertigo happens rarely, but balance problem are one of the more consistent symptoms I’ve noticed. It happens when it’s dark and I don’t have a visual reference to help stabilize. It also happens if I’m moving and turn my head quickly. Or if I twist to look up and behind me.
Another frequent symptom I experience I didn’t actually know was a symptom of MJD: frequent urination. Yeah, overactive bladder. This one I’ve had for a decade or more, though I attributed it to my blood pressure meds. Maybe that was the case, but it has definitely increased in recent months, to the point where just about whenever I get up from sitting I want to pee. TMI? Sorry.
While each of these are fairly minor, together they usually conspire to do one of the things that most people who have MJD complain about: sleep disruption. Yeah, it’s hard for me these days to actually sleep solidly more than about four hours. Typically I take my usual pain meds (for chronic problems) and crash, then wake about four hours later to have a pee and take the next round of pain meds. In the past I’d usually be able to get fairly soundly back to sleep quickly, and sleep another three or four hours. Now, almost always one or more of the above symptoms will either stop me from getting back to sleep, or wake me frequently for the next couple of hours. At best, I doze in a light and fitful sleep.
So, there we go: a nice summary of where things stand for me.
Of course, that’s the physiological stuff, not the psychological stuff. Because yeah, there are stresses involved with this disease. Knowing what it can do. Knowing what it means. Knowing that there is no cure, and only limited treatments that have been proven effective. Knowing that it is rare to the point of almost being unknown by those outside a few medical specialties and the other families that have the genetic disorder. I was startled the other day when I was on Reddit (a huge online community/news site) looking for something else, and thought to see what kind of support groups exist for people with MJD. There aren’t any. None.
But then, the best estimates are that only about 3-5,000 people in the US have MJD. About one person in a hundred thousand. I’m guessing that I won’t be able to find a local support group, either.
So, thanks for being there, dear reader.
Jim Downey
Filed under: Book Conservation, Connections, General Musings, Health, Machado-Joseph | Tags: arthritis, ataxia, blogging, bookbinding, dystonia, fasciculation, health, jim downey, Machado-Joseph Disease, medicine, MJD, neurology, neuromuscular disease, pain, peripheral neuropathy, SCA3, science, spinocerebellar ataxia type 3, Tramadol, Wikipedia
This morning I’ve been experiencing a typical episode of one of symptoms of MJD (for me): hand weakness/spasm/pain. I’m writing about this to document the disease and to give people a sense of how odd it can be sometimes. At this point, the episodes I experience aren’t constant; they last for a few hours, then disappear for a day or three.
Now, normally I have very strong hands. Building on my basic physiology (large hands, good musculature), 30+ years of bookbinding have made my hands strong, as you would expect. But not this morning.
The first thing I noticed was a slight tingling was through my hands, extending into my forearms. Almost like they were ‘falling asleep’, or like I had held onto a vibrating machine like an orbital sander for too long.
Then there was a feeling of weakness. Like I had been handling bricks for hours, or using a heavy hammer to break blocks or beat metal. My hands were tired, though I hadn’t done any work with them. When I was making coffee, I was sincerely worried that I’d be able to hold onto the mug securely. Popping off the top of my Tramadol Rx pill bottle actually took effort.
Know how when a muscle (group) is particularly tired, it can develop a slight tremor or spasm? Like it has been over-worked and the nerve signals are getting wonky? Yeah, that’s also typical of these episodes. My hands don’t really shake like I have Parkinson’s or something. Rather, they just feel like if I demand anything much from them then they will spasm. The medical term usually applied to this is fasciculation, and is common in neuromuscular diseases like MJD. There’s an … uncertainty … or maybe an unreliability to using my hands. Motions aren’t fluid, graceful, confident. I question whether I am holding the coffee mug securely enough. My typing suffers (the number of corrections I’ve made while typing this is rather startling).
Actual pain isn’t too bad today. It’s more like an ache. But there is a memory of pain there. A hint of things to come. More than just muscle pain, but different than arthritis pain. Almost like the pain from a broken bone, partially healed. In some ways it sounds like peripheral neuropathy, though I’ve never had a diagnosis of that.
None of this is debilitating. I’ve been able to make breakfast. Get showered. Run an errand. Feed the cats. Get lunch. Even get a little bit of work done at my bench. It’s mostly just annoying. And it will likely pass in a few more hours.
But it is tiring and distracting.
I’m looking forward to seeing if there’s something we can do to help manage it, and the other symptoms.
Jim Downey
Filed under: Book Conservation, Connections, General Musings, Health, Humor, Machado-Joseph, Promotion | Tags: arthritis, ataxia, blogging, book conservation, bookbinding, dystonia, health, jim downey, Legacy Bookbindery, Machado-Joseph Disease, medicine, MJD, neurology, pain, SCA3, science, spinocerebellar ataxia type 3
Wait, how can MJD be “hilarious“?
Well …
… so this morning (4/9/22), I got a call from a book conservation client. Since I screen my calls, he left a message, identifying himself, and said he was someone in the University administration. The call was otherwise fairly routine, asking if I could refer them to someone else for a small job they needed done, since I am not accepting new clients. [My website says it’s due to arthritis, which I do have in my hands, but it’s really due to the problems with the onset of MJD. I’ll change the website once I have an official diagnosis.]
I called the fellow back, as I would for anyone who called and left a message. We discussed what the client has, what he thinks he needs, etc. As is commonly the case, he didn’t really need conservation work done (the book is more valuable leaving it alone), but instead just needs a proper archival storage box. Since he’s here in town and associated with the University system, I ask if he is familiar with Special Collections at the University library, since they could easily make a custom box for him (and would likely welcome the chance to do a favor for someone in the administration). He admits that he’s only been in town for a year or two, and was mostly involved with the Medical School. So I tell him who to contact, and what to ask for, and to say I referred him. Easy, simple solution, but the sort of thing that makes people very happy for the help provided and has earned me many references and additional clients over the years.
Then, in chatting a bit, he asked why I was no longer taking clients. I explained that it was due to arthritis, and just left it at that. I told him I’d send him a text with the contact info again, just as a courtesy (as I would do for any client).
After I hung up, I looked again at the name he’d given me. It was a little unusual, and for some reason rang a bell. Then it hit me — he’s the attending physician in the Neurology Clinic I have an appointment with to evaluate my MJD in about six weeks.
I mentioned that I had an appointment with him in my follow-up text with the info I’d promised. We had a bit of a chuckle back & forth over how it’s a very small world sometimes …
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
Communion Of Dreams 