Communion Of Dreams


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



Three weeks in Wales, Part 4: Take a walk on the wall side.

Part 1. Part 2. Part 3.

That’s the famous Laburnum Arch at Bodnant Garden in North Wales. Calling it an ‘arch’ is somewhat misleading, since it’s actually 55 meters long. Here’s a better image of it from Wikipedia:

We decided to kick off our week in North Wales with a trip to Bodnant Garden, particularly since Martha knew that the Arch would be in bloom. She’s wanted to see it in it’s full glory since we first went there almost 20 years ago.

And much of the rest of the garden was in bloom, as well:

If you plan a trip to Wales, particularly anywhere in the north, you really should include Bodnant in your itinerary.

After enjoying the garden, we decided to pop into Conwy for a bit of lunch, enjoying the old town, and seeing the amazing City Walls:

(Not my images, found on Google.)

From there, we decided to drive back to our cottage in Dolgellau via Betws-y-Coed, so we could check out Swallow Falls:

And here’s some video of it:

The next morning we headed to Ynys Môn, more commonly known in English as Anglesey. The first stop was St Seiriol’s Well, Penmon. We visited the well:

Then checked out the medieval dovecot, and went out to the beach for a nice stroll. We drove further inland to check out a site I had again *thought* that I had visited previously, but that turned out to be only in my fiction: the Lligwy Burial Chamber, a neolithic burial site. And nearby is the Lligwy hut group, a Roman-era defensive village which is really quite delightful, even if it is only foundations:

After the Hut Group, we stopped at a roadside pub for some lunch, then went exploring Anglesey, taking in the views, hopping fences to get up close to some wind mills, and enjoying the many scattered Standing Stones.

Jim Downey



Why yes, as it happens I *am* still alive …

The past few months have been … eventful.

* * * * * * *

A couple of weeks ago I got back to work on St Cybi’s Well. Yeah, the break since I finished Chapter 14 was much needed, as I had hinted in my last cluster of blog posts at the end of May.

Why? What happened?

Well …

… in no particular order:

  • Discovery, and subsequent treatment, of a major cardiac health problem.
  • Completion of a full course of cardiac rehab.
  • A substantial change in our financial situation resulting from the sale of property we owned.
  • A bunch of resultant legal and investment research, planning, and changes which every adult should do but few of us ever get around to actually completing. Something about almost dying tends to focus the mind on such matters.
  • A couple of extended out-of-state trips.
  • My starting to train someone from the MU library staff in proper conservation techniques a couple of afternoons a week.
  • A complete new computer system & software upgrade, with all the fun of transferring archives and working files.

And then there’s all the usual business of living and working. Having a couple of months of my life sucked up by dealing with the cardiac problems & treatment meant a lot of changes and trade-offs … but it sure as hell beats being dead from a massive sudden heart attack.

* * * * * * *

So, a couple weeks ago I went through and re-read the entire text of SCW to date, then started working to pick up the story again and bring it to a satisfactory conclusion. Here’s an excerpt from the next section:

Darnell looked out Megan’s bedroom window, across the little lane into the large field beside the Tanat. The field, where so recently cattle peacefully grazed, was now a small village of tents and temporary structures. Most prominent among them was a large marquee someone had found and brought from a nearby town. Make-shift walls had been constructed of large plastic-wrapped round bales of hay from down the road, their tough skin making them weather and even somewhat fire-resistant. The marquee was the main recovery center, where people would be brought from the church after healing, allowed to emerge from the deep sleep at their own pace.

He turned and looked at his sister, who was sitting on the side of her bed. “There’s no reason for you to get up. We can handle it. Go back to sleep.”

There was a faint blue-white shimmer to her skin which never left her now. It wasn’t like she was glowing, exactly, but more like she had a permanent echo of the healing energy which she had used so much in the past couple of weeks. She shook her head. Darnell wasn’t sure whether it was in response to his comment, or just an effort to clear away cobwebs of sleep. “It’s better if it comes from me. I’m known as the Guardian of the Shrine. That carries some official weight with the Church.”

* * * * * * *

I got my garden in late this year. No surprise, given how things went with spring and the early summer. So my tomato plants were not as far along as they could have been when the first waves of heavy storms hit in June. Since then we’ve had fairly regular poundings of storms. And it looks like the tomatoes are almost at the end of their producing for this year — a full month or so early. But between what I harvested, and extra tomatoes picked up at the farmer’s market, I’ve put up about 60 pints of chopped tomatoes. Not quite as much as I would normally like to have, but not bad considering the situation.

And my habanero plants seem to be doing OK this year. Won’t be a bumper crop, but it ain’t nothing.

* * * * * * *

The past few months have been … eventful.

And a lot of things which normally get done, didn’t. Or were handled in a more superficial way than I would usually do.

But that’s OK.

 

Jim Downey



Writing is a process of discovery …

It’s an annual ritual. Salvaging what I can of the deer netting, pulling up the long lengths of rebar which pin the support towers in place, packing up chickenwire. While it wasn’t as good a year as it could have been, it was a better year than I thought it would be, and I hope that the next year to come will be even better yet.

* * *

This is good. Relevant excerpt:

You know what writers feel like when they’re not writing?

Guilty. Incredibly guilty.

Miserable.

They don’t need anyone to come by and kick them while they’re lying there, writhing in the seventh circle of hell, telling them, “Oh, remember those ten books and multiple short stories you wrote? Well, sorry, you stopped writing for a year so none of that counts. You’re no longer a writer.”

* * *

We live in a disposable world. Disposable electronics (when was the last time you tried to fix a tablet, laptop, or television?). Disposable water bottles. Disposable people.

Last week, I did this:

20151104_095415

20151104_110513

That is, I detached the deer netting I had put on my tomato towers, folded it up, secured it, and stowed it away to reuse next year.

This, actually, was a stupid thing to do. That’s about $20 bucks of deer netting. It took me about 90 minutes to salvage it. The amount of my time (in terms of billable hours) which went into doing that is literally 10x the value of the netting I saved. Stupid.

I don’t mention this to tout how environmental, enlightened, or noble I am. None of those things explains why I did what I did.

Well, OK, I try to be environmentally conscious. But I’m not fanatic about it.

No, I did that because in this instance it wasn’t about economics. Gardening, in purely economic terms, is fairly dumb. I don’t do it to save money. I do it to save my sanity.

* * *

By nature and profession, I save old things. It’s just part of my life. And I’m good at it.

Now, that deer netting above isn’t old, or valuable. And how does spending 90 minutes on a weekday morning fiddling around with rusty twist ties and uncooperative lightweight netting save my sanity?

Well, because it gives me time to think.

And thinking is how I spend the vast majority of my time & energy writing.

* * *

It’s an annual ritual. Salvaging what I can of the deer netting, pulling up the long lengths of rebar which pin the support towers in place, packing up chickenwire. While it wasn’t as good a year as it could have been, it was a better year than I thought it would be, and I hope that the next year to come will be even better yet.

Yeah, I’m talking about my garden. But I’m also talking about St Cybi’s Well.

I should have been finished with the book two years ago, according to my Kickstarter plan and promises. Hell, even at that point, I thought I would be done with the manuscript early in 2013.

But writing is a process of discovery. Self discovery. I knew this, but having it driven home during the last couple of years has been … sobering.

Other than periods when I’ve struggled to sort out some particular issue with the book, I haven’t suffered the writer’s block which J.H. Moncrieff discusses in her blog post linked above. But upon occasion my writing has made me feel miserable. And guilty.

Part of that is just a sense of failure because I grossly misunderstood what it was going to take to finish this book. Yeah, I’m talking about the time & energy commitment. But I’m also talking about the psychological challenge of writing a book about the onset of the end of the world we know. Thinking through the details of that takes a toll.

Recently I asked an old friend to read the book so far, and give me feedback. As I told him, I have been so deep in this thing that I had lost my bearings — I could no longer tell whether the thing was any good or not. And that was true.

But the deeper truth was that I could no longer tell whether I was any good or not as a writer.

He says it is. We’ll see if I am.

 

Jim Downey

 



When hope is real.

Last April:

I took advantage of the cool but beautiful Spring day and did this yesterday:

Done 2015

That’s about 40×40. Good deep churn to about 10″ depth. The soil conditions were just about perfect. Now it’s prepped for me to plant things in a couple more weeks (it’s still a little too early here for tomatoes and Habaneros). And surprisingly, I don’t hurt nearly as much today as I expected.

Back to work on St Cybi’s Well. All that time tilling yesterday gave me a chance to think through some things.

* * *

From page 261 of Communion of Dreams:

“Who, Darnell? Who was she?”

The old man blinked, focused on Jon. “My sister, Megan. Somehow, she found th’ courage t’ step up, t’ look past th’ things that they’d always taught her about science ‘n medicine, t’ trust a small blue-white light that she could feel grow under her skin whenever she came close t’ someone with th’ flu. That light would seep out ‘n into ‘em, givin’ ‘em strength ‘n th’ ability t’ fight th’ virus.

“That was hope. That was real. Some of us were able t’ learn t’ find th’ light ourselves, ‘n she showed us how t’ allow it t’ grow, t’ become strong enough t’ reach out ‘n heal th’ others.

Guess what happens in the chapter I am currently writing.

* * *

Hope isn’t always real, or realistic. I’ve seen far too much of life to think otherwise. Likely, you have as well.

But sometimes it is. I picked these today:

20151011_115455

That’s probably about 250 peppers. Most of them Bhut Jolokia. But a fair number of Carolina Reaper and a few Moruga Scorpion peppers. All three are recent or current “world’s hottest pepper” record-holders. And like last year, I’m going to box them up for a few days so that they can become fully ripe. I have not quite as many others I picked earlier and have frozen.

So, what does hope have to do with these?

Well, we had a *very* wet spring and early summer. Enough so that I thought that this year’s Habanero crop would be a complete loss. The plants were stunted, sickly, and very late to blossom.

But sometimes late bloomers will surprise you.

 

Jim Downey



Out there … and down here.

Via Laughing Squid, a nice little animated exploration of the Fermi Paradox:

(Does not contain spoilers for Communion of Dreams. 😉 )

* * *

Been a busy week. Part of it was putting in my garden:

Garden

(That’s just the tomato plants — the super-hot peppers will go in next week.)

Part of it was a MASSIVE job converting a 16 x 16 storage space into the beginnings of a workshop:

Shop

(There’s still lots to do, but man, what a change from being hip-high in grungy boxes and scattered junk!)

And part of it was we have a new addition to the family:

Kitten

(He’s just 6 weeks old, entirely too cute, bold & adventurous, and tiny. For now. No name yet, though given his grey color I suggested perhaps we should go with Dukhat … )

* * *

I’m just now finishing up the first major revision to the working copy of St Cybi’s Well. I already have a couple of people lined up to take a look at it with fresh eyes, but if anyone else is interested also having a preview, leave a comment and I’ll get in touch with you.

Lastly: for Mother’s Day weekend, the Kindle edition of Her Final Year will be available for free. Check it out, download it, share it with others!

Jim Downey



Hope springs eternal.

Because I want this:

20130830_111300(0)

And this:

I took advantage of the cool but beautiful Spring day and did this yesterday:

Done 2015

That’s about 40×40. Good deep churn to about 10″ depth. The soil conditions were just about perfect. Now it’s prepped for me to plant things in a couple more weeks (it’s still a little too early here for tomatoes and Habaneros). And surprisingly, I don’t hurt nearly as much today as I expected.

Back to work on St Cybi’s Well. All that time tilling yesterday gave me a chance to think through some things.

 

Jim Downey



Scary. Just plain scary.

Behold this year’s Habanero experiment:

The Compote of DEATH.

The Compote of DEATH.

What you’re looking at is pureed super-hot Habaneros. With just enough vinegar and salt to preserve them for canning.

OK, the full story …

This morning I made a batch of my Habanero Sriracha sauce. One of my standards. But I decided to do it a little bit differently this year. Rather than use a limited amount of Habaneros in it, and just blend them in, I added a full two gallons of frozen habs to the vinegar & tomato sauce mixture and cooked them until soft. Then I removed all the peppers, setting them aside to cool. There’s still plenty of heat and flavor from the habs in the Sriracha (recipe below).

But then I added some more salt and a little apple cider vinegar to the cooked Habaneros, and ran the whole lot through a food processor. And that’s in the pic above.

The idea behind this isn’t that you’d actually use this stuff directly on food, like you would a hot sauce or some of my Habanero Dust from a spice grinder. Rather, it’s intended to be used in very small amounts as the basis for some larger dish or sauce, giving you just concentrated (almost pure) Habanero flavor and heat. I’ll put it into 4 ounce jars for a very limited number of insane friends.

Just thought I’d share the idea.

Jim Downey

This year’s Sriracha recipe (variation on the theme of recipes you’ll find elsewhere):

  • 5 quarts homemade tomato sauce
  • 2 cups apple cider vinegar
  • 2 cups dark honey
  • 1 cup brown sugar
  • 3 heads of garlic crushed
  • .25 cup of Kosher salt
  • 3 Tablespoons of Fish Sauce
  • Habaneros (mix of super-hot varietals)

Add all ingredients into large pot. Heat to simmer, stirring often. Remove Habaneros. Simmer rest for half an hour or so, then allow to cool.

EDIT NOTE: After trying the Sriracha prior to canning, I decided that it was just plain too hot, so added another couple of quarts of tomato sauce and changed the total above. Everything else still seemed pretty much in balance after. I think the heat level was masking some other problems earlier.



It’s a gas, gas, gas!*

It’s Habanero season again!

I noted a few weeks back that I had harvested the first of this year’s crop, and that I thought that things looked promising, if the weather held. Well, all together I harvested about 200 fully-ripe peppers, seeding freezing them in small batches, and I’ll make some of my Habanero Sriracha with that later. But last Friday we had the first hard freeze of the season, so I picked all the rest of the remaining fruit off the plants. Here’s a the pic of that:

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Now, since turning whole peppers into hot sauce is the sort of thing which can drive any sane person out of the house, I waited until today to do this year’s production. Why? Because my wife is a poll worker, and so is gone all day. Well, here’s that exact same box of peppers, which had just been closed up since Friday:

20141104_122814

Fun, eh? Welcome to ethylene gas. Yup, the peppers are a LOT more ripe, just from being shut up for a few days. Not as ripe as last year’s end-of-season harvest, but not bad at all. And since my version of Sriracha is fairly sweet, I decided to make a less sweet batch of sauce out of the above, since it will tend to accentuate the citric qualities of the not-entirely-ripe peppers. So, here’s this year’s recipe:

  • Approximately 335 peppers, crown removed and cut in half
  • Not quite a gallon of natural apple cider vinegar
  • 8 tablespoons of Kosher salt
  • 3 heads of garlic
  • 2 large yellow onions, rough chopped

Prepare all ingredients. Saute onions and garlic until soft. Add vinegar, salt and peppers to 5 gallon stock pot, simmer until soft, stirring often.

Scoop into blender, do a rough blend for 15 – 20 seconds. Then pour into Foley food mill, and crank until just seeds and skins are left.  Transfer to jar, can.

 

Edited to add later:  Total of 22.5 half pints. Which works out to about 2 habaneroes per fluid ounce, which is what my standard ‘Evil Green’ (previously my hottest sauce) runs, except that this has a much higher % of fully or mostly ripe habs.And this is clearly hotter than anything else I’ve ever made. Pic below – need a good name for it. It’s the one on the right, the reddish one is my Sriracha (about the color of tomato sauce) for comparison.

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Jim Downey

*With apologies.



Don’t fear the Reaper* …

… but *do* have a very healthy respect for it.

The SMOKIN’ ED’S CAROLINA REAPER® pepper, that is. Here’s a bit about it from Wikipedia:

The Carolina Reaper is a hybrid cultivar of chili pepper of the Capsicum chinense species, originally named the “HP22B”, bred by cultivator Ed Currie, who runs PuckerButt Pepper Company in Fort Mill, South Carolina, United States. It’s the world’s hottest hybrid pepper. The original cross was a red naga pepper and a red savina pepper. [1] The “Carolina Reaper” was rated as the world’s hottest chili pepper by Guinness World Records according to 2012 tests,[2] averaging 1,569,300 on the Scoville scale with peak levels of over 2,200,000 Scoville Heat Units (SHU). The previous record-holder was the Trinidad Scorpion Butch T.[3]

There are some included in today’s harvest of peppers:

2014habs

How are they? Oh, baby!

No, seriously, trying one of these peppers is sort of the equivalent of seeing a live, active volcano. Sure, it’s insanely hot (I ate the smallest little piece, about the size of an apple seed, and it did the whole ‘mouth numb, face flushed, lips melting, nose running’ thing). But it’s also insanely cool to just experience the thing … if you exercise a little respect for its power.

And they have the same flavor profile as other super-hot Habaneros, which is actually why I like them. It’s a deep, smokey, lasting peppery flavor.

I’ve only harvested about 60 peppers from my plants so far this season. For some people, that would be about 59 too many. But if the weather holds, perhaps I’ll have a total harvest along what I’ve gotten in years past.

 

Jim Downey

*Of course. And if you would like to order your own fresh super-hot peppers, you can do so from the same place I get my seedlings each year.