Communion Of Dreams


Machado-Joseph Disease: Home, sweet home.

Yesterday I spent the most I’ve ever spent on a glass of water. And I couldn’t be happier about it.

See, my wife had a business function at a conference center at the Lake of the Ozarks. She’s retired, but still somewhat active with the AIA in a volunteer/mentor capacity. We drove down, checked into the hotel, dropped off our bags, and changed to go to a reception we thought also included dinner, awards presentations, and a full evening. I had a glass of water to take my routine evening meds, and we went off in search of the reception.

All went fine, but it quickly became clear that our expectations for the evening were somewhat at odds with what was actually planned. This was the first time this particular event was being held post-Covid, and contrary to the pre-pandemic routine (we’ve attended a number of these events), there was just a reception and brief awards ceremony. It was good that we were there, since my wife is a previous recipient of the same big award, and she was happy to support the new recipients. It was also important for her to be recognized for her past work on behalf of the profession.

But it was all over by 7:00 PM.

Now, since it was just an informal reception, we’d all be standing around chatting (and then listening to the presentations) for a couple of hours. A couple of hours during which some of my MJD symptoms made it abundantly clear just how much had changed for me since the last time I’d attended such a function (pre-Covid). I wasn’t miserable, but I was painfully aware of just how fragile I was feeling (as noted recently). Just being around people was work — even moreso than my usual introvert reaction to such events.

So, as we walked back to the room from the reception, we talked about just checking out and heading home. In just 90 minutes we’d be back in our refuge, I could sleep in my own bed and get back to my usual routine. It meant forfeiting the room cost, though.

I decided it was worth it. One of the lessons I’ve learned in my life is that sometimes you just have to write off the sunk costs of a decision, and get on with things.

So we changed again in the room, gathered up our things, and left. Got home safe & sound, played with the cats, crashed. I slept well (which I never do at a hotel), and this morning was able to get in my usual sunrise walk and exercise routine.

This wasn’t entirely due to MJD, of course. Partially it was due to the weird almost-over-but-not-really pandemic reality we’re still adjusting to. My ‘extrovert batteries’ aren’t what they once were, due to lack of use. And there was a breakdown in communications as to what was happening, so our expectations were at odds with what actually happened. Had we known that the event was going to be over so quickly, we would just have planned all along to return home after it was over.

But I do have to say that the changes I have experienced due to MJD were a major factor. None of my symptoms were noticeable to anyone else, and we didn’t discuss it with anyone. Yet I felt it, and it took a toll.

Lesson learned, and adjustments made.

Jim Downey



Machado-Joseph Disease: oops.

As I’ve mentioned, I’m in a long-term project to repair and repaint our 1883 historic home. Most days I put in several hours of work on it, according to what my symptoms allow.

The pic above was from yesterday, just as I was getting to work on painting the ceiling of our large carport. The six-foot stepladder is sufficient for this chore, though not ideal — I need to hold onto the roller pan or paint bucket while I work above my head. Well, just as I was getting started, I went up the ladder … and promptly dropped the roller pan. It just slipped out of my hand.

Now, like everyone, I’m occasionally clumsy. Always have been. And I’ve made bigger messes than that shown.

But in the past, my clumsiness has always been related to some other factor. I was distracted. Or I was doing something I knew was marginally safe/balanced. I’d drank too much. I had a migraine. I hadn’t slept. Et cetera.

Not this time. I felt fine. I’d had a good morning, getting in my walk and exercises. I’d had breakfast, and was sufficiently caffeinated. I’d set up everything properly to paint, and the ladder was stable. There were no unusual or unpredictable factors at play.

Except MJD.

And that was enough. My hand … just let go.

As I picked up the roller and tray, and cleaned up the mess, I was pissed off. And feeling very, very fragile. It was a rude reminder that I have a disease I can’t control. All I can do is manage the symptoms to the best of my ability.

In the end, it was just an hour or so delay before I got to painting. And a lesson in not taking things for granted I have always taken for granted.

Jim Downey



Machado-Joseph Disease: Management trainee.

This morning I picked up my monthly meds. Basically, the same set I have been taking for almost a decade.

And as I was going through and organizing things, I realized something interesting: I’m taking *way* less of my opioids (Tramadol and Tylenol #3) now, thanks to my Medical Marijuana.

The growing MJD symptoms had started cutting into my small reserve of the opioids which had been more or less stable for years. I mentioned this in one of my first posts about MJD:

But of course, being aware of — even moreso paying attention to — more pain is, well, painful. Distracting. Annoying. So in terms of my perception, my ambient pain levels have gone up significantly in the last few weeks. I noticed recently that my use of my prescription pain meds (Tramadol, Tylenol 3 with codeine) that I’ve been on for about a decade for an intercostal tear has ticked up recently. Now, that happens, particularly when I am doing some strenuous exercise/project. There’s a sort-of natural ebb & flow to it through the year, with some months being a little higher usage, some being a little lower usage. But since we finished installing a new stamped copper ceiling in the kitchen, I haven’t been engaged in anything very physically demanding. That was six weeks ago, and I should have reverted to something closer to baseline. I haven’t.

By the time I got my MMJ card two months ago, I had pretty much used up the small reserve I had. That was a little nervous-making, since I really didn’t want to increase either the power or amount of opioids I took.

Well, in just two months of having access to MMJ, and about a month of understanding how I can best use it for my needs, things have changed. A lot. Like, I’m now taking half the amount of opioids I was (same for alcohol intake). In this short time I have already replenished my reserve. I could probably cut that further, but I’m still just using the MMJ products in the evening (very mild dosages) and overnight (mild dosages).

This disease, and the version I have, is progressive. With luck, however, I should be able to manage the symptoms, and particularly the annoying pain issues, without increasing my intake of opioids for a while. We’ll see — it’s all about learning how to manage things.

Jim Downey



Machado-Joseph Disease: Not Dead Yet*.

So, I got the genetic test results today: I have a mild version of MJD.

That’s not an official diagnosis. I probably won’t have that until sometime next year, after I have different insurance (Medicare) and can find a local Neurologist to work with. Because I won’t go back in to the Neurology Clinic at the local large-institution university hospital which shall remain nameless, for reasons outlined previously. And because they didn’t bother to send me the results — which they have had for over a month — until I called them up. And they’re supposed to post all such results to the ‘patient portal’ within two days of getting them. Grrr.

But the results are clear. And since there is little or nothing that modern medicine can do for me that I’m not already doing, I’m happy to just wait.

Knowing the results makes a difference. And while it’s not good news, it could certainly be worse. I know what is going on, and what to expect. Thanks to my sister’s experience, and the experience of my other family members, I know most of the best strategies to manage the disease. Because of my age of onset (about 4 years ago, I think, so about 60 years old), and the type I have, I should experience a normal lifespan and slowly progressing symptoms. I can plan and work with this information.

I intend to continue to write about this, but those posts will probably be just occasional updates when I feel like I have something interesting to say.

Thank you for your good thoughts and support — it’s helped me these past months while I have navigated this experience.

Jim Downey

* https://www.youtube.com/watch?v=Jdf5EXo6I68



Machado-Joseph Disease: all things being equine …

My feet hurt. My hands hurt.

Actually, let me be a little more specific: the backs of my hands and wrists hurt. The tops of my feet and the fronts of my ankles hurt. If I were on all fours, you could see the symmetry of the places that hurt. It feels like all four joints were hyper-extended, and now hurt from it.

I put it into these terms because there’s value in understanding something about MJD: it affects the cerebellum, that part of the brain that controls movement and coordination, as well as having a role in pain awareness and some emotional/intellectual controls. This is an ‘old’ part of the brain that we share (in evolutionary terms) with most vertebrates.

Like horses.

Why do I mention horses, specifically?

Because of this:

Episode 51: Shivers Horses and Its Association to the Cerebellum

In this episode, Dr. Audrey DeClue focuses specifically on the association of the cerebellum to shivers horses. She shines a light on the existing, published research on shivers and the trouble with conclusions based on a small sample size. She also includes an interview with longtime friend, Celeste, to provide a powerful story of what it is like to live with a degenerative cerebellar lesion.

The ‘Celeste’ there is my sister. Who first experienced the onset of MJD symptoms about 20 years ago, and has been living with the disease since.

The whole podcast is well worth listening to, but the discussion with my sister starts at about the 28 minute mark, and lasts for about 20 minutes. In the interview Celeste is forthright about the challenges and pain associated with the disease, and how her symptoms have evolved over the last two decades. In listening to it, I was struck not only by her astute awareness of her body and how it has changed over the years (she is very smart and observant, no surprise) but also in just how much her early experiences echo what I have been experiencing the last couple of years (though my symptoms are mild compared to hers). That includes not only symptoms, but the frustrations of trying to get doctors to actually listen to her describe her subtle symptoms early on.

It’s an excellent interview, and Dr DeClue is a sensitive and talented interviewer. I encourage you to give it a listen if you are interested in this rare disease, and what it is like to live with it.

Jim Downey



Machado-Joseph Disease: ∞

Eight weeks.

8

Which, on its side, helpfully looks like the infinity symbol: ∞.

Because while it’s been eight weeks since my blood sample was drawn for the genetic testing for MJD, it feels like I’ve been waiting an eternity for the results.

Of course, it took a full month for the Neurology Clinic at the local large-institution university hospital which shall remain nameless to order the test.

And I waited two months before that to get in to see those neurologists, because I thought I needed a referral.

And I waited three months before that in order to get in to see my GP in order to explain why I wanted the referral.

Yeah, count back, and that means I have been waiting all this year in order to get a diagnosis for the disease I’m reasonably certain I have. Little wonder that NORD (the National Organization of Rare Diseases) says that typically, a correct diagnosis for someone with a rare disease (such as MJD) will take upwards of five years. I’m already most of a year in, and I even KNOW the disease actually runs in my family. Imagine what it would be like if it was just a random mystery disease, and we had to start from scratch to determine what was going on.

>sigh<

Yes, it’s frustrating. Friends and family keep asking (just being supportive, not annoying), and I keep telling them the same thing: no results yet.

Meanwhile, I continue to just deal with the symptoms as best I can. And things do continue to evolve. Balance issues are now fairly routine. Hand & feet pain and Restless Leg/Arm Syndrome less so, but seem to be happening more often. And I’ve started to experience occasional vision difficulties (focus/double vision problems) that I can usually ‘reset’ by changing my point of focus to something far away, then shift back to a closer item. It’s not an actual double image, but rather the sort of thing you experience when trying to look through the wrong part of progressive lenses, then shift your vision so things slide back into focus.

The good news is that the MMJ does help most of these symptoms quickly, and I have cut my mild opioid intake by about 50% since I figured out what worked for me.

Meanwhile, I wait. I check to see whether the results have been posted to my account on the diagnostics site or my patient portal for the local large-institution university hospital which shall remain nameless. And I get on with life.

While waiting.

Jim Downey



Machado-Joseph Disease: not immune.

I just spent about 20 minutes sitting on a toilet. And so far this morning, a total of about an hour doing that.

TMI? Yeah, sorry. But I mention it because it has demonstrated a truth of which I was only partially cognizant of previously: there is no immunity from other illness when you have a chronic disease.

I mean, I knew this, but until you live with it, it’s not something you think about a lot.

See, a couple of weeks ago I started to notice a pattern of gut-grumbles I don’t normally experience. And about ten days ago it resolved into something I thought I recognized: giardiasis. Twenty+ years ago I had a bout of this, likely picked up from tainted water on a camping trip. Where I got it this time, I have no idea.

But after recognizing the symptoms, I contacted my GP clinic and got in to see a doc. Who did the necessary exam, discussed options with me, ordered the appropriate tests, and prescribed a powerful antibiotic which is the standard treatment for giardiasis, and which cured me the last time. I’m now in day 7 of that treatment, and while there are *some* indications it is working, well, I still wind up sitting on the toilet with nasty spasms every 6-8 hours or so, unless I really load up on OTC anti-diarrheal treatments. Even so, I don’t dare get very far from a bathroom for very long.

Of course, through all of this, I am still experiencing the random rotation of MJD symptoms. Joy. Now I have TWO reasons to compulsively check my health/testing accounts: to see if the MJD test results are in, and to get confirmation of the giardiasis. Because yeah, even though tomorrow will be seven weeks since the genetic test samples were collected, I’m still waiting on those results.

No one who has lived with a chronic disease will find any of this surprising. They know that it just goes with the territory. Hope you never have an opportunity to experience it for yourself.

Me, I’m going to take some pro-biotics and get a nap. Maybe the test results will be in after.

Jim Downey

Edited to add several hours later:

Spoke with my GP’s office. Turns out I did have giardia, but happily there was no sign of c. diff, which was a possible concern. So after a week of taking Flagyl, I can now stop that (that alone was probably part of the ongoing spasms & diarrhea) and work to get my system working normally again. That means increasing my probiotics, getting back to normal eating habits (with my relatively high fiber diet), and taking it easy just to let my body recover.

So, no news yet on the MJD test, but at least this other problem should resolve in the coming days.

JD



Get a grip. Or make one.

[For the AI’s own inscrutable reasons, Facebook considers my ballistics blog “spam”. Unable to 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 shooting stuff isn’t of interest.]

A couple of weeks ago I posted about finishing a Liegi Derringer kit, then doing the laser work to customize the grips. It turned out well enough, so I decided to finish and laser a second kit, to use as a door prize for a black powder workshop I’m doing at the annual meeting of the Liberal Gun Club this fall in Las Vegas.

All went well until the time came to mount the grip to the receiver. The top mounting screw went in fine, after drilling a pilot hole. But the bottom one broke loose as I was tightening it. I took the top screw back out so I could see what the problem was. This is what I saw:

Almost looks like a tiny wooden shoe from this angle, doesn’t it?

[The entire post about this project can be found here.]

Jim Downey



Machado-Joseph Disease: I’m W A I T I N G !

Tomorrow will be five weeks since the blood draw for my MJD genetic test.

I just checked (for the fifth time so far today), and neither my patient portal for the Neurology Clinic at the local large-institution university hospital which shall remain nameless now the diagnostics lab that handled the test has results back yet.

>sigh<

I don’t really have much to say that I didn’t say two weeks ago in this post, other than the fact that it’s been two more weeks of waiting. Everything there still applies.

But I wanted to whine a bit.

Not that it will do any good, other than allowing me to vent my spleen.

Which sometimes is enough.

Barely.

Jim Downey



Lookin’ Sharp(s).

[For the AI’s own inscrutable reasons, Facebook considers my ballistics blog “spam”. Unable to 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 shooting stuff isn’t of interest.]

As anyone who has read much of this blog probably knows, I (and the other BBTI guys) like weird guns. Anything that is innovative, or unusual, or uses a transitional technology, is likely to catch my eye.

One of those I got to try this past weekend is a reproduction Sharps Pepperbox. It was designed by Christian Sharps (of Sharps Rifle fame) in the middle 1800s , and proved to be a popular little hide-away gun in early .22. .30, and .32 rimfire cartridges.

In the 1960s Uberti produced a little .22short reproduction with a brass frame and plastic grips. Here’s one recently listed on Gunbroker which has an excellent description of both the reproduction and the original: Uberti Sharps Pepperbox 4 Barrel Derringer.

And here are some pics of the one we shot this weekend:

[The entire post about this little gun can be found here.]

Jim Downey