Tag: HD

Apathy, Anxiety, Memory

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In trying to master the decline, I’m also documenting it. These are the more difficult entries, both to write and to post.

You’d think apathy and anxiety would cancel each other out. But, no.

I’m traveling again. And I don’t know how long I’ll be able to do it. The planning is harder, the sleep disruptions are more disturbing. I still love it, even if I can’t fully feel all the feels.

In the last 24 months, there is a noticeable shift in my perceptions.

Apathy

I can’t get excited about things the way I used to. But I haven’t felt the usual rush being someplace different. And it is very different. Even 2 years ago I would’ve been preoccupied with all the little things, figuring out the new systems. People watching, museum-going, taking photos of buildings and plants.

I still do those things. I can’t put my finger on why I still do it, if it feels so… artificial.

I’m flat. Flat + anxious, honestly.

Anxiety

I had anxiety before the flight, before taking the bus for the first time, and going to a restaurant for the first time. Part of the pre-travel anxiety was doom-related. Not a voice in my head asking if this is the last time I’ll travel or what happens if the plane goes down. Instead, a fog. A grey mood around something unseen, blurry, in the peripheral vision in my head.

I used to have the kind of anxiety that manifests as depression. That’s what I thought it was. In the freeze reaction I used to have about imposter syndrome on the job. Terror at being found out (even though I was good at my job) turned into the inability to get out of bed in the morning, leading to bad job performance. That’s hard to admit. It got worse closer to the HD diagnosis in 2017. I even quit working in 2018.

Memory

A bigger part of pre-travel anxiety came from a vague sensation of forgetting something. Again, no voices in my head asking about passports or phone chargers. Just that sense that plans could be incomplete in some way that I don’t know that I don’t know about.

Someone shared childhood memories that 10 years ago we had. Today I don’t. Absolutely no memory of it. It doesn’t disturb me in the way you might think.

Part of the reason traveling works for me is that it avoids the groundhog day effect. When every day feels repeated, time passes so fast that I feel like I’m running out of life. This runs counter to the advice: give people with memory problems a very consistent routine. Maybe it works with Alzheimer’s and dementia, but for me the quality of the memory loss is distinctly different, needing a different solution.

I’m apathetic about my memory loss, but it still makes me anxious. What?

Conclusion

There’s an interaction between memory and anxiety that I haven’t read about in the HD literature. Anxiety that isn’t nervousness or specific voices. Just a vague sense of forgetting something important: an appointment, the keys just before you shut the locked door, or food on the stove. All of which I do more of, so is it another case of “just because you’re paranoid…”? I think it’s a legitimate reason for distress.

At the same time, the interaction between apathy and anxiety means that I’m happy enough, I guess, most of the time. I’m not particularly *bothered* by my anxiety. Also something I don’t read about in literature about either HD or anxiety.

I’ll just keep reading what I can find. And writing about things I don’t.

I Don’t Get Post-Workout Muscle Soreness

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Here’s my protein ‘recipe’ for a less painful workout and overall strength:

During the day, 1g protein/ lb bodyweight.

3-5g creatine 30 minutes before working out.

1/2 to 1 ‘serving’ of a BCAA supplement (leucine, isoleucine, and valine) up to 30 after working out.
Other amino acids can actually interfere w/ BCAA effectiveness, so I avoid the ‘extra mega everything’ kinds of supplements.

At bedtime, 1 serving of a casein protein shake. Adding it can improve muscle building, and casein is slow-digesting so in theory better for overnight recovery than a faster digesting one.

Oh, and I’ve gained about 2kg while wearing looser clothing. I have measurable strength gains in the last couple of months.

Apathy And A Little Brainwashing

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Huntington Disease causes apathy, but apathy is more than one thing, it lives in three domains. In some people with Parkinson Disease, emotional apathy remains, even when behavioral and social apathy is absent.

Effort for reward (motivation) is the behavioral domain. For me, this is pretty much wiped out.

Effort for other people is the social domain. Pretty much intact for me: I’m willing to do things for other people and groups.

Emotional responses and sensitivity is the third. My emotional responses feel somewhat blunted, but I am sensitive to others’ feelings. (With a caveat: I’ll need to accurately read their faces and body language and voices.) I’ve lost memory around what something felt like, emotionally. So I can remember an event, but not, sometimes, what I felt about it.

In the Parkinsons study, there was a correlation between having behavioral and social apathy and having anhedonia, the inability to enjoy doing pleasurable activities.

I don’t exactly have anhedonia. I’ve lost the memory of how enjoyable an activity is. So I won’t start. But once I start, I enjoy it.

So… I have a really hard time getting things done. Even things that, in theory, I like to do.

I’m trying to do a little brainwashing on myself, and ran into this video from Chase Hughes. It’s been super helpful 🙂

2019 Letter To My Friends About HD

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HD is inherited. A faulty protein kills neurons, with symptoms similar to Parkinson’s, ALS, Alzheimer’s, and/or Tourette’s. It’s progressive; it won’t go away. It can develop earlier and faster in each generation.

It causes spontaneous muscle movements, contractions, and weakness. I stutter more and am slower to speak. It’s harder to swallow correctly, button, zip. My coordination and proprioception is impaired like a drunk’s.  It’s hard to tell where my body is in space.

You may not notice anything. Or you might.

I’m likely not drunk, bored, impatient, or needing to go to the bathroom (usually). My face may make expressions unrelated to what I’m feeling. And I’m capable of nuanced thought even when I’m not articulate.

I have good days/moments and more symptomatic ones.

I fall sometimes. I still exercise. I meditate. During both I have fewer symptoms. I don’t mind talking about it. I’m a realist, so this may sound a little dry, but it’s the facts, presented with love. ♥️

Where Is My Mind?

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OK, things are getting worse. Yesterday my therapist asked for the usual credentials at the beginning of a session: name, DOB, address. I gave name and address and hesitated. Took me a minute to remember what else she’d asked for. We have done this weekly for over a month, and still I couldn’t remember.

Later the same day, I host a zoom meeting. It’s protocol to thank each person who makes a comment in their allotted time. On the very first one, I forgot. The procedurals that are automated are going downhill.

I’ve had bad days, but this was a new low. With a degenerative disorder, new lows are the new normal.

This isn’t bad or good, it’s just what is. I don’t want to post this. But the purpose of this blog is to document the decline in detail. In realtime. And I don’t feel terrible. Just a sense of urgency to get done the things I won’t be able to do.

I play musical instruments, mostly guitar and piano. And I have a back catalog of about 300 songs. I’ve recorded a couple dozen. The others are mostly lost to weird made-up notation (not tab or sheet music) and lyric fragments. Some jpegs of literal fragments of paper. I’m not sure what’s worth saving. And it’s daunting to look at it and try to sort it. Much less re-learn and re-record it.

I’ll get done what I get done. Or as my mom and her father used to answer, to lots of things: “either it will or it won’t.” And I can choose to be ok with that, too. 🙂

Memory, Intelligence, Coherence

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What It’s Like In My Head in 2021

It’s been 3 years since the Huntington Disease diagnosis.

My drafts are composed by dictation. Sometimes I can’t open the app fast enough to remember what I’m going to write about. Today I finally remembered two things.

Memory is not intelligence.

Sure, they’re integrated. So much that you won’t notice until only one of them is affected.

There are thoughts you keep in your head for multitasking; subconscious, and never written. They’re the basis of the small day-to-day decisions about automated tasks. I’ll remember that I have decided to execute a habitual tasks in a certain way or in a certain sequence, but can’t remember the rationale.

Like brushing my teeth before putting on makeup so I don’t drool it off my chin.

Where do I sit in the room to meditate and why? There’s a particular chair that doesn’t sink as much as the couch. It’s in the sun in the morning. I do it without shoes because I don’t want to have them on the seat. If it gets worse I’ll workaround with a post-it in the chair: “Meditate Here.”

I record music and there’s a reason why I route my guitar a certain way through the DAW. I disconnect the power source to reduce hum. Do I remember that? Nope. Every time I plug it up I go through that same routine. Which I can do, once I am in situ.

That kind of knowledge is stored differently than the memory of going someplace yesterday, news heard last night, or things learned about mRNA vaccines. Those are intact for me, for now. It’s the automated memories that don’t seem to be there.

The ability to produce coherent thought is not the same thing as having coherent thought.

Sometimes I can’t find a word, but the concept is there, intact, in my head. Sometimes I’m slower to speak, slur, or stutter. I haven’t lost the subtleties of thinking. I’ve lost the subtleties of fluid speech. We’re (ok, I am) used to using speech to estimate intelligence. It’s a misconception. See Parkinson’s research.

As an HD patient, what do I need from friends and family and caregivers?

Don’t treat me like I can’t make decisions for myself. If I can’t find a word, it doesn’t mean I can’t reason. Don’t assume. Ask. And give me space and time to answer.

So far I’ve had great support from friends and family, but I’ve seen others who haven’t been so lucky.

Gaps in Care of HD

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There’s More To Huntington Disease Than Neurological Symptoms

2019

Last visit to the neurologist, mom saw 8 different people in 25 minutes.
The nurse collected the vitals.
The neuro’s assistant went through an annual questionnaire, extending vitals.
The nutritionist gave nutrition advice without asking what mom eats.
The social worker gave us a card and said she could be contacted for things like getting medical insurance, making doctor appointments.
The physio had her walk and do a couple of cognitive tests. No one asks: Do you exercise? In what way and how much?
The psychologist asked a standard battery of yes/no questions. Do you have suicidal thoughts? Depression?
The speech therapist asked another set of yes/no questions.
The neurologist gave the standard UHDRS tests.

In an office on the other side of the country, my exams are exactly the same. I am asked the same questions, tested in the same way (though I only see the neurologist).

The UHDRS is an annual test that measures HD symptoms that fluctuate hourly.
Patients aren’t asked: are you having a good day or a bad day in relation to your symptoms? Are they worse in particular times of the day?
The tests measure symptoms at a super low baseline. In 2017, I didn’t fall down when pushed and could walk a straight line. Same in 2018. But there were changes in my balance and coordination, undocumented by this standardized battery of tests.

It’s assumed that the checklists are both relevant and comprehensive.
There are lots of yes/no questions that ignore subtlety. Without defining ‘difficulty’ the speech therapist asks, “do you have difficulty swallowing?” Mom says no, because she can swallow. Though she aspirates food or water several times a day.
We aren’t asked to give perceptions of the disease progression or new or bothersome symptoms. The physio didn’t notice mom’s knee problems (due to pronation due to flat feet, all new).

We are given no comparative data or results.
I don’t know what the data is used for. We are given updated prescriptions, if needed. But the tests aren’t explained and we get no feedback on progress.

The lack of information – on the part of medical providers – about non-neurological HD symptoms and how best to care for them leads to a gap in care.

Symptoms that are related to the disease can and are overlooked.
The neurologist is considered to be the specialist managing the HD in toto. The GP is not familiar with the illness, much less the symptoms.
The specialist is only concerned with what’s going on with the neurodegenerative part of the disease.
In my family, symptoms like a super low heart rate, weird insulin response, sudden change cholesterol were all written off as unknown etiology and unrelated to Huntington’s Disease. Musculoskeletal problems blamed on chorea though they appeared a decade before it.

Huntington’s Disease is a Multi-system Disorder

OK, here are a bunch of references, linked.

Even the Merck Manual HD entry describes it as a neurologic disorder, without any mention of other potential system involvement.

The Exact Function of the Huntingtin Protein Is Unknown
Since we don’t know what the role of the normal Huntingtin protein is, we can’t know how the mutant protein affects other organ systems. HD affects bone density, muscle loss, cholesterol in ways we don’t understand.

Diabetes: HD affects insulin and metabolism, and increases the risk of diabetes.

Aberrant cardiac conduction: In one study, 28% of early stage HD patients had bradycardia. This is especially important, as 20-30% of HD deaths are due to cardiac problems.

Muscles and bone density are affected. Cholesterol, too. I suspect that the chronic enthesopathies are very much related, but there isn’t any research into connective tissues and HD. Yet.

What To Do?

So what’s the conclusion? Find other specialists and convince them of the connection? That’s my first instinct, and it’s the path I’m on. Though since cognitive deficit is another symptom of HD, that’s not an option for a lot of patients.

About Me and HD

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Before Huntington’s Disease I played around with sports. I haven’t been able to run in almost a decade. As of 2019, I’m having trouble with escalators, eating, talking, thinking, and sometimes walking. I have some chorea.
When I found out I had HD, I really wanted a first-person account. I found stories on testing, family dynamics, and pre-symptomatic life. Most writing about symptomatic HD patients came from caregivers and family.
While I’m not a writer, I’m making a record of my HD progression, symptoms, experiments, and hacks.  I do this on the borrowed time when I’m coherent.  Dedicated to my family’s next generation, status unknown, with love.