Month: February 2020

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.

Vertigo

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Related to Huntington Disease? I don’t know. Maybe it’s the autoimmune genes fault, this time.

2019

Episodes of vertigo started 15 years ago – severe, but not frequent. Sometimes it would put me in bed for a week. Changing position incrementally (I couldn’t just sit up from bed), I’d have to gradually tilt my head about 15 degrees up and wait a minute.. repeat until sitting. Now it’s persistent but subtle, about half the time, with a duration of a week or two.

Suggestions for people with Ménière’s disease and other vestibular disorders: regulate intake of food and water so that there are fewer *fluctuations* in sodium input and blood sugar levels. Avoid alcohol, caffeine, and MSG, and stay hydrated.

In 2003 I had a doc suggest that I drink alcohol for its diuretic properties. It was somewhat successful, but it’s hard to tell since I was also reducing sodium. I’m more likely to have a mild episode that lasts a few hours after eating a lot of salt, which typically I don’t.
BPP exercises (the Epley manoever is one) doesn’t generally give much relief – it worked once in 2012.

What Works For Me

Every morning I do a set of exercises my mom’s physical therapist gave her and it has reduced the severity. The debilitating episodes happen only once every year or two.
Move the head in three planes, gently, to the extent of range of motion, and without discomfort:
* chin up and down supporting the neck in the back
* looking left and right without moving the chin higher or lower
* tilting the head left and right while keeping the face forward and the shoulders down

I’m also targeting balance more generally. Walking heel to toe on a treadmill (on it’s very slowest setting), walking on the treadmill while doing the head-ROM exercises, standing on a balance trainer and doing them (with a wall for support). The mantra: find what is hard and do more of it.
In 2016 I had my first surf lesson in the middle of a week-long mild episode. It was definitely harder, but doable. 😉

Diet (2019)

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Most of the advice for people in the early stages of HD is the usual, generic advice for good nutrition. Later, losing weight is a thing. So is swallowing. “There is also some evidence that maintaining a body weight slightly above ‘desirable’ weight will facilitate control of the disease.”
There’s no verified causal link between particular foods/groups and HD symptoms (for better or worse). But there are recommendations, sometimes for specific supplements, like calcium.
I’ve experimented enough with dieting content and timing (fasting, keto, low carb, FODMAP, diabetic restrictions) to know what my body is comfortable with.

My goals:

  • stable diet, so that I can monitor changes in supplements as I experiment
  • log diet info, so that I can supplement nutrients as needed
  • shop with a super low budget
  • eat mostly whole, single ingredient foods
  • eat mostly foods that don’t bother me for any reason

Macros and Supplements

I was craving carbs, hungry all the time. I’ve tried everything I could find online to mitigate this, promise. I feel ok with keto, and even better fasting, for a day or so. But it’s not something I could maintain long-term. For me, the trick was counting carbs so that the total is about 150, but only about 50 at any one time. I’m notoriously bad at sticking to a recipe (ok, following instructions in general), but I’ve started measuring food. And I’m good with averaging: I eat 2300 kc one day and 1500 the next. I don’t pay much attention to the other macros.
I was getting most of the official RDA for everything but vitamins D and E, so I added them to the daily mix. I also like to tweak the recommendations to include the AREDS2 list. Following that, I was also low on a couple of minerals. I have a few bottles of multivitamins, so I’ve been taking 1/3 of one every day, to add some extra minerals and Bs.
My serum Mg was low. I poop normally when I am taking the Mg supplement. So I take it. And it’s recommended to take Calcium if you’re supplementing Mg, so I take that, too.
The glucosamine is a definite plus, I’m still experimenting with the collagen.

Foods

I’m losing weight (very slowly) but at this stage in my HD progress, that’s fine. I need to lose a little for my knees and hips. Here’s what I ate one day:

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What I Ate One Day
  1. coffee + cream, 3 eggs and some spinach sauteed in olive oil
  2. a half cup of lentils and a half cup of collards (some olive oil in both)
  3. a sweet potato and a boiled egg
  4. a cup of lentils and a boiled egg
  5. .8 cup of rice and a boiled egg and half a cup of collards

Plus some water, tea, and diet drinks. I didn’t feel hungry, it was about 2,000 kc. I cooked the lentils and rice from dry; sweet potato, collards, and spinach from fresh. Most days I don’t eat so many eggs and have some tuna with yellow mustard and sugar free sweet pickle relish. I usually have some frozen berries (cherries or blueberries). And popcorn in olive oil. I use salt and fresh pepper in everything, ginger in green tea.
I prefer fresh, will make do with frozen. I’ll sub any greens for collards, chick peas for lentils. On the road I’ll get canned veggies, fresh fruit, and precooked eggs.
I try to eat to reduce inflammation. I add hot spices (ginger, peppers), especially to inflammatory foods like meat. I eat yellow mustard for turmeric, pickles and an occasional beer for gut health. (I hate yogurt, sorry, mom.)

What I Do Eat (90%): It’s easier to concentrate on what to eat, since it’s a shorter list and it makes shopping and decisions quick.

  • Rice and corn without anything added and stuff made from it (tortillas, rice cakes, popcorn)
  • Green and yellow vegetables (greens, salad greens, cucumbers, asparagus, cactus, artichokes, squash, yam/sweet potato, carrot, pumpkin), avocado, olives, pickles
  • Berries (all of them) and cherries
  • Eggs, fish, and occasionally chicken
  • Coffee, tea, water, fizzy no sugar drinks (prefer stevia, then splenda)
  • Rarely: sunflower seeds, walnuts

What I Don’t Eat Much Of (10%): the anti-list (why it’s off limits for me):

  • Restaurant food (too many surprises)
  • Sauces and anything in a packet (ditto)
  • Sugars – honey, agave, corn syrup, all of it (inflammation and blood sugar regulation)
  • Fruit that is not berries, large quantities of tomatoes, and any fruit juice (ditto)
  • Garlic/onion (GI issues)
  • Beans other than chick peas or lentils (ditto)
  • Regular dairy products (ditto)
  • Grains other than corn and rice (inflammation)
  • Red meat (expensive, sustainability issues, often has surprise additives like sugar in jerky)
  • Nuts (expensive)
  • Pretty much anything not on the other list

Budgeting $55-$65/Week + The 10%

  • $15/week for the veggies, beans, and rice
  • $7 for eggs/tuna
  • $7-12 for frozen fruit
  • $15 for carbonated flavored water or sugar free sodas*.
  • In hot weather I try to drink an electrolyte powder every day, another $3.
  • Every month or so it’s another $10-15 for yellow mustard, olive oil, spices, green tea, coffee, etc.

Tools

I log all the supplements, food, drink and exercise. I don’t check it much, now that I got the carb and nutrient targets where I wanted.

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I’ve been using Cronometer. It’s free, and the app is super quick to use.

I set it up by adding the food I ate everyday (it does a lookup on a partial text). I added all the supplements. Now I just copy the previous day and edit individual values.
It auto-calculates the nutrients for everything. You can choose which are highlighted, and you can set the range of acceptable values.

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Week Averages

Making It Interesting vs Making It Work

I love food and eating. I’m a creature of habit but also sometimes bored. So I’ve tried things like making jello from plain gelatin and electrolyte powder and adding frozen blueberries at the end. I take peach fizzy water and mix it with stevia and matcha and sprinkle ginger on top. It helps that I love the taste of olive oil + salt + pepper + any vegetable. That I love eggs. And olives. That I tolerate fish pretty well with olive oil and heat or cool and pickles, sweet or not. That I love salad and avocado and olive oil. I like to saute jalapenos and then add the eggs or veggies. That I use curry powder and cumin in the lentils. And/or the greens. I use chai spices on the squash and yams. And did I mention olive oil?
So this works for me because I already like the food on the list. I imagine it would be harder to transition if I didn’t like veggies so much.

And when I stick close to it, I feel noticeably less fatigued during the day. So this is what works for me, for now.

Glucosamine/Arthritis

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Glucosamine and Arthritis 2019

I stopped taking glucosamine at the start of this experiment. It took a week or so, but gradually joint pain increased. Things that hadn’t hurt started to hurt again, others that always hurt were worse.

I don’t have typical Rheumatoid Arthritis joint pain, with redness and swelling. My joint issues are soft tissue disorders, the worst being enthesopathies. I was playing soccer, landed on one foot and twisted, and heard a pop. The obturator internus separated from the hip bone. So did a third of my hamstring.

I’ve been diagnosed with everything highlighted above. Below is a pelvis. It’s not my pelvis. But I did have imaging of all kinds, revealing, the avulsion injuries, calcification on both ‘sit bones’ (where this image is pointing), and tendinosis on the upper outer hip.

When I’m at the gym, if I increase weight or reps to reach muscle fatigue, the limiter is a joint: knee, shoulder, elbow, wrist, or hip. If I push through the pain, I’ll end up with a tendinopathy that will keep me sidelined for weeks until it’s healed.

Not My Pelvis

Legendarily bad hips run in the family, in the very same people that had HD. My grandmother, who didn’t walk for the last 20 years, blamed the hips on her father. My aunt died from HD after 2 hip and 2 knee replacements.

Rheumatoid Arthritis and HD

“Depleting rheumatoid arthritis cells of HIP1 decreased their invasion capacities by nearly half, a property required by these cells to properly recover after cartilage and joint damage.”

Result?

I still have no answers for the joint/tendon issues. I suspect that since both skeletal muscle and bone density are affected, there will be further information on other connective tissue. Someday.
Until then, I’m back on the glucosamine. Within a week I was feeling better, exercising longer.

Melatonin/Sleep

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2019

Circadian Rhythm Disturbances in Huntington Disease

I stopped taking melatonin a few weeks ago. It wasn’t initially harder to sleep. In the last week or so, I’ve felt the cycle shift. I’m up about an hour later (both in the morning and at night) almost every day. A couple of weeks ago I was sleeping (and being sleepy) at 9pm, waking at 6am. Now I don’t sleep until 2am and can’t get up until at least 10am. I’ve also been napping mid-day, sometimes several times, so tired I couldn’t get up to work. I’ve gone through my usual attempts to have more energy. Resisting napping. Regular exercise. I take Zyrtec for asthma from seasonal allergies. It shows up it its most annoying form as fatigue. But that’s not it, this time. I’m still sleepy for hours after I wake up and immediately after naps. And I sleep 12-14 hours a day.

Melatonin in HD: lower levels and improper timingMelatonin is a hormone that helps the brain decide when to sleep and wake.

As a teenager I was a ‘night owl’ and required the usual extra sleep we now know they need. Later I had kids, was working and going to school, and was consistently sleep deprived. As an adult, I’d wake up after 7.5 hours, like clockwork – as long as those hours included the interval 4-6am. In the last decade with HD, it’s increased to 9-10 hours. It’s not just how long I’m in bed – it’s actually sleeping.

I bought a light box a few years ago and use it in the winter. I have great sleep playlists and use earbuds if necessary. I do all the usual recommended sleep hygiene including limiting screen time at night, not eating late, sleeping in the cool and dark.

Brain disorder-specific sleep issues

Hypersomnia, melatonin disturbances, and circadian rhythm disturbances can’t be fixed with normal sleep hygiene.

Neurological diseases cause sleep problems. So for the next part of the experiment, I’m resuming the melatonin and I’m going to try to reset the clock. If I can’t back it up, I’ll do a progressive advance. I’m shopping the melatonin TODAY. And I’m in a place with lovely amounts of sunshine, so the therapy starts in the morning.

2019 May 13: I’ll update over the next couple of weeks.

Update 1: that day, I took 4x 3mg melatonin at 8pm and, as a last resort, and advil and a quarter bar xanax. Woke up at 1am and took another advil. Was back to sleep in half an hour. Woke at 9am, napped at 10:30. Groggy all day, blamed the xanax.

Update 2: the next day, took 4x 3mg melatonin and an advil at 8pm. Slept 9pm – 9:30 am only waking once, briefly. Groggy all day, blamed the melatonin. Planning to half the dose tonight.

Update 3: It worked! The next few days I started with 5mg and increased to 9mg and sleep was increasingly regular. I moved the dose to 7pm, as it takes a couple of hours for me to get sleepy. I’ve now been sleeping about 9pm, waking to the alarm at 7. I don’t wake during the night unless there’s some other issue. And I don’t feel groggy all day. This is consistent over a couple of weeks.

Exercise (2019)

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I’m constantly in the process of creating an exercise routine that works best for me.
A lot of practices claim they’re a fountain of youth: qigong, yoga, 20th century fitness, and 21st century ‘movement culture’. My goal is slowing the progression of HD. My quest is for something similar, even if I’m reluctant to admit it. I don’t want to live forever, I just want to live with the best quality of life.

Learning to Juggle. And Hackeysack.

My body isn’t the same from year to year (or day to day), so it needs to have a core set of goals that can be approached with flexibility.

Differences in anatomy and physiology (visible or not) aren’t built into any system I’ve studied so far. The tall, lanky basketball body versus the sprinter versus the gymnast. Differences in fast/slow twitch muscle fiber ratios. Tendencies to build muscle or not. It’s also super-important to consider pre-existing injuries and tendencies to re-injury.
Most systems work for one or two body types better than others. None work for me unmodified.

Incorporating Multiple Movement Systems

I’m ignoring systems designed to achieve some spiritual purpose (Kundalini yoga, Gurdjieff movements) unless they have integrated movement and fitness goals. Great stuff, but incorporated into other daily practices. Here’s a summary of my sources.

Hatha Yoga / Pranayama

The standard Ashtanga flavors of yoga have a lot of variation to offer, but I have to modify in a couple of ways.
Long-held static postures are a no-go. It makes my joints and tendons stiff. My body doesn’t care if it’s sitting on the couch, cross-legged for meditation, in corpse pose, or in an active balance. If it’s too long, it causes pain and seems to be counterproductive. This rules out Bikram and Iyengar.
Forward folds have to be limited: I have two torn tendons in my hip, one being a hamstring. All it takes for me to be out for weeks is to get serious in a folding-intensive class. Once.

Qigong

I’m looking at Yi Jin Jing for specific movements and combining energy work with the exercises, from qigong or other sources. “You can do vital breath through everything” – my instructor.
The stimulation from tapping seems to increase proprioception. People with HD misperceive where their bodies are in space. They won’t be aware that a hand is waving like it’s trying to flag a taxi. They often have injuries they don’t even notice.

20th Century Exercise Routines – J.P. Muller

In the 20th century, pre-physiologists like J.P. Muller and Eugen Sandow created daily exercise routines. Muller’s goal was lifetime fitness, Sandow focused on building muscle and strength. What they have in common with qigong, tai chi, and yoga is an emphasis on how to breathe while moving. Muller even wrote a 2nd book on his breathing exercises and focuses on fluidity in the movements. Muller created something that could be done daily, in 15-20 minutes.

21st Century Fitness: ‘Movement Culture’

Ido Portal took notes from Claude Victoria and capoeira. He stripped movement from context and created a philosophy of physicality based on play. Actions are given degrees of freedom and spontaneity that linear, proscribed fitness routines and physio training lack. That reduces the repetitive motion issues quite a lot.
I’m not into the extreme sport aspect of it: the one-handed handstands and flagpoles. I am one of the “people who weren’t willing to train many hours a day, six or seven days a week”. I met a movement guru who is more interested in the physiology and adaptability, and incorporating philosophy and health rather than competing like a bro.

Sport and Physical Therapy

I’m excluding sports generally and endurance sports in particular. They’re out mostly because I don’t have the coordination (HD), but some were off the table earlier.
This is not a humblebrag, I’m more of an experimenter than an athlete: I stopped triathlon training, HIIT sprints, and roller derby when I tore a hamstring in the 2000s. In the early 2010s, I took classes in trapeze, silk arts, parkour, crossfit, and gymnastics. I started climbing and capoeira.
The repetition of sport-specific training was too intense. Hand-intensive and impact-heavy sports were dropped because of the joint issues and tendinopathies: crack climbing, silks, parkour, gymnastics. I tried surfing (because I was terrified of being alone in deep water) and got surfer’s neck and flared a shoulder tendinosis from paddling out.
What playing with sports gave me was a range of training styles, exercises, and intensities.

Physical Therapy

After spine, hip, and knee injuries I went through several rounds of physical therapy and realized I didn’t need to spend 3 hours at the gym every other day to make a difference in how I move and feel.
I’m taking anything from PT and sports that hits a sweet spot: particularly efficient movement that exercises an otherwise overlooked area of my body. Rolling the IT band, wall squats, vibration therapies, superman (dorsal core, good for HD dystonia and ruptured disks).

Capoeira

The Afro-Brazilian (martial) art capoeira, is evolving proscribed movement (like a sport) and a philosophical worldview. What it has that other paradigms lack are the focus on inversions and balance in motion. Using the arms as much as the legs for stability on the floor adds a lot of fall resilience. Since I’m given the ok to modify in class as much as I need, it’s the only sport I’m (occasionally) continuing.

Thanks, Mom

Later than anyone would have expected her to be exercising, and long after she couldn’t drive, mom was at the gym. She was in much better shape than her family members at the same age with HD.
She “went to the Y for a yoga class.” Not mentioning the 20-odd minutes of cardio on the bike she did before walking (with her cane) to each of the weight machines. Before yoga class.
Mom’s the reason I’m including the strength training, too. HD increases bone loss as well as muscle loss.

Goals

The ultimate goal is a short, easy to remember, daily routine that incorporates everything in this checklist.

  • Breath and Energy (Pranayama/Qigong)
  • Complete ROM, All Joints (Fluid/Non-Impact)
  • Core Fitness
  • Neck and Spine Flexibility
  • Extremities Strength and Flexibility
  • Inversions (Lungs and Spine)
  • Tapping, Vibration (Proprioception)
  • Balance
  • Non-Impact Cardio (Heart and Lungs)
  • Oh, and under 40 minutes.
Routine Cheatsheet, 2019

Details of the new 2020 routine (and an explanation of the 2019 cheatsheet) in a later post.
I’m planning to post a breakdown of each of the movements, its purpose and effects.

Hopefully what you’d take from my process is not my routine – but how to create yours, starting with your goals and listening to your body. Experimenting and noticing reactions, short and long term.

Happy moving!