Weight Reduction
This post is about my personal challenges with weight, and managing it in light of a metabolic disorder. None of it is meant to be self indulgent or congratulatory, and I promise there are some geeky bits near the end for those patient enough to wade through the content or who want to skip to the end. Sorry for being boring, but not for being me. On with the story:
Back in the day I could have been considered a "bean pole." I was highly athletic, and though moderately muscular any of the definition was overshadowed by being wiry (though I eventually outgrew the designation "gangly," thank heavens). This is back when I could afford to be so physically active, and before the effects of the McArdle's disease steadily clamped down on the stamina. More importantly, it's when I was also getting sufficient rest to compensate for the effects of the activity I pursued regardless.
I got married, and gained the requisite 10-15 lbs. that generally comes with it, simply due to a change in lifestyle - eating more frequently, and in the company of others on a schedule of well prepared food rather than "when it suits me" and consisting of "whatever's around." Even with that gain though I was smack in the middle of the Healthy band of the BMI (though individual results vary depending on body type, I'm fortunate to be a good match for the algorithmic archetype). That weight stayed steady for another year.
Then: The Desk Job. In the interest of advancing my career and furthering the capabilities and savings of our family, I changed from the more free-form telecommuting gig to one that met those requirements but also required standard office attendance. I have no issue with the work, or with good work ethic, but I had been liberally making use of the telecommute arrangement to rest as necessary in order to recover from the day's strain. With the desk job, that luxury was forfeit. Ignorant of the specific nature of my disorder at the time, I didn't realize how disastrous this would be.
Seated at the console, plugging away on the code (Object Pascal cum Delphi RAD environment for MS IIS ISAPI filters on the MetaStorm e•Work product) I found myself wearied as usual, but unable to deal with it. I compensated by being exceptionally stubborn, and when that fell short, supplemented with anything available to increase my blood sugar (I was also drinking a lot of water, which I attributed to the poor transition from Washington's temperate and moist environment to the Utah dessert two years before - but probably only half of what I now know would be required. Whatever the case, whenever Rachelle called I seemed to be in the bathroom). This latter strategy came with predictable side effects, and in the course of five months I put on some 40 pounds.
The rapidity of weight gain was certainly off-putting and distressing, especially as the combined weight of the entire supplemental caloric intake during this time didn't seem to be adequate to account for the change. I did my best to stabilize and cut back on the snacks, suffering through the stupor instead, and plunging into another round of medical inquiry to find out why I was so tired so I could hopefully do something other than eat to manage energy levels - these of course were ineffectual. Later, changes in jobs, the production of very very cute children, and general demands of the conditions of life slowly edged the weight upward until finally peaking at about 250 lbs. (113kg), at the same time that the last vestiges of regular physical activity (Kishindo martial arts) feel by the wayside.
I was frustrated, to say the least, but also largely to blame - by putting myself and my needs very last, I was doing myself this dramatic (and visible) harm until it interfered with my ability to continue to serve my family. I was so bogged down, waking up exhausted (and frequently nauseous), barely pushing through the day, and repeating. Minor gains in understanding, not necessarily of my condition but my reaction to it, gave me some latitude for correction and over the course of about 2-3 years managed to move from 250 back down to about 220.
The most recent improvements have come from a very complete comprehension of the condition and my situation. The management strategy I'm pursuing now allows me to walk that thin line between capacity and injury, and to retain cognizance throughout even the long days (with few exceptions). Combined with that, I've been able to leverage the inability to utilize stored glucose energy to maximize natural ketosis (the same condition the Atkins and other low-carb diets attempt to induce artificially) and consume stored lipids instead - this time without the crippling side effects causing near-comatose stupor. This means that from the first day of the diet I've been able to realize dramatic results, as detailed below:
Down, down she goes!
The weight log starts in late January, testing out the Wii Fit I'd gotten the family for Christmas (but was unable to play with myself, having been separated from them). Return visits to the family explain the infrequent initial measurements, followed by my more permanent return in lat March. Throughout April and May I was completing my research into the effects and side effects of McArdle's before finally establishing the current regimen. This failed to produce any effects on the weight though, so I sought out the advice of my buddy Joel - the most successful dieter I've ever seen. Over the course of a year his appearance completely changed, to the point that when we were re-united for a get-together I recognized his wife, and wondered who the heck was with her. Thus was born the references to the "Sexy Sexy Joel Diet" (as the diet belonging to and responsible for Sexy Sexy Joel, as opposed to the old Joel).
His secret? Has nothing to do with all of those "By following these X simple rules!!1!" ads that are all over the internet, nor with fad products, "cleansing," etc. He wasn't even exercising - just following the old Weight Watchers™ formula for calculating caloric intake in terms of points (calories / 50 + ( grams of fat / 12 ) - ( min{ grams of fiber, 4 } / 5 ) ) and his allowance for consuming them. Using a calculator and log on his iPhone he followed the simple principles over time to great effect.
I adopted the same pattern, and am pleased to report that, per the graph above, have dropped 32 lbs. in the course of just over 3 months. At this rate I have another month and a half or so to meet my long term goals, after which I can settle into a maintenance pattern. Looking at the trend line it's very slowly leveling out. It also has that cyclical up-tick I'm at a loss to explain - though I also admit that I haven't gone into an in-depth analysis for them yet, as they are inconsequential to the average / long view.
For the geekiest bits: the point system, whatever its failings, works. In order to make sure all measurements are taken consistently, they've all been done within the same hour of the day, in the same general outfit (light sweats, t-shirt), immediately following my carefully metered exercise (metered to avoid undue metabolic or toxic distress) in the morning before any kind of intake. This does mean that I've artificially skewed the measurement to the lowest point in a day's fluctuation, but given that I'm moving 10-15 lbs. of water though my system throughout the course of a day it makes sense to take a low baseline, since the peak water retention will fluctuate unpredictably (or according to parameters and timings I'd rather than make explicable by mapping when there's an easier solution). Doing it the same way every day is what makes the biggest difference in collecting the samples, which allow me to apply a general trend analysis instead of a specific margin of error per day. All measurements have been taken on the Wii Fit in order to keep the analysis consistent and to provide a log of the output as well, which I have carefully transcribed and reproduced above using Perl's GD::Graph module.
I have several other observations as a result of the experience, mostly regarding psychology and sociological trends, which I'll save for another time. For now I'm excited for the day when I can stabilize enough to buy new pants (which I'd rather only do once, an account of being a cheapskate). Also, while it's true that I may have an unfair advantage due to the super-charged ketosis, Joel's experience demonstrates the viability of the approach without such an inside track (a track, I might add, that is not worth the side effects if it can be avoided).
Bon (pétit) apétit!
The Long Journey
I've written a lot about my health over the past few years, and my efforts to try and do something about it. Each little clue gave me new hope, into which I threw my full energy. I took every inch and reveled in it, moving as far as I could before inevitably declining once again (increased effort met with increased resistance). These were genuine steps forward though, rather than false starts: I lost weight, gained focus, and started sleeping better, so each time I was really complaining from a new place.
The biggest gains came from discovering sleep apnea, candidiasis, and suspicions of mitochondrial myopathy. None of these are naturally indicative of McArdle's Disease (the eventual diagnosis) though, nor are they typically concomitant. They didn't fall into their place in the puzzle until the picture was already becoming clear, when it all suddenly came together.
I should start by explaining a little about the condition. Glucose is the primary source of energy for nearly everything in the human body, the whole process referred to as "glycolysis." Most cells, especially muscle cells, have an internal reserve of it in a compact polymer-like form called glycogen. When demand for energy increases, molecules are trimmed off the end of the chain and made available to the mitochondria to do their business of converting it into active energy (adenosine triphosphate, for those taking notes).
This store of energy is pretty high - enough for 12-20 hours worth of activity before needing to be replenished. That replenishment happens on a regular, ongoing basis, to keep stores topped off whenever spare glucose is available, and any surplus is either excreted or converted into starches and stashed elsewhere. Normally this is enough to keep the body readily burning glucose between meals without running low - an exception would be fasting (more than a day), starvation, or extremely high demand: marathon runners, for example, can completely deplete glycogen stores after about 20 miles of continuous running, a phenomenon they refer to as "hitting the wall." At that point continued activity requires use of the stored fats as an alternative fuel, and risks damage to those tissues unable to do so.
McArdle's refers to a defect in or absence of myophosphorylase, which is a fancy name for "the enzyme that breaks glucose off the glycogen chain." This deficiency means that the primary source of stored energy is either completely off limits or so impaired as to be unable to meet the body's demand. This results in dramatic exercise intolerance, and in some cases cramping or seizing of muscle fibers (which require energy both to contract and relax) in a disparate and uncoordinated state so severe that they can actually rupture (rhabdomyolysis - this will be on the test), spilling their proteins into the blood and straining renal functions. Alternatively, the secondary fuel source based on fatty acids can be engaged (lipolysis).
An interesting side note here is that this is the primary intent of low-carb diets: by lowering the intake of glucose (and things easily synthesized into it by the metabolism), glycogen stores are exhausted and the body has no choice but to turn to lipids in order to remain functional. It's a hack, but a potentially effective one - your mileage may vary though, and not every system can handle the kind of stress this creates.
To point, too heavy a reliance on lipolysis floods the body with its waste products and increases the acidity of the blood (ketoacidosis). Healthy folks don't normally get to this point - diabetics can be affected in pretty nasty ways though. Those with McArdle's suffer a similar fate since the reliance on it is more absolute, and constant: this happens on regular diets without regard for carbohydrate intake. I had hit on a form of metabolic acidosis in my investigation earlier but had come to it from the wrong side, thinking that it was an inhibition of the mitochondria in making use of available oxygen, when in fact the mitochondria work wonderfully and are simply making-do with limited materials on hand. The effect is the same though: fatigue, memory disruption, stupor, and eventual unconsciousness.