After my hospitalization last summer, I decided to fly to Atlanta to see a specialist and get an ER protocol letter. I figured that being kept waiting in the hospital ER for hours while my muscles digested themselves was more than enough incentive to make sure that never happened again. I still believe that if I had been given a D10 drip immediately upon arriving (which I asked for but wasn't taken seriously), I would have been hospitalized, at worst, for a day or two. As it was, the extra hours of muscle breakdown left me hospitalized for a week.
So last weekend I experienced some shortness of breath. Since Dr. Kendall told me that cardiac myopathy is a possible side effect of CPT2 events, I don't just blow off signs that there might be a heart issue. (Spoiler alert -- things seem fine, will confirm with echocardiogram on Thursday). But I arrived at the hospital with my ER protocol letter in hand, and proudly announced that they just need to put me on a D10 drip and then they could keep me waiting as long as they needed to. Well, that didn't happen. I asked them why they were ignoring the protocol letter and they don't me "we aren't ignoring it, we just don't have space so we can't follow it". Instead they gave me apple or orange juice every 30 minutes. That is in fact not a terrible alternative, but given that the protocol letter was really clear about the importance of a D10 drip, I was really surprised that they took the chance. Yes, it was Saturday night. Yes, they were busy. But I'm willing to bet that I was maybe the 10th FOD patient they'd ever had, and the only CPT2 patient they'd ever had. So it isn't like they knew what they were doing from massive experience.
I even pulled out my trump card: "Please let your risk management department know that you are not following the Emergency Room Protocol letter". The response: "We're too busy to do that".
So I'm not going back to that ER. But it raises the question of what we should do in this situation.
In the meantime, my CK test showed an abnormally high CK level, but not the "above the maximum the test can measure" like it was last time.
Oh, the best piece of advice? I was told that because I was "walking and talking" they put me at the lowest level of urgency. Next time I'm slurring my words and falling over.
Sunday, June 7, 2015
Tremors
I didn't think anything could be worse than Kevin Bacon's performance in Tremors, but I'd rather have to sit through that movie every Saturday night than live with the tremors I'm now experiencing.
This is all new to me. Basically, when I saw Dr. Kendall in Atlanta, she observed some neuromuscular damage to my thighs. When she pressed down on my thighs, there was slight tremor when I resisted the push. My arms and other parts were normal, no tremor.
That has all changed. About two months ago, I started experiencing tremors in my arms and hands. Sometimes the tremors were so bad that I couldn't write or type. I discontinued the statin I was on (Livalo 2 mg), and the tremors seemed to improve slightly, but didn't go away.
There is a lot of stress in my life right now. The patent system has taken four big hits in the past few years (Bilski, the America Invents Act, Myriad, and Alice), each of which either weakened the ability of independent inventors to enforce patents, made them more expensive to obtain, or just did such a poor job of explaining the law that they increased the attorneys fees involved in getting a patent. Since I've primarily worked as an independent inventor for the past decade, you can imagine how this impacts my work and income. At the same time, one of my kids is going through some very expensive and tough times and that's stressing me out -- a lot. Finally, given the finances, I've mostly stopped the hobbies I had been using to destress. [Update 6/8/15: Looks like there is some validation for my subjective observation that patent value and liquidity have crashed in recent years, per this post].
Since we know that stress is a major trigger for CPT2 episodes, I'm left to wonder whether the tremors are an indication that I'm now in a constant state of baseline muscle breakdown. My last 2 CK tests, taken when I wasn't feeling particularly symptomatic, were both several times above normal (of course, several times above normal isn't a huge deal, given that I was hospitalized at several hundred times above normal last summer).
I've tried eating more, exercising less, meditation, medication, less medication, just about everything, and I'm still experiencing tremor. I was at dinner last night with my wife, the kids were in the care of my mom, and I looked down and saw my fingers trembling. It is all very scary and weird.
I'm fairly convinced it is stress, but fairly convinced and convinced are miles apart when it comes to health.
I've been advised to get a brain MRI just to make sure it is not a brain issue. I'm going to do it, but even the doctor recommending the brain MRI is doing it just in case -- we're all pretty sure it is CPT2.
On the bright side, at least the tremors aren't debilitating, and I did learn to use Dragon Dictate for when I have trouble typing. So at least there's that.
This is all new to me. Basically, when I saw Dr. Kendall in Atlanta, she observed some neuromuscular damage to my thighs. When she pressed down on my thighs, there was slight tremor when I resisted the push. My arms and other parts were normal, no tremor.
That has all changed. About two months ago, I started experiencing tremors in my arms and hands. Sometimes the tremors were so bad that I couldn't write or type. I discontinued the statin I was on (Livalo 2 mg), and the tremors seemed to improve slightly, but didn't go away.
There is a lot of stress in my life right now. The patent system has taken four big hits in the past few years (Bilski, the America Invents Act, Myriad, and Alice), each of which either weakened the ability of independent inventors to enforce patents, made them more expensive to obtain, or just did such a poor job of explaining the law that they increased the attorneys fees involved in getting a patent. Since I've primarily worked as an independent inventor for the past decade, you can imagine how this impacts my work and income. At the same time, one of my kids is going through some very expensive and tough times and that's stressing me out -- a lot. Finally, given the finances, I've mostly stopped the hobbies I had been using to destress. [Update 6/8/15: Looks like there is some validation for my subjective observation that patent value and liquidity have crashed in recent years, per this post].
Since we know that stress is a major trigger for CPT2 episodes, I'm left to wonder whether the tremors are an indication that I'm now in a constant state of baseline muscle breakdown. My last 2 CK tests, taken when I wasn't feeling particularly symptomatic, were both several times above normal (of course, several times above normal isn't a huge deal, given that I was hospitalized at several hundred times above normal last summer).
I've tried eating more, exercising less, meditation, medication, less medication, just about everything, and I'm still experiencing tremor. I was at dinner last night with my wife, the kids were in the care of my mom, and I looked down and saw my fingers trembling. It is all very scary and weird.
I'm fairly convinced it is stress, but fairly convinced and convinced are miles apart when it comes to health.
I've been advised to get a brain MRI just to make sure it is not a brain issue. I'm going to do it, but even the doctor recommending the brain MRI is doing it just in case -- we're all pretty sure it is CPT2.
On the bright side, at least the tremors aren't debilitating, and I did learn to use Dragon Dictate for when I have trouble typing. So at least there's that.
Tuesday, February 17, 2015
Off to Atlanta
I'm flying to Atlanta tomorrow to see Dr. Kendall of VMP Genetics. This is the first doctor I've encountered who claims to understand CPT2 and is able to take me as a patient. I've never had a long distance physician before, but when you've got a rare disease, I suppose it is an acceptable compromise.
There is a bit of irony here, though. One of the big triggers for CPT2 events is cold. Extreme cold comes with significantly elevated risk. I'm flying from home, where today's high is 72 and low is 47, to Atlanta, where it will be 26 degrees when I land, 15 degrees with wind chill. I'll head to the doctor the next day when it is 17 degrees, 3 degrees with wind chill. This is precisely the kind of weather I avoid.
I would rather not spend a fortune on a taxi, but public transit with these temperatures seems a bit risky. We'll see how it goes.
There is a bit of irony here, though. One of the big triggers for CPT2 events is cold. Extreme cold comes with significantly elevated risk. I'm flying from home, where today's high is 72 and low is 47, to Atlanta, where it will be 26 degrees when I land, 15 degrees with wind chill. I'll head to the doctor the next day when it is 17 degrees, 3 degrees with wind chill. This is precisely the kind of weather I avoid.
I would rather not spend a fortune on a taxi, but public transit with these temperatures seems a bit risky. We'll see how it goes.
Friday, January 23, 2015
CPT2 in the news
Somebody on the CPT2 Google Group just posted a link to a story about a hockey player who has CPT2. There isn't much press coverage of CPT2, so I'm going to start collecting links to press coverage here. I'll update this page as I find new links.
http://www.collegehockeynews.com/news/2015/01/22_fighting_back.php
https://www.washingtonjewishweek.com/you-should-know-allison-isaacson/?fbclid=IwAR3rjccJNS-1FroRTjZq6egZxGaHGBbKmM4poL8L3-H5XI0l-l82rUXkPzU
http://www.collegehockeynews.com/news/2015/01/22_fighting_back.php
https://www.washingtonjewishweek.com/you-should-know-allison-isaacson/?fbclid=IwAR3rjccJNS-1FroRTjZq6egZxGaHGBbKmM4poL8L3-H5XI0l-l82rUXkPzU
Friday, December 12, 2014
MCT Oil Resources
Medium Chain Triglycerides are a strange thing when it comes to fatty acid oxidation disorders. For those with CPT2 deficiency, MCTs are fantastic -- they can be metabolized easily and quickly despite CPT2 issues. For those with other fatty acid oxidation disorders (i.e. MCAD), they can be deadly. One thing that both ends of the spectrum have in common is a need to understand MCTs -- where they are found and what they do. I couldn't find a good list of MCT resources, so I'm going to use this post to gather some MCT resources. I'll update it periodically.
http://mrvitaminsnews.com.au/weight-loss/how-to-get-more-mcts-those-amazing-fats-that-make-you-thin-and-other-fat-facts: "In the vegetable kingdom there is one other oil with a similar saturated fat profile to coconut oil and that is palm kernel oil. This too is high in MCTs.... [However], although from the same plant, palm oil has a very different composition to palm kernel oil and is high in polyunsaturated fats that are not good for your cholesterol levels." They also identify the following as having high MCT content: Sheep milk, yogurts and cheeses made from sheep's milk.
http://nutritionreview.org/2013/04/medium-chain-triglycerides-mcts/ : A good article describing how MCTs are metabolized. It provides pretty good detail (for example, MCTs have 8.3 calories per gram vs 9 calories per gram for LCTs, and that MCTs enjoy accelerated metabolic conversion).
http://en.wikipedia.org/wiki/Medium-chain_triglyceride: Well, it's Wikipedia so I can't predict what the page will look like in the future. It currently contains useful information ("MCTs lack the cholesterol raising Myristic and Palmitic Acids") as well as odd information ("The milk fats of horses contain large amounts of medium-chain fatty acids").
http://www.livestrong.com/article/316430-sources-of-medium-chain-triglycerides/: Easily summarized: MCTs are found in coconut oil and olive oil (note that other sources do not list olive oil as an MCT source, so it is unclear whether livestrong has this right). They don't break down the ratio of MCT to other fats in either oil, however.
http://healthimpactnews.com/2014/mct-oil-vs-coconut-oil-the-truth-exposed/: I'm not sure about the value of this article vis-a-vis CPT2. The article attempts to describe how coconut oil differs from artificially created MCT oil.
http://www.coconutresearchcenter.org/article10612.htm: This article was published at "coconutresearchcenter.org", so it may have pro-coconut bias, but it is still a fantastic resource. It provides a lot of detailed information about the benefits of MCT in coconut oil.
http://www.greenmedinfo.com/blog/mct-fats-found-coconut-oil-boost-brain-function-only-one-dose: This article describes a study that purportedly shows that "MCTs ... almost immediately improved cognitive function in older adults with memory disorders."
http://www.bodybuilding.com/fun/issa23.htm: This article looks at MCT oil from the bodybuilding perspective.
http://www.meltorganic.com/what-are-medium-chain-fatty-acids-and-why-are-they-important-to-your-health/: General description of MCT oil.
http://www.latimes.com/health/la-hew-askus25jun25-story.html: Expresses concern that long term use of coconut oil may have adverse cardiovascular effects.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2874191/: A scientific journal article that reports on a study of health impacts of MCT oil. "Our results suggest that MCT oil can be incorporated into a weight loss program without fear of adversely affecting metabolic risk factors." Very well sourced article with 32 footnotes.
https://louisville.edu/hsc/compliance/site-medschool-pediatrics/clinical/wcec/genetics/nutrition/about-mct/recipes/MCT%20Recipes.pdf/at_download/file: MCT oil cookbook
http://healthimpactnews.com/2014/mct-oil-vs-coconut-oil-the-truth-exposed/ Differences between MCT Oil and coconut oil.
http://mrvitaminsnews.com.au/weight-loss/how-to-get-more-mcts-those-amazing-fats-that-make-you-thin-and-other-fat-facts: "In the vegetable kingdom there is one other oil with a similar saturated fat profile to coconut oil and that is palm kernel oil. This too is high in MCTs.... [However], although from the same plant, palm oil has a very different composition to palm kernel oil and is high in polyunsaturated fats that are not good for your cholesterol levels." They also identify the following as having high MCT content: Sheep milk, yogurts and cheeses made from sheep's milk.
http://nutritionreview.org/2013/04/medium-chain-triglycerides-mcts/ : A good article describing how MCTs are metabolized. It provides pretty good detail (for example, MCTs have 8.3 calories per gram vs 9 calories per gram for LCTs, and that MCTs enjoy accelerated metabolic conversion).
http://en.wikipedia.org/wiki/Medium-chain_triglyceride: Well, it's Wikipedia so I can't predict what the page will look like in the future. It currently contains useful information ("MCTs lack the cholesterol raising Myristic and Palmitic Acids") as well as odd information ("The milk fats of horses contain large amounts of medium-chain fatty acids").
http://www.livestrong.com/article/316430-sources-of-medium-chain-triglycerides/: Easily summarized: MCTs are found in coconut oil and olive oil (note that other sources do not list olive oil as an MCT source, so it is unclear whether livestrong has this right). They don't break down the ratio of MCT to other fats in either oil, however.
http://healthimpactnews.com/2014/mct-oil-vs-coconut-oil-the-truth-exposed/: I'm not sure about the value of this article vis-a-vis CPT2. The article attempts to describe how coconut oil differs from artificially created MCT oil.
http://www.coconutresearchcenter.org/article10612.htm: This article was published at "coconutresearchcenter.org", so it may have pro-coconut bias, but it is still a fantastic resource. It provides a lot of detailed information about the benefits of MCT in coconut oil.
http://www.greenmedinfo.com/blog/mct-fats-found-coconut-oil-boost-brain-function-only-one-dose: This article describes a study that purportedly shows that "MCTs ... almost immediately improved cognitive function in older adults with memory disorders."
http://www.bodybuilding.com/fun/issa23.htm: This article looks at MCT oil from the bodybuilding perspective.
http://www.meltorganic.com/what-are-medium-chain-fatty-acids-and-why-are-they-important-to-your-health/: General description of MCT oil.
http://www.latimes.com/health/la-hew-askus25jun25-story.html: Expresses concern that long term use of coconut oil may have adverse cardiovascular effects.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2874191/: A scientific journal article that reports on a study of health impacts of MCT oil. "Our results suggest that MCT oil can be incorporated into a weight loss program without fear of adversely affecting metabolic risk factors." Very well sourced article with 32 footnotes.
https://louisville.edu/hsc/compliance/site-medschool-pediatrics/clinical/wcec/genetics/nutrition/about-mct/recipes/MCT%20Recipes.pdf/at_download/file: MCT oil cookbook
http://healthimpactnews.com/2014/mct-oil-vs-coconut-oil-the-truth-exposed/ Differences between MCT Oil and coconut oil.
Wednesday, November 26, 2014
MCT Oil
I've been working hard to take control of how my diet interacts with my CPT2. My brother has taken MCT oil regularly for years -- and he has also been less symptomatic than I am. I had long assumed that this was because he is a good weight for his height, whereas I struggle with my weight. I still think that this has something to do with it, but there is a bigger role for MCT oil than I thought.
Over the past month, I've been regularly adding MCT oil to my diet. My muscles have behaved much better with added MCT oil. I'm going to stick with this for several more months and reevaluate, but my current thinking is that it is going to end up being a permanent dietary change.
MCT oil apparently has a smoking point of 350 degrees, so it is a bit limited as to how it can be used. An easy way to incorporate MCT oil is substituting it for regular oil in cookie and brownie recipes and boxed mixes. If using boxed mixes, look for ones that have minimal fat already in the mix. Pillsbury makes a brownie mix with less than 5% of calories from fat in the boxed ingredients.
Because the goal of adding MCT oil is to develop a tasty and well-tolerated MCT delivery system, I haven't cut the amount of MCT oil in the recipes. That said, MCT oil appears to saturate the food far better than regular oil -- the food is far more moist than with normal oil. It hasn't been an issue for me (kind of a plus), but there is a difference in texture.
Also, be sure to label your food as "MCT Oil". Hypothetically, if my daughter's friend slept over, smelled freshly baked cookies, didn't know they had MCT Oil, ate a dozen of them (without asking!) and then destroyed the bathroom, that wouldn't be good. MCT Oil is tough on the stomach. So label, label, label.
Update 12/12/14: Here is a mini-cookbook for MCT oil: https://louisville.edu/hsc/compliance/site-medschool-pediatrics/clinical/wcec/genetics/nutrition/about-mct/recipes/MCT%20Recipes.pdf/at_download/file [UPDATE: Looks like that link is dead now]
Update 12/12/14: Here is a mini-cookbook for MCT oil: https://louisville.edu/hsc/compliance/site-medschool-pediatrics/clinical/wcec/genetics/nutrition/about-mct/recipes/MCT%20Recipes.pdf/at_download/file [UPDATE: Looks like that link is dead now]
Tuesday, November 18, 2014
My belated response to the Invisible Illness Week list of questions
I was recently given a link to a page about invisible illnesses. http://www.butyoudontlooksick.com/wpress/articles/written-by-christine/30_things/
CPT2 seems to qualify: It is difficult to know somebody is experiencing an episode, and medical providers often have only the patient's self-reported description to go on. Given that, I thought it might be interesting to answer the 30 questions myself:
30 Things About My Invisible Illness You May Not Know
1. The illness I live with is: CPT2 deficiency.
2. I was diagnosed with it in the year: I was diagnosed December 8, 1992.
3. But I had symptoms since: I can remember having symptoms around kindergarten. I actually can't remember a time before I had symptoms.
4. The biggest adjustment I’ve had to make is: Practicing mindfulness about my physical status and limitations.
5. Most people assume: I'm fine. Even those who know I have CPT2 have a hard time understanding that somebody can have a significant condition yet be perfectly fine almost all of the time.
6. The hardest part about mornings are: I have three daughters, and getting to see them first thing in the morning is always great. Actually, I feel great most mornings -- being nearly motionless for eight hours isn't exactly a trigger for a muscle condition.
7. My favorite medical TV show is: Mystery Diagnosis. CPT2 was featured on it twice. At least I have something objective to point to to help people understand my condition.
8. A gadget I couldn’t live without is: My brain. Seriously, I love computers, hardware and software, but my brain -- my "wetware" -- is my favorite gadget.
9. The hardest part about nights are: Trying not to eat late at night. If I eat late at night, I replenish my carbs for the morning, but 99% of nights, my carbs are already just fine. I just have trouble telling when it is one of the 1% of nights that I actually need to eat.
10. Each day I take: Bezafibrate (Bezlip) 400 SR in the morning. There is some evidence that it up-regulates CPT2 production.
11. Regarding alternative treatments I: Don't have a choice. There are no actual, scientifically validated treatments (other than Bezafibrate, which is only partially effective). There are so few people who have CPT2 that anecdotal evidence ("MCT oil works for me", or "Corn starch helps") carries a lot of weight.
12. If I had to choose between an invisible illness or visible I would choose: I have never known life without CPT2. I love myself for who I am, flaws and all. I do wish that I had been diagnosed earlier, I could have avoided a lot of pain.
13. Regarding working and career: I get distracted at work and don't remember to eat, which then causes me to have trouble working. When work stresses me out, that can sometimes trigger an episode.
14. People would be surprised to know: How terrifying it was before the Affordable Care Act, when I lived in fear that I would lose my insurance and be unable to get new insurance (because of the preexisting condition I was born with). I can't imagine being unable to afford to get medical help when I need it.
15. The hardest thing to accept about my new reality has been: Well, it isn't a new reality. It is an evolving reality. As I learn more about my condition (when I was diagnosed, there were fewer than 50 diagnosed cases -- now we're in the hundreds), I try to change my lifestyle to fit. Old habits being hard to change and all.
16. Something I never thought I could do with my illness that I did was: This question doesn't fit my experience. People with CPT2 can do anything "normal" people can do, we just have to be very careful about how we do it. I suppose if I ran a marathon without constantly taking in carb calories I would be in deep trouble, but my limits are always in combinations: If I do X and Y happens, I have a problem.
17. The commercials about my illness: I wish there were commercials about my illness. Too few people have it for it to be of even the most remote interest to the drug companies.
18. Something I really miss doing since I was diagnosed is: Nothing. I don't remember a time before I was aware that something was amiss, and by the time I had a formal diagnosis, I had already structured my life around my limits.
19. It was really hard to have to give up: Eating whatever combination of carbs/fat/protein I wanted.
20. A new hobby I have taken up since my diagnosis is: I think my diagnosis had a subconscious effect on my choice of hobbies. Before my diagnosis I took up scuba diving, mountain biking, swimming, etc. After my diagnosis I took up far less physical hobbies. I never spotted that pattern before today.
21. If I could have one day of feeling normal again I would: I'm lucky. I have many days of feeling normal. If I had one day when I didn't have to worry about CPT2, I'd go on a long hike (exercise = trigger) in the snow (cold = trigger), and I'd power it all by eating fat (high fat diet = trigger) and protein.
22. My illness has taught me: That it is hard to miss that which you never had. I've always had CPT2, I've always lived with the symptoms, but I've never thought of myself as disabled. There have absolutely been times when I couldn't walk from the bed to the bathroom, but my internal dialogue is not "oh, poor disabled me".
23. Want to know a secret? One thing people say that gets under my skin is: "You got the right answer, but you got it the wrong way, so you're wrong." I know, it has nothing to do with CPT2, but as an inventor, it makes me nuts when people say that.
24. But I love it when people: Open up about their own life experiences in response to my opening up about my condition. As humans, we have so much in common -- but we find ways to pretend we don't. Honestly discussing my own vulnerability gives people a safe place to talk about their own issues.
25. My favorite motto, scripture, quote that gets me through tough times is: I love myself.
26. When someone is diagnosed I’d like to tell them: Research the disorder. Track your own experiences and how your body responds. Network with other people who have it.
27. Something that has surprised me about living with an illness is: I've always lived with it. So no surprises.
28. The nicest thing someone did for me when I wasn’t feeling well was: Believe me. This is a disorder that often involves no outward indicators that anything is wrong.
29. I’m involved with Invisible Illness Week because: Somebody sent me the link, and it resonated.
30. The fact that you read this list makes me feel: Like I accomplished something.
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