Tremors, Part II

I have now had a 2 hour EEG, a brain MRI, and a 5 day ambulatory EEG.  The results of the first two exams show nothing that would be causing the tremors, and I am awaiting the results of the 5 day EEG (but I strongly suspect they too will not reveal the cause of the tremors).  This leaves two possible causes -- CPT2, or that terrible Kevin Bacon movie.  I'm guessing CPT2.

While visiting Utah, I had the opportunity to climb a mountain to visit some caves.  There was an approximate 1,200 foot elevation gain over around 1-1/2 miles.  I stopped (very) regularly to eat simple carbs (um, Skittles if you must know).  We even got to enjoy a thunderstorm cell that passed over us mid-hike (drenched, but was fun, lightning was quite distant).  

I did not experience CPT2 breakdown in the way I was used to, but I did ask my daughter to push down on my leg while sitting at various places on the climb.  There were slight tremors at the start of the hike, and by the end of the hike they were much more noticeable.  I've done similar tests when I haven't eaten enough, etc., and there does seem to be a correlation.  

So what I'm seeing is negative results from the neurology side, and positive results from exercise or fasting.  I'm nearly positive that the tremors are a "normal" side effect of CPT2, and nothing worse.  I can live with this -- literally.  Sure, tremors suck.  It makes certain things harder.  It is another way that CPT2 qualifies for accommodation under the Americans with Disabilities Act.  But it isn't going to kill me.  In fact, I wonder if it has been there all along and I'm just now noticing it, or if it is a side-effect of the big muscle breakdown I had last summer that ended up with a week in the hospital.  Either way, assuming that the ambulatory EEG is also negative for tremor-inducing indications, it is exactly as I expected -- caused by CPT2.

On a side note, you really don't want to do an ambulatory EEG.  They suck.  Electrodes are literally glued/cemented to your head and you are wired to a measurement device for almost a week.  No water is allowed to touch the device, so sponge baths and no hair washing.  I'd really rather not do this again.  Ever.

In case you were wondering what I meant about the Kevin Bacon movie, here it is:

  [edit:  Looks like blogger.com blocked the image I posted.  I'm convinced it fell under fair use, but whatever.  Here is the link to the IMDB page.]

My trip to the hospital last weekend, and why ER Protocol letters aren't all they're cracked up to be

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.

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.

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.

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

https://www.firstpost.com/health/a-new-netflix-series-reveals-the-mysteries-behind-a-good-diagnosis-episode-1-focuses-on-a-rare-fat-metabolism-disorder-7247851.html