Insurance Companies Don't Understand CPT2

I've been fighting to get coverage of Praluent, a cholesterol control medication that doesn't trigger rhabdomyolysis episodes in CPT2 patients.  Statins, by contrast, put me in the hospital.

Anthem Blue Cross (California) refuses this coverage, preferring (whether expressly or simply as a result of a poorly designed policy) to pay for another hospitalization (the last one cost more than a lifetime of Praluent would have cost).

Please add in the comments section your own experiences with insurance companies insisting that you do things that are contraindicated in CPT2 patients.  Please include the name and state of the insurance company.  Good experiences, bad experiences, and neutral experiences are all welcome in the comments.

It is open enrollment season, and the more data the better.

Is Gan Mao Ling a trigger?

About 2 weeks ago, for the first time in my life, I had a CPT2 episode without any identifiable trigger.   I keep a list of known CPT2 episode triggers online, but didn't see any that applied.  The episode was different from "normal" as well:  It was pain in the front, upper half of my legs.  It felt like the muscles were contracting on their own, almost like cramping, but they also hurt.  I wasn't sure it was a CPT2 episode.

A few days ago, the same symptoms returned.  This time, I checked my urine and found the protein levels were too high.  I went to the doctor, and I'm awaiting the results of my CK test.

In the meantime, I identified something that happened right before each of the two prior episodes:  I started to feel sick, and I took the herbal supplement "Gan Mao Ling".  Within about 12 hours of taking it, I was symptomatic with CPT2 (at least, I think it was CPT2).

The tricky thing about CPT2 is that mild episodes can feel a lot like other things -- exhaustion, cramping, too much exercise, etc.  I can't even say for sure that the supplement was a trigger, but I'm going to avoid it just in case.

The primary reason I'm blogging this one is so that it can be found by others with CPT2 who are considering taking the supplement.  If you do, please provide feedback.  I'm curious if others have the same experience.

PCSK9 Inhibitors and Statins -- some actual numbers

I was taking a 75 mg/mL dose of Praluent (alirocumab) in addition to a 2 mg dose of a statin (Livalo) when I had my blood work done in November 2015.  My doctor then discontinued the statin and raised the dose of Praluent to 150 mg/mL.  I had a blood draw in mid-February.  In comparing the results, the quick and dirty analysis is that my LDL went up slightly, but is still well under where it needs to be, my HDL went up moderately, my triglycerides went up moderately, and my liver enzymes were in the normal range for the first time since I started statins.

Bottom line:  I have yet to discuss with my doctor, but my instinct is to stay off of statins (reducing the risk of CPT2 episodes) and continue with the Praluent.

Here is a brief summary of my results:

	Aug. 2014	Dec. 2014	Nov. 2015	Feb. 2016
	No statin, no Praluent	Livalo 2 mg	Praluent 75 mg, Livalo 2 mg	Praluent 150 mg, no statin
LDL	198	140	77	91
HDL	40	47	42	53
VLDL	45	24	20	21
Triglycerides	305	150	144	182
AST	25	31	44	25
ALT	60	44	64	39
Creatine Kinase	173	164	192	118

Obviously, these tests are a snapshot in time.  It is also worth noting that between the November 2015 test and the February 2016 test, I relaxed my vigilance about consuming cholesterol, so I ate more foods with cholesterol.  However, it seems pretty clear that my liver (AST, ALT) and my CPT2 (Creatine Kinase) do much better without the statin (yes, I'm aware that a 192 CK is within the normal range, but it is worth noting that my "baseline" CK is lower in the Feb. 2016 than I ever remember seeing it in any test while on a statin).  My LDL went up by around 18% when dropping the statin and increasing the Praluent, and is still 35% lower than it was on the statin alone.  However, my HDL also went up -- by 26%, a good sign.

On the non-testable front, I feel better without taking the statin.  I've had fewer muscle incidents as well.  I haven't had any side effects from the Praluent except for slight bruising at the injection site the one time I injected it in the stomach area.  Although the instructions say you can do that, it really hurt and I won't be picking that area again.

PCSK9 Inhibitors and CPT2

I stopped taking a statin and switched to a PCSK9 inhibitor.  I'm taking 150 mg of Praluent.  My LDL dropped dramatically, to around half of what it was before.  Much more effective than the statin, and none of the muscle damage that was happening with the combination of a statin and CPT2.

However, Anthem Blue Cross is currently refusing to cover Praluent, and I can't afford to pay for it without insurance coverage.  If I go back to a statin, I'm likely to end up in the hospital again.  The last time, it was 6 days and probably cost the insurance company $100,000 or so.  So I'm at a loss as to why they don't want to cover it.  I'm still trying to make coverage happen.

Bottom line, though, is that if you can afford to switch to a PCSK9 inhibitor, you should definitely talk to your doctor about it.  My CPT2 symptoms have dramatically improved on the PCSK9 inhibitor, and I'm not looking forward to having to get off of it and back on a statin.  Hopefully the insurance company will do the right thing and I won't have to.

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.