Thursday, November 10, 2016

It finally happened -- An illness that stopped me from eating

I suppose it was only a matter of time.  I've been highly susceptible to strep throat my entire life.  I was scheduled to have my tonsils removed, but given the malignent hyperthermia risk with anesthetic for CPT2 patients, that surgery never got scheduled.

I was set to spend election week in Vancouver, Canada with my family.  I started having throat pain a few days before we left, but I got on antibiotics and was sure I would be ok.  I wasn't.  Soon enough, the strep had caused lesions on my tonsils.

A quick word about the Canadian health care system:  Even if you are a cash-pay patient, it is way cheaper than in the US, and the quality was great.  I know this because I visitied a doctor here to change my medication, as I was pretty sure the antibiotic I was on wasn't effective against strep.  They switched me to a new antibiotic.

Soon enough, the my lesions were healing.  I'm still feverish and symptomatic, but on the mend.

With that as the background, here is the CPT2 issue:  I couldn't eat.  Literally, even swallowing water triggered my gag reflex.  When there are lesions on your tonsils, your brain freaks out and thinks each lesion is a piece of food ready to choke you.

As a result, I had two CPT2 episodes.  Remember that fever (check) and illness (check) are triggers.  Lack of carbs is the major trigger (check).

What was the solution?  Well, it gets complicated.  Normal anti-nausea medications are off the table for me since promethazine causes me to develop permanent dyskenesia.

Sounds like check-mate, right?  I can't eat because I have nausea.  I can't treat the nausea because the treatments expose me to unacceptable side effects.

It wasn't check-mate.  My life was saved by medical marijuana.  A very small amount of Indica (a strain called "BC chemo" bred to suppress nausea) was enough to override the nausea and let me eat.  Both times I had muscle breakdown, I was able to reverse it quickly with BC chemo.  The alternative would have been being hospitalized and being fed through a stomach tube or intravenous drip.

I still feel bad.  I'm still sick.  But my muscle disorder is under control.

Monday, October 31, 2016

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.

Friday, May 13, 2016

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.

Thursday, February 25, 2016

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
Creatine Kinase

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.

Monday, February 8, 2016

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.