Tuesday, August 20, 2019

My ER visit triggered CPT2 and landed me in the hospital

I have a card with my ER protocol on it.  I've reproduced it below:

I was sick from Friday until Monday.  I had a bad sore throat and a fever.  I decided to go to the walk-in clinic on Monday and was given antibiotics and a strep swab.  While waiting for the antibiotics, I took care of an outstanding labwork order that included a CK measurement.  My CK was 109, well below the 165 that is the upper limit of normal.  In other words, careful diet management over the weekend avoided my illness turning into a CPT2 episode.  If only it had ended there.

I started having a cough and when my pulse oximeter read only 91, I decided to go to the ER.

When I arrived, the first thing I did was hand them my card.  They said basically "no thanks, hand it to the triage nurse".  A while later, I was called to see the triage nurse.  I again said I have CPT2 and handed her my ER protocol card.  She held it for perhaps 30 seconds glancing between me and the card.  In retrospect, she was either a remarkable speed reader or just pretended to read the card (or maybe she skimmed it).  It was a very busy day in the ER and I suspect she was under a lot of pressure to move the line along.

After waiting for a long time (total time sitting in the ER waiting room was around 6 hours, so the blood draw was likely after about 90 minutes) I was given a blood test, but they didn't order CK. At this point I was worried that the stress of being in a standing-room-only waiting room could potentially trigger a CPT2 episode.  When they first said they would order a blood test, I asked them to please add CK, and they said they would, but when my blood was drawn, the order didn't include CK.  I asked the person taking the blood to please fix this and add a CK reading.  I later learned that CK wasn't run initially, and it wasn't until much later, when I was symptomatic, that they read the CK.  It turns out that it was around 1,000 at that time.

It is worth remembering that had they followed my ER protocol, I would have already been on a D10 drip, so I'd be home now instead of writing this from a hospital bed.  If they had fed me as the protocol required, that also would have avoided this whole episode.  But they didn't, and I was sent back to the waiting room to, well, wait.  And wait.  And wait.

There was a vending machine there, but all of the solid food snacks were too high in fat to be helpful.  However, there was also a soda machine that had orange juice, so I kept up my carb intake that way.  Sadly, orange juice failed me.

I started to feel leg pain, but what is scarier is that my earlier cough was masking muscle breakdown pain in my chest, upper back, and most frightening, my diaphragm (you know, the muscle that lets you breathe).  I figured the pain was from the coughs.  It wasn't.  Soon I was in pretty severe pain.  Luckily, my long ER wait was finally up and I was called back.  At that point, all of the breathing issues that brought me to the ER in the first place had resolved on their own.  However, I now had a much bigger problem.  They re-ran my CK and it was up to 3,000. I was finally started on D10 and given juice.  A couple of hours later it was 6,000.

So what went wrong?  First off, they didn't follow my ER protocol.  That sucked, but I should have been way more pushy about it.  While I lived in the US I would have been (I know this because I once asked a US hospital's ER staff to let their risk management department know they were ignoring my ER protocol).  But this is Canada, and that kind of behavior just isn't done (though it will be done next time).  So my second error was not becoming hyper-insistent and pulling out my "I'm a graduate of Harvard Law and I know you should be following this -- who is your supervisor" persona.

My third error was not adding up triggers.  I know triggers.  I maintain a well researched and sourced list of triggers.  Infection is a trigger, and I definitely had that.  Fever is a trigger, and I'd had that all weekend and probably part of Monday.  Anxiety is a trigger -- and waiting in that ER lobby for endless hours certainly stressed me out.  I've never had a CPT2 episode with less than 3 triggers (with one exception -- the dual triggers of not eating enough food and exercising too hard).  However, I've had plenty of episodes with all kinds of mixtures of three or more triggers.

Again, the ER protocol was designed to avoid just this scenario.  CPT2 episodes can cause what my wife has aptly named "brain fog".  It is likely a result of hypoglycemia that is part and parcel of the CPT2 experience.  In fact, this cognitive fog was once misdiagnosed as a severe case of ADHD.  By the time I had my blood test, it was too late for me to avoid this on my own, as the cognitive fog had set in.  The reason I had cards printed in the first place was because CPT2 episodes often made me too scattered to properly advocate for my own care.  As a side note, like most invisible illnesses, CPT2 is only an issue during an episode.  There is no cognitive fog unless I'm in the midst of an episode.

The good news is that the VCH Metabolic Disease Clinic consulted with the ER doctors by phone late at night, and visited me the next morning.  I feel like they are on top of my recovery.  That same clinic was the source of the text of the ER protocol on the card, which makes it really beyond comprehension that the triage staff at the ER didn't follow a protocol written by specialists using phrasing that no lay person would use.

The lesson here is that ER visits are very stressful, so I need to treat them the same way I would treat a long hike -- by reducing the risk of a CPT2 episode.  That means carb-loading prior to visiting the ER, bringing lots of carbs with me to eat in case I have to wait, and having somebody with me in case the cognitive fog kicks in.

With invisible illnesses like CPT2, we are absolutely fine until we're not.  Then we're absolutely fine again.  There is nothing visible from the outside that tells people we're at higher risk than the person sitting next to us.  That said, I actually handed the ER protocol card to literally every health care provider I saw on that visit, and nobody followed it, so I'm not sure what more I could have done to make my invisible illness more visible. Of course, once a standard method of evaluating body damage showed damage (i.e. the CK results), the invisible illness was suddenly visible to all.  But the shame of it is that the entire episode could have been prevented if any one of the providers had taken the ER protocol seriously.

CPT2 returns to TV

Netflix has a new show called "Diagnosis". 

The description of Season 1, Episode 1 is "Detective Work: The once-athletic Angel, 23, suffers from episodes of muscle pain so severe she often can't move.  As she begins a nursing career, she needs answers."

Given the title of the blog I'm posting on, it shouldn't surprise anyone that her disease is diagnosed as CPT2 deficiency.

First, the fact that she spent nearly a decade trying to figure out her diagnosis means that I personally consider this blog to have failed her.  The idea of posting my experiences was to help people like her (seeking diagnosis) by giving them something to discuss with their doctor.  It also means that the super-helpful Facebook FOD group and the great, more specific "What you can do despite CPT2" Facebook group have failed her. They are great resources, but somehow didn't place high enough in search results for searches done for the key symptoms of CPT2 episodes.  I personally think the quickest answers come from the Google Group for CPT2, but that too didn't help.  As much as the CPT2 community is a strong one, we need to do much better at getting listed in response to searches for symptoms of CPT2 attacks.

So, here is a quick search-engine-friendly, somewhat incomplete list of symptoms (note that I do not have advertising on this blog and I'm not trying to monetize any improved search engine results, I just want people with CPT2 to be able to find the resources they need).

Coca-Cola or Coke colored urine, dark pee, dark red or other evidence of blood in the urine (since many quick tests just test for iron in the urine and call it "blood", somebody with muscle fibers in their urine will test positive for blood in the urine if the test just looks for iron).

Muscle weakness, exercise makes my legs hurt so much I can't bend them, I suddenly get very stiff and painful muscles.

When I'm stressed out, I get muscle pain.  When I don't sleep enough, my muscles hurt. When I eat meals with too much fat, my muscles ache.  When I don't eat enough, I can't walk very far before I get muscle failure.

I got propofol infusion syndrome PIS or hyperthermia from an anesthetic that act on fatty acid.

When I tried Atkins, Paleo, low carb, no carb diet, my muscles hurt a lot.

My doctor doesn't know what's wrong with me, but my CK is sometimes really high.

My CK was over 10,000.
My CK was over 20,000
My CK was over 30,000
My CK was over 40,000
My CK was over 50,000
My CK was over 60,000
My CK was over 70,000
My CK was over 80,000
My CK was over 90,000
My CK was over 100,000

I got rhabdomyolysis.

If these help one person find this blog and then go on to the community groups I linked at the top, I'll call it a success.

Thursday, July 26, 2018

What I learned spending a year eating under 15% calories from fat

Put briefly, it works.

My diet was:

1. Prescription grade MCT Powder every morning, approximately 140 calories (provided free of charge by the BC government -- Canadian health care is one of the things that makes Canada awesome!).

2. Eating at home: Under 15% of calories from fat. Sounds simple, but it isn't.  Let's put it this way:  100 grams of corn has 96 calories and 1.5 grams of fat.  At 9 calories per gram, that is 13.5% of calories from fat. The baseline fat in many foods we consider fat-free is actually more than zero.
   2(a): Watch out! In the US, if it is less than 0.5 grams of fat, they may simply say "zero".  You don't normally see food labels list 0.3 grams of fat.
   2(b): Canadian products, including "fat free" dairy products, actually DO list fractional grams of fat.  Surprise! Many "fat free" dairy products do have fat in them.  Read the label.
   2(c): I normally want every single ingredient to have less than 15% of calories from fat.  That way I actually eat less than 15% calories from fat -- because I assume that through the course of the day I may make errors and this lets me make a mistake (such as when they give me "skim milk" creamer at a restaurant that is really 2%) without blowing the 15% mark.

3. Eating away from home:  This requires a bag of tricks.
  3(a) First, nothing on the menu is going to work.  Seriously.  I can count on one finger (or less) the number of times I was able to just order a menu item without having to have a conversation with the server about fat content.
  3(b) Second, pizza.  Pizza is a fantastic choice, with a giant caveat: "I'd like to order the Margherita Pizza, but with no cheese.  If there is any oil in the pizza sauce, please just put some tomato slices on it instead."  Done this way, it is basically bread and vegetables. Some breads have more than 15% of calories from fat, but it isn't going to destroy your diet ratio.  Just offset it...
  3(c) Third, offset it.  Order some rice or a dry baked potato to eat with the meal. This gives you extra carbs and reduces the overall percentage of calories from fat.
  3(d) Fourth, your dessert is always fruit.  Always.  You don't have a choice (except periodically sorbet when it is available).
  3(e) Fifth, you don't get to order fancy coffee either unless it is fat free.  One regular latte pretty much blows your ratio for the day.
  3(f) Sixth, get creative. Tell the server that you have a muscle disorder and you're medically limited to 15% of calories from fat and you'd like to order a piece of grilled chicken on a roll with no mayo, no cheese, nothing else with fat.  Then smile and say "I'm not allowed to eat anything fun anymore, so instead of fries can I have a salad with no cheese, no dressing".  The self-deprication prevents the server from getting annoyed with you.

4. Learn math tricks.
  4(a) 15% is a difficult thing to calculate on the fly, particularly when you're at the supermarket and looking over dozens of nutritional labels in an hour.  What is 15% of 145 calories?  Yeh, I don't know either.  The good news?  It doesn't matter -- use the trick in the next paragraph.
  4(b) Long chain fats have 9 calories per gram (medium chain fats have 8 calories per gram, but those with CPT2 can metabolize them, so they don't count against the 15%).  It turns out that 9/60=15.  In other words 1 gram of fat = 9 calories = 9/60.  This makes life simple:  You can eat anything that has 1 gram of fat per 60 calories or less.  If it has more than 1 gram of fat per 60 calories, you don't get to eat it.  Quick test: 4 grams of fat in 218 calories -- OK or not OK?  Easy answer:  4x60=240.  240<218, so all good, eat away.  What about 6 grams of fat in 250 calories?  6 x 60 = 360.  360 calories > 250 calories, so the amount of fat is too high (i.e. 6 grams of fat requires at least 360 calories to be 15% or less).

5. Protein:  I was told by a former nutritionist to stay away from protein. That is not the advice my CPT2 specialist gave me. During an active muscle breakdown episode, protein can be risky since metabolizing muscle fibers and protein both happen in the kidneys, putting them at heightened risk of failure.  If I'm not having an episode, I no longer limit my protein (except almost all proteins have fat in them, so I do the "no more than 1 gram of fat per 60 calories" trick.


I had my second visit with an Adult Metabolic Disease Clinic nutritionist last week, and learned I was doing things almost right.  The "almost" was a trick that I'm not allowed to do anymore.  I was adjusting the total number of meal calories by ordering juice or pop (with sugar) with my meal so that it would offset the fat calories.  It turns out it is far more complex.  Yes, you can offset, but only with complex carbohydrates.  So a side order of whole wheat bread is all good, but a Pepsi, as delicious as it is, is not a legitimate way for CPT2 patients to offset calories.  You can still have sugar pop drinks, but just don't use them to offset calories.

Importantly, I was told to make the carbs I eat as complex as possible.  This is itself complex, because whole foods often have more fat than their simple carb counterparts.  For example, brown rice has 1.8 grams of fat per 8 ounces.  It is still ok, because it has 216 calories (rounding up, 2 x 60 = 180, so brown rice is just fine). But in terms of a fat-free food offsetting other foods with fats, complex carbs aren't a magic bullet.

Also, now that I've moved to Vancouver, I will say that Kombucha is awesome.  My nutritionist was clear that Kombucha is sufficiently complex that it is just fine to drink.

Tuesday, March 27, 2018

Getting close to asymptomatic

After visiting the Vancouver Metabolic Disease Clinic, I made two big changes in my approach to CPT2:

1. I now have at least 140 calories, and sometimes 280 calories, of pharmaceutical grade MCT powder.  The first 140 calories are consumed right after I wake up.
2. I have strictly limited my diet to 15% of calories from (long chain) fat at a maximum.

I've felt CPT2 symptoms hint that they were coming on.  One day I forgot to eat breakfast and lunch and by dinner I had some shaking.  But it never progressed beyond the worrisome early signs.  As far as I can tell (mostly from looking at my urine), I haven't had significant muscle breakdown since I started MCT and lowered my fat intake to under 15%.

Will this work for everyone?  Probably not.  Is it unlimited protection from CPT2 episodes?  Surely not.  But it is still the best my muscles have done since I became an adult.

Monday, September 11, 2017

Beware the risks of recessive CPT2, at least with statins

I ran across an study today that everybody with CPT2 deficiency should be aware of.  It shows that those who are heterozygous for CPT2 (so not symptomatic) are 13 times more likely to experience muscle myopathy when given statins.

We know so little about fatty acid oxidation disorders that it makes sense for us to give our relatives a heads-up that they should let their doctors know they may be recessive for CPT2.  This is particularly important to discuss when getting statin prescriptions.

Click here to see the article and search for "CPT" to find the relevant paragraph.

Here is the relevant section:

Thursday, July 6, 2017

Visit to Vancouver General Hospital Adult Metabolic Diseases Clinic

Today was my intake appointment with the Vancouver General Hospital Adult Metabolic Diseases Clinic.

Let's start with the startling:  They know way more than I do about CPT2.  This may not sound surprising, but it is.  My normal routine is to teach my physicians about CPT2.  They then look up more information about CPT2, ask me questions, and eventually get up to speed.  None of that here.  They know this disorder, and I could not be happier to have this amazing resource in my (new) backyard.

I learned a few new things today:

(1) Carnitine (and therefore Carnitor) is associated with increased cardiac risk, and may be a primary mover behind the cardiac risk associated with red meat.  On balance, it is positive for my health to take Carnitor, but for the first time I learned that there is a downside to it as well.  I haven't eaten red meat in 34 years, and learned today that this is actually positive for my muscle health (Carnitor takes care of my carnitine levels, and red meat has a lot of long chain fats that are bad for my muscle condition).

(2) MCT oil in coconut oil form isn't a good idea.  Despite the medium chain fatty acid content, coconut oil has too much long chain fatty acid content to be a good choice.  Instead, refined MCT oil is preferred.

(3) There is a prescription grade MCT available in liquid or powdered form, as a prescription medication.  I'm going to try it.

(4) I don't need to fear protein.  My diet should be as low in fat as possible, preferably at or below 15% (I knew this already), but the protein component is recommended at 15% strictly because of the number of carbohydrate calories I should consume.  Until today, I feared that protein would present renal risk for CPT2 patients.  I was told it does not, as long as my kidneys are currently functioning properly.

(5) Raw corn starch is good for young kids, but in adults it is better to eat complex carbohydrates at intervals throughout the day.  Raw corn starch has little or no nutritional value beyond being a slow release carb.  I find raw corn starch difficult to stomach (literally, it gives me stomach aches), so I'm pretty happy to leave that behind in favor of regular bites of complex carbs.

(6) No more deep fried foods.  Watch out for Chinese food, salads loaded with dressing, and other high fat sources.  I'm letting this sink in.  No.  More.  French.  Fries.  That's a triple sad face, and it is going to strain my willpower to keep it up.

(7) It is possible to have a CPT2 episode without muscle breakdown.  I don't fully understand this, but I guess it is the period between normal and rhabdo when the muscles hurt but still function.  In some ways I have been aware of this, but I never thought of the pre-rhabdo pain as caused by CPT2.  Now I know better.

(8) The Canadian healthcare system is a far better place to have a chronic illness than the US.  There is no fighting with an insurance company to convince them that it is better to test for a metabolic disorder and treat it, instead of waiting until one is hospitalized with it.  The doctors, nurses, and others seem to actually care about me.  Because the doctors are salaried, they can switch between research and clinical work easily, allowing their work with me to benefit other patients.  I can also contact them any time -- and they don't need to worry about spending time on the phone without being paid, because the pay structure doesn't make that a problem.

(9) I need to be far more proactive in managing CPT2 via diet.

I am thrilled that I have this resource two subway stops away from my home.

Wednesday, February 8, 2017

Released from the hospital -- and near immediate partial relapse

Here is a tip:  Getting released from the hospital often means coming home to a bunch of overdue stuff.  Bills, overdue work assignments, fights with insurance companies over the hospitalization, friends, co-workers, and family members who subconsciously resent your unplanned and undesired hospital "vacation" -- there are plenty of stress points.

The problem with stress points is that stress is a primary trigger for a CPT2 episode.  Coming out of the hospital, you will likely be exhausted, sleep deprived (unless you can sleep through blood draws), stressed out, and a bit disoriented.  Exhaustion and sleep deprivation are also triggers.

So with that as prologue, I came home yesterday, and by mid-morning, I was feeling moderate muscle pain.  My urine was back to being cloudy.  I took that as a cue to pretend I'm still in the hospital and cut out as many trigger events as I could.  I'm going to try to get my CK checked tomorrow and hopefully today's episode was just a minor blip.  I really don't want to be readmitted.