Thursday, September 8, 2022

British Columbia could use some Canadian-style healthcare

My Years in the USA Advocating for Canadian-Style Healthcare

I was born in Montreal, but my parents moved to California when I was 9. My dad was a doctor, and frequently argued with the other doctors in his practice group about how in Canada, he was free to practice medicine while in the US, his hands were tied by the byzantine private insurance system. Sure, he'd say, he's earning more money, but he'd much rather live with the Canadian system because it would let him worry about the health of his patients as his first and only priority. There would be no arguments about whether a procedure was too expensive for an insurance company's shareholders.

In my early 20s, I was diagnosed with a fatty acid oxidation disorder.

For years, I kept my invisible illness (called CPT2 Deficiency) a secret from my doctors. It wasn't that I was embarrassed by it. Instead, it was a deep worry that if my medical records were festooned with references to CPT2, fatty acid oxidation disorders and rhabdomyolysis, I would become uninsurable under the United States' "if you're uninsured, prepare for bankruptcy, death, or both if you get sick" medical system. To protect my kids' ability to get insurance, I never had them tested for CPT2 until after I moved to Canada. Instead, I would check their urine color (and when they were older, asked them to check their own) for signs of rhabdomyolysis. My father's observation that the US medical system was not compatible with obtaining the best health outcomes seemed proven right by my experiences with CPT2.

In 2008, my former classmate (a year ahead of me in graduate school, so while I knew a lot about him, I didn't personally know him) Barack Obama started climbing in the polls and then accumulating lots of convention delegates. His competitor in the primary was Hillary Clinton. While both promised to implement substantial medical reform, I wasn't ready to disclose my illness for fear that McCain would win or that medical reform wouldn't pass Congress. I joined the Obama campaign and worked on his Legal/Voter Protection Team in the primaries. I spent a week in both Pennsylvania and Texas as part of that team.

As the primaries drew to a close, I was given the opportunity to work as a Regional Field Organizer for the general election campaign. I started out managing Fresno County, but eventually ended up in charge of the campaign's efforts in many neighboring areas.  There were plenty of reasons to want Senator Obama to become President Obama. There were reasons that were high priority for me even though they didn't directly impact me, such as diversifying the federal judiciary and enforcing federal civil rights laws.

The most powerful incentive for me to work on the campaign, however, was his promise to treat medical care as a basic human right. He would get rid of the ability of insurance companies to refuse coverage because you were born with a problem (that's me); he would require mental health care to be covered in the same way as physical health care; he would, I believed, work hard to decouple poverty and bad health outcomes. I took months off of work to campaign for him.

Part of the training for Regional Field Organizers was to attend "Camp Obama", a multi-day gathering where we learned how to reach voters. A key lesson was to make policy personal. Rather than talking about a universal healthcare system in the abstract, we were told to put it in context by sharing how it impacts us personally. I was inspired and dedicated enough to decide that it was time to "come out" as having an invisible illness. As it turned out, every time I shared my fears about testing my kids for CPT2 deficiency, I moved the needle a lot. Personal stories matter in formulating policy and shaping elections.

At this point, I was "all in". If medical reform didn't pass, I would have a record of a "preexisting condition" -- a condition that, while not necessarily medically fatal, could be fatal to my ability to get medical insurance if I lost my existing coverage.

The Affordable Care Act became law and has survived more than a decade of efforts to dismantle and undermine it.

When the 2016 primary elections delivered Clinton and Trump as nominees, I started telling people I would move to Canada if Trump won. I felt he would be the beginning of the end of the US as I knew it, and I worried deeply that Trump would sabotage the Affordable Care Act, or worse yet, sign legislation to overturn it wholesale. 

I moved to Canada weeks after Trump was sworn in.

Canada, Before COVID

When I first moved to Vancouver, I heard rumblings about issues with finding a family doctor, but I had no trouble finding one just a few hundred meters from my front door. I had nothing but good experiences with the system. I was aware that some people were complaining about long waits for procedures, but it seemed to me that the long waits primarily impacted people who didn't have a critically time-sensitive problem (not that long waits are ok in any case, but triaging procedures explained a lot of it to me).

As some people have painfully learned, British Columbia's Medical Services Plan (MSP) doesn't start until start of the fourth calendar month after one moves to BC. I moved in February 2017, which meant that I was covered only by travel insurance until May 1, at which point government care kicked in. Of course, because things just sometimes work that way, I was hospitalized at the end of April -- and my travel insurance refused to cover it, because I had previously been hospitalized for a CPT2 episode. In other words, my long-feared lack of insurability due to the pre-existing condition of CPT2 was finally realized during the last two weeks I ever had to fear it.

My experience in the hospital was as good as I could have expected, and much better than it was during my prior two CPT2 hospitalizations in the United States. Other than a stubborn refusal to give IV opiates as part of the effort to avoid creating new addicts in a city with a horrible overdose problem, which I understood but disagreed with as a blanket one-size-fits-all policy, I had no complaints.

I was seen numerous times a day by physicians. I loved the opportunity to talk to a variety of interns and residents about what it is like to have a fatty acid oxidation disorder (the more doctors who know about it, the better others with FAODs will fare). The nursing staff was unbelievably great. Even the food was acceptable (though it would never be mistaken for gourmet food). When they learned I was paying out of pocket for the care, my primary doctor came in and told me that if I was staying as part of government-provided health care he would keep me for a few more days, but he gave me the option of caring for myself at home to save money. As this was literally my life and health we were talking about, I ended up deciding to just eat the cost of the extra days in the hospital.

Right before COVID hit, I was hospitalized in Canada a second time (my fourth overall), this time at Vancouver General Hospital. By then, I had been accepted as a patient of the Vancouver General Hospital Adult Metabolic Diseases Clinic. In addition to a parade of physicians and nurses seeing me daily, the clinic sent a doctor every day to make sure I was doing well and that the doctors treating me as an inpatient had all of the information they needed to help me heal.

It was at the end of that second hospitalization that I realized that I had been absolutely right to fight for Canadian style healthcare in the United States. I was treated with care, dignity, and a complete disregard for whether I had money. I stayed as an inpatient for a full week, and the only time finances ever came up was when they took my medical services plan number when I first got to the hospital. There were no bills, no copays, no fights to keep me hospitalized when an insurance company decided it was just too expensive. All that mattered was my health. I'd been vindicated in trying to change US healthcare to be more Canadian -- I thought. 

Canada, 2020 to Now

 I personally witnessed a lot of abuse of medical personnel during the first two years of COVID. A tiny woman (maybe 5 feet tall and 90 pounds if I had to guess) working the front door at VGH's ER asked a huge man (at least 6 feet tall and well over 200 pounds) to please follow the mandatory masking policy. Without hesitation or humanity, he got in her face and screamed -- in the process demonstrating his lung capacity and breathing were undiminished -- that he couldn't wear a mask because his lungs aren't strong enough. Then he called her the N-word (although she looked for all the world like her ancestors were from India, I guess when a racist is searching for a way to insult somebody, all skin tones darker than chalk look alike). He was escorted out by security.

This was a story I saw repeated nearly every time I sought medical care. People intentionally pulling their masks under their noses while in a waiting room (with people, like me, who were extremely clinically vulnerable) and yelling at staff when there were politely asked to cover their noses.

By this point, my family doctor was Dr. Sangeetha Nadarajah. I've had some pretty great doctors in my lifetime, but Dr. Nadarajah was in an entirely different category. She deeply cared that I stay healthy. She understood how CPT2 works by the second time I saw her (so she did the research on her own time -- something no other non-neuromuscular specialist had ever done). She didn't just guide me on my journey as a CPT2 patient, but made me feel she was walking that journey with me. She was caring, but in a way that grew from deep empathy -- a bedside approach that one can be born with, but that can't be taught. She is somebody I consider a mensch (I'm Jewish and being a mensch is a good thing). 

In 2021, I started hearing stories about doctors (many in family practice) quitting because of the stress of dealing with COVID. Not treating the disease. Not helping those with long COVID. Not advising on avoiding COVID. Instead, they were quitting because of the hostility and anger they constantly had to deal with from people wanting vaccine and mask exemptions for no reason other than misinformation about vaccine risks and mask efficacy; because of the dangers of simply being identified as a physician by COVID deniers; because of the inability to keep themselves safe around the worst 5% of Canadians who treated COVID as a fabricated inconvenience instead of the pathogen it is.

In 2022, Dr. Nadarajah told me she was quitting. I immediately felt a combination of sadness and terror. The best doctor I'd ever had no longer felt she could practice family medicine in British Columbia. In a province where one in four people are searching, fruitlessly, for a family doctor, I felt the terror of joining their ranks. Worse, I had an incurable genetic disease that could only be properly managed with a family doctor on board.

Shortly thereafter, Dr. Nadarajah said that she had discussed my case with the doctor in charge of the clinic I had attended, and that he had agreed to take on some of her very high risk patients, me included. After effusively thanking her, I finally let myself exhale. It was going  to be ok. I would still have a  family doctor necessary to manage my health.

If only the story had ended there, but it didn't. Shortly after being told that the new doctor would be my family doctor, he sent a letter saying that he was closing his practice. The letter included this passage:

I was truly confused because the British Columbia College of Physicians and Surgeons has a practice standard that says that when a physician "decides to end the patient-registrant [doctor] relationship after thoughtful consideration, they must ... where possible, assist the patient in finding another registrant [doctor] or clinic where care can be transferred" and "ensure medical services are provided in the interim period..."  I'm not licensed to practice law in British Columbia, so it is entirely possible that I've read the standards as requiring things they do not require, but I was nonetheless shocked that a patient with a serious metabolic condition was terminated by email, and that the efforts to find me a new family doctor were limited to providing a list of websites and mentioning that maybe a friend would help me.

This is when I decided I needed to take out an ad. The math was simple: Without a family doctor, my risk of an early death was unacceptably high. I have three daughters -- 21, 15 and 13 -- and my death or a serious disability would be a disaster for them. With the horrible shortage of family doctors, I was aware that I needed a doctor capable of understanding my condition and properly triaging patient needs. With the existing clinics throughout Vancouver's Lower Mainland bursting at the seams with patients, I knew I'd need a clinic near where I live so that I could jump on any cancellations and avoid travel while suffering an episode. After all, if a 90 minute drive to Delta (near the US border) was followed by a 5 hour wait and a 90 minute drive back, that's all day. Worse, I had already been hospitalized overnight when the anxiety of waiting for eight hours in the VGH waiting room triggered a muscle episode (I was waiting to be seen for lung function issues, and those resolved on their own while I was waiting -- but not before rhabdomyolysis from the stress had set in). 

If I couldn't find a doctor within walking (or 5 minute Uber) distance, I would need a doctor who would not have a long wait once I arrived. I have a Nexus card, so I can normally cross the Canada/US border in ten minutes or less. I decided that finding a family doctor just south of the border would be the best course of action if I could not find one locally. At least I'd know I could get treated without waiting for hours among the sick and coughing in a waiting room.  The cost of US health insurance would run around $20,000 per year. The cost of cash-pay for services would run $5,000 or so per year (based on a bunch of assumptions -- obviously there is a lot of guesswork involved in calculating how often you'll see a doctor).

I decided that the financially and medically responsible thing was to run an ad and offer $5,000 on a one time basis to anybody who could hook me up with a local family doctor who could meet my requirements. Of course, it is illegal for a physician in BC to take money to see a patient who is covered by the Medical Services Plan, so I knew that no doctor could take the money. However, I figured that it might be enough to get patients of good and available family doctors to reach out to me with leads.  This is the ad that ran in the local papers (I cut it off right before the email address and phone number):

Right after placing the ad (and before she saw it) Dr. Nadarajah reached out to me to ask if I was doing ok. I told her about my situation, and she was not at all happy to see one of her high risk former patients without a family doctor.  She said she would make some calls and see if she could find me a family doctor I could walk to (or, during an episode, quickly Uber to). Bear in mind that she had already met her obligation to transfer my care to another family doctor, so this was going above and beyond her professional obligations. She was also careful to encourage me to keep looking on my own in case she couldn't find a place to place me.

I thought I'd get a few calls, but my years of living in the United States left me unprepared for the kindness that beats in the hearts of Canadians. I was (and still am) overwhelmed by the number of leads. Moreover, the only people who mentioned the reward mentioned it as part of a phrase communicating "I'm not interested in the money, but here's a lead..." or "I'm not interested in the money, but here's a charity you could consider". Within a day, I had around 100 leads.

I'm not going to link to my appearances in the media, but my story was picked up by the CBC and CTV, and reached a substantial audience through social media as well. Bear in mind that stress is one of the triggers for a muscle episode, and I found myself starting to stress because I wanted to personally thank everybody who helped -- whether they had a good referral or not. However, as the number of responses kept growing, I knew I didn't have the bandwidth to do that.

I lined up several of the best leads and was starting to reach out when Dr. Nadarajah reached out to tell me that she was able to find a doctor she trusts to take me as a family patient. This was the figurative life preserver I needed, and she was able to throw it to me. The only caveat was that the doctor she referred me to had a huge waiting list, and he would need to meet with me before deciding that my condition was serious enough to merit taking me on immediately.

I contacted the doctor that Dr. Nadarajah referred me to. Within a week, he met with me, went over my condition in detail, and agreed to take me on as a patient. I felt enormous gratitude and relief - even as I worry about the more than a million BC residents unable to fix their lack of family/primary medical care.

Guilt, Privilege, and Fixing Things

This is probably the right spot to air my guilt. I know in my heart that this entire situation was caused by the government of British Columbia letting the healthcare system fail. Whether the collapse started under the BC Liberals (ironically named, as it is the party representing conservative positions) or the current BC NDP party (the party representing liberal positions) has been the subject of a lot of debate, but ultimately it doesn't matter. The buck stops with the current party in power, and with a majority government they could set about fixing things. Instead of fixing things, though, they got busy complaining that the federal government wasn't contributing enough money to the provinces (in Canada, the provinces are in charge of medical care and the federal government provides subsidies). When talking with the media, I noted that finger pointing isn't solving the problem, and that I wished that BC would raise taxes on me and those in a like position and use the revenue to fix the medical system, perhaps seeking reimbursement from the federal government later. After all, a period when patients are dying for lack of care is exactly the wrong time to let affixing blame get in the way of fixing the problem. It turns out that they wouldn't even need to raise taxes, as days after the interviews BC announced it had a very healthy annual budget surplus, well in excess of one billion dollars.

As the previous paragraph indicates, I'm well aware that I'm not the cause of this problem. However, emotionally I can't get over the fact that there are others (around a quarter of people in BC) who are looking for a family doctor but unable to find one. And I literally jumped the queue because of my medical condition. Were it not for my responsibility to my children, I would find that difficult to live with. However, I think most parents would do the same to make sure they were around for their kids.

The true guilt, of course, would have surfaced had my privileged position (having enough money to offer the large reward) made the difference. While knocking on doors and calling voters for the Obama campaign, I never thought that a progressive government (like the BC NDP is supposed to be) would allow single payer healthcare to languish. It never occurred to me that prohibiting free market provision of medical services in favor of a government pays system meant that an incompetent or indifferent government could endanger the health of everybody. It definitely never occurred to me that I would need to offer a reward to find a family doctor.

The bottom line is that the Canadian system was never supposed to work like this. There was supposed to be enough money to pay doctors. There was supposed to be a large enough number of doctors to treat the patients. The egos of those in provincial leadership weren't supposed to lead them to use growing anger over healthcare failures as a lever to get money from the federal government -- rather than simply fixing the problems.

If I was in charge, I would take the following steps:

1. Identify jurisdictions with physician licensing standards similar to British Columbia, and allow licensed physicians from those jurisdictions to provide telemedicine services to residents of BC.

2. Welcome foreign-trained physicians to qualify to practice in BC, providing each with an advocate who will help them navigate everything from immigration to housing to training and licensing.

3. Raise the pay for family practice physicians to be competitive with the pay received by specialists so that the financial incentive to avoid family practice is eliminated.

4. Prohibit family physicians from charging people without government healthcare more than they collect from the government for seeing people with government healthcare. This alone would likely alleviate much of the crisis. Family doctors make far more money on a per capita basis seeing foreign patients than they do seeing BC residents, creating an incentive where non-residents can easily be seen by the same family doctors who claim their practice is too full to take on new BC residents as patients or walk-ins. This is a much bigger issue than most understand, and there are even companies that arrange for international travel to BC for private-pay medical care. 

5. Include mental health professionals, such as therapists and psychologists, within the government paid programs. From the overdose crisis to medical service providers such as nurses quitting from the stress of working with COVID patients, it is clear that the cost of providing mental health care is far less than the cost of not providing it.

Was I Wrong in 2008?

The failure of the BC healthcare system and the extraordinary measures it forced me to take have made me reflect hard on whether I was wrong in advocating for a Canadian-style healthcare system in the US during the 2008 election. Ultimately, I do not think I was wrong as a matter of public policy. It is unacceptable to treat medical care as anything other than a basic human right, and a system that conditions medical care on wealth was an utter abomination. The Canadian system, warts and all, is better than a cruel system where poverty plus illness equals death and where insurance companies have a bigger say in healthcare than physicians do.

However, calling a medical system "better than the US healthcare system was in 2008" is faint praise indeed. Furthermore, it is far from clear that the current system in BC is better than the system in place in the states that accepted Medicare funding under the US Affordable Care Act. Yes, the paperwork is crazy-making. The co-pays and deductibles are inhumane. But at least the poor get free healthcare, the middle class gets a large subsidy, and the wealthy, as usual, can take good care of themselves -- and there are enough doctors to provide the care required.

Medicare should be the pride of British Columbia, not a festering and deadly embarrassment.

Everyday Canadians need to insist that their elected representatives show the same level of love and care for their neighbors that everyday Canadians show. That means, if nothing else, making sure that the health of BC residents is a top financial and legislative priority. If elected officials can't treat their neighbors and constituents as they would wish to be treated, they have no business calling themselves Canadians -- and they certainly have no business holding elected office in British Columbia.

The family doctor crisis is an easily fixed problem being cast as an intractable one by politicians who can't be bothered to (or who are afraid to) take the steps necessary to fix it.

Monday, June 20, 2022

COVID didn't kill me

 Ever since the early reports about a new virus in China started coming in during late 2019, I worried about how the virus would interact with CPT2 deficiency. My worries were magnified greatly when I heard two anecdotal reports from others with CPT2 that they had asymptomatic COVID yet ended up hospitalized because viral infections can trigger an episode

I tested positive for COVID on a rapid test on January 18, 2022. At that point I'd had a total of four vaccinations (the regular 2 mRNA vaccines and 2 boosters).

I was barely symptomatic. I had a headache and sniffles. However, because of the known risk of any viral infection to somebody with CPT2 deficiency, I stayed off my feet for a week and drank an ungodly amount of Gatorade.

I recovered quickly, and the only thing that has changed is that I may be slightly more tired. Frankly I think the fatigue is due to things other than long COVID, but who knows.

I'm so grateful I was able to get vaccinated and boosted. It turned what could have been a CPT2 disaster into more of an annoyance than anything else.


Note that there are many genes involved in CPT2 production. I have two different kinds of mutation, one on each allele. If you have CPT2, it may well involve different mutations, so my experience does not predict your risk of a CPT2 issue with COVID.


Added 22-06-22: This raises the question about how to deal with COVID going forward. My risk level is pretty much "same as others in my age range" (now that I know it didn't negatively interact with my version of CPT2). I asked myself "what even are you waiting for in order to relax your restrictions?"  I've now had 5 immune events (vaccines + infection), and as far as I know there isn't anything on the way that will be able to outpace the virus' ability to mutate into a version that has pretty good immune escape. I worry a lot about long COVID, less so about acute COVID (because vaccines do mitigate the risk of death down to an acceptable level, and Paxlovid is there to fill in any gaps).

The bottom line is that I can't identify any change that will be a clean demarcation between "be super careful" and "back to normal".  The choice is thus between staying super careful forever and figuring out how I want to live for the next decades. If British Columbia took seriously the risks faced by immunocompromised people and those with chronic illness, I'd absolutely be part of that effort -- it is something I've been doing for years now. However, I don't know how much risk mitigation I'm providing others by being the only person in the grocery store wearing a mask.

Ultimately my conclusion is that (a) I've taken all of the steps I can to reduce my risk of serious injury or death; (b) everybody I'm in regular contact with is low risk (either via vaccination or other factors such as age); (c) given the piss-poor public health efforts in place in British Columbia where I live, any steps I take to protect others (primarily wearing a mask whenever I'm not home) probably only make a de minimis change - if any - in the risk level of those at high risk. 

The logical answer - I think - is that I can relax my protocols significantly, to a level where I'd be ok with those staying in place all of my life. Basically, mask when indoors for more than a few minutes in proximity to somebody at high risk, isolate for 2 weeks if I test positive, and respect any request to put on a mask. 

Wednesday, September 8, 2021

Invisible Illnesses and Covid-19

 It is terrifying to have an invisible illness during Covid-19. Mine is a fatty acid oxidation disorder (CPT2). I know of somebody with CPT2 who ended up on dialysis after catching Covid-19. His only Covid-19 symptom was a high fever (vaccinated, so asymptomatic other than the fever), but unfortunately high fevers and viral infections are well known and powerful triggers for CPT2 muscle breakdown (and loose muscle fibers are metabolized in the kidneys, up to the point of kidney failure).

The thing about CPT2 is that we look totally normal. Unless I'm having an episode you can't tell I'm a mutant no matter how hard you look. This means that when I ask somebody to please pull their mask up to cover their nose, I'm asking because catching Covid-19 means I'm almost guaranteed a trip to the hospital. Apart from my own suffering and injury, that trip to the hospital means that some other person who needs a hospital bed will be denied one. I'm not being paranoid. I'm not being selfish. I'm taking a reasonable precaution from a place of very high vulerability.

If I had a visible illness like ALS, I'd be far less likely to be yelled at or even violently attacked for simply asking somebody to follow the rules. But Fatty Acid Oxidation Disorders are not visible.

We need more awareness of invisible illnesses. I've been made to wait as long as 8 hours in the ER because I'm not visibly bleeding -- but when they finally see me and do a blood test for CK, the doctor inevitably says something like "wow, you shouldn't have been kept waiting, this is an emergency" and I get admitted. Unless you're bleeding or claiming heart issues, you are pretty much at the bottom of the triage list. This needs to change. Of course, for my fellow CPT2 patients, a quick tip: I've printed a card explaining what CPT2 is and carry it on me; I give it to the triage nurse and add "I'm really worried because I'm having massive muscle breakdown and my heart is a muscle". That at least gets me a quick blood draw, and during an episode, the blood draw results always bump me up to the top of the triage list.

Saturday, December 14, 2019

Anything that slows digestion is a terrible idea for CPT2 patients

I went to my cardiologist last week and he said that thanks to my PCSK9 inhibitor, I had fantastic cholesterol numbers.  He was concerned -- as am I -- with how difficult it is to lose weight.  He also let me know that studies are starting to show that Trulicity is highly effective at reducing cardiovascular risk, with the side benefit of causing weight loss.  So he prescribed 150 ml Trulicity and I took my first shot Monday night.  I then had some Indian food leftovers and went to bed.

Just stop reading for now if you're eating or easily grossed out.  Because what follows sucks.  And is not at all compatible with avoiding nausea.

On Tuesday I was feeling mostly ok until mid-day, when I started to burp with a sulphur smell. That was super-weird and had some nausea, but I figured maybe I ate something that was causing it.  By Tuesday night I was feeling sick but at that point I'd only eaten an english muffin for breakfast and was worried that my carb intake was too low, putting me at risk of a CPT2 episode.  I used cannabis as an antiemetic (thank you, Canada, for making that easy to do) and I was able to eat two pieces of bread.

When I woke up on Wednesday morning, I was feeling very sick and very full.  A side effect of Trulicity is that it is capable of slowing digestion.  What I didn't know is that it would effectively stop my digestion.  I couldn't eat more because there was literally no place for it to go.  I tried to stay hydrated and had sports drinks with glucose to stave off muscle breakdown, but by Wednesday evening I was feeling pretty weak.  On top of that, I tried using cannabis as an antiemetic Wednesday night but I still couldn't do more than slowly sip liquids.  Nothing was happening in my digestive system -- nothing but liquid could go in, and nothing was coming out (nothing had come out since Tuesday morning).

On Thursday I mostly stayed in bed and felt miserable (though I was able to get work done thanks to my laptop), and I was hoping that by staying largely immobile I would avoid triggering muscle breakdown.  By midday, though, I was in terrible stomach pain (which I would have just lived with) but I was also feeling muscle breakdown (which is an immediate trip to the hospital).  I packed a hospital bag, mostly in a daze, and went to Vancouver General. I have large, 4 x 6 cards printed with my ER protocol on it and they have notes in their system about how to handle me, so I was in the back having a D10 drip in under an hour.  This probably kept me from being hospitalized overnight.  Another hour or two without IV sugars and I would have been in real trouble.  They also administered carnitine and an antiemetic.  You'd think the antiemetic was a good idea, because I was able to drink juice and eat a total of four small crackers, but it created the false impression that I was on the mend.

My CK results came back and they were only about double the top end of normal.  For people with regular muscle metabolism, that might be a problem, but for a CPT2 patient, anything under around 3,000 is pretty welcome news.  I was actually under 500.  The rest of my bloodwork showed inflammatory markers and indications of a viral infection.

Going through my CPT2 triggers,  I had quite a few.  The nausea meant that I wasn't getting enough carbs and that I wasn't able to get enough sleep -- two triggers right there. A viral infection is a trigger (even through I was outwardly asymptomatic for a virus, though I got a slightly sore throat a day later).  I am in the middle of a few work emergencies, so I also had stress, yet another trigger. And to top it off, keeping the room cold felt good for my nausea, but temperatures extremes are yet another trigger.  Normally it takes me at least three triggers to become symptomatic (the exception is not eating enough combined with exercise, which are the two triggers that are enough in combination).  But I was now looking at five triggers, and one of them was the big one -- not enough carbs.  Making it even worse, it wasn't a trigger I could fix on my own because the Trulicity had stopped my ability to eat and even limited my liquid intake.

Back at the hospital, though, my second CK came in an it wasn't climbing.  Because the hospital has shared rooms and the stress and insomnia caused by sharing a room with a really sick person has, in the past, made me actually get worse in the hospital, the doctors told me it was my choice -- I could go home or get admitted (because I knew my body and how it responds better than they did). After going over the pros and cons, I decided that going home would be the right move.  So I went home Thursday night.  While the antiemetic was still highly effective, I was able to do work that night (so less catching up to do later).

On Friday I had a work meeting that I just couldn't cancel or move (can't elaborate -- when you work with high tech and are a lawyer, everything has a non-disclosure agreement or privilege, sorry).  After that meeting, I was hoping that I'd eliminated enough of the Trulicity that I could try something more substantial than sports drinks.  I had a bowl of matzoh ball soup.  Boy, did I regret doing that later.

By Friday mid-afternoon, I was experiencing severe stomach pain and an incredible amount of reflux (despite being on Losec, or as Americans know it, Prilosec).  I couldn't eat.  The sulphur burp thing was making me crazy.  My muscles really hurt, but I thought that it was soreness from the earlier breakdown rather than renewed breakdown.  I got into bed, cancelled my evening work thing, and this atheist, non-practicing, culturally Jewish man suddenly found found himself asking G-d to please start my digestion again and make me ok.

I kept thinking I was going to throw up, but never quite got there until early evening. I went to the bathroom and had dry heaves for maybe 30 minutes.  But the weird thing about it was that when you throw up, you normally feel something in your stomach.  All I felt was pain between my solar plexus and my throat.  I went back to bed and realized that I'd been taking my medication all week but wasn't digesting anything.  I was -- and still am -- terrified that all my meds will get digested simultaneously once my digestion starts back up.  I don't think that's the case, because I have BPH and the Flomax was clearly still working, so at least one pill was being at least partially digested every day.  But it is something I wish I'd through to ask about while I was at the hospital.  Had I thought about it, it would probably have switched my choice from "go home" to "get admitted".  Hopefully I never again have the circumstances necessary to put that lesson to use.

I thought I was literally unable to throw up because the Trulicity had stopped my digestive system well enough that even putting it in reverse wasn't possible.  If that's true, then the Trulicity was finally wearing off, because by around 9 or 10 pm, I went to the bathroom with more nausea.  This time, however, it was literally the most violent vomiting I'd ever experienced.  My entire body seized up in pain.  Making this a weirdly iterative experience, the vomit itself smelled a little like, well, ok this is gross but feces.  "Fecal vomiting occurs when the bowel is obstructed for some reason, and intestinal contents cannot move normally".  I need to thank my late father for giving me the courage to share something this embarrassing so that my experience could help others (he experienced it while battling cancer). This is now a red line for me: If I experience it again, it means that there could be a blockage and I have to go in. I'm pretty sure this was a quasi-blockage caused by the Trulicity, but if it recurs I can't take the risk, just in case.

In case you were wondering why I mentioned the Indian food leftovers I'd had on Tuesday, here it is:  I threw up a lot.  First came the matzoh balls, then the Indian food.  It had been sitting there for 72 hours.  My theory is that it was being held, undigested, at room temperature for 72 hours, allowing bacteria to grow.  Basically, food poisoning that  resulted from the unsafe food handling practice of leaving food at body temperature for 3 days -- but a practice that was caused by my own body's failure to clear any of it from my stomach.  I could be wrong as to that piece, but it doesn't matter to the outcome.  It was the most pain I'd ever felt from throwing up.  I was positive I was going to aspirate the vomit, which added an element of terror to the whole experience.

After several more sessions in the bathroom, I got into bed around midnight.  But after 30 minutes of trying to sleep, I couldn't.  Every time I moved my body, I felt stuff gurgling in my stomach and felt like throwing up.  The hospital's antiemetic had apparently been cleared by my body.  So I went to the next best (and much easier) thing:  More weed.  After hazing about 1/4 gram of cannabis, I felt my eyes grow heavy and I was able to move around on the bed without feeling I was about to vomit.

When I awoke this morning (Saturday, since I expect I may add more to this if something else happens from this episode), I felt somewhat better, but still knew that my digestion wasn't working.  After all, I hadn't voided my bowels in 96 hours, and to put it lightly, there was nothing in the chamber.  Given that last night I threw up food I had on Monday, it isn't surprising that there was nothing to void.

As a side note, this entire time I was monitoring the colour of my urine.  This isn't optional for CPT2 patients.  If it is dark you see, you need an IV (hey, just made that up!).  It got slightly dark from dehydration, but that quickly reversed when I had water.  So I haven't (yet) gone back to the hospital.

As I sit here writing this, I'm feeling pretty sick.  I still can't eat solid food.  I can't even get myself to drink juice.  Just sugar water (like sports drinks).  And just enough to keep my muscles from breaking down.

The half-life of Trulicity is about 5 days, so I might have a few more days of misery.  But I'm hoping that the worst is now behind me.  I have an exciting new project I'm working on with a really smart AI programmer here and I've got two new patent applications to file, and those are things I enjoy doing.  So I'm hoping that I feel well enough to function by tomorrow morning (I'd say tonight, but the combination of living in a fact-based reality and the fact that I still can't even drink juice tell me it isn't about to happen).

So, what are the lessons?  First, we need to proactively ask about any digestive effects of any medication we take.  If I was in charge of the FDA or Health Canada, I'd list Trulicity as contraindicated for any FOD/CPT2 patient.  But CPT2 is so rare that of course it isn't listed as something that makes Trulicity contraindicated.  However, I have a new rule:  Ask whether any new medication, or new dose of existing medication, is capable of causing any impact on the speed of digestion or the ability to absorb carbohydrates.  Anything that inhibits my ability to get sugar into my blood needs to have benefits that are absolutely necessary to survival.  Otherwise, it is just too great a risk.

I can't say that Trulicity is a bad idea for everybody with CPT2. It does have benefits, and maybe the effect it has on me is much stronger than on the average person.  Maybe.  But I can say that before taking it, a long discussion with a doctor well versed in fatty acid oxidation disorders is strongly advised.  Take it from the guy who hasn't digested any solid food or opaque liquid in four days and therefore got so tired writing this that he's heading back to bed.

After all that gross stuff, here is the mandatory unicorn chaser:
(image by wikimedia Author
User:Yamavu, User:Hashar & User:Kalki)

[Update 1]  It is Sunday morning, and I am finally starting to feel more normal. I started feeling new muscle pain and knew that sports drinks alone weren't going to stave off a CPT2 episode forever.  I had a little bit of pasta last night and despite some unsettling stomach pain, it didn't come back up.  As of this morning, it feels like it has been digested.  I'm going to have more pasta in a few minutes and if that stays down, by tonight I'll be able to eat more.  I'm optimistic that I'm past the scary part, but as my head clears I'm all the more surprised that I didn't realize the risks associated with slowing my digestion would be exponentially more dangerous than for somebody with normal fatty acid metabolism.

[Update 2] It is Sunday afternoon (though around 4:45 pm in Vancouver, in December, it sure looks like night outside). I was able to have Annie's brand shells and cheese (exactly 15% of calories from fat, so I'm allowed to eat that!).  I ate it for lunch and I feel ok.  It isn't digesting at the normal speed (I'm definitely not close to hungry), but it is digesting.  I know it is because the muscle pain has finally subsided and there are no signs of muscle fibers in my urine (I know, gross, but dark urine is a give-away that you need to get looked at by a doctor).  I'm pretty hopeful that I'll be ok in the morning.

[Update 3] It is Monday late morning and I had a pretty normal amount of food yesterday (bland, but normal). When I woke up I was slightly nauseous but it seems to be clearing up. I'm not hungry at all today, so it has been sports drinks this morning for CPT2 safety.   The elimination half-life for Trulicity appears to be 5 days.  I'm a little over 6-1/2 days from taking it.  So I'm not surprised I'm still slightly symptomatic/nauseous.  Yet another lesson:  Check half life of a medication before you take it at that dose for the first time. 

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.