Tag Archives: Trikafta

A Definitive Ranking of Prescription Drugs

This is most of what I need to travel, minus a cooler bag and a 22 pound vest.

For reasons simultaneously mundane and complicated, I find myself traveling a lot. The distance affords me both time for reflection and a tremendous amount of opportunities to forget the various pills and accessories that keep me alive. Although I have a 94.2% hit rate, that ain’t 100%, so here’s a list of the things I could forget, tiered by how much it sucks when I forget them

Tier 1 – Over the Counter

Pepcid (acid reducer), Claritin (allergies), Flonase (allergies and nasal polyps), Vitamins

The penalty for forgetting any of these is taking $10 to Wal-Mart and buying enough to get through the weekend. I have them listed from most important to least important above, with Pepcid topping the list because it’s actually a drug one of my other drugs takes. Also, I listed them with brand names to save everyone some Googling, but I’ll be goddamned if I’m paying double for top of the line over the counter drugs.

I have to take special vitamins as well, which technically have to be mail ordered, but I can get by for a few days on some whack ass Centrums if I really have to.

Tier 2 – Quality of Life Prescriptions

Albuterol (fast acting bronchodilator), Stiolto (long acting bronchodilator), Zithromax (maintenance antibiotic), Losartan/Hydrochlorothiazide (blood pressure reducer), Inhaled Saline Solution (mucus seasoner)

I can go a couple of days without these, but depending on the weather and my health, they may not be very fun days. Albuterol is the opening act of my daily treatments, both in that it’s the one I do first and it opens up my airways. Stiolto is also a bronchodilator, but acts long term as opposed to the quick hit of the albuterol. As such, it takes a couple of days without Stiolto before I start to think “oh yeah, I guess that was doing something”. Losartan/Hydrochlorothiazide is for my blood pressure. I haven’t missed it yet, but I imagine it’s not pleasant. Zithromax is a prescription I imagine a lot of you out there have sampled, but I take it every Monday/Wednesday/Friday to either inhibit bacteria growth or make superbugs, I don’t know. Either way, they keep giving ‘em and I keep taking ‘em. The saline solution goes in a nebulizer to salt up my mucus for easy removal. I can miss about a day of that before things start to feel a little tight.

With the exception of the Stiolto, I could afford to fill these prescriptions without insurance, though I wouldn’t be happy about it.

Tier 3 – Accessories

Monarch Vest and Power Supply (mucus shaker), Nebulizer, Air Compressor

The Monarch Vest is pretty much a $10,000 Brookstone massager. It’s supposed to shake my bronchial tree and loosen up the mucus so I can cough it out. It works fine, though we all pretend it works great because it’s more convenient than the previous version of The Vest. I rarely forget the vest, but I have forgotten the proprietary power supply. It has a battery, but I have to use it twice a day for 30 minutes at a time and the battery will only last about 3 and a half sessions. After that it’s just a 22 pound fashion statement. Luckily, it’s function can be replicated by getting someone to drum on my ribcage for a little bit.

The nebulizer and air compressor are necessary for both the saline I talked about above and another important inhaled drug that’s coming up. You’d think one would be able to stroll up to the drug store and purchase either one of these on a whim, but it’s actually surprisingly complicated, as both need a prescription. There are some websites out there that will sell you both on the honor system, but I’m not going to out any of them here. But alas, even express delivery isn’t super helpful if you need the parts right fucking now. In light of these developments, I own a backup compressor and many backup nebulizers.

Tier 4 – Diabetes Drugs and Supplies

Continuous Glucose Monitor (blood sugar meter), Novolog (short acting insulin), Basaglar (long acting insulin), Pen Needles (stabbing)

Novolog is a short acting insulin, so I need that to eat anything with carbs and basaglar is a long acting insulin, which essentially acts as a support for the Novolog. If I forget either one of these I can just stop eating carbs or try to cut a deal with a local diabetic, which is somehow easier than getting refills at a pharmacy I don’t usually use. The pen needles are used to get the insulin into my body. Apparently these are available over the counter now, which is nice, because while I can stretch the supply, I really do love a fresh needle.

The continuous glucose monitor holds the title of “thing I’m most likely to forget” because I have to change it every 10 days and it’s somehow always a surprise to me when it expires. I do have an over the counter finger prick meter I bought for those times when my calendar calculations are a little off, but if there’s one thing 10 straight days of pure blood glucose data has taught me, it’s that a one time reading is essentially useless. I’d love to sit here and tell you I’m willing to prick my finger every half hour to keep up, but it wouldn’t be long before I switched to an all Slim Jim diet or used the “well, I’m still upright” model of glucose monitoring. Neither comes recommended.

Tier 5 – The “Gotta Eat” Tier

ZenPep (digestive enzymes)

This one gets it’s own tier because without my digestive enzymes, eating is just the process of chewing things up so they’re easier to pass an hour later. That’s an exaggeration; it’s actually about 24 hours before things literally go south. It always seems these wings of wax are going to hold out, then the sun rises and I start blowing my hole out.

A few years back my stepsister got married in Vegas. It was a big family trip, so we had a big family meal at the airport Hooters the night before we left. I forgot my enzymes back in the hotel and didn’t feel like asking a van full of people to turn around. I figured I could just take them afterwards and be fine. And I was, right up until I was flop sweating on a 5 hour plane ride. I held out for about three hours, but when I finally did my business, it looked like someone shot the bowl with a paintball gun. For the next 4 days, everything I ate flew right out, regardless of enzyme consumption.

However, the “joy” of digestive enzymes is that enough people take them that after some negotiation, it’s possible to get someone to call in a few days worth to a pharmacy. It’s not cheap—they’re about $15 a pill and I take 6 per meal—but at least it’s possible. There are generic enzymes too, but those are just as good as taking nothing, even if the last time I had them they came in a blood red capsule that was stylish if not effective.

Also, these are the pills that I have to take Pepcid for, otherwise my stomach acid tears them up too fast.

Tier 6 – The Turn Around Tier

Pulmozyme (inhaled mucus thinner), Trikafta (protein modulator)

This is the turn around tier, because if I forget one of these, I’m just going back home.

Pulmozyme is an inhaled medication that splits the DNA strands of my mucus and thins it out a bit, making it all easier to bring up and pass. It has a special place in my heart because for years a one month supply was the most expensive thing I’d ever bought. The full story is in my book, but my state insurance lapsed, I couldn’t get pulmozyme, started coughing up blood after a week, and had to convince the pharmacist to let me charge it for $1,900. I paid $400 more than the insurance company because my negotiation skills are poor, I guess.

However, as nostalgic as I am for those blood spewing days of making it rain at the pharmacy, things have changed since then. For one, spending 4 figures on a drug is no longer a novel experience. Every January I get to start off the new year with a cool $1,400 copay before embarking on a journey of $4k in total out of pocket expenses. And my insurance is actually one of the better plans!

But there’s also a new top drug in town. Trikafta moves and unfolds the misplaced and messy cystic fibrosis transmembrane conductance regulator protein in my cells which helps water and salt move more normally through the cell. That means my mucus can no longer caulk a bathtub. I’m also at—or slightly above, depending on your perspective—a healthy weight for the first time in my adult life. It’s not a cure and it hasn’t lessened the number of prescriptions I’m on, but it has made CF a lot easier to manage.

Trikafta is a tremendous life changing achievement. I need to reiterate it’s not a cure as, in the words of Warren Zevon, “My Shit’s Fucked Up” and I’ve had CF long enough that a full cure is probably out of reach for me. But it has absolutely changed my life and the lives of many others for the better in a lot of ways. That makes it difficult to talk shit on it. Or at least it seems to make it difficult for most people. I’m a natural born hater. I was born to pass mucus and talk shit, and thanks to Trikafta, I’m all out of mucus.

Trikafta is fucking expensive. I’m sure it was expensive to develop, even with the $40 million that Vertex Pharmaceuticals (then known as Aurora Biosciences) got from the Cystic Fibrosis Foundation, but here is a study that estimates the manufacturing costs at less than $6,000. Looking at the last Vertex quarterly report, we can see they made a cool $915m in profit in the second quarter of the year alone. The current yearly price that shows up on my pharmacy receipts is $306,816, which makes me about .003% of their quarterly revenue before costs. Neat!

It used to be less, but they actually upped the list price after a couple of years on the market, which is quite a move if you can pull it off. I, of course, do not pay that price directly, my copays come out to about $720 a year for it. Funny enough, while I was writing this, I got a call from their patient assistance program and they were finally able to get my pharmacy to use a copay assistance card, which is something I’ve been trying to do for years. I have a lot of negative opinions about the impact of high drug prices, but at least Vertex’s customer service is tremendous. First time in my life I’ve felt like a high roller.

But that’s not the point of this. The point of this is that I forgot my Trikafta this weekend and had to turn my ass around. It comes in 7 day packs and after 3 years and a few missed doses, my 7 day cycle now starts on Saturday, so if I’m packing on a Friday night, I have to remember to throw the new pack in my medicine bag. Given that one day’s dose would be just a little less than if I bought 2 PS5s, I can’t solve this one by swiping the plastic. Of course, most pharmacies don’t stock it anyway and even if they did, it’s not like they’d break up a pack and sell me a couple of looseys. Or maybe they would, I don’t know. It seems like an expensive pain in the ass, so I just turned around.

Of the various wounds inflicted by the current prescription market, changing travel plans is a mere paper cut compared to the gaping wound of the 18 million Americans that can’t afford their prescribed medications but like a paper cut, it sure is annoying.

Things I Like

Speaking of genetic mutations, I loved the new Ninja Turtles movie. I was a big fan when I was younger, though it’s not necessarily something that’s stuck with me as I’ve gotten older, even if I did manage to buy all the Ninja Turtles as Universal Monsters figures that came out last year and I play more TMNT video games than the average 40 year old (maybe?). In any case, I think TMNT: Mutant Mayhem is about as good of a franchise update as I’ve ever seen, making some smart story adjustments and presenting a mostly fresh version of a thing that’s older than the intended audience.

The Old Amorphis Logo on a Bottle of Pills

Death Metal Improved My Lung Function

I don’t know how many of you have done a Pulmonary Function Test, so here’s a quick rundown: inhale as much air as you can then exhale it into a machine as hard, fast, and long as you can. Here’s some footage of Nick Nolte passing out while doing one:

The fun starts at 0:34

Nick has made a crucial mistake here and it’s one that I made for actual decades: force does not equal volume.

The problem with the Pulmonary Function Test is that it’s effort dependent. Whereas I can test my blood sugar with no more effort than pricking a finger or attaching a little sensor to my stomach every 10 days (shout out to the Dexcom G6–you blew a ton of my money on a Super Bowl ad and sometimes you come up with a ridiculous reading, but I’ll love you right up to the moment that they finally crack non-invasive glucose testing)–a PFT requires a lot from me: at least three of my hardest, longest breaths (repeatability matters!). 

If I were an athlete, I’d say it’s the difference between a drug test and running a sprint, in that one is passive and the other can be affected by the weather, my mood, or even the time of day. But I am not an athlete, so I will not say that.

I will liken the PFT to singing, but I’ll do it later. First, a small detour through medication.

In December 2019, I started Trikafta, the latest Cystic Fibrosis drug from Vertex Pharmaceuticals. Like Orkambi and Symdeko before it, Trikafta uses a combination of drugs to help out my busted Cystic Fibrosis Transmembrane Regulator (CFTR) protein by getting it to the surface of my cells and making it function in a more normal way with it gets there. Basically, more salt and water can pass through my cells and my mucus is less sticky. It works pretty well!

It also costs roughly $300,000 a year, but for now I will simply mention its price, partially charity funded development and incredible profit margin in passing so you know how I feel about high drug prices (against!).

The phenomenal shareholder value of Trikafta does shade my view of it slightly, though I have to say I feel better than I ever have. Some of that has to do with circumstances–working at home has done wonders for my health–and Trikafta hasn’t taken any medications off the table, leaving me with a current total of 12 daily medications, give or take a nasal spray. Plus I did pretty well on Orkambi and Symdeko before moving to Trikafta, so my lung function didn’t take a huge leap on Trikafta. Not at first, anyway.

When I started Trikafta there was a month or two where I brought up stuff that felt like it’d been in my lungs for years. Neat! It’s very satisfying to cough up a crusty piece of brown gunk that’s in the shape of your bronchial tree. But for the first official clinic PFTs I did 3 months after starting Trikafta, my lung function actually dipped down a little bit. That was disappointing when I was expecting record highs. If crusty old mucus wasn’t holding me back, that meant that maybe my lungs were just beat up after so many years of abuse and this was the best they would ever be.

As it turns out, I was holding back my lung function.

PFTs measure a lot of things, but there are really only two things that I care about: FVC and FEV1. FVC is Forced Vital Capacity which is a measure of the total amount of air exhaled. FEV1 is Forced Expiratory Volume in 1 second or the amount of air you can blow out in 1 second. When asked to move as much air as I can in 1 second, what I hear is “how quickly can you give yourself a headache?” I was taking the “forced” in Forced Expiratory Volume too literally.

Pre-pandemic I made two incredible decisions though only one is really relevant here: I bought an electric drum kit in December 2019 (if you’re curious, the other decision was not taking a job in a city that got rocked by ‘rona). At the time, my drum skills were limited to some Rock Band I’d played 10 years prior, so I essentially made a $400 bet that I’d actually learn and play the drums. About 10 weeks later I was furloughed from my job for about two months. Guess who’s an adequate drummer now?

I learned that if you want to get better at something, do it every day. Even if you do it poorly at first, you’ll eventually get better through sheer repetition. Maybe not great–I also spent some time trying to learn piano and my progress there has been slow–but better.

So if I wanted to get better PFTs, I should do them everyday.

Just sitting on my ass and breathing as hard as I can isn’t the same thing as putting up big lung numbers. The process of PFTs requires a feedback loop; I need a number that I can beat myself up about. Luckily, since I’m enrolled in a continuation study for Trikafta and it wasn’t/isn’t exactly safe to go to the hospital just for fun, I got mailed a handheld spirometer that hooks up to my phone.

The research coordinator was a little nervous when I said she’d be shocked by the amount of data she got from me, but so far no one has said anything. Immediately after my morning medicines, I do a couple PFTs to see how the lungs are. For the first few weeks, the efforts weren’t great, essentially matching what I had done at the clinic. I had assured everyone for years that if they just let me practice I could put up some big numbers and it was starting to look like I was full of shit instead of air. But I eventually realized that in forcing out the air so hard that I got light headed, I was actually closing up my airways. 

It wasn’t until I was listening to Amorphis’s tremendous 1999 album Tuonela that I figured out what I had to do: I had to growl.

I think Tuonela is the first album I ever owned with growls on it. It’s not really a death metal album–more of a dark progressive rock thing–making it somewhat controversial among the fanbase and somewhat ignored by the band. I celebrate the entire catalog though as Amorphis are a beast too beautiful to be contained by one genre and Tuonela is one of my favorite albums ever.

The song “Greed”–the middle in a triptych of absolute bangers–has a very long and low growl in the beginning that I’ve never been able to replicate, though I’ve tried for roughly 22 years. Possibly because I have bad technique, I noticed I move a lot of air when I do it, far more than I was moving in my PFTs. So I growled into the spirometer.

Ladies, gentlemen, and non-binary friends, we had an all time record.

The growl happens around the 0:45 mark, but the whole thing’s a treat.

It doesn’t really count if you can’t hit it more than once, so I did it every day until I could. In doing so, I modified and refined my technique. My PFTs got a little worse at first–any time I make a “breakthrough” learning anything, I tend to get a little worse at said thing before I get comfortable with the new technique–but I eventually learned to play my lungs like an accordion. It still makes a weird noise when I do the test, but now it’s less like a dinosaur roar and more like a ghost taking a dump. See for yourself!

This video is a demonstration breath that I did after my normal testing, so I’m a little tired and I sort of cough it out at the beginning, but you get the idea.

For those that would like to play along at home, first I inflate my chest as much as possible, using my diaphragm for overflow storage. I think it might help to practice a few inhales while not inflating your rib cage, but when it’s showtime, you want air everywhere you can put it. Then I like to visualize doing a long, low growl and really focus on keeping every airway as open as possible. Then I send out as much air as I can, making sure to not do it so hard I start closing off airways (though I still don’t get it right every time). When it works, the whole house gets to hear the resonant frequency of my lungs/throat, a sort of low “ugggggghhhh” sound.

The first time I did this in an actual hospital setting–hitting the note in the rehearsal room is one thing, but it’s doesn’t mean shit if you can’t do it on the big stage–I warned the technician that I would be making a weird noise and would probably breathe out for longer than they’re used to. Thankfully, the larger apparatus of the clinic spirometer ate up most of the noise, but I think they were wildly unprepared for how long I would take. Not only did I hit a personal record, but I bested my previous FEV1 by .2L, which is kind of a lot. I had done about a 3.47L three months prior, which was a small bit away from my previous best, 3.54L which I did about two years prior to Trikafta. My new clinic record is 3.69L (nice).

This is a graph of my lung function over time. FVC is in blue, FEV1 in orange. I made Predicted FVC gray and Predicted FEV1 yellow. I also included some linear trendlines so you can see that by 2022 I’ll be unstoppable.

I expected a parade to accompany this new record, preferable one where the ticker tape would be my old medical records that they no longer needed because I’d never have to come back. Instead I got, “we just want to make sure you’re still using your Acapella device and doing regular airway clearance”. I hate the Acapella–I’m a threshold PEP man, thank you very much–because my hot breath renders it useless, but if you say that to a medical professional they essentially view it as suicidal ideation, so I usually just say “yeah, I’ll try that out again”. Sometimes it’s just easier to tell them what they want to hear. 

What they don’t want to hear is that my airway clearance regimen includes death growls and attempting to drum Judas Priest’s “Painkiller”. In their defense, there is more clinical data on the Acapella device and I don’t think any of them have heard “Painkiller”.

My lungs still aren’t perfect–my FEV1/FVC ratio is on the high end of low because I screw myself by coming in for too soft of a landing–but maybe there’s a song that can help me with that.

To be clear, I could not have gotten to this place had Trikafta not cleared out the nastier corners of my airways, making it so I could get out more growls without choking on mucus, but it alone was not enough. I also required 10-11 other medications, practice, luck (my health took a big upswing when I started working at home), money (those prescriptions aren’t free!) and Amorphis. But hey, don’t let your disabilities hold you back, right?*

*The current state of discourse on the internet makes me feel like I need to fully explain myself here and say that there are very few–if any–people who are actually attempting to be held back by their disabilities but are instead crushed by a system/society that does not give one single fuck about their situation.