Trikafta: Six Months Later

Six months ago today, I stood in my office (who even remembers what that feels like these days? LOL), anxious, eager and cautiously optimistic to find out what this box and the little pills inside would do for me. This medication was an answered prayer for so many. It was a miracle drug that many had waited for their entire lives. Personally, I was one of the lucky ones because this was not the first miracle drug I was able to take. The truth is my first miracle happened almost five years ago when the FDA approved Orkambi in July 2015.

Prior to Orkambi, I was admitted to the hospital on average 4-5 per year. Since starting Orkambi in August 2015, I’ve been hospitalized three times total. Three times. In five years. To me, this was more than enough. I didn’t care that my lung function hadn’t gone up, I didn’t care that I didn’t gain weight. I just relished in the fact that I finally had the chance to live something that more closely resembled a “normal life”. I cherished that I didn’t constantly feel sick and that I didn’t repeatedly have to put my life on hold to go spend two weeks in the hospital every couple months. What Orkambi did for me was life changing and I couldn’t imagine how it could get any better. Man was I wrong...

Last November, I started Trikafta, the newest and best CFTR modulator developed by Vertex Pharmaceuticals to treat the underlying cause of cystic fibrosis, and once again, my life changed in ways I couldn’t even fathom. 

Today, my FEV1 is sitting 6-7% higher. This might not seem like a lot compared to some of the stories many of us have heard from others in the community, but it’s the highest my lung function has been in over 3 years and considering I started this medicine at just 27% FEV1, 7% is pretty solid net improvement. I’ve also gained over 12lbs and I currently weigh more than I have in a decade (and it’s still going up!) Even more remarkable is that I’ve been able to cut my tube feeds down to just 2 nights per week instead of 6-7 and my weight has remained stable. Why is this so great? Cause it means I actually get to sleep through the night and get a full, uninterrupted 8 hours of sleep, five night a week, instead of waking up to turn off the pump at 4am and then trying to go back to sleep, and not waking up nauseous in the morning because I just had 30+ oz of formula pumped into my stomach. You know how full you feel after a giant chocolate shake? Yeah, now imagine feeling that way first thing in the morning, every single day.

One of my favorite little guys
sharing one of his favorite new books
because it reminded his mom of me 😂

My appetite on Trikafta is consistently in what I like to call “baby dinosaur mode.” I am pretty much always hungry and cannot stop eating — a stark difference from my pre-Trikafta life. In fact, back in a pre-COVID-19 world — you know, when people were actually allowed to go shopping — I was at one of my favorite thrift stores and came across a couple pairs of really cute dress pants that were a size 1 and size 2. Normally, I wouldn’t have even bothered because I have worn size 00 since I was in the 6th grade. But I knew I had gained some weight so I decided to give it a shot… you can imagine my surprise when they did actually fit! I also may or may not have broken a button on a dress trying to get it to fit a couple months ago as well… Wild, right??

My weight has been a never-ending challenge ever since I was diagnosed with CF at 3 months old. It’s actually the very reason I was even diagnosed. I was losing weight as a newborn, instead of gaining. My little body was literally starving to death and I was diagnosed as failure to thrive, before additional testing confirmed the cause was cystic fibrosis. Ever since, I’ve been a very petite person and I have always struggled to consume enough food on a daily basis to maintain, or god forbid gain, some weight. It’s common for people with CF to have higher daily caloric needs due to constantly fighting off infection. That coupled with not being able to properly absorb fat due to pancreatic insufficiency leads to a constant and uncomfortable daily battle of forcing yourself to eat more. Which many times leads to food aversion. There's been so many times that even the thought of eating make me want to puke. I have taken appetite stimulants for over 16 years, have a feeding tube that gives me 1500 calories at night for the last 14 years, and I still can’t seem to eat enough during the day to keep my weight (head) above water. On Trikafta though, I no longer have to force myself to eat... I actually want to do it! And my life is not literally completely dependent on my feeding tube. A year ago, only doing my tube feed 2 nights a week would’ve resulted in me dropping 5-6 lbs in a single week. And then spending probably 5 or 6 weeks to gain it back. I know most 28 year old females would cringe at how excited I sound about gaining weight, but for me, it's a relief and a welcomed change of pace. 

At this point you’re probably thinking, “Wow, that is amazing!” And I would say “Yeah it really is… but wait there’s more!”

While actually having a justifiable reason for how often I buy new clothes is freaking fantastic… the most amazing thing I’ve experienced as a result of Trikafta is that my cough — the cough that has tormented me for the last 28 years — is all but nonexistent. Seriously. I barely cough anymore. Coughing has long been my “tell” and it’s usually the first thing that gives me away when I meet someone new who doesn’t know about my CF. (Me: *Coughs four times in the first 12 minutes of meeting someone* Them: “Are you sick? You seem to be coughing a lot”. Me: “Well no, not really but...” ) On Trikafta, I don’t have to repeatedly explain to complete strangers that “No, I’m not sick” and I often find myself sitting in bed at night realizing I haven’t coughed all day. Every once in a while, I catch myself actively trying to remember the last time I did cough because it's been so long. I still do all my breathing treatments and airway clearance therapies, but when I do, I have to force myself to cough and even then, it’s still few and far between. It’s so noticeably gone that I’ve had literally dozens of family and friends point out to me over the last six months that I am not coughing.

As a result of not coughing all day long, my energy levels have skyrocketed. I feel great and am honestly just less tired than I have been in years. I’ve said before that one of the most exhausting parts of having cystic fibrosis is coughing but I don’t think I fully understand my own statement until I didn’t have to experience it every single day. Coughing is exhausting and it use to consume so many aspects of my life. Before Trikafta, damn near everything made me cough. Laughing too hard? Cough. Crying too hard? Cough. Step outside and the air is too cold? Cough. Singing along to my best Spotify playlist? La la la la... Cough. Try to use hairspray or perfume? Cough. Try to blow out a candle or use an air freshener and the fragrance was too strong? Cough. Carry a bag of garbage to my apartment complex dumpsters? Cough. Try to talk while also walking quickly? Cough. Try to do any form of cardio exercise? Haha no, how about I just cough instead? Need to vacuum or move some heavy items around my apartment? Cough. Try to run after my dog who just saw a squirrel outside? Yell NO BENJI... then cough. Take too deep of breath to try and stop coughing? Cough some more.

I could go on, but I think you get the point. It’s not a secret that my disease progression is severe, and I had reached a point with my CF where even minimal physical excursion often left me gasping for air, which then usually turned into a brief uncontrollable coughing fit. Now that it’s gone, I feel like a whole new person. I can do the simplest of tasks without having to stop and cough. I can talk on the phone and not have to ask someone to repeat what they just said because I started coughing and couldn’t hear them. I can laugh, cry and sing, without coughing. I can play with my dogs, without coughing. I can meet someone new and not have to dodge judgmental glances and/or disclose that I have a chronic illness within 15 mins of meeting them to avoid more awkward glances because I was coughing. I can just breath, without coughing.

“Just breathe” has been a mantra for people with with CF for many years. It is one of the most natural things our body does, most of the time without us even realizing it. It’s something that many people take for granted. I always thought I was pretty good at “just breathing” despite my CF, but over the last six months, I’ve realized that I had absolutely no idea what that meant and even my best days on Orkambi pale in comparison to what I experience every day on Trikafta now. They say “you don’t know what you don’t know” and sitting here typing this might be the first time in my life that I truly understand what that phrase means. What I thought was “just breathing” is nothing like how it feels now to actually just breathe. Not cough, just breathe, deeply and freely.

I am so thankful for this newest miracle drug. Did it give me groundbreaking results and reverse years of existing damage? No. Did it completely erase my cystic fibrosis? No, of course not. Did it drastically reduce the laundry list of medications and therapies I have to do on a daily basis to maintain my health? Also no. Will it prevent my disease from ever getting worse or take transplant completely off the table? Probably not, but who knows! Did it change my life by giving me a chance to live a different kind of reality, one that’s easier and less painful that I have ever experienced before? A thousand times yes.

Cheers to six months of a new kind of normal, and a new kind of "healthy." Thank you to everyone who has followed along and supported me on this new chapter of my life with CF. If you’re up for a (much needed for so many reasons) post-global-pandemic celebratory shopping trip and/or happy hour, you know where to find me (hint, it’s the same place I’ve been for the last two months because of said global pandemic 😋)