Note: This piece may feel repetitive/circular in some ways. Know that’s largely intentional, as that’s how my mind currently deals with the all of the complicated feelings and thoughts surrounding Trikafta.
Sometimes, I miss the comfort of knowing that I’ll have an early death. Prior to Trikafta, I was almost certain that I’d be dead before I reached forty, which gave me around twenty years to live. This gave me comfort, because I knew that my time was short, and therefore spent my time doing what I could (given my condition) to make that time worthwhile.
In a way, having an early death gave my life more meaning, more beauty, and, in a strange way, more peace. After all, I had a pretty good idea of how I was gonna die. In ten to twenty years, my Cystic Fibrosis would progress to the point that I could no longer live. I’d die in hospice, surrounded by loved ones, and in no real pain thanks to morphine and other opiates. Of course, my death wouldn’t be pretty, but it would be peaceful and expected.
But, then… another unexpected miracle happened. One that literally nobody saw coming (it was very suddenly approved by the FDA one day; coincidentally the day I remember rage-praying to God, begging for a cure to my pain as it was worse than ever). A few months later, just after Christmas of 2019, I got my first box of Trikafta, and the threat of an early death was erased literally overnight.
It’s damn near impossible to properly put into words what it was like to experience my body, quite literally, transform into something new. What it was like to essentially experience a rebirth. Two weeks after taking my first dose of Trikafta, my body was completely different. Over eighteen years’ worth in built-up mucus was expelled from my body. For the first time ever, the inflammation throughout my body reduced to the point that it was completely reversed… and then some. I had no more joint pain, no more migraines, no more tightness around my chest.
Even my pancreas was healed. Not completely, but enough to where I no longer had to worry about Cystic-Fibrosis-Related-Diabetes (CFRD), and I was able to reduce my Creon (enzyme medication) intake by one or two pills per meal. Gaining and maintaining weight was no longer a constant struggle; I no longer needed to shove 4,000-5,000 calories of food down my throat each day to stay at a healthy weight.
I also gained a lot of muscle, especially during the first six months of being on Trikafta. As my strength increased, so did my tolerance for cardio exercise and weight-lifting. My hair thickened, my hands and feet warmed up, my skin became less pale and blue, my toes and fingers became less clubbed. My base PFTs shot up by over twenty percent as my lungs were cleared of mucus completely. For the first time in my life, my sinuses completely cleared, enabling me to smell and taste things like never before.
The list of miracles goes on, all thanks to three daily pills.
But, this new change, as awesome was it was, didn’t come without a few costs. The minor costs include my eyesight and my anxiety. Both things have gotten significantly worse since starting Trikafta. I can still see relatively okay without my glasses, but I can't read anything greater than arm's length from me, and objects get "choppy" starting at five yards away from me.
My anxiety, while better than it was prior to me taking anti-depressants, is still gnarly. I still have a tendency to freak out over every little thing, and severely limit myself out of fear and anxiety. Logically, I know that I'm perfectly okay and capable doing a lot of things I couldn't do pre-Trikafta, such as swimming, camping, and going to college (I know I've been going to college for four semesters now, but I still have a lot of doubts and what-ifs). But, I still harbor a lot of old habits and thoughts from my past; habits and thoughts that kept me alive for over eighteen years. Which stand in my way of... well... living.
Indeed, I don't know how to live!
After all, I’d spent my entire life up to Trikafta preparing myself for an early death. I never expected to make it to twenty, let alone surpass my thirties, let alone make it to old age. Yet, thanks to Trikafta, I will make it to old age, assuming I didn’t get struck by lightning or get run over by a speeding semi. The threat of an early death is no longer greater for me than it is for anyone else my age, and that… well… it brings about a lot of complicated thoughts and emotions. Complicated thoughts and emotions I really dare not to voice to anyone.
I got exactly what I’d rage-prayed for on a cold, windy fall day in 2019. The day I shouted at God while I was nearly frozen to death in my car, demanding Him to fix my health problems right then and there, was the day the FDA unexpectedly approved Trikafta for patient use. Three months later, I got my first box of the stuff. A month after that, I went to my first doctor's appointment with Trikafta in my system. The results from that first doctor’s appointment brought everyone in the room to tears, because my stats and blood draw results were absolutely incredible! For the first time ever, I was just as healthy as my normal peers. Healthier, actually.
Talk about a major medical breakthrough. Or, a cure-lite for Cystic Fibrosis!
But, a part of me felt… bad. And not just because of the survivor’s guilt I felt (and still feel).
I was (and am) very afraid of my future, now that I was no longer set to die young. I was (and am) completely at a loss of what to do next, now that I had an entire long, fulfilling life to live. Most potent of all, I began to miss the security an early death gave to me.
Because I anticipated an early death from such a young age, instead of spending time thinking about my future, I spent that time meditating on the fact that I didn’t really have a future. I grew up being told, over and over and over and over again, that I wasn’t expected to live very long. My deteriorating health only cemented the fact that I wasn’t going to live for very long. So, I spent many hours over many years preparing for death; meditating on my mortality to make myself unafraid of it.
I eventually came to accept the fact that I wasn’t gonna live as long as my peers. Granted, I wasn’t happy about that. I desperately wanted to live and stay as healthy as possible for as long as possible. I did everything in my power to delay the inevitable, in hopes of giving science and God just a little more time to save my life. I ate extremely healthily, I exercised to the best of my ability no matter how I felt, I took all of my medications and did all of my daily CF treatments, I went to all of my doctors’ appointments and did everything they asked me to. I joined a scientific study in which I snorted phage viruses to combat my antibiotic-resistant Pseudomonas infection, when nothing else worked!
I did everything in my power to stay healthy and alive for as long as I could, because I wanted to live. My entire purpose in life was to live just long enough for science and/or God to move in drastic ways, even though I didn’t really believe I’d make it that far (especially when Pseudomonas took hold of my lungs in the fall of 2017). But then… I did. And I’ve been taking Trikafta for three years now. In these three years, my health has only improved. In fact, it’s still improving.
This is where the complicated feelings lie; in the fact that I’m going to live to die of old age (assuming I don’t get crushed by a meteor). Because, now I’m having to contend with questions such as, “What do I want to do for the next fifty-plus years?”, “What do I want to do as a career?”, “What do I see myself doing in five, ten, twenty years?”, etc, etc.
And the answer to all of those questions (and every other question like them) is, “I don’t have a damn clue.”
I don’t know what I want. I don’t know what to do. I don’t know what I’m doing. I don’t know what I’m good at. I don’t know how to set long-term future goals. I don’t even know who I am. I just… don’t know. I’m completely uncertain and petrified by the endless opportunities that lie ahead of me.
Prior to Trikafta, I already had most things figured out (or, at least I felt I did). My main goal was to just make it through each day the best I could, not giving much thought towards the days ahead. I had no future plans, because I didn’t think I had a future to plan for. My health was all over the place, especially in high school. It was impossible to plan ahead when I could be fine one day, and be completely bedridden and/or in the ER the next. Plus, I was gonna die early anyway. So, what was the point of setting long-term goals and dreams when I was too sick and short of time to pursue them?
I already had an idea of what I wanted my funeral to be like for when my health inevitably failed (I still have the notes written down somewhere, though they’re not official and I don’t want them to be). I studied religion, theology, and philosophy to prepare myself for what may or may not be waiting for me after death. Around my family and friends, I put on a facade of hope; hope that I’d live to die of old age. But, deep down inside, I knew my time was short, and I prepared myself accordingly.
Then… Trikafta came along. And everything changed dramatically. My plans to march into God’s Throne Room with a million, “What the fuck?” questions were postponed by at least fifty years. The threat of an early death no longer loomed over me like a great plains thunderstorm. I was free from the shackles of Cystic Fibrosis, so long as I kept taking Trikafta.
In basically every way, I was made “normal”. CF had been successfully defanged by Trikafta. My battle for survival was no longer a battle. Three little pills basically guaranteed that I’d live to die of old age.
I’ve said this before, and I’ll say it a million times more: I was not prepared for this, in any way, shape, or form. I spent my childhood preparing for an early death. Never once did I think I’d live to die of old age. Never once did I think I’d get better as time went on, considering the fact that Cystic Fibrosis is considered a progressive condition; a condition that only gets worse with time. And before Trikafta, that was very true for me.
Yet… thanks to medical science and the grace of God, here I am. Healthier than ever. Getting healthier still. More than likely to live to die of old age.
What in the hell happened?!
I don’t know how to put into words what I’ve been experiencing these last three years. I mean… how can I explain the impacts Trikafta’s had on me to those who never have (and never will) experience such a drastic change in their lives? I guess it’s like describing the color red to someone who’s red-green color blind, only it’s much, much greater than that. One can live a long, healthy life even if they’re red-green color blind. But, nobody can live very long without the ability to properly breathe or eat.
See what I’m trying (and failing) to say?
Perhaps, all of this explains why I've been struggling so much lately, despite having it easier than ever before. I spent my entire life living in "survival mode" until Trikafta came along. Then, shortly after that, covid arrived, and I got to get back to living in "survival mode" for another year or so. But now that I've since been vaccinated, and science has figured a lot about covid, I've no longer been living in "survival mode". Frankly, I don't know what mode I'm currently in, but it isn't "survival mode", and it's not "vacation mode" either.
It's something entirely different that I've never experienced before. It's something I can't put into words. It's something I don't understand. I'm sure there's a term for what I've been experiencing since April of 2021. I'm sure there's an explanation as to why I currently feel the way that I do. I just don't know it. And so far, no one I've discussed this with has any clue what to call it either.
That said, whatever I'm feeling right now isn't bad. The way that I'm currently living my life isn't bad. It's actually objectively good. I mean... I'm not worried about dying anymore. I don't spend the first two hours of my morning hacking up mucus from my lungs anymore. I don't wake up or go to bed sick as a dog. I have more energy to do things than ever before... physically at least. I'm physically stronger and healthier than ever before, and my physical strength and health are still only increasing with time. I'm no longer severely limited by my body. I can, quite literally, do anything I put my mind to, with no greater risk to my physical health.
And, that overwhelms me. The opportunities before me are so great that I'm paralyzed by them. I'm scared shitless because I didn't get to spend my childhood preparing for this time in my life. I mean... I thought I'd be dead by now... or at the very least, way too sick to really go anywhere or do anything. In a weird way, I feel that I was supposed to die, but somehow cheated the inevitable. I stepped into an alternate universe where I no longer have CF, and now I'm having to learn, through trial and error, how to live like everyone else.
My prayers were answered. But, there is a small part of me that keeps repeating the mantra, "Be careful what you wish for..."
I begged for life. I fought like hell to stay as alive and healthy as I could for as long as I could, while simultaneously preparing myself for if/when my body failed, trying my best not to think about the future because... well... growing up, my future was bleak. It was full of suffering and dying young. That fate was literally coded into my genes. Yet, somehow, scientists and doctors found a way to fix that code. Sure, Trikafta's not a perfect fix, and it's not a cure either. But, it's one of the most exciting, ground-breaking scientific creations the world has ever seen. Most people just don't realize it yet.
Prior to Trikafta, gene-editing someone who has already been born was straight-up science fiction! But, now it's reality, and I'm among the first 20,000 people in the world to test this new thing out. As great as it is, as thankful for it as I am, I'm still terrified of it. Nobody knows what to expect long-term. The impacts of Trikafta continue to be studied, but since I'm on the cutting edge of this stuff, when something new crops up, nobody has any real answers for me aside from, "Keep monitoring the situation and let us know ASAP if things get much worse."
What does one say to that? What does one do with that? Well... I personally do what I can to get it out of my mind. Whatever Trikafta does is almost completely out of my control, just like how CF was (and still kind of is) completely out of my control. Trikafta's gonna do what Trikafta's gonna do, and all anyone can do is watch it like a hawk, and take note of the good, the bad, and the ugly. All while trying to get on with life as a "normal" person, because chances are, I'm gonna live a long, healthy life.
But, how can I? How can I get on with life like everyone else, after all I've been through, and also while I'm trying to keep calm while Trikafta changes each and every cell in my body? Well... I've been going to college since the fall of 2021. While college has given me a little structure to my life, it hasn't made me feel any better about myself or my future.
When I went to college, I expected to find people to connect with there. Community colleges are hugely diverse schools. People from all over the world and from all walks of life attend community college. My college offers hundreds of classes, dozens of clubs, and so much more. And yet... I still feel very alone in that vast sea of college students.
I'm the only person with CF (to mine and my college's knowledge) who currently attends ACC (each semester, I make sure to ask if there's anyone else with CF on campus, so I don't exchange CF-specific lung germs with them), and that's been the case since I started. That's not surprising, as there are only 35,000 Americans with CF (roughly 100,000 people have it worldwide). I'm very glad that so few people have such fucked up genetics. But, goddamn... does that make me feel so alone.
So disconnected from everyone else.
I guess facing death at a young age (or any age, really) drastically changes a person. Most of my peers have never had to face death or struggle to survive, which is awesome! I don't want anyone to go through what I've been through. But, there is a huge maturity gap between myself and my peers, contributing to my feeling of loneliness.
When I mention my loneliness, most people just urge me to join more clubs, attend more classes, get more phone numbers, etc. etc. But, I've been doing that since I got my covid shots, and it hasn't really helped. If anything, the more people I meet, the longer I attend college, the smaller the world feels, the more alone I feel. It's hard to explain why this is. It's almost like I see and experience life very differently from most people around my age (and older). And that puts a barrier between myself and everyone else, making it much harder for me to make true connections with other people.
I can't really describe what that barrier is. I can't really explain why I feel so... old... for my age, if that makes sense. I mean... I can understand it on a surface level. As I said earlier, death changes people in dramatic ways. But, I can't really describe what those changes are. I just know that something about me just doesn't seem to... well... connect with most other people under the age of sixty too well. I don't think it's arrogance. I don't think it's fear. I don't think it's any of that stuff. It's just... a barrier... an acrylic glass wall that splits me from everyone else, and I don't know how to get through that barrier to others, and vise versa.
The reality I grew up in is completely different from the reality I live in today. I spent over eighteen years of my life preparing for death, only to be expected to be prepared for life. And, I don't know what it means to "be prepared for life". I don't know how to "act my age". I don't know what I want to do with myself for the next fifty-plus years, assuming Trikafta holds up for that long, and I don't die in some freak accident. In college, I'm just barely starting to figure out what I'm good at, and what skills of mine I can make a decent living off of. I don't have the slightest clue of what I ought to do with my life moving forward.
In short, I'm just paralyzed. I feel just as stuck and alone as I did three years ago, and I don't know what else to do about that. Aside from what I'm already doing.
Recently (as of a few days ago), I came across a new Trikafta study, which basically said that people taking Trikafta can expect to live to be an average of 71 years old, with that number being higher (77 years old) for someone who started taking Trikafta before the age of 25 (which I did).
77 years old. I'm gonna live to be 77 years old, at least. I know it's just one article on one study, but still. Holy shit!
Of course, I'll reiterate that this is an objectively good thing. It's good that I'll live to be an old lady, and I'm tremendously grateful for that. Still... I... I don't know what to think about that. And I sure as hell didn't when I first read the article a few days ago. So, I did what I knew to do best: I went for a walk with Toby around Clement Park.
Clement Park will forever hold a special place in my heart, as it was where I first began to heal after successfully fighting off Pseudomonas with phage viruses in eleventh grade. The library where I met with my teacher is at that park, and I agreed to walk to and from the library every day, five days per week, so long as the weather was bearable and I wasn't too sick. Needless to say, I got better very quickly thanks to those walks and much-needed outdoors time.
Though, I will say, I never got back to my pre-Pseudomonas self, unfortunately. Not that I was expecting to, anyway. At the time, I was just glad I could walk a couple miles a day and not keel over along the way.
A little less than two years later, Trikafta happened. Three years after that, I took Toby to Clement Park for the first time. And... oh boy... as I was walking the loop trail around the lake with Toby trotting beside me, I fought hard to swallow my emotions (I didn't want to burst into tears around lots of other people, many of whom were eager to pet my puppy). For the record, most of the emotions I was feeling in that moment were good. My heart overflowed with thankfulness for Trikafta, for my health, and for my life as it currently was (and is).
Alongside the thankfulness and joy, I also felt... intensely sad... for my 16-year-old self, for lack of better terms. A part of me desperately wished I could go back in time, and reassure my younger self that everything would turn out okay. In fact, I had a dream like that once, about a year or two ago. My memory of the dream has since faded quite a bit, but I vividly remember waking up crying tears of happiness and relief upon waking up from it.
I guess that dream gave me some closure, though not complete closure, given that I still feel so... I can't even explain it. Lost? Unsure? Wishing that dream was reality and I could relive it? I don't know. I sure as hell can't put into words what I don't know, let alone what I do know.
Point it, emotions are hard. It's emotionally difficult to deal with the consequences of Trikafta. As I make my way through college and continue to explore my likes and dislikes, abilities and disabilities, strengths and weaknesses, opportunities and possibilities, etc. I get more and more overwhelmed... but not necessarily in bad ways. I just feel greatly overwhelmed by the possibilities that lay before me.
Prior to Trikafta, living with CF was like living as a bird born in a welded-shut cage. I just had to accept that my life would be short and severely limited. But then, Trikafta came along. All of a sudden, after over eighteen years of living like I was, the cage was completely done away with, and I was (and am) free to fly out into the world, like everyone else. So, while I am, in theory, free to do whatever I want, truth is, I can't just spread my wings and fly. After all, I never dreamt of flying, let alone ever had the chance to learn, so what am I supposed to do?
Well... I'm just now learning how to fly. Again, I'm doing this by simply throwing things at the wall and seeing what sticks, while also taking suggestions and advice from the people in my life who know how to live long, functional lives.
If covid taught me anything, it's that I don't find any joy whatsoever in doing absolutely nothing with my life. During the covid days, I spent most of my time sitting at home playing video games, and only leaving the house to run basic errands and get some fresh air. 95% of my socializing happened over Zoom and Skype. I never stepped foot into a restaurant, and most of my groceries were delivered to my doorstep. I avoided people like the plague, and wore an N95 or gas mask whenever I had to brave indoor public spaces.
Needless to say, it sucked major ass for me, and everyone else in the world. By the time I got my vaccines and could get out into the world again, not only did I have to re-learn a lot of social skills, but I was ready to attend college, for no other reason than it would give me some structure to my routine and face-to-face, in-person interactions with other people my age.
A little over a year later, now that the initial shock of attending college has largely worn off, I'm trying to figure out what I want to do with my life as I'm running out of general pre-requisites to take.
For the record, I haven't come up with nothing. I just don't know what to pick! On one hand, it's a very good thing that I'm figuring out that I'm good at a lot of stuff I never thought I'd be good at. But, on another, it's paralyzing. I've only just started to create a web map of all of the different careers I could take that require a college degree, and it's already huge. I haven't even included careers I could try that don't require a college degree, of which there are many! Nor have I included careers I have little-to-no interest in, such as careers related to real estate and education.
What am I supposed to do with all of this information? How am I supposed to narrow down a single career out of hundreds of thousands? Hell, what happens when I do narrow down a career? How does college graduation work? How do job applications work? How to job interviews work? What will the job market look like once I finally graduate college? What will the real estate market look like? What will the economy look like? What do I do with my first paycheck? How do taxes work? How does retirement work? How does health insurance work, and how do I make sure my health insurance covers Trikafta? How does politics work, and what will the political landscape look like when I'm out of college? On and on and on...
Such questions are why I considered an early death a security blanket of sorts. While I didn't (and still don't) want to die, dying early meant I would get to skip most of the complicated "how does life work?" questions every functional adult has to wrestle with. Now that I'm no longer dying for the first time in my life, I'm having to face all those "how does life work" questions for the first time, too.
Truth is, to me, death is far less scary than living. When I say that to people, they usually don't know what to do with that sentence. At best, they just look at me like I've got two heads. At worse, they ask if I'm okay and in therapy. To be fair, I don't blame people for getting so... uncomfortable... whenever I bring up the fact that I should've died a long time ago. But, whenever I do bring it up, it's simply because I wish to tell my story from my perspective, to anyone who's willing to listen. I also hope to hear some advice from people who grew up preparing for a long life, because I sure as hell haven't a damn clue how to prepare for a long life.
Unlike dying, I haven't spent years meditating on living. I have a pretty good idea of what dying and death are like. Technically, I've been dead before, and I've come near to death too many times to count. I've witnessed the death of loved ones, whom I will forever grieve till we meet again. I've made my peace with God (for the most part), and talk to Him every day, throughout the day, like I would a close friend. I've experienced and witnessed miracles of all kinds, the most baffling of which was when my heart valve grew back when I was in kindergarten. To this day, my cardiologists can't explain that one (and I ask every few years when I go in for an echocardiogram).
Evidently, while I often say, "I should've died...", the truth is the opposite. For reasons nobody can explain without Divine Intervention, I'm still here. Healthier than ever. Clearly, I'm supposed to be here. If I wasn't, I would've died as a newborn, or in elementary school, or in middle school, or in high school. But... I didn't. I came very close, which is why I've grown so comfortable with dying and death. But, I didn't just survive my childhood. I'm healthier as an adult than I ever was a kid, and I'm still getting healthier as time goes on.
Because of this, people often urge me to just "let go of the past" and "look forward to the future", and to shut the hell up about death and dying. But, how can I? I can't simply "forget the past" and move on from it. My past shaped me into who I am today. That said, I don't base my entire identity on my past. But, the past is certainly worth remembering, reflecting upon, and listening to. Especially with those who were there when I was at my sickest.