It has been over two years since I started Trikafta
A meditation on my experiences on Trikafta, cystic fibrosis, where we are now, where we can go in the future, and more
It has been over two years since the FDA approved Trikafta in October 2019. My experience has been mostly positive, but not everybody else’s has been. This essay is a meditation on Trikafta and life since I started it, but it’s bigger than that: It’s also a reflection on being disabled in an ableist world, on cannabis, on how issues that affect the CF community are linked to broader societal issues like civil rights, the drug war, climate change, and more. I hope you take the time to read. If this resonates with you, please share it with others on social media. Your support means the world to me. I would also love to hear others in the CF community’s perspectives and also the perspectives of people not affected by CF.
Trigger/content warning: This essay will touch on many sensitive subjects including death, grief, suicidal ideation, mental health struggles, and many others. I do my best to write about these sensitive subjects as gently as possible, but as a firm believer that writing requires these types of real subjects to be covered in a way that conveys their heaviness, sometimes, I intentionally talk about my experiences in harsh terms. If you are struggling with anything of the sort, please reach out to me (or here) and I will do my best to be a support beam during a time of difficulty. You are loved, and the world is better with you in it.
It is critical that I open this essay with the acknowledgment that I am privileged beyond belief. This is true across my identities. When it comes to CF, my health for the majority of my life has been stable whereas most others, including my sister, aren’t nearly as fortunate. I also have the most common mutation on both my chromosomes which, while it is considered a more severe mutation than many others, receives the most research interest thus providing me a sort of “mutational privilege” that I’ve begun to call it. I am also a white, cis-gendered male which provides me a level of privilege in life that does trickle down into the CF world. I have also been very fortunate in other ways; financially, throughout my life, my parents have ensured neither me or my sister ever struggled to have what we needed, including healthcare and prescriptions and my mom was able to be a full-time caretaker for both of us which allowed us to have guaranteed support at every appointment. On top of all of this, I’m also simply lucky that Trikafta has been as effective for me as it has been.
This level of immense fortune is not lost upon me. I can acknowledge that I have been disciplined in learning to understand CF and do my best to take care of myself and advocate for patients but we only have so much influence on our health. My parents worked very hard to provide for us. But it’s this fortune that has driven me to work every single day to better understand how the world works and to internalize those understandings to help build a better world for people with CF, people with other chronic diseases, and everybody more broadly speaking.
All of that being said, my journey to this moment has not always been clear or paved; there have been gravelly, dusty roads, there have been roads full of hazards, roadblocks, detours, and deep potholes, there have been short stretches of empty highways. We have all experienced journeys like this; our routes are all full of different proportions of these hazards, followed by short, moderate, or long stretches of ease. The more I learn about the human experience, the more I have learned that everybody is resilient, and everybody is just trying to make it through the day. Everybody has suffered in one way or another, and the world becomes a lot gentler when we recognize that that person who cut us off might have been rushing to the ER or they might have just gotten some tragic news, or maybe they’re simply in a rush because they’re frightened that being late will result in them losing their job. Or maybe, they’re just driving like an asshole. I don’t think one asshole driver should be enough for us to lose sense of our shared experience as humans navigating the difficult experience of being human. Trikafta has made my journey a lot smoother these last two years.
Trikafta has been a miracle drug for a lot of people. But CF and our community are not defined by Trikafta, just like the CF experience or community has never been defined by any other medication or breakthrough before this one. The CF community is defined by a shared commitment to one goal: To create a world free from CF and the suffering that comes with it. Our community is not united on every issue. There are plenty of issues that members of the community are split on, but I can confidently say that disagreements are rooted in approach, not end goal. The end goal might be the same, but differences in opinion around approach is good. These differences make us better as a community as they drive us all to interrogate our opinions and biases, and hopefully, if both engage in good-faith, people at different sides of a discussion will come away with an improved perspective and renewed belief in working with others to make the change move in the right direction.
Since being on Trikafta, my life has changed dramatically. On most days, the daily burden of care and clinical manifestations I was used to for so many years are barely there. I don’t have to do my vest, inhaled medications are a few times a week thing, I don’t cough much and when I do, I don’t produce much sputum, and my sputum, when produced at all, is thin and clear. The occasional exacerbations I’ve had have been insignificant and quickly resolved with a course of oral antibiotics. I don’t even need to see my care team nearly as frequently. The biomarker I used to obsess over — forced expiratory volume in the first second (FEV1), or just lung function — is something I rarely think about anymore. My life is stable and CF has become a non-issue.
For extremely positive cases like myself, I’ve begun using a term that I hope gains broader traction: CF remission. For “CF remission” to become a medically validated term, it would require the CF Foundation and researchers to look deeply at what the long-term prospects truly look like for people experiencing these robust benefits but for a lot of people, Trikafta has opened up the future in a way like no other drug not only in CF history but in the rare disease world. I no longer assume I am going to die young and I have dreams of aging and retiring and having grandchildren. I believe I have a real future for the first time in my life. It has taken some time, but I think even my parents have reduced their worrying about my health to the level of most other parents without a child with cystic fibrosis. Words can’t describe the relief I get knowing that there are people who care about and love me — and people who cared about my sister — that are witnessing the reversal of the prospects of my life. Many times as a child, I cried from rage and despair about having a shortened life expectancy. Today, children that are eligible for Trikafta could very well see a cure and live mostly normal lives. I’m happy for the adults who have benefited, but I’m ecstatic for the children and their parents.
I began Trikafta two years and two months ago in November 2019. About four months after starting Trikafta, the US began grappling with the COVID-19 pandemic. In the timeline of my life, the beginning of Trikafta roughly corresponds with the pandemic. As for my thoughts on the pandemic, which could be an entire series of essays on its own, one overarching message I’ve learned since we first entered lockdown in March 2020: Disabled people have a right to live and we should not have to argue our livelihood every single day. Yet we do. COVID-19 didn’t expose any fractures in society that did not exist before; instead, just as sunlight is the best disinfectant, the pandemic shone a light on these issues in a way like never before. Disabled people knew about many of these issues before the average person had ever learned of SARS-CoV-2. The lessons and experiences I’ve learned from the pandemic have been disappointing at times but transformative for my worldview and I’m more optimistic than ever that a better world will exist. This is true of both society post-pandemic and the CF community post-Trikafta. With circumstances that receive extensive media coverage, the lessons each individual gleans will be radically different. That is where it is on us to take these opportunities to make something positive happen.
Everything is a spectrum. Cystic fibrosis is a broad spectrum of different experiences; Trikafta has been a broad spectrum of different experiences. There are positives to suffering, despite suffering being a generally unpleasant experience that we would all prefer to avoid.
The year leading up to Trikafta was filled with some moments that have outsized importance in my mind. Over the summer of 2019, I had been invited to give the keynote speech at the CFF’s Research Conference in Stowe, Vermont. It was one of the first trips I had done entirely on my own in a picturesque location during summer, and it was to deliver a talk on two of my favorite things: life with CF and science. It was during that trip that I felt like life was attainable, that I could make a contribution to the worlds of CF and science and I could continue using my experience to ideally drive scientific and communal progress in a realm that was as near to me as life itself. The trip’s positive message was compounded by the fact that I was alone for the majority of this trip and I delivered the biggest talk of my life. I realized then how many limitations I had imposed upon myself. That trip put me so far outside of my comfort zone, with the only thread to my comfort zone being the opportunity to talk about my personal experiences with CF, that it was filled with adrenaline for the flights there, back, and every moment in between. I realized during this trip that the life I wanted to live was outside my comfort zone. That experience would teach me the importance of taking chances that terrified but excited me.
The biggest talk of my life in Stowe would be surpassed three months later thirty minutes north of Washington, DC, in Bethesda, Maryland. I was invited to the CF Foundation to deliver an in-person talk to the employees at headquarters that was to be live-streamed to every chapter. I would cover my personal experience with CF, my sister’s life, my research and writing careers, and then open the floor for questions. The day of that talk, I was also able to have my parents and a few family members there. By the end of my talk, in a moment that feels poetic, my father was answering questions standing next to me and my professional mentor (shoutout JP Clancy). I also had the opportunity to meet with several teams of the CFF where I could learn the ins-and-outs of what they did and provide my perspective.
The day before, I was invited to join the CFF on Capitol Hill where I went to the offices to advocate on behalf of the CF community of my then-state representatives. None of these representatives would vote for the issues I had advocated for, but this part doesn’t matter. For me, it was the largest single act of direct action I’ve done for a community that I love as dearly as family.
After I finished meeting with the different teams at CFF, I ubered from Bethesda to Arlington, Virginia where I met up with my family. I didn’t realize it at the time, but as we were driving to Arlington, we drove alongside the beautiful Potomac during September, one of the prettiest times of year in the area. Usually I talk the entire drive with ride share drivers, but on this occasion, I needed to reflect on the day while listening to music. This ride home is a moment I recall fondly; it was the moment that I realized that I wanted to pursue something much bigger and I felt like I had a chance to do so. It was then that I also realized I wanted to come to Washington, DC.
I feel it is important to share these moments because these were lessons I learned that I want others to hopefully learn from me if they themselves have not yet learned these lessons. In a span of a few months, I learned that life was meant to be lived, and more specifically, it’s meant to be lived on the fringes or outside of our comfort zone. If I hadn’t taken those chances despite being terrified and excited to do so, I don’t know when I would’ve learned the lesson that would come define my future.
Two months after that talk, I started Trikafta. The lessons I learned during those trips aligned with a belief that I was being given a second chance at life. Pretty quickly upon starting Trikafta I noticed how much better I felt physically. In March, just as COVID-19 began to close the world, I was offered a job in Pensacola, Florida. I knew little about Pensacola but after a trip there, I decided I would be leaving my home and friends in Kentucky for a major personal and professional leap. Who knows if I would have taken that job if not for those two talks half a year earlier. Life since that weekend in Pensacola has moved rapidly.
At BioNews, I would focus on patient-reported outcomes across 60+ disease communities. The goals of these surveys were to research every factor of the patient experience to better understand what affects their quality of life, what these communities need, and so much more. Through this research, my committed belief that patient communities, no matter the type of disease, are united by far more than we are divided by would deepen even further. Every patient experience is different but we all learn very similar lessons about the way the world works and we all quickly learn how to find peace in a world that is neither built by nor for us, and a society that refuses to fully acknowledge the spectrum of human existence in terms of ability, work, and experiences. This experience alone is enough to warrant a devotion to solidarity, but as of yet, we do not have enough cross-disease and international community movements to realize the solidarity needed to advance these issues. This role would also inform my understanding of internal inequities within my disease community and others. The racial, sexual, gender, and class inequities that exist in American society are present, and sometimes worse, in disease communities. I have long said either everything is political or nothing is. Healthcare is political, disability is political, inequities are political. To entirely resolve socioeconomic inequities within the CF community cannot be done by the CF community alone; socioeconomic inequities must cease to exist in society full stop. And by resolving inequities in society at large, the gaps narrow in every disease community. Our interests align more than we usually realize.
Not only would I learn these lessons from the research we conducted, I realized how beautiful the spirit of humanity is. Everywhere — without exception — there exists suffering, there also exists in equal or greater parts human spirit, resiliency, compassion, kindness, and resolve. We must speak truth to power and challenge those in power who perpetrate or perpetuate human suffering, but we must refuse to allow them control over our minds and spirits. We can focus on those that experience this suffering and instead of becoming cynical or cruel themselves, they commit to building a better world for the future, even if they may never see it. There will be times where we make sacrifices for somebody we don’t know because it is the right thing to do. These sacrifices, sometimes small, sometimes large, compound and spread compassion outward. It’s through millions of little acts of goodness that a better world is built. But these have to start somewhere.
My experience in Pensacola would not only benefit me professionally; it was also great for me personally. The beach and ocean are sort of spiritual to me. Hypertonic saline, a key tool in the CF arsenal for the last several decades, was discovered by Australian researchers who found people with CF who lived closer to the ocean fared better. They deduced the salt in the air thinned the mucus in the lungs of people with thick, sticky, CF mucus. I don’t love the ocean for only that reason. I find the ocean to be a mesmerizing part of the earth. Life arose there, life thrives there. The breadth of the ocean is incomprehensible. Though forests are the lungs of the planet, oceans strike me as similar to hearts; the pulsating waves, the life and habitats, the connection to faraway places. I spent many, many mornings and evenings near or in the ocean. Some evenings, I’d sit on the beach transfixed by the ocean painted against the night sky, contemplating my place in the world, humanity’s place in the universe, and the beauty of both the ocean and night sky. Earth is large, the universe larger. During difficult times, I look up to the night sky to remind myself that we are parts of the universe and connected to the very moment the universe began. Existence and sentience are beautiful things.
Another major component of my experience in Pensacola was medical cannabis. Out of concern of the stigma and retribution to my career, I haven’t written much about cannabis but it has become an important part of my journey. I have struggled with anxiety and depression for so long that I knew I wanted to utilize medical marijuana in an attempt to resolve my longstanding issues. The benefits of cannabis for me were significant, but most notably was that cannabis didn’t only help when I ingested it; it helped me in the days afterward. I certainly don’t dismiss the importance of anti-depressants and anxiolytics, but for me, they only seemed to lightly ameliorate my issues. I felt that they were reactionary and treated the symptoms. Cannabis acted as a prophylactic; cannabis helped to reframe many of my struggles. I felt that cannabis helped me maximize my positive passions — like caring compassionately about educating others and fighting for issues like curing CF, resolving climate change and COVID-19, improving inequities — and minimize my negative ones — being angry at politicians but not doing anything to fix it, fixating on our failures as a society to mitigate COVID-19 or climate change, or personal insecurities like feeling like a scientific failure. If you notice, most of the issues are similar but framed from different angles. I never feel like my mind is dulled with cannabis. Instead, my mind feels calmer, more acute.
Since I started using cannabis, it has had a profound impact on how I understand my own mind. My experience with cannabis allowed me to kindly confront a lot of my internal struggles with cystic fibrosis, my sister’s life and death, survivor’s guilt, my parents, my family, my friends, my career, my place in the world, my guilt and shame, my anxiety and depression, my self-loathing, my suicidal ideation, and everything else you can imagine. I hesitate to attribute this to one thing, but cannabis played a major part in me navigating suicidal ideation by allowing me to confront those thoughts compassionately and fully interrogate why they were racking around my mind uninvited. Cannabis played a major part in saving my life from myself. Cannabis also provided me the runway to navigate something I’ve subconsciously wondered about for a long time: That I might have ADHD or be neurodivergent. I’m now being evaluated for ADHD but even if I don’t have it, cannabis has played a significant role in me better understanding how my mind works. This is not to be taken lightly; understanding how my mind works has had many downstream benefits in helping me navigate my dreams, goals, careers, insecurities, and general well-being. Since using cannabis, I have experienced maybe one or two minor panic attacks.
Cannabis is not perfect, just like anything else, including Trikafta. But the safety of cannabis is widely documented and, with appropriate use, can be a genuinely positive thing for most people, chronic disease or not. But I can’t discuss cannabis without discussing something else that has become an increasingly important issue in my mind: the decades-long drug war that has heavily stigmatized compounds, including those with genuine medical potential. The US drug war, from its racist crackdown on crack to its ongoing prohibition of cannabis that disproportionately harms Black and brown Americans, has destroyed countless lives and essentially impeded all clinical and scientific research into the substances thus stealing from all of us the medical research that could’ve changed many lives. The drug war has also ruined the lives of millions of Americans by flattening why some people might use these substances for complex, multifaceted reasons that should not be dictated by the US government. I have benefited from cannabis, but as of yet, there are two Americas: the states where medical or recreational cannabis is legal and the states where it is not. Furthermore, in even the states where it is legal, there still remain people behind bars for non-violent cannabis-related crimes. This is inhumane and cruel. Cannabis should be legalized recreationally and medicinally and everybody currently in prison or probation for non-violent cannabis-related crimes should be released and have their records expunged. Cannabis, like LSD, psilocybin, and ketamine, should also be studied further for its many potential therapeutic uses. It’s the ethical thing to do, especially while many white Americans are collecting profits while Black Americans face inhumane detention, including the horrible conditions at Riker’s Island where many people are detained even after never being convicted of a crime.
None of these issues exist in a vacuum. Cannabis, the drug war, civil rights, racial, social, class, and gender inequities, disability rights, even climate change; all of these issues dovetail. Cannabis could be extremely helpful for people with disabilities; people with pulmonary diseases will experience the first harm from climate change due to increased air pollution; climate change will disproportionately harm Black and brown communities, who have been harmed by the drug war, and who also experience other inequities in the US; inequities across races are also present in disease communities. All of these issues affect one another. Everything is political or nothing is.
Trikafta is a major accomplishment in the book of CF, but it is most certainly not the final chapter. Until everybody on planet earth with CF is cured and has a chance for a full life unencumbered by a defective protein, the history of CF is incomplete. While it’s important to celebrate Trikafta for the many people and their loved ones who are now suffering less, we must acknowledge the other pressing issues within our community.
There is no American race, so we must emphasize that CF is not an American or white disease. This distinction is important because while statistics seem to show that CF is most prevalent in the US, I am confident that the number of people across the world with CF is vastly undercounted. This is due to logistics; many nations don’t have the capacity or understanding of CF to efficiently and accurately test. This is also due to tragedy; I suspect in developing countries, there are probably many infants with CF but who are never diagnosed that die due to malnourishment, infections, or other complications. In every nation in the world, CF care is lacking. In countries like England and Canada, care is pretty much on par with the US, but access to necessary medications that receive FDA approval lags. In other countries, they don’t even have access to basic CF necessities like hypertonic saline or Pulmozyme. In the US, people with CF who are unable to work are unable to effectively afford more than basic necessities due to earning limits imposed by the US government in exchange for access to healthcare. Disabled Americans still struggle to prove their livelihood as we’ve witnessed during the COVID-19 pandemic. As I’ve hammered home, inequities — racial, sexual, gender, class, mutational — exist in the CF community. Many Americans with CF are either ineligible for Trikafta or other modulators, or these modulators aren’t well-tolerated. This is creating a further divide in outcomes within our community.
Many Americans with CF report physically benefiting from Trikafta but experience severe mental health issues. I cannot affirmatively say Trikafta hasn’t negatively affected my mental health since I have struggled with it at times, but I do feel that my body aches a bit more, sleep seems more difficult, I feel fatigued at times, and I don’t feel perfectly healthy. To that end, it must be emphasized that Trikafta is not a perfect drug for really anybody, and for some, they reasonably decide their physical health is not worth the exchange of their mental health.
Trikafta also shines a light on another issue that is under-discussed in the US: What are people with CF supposed to do now that some aren’t (legally) disabled anymore? Wages in the US are already egregiously low and stagnant, disabled people often don’t have the ability to work full time or go to college so disability can influence resumés which disabled people then feel pressured to disclose and justify in interviews to explain gaps, and Americans are struggling more than ever to save for retirement. Many disabled Americans can’t save at all. So what are people supposed to do when they’re suddenly healthy enough to work full time but don’t have the income to support themselves, or don’t have any savings, or have to start with low-paying jobs since they lack experience? Now consider this is all during a respiratory pandemic that puts those of us with CF at even greater risk. The simple answer is not “just go work.” There are so many complexities here that “just go work” doesn’t consider. Many people are forced to remain legally disabled to qualify for low-income housing and Medicare and Medicaid with no real opportunity for economic advancement. This is why we often say capitalism is ableist. Humans are not meant to be singularly defined by their ability to yield economic output; disabled humans, despite often being unable to work full-time, contribute so much love, beauty, intellect, problem-solving, value, and creativity to society. Disabled people are also allowed to just be people without the belief that, despite not being able to work full-time, they must sacrifice themselves in some other way. Humans, disabled or not, must not be defined by something as inhumane and dull as the ability to generate economic revenue.
I often say the world can be better. This is a core belief that helps me get out of bed every day. I believe this because humans have faced terrible suffering across hundreds of years and always, no matter what, there are people in every pocket fighting against cruelty and working to reduce suffering. It doesn’t happen overnight, but humans have overcome so much in our hundreds of years of society that I find inspiration in the people who fought to resolve these issues in eras long ago. There is no doubt in my mind that we can continue to overcome the most pressing issues afflicting humanity.
After living in Pensacola for a year and a couple of months, I ended up getting a new job and moving to Washington, DC. It was not easy choosing to leave Pensacola. Especially the warm weather. I had come to love the people there and the proximity to the beach. This decision, like the Pensacola one before it, was made with a mixture of excitement and fear. I knew I loved DC already, but I knew I was entering a place like none other I’d lived before. Ultimately, DC has proven to be great for me. I adore the city and the area and it’s filled with so many people working to build a better world. I’m also fortunate enough to be able to live alone and have found that I really enjoy the solitary time and the ability to have a place be my own.
Trikafta has opened up the world for me and for that I’m eternally grateful. Yet, our community has so much more to go before the book on CF is closed. I hope our community can come together with one another to fight for the world we all deserve, and I hope our community can link arms with other communities in the US and abroad to build that better world for all.
I want to conclude with an excerpt from an essay I wrote a few days after Trikafta was approved:
[Trikafta] is a tremendous win. There is no other way around it: The combination of fundraising, advocacy, medicine, science, and common humanity over the past half century has led us to this moment. It is nothing short of a miracle of superhuman proportions. The victory is built on the backs of hundreds of thousands of people. The victory is also built on a graveyard. When CF is one day cured or at the very least not any different from a diagnosis like diabetes, it will come with the knowing that tens of thousands of young people died without a chance of a full life.
This remains true to this day, which is why it is more important than ever that we continue fighting for better medications and a cure for everybody with cystic fibrosis
Thanks for reading. Your support means a lot to me. I plan on writing more this year. If you’ve been a reader of mine for a while, thank you sincerely for your patience as I navigate my writing career.
tl
Great read Tre! Love you cuz
Awesome article’. I love reading everything you write. You’re the best!!!! I love you.