This will be my final written post of #31DaysOfTré. I have a cool idea that I'll be doing for tomorrow and I'm looking forward to sharing that with y'all.
First, I want to give myself a little pat on the back. Though the project wasn't exactly what I hoped it would be, I have written a post for every day so far. That means a lot to me because I look forward to projects in the future and I'm proud of what I've produced with this.
Secondly, I want to very sincerely thank anybody that has taken the time to read what I've written, whether that's any post for this project or just anything I've written in general. That means so much to me. I think my platform has expanded since starting this project, and admittedly, it could've definitely been produced better. But considering it's been the hardest stretch of 3 months in my life, I give myself credit for achieving this, but I wouldn't be here without the support of so many.
As for what's next. I have lots of ideas for writing projects, pieces, and other creative projects I want to work on. This was just the beginning. I hope you're looking forward to keeping up with me in the future. I am so, so thankful and feel like this blog can finally blossom into something much better
And while it can be hard at times, never forget:
Dum spiro spero.
While I breathe, I hope.
I know Lyss would be proud of this project. Just wait till you see what else I have in store. I miss you, sista, but I promise I'll live in your honor until we meet again.
Through 28 posts, I've managed to avoid any political topics, but that comes to an end here. If you know me at all, you know I'm very outspoken – some would say to a fault. I recognize that my passion can sometimes be construed as a bit too much at times, but I firmly believe that my passion is ultimately rooted in compassion, which can lead me to get worked up. I also give myself a bit of credit that I do try my best to be educated about the issues of which I discuss, but I'll admit I can start to get a little myopic in regards to my own views.
Let me start by saying one thing: I passionately believe that every American should have healthcare; in fact, I believe healthcare is a human right. We live in an era with unprecedented wealth, technology, and medical resources. I recognize that this is not an easy task with a simple solution, but I also refuse to accept that the country with the world's largest GDP has some shameful morbidity and mortality rates, uninsured rates, and other typical markers of nationwide health. America is the richest country on earth, and I believe with that responsibility comes the responsibility of guaranteeing health care to all people here.
Earlier this year, I read a book titled "An American Sickness" by Elisabeth Rosenthal. Rosenthal is a former physician turned journalist that has spent the last twenty years investigating why healthcare has become the big business that it has become. (Note: healthcare, in my usage, is intended to mean hospitals, pharmaceutical companies, insurance companies, providers, foundations, etc. There are ad nauseam moving parts here.) I've been meaning to write a post summarizing the conclusions in her book, with sourced material, citations, etc. It will be a long post with the goal of cliff-noting it so people don't have to read what is essentially a dissertation to reach an understanding of how bad the situation has gotten and how to cure it.
I will write that post, but for now, I'm going to appeal to emotion rather than facts. This post is written from the perspective of somebody viewed as a commodity to the industry. I am not writing to you from the perspective of a liberal, or a scientist, or from politics, but rather, from somebody that is adversely affected by viewing the patients as profits.
This may sound cynical, but when you get into the thick of it, viewing patients as commodities is an inherently capitalist viewpoint. A drug that I'm on – Orkambi – cost nearly $260,000 a year in 2015. Proponents of the free market will usually use the talking point that drugs for orphan diseases, which includes people with CF, typically cost about $1bn to develop. I understand that the money must come from somewhere, but insurers end up facing the brunt of those costs, which only perpetuates the issues we currently face.
But a part of this means that patients with orphan diseases have to be constantly concerned about the fiscal implications of the drugs designed for their diseases; we are conditioned to believe that companies – companies that will eventually profit; executives will oftentimes get multimillion dollar bonuses – are doing us an altruistic favor for developing drugs for us. If a drug can be successful for the community, we have to be worried it will benefit the shareholders. A drug could literally be life-changing for a subset of people with CF – as better modulators are developed for patients with rare CF mutations, the subset can theoretically become as small as less than 1% of the CF community – but since it isn't profitable, it may never reach those patients.
Let me make another point: I work in a lab sponsored by the CF Foundation; I work closely with people at the CF Foundation; I work with reps from pharmaceutical companies; I know people that work for insurers and providers. I am not demonizing the people that work in this industry; in fact, these people are why I'm so passionate about science, medicine, and better healthcare. There are great people at every level in this current framework; unfortunately, the bad actors can easily exploit the current framework to profit and perpetuate existing social, cultural, and economic issues, and oftentimes, I don't believe that the genuine selflessness of some will outweigh the greed of the few. (This is probably a jaded perspective, but being fully optimistic is probably the wrong mindset to take when approaching an issue like this.)
I believe we are currently working within a framework that exploits the people it's intended to protect: the patients. I also believe that free market principles will not solve the ailment that is our current healthcare crisis. Allowing healthcare to be approached like every industry disregards the reality that it is fundamentally different than every other industry; in fact, even calling it an industry (definition "c" from Merriam-Webster of industry: a distinct group of productive or profit-making enterprises) is an inherently self-defeating exercise; it is literally calling parts of the healthcare system "profit-making enterprises," meaning patients are fundamentally necessary for profit. I suppose there is a school of thought that believes it is okay to allow all pillars of healthcare to be for-profit; I, for one, do not subscribe to that belief as being the best healthcare system for all peoples.
And to those people, I ask: what would you say if you or your child was a commodity to other people? To be completely frank, we're all commodities in this framework; not everybody, however, is so blatantly commodified as those with chronic and/or orphan diseases.
For transparency purposes, I'll admit I have a strong opinion. I admit I can strengthen my thesis with more soundly-stated facts, but to begin to broach a societal (no, healthcare is not plainly a political issue) issue like this, disregarding emotion is fruitless. Emotion matters here simply because humans are affected and to disregard emotion is to disregard their humanity.
I invite you to consider my perspective and to reach out and challenge me. I want there to be disocourse. Part of being a writer means exposing myself and generating discussion. Let's get moving on making our society a better place for everybody.
I'm not sure if there is a lot of benefit to me to going through the motions of what I have to do daily and weekly for my CF. I like to educate people – I mean, writing 31 pieces is obviously intended to do just that – but sometimes it feels as though I'm just spending time talking about something I can't change.
What's the goal, then, when I explain my daily routine? I suppose to use my platform to be honest and transparent, and sure, maybe, educating others means they may feel sorry or sympathize with me. Ultimately, I hope people empathize with people that suffer from health issues.
Every morning, I do an inhaler and three nebulized medications. The inhaler, albuterol, is a bronchoilator, intended to open up the airways so the medications can reach more of my bronchii. The nebulized meds are done as follows: hypertonic saline, pulmozyme, and an antibiotic. The hypertonic saline is a mucolytic, which means it's intended to thin the thick mucus in my lungs. The pulmozyme, also called a DNAse, cuts up the DNA in white blood cells, which helps to thin the mucus even further. Lastly, I do the inhaled antibotic. I cycle Colistin and Cayston. These are cycled because it helps to "confuse" the bacteria, to hopefully reduce the likelihood the bacteria will develop resistance to them.
While doing all of these nebulized treatments, I'm actively doing my chest physiotherapy, the vest, to break that mucus even further. I feels wonders better after doing my treatment in the morning. (Since starting Orkambi, I've felt generally clearer. In the years before starting Orkambi, I noticed my chest was feeling very constricted before doing my treatments in the morning.)
In addition to actually doing these treatments, I also have to be sure to keep the nebulizer cups sterile and to clean them daily. All in all, the process is usually 45 minutes to an hour every morning and night. It's a burden that we get no respite from, which weighs on me quite a bit here and there. I've written about how this stress can force me to feel compelled to rebel, but then I remember how sick Lyss felt whenever she was sick, which quells that urge pretty quickly.
I also have to take medications every morning, every night, and every day when I eat. The medications I take in the morning are several vitamins, Orkambi, an anti-inflammatory, a medication that helps my liver, and an acid reflux medication. When I eat, I take enzymes called Creon. These are comprised of several enzymes: protease, lipases, proteases and amylases. Together, these help to break down fats, proteins, starches, carbs, and whatever molecules my body can't break down itself.
Each week, on Monday morning, I spend time preparing my pills for the week and, during Colistin months, preparing my inhaled antibiotic. Fortunately, I can kill two birds with one stone by doing this while doing my Vest, but still, it isn't particularly fun.
Having these types of side tasks on top of all the physical burdens of CF can feel like insult to injury which is why I struggle sometimes. But embracing it, searching for normalcy, being hopeful, and having a good mental health plan are the keys to making it day by day.
Yesterday, in regards to my post on exercise, I received a couple of comments about my motivation. I thought this was really interesting and a good topic to write on.
Truthfully, my motivation resides in what I believe will keep me alive the longest. That’s an unfortunately morbid way of looking at it, but I’ve spent a majority of my life seeing my sister sick and being scared of ever getting that sick myself.
That’s a weird feeling: to fight so hard against this disease with the realization that I’m fighting to avoid my sister’s fate. That’s what’s different about our strengths. Her strength was despite all she went through; my strength is to prevent what she went through.
Alyssa has always been my motivation. For a long time, it was just to capitalize on my healthier baseline and trying my best to avoid a steep decline. Now, its to avoid that, but it’s also to fight in honor of her. I’ve struggled with bouts of depression where I didn’t take care of myself in terms of exercise or sleep, but now I find motivation in knowing that I feel like she’s no longer fighting her disease and she’s on my side, fighting this with me. I find my motivation through her and I always have. I think I always will.
I don’t believe I’m qualified to comment on this, but I suppose my advice for finding motivation comes down to this: find whatever it is that makes you want to be alive forever. And when you find that, find ways to maximize that feeling. I know I’ve struggled with this a lot, but remembering how Lyss didn’t have that luxury the last five years reminds me to be grateful for what I have too.
Motivation comes from within. You will never achieve anything if you don’t first want to accomplish it in your heart. Most achievements require the help of others, but it’s critical to find that initial motivation within. I fall short every single day. But I’m accepting of those shortcomings and I make it a priority to be better after recognizing those shortcomings.
Every morning and every night, I do what's called chest physiotherapy. The specific CPT I do is called the Vest. The vest is intended to help loosen the thick mucus in my lungs. In addition to the vest, I also exercise 1-2 hours a day 6 days a week. I'm active every day of the week, whether it's through taking Duncan to the park or just a jog, I make it a priority to get some exercise in.
I love exercising, but I don't love running. For cardio, I mostly box and do HIIT. I also lift 6 days of the week.
With CF, unfortunately, exercise isn't really a choice. Conditioning helps to loosen the mucus up, but it also improves cardiovascular function, which in turn can improve lung function by opening the airways. With CF health, there's a lot of luck, but for what part of my good health I am responsible for, I attribute it to my adherence to my therapies and my dedication to being active.
I also exercise for the amazing stress relief it provides. There are definitely circumstances where an intense anti-depressant and anxiolytic regimen is necessary, but for me, boxing and lifting provide my mind a respite. It provides me a sense of control over my disease. I'm a broken record, but finding those strategies to provide a sense of control over CF are some of the most important facets of my life. It's how I find a way to normalize myself. It's how I rebalance my hope and excitement for the future.
When I'm in the gym, I don't feel like my CF is out of my control or a cemented future. I feel like I can combat the decline, and honestly, when I box, I imagine that I'm literally fighting my CF. That's cheesy, but I promise, acting like I can literally beat the hell out of CF is a pretty damn good motivator and stress release. It's definitely hard at times when I'm not feeling well – I actually have a cold right now – but I also believe that pushing myself to get a little sweat in helps to beat colds and infections quicker.
And don't worry, I'm fully aware of how big of a tool I am for this post. I have a lot of work to do, but I'm definitely proud of the work I've put in over the last year and a half (oh, and if you didn't know, I reached Peak Athlete last year when I ran the Flying Pig).
Having a chronic disease means you're more likely to have to explain your life to others often. Which means that you learn to navigate the interesting waters of telling somebody you have a chronic, progressive disease – that most people know of because it's life-shortening – and it gets even more interesting when you have a sibling that succumbed to complications from it.
One of the best lessons I've learned from discussing my CF is how important it is to develop the skill of empathy. I've written about this before, but I feel like it's important to really harp on this topic.
It can be difficult for the human mind to comprehend suffering. I understand that there are many others that have it far worse than me, and comprehending their suffering is difficult, but there are also many people that will never have health concerns until they're very, very old. That puts me in the unique position of understanding suffering, but attempting to not become bitter for my complicated situation.
Writing about this is also difficult because I don't intend to sound arrogant or pandering when discussing this, but when you start to realize how difficult others' lives can be, and probably more importantly, how little you really know about what others deal with, you start to become a more compassionate individual. You develop the ability to view the world from the lens of love and compassion, as opposed to bitterness and anger. Trying to imagine how you would feel in someone else's shoes – and being honest with yourself about how you would feel – allows you to better understand the plight of others, and hopefully, allows you to become a better person in the process.
Some of the most amazing conversations have come when I talk to people about my situation and they come away better understanding in the end. They come away realizing how intense CF can be, but hopefully it isn't founded in pity, but rather empathy. It means the world to me when people take the time to learn and understand. It gives me a sense of control in how others perceive me and CF in general. It also makes me happy because it shows that other people care about me. Even people readin my pieces means so much to me because I think it allows people to do an exercise in empathy by trying to learn more about me.
I admit I have a long way to become a better and more compassionate person. I think utilizing empathy and practicing it is the first step to contributing to the greater good. I implore everybody to try to be empathetic today and every day.
One of the coolest advancements in CF medicine in the last decade are modulators. These are drugs that are a capable of successfully improving CFTR function, which means they correct/potentiale the underlying issue with CF: the defective CFTR mutation.
Modulators are so impressive because they show that it is feasible to hope for medications that are going to be so good at fixing the mutations that patients can reasonably expect to see a significant decrease in their lung function decline.
There are three major modulator therapies approved, all produced by Vertex: Kalydeco, Orkambi, and Symdeko. I won’t get into too much of the basic detail, but Kalydeco is the most effective because it potentiates one of the easier mutations of CF. Orkambi is effective in patients with the most common mutation, but since that mutation is more complex, it is more difficult to fix, and therefore it doesn’t improve CFTR function as well as Kalydeco. Symdeko is a newer generation of Orkambi for a similar group of patients, but it’s shown to be safer for patients, though about equally as effective.
Modulators are the reason it’s okay to be excited about CF medicine. They’re groundbreaking drugs that, incredibly, improve the underlying issue with CF. I’ve only been heavily involved with CF science for about 3 years, but I’m not sure if it was previously believed you could have such a significant improvement in CFTR without doing gene therapy.
I want to be careful of prognosticating, but I do believe the cure to CF will be some sort of gene therapy. With that being said, modulators are something to be very, very excited about.
When I think back to my childhood, I realize that one of my biggest sources of anxiety were my CF appointments. Since I was so healthy, I wasn't consumed with my CF all the time and, aside from my treatments, I didn't think much about these. I resented my appointments because they were reminders of my life with CF. As I get older, I've started to understand that these are opportunities to take stock of my health.
At routine CF appointments, you see a lot of providers. I see a dietician/nutritionist, a pharmacist, a therapist/social worker, a doctor, and whomever else feels the need to stop by and check in. Since I see these providers usually half a dozen times a year, I've become close with all of them over the years.
Each appointment, I perform a pulmonary function test or a spirometry. This is how my lungs are measured. There are several measurements in these PFTs, but there's one main one that we talk about. Whenever I say "lung function," I'm referring to my FEV1, or "forced expiratory volume in the 1st second." This is the measurement that helps to measure lung disease, long-term survival, and other important aspects of life with CF.
Over the last couple of years, my lung function has been relatively stable between 80-90%. For aa 24-year-old with CF, that's considered to be mild lung disease. I take a lot of pride in my compliance and adherence to my treatments and exercise and so I worry myself about my lung function quite a bit.
Last year, in January, I did a study trial for a bacteria in my lungs – Pseudomonas aeruginosa, one of the most common ones in CF – for an upcoming antibiotic. After this trial, my lung function improved significantly for me, so my lung function has been a stressor for me over the last year. In November, my FEV1 reached a looooong-time high of 97%.
Today, they were 89%. A year and a half ago, I would've been thrilled with 89% because my baseline was about 85%. I've been stressing about my lung function a lot, but I feel great, so I know that that's just a number. FEV1 can vary drastically day-to-day and there is a 3-5% margin of error per measurement anyhow. So, it's just that, a number, that is used to be a part of care, but it isn't the only part.
My appointment today was a fine one. It wasn't great, but it wasn't terrible. I'd like my baseline to be 95%, but it isn't the only way I'll be content.
There is a lot more that goes into these appointments, but I feel as though it would only get clunky and superfluous if I discuss more of the minutiae of these appointments.
Today, we moved most of our belongings out of the house that we’ve occupied since September of 2015. Since 2008, my family and I have lived in 11 different locations. After next week, I’ll have spent time living in 13 locations.
I’ll be the first to admit that I romanticize nearly everything. I do this because if not, then I feel like I don’t appreciate something. I don’t know if this house meant that much more than our last couple of houses, but this house will forever carry added significance. It was the last house Lyss lived in. It was where I moved after graduating. It’s the house we’ve celebrated at for the last 3 CF walks. The house does mean quite a bit to me and I’m sad to leave it. That’s probably why the last couple of days have been so hard for me.
This is the first major part of moving forward with our lives. I know we need to. I know that I need to stop viewing the past with the bittersweet sheen of nostalgia. Alyssa’s life was full of tough battles and she was miserable a lot of the time.
A difficult part of nostalgia is that you no longer view those moments from the present tense; you view them with the knowledge of what happened. Of course my time in Lexington seems like it was fantastic, I was a college student. In reality, I was stressed constantly with school, missing my family all the time, unsure of my future.
That’s what I’m feeling now. I’m sad that I’m leaving the house that ended up being my sister’s last. This house will forever be that for me and that will always be hard. But it also means I’ll be starting fresh elsewhere. This is hard for my parents and me for different reasons, but it’s a step we need to take.
I recently learned a piece of advice that has fundamentally changed how I make decisions. Instead of spending an inordinate amount of time deliberating on a decision, weigh the pros and cons relatively quickly and make a decision. Afterwards, instead of obsessing over if it was the “right” decision, commit to ensuring that it was the right decision by taking control.
To give an example: I’m anxious about moving somewhere new, but instead of obsessing over how I would’ve rather spent time at this house all summer, I’m committed to using this discomfort to work on a lot of these ideas I’ve had brewing for a lot of time. That’ll ensure that moving into a new place will make me better in ways I’ve wanted to work on for some time, in ways that Lyss would’ve been proud and even encouraged me to work on.
I think being romantic about the past is okay, because it allows you to reflect on how you became who you are. But I think always pretending that the past was the golden years prevents yourself from enjoying the moments as they come.
Not to be redundant, but I’ve had a really tough couple of days. When I started this project, I didn’t expect there to be so many sad pieces. But I’ve willingly accepted that being open and public about my grief may be able to help others. It’s so cathartic for me too. It also means that my writings reflect how I’m feeling on a day-to-day basis. So today’s piece contrasts with yesterday’s quite a bit.
Here’s to the future. Maybe I can channel Lyss’s strength to find that hope.
When I think of Alyssa's journey, it is typically bracketed by the last 5 years. It was on this day five years ago, in the middle of the night, we told Lyss goodbye as she was wheeled off to get her first double lung transplant. The journey began weeks earlier, throughout the entire transplant evaluation process and the move from Kansas to Houston, but for whatever reason, it's always been receiving that call that her donor lungs were ready that feels like the starting point for me.
Over the last five years, this date has carried a lot of significance. Alyssa typically posted about it with a thank-you note to her original donor, Deanna. Lung transplants are not cures for people with CF, but Lyss's first transplant felt like an opportunity to be excited.
But right now, I'm struggling. Everything she went through, the surgeries, the procedures to reverse rejection, the infections, the constant stress, it all feels like we have nothing to show for it. I feel terrible saying that, but it's true. I feel like I watched my sister go through hell for years, only for us to have a taste of hope, a taste of a life of normalcy – the thing I wanted most for my sister – only for it to be five years of a quality of life arguably worse than her years leading up to it.
See, the thing about hope is that maybe it isn't an infinite resource. Maybe it's finite. I'd like to believe with everything in me that we humans are capable of an unlimited amount of hope. Our letdowns have been devastating. It feels like every time we get a surge of hope that the letdown is somehow bigger than the last, despite our familiarity with disappointment. How do I find it in my heart to continue hoping, when it almost seems like forgoing hope for a better future would allow me an emotional sanctuary?
I'm scared of the future. I'm scared of creating more memories without Lyss. When I look at what we went through over the last five years, the thought of my future five years is alarming. I'm 24; it isn't irrational to think my health could deteriorate quickly, but with the advent of better drugs, it's also not out of the realm of possibility that CF could have effectively life-changing drugs come out. So do I remain hopeful that I can start to think ten years into the future? Do I start planning for the possibility of actually having a child? What happens if I start to get hopeful that I may finally have a stretch of several normal years, only to have my lung function decimated by a particularly nasty lung function? The thought of going against everything I've been conditioned to understand about my condition – it's terminal, progressive, painful, life-shortening – may not be in my future into my 30's would be paradise. But the thought of starting to hope for that, only to need a lung transplant and to go through what I watched my sister go through is a fate ten times more painful than the joy of the alternative. I fear the fragility of my mind of having to go through that disappointment.
Nearly two and a half months ago – 57.5 months after one of the happiest days of my life, her transplant – I watched my sister die. I watched as her heart rate spiked, only for it to beat slower, and slower, as her body desaturated, as her organs gave out. I have now been in the company of three of the people that I've loved most on earth as they drew their last breaths. I want to remain hopeful, but these types of experience have a nifty way of draining some of your youthful vibrancy. I realize that abandoning life is something I've contemplated far too much over the last two years and I wish to continue towards a future that is hopefully filled with happy experiences.
But the letdowns. Man, the damn letdowns.
I recall the last five years. When they fly by through my mind, they're accentuated by the best and worst memories, as our lives are defined by those. I recall watching my sister be wheeled back for two separate lung transplants. I watched as she was told, this last time, they had no options. I watched the hope dissipate from her face. In some ways, that news, the doctor then coming to hug her, the disbelief in the room as we realized that there was no timeline where my parents, Alyssa, and I would be together much longer, was the last day of Lyss's hope in life. She wanted to be evaluated elsewhere, she wasn't ready to give up fighting yet. I'm not sure if that was for my parents or my sake, or if she really wanted to keep living. She did not enjoy herself in LA and the fact that she looked so healthy in her pictures accentuates her life so well; she was able to present herself in such a beautiful manner, as her mind was the polar opposite.
I reflect on the happy times, and even those, for me, in retrospect, feel as if there was a gloomy cloud over them. Maybe that's because I know the end result, but I always felt as though rejection could be imminent. It made celebrating monthly milestones feel like we were drawing attention to the fact that we knew Alyssa would not get a lot of mileage out of those lungs.
Writing this piece makes me feel like a terrible brother and especially a terrible person. I feel like I've been pessimistic this whole time. But this day feels like the culmination of an unfair half-decade. I'm heartbroken in thinking about how hopeless I feel right now. I hate this disease so fucking much and I just want to feel normal. Lyss did not deserve the hell she dealt with and the legacy she left only serves as a constant reminder of her breathtaking strength.
I believe I feel this way because this weekend was the biggest reminder in two months of what the world lost when we lost my sister. Today was probably the hardest day in the last two months for me.
I feel hopeless in many ways but at least I'm hoping I can start hoping again.
I want to write a sincere thank you to the many individuals that came together to support my family this weekend.
Life with CF is difficult, but the amount of love and support I felt this weekend was breathtaking. I missed my sister so much. She was always so lively during these events, though she didn't always feel well. It still doesn't feel like she's gone forever.
I have no idea what I would do without the support of my friends and family. Through everything, this support reminds me there is good in this world; it reminds me why I'm so grateful to be alive. It reminds me that there might even be a perk to life with CF.
I think a lot about altruism. Is it possible in humans? In animals? Is it possible to be completely selfless; to do something for absolutely no benefit to yourself? Parts of me say no, even donating money or time triggers the reward parts of the brain and produce serotonin, meaning that a benefit is feeling better about ourselves.
But then I look at this support. I look at the love I have around me. I used to think people pitied me and gave me the benefit of the doubt because I had CF. When I was younger, I always wondered if people were less likely to get angry with me because I dealt with this. As I get older, it seems clear to me that people don't love me because of my CF, they love me despite my CF.
I'm open about my CF because if I want people to know my heart, I want to be open and transparent about the biggest part of my life. This weekend felt like a celebration for my sister. It felt exactly as she would've wanted for me. She would've wanted me to be enjoying myself and celebrating with the people that I love the most, that love me dearly, and that care and miss her so much.
I am so incredibly overwhelmed with emotion. I miss my sister. There is nothing I want more than to see her smiling or to tease her about something. This weekend allowed my mind a respite, but Sunday evening continues to be the hardest part of the weekend. We accomplished another first without her, but the path ahead continues to appear unexplored. I still feel as though I'm navigating an impossible battle.
I wish I knew how to repay the support system I have. I hope all of you know how dearly I love you. As hard as this battle is, this weekend proves to me that there are so many people in my corner and that I'll be okay.
And to The Cold Boys, a special note. I know I've thanked you guys a lot for everything. But I need to do more than that right now. I lost my sister just over two months ago. I know I wasn't the only one that lost somebody important and that this grief affects everybody differently, and Lyss loved you guys so much. She considered you guys to be best friends and little brothers. Over the last five years, I've shamefully been scared of feeling like an only child after losing Lyss. Thanks to you guys, I don't feel like an only child. I sincerely consider you guys to be my brothers and I hope you all know that. I love y'all so much.
Thank you to everybody. This was all for you, Lyss.
Oh, and check out these calves. And these socks. Lyss would've loved them.
I know exactly what she would say.
"I'm fabulous and I'm famous."
We miss you so much, Sista.
Today was the Great Strides Walk. As I wrote the other day, I'm so grateful of people showing up to support Alyssa and me. I've been spending time with them all day. Thanks so much, very much love.
Fundamentally, writing and science have the same pursuits. While one is definitely more romantic and poetic, they both seek to uncover something. The overall undertaking of science seeks out to uncover the basest reality of the world we are experiencing. Writing endeavors to uncover deeper layers of the human experience. All types of writing intend to make you think; fiction oftentimes tries to resemble reality in a way that we'd believe or in a way we wish existed; nonfiction seeks to teach, using words and sentences combined together to educate the reader.
I think this is why I'm drawn to both. Writing is the poetic side of my brain; science is the logical side. Since graduating, I have been trying to find different ways to blend these two seemingly divergent passions. Science can be mundane or awe-inspiring; writing can be cumbersome or inspiring. I feel both whenever I study both. There are times I have an idea for a piece and I'm ecstatic as I start writing, only to get more than halfway through and feel like I'm throwing darts at a keyboard and sounding incoherent. A benefit of this project is that I realize that the writing process is not always going to be fun, but to stick to it, and the rewards will show themselves.
Science is also that way. There are months that go by with zero progress in our lab; we all start to wonder if what we're doing matters. Then, suddenly, one day, it all comes together and it feels like we made a massive leap. In reality, those months are incremental changes that are harder to see. An interesting way to think about this is to think about a sunset; during the entire day, a minute doesn't change lighting much. 2:30 vs 3:30 doesn't look all that much different. But the last hour or two of daylight are full of interesting lighting and shadowing changes; the golden hour shrouds the grass and flowers and subjects in a beautiful golden hue. As the sun sets further, I'm consistently overwhelmed by the sheer beauty of our earth; a beauty that doesn't seem as magnificent when the sun is high in the sky. Science and writing can feel this way at times.
I believe part of the human condition is the desire to be a part of something bigger than ourselves; to be a part of something more concrete than just our internal mind. For me, writing and science are the two best ways of doing so. Studying science and biochemistry have provided me a unique feeling of understanding that is almost intoxicating; writing provides me an outlet to use my words, experiences, rhetoric, and passions to spread a message, to uncover the layers of our collective human psyche, with the ultimate pursuit to share in the feeling that I'm a part of something bigger. The feeling that I am bigger than myself by understanding what makes me the human being that I am and the feeling that I will live on long after I've expired through my writings.
It's a romantic feeling. It's a feeling that I am passionate about pursuing on a daily basis. One of the most fulfilling feelings for me is making people experience an emotion that transcends the moment. I most enjoy making people think and laugh and feel. Writing and science provide me that.
Today at work, a guy in the lab caught a glimpse at the tattoo on my right forearm. Since I have three tattoos on my arms, it's very common for me to get asked about them, but something interesting about the tattoo on my right forearm is that it's an organic compound. Whenever scientists see it, they're always immediately interested in what compound I have tattooed on me.
The tattoo I have on my right forearm is the amino acid phenylalanine. In the mutation of CF I have – ∆F508 – a phenylalanine is missing from my copy of the CFTR protein. I'm fascinated with the concept that lacking a single tiny amino acid can disrupt the structure of an entire protein, which then causes a shit ton of defects on an enormous scale in an organism. (It's a weird feeling to be simultaneously fascinated and overwhelmed by how your disease is caused by some interesting biochemistry.)
Anyhow, for this tattoo – it was my second – I wanted something to represent my love for chemistry and science, but I still wanted something meaningfully related to my CF. I didn't want to do some intricate design of a compound so I went ahead and got a simple organic compound that represents how a tiny amino acid has caused my disease. I enjoy it a lot because it's nerdy, much like myself.
The other three tattoos I have are also symbolic, but I will talk about them in chronological order.
The first one I got is a purple rose with "Dum spiro spero" written above and below it. The purple rose – and accompanying green and yellow stem – are symbolic colors. The purple symbolizes CF awareness while the green and yellow symbolize organ donation. That's also why my blog theme is purple and green. "Dum spiro spero" means "While I breathe, I hope" in Latin. It's meaningful to me because hope is such an essential part of dealing with CF; without hope, my adherence, compliance, motivation, and happiness would inevitably fade.
The second and third ones I got at the same time. The second one was the phenylalanine that I discussed at the beginning and the third is a mute symbol that I got in honor of Aaron. Aaron actually got a tattoo for CF after we graduated high school (also has the quote "Dum spiro spero" on it; he was the second to get a CF tattoo). He joked afterward that he expected me to get one in honor of his auditory processing disorder. Five years later, I surprised him with this tattoo. While it's sort of a "joke" tattoo, I chose it to symbolize my love and dedication to friends and family. I have several friends that have tattoos for Lyss and I, so I thought this tattoo would be a good representation of them and for me to also not take myself too seriously.
My most recent tattoo is the most meaningful and significant, though.
A few weeks before she died, Alyssa wrote this note on a card:
Brah – I love you so much & very proud of you!
Fortunately, I was able to get that note tattooed on my forearm in her handwriting. It's a daily reminder of our relationship. I feel like she's with me everywhere I go and I feel like we're continuing this fight together. She'll forever be by my side.
I think the idea of a legacy is very similar to the idea of a reputation. We can only control them so much and preoccupying our mind with either probably doesn't make that much of a difference in how people view us.
But is that to say we shouldn't be concerned with our legacy at all? I think how we are remembered should mean something to us. How we want to be remembered is reflective of our character, our ethics, our moral code, and the way we made a difference in the lives of others.
I can't remember the exact quote right now, but I believe it was Maya Angelou who said something along the lines of this: People will never remember your words, but people will always remember how you made them feel. That concept sums up pretty well why I've been so consumed with the idea of creation over the last couple of years.
I've pretty sure I've written more pieces on this blog in the last two weeks than I have since I started it, but I feel like I'm finally committing to my aspirations as a writer. I enjoy writing for a number of reasons, but I enjoy putting my thoughts out there into the ether probably the most.
I was talking with a friend the other day and we were discussing the idea of how we are remembered. I'm not sure how we got on the topic other than we were both discussing some recent struggles with grief. Since Alyssa died, I've thought about what her legacy is. It's an American tradition to be very weird and abstract with death; we don't like talking about it and we definitely don't like acknowledging it.
I think that the reason Americans react so adversely to conversations about death is that here mortality and morbidity is constantly getting better. Incidental death is no longer common and medicine has gotten so good at treating illness that people don't die like they used to. Alyssa and I - and tens of thousands of other CF patients, as well as millions of other people that have chronic, life-shortening diseases - don't have the luxury of ignoring death. I view my writing and my situation as an opportunity to change the conversation surrounding our relationship with death. I don't believe we should fear death and I don't believe the reality that we all die should dictate our lives at every turn, but maybe we should confront that reality more concretely.
I think a way of going about this is by zooming out of our day-to-day existence and wondering how people would remember us. I listened to a podcast a couple of years ago that advised the listeners to write the eulogy you believe would be written aboutyou, and then write the one that you would want written about you. The idea was that you would then try to make the decisions and change your existence to suit the one you preferred.
When you consider it all, and when it's all said and done, our day-to-day lives matter so minutely. Sitting in traffic may be the most infuriating inconvenience to happen in your morning, but when you recall some of these seemingly terrible moments, does the feeling you recall of that time you sat in traffic 3 years ago evoke the equal magnitude of anger you felt when you were sitting there? Absolutely not, because you made it to work and realized the consequences of being late weren't that severe. Look, I think there's a reason we get mad about little stuff: because we can. Our lives have reached a point where we can stress about minute things. To some degree, that's a representation of our far we've evolved to not be constantly preoccupied with out own survival. It's okay to be upset about these daily annoyances or else you wouldn't be motivated to get to work to time in the first place. But it's also important to recognize them for what they are: minor annoyances.
I wrote a couple of pieces in January that were intended to generate conversation or at least evoke some thoughts out of the reader. I wrote about the question of what is a life well lived and I also wrote about coming to grips with my mortality. I mentioned this in a previous piece, but both of these pieces were my own internal reflections on Lyss's predicament at the time. I knew, based on the evidence and the way things were going, that Lyss neither had much time nor a high likelihood of getting another transplant. This forced me to confront my own mortality and also to take a step back to ensure I'd be actively pursuing my goals to satisfy the "well-lived" prerequisite.
In creating anything, it's difficult to balance "quality vs quantity." Is it better to produce a lot of mediocre content, or produce very little great content? In my experience with writing, the answer is that neither are true. All great artists produce both great content and bad content, from the perspective of the viewer and from the perspective of the creator. It is not possible to create great art without creating some bad art. Creating is fulfilling because the pursuit of perfection is never-ending, but the result of producing good content is ever-satiating.
I cannot totally control my legacy. I enjoy writing pieces that are essentially just essays on things I think about. I will not make a judgment on if they are great or good or bad or pointless. I have learned that the act of creating itself is fulfilling, but creates an opportunity to hone my craft, which motivates me to create more. I hope my legacy is dynamic and viewed from different lenses by everybody that has ever come in contact with me or my writings. If my legacy is the same to everybody, then that's no legacy at all. My relationship with everybody I know is different so I hope that I'm remembered differently by everybody; my writings should affect everybody differently, as all art does, because polarization can be the instrument of change. I understand that I have been abrasive to some, and while I hope to never do harm, I realize that my vocal nature is an outgrowth of my passion.
Alyssa's legacy is different for everybody. For me, her legacy inspires me to confront my tribulations head-on. I'm motivated to be myself and love myself for who I am, despite my predicament at any given moment. For others, her legacy inspired them to be appreciative of their health and to try to outwardly display positivity.
At best, when I write, I hope I make a difference in someone's life by filling them with some sort of emotion, be it hope, or laughter, or an epiphany. At worst, I hope the reader at least sees a new perspective, though maybe it doesn't affect their daily life thereafter. Maybe my legacy will simply be motivating people to consider their legacy. Maybe it will be nothing more than me exploring life outwardly as a writer. Who knows, it could be something I don't even know I'll be interested in in the coming years.
I know I can't control my reputation or legacy; I can only control my life.
And I think I'm beginning to be okay with that.
At first, I thought it might be a bit odd to wax poetic about Duncan for several hundred words for this project. About 2 seconds later, I realized a memoir or biography of my life would be incomplete without a chapter about how big of a part of my life Duncan has become.
If you read my first post, you know that I do not take anxiety or depression lightly. But I can say, aside from Zoloft, the single biggest help for my anxiety and depression has been Duncan. The amount of love I have for this dog is genuinely hard to put into words. Developing a bond with him has been so important to me and I can't stress enough how much I care and worry about him.
Humanity's relationship with dogs is an interesting one. In some ways, I almost feel guilty about our collective domestication of dogs. I feel weird when I realize that he ultimately has no control over his life and that he fully relies on me. But I also feel responsible for his well-being and that has taught me a lot. I joke a lot about how I have learned what it's like to be a father because of him, but that's actually true in a lot of ways. I can now understand why my mom worries about me so much; I can see why my dad wants to steer me in the right direction when it's clear that I'm making a mistake.
Duncan is such a precious part of my life and has been such a crucial stress relief. Whenever I'm having an anxious time or need some time alone, I take him on drives or walks at a park. In those moments, it's the most surreal peace I feel during my entire week. I don't feel consumed with the stress of CF or my job or the burden of the world or my grief on my conscious. Spending these moments with Duncan is what gets me motivated to pursue my passions. Trying to put this dynamic into words feels fruitless. At this point, this piece feels emotionally weak and pointless, but, if you've seen me interact with Duncan, you have seen that there is never a time I'm not excited to see him.
I talked about him during my talk at CF Family Day the other day, and when the picture of him and I pops up on the screen, it's obvious how much I love this dog. Talking about him makes me happier than anything else. He's my best friend, so why wouldn't I write this about him?
Spending these moments with Duncan feels like how it felt to be a kid. Having Duncan around means I never feel totally alone, it means I feel like I always have a reason to continue on. I'd be lying if I said I don't vent to him even more than my therapist. He's the best listener and gives great advice (likely because I even talk as him so I give myself my own advice lol).
Growing up, our family dog Bo loved my mom more than anybody else. I always teased him so he didn't love me very much and would growl at me all the time. But Bo, then Coco, were both dear parts of our family. Having the dogs around made Alyssa and I happier. I was so thrilled when I realized that Duncan has a very similar relationship with me as Bo did with my mom.
Whenever you're fully prepared, get a dog. Treat them with the utmost love and give them a chance to develop their own personality, and they will reciprocate a love that humans can't mimic.
Some scientists believe that maybe dogs domesticated us; since we provide for them and take care of them and they have to do practically nothing to survive, then maybe they're our masters. It's an interesting thought, and based on how Duncan pretty much gets whatever he wants, I think there may some truth to that.
Every year towards the end of May, there are hundreds of walks across the country to raise money and awareness for CF. This weekend, on Saturday May 19th, the Cincinnati Chapter of the CF Foundation is hosting the walk at Sawyer Point. For as long as I can remember, these weekends have been some of my favorite.
During CF walk weekends, Lyss would always joke about how she was famous, and by extension, so was I (on a serious note, Alyssa knew a lot of people so she definitely seemed famous sometimes), based on how many people would attend in support of our team, 65 LaRosas.
Rather than enjoying these weekends because they bring so much attention to CF, I actually think I enjoy them in spite of them focusing on CF. Or maybe it's less that it's about CF and it feels like it's the only weekend of the year that I allow myself to recognize how much Alyssa and I have dealt with over our lives and it feels like a weekend where I allow us to be celebrated.
I enjoy being able to spend some time with my worlds clashing; my friends and family all in the same area and celebrating afterward. It's so much fun to see everybody interacting and taking the time to come together for a bigger purpose in pursuit of a better world for people with CF, and if I'm being honest, for people to show us how much they care about Alyssa and I.
For obvious reasons, I feel anxious about this weekend. Lyss was a social media savant and was probably responsible for the majority of our fundraising every single year. Though this weekend is supposed to be for CF, to me, this weekend is not about that at all; to me, it's about my sister. I want to celebrate her life and I want to celebrate how instrumental she was in spreading so much awareness. I want to honor and remember her because I miss her so much and the world keeps spinning without her, but my world is eking along.
I'm so grateful for how much support I've had over the last two months and I'm looking forward to that coming together this weekend in the form of dozens of friends and family being there at the walk. I have a little surprise for everybody and I'm excited to show everybody.
There is still time to sign up to walk with us and donate (you'll have to copy & paste the link into your browser because I don't have an embed link, sorry):
Also, special shout-out to my coworker Cindy for buying a shirt and rocking it with me last week!
Today almost feels like insult to injury. How is there any way we can celebrate today without Lyss? My birthday was hard for me, but a day designed to celebrate mothers just two months after losing a child feels unfair. I'm upset today so I can't imagine how my mom must feel. So this piece is a letter from me, on behalf of both Alyssa and I, to my mom.
Alyssa and I could not have had a better mother in any world imaginable. I am so sorry that you have had the stress of worrying about our health, but I hope you know that Alyssa loved you more than she could've ever put into words. You were her best friend, her biggest fan, and her biggest advocate. I know I can confidently say that it bothered her to her core that you had to worry about her so much for so many years. I also know she was so worried about how you would handle all of this. She was so scared to leave you, but I am so proud of you. I'm so proud of how you've handled everything and kept your head held high. She is beaming with pride in Heaven looking down on you.
A weird part about siblings with a chronic disease is that one is inevitably going to be healthier, especially when considering the fact that females with CF tend to be sicker than males. This means that one child is naturally going to require more effort and worry, but I hope you know I've never felt unloved or that you focused on Alyssa more. I've taken my care into my hands so much for that exact reason: so I could relieve a good amount of that burden in whatever way I could. I know you worry about me so much and – as a father of my perfect son, Duncan – I'm realizing as I get older that I have to let you be my mom sometimes. I'm thankful to have you worry about me so much because it shows how much you care. I try so hard to be compliant and adherent to alleviate that stress. You seeing me sick is one of my greatest concerns and it is one of the most important reasons I fight so hard.
I have no words to thank you for being my mother. I will never be able to thank you for everything you've done for me in my life. I am truly so genuinely fortunate to have you and dad in my life. I hope to always make you both proud in everything I do. I promise to always fight for myself and for you guys and in honor of Alyssa.
Today sucks and there's no way around it. I know that Mother's Day will be hard every year from here on out. How do we navigate this day in the time after Lyss? Do we try to start new traditions? I don't know. I hate that this day pushes all of that grief right into the forefront of our mind yet again. But I hope you can reflect a little and find some solace in knowing that Alyssa's quality of life – and everybody's who knows you – was made infinitely better by having you in it. She knew that and I know that was probably the hardest part about all of this for her.
I love you, mom.
Your kids forever,
Lyss & I
This morning I spoke at CF Family Day in Lexington. I also spoke last year – since I've gotten a lot more comfortable, I think I did much better today than last year – and am so thankful I've been able to do so both years. I was super anxious and missed Alyssa all week thinking about doing this talk, but I'm glad that I ended up doing well in honor of her. She was so passionate about advocating for CF that I feel like I'm continuing her legacy in this way.
The talk today was a nice reminder of why I'm doing this project. Since I was talking to an audience of family members of people with CF (CF patients aren't allowed because of infection policy so yours truly was the only mutant present), I was able to not focus on the specifics of life with CF which aligned with my goal of this project. I focused on my goal of normalcy (and of course discussed why I dislike that word) and how I've coped with all of this for so long.
I try to avoid self-aggrandizement, but today's talk included me discussing some of my accomplishments. I don't view myself as a role model, but I can imagine how difficult it may be to not know the future of your child (I mean, I stress out about Duncan soooooo I already know I'm going to be a high-strung parent), so I hoped that my discussion of how I've embraced CF was some comfort to the family members and a reminder that it's possible to have some control over how we handle all of this.
I feel so motivated today. I feel like I'm getting better at speaking publicly and delving further into the social commentary around CF. I feel like Alyssa would've been so proud of me today and I'm thrilled with the speech that I gave (not self-aggrandizement, it's self-love!!) because I was pretty anxious about it all week.
To any CF families reading this post: the future is bright for CF medicine. It's hard to see that sometimes. Trust me, I know, I feel so sad that Lyss isn't here to see this, but I'm feeling more passionate than ever. I'm happy with how this project is going! People have given me fantastic feedback and I'm enjoying the challenge. In some ways, it's becoming a bit of a diary.
I'm feeling full of love today. Much love to all and thanks so much for being a part of my life.
"Normalcy" is a bizarre concept. I'll be the first to admit that I hate social constructs and the just going along with the status quo for the sake of it. But feeling normal is a critical part of growing up. Having CF meant that I grew up not feeling normal at all. I got adjusted to delicately explaining CF at a young age. There were definitely weird learning curves and it wasn't always easy. But I am eternally thankful for my teachers along the way for making things easier on me.
When you're growing up, from 1st through 12th grade, you spend about 35 hours a week at school. CF presents a litany of problems that are tough to deal with at school so having understanding teachers that navigated the strange waters of dealing with my health was one of the most valuable parts of my adolescence.
I had so many teachers that worked with my family and I to alleviate whatever burden I would have when I was sick or when I had surgery. I had help from the administration to find ways to assist in the issues that come with being a student and having a chronic disease. In terms of my academic career, I felt like these teachers helped me obtain a level of normalcy I wouldn't have been able to obtain otherwise.
Maybe more importantly, my teachers believed in me. They believed in my passion and my drive and they encouraged me to fulfill my potential. I believe in myself because of my teachers believing in me. I would be mistaken to not give my teachers credit for their support because I can say wholeheartedly that I would've never fallen in love with learning without their drive to be the best teachers in the world. I can say that writing would not be a dream of mine without them instilling a passion in me for learning, self-evaluation, and dreaming big.
Life with CF can be difficult in more ways than I can write. But life with CF provides me with an opportunity to allow people's true colors to flourish. Unfortunately, I've seen people's true colors be full of hate, but that also means that I seen people show me there are genuinely well-meaning souls that are comprised of optimism and love. Some of the most important people I've developed relationships have been my teachers over the years, and their sympathy and empathy for my CF is what drew me so closely to so many of them. I am so thankful for so many teachers over the years for believing in me.
When I try to remember her voice, her laugh, her face, my mind fails me. It hasn't even been a full two months and my memory seems to be betraying me already. I remember what we'd talk about, what we would fight about, but I can't hear it in her voice anymore. I remember how she would say things, but I can't hear the inflection in her voice. I can't hear her laugh. I can't picture her smile.
Today, I was scrolling through recent Facebook pictures to find this specific one of Lyss and had one of the most haunting realizations I'd ever had. I looked at a picture of her, one from just a few months ago, and she looked...different. I can't explain how she looked different, but in a way, almost unrecognizable. She looked like someone from my past, in the way that you may recognize somebody that you knew in high school, but never spent time with. In a way where I can't believe I had over two decades of many memories with her.
If this is already what's happening to my memory of my sister after 59 days, what is is going to be like a year out? Several years out? Will all of my memories of her be real or will they just be my own internalized creation of who she was?
Grief has a funny way of reminding you that you aren't supposed to feel okay. I go through my days and the relief of not worrying about my sister has provided a lot of comfort. But the specter of grief reminded me today that, in the absence of worrying about Lyss, my mind is filled by a newfound absence: fading memories. When I notice that it's been almost two months already, my mind spins. Two months seems like an eternity and an instant. I don't have a grip on how long a minute is anymore. I've struggled to sleep a full night's rest every day for two months; I've gone about my life, searching for fulfillment, towards an uncertain future, every day for two months; I've filled my mind with new memories, in a world without my sister, for two months.
I often wonder if our minds are capable of holding only a finite amount of memories. The science seems to support that to some extent. We don't necessarily push them out in the order we experienced them, but rather, each time we recall a memory, we coat it with the sheen of who we are at the time of recollection. I think there's some truth to the fact that our mind operates on autopilot sometimes to conserve space for the upcoming valuable memories, yet I wish I could just permanently save my most treasured memories of Alyssa and to hell with any upcoming ones. My memories of Alyssa over the last five years are shrouded by my growth into her little, yet protective brother. The fact that I grew into an adult, and as I grew older, my opinion mattered in her medical decisions, means that in some ways, I felt like her older brother. She was my big sister in mostly every way, yet that she respected my inclinations to be always looking out for her health.
Over the last two months, I've recounted some of my last experiences with Lyss and some of her last times doing some things. There was a last time she laughed, a last time she felt love, a last time she thought about me or my parents and so many other people, a last time she worried about my health, a last time we talked, or a last time I teased her. Life is perpetually unfair, but death, death is so, so final. For every day of my 23 years and 9.5 months, Alyssa was in my world. All of my memories, whether she was by my side, or halfway across the country, my mind was creating those memories with the knowing Alyssa was actively in my life, until she suddenly wasn't. My brain has spent the last two months trying to create new memories, all while realizing she isn't there.
I think that when I've been creating new memories, the pain of her death is still so palpable, my mind has been numbing some of the triggers. Maybe, as a sort of defense mechanism, my brain didn't immediately recognize Lyss to save me the pain that seeing her face or hearing her laugh would cause. Surely, my subconscious brain should've then predicted that the emotional pain I felt when it seemed like I didn't recognize my own fucking sister would've been exponentially worse.
Grief is so personal; it is a newfound territory that we all have to navigate differently. There is no guidebook that can conform to what we need it to. What could anybody say to me that would provide me solace today? I didn't want to talk about it to anybody, I only wanted to write about it.
Since cystic fibrosis is the most common genetic disease in white Americans, it's important to understand how it is inherited. As we understand more and more about CF every day, we are recognizing that it is a more complicated disease than previously thought. But for simplicity's sake, we will discuss inheritance of the most common mutation, ∆F508 (pronounced delta-F-508), which Lyss and I have.
∆F508 means that there is a deletion (indicated by the ∆) of a phenylalanine (the amino acid) at the 508th spot in the gene that encodes for the CFTR protein. As I mentioned in a previous post, CF is almost more like a collection of diseases that are caused by different mutations that end up disrupting function of the CFTR protein, and these mutations can disrupt the protein in different ways. These different ways are usually grouped into five classes, but for now (again), I will not be going too deeply in the biology and biochemistry of it all.
We inherit a copy of each chromosome from each of our parents. Cystic fibrosis is inherited through what is called an autosomal recessive pattern. This means that, to inherit CF, you must received a mutation from both of your parents. This essentially means that parents of children with CF – carriers, as they're called – have worse CFTR function than wild types, or people that have both copies of a normal CFTR gene. It isn't known exactly, but the common belief is that at most 50% CFTR function is required to have zero clinical presentation of CF. That could mean that, for some people, you only need 20-30% function. It's impossible to tell right now due to so many other genetic factors. I'd surmise, by my lab experience and own background, people with ∆F508 typically have less than 5% CFTR, and probably closer to 1-3%.
In biology, a way to demonstrate inheritance patterns, especially one that obeys such simple Mendelian genetics like CF, is what are called Punnett squares.
(For the record, I am trying to get better at photoshop and stuff and spent about 20 minutes trying to make a graphic to better describe this and it didn't go well. I'll get better soon, I promise.)
In that chart about, the top row indicates the two copies of the gene in parent #1. The large C's represent wild type copies of the chromosome, while the small c's represent mutant copies, or "CF" copies of the CFTR gene. The first column represents parent #2. As you can see, both parents are carriers of CF, while not actually having CF. The term to describe them are "heterozygotes."
Punnett squares are used to map out the possible outcomes of two people having children, but they are simply a formalism to demonstrate likelihoods. In reality, it possible for two adult carriers to have 4 straight children with CF or 4 straight children that don't carry a copy of the mutation or any iteration in between.
This means, when two carriers have a child, there is a 1 in 4 chance that child will have CF, as indicated by the "cc" box. This also means that there is a 3 in 4 chance that child will not have CF, as indicated by the "CC" and "Cc" boxes. However, there is a 1 in 2 chance that their child will be a carrier, indicated by the "Cc" boxes, or heterozygotes, otherwise called "carriers" like their parents. While that child will be healthy, it is possible that CF will still pop up in future generations. The other 1 in 4 chance, the "CC" box, is what called a homozygote recessive, which means that child has both normal copies of the CFTR gene.
One of the most common questions I get asked is "Man, so what is the likelihood that both you and Alyssa would have CF?" Even though each child has exactly the same chance of having CF, it is still possible to calculate the likelihood that both children will have CF.
To do this, you multiply the likelihoods. Since Alyssa and I both had a 1 in 4 chance independently, or (1/4), to determine the combined chance, you multiply those together. The likelihood we would both have CF was a lowly (1/4)*(1/4) or (1/16) chance. In a percentage, that's 6.25%...so not very likely.
A common discussion in the CF community is whether or not parents should have a second biological child once they have already had one with CF or once they are aware that they are both carriers. That's a discussion for another day, and to be honest, that discussion becomes a bioethical, moral, philosophical, and even religious discussion for some so I'm not sure I'll touch on that (I definitely would like to so I probably will. We'll see.). I'll explicitly state: neither Alyssa nor I hold absolutely any ill will towards our parents for choosing to have multiple children, especially in my case, knowing that it could happen again.
Lyss and I were close for a lot of reasons but I think having CF drew us closer. The life we had as siblings was undeniably defined by our concern for each others' health and well-being. In that way, I'm glad we were able to battle together, so maybe that 6.25% chance was a victory in some way. I'm not too sure but I hope I explained all of this clearly.
Part of being outspoken and transparent about your chronic disease is that it immediately grants you a platform. As demonstrated by this project, I'm trying to be opportunistic about this and I'd be lying if I said I wasn't trying to expand that platform to a bigger audience. But, as a scientist, writer, and human, I feel a lot of honor and responsibility by having this platform.
I'm writing this because this Saturday, I'm speaking at the CF Family Education Day at the University of Kentucky clinic and just a couple of weeks ago, I spoke on behalf of Alyssa and myself at the CF Foundation Cincinnati's Finest Event.
I realize that my background as a scientist – and ahem, a person that's dealt with this disease for 24 years and counting – means that people give me the benefit of the doubt that what I'm saying is at least somewhat accurate. I recognize that my opinion and my statements hold real estate in people's mind when I talk about topics that people respect my opinion in. As a writer as well, I understand the immense power that words and rhetoric are capable of. I also believe that when people weaponize words, in whatever form that may be, it's important that they're held accountable. (When I say "weaponize" words, even my posts that are intended to elicit empathy use that technique, I merely mean when words are intended to have an effect, which spoiler: whenever someone's writing, they should always be intended to have an effect, so my point here is people should expect to be held accountable for the words they say or write.)
I consider a platform, even one as small as mine, to be both a great privilege and a tremendous responsibility. I believe strongly that when individuals develop reputations that they need to be honest about their intentions and their motives and I hope to be clear about that whenever I speak on topics related to CF or really anything. I take great pains in researching the topics I discuss and being careful about the words – for example, in my "Some CF Stats" piece, I intentionally cited the pages where I used numbers for populations and stadium capacities; some of the pages cited were Wikipedia which IS a good source for a lot information, by the way, but that's for a different day – I use.
In science, it is of utmost importance to be clear. It is also fundamentally important to be concise. While my inclinations to speak in prose may inhibit my ability to be concise (unintentionally superfluous), I sometimes use more words to explain things with the goal of being clearer. That's partially why I haven't written that many pieces about specific CF stuff – inheritance, mutations, etc – because I know I will feel compelled to try to explain background biochemical and biological topics that will overcomplicate the entire message for probably no good reason. But I also feel like explaining other parts of dealing with CF – having the responsibility of a platform, why the term "sick" can have a bad effect on us, or just being honest about my feelings – can have the same intended message that life with CF is vastly complex.
In a community of such a small amount of people, we must be careful that we do not abuse our platforms. I'm active on social media, so I see many people with CF and affected by CF being active in this month of awareness. Unfortunately, I've seen people abuse the power that comes with others assuming that our experiences provide us with expertise in areas we may not have expertise. If we're not careful, we can inadvertently infect the discourse surrounding life with CF. I absolutely do not consider myself to know everything there is to know about CF, but I do know that my experience in the lab as well as my degree provide me a wide breadth of knowledge in this field. I'll speculate based on my know-how, but I hope I can always make an informed comment with the hedge that it's speculation. This is part of my platform that comes with my science background. (I sincerely hope this doesn't sound self-aggrandizing because it absolutely isn't supposed to be. If you know me, you know I'm very, very hard on myself. I'm only trying to speak to the fact that I try to be careful about what message I'm sending because I want it to be as honest and factual as possible.)
I feel like Alyssa left a great legacy on earth because of her platform, yet I also feel like Lyss never felt like she could feel sick and just feel sick because of her platform. I could be wrong, but I know Alyssa took a lot of pride in her reputation as somebody that always embraced the good and looked forward with a hopeful disposition. That platform, in a paradoxical way, was what gave her a will to fight and also what burdened her in her fight.
Sometimes, I reflect on where I am and notice that I could've never imagined I'd be in my current career path. I always thought CF gave me an inherent passion to pursue in medicine, but now that I feel like my career path has been hugely altered since college graduation, that only I am capable of being in this position due to my unique situation in life. Life with CF has given me something to force me to reflect on life and a ton of interesting experiences. Watching Alyssa go through what she went through provided me with a type of suffering the most people cannot even fathom. I wish both weren't true, but great tragedy can be fodder for great art (bold reminder here from Day 1: DO NOT under any circumstance romanticize your depression as the fodder for great art; it is not the depression or anxiety, but rather the experience itself that can be the good material; again, please seek help if you need it).
So I embrace that platform. I embrace the legacy Alyssa left for the world and the legacy she left for me. I admire the path she carved, but I plan on carving my own. I hope to use my writing to show a human being going through human emotions. It took awhile for me to speak about my depression and anxiety and even longer to write about it, but I received some really nice messages about others having gone through something similar. If I helped a single person, that piece was a success. I write with the goal to inform others of my story. I don't consider myself to be "inspirational" and I don't love that word anyway. I enjoy writing because it's extremely cathartic for me. And I enjoy having a platform because I love the idea of using my experiences to make a difference in the world.
I enjoyed talking at Cincinnati's Finest a few weeks ago. Most of the people that raised thousands of dollars did not know much about CF. I enjoyed being able to personally thank them. Alyssa wanted to speak at that event badly so I enjoyed speaking because I know she would've been proud of me.
I look forward to speaking this weekend (my second time at the UK family education day) to be a testament that people with CF can live fulfilling lives and embrace their experiences to hopefully one day make a change, just like I hope I can do and I know Alyssa did.
Please hold me accountable if it seems I am being disruptive or inaccurate. I look forward to the challenge of having this platform and I hope I can become a successful writer, advocate, and human.