Stowe, Vermont – CFF Research Conference

Vermont, June 23rd, 1pm Eastern Daylight Time

I’m standing near the summit of the tallest peak in Vermont: Mt. Mansfield. The wind is gusting, visibility is mostly clear with the sun out and blue skies, punctuated by occasional poofs of airy clouds. I can see what I think is Lake Champlain? Is that the direction of Maine, of Canada? Somebody had mentioned Lake Placid is near, too, I think. It becomes evident to me that my geographical perception of northeastern America is lacking, but oh well.

Vermont is exactly I envisioned it. And yet still more beautiful when seeing it in person. Where I stayed was a world away: two flights followed by a 50 mile drive through mountains, folksy towns, lucidly lush, green tree-lined roads. The people in Vermont were some of the kindest people I’ve ever met. The forty-eight hours I was there flew by. Still, I felt like I did so many things. 

As I stood in one of the most beautiful places I’ve ever been, panting due to thin air and a rushed heartbeat, the last 25 years of my life crystallized in front of me. The geography in front of me felt humongous, accessible, open. It mirrored my life going forward. A million pound weight rose – no, floated effortlessly – off of my shoulders. 

Kentucky, The week of March 12th, 2018

When you’re in the depths of grief or depression, an open world with possibility feels as far off as the end of the universe; It is unimaginable, unachievable, undesired. When you’re at the top of a mountain, the path behind you, the path already traveled has been etched into history. If I can surmount that, what else can I do? 

In the days leading up to my sister’s death, my world hung in the air. I was wishing that I could just begin the process of grieving before she was already gone. As I watched my sister die, I felt a visceral sense of dread. My life was being irrevocably split in half. For so long, I struggled with my own happiness. My happiness was a bargaining chip. I was nearly convinced that in a past life, I accepted a devil’s bargain: Stable physical health in exchange for never experiencing true happiness. I struggled as I watched my sister spend day after day feeling like shit, incurring physical scar after physical scar, mental trauma after mental trauma. It was not lost upon me that this is possibly the life I may one day have to experience. The mental burden of watching my sister’s flailing health for two decades was my punishment for being healthy. As she experienced such physical agony, I was characterized by mental anguish. That was only fair. How does one look forward to a life confined to scarred lungs as they filled with mucus inhabited by antibiotic resistant bacteria? I believed my circumstances made me stronger. What could be worse than watching your best friend – the one person in the universe that you feel like you can genuinely relate to – die? What could make you stronger than going through such a horrible tragedy?

It was in the days after her death that some important seeds were planted. My family was surrounded by immense support and social media saw an outpouring of kind words about my sister. People shared their stories of experiences with Alyssa over nearly 30 years. Many said they found inspiration in my sister’s relentless spirit, always pushing forward no matter how much pain she was in. Some mentioned how they adored her candor about her experiences, particularly when she felt badly but still shared pictures of her looking upbeat. Of all her passions, her love for fashion was the most talked about. The week after her death solidified one of my long-held beliefs: Humans are inherently good. They want to help, to support, to show love. Sometimes it takes tragic circumstances for that to show, but the important point is that it is shown. 

The other, possibly more important, seed that was planted that day: Life is short and we need to find a way to maximize our days. For two years before her death, I didn’t call myself a writer. I hesitated to share my deepest thoughts on heavy topics like death, spirituality, life, fulfillment, grief, or mental health. Although these topics were important to me and I had already spent so much time thinking about them, I felt I was too young, too naïve, not damaged enough, to share them. Once I accepted that my trauma was real and how my experiences had transformed my world in uncommon ways, I stopped being afraid that people might dismiss me. That was up to them and clearly people could make their own decision, but I needed to do what I needed to do to be content with my life. I needed to make sure I continued my sister’s legacy and part of that was learning how to proceed with my life.

Things didn’t change over night. It still took time: I wrote pieces that went unpublished and published pieces that went unnoticed. My friends and family believed in me, but believing in one’s self is the most important part of the recipe. 

The week after Alyssa’s life and this past weekend in Vermont are inextricably linked in my life. Back then, no one could’ve convinced me that I would’ve had the courage or optimism to fly to Vermont and speak to a room of 150 of the most prominent CF researchers from across the world. In the audience, and seated next to me at dinner, were the current CEO of the CF Foundation, the incoming CEO, the chair of the board, and a few other senior directors. Speaking candidly in front of this audience was one of the proudest moments of my life and one of my biggest achievements. 

My talk ranged from my personal life with CF, why my parents inspire me so much, some of the toughest parts of Alyssa’s life, the mental health burden that comes with CF, my dating life, science at large, and my experience in the lab. I joked with the crowd that I was thankful for them for two reasons: The amazing research they do, and for providing me a stage where I could talk to 150 therapists listening to me bare my soul. This version of my life doesn’t exist without the week after my sister’s death where I was the shown the power of the human spirit and the world around me – and the future that I had just lost – convinced me to allow myself to begin living.

In the second act of my life without my sister, I’ve had many weeks where I’ve felt generally okay and many weeks where the most difficult task in the world was rolling out of bed in the morning. There have been times where this all feels for naught and also moments where I feel more alive than ever. I’ve learned how to balance these two extremes in the most human way possible: By sitting with them. They are natural emotions and it’s taken some time and the support of a mental health professional for me to recognize I am not inherently flawed for feeling this way.

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In Vermont, I stood on three mountains this weekend. The first was Mt. Mansfield, the second was the platform at the CF Research Conference, and the third was the peak of my life since I lost Alyssa. 

The world around us is beautiful and expansive. We are stronger than we know. I am thankful to be alive. I am thankful to have had almost 24 years with my sister. I’m lucky to have the lessons she taught me about life, fulfillment, believing in myself, and perseverance. 

Life is inexplicably hard at times, but it’s also the most beautiful experience. We come to believe there will always be another day for us to enjoy the weather, to start a new hobby, to have quality conversations with friends and family, to take that trip.

It isn’t until we learn that there won’t always be another day that we can begin living the life we deserve.

Because there won’t always be another day.

TL


My Thoughts on "Five Feet Apart"

Back in October, the trailer for “Five Feet Apart” was released. I made a video of me reacting and offering commentary on the trailer. I also had a passing thought that maybe I would try to organize a private screening of it at a nearby theater since it would be be coming out right around the anniversary of Alyssa’s death. When I saw the trailer, I had immediate reservations. As far as I know, it wasn’t particularly well-known in the CF community that it was being produced. I learned that a few adults with CF and clinicians, including Claire Wineland (a well known CF advocate), had been consulted.

One thing about the CF community, however, is that it is a population of over 30,000 people in the US. CF is also a disease that affects every single person differently, which means our experiences with the disease are incredibly wide-reaching. That also means that our opinions about all kinds of issues related to CF are also wide-reaching: Many of us have strong opinions in both directions about the CFF, Vertex Pharmaceuticals, “inspiration porn,” and so, so much more. The reality, then, is that producing a film and not a documentary will inevitably not encapsulate a very wide spectrum of experiences and opinions in the community. If the film were to be produced well, it is possible that it could do great things for our community. On the other hand, if it were produced poorly, the community could be seriously adversely affected.

Most people are familiar with the expression “All press is good press.” That was the response I saw every time somebody rang the alarm bells about “Five Feet Apart.” It is true that people simply being familiar with CF will likely mean that more money will flood into the coffers of the CFF and other related foundations. Press and more money for foundations probably means better awareness and better drugs on the horizon. In the long run, that’s probably a good thing. With the wrong portrayal, all of this may still be true. With the wrong portrayal, however, our day-to-day lives could be negatively affected. Ableist language is already so common in our everyday language, especially when people talk to those of us with CF. People with CF are so commonly referred to as being “inspirational” for simply waking up and keeping ourselves alive. The damage from a poorly produced film could lead to us having to always stifle off pity when all most of us really want is empathy and to be treated like we are normal human beings because that’s exactly what we are, we just have shitty CFTR function.

These were my reservations about the film. Sure, we shouldn’t have strong opinions about something until we see it, but raising points early on in the press tour was the community’s effort to ensure that our voice was heard which, if the film is being produced for the “right” reasons, is exactly what our community deserves – our voices are what need to be at the front of this conversation. That includes the voices of those pissed off about the film, those that are ecstatic about it, those that refuse to see it, and it also includes all the voices in between!

So I suppose it is time to share my thoughts on the film. All in all, I was fine with it. It was clear that director Justin Baldoni took some creative liberties with the film, the most blatant being the hospital stay being portrayed as such a fun experience with the pool, the game room, the exercise room, the rooftop and the constant hang out sessions with others with CF (albeit six feet apart from one another). The benefit to the hospital being portrayed this way, however, is that our lives are interspersed with terrible and annoying shit – our treatments, medications, coughing, and all that – and fun lives being lived. We are humans, after all, with a full spectrum of emotions and experiences. The heartbreaking truth that CF interrupts our lives was palpable. I felt that best represented when Stella has to FaceTime her friends from the hospital when they are out partying and she’s confined to a small white-walled room. Stella, like Alyssa used to, made her hospital room her home and I loved that part. These hospital stays are psychologically damaging, so finding a way to make them feel like home is so important.

There were a few things that made me uncomfortable. At times, it felt like an advertisement for Afflovests and Vogmasks. Vogmasks aren’t exactly endorsed by the CFF, though they are very commonly used (my sister had them too). The Afflovest is used by some in the CF community, but I’m pretty certain Hill-Rom’s The Vest and their newly released Monarch portable vest are the gold standards. That isn’t a huge nitpick – chest physiotherapy and aerosolized medications were still featured prominently which is what is important – but product placement is a bit of a bad look in my opinion. I also don’t think the transplant process was portrayed nearly as detailed as it could have been, but it’s likely that wasn’t a focus point due to time constraints.

I do still feel a bit uncomfortable about it being a love story and how that is supposed to wholly represent the struggles of us not being able to be close to one another. In my view, people with CF don’t hate cross-contamination risks because it means we can’t date or hook up with one another. We hate cross-contamination risks because it impedes us from ever being able to have a true friendship or relationship with the very people that can most empathize with us. I was lucky growing up because I had my sister. While cross-contamination is a real risk for siblings, it wasn’t one that was going to prevent us from being siblings. I had Alyssa so I could talk to her about CF. Since she died, I’ve become closer with a lot of people in the CF community and those bonds are real. It does suck I can never hang out with them in person, but for different reasons than how the six-foot rule is portrayed in “Five Feet Apart.”

While the love story is obviously chosen to appeal to a young audience, I think one of the most important points of emphasis in the film was its sheer boldness in talking about mortality. I’ve been very open about my disillusionment with how CF steals life away from young people and how it forces us to think of such heavy topics at a young age. I’ve also been very open about my opinion on how CF has provided me with unique wisdom, experiences, a career, and a platform. 

I don’t think there is a lot of benefit to going through the film and dissecting every little detail. In all movies, and even in documentaries, creative liberties are taken, so it’s genuinely impossible to get an entire picture of what the CF experience is like. The biggest point I will make is that this movie essentially shows the experiences of three teenagers with CF. Some people are meticulously compliant whereas others don’t quite see the point. Both experiences are valid. I want to emphasize that every single experience and opinion about the film is valid. It is totally okay that some are weirded out by it and it is okay that some are thrilled to finally have a decent portrayal of CF in pop culture. Ensuring our voices are elevated, respected, and included in the conversation is of the utmost importance. I also don’t want to neglect to mention the tone-deaf Instagram “influencer” ad campaign shared last weekend. I imagine that idea did not go through any committees or focus groups composed of adults with CF because it would have never been allowed to air.

I’m going to pay myself on the back for how well the private screening went. We sold 128 tickets which was far more than I expected. I was impressed with how smoothly it all went. Admittedly, I was anxious and I was worried that I would crumble once the film started. Memories of my sister’s hospitalizations flooded back. The talk about death, sickness, and transplants inundated me with memories that have been mostly cemented over in the last year. Afterwards though, I was composed as I gave thank you remarks to the audiences of both auditoriums. It was during these thank you remarks that I admitted my ulterior motive for the private screenings.

I want the conversation after “Five Feet Apart” to be led by those of us with CF in the CF community. I want us to speak as best as we can on behalf of all of those with CF and I want people in the community at large to recognize our autonomy and how these conversations should be led by us. Putting together the private screening was my attempt to begin that conversation. It was a way for me to get people to the theater so they could see some of the ugliness of CF and for me to then be able to be a voice that talks about the film. In that way, I’m so thankful for “Five Feet Apart” for giving me that opportunity.

In addition to all of this, I’m also thankful that a kind friend told the local news station WLWT about our private screening. I was interviewed and given the opportunity to talk about my sister, “Five Feet Apart,” my role in the community and more. It was awesome and brought even more awareness to all of the topics that I care so deeply about. And I’m not going to pretend that it didn’t feel a little cool to see myself on TV (Alyssa would have obviously been a prettier face, so sorry about that). 

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Where do we go from here? This is the really important point. While the film could have been better in some ways, it could have also been a hell of a lot worse. I’m thankful for how it was portrayed and how it will show a lot of people some of the ugliness that isn’t as obvious from how us advocates tend to talk about it. I hope people respect our autonomy, respect that we are human, and respect our wishes in how the future unfolds for our community.

I hope Justin Baldoni, Cole Sprouse, Haley Lu Richardson, Moises Arias, and co. continue to elevate the voices in our community so as to prove that the film is really about continuing to make the world a better place for every single person affected by CF. I hope our community is one that becomes more well-known and that we can continue to make leaps and bounds.

Thanks for reading, thanks for watching, and thanks for supporting. My sister would have had some opinions about the film, I’m sure, but I think the more important point is how excited she would be about our private screening and our family’s WLWT appearance. Thank you all for providing me a platform. 

TL

1/31/18

One of the weirdly comforting luxuries of CF progression/decline is that it is episodic; meaning it isn’t necessarily consistent decline, but rather stepped. Our lung function doesn’t decrease some noticeable percentage daily. With each infection “flare-up,” we just may not recover all of the lung function we lose from that instance. So for six months, my lung function may remain around 90% that entire time until in month seven, I have an exacerbation (what CF infection “flare-ups” are called) where my lung function drops down to 75%. After a course of antibiotics, I may only get back to 83% – my new baseline. This is typically how CF disease goes, but not always. 

As I said above, this – at least to me – is weirdly comforting. Since it’s episodic, we can learn to live with our new baselines as they come. It’s almost as if we have time to come to grips with them, find a way to be okay, then figure it out when the next drop comes. Don’t get me wrong: living like this sucks, but we don’t have a choice, so we find ways to feel okay about it.

Alyssa was excellent at rediscovering how to live with each new baseline. Every time she fell to a new, lower, harder baseline, she didn’t sulk or wax poetic about days gone by, which is something I’ll most likely do whenever I’m not as able to play pickup basketball or run with Duncan like I can now. Post-transplant life afforded her an entirely different set of circumstances; the threat of acute or chronic rejection was one that weighed heavily on our hearts and minds, meaning figuring out how to live in this world was a challenge I’m not sure we ever fully got adjusted to.

It seems that life after a lung transplant goes two ways: there are people that are in spectacular health for many years after transplant, never struggling with rejection or any of the concerning roadblocks that are somewhat common, and then there are people that may have decent days, but the roadblocks are so prominent and often, no obviously new baseline is established. The latter was the fate Alyssa and my family were confined to.

I can confidently say that after Alyssa’s first transplant, she didn’t go a full three months without some circumstance that upended her life for a time. She had medical issues – bouts of acute rejection, seizures, bowel obstructions, pneumonia, sinus infections, and more. And she had personal issues – losing her hair was one of the most difficult things she ever went through, she was unable to work consistently or move out on her own. (An aside: watching my sister as she sobbed when she was losing her hair – one of her absolute favorite things in the world was her hair – in Houston back in 2013 will remain in my mind forever. After everything she had gone through, that felt just downright insulting and unnecessary. I’m still distraught about how difficult that was for her.) The point of all this is to say that transplant was supposed to allow my sister to begin living a full life, or that’s what we had hoped. It’s hard to convey how hard it is to live daily when you believe you’re so close to a life of freedom from health worries when those health worries are just so damn frequent. We all want to believe that transplant does afford everybody that gets one that life. This isn’t reality, unfortunately.

As burdensome as this life was, we remained hopeful. I will never act like the LaRosa family is one that never struggles, worries, or feels hopeless, but I will also say the unique blend of personalities that we four had allowed us to be strengths to complement each other’s weakness. My mom has always been a momma bear, understanding yet protective. My dad has always been one that’s compassionate but reasonable and detail-oriented. Not to get too bogged down in all of this, but I think that our complementary skills sets enabled us to feel that, as a family, we could conquer just about anything, but we needed all four of us to do so.

I’m now going to try to put into words just how fucking difficult it is to sit in a room together where doctors are telling your family that your daughter/sister is, for the first time in her life of near-constant woes, officially terminal. We are not the first family to go through this and we are absolutely not the last. It is not my intent to be dismissive of other’s struggles or own heartbreak; in fact, my goal is to amplify that heartbreak and to write a message of love and support for that pain.

The fear after an organ transplant is rejection. That’s the one word. It’s a word you carefully tiptoe around saying so as to not jinx your fortune. For people that have had any sort of transplant, they’re prescribed immunosuppressants for the rest of their lives. Our bodies have a way of recognizing what is part of us – the “host” – and what isn’t. It’s obviously a bit stressful on the body to remove the organ it recognizes and place something totally foreign there instead, so immunosuppressants are used to suppress the mechanism that would typically recognize and attack those foreign invaders. 

There are a couple of problems with this strategy. First, your immune system is mostly a good thing, so turning it off has some bad effects, probably the most significant of which is the exposure to viruses and bacteria. Secondly, immunosuppressants are taken orally. Our bodies metabolize medications differently and sometimes doses have to be occasionally changed to ensure that the proper concentration is in the bloodstream. If the concentration is too high, hair loss or brain swelling can occur, as it did in July of 2016 when my sister had such a serious seizure that she stopped breathing and had to be resuscitated (another in the long list of horrible things she went through and we witnessed). If the concentration is too low, that immune response mechanism resumes and can begin recognizing the organs as foreign, or in other words, rejection.

The biggest lifestyle difference between the chronic disease that is CF and the chronic disease that is post-transplant life is that while CF may be mostly episodic decline, rejection can onset suddenly. Alyssa would be feeling healthy for a couple of days, then wake up with a cough and sore throat, indicating maybe a virus or worse, acute rejection. There are a handful of options that can rapidly reverse acute rejection and most of those are decently efficacious, but Alyssa’s body did not metabolize immunosuppressants well, so she seemed more likely to go into acute rejection. This type of lifestyle is difficult – since rejection can so rapidly come on, that concern is always present.

Acute rejection is mostly treatable; chronic is not. While their mechanisms are not completely understood, the biggest difference between the two is their prognosis. It’s possible for your body to be in acute rejection at one moment, then after a few days of treatment, no longer be in acute rejection. Chronic rejection is not that way; your biggest hope once diagnosed with chronic is stabilization. Strangely, in a weird twist, it’s sorta like CF decline. Neither can be cured, so the goals of treating both are to minimize rate of decline. Chronic is closer to end-stage CF, however, where “stabilization” is still relatively poor quality of life. With end-stage CF, hope still remains: a double lung transplant. After chronically rejection two double lung transplants, you’re no longer considered a good candidate, so the road is over: no other options.

So we sat there in that hospital room in Lexington, Kentucky, on that cold day at the end of January, where the following words hung in the air: “We’ve known that Alyssa was probably in chronic rejection for several months now, but the treatment we’re doing isn’t working. It’s clear that Alyssa is not a great candidate for a third double lung transplant, as we are unsure she’d even survive the procedure and we believe she would most likely immediately reject the new lungs. We encourage you to make the most of the time you have left. We are so sorry.”

I’m not sure what we expected before that appointment. This was Alyssa’s third time feeling this physically depleted and constantly short-of-breath. The signs were there, and we all probably knew in the back of our minds. But hearing those words from her doctors meant that there were no more reasons to be hopeful, aside from hoping she would not suffer. I hate to admit this, but that was when Alyssa first began to pass away; there were only a few moments where Alyssa would be totally caught up in the vibrance of a good moment for the rest of her life. My sister would only survive six weeks after this appointment. 

For the first time in my life, Alyssa’s path broke from ours. After this day, we would have to hope for no pain and a quick end. How do you find a new baseline when the next step is death? How do you find solace in that? After decades of experience, Alyssa was as good as anybody at finding reasons to be hopeful, but hope is based in believing something positive is in your future. There are very few instances where death is a reason to be hopeful. What did Alyssa have to be hopeful for? I suppose part of me wants to believe she was finally hopeful to enter into a perfect rest, where pain and suffering no longer existed. I don’t know if she reached that point. My sister loved life and her zeal for life was sincerely paralleled by almost no one. My sister did not want to die so young, even if it meant more hospitalizations and pain. We talked a few times when she was doing okay if she’d consider a third transplant. She said she didn’t want another, but always hedged that she was thankful for the extra time she did receive so it would be considered. 

With every fiber of my being, I despise 1/31/18 with the strength of a thousand suns. It was this day that stole my sister’s life. She knew she would die soon, but when was soon? Why do anything if she’s going to be gone in 6 months, or 3? My sister was forced to make an impossible choice: continue on doing all of her treatments and maybe get a bit more time or forego them and maybe still get the same amount of time. She was forced, by no fault of her own, to confront her existence and her fate before anybody should ever have to. If you know my sister and me, you know we’re very different types of thinkers. I don’t know what she was thinking in the time after this day, but I do know that she was terrified for herself and for how we would process this. 

I feel powerless when thinking about what must have been going through her mind. Hell I feel powerless when I think about how I felt. We don’t think about death until we have to. It is impossible to fathom that we will one day cease to exist; it’s even more impossible to imagine that day coming in the near future. Suddenly your most menial concerns dissipate. Who gives a shit about followers or exams or someone making fun of your outfit that one time when you’ll not be here in a matter of months. Only the most important concerns matter anymore – how our loved ones will move forward, if we’ve said what we’ve wanted to say to those that we love, and if we’ve lived our lives well. 

1/31/19 marks the anniversary of my life beginning to change. While March 12th is clearly the climactic date that divides my life in two, it was 1/31/18 that I had to start changing. I began preparing and rationalizing all of our futures. There were moments that I was forced to remove the emotional component of my brain when I was consoling my mother. I will never forget my mom asking me if I believed there was any chance of Alyssa’s chronic rejection reversing and her living longer. In what I still feel like was the coldest moment of my life, I had to flat-out say “No.” No hedging or anything. There was no hope that my sister would or could “beat” this. That is simply not how chronic rejection works. I began to feel anger and resentment at any mention of a “miracle.” Alyssa and I hated the discussion of miracles. We believed that was not how we should live life. The hope of a miracle removed ourselves from reality. I understand that I was not wrong in that moment when I talked to my mom, but it meant that I was already putting my sister in my past, I was already beginning to griever her. I hate that we had to go through that. 

In this moment right now, the hardest part of death and grief is how it removes any sense of control from us. We could not control what would happen with Alyssa. We were forced to sit and watch, with no way to provide her with any comfort other than our love. I made promises to my sister that I hoped would allow her to feel like she had change the world in some ways and that her legacy would live on for a long time. That was the extent I knew I could maybe provide some respite. 

I recognize ten and a half months is a short time, but there are moments I feel more lost than I did in the first couple days after Lyss died. I worry about the next six weeks. That anniversary mark scares me; it’s when I feel like I need to turn a corner, even though I know that isn’t how grief is supposed to work. How is it that nearly a year later I can hardly remember certain things about my sister and yet it feels like she’s not even gone? How do I accept that memories of my life with her are going to fade? How am I going to respond or make it through rough days when my lungs decline? These are thoughts and fears I can’t control and they are continuous reminders of my life without my sister.

I wanted to remove myself from my sadness today so I took Duncan to a park. I felt connected to nature and realized that I can feel whole in moments, even though Lyss is gone. As I turned the corner towards a trail, I caught a glimpse of a red cardinal – what we have found to be Alyssa’s way of letting us know she’s still with us.

TL

five months

I'm not sure how this started, I'm not sure if someone told us this or if it was something we read somewhere, but ever since March, whenever my parents and I see a cardinal, we believe it's Lyss saying hello. I feel an overwhelming sense of calm whenever I see a cardinal now. As a matter of fact, I saw a cardinal the morning I drove to the tattoo shop to get Lyss's handwriting inked on me. I felt like that was her way of endorsing the tattoo. 

Other than that, however, I have to admit, I felt like Alyssa wasn't present, mostly because I wasn't seeing very many signs of her. I'm sure most people know that feeling of chills you get when something seems too coincidental to be just a coincidence. Besides a few of those moments of seeing a couple of cardinals, I haven't had that feeling. I've been struggling a lot because of this. I miss my sister so much and I need those signs from her.

That changed in the last week. A couple of things have happened in the last week that have provided a sense of comfort I haven't had in a long time. I knew that I'd struggle after a couple of months because people would begin to forget that this grief is still a daily struggle for me and my parents. Trying to put on a face that I'm doing okay and moving forward for everybody has taken a serious toll on my mind, and that's been reflected in some of my recurring anxiety and depression symptoms that had gone away for a couple of months. 

I think everybody wants to have a legacy, but living with a chronic disease means your timescale is condensed, so the drive to have a legacy is magnified for people those of us that are younger. In the last week, there have been two particular events that have happened that have fired me back up to be motivated towards the future to build a legacy for Alyssa, myself, and my parents. The first one was a conversation with a friend. This friend and I talked about the two projects I'm actively working on, and he had a connection to both of them; both of these connections felt too coincidental to just be coincidences. These two projects I'm actively working are giving me hope for the future. I'm not going to discuss those projects yet because they are quite some time away. I'm really excited to keep working on them because I think they have a very real chance of providing me that connection to my sister and they are both going to honor her in ways that she would love. I felt like that conversation I had with that friend confirmed that I need to commit to these projects.

The second was a dream that I had the other day. I'm not usually one to believe that our dreams mean anything more than maybe it was something we were thinking about just as we fell asleep, but I woke up feeling more comforted than I have been since Lyss passed. She was in my dream for the first time since she died and she told she loved the ideas I was working on and that she was proud of me. She told me to stop searching for signs of her, that she would make it obvious to me when she was around. I don't have any idea if my mind concocted this dream to comfort myself. It could've been a defense mechanism to get through all of the continuous pain. Or it could've been her way of nudging me further towards these goals. Seeing her in this dream after having that conversation with my buddy a couple days earlier gave me the chills I hadn't had in quite some time. I don't know what it was but seeing her felt so real. I woke up feeling as if she was alive for the first time in five months. That is overwhelming to feel immediately upon waking up. There was even a period of a few seconds where I had to look at the tattoo on my arm to confirm she really was still gone. It hurt but it was comforting.

I still feel lost in the world I'm in. I still feel like this is just a fever dream and that I'm going to see her posts pop up on social media. I'm proud of my family for finding reasons to continue waking up and pushing forward. As has been the story for three decades, my parents are strong beyond words. 

I feel like I had better clarity of my place in the world in the couple of months directly afterwards. I feel more lost now than I have in a while, even with these projects I'm working on. Part of battling my anxiety has been me realizing that anxiety is a powerful mental enemy that convinces you that you're failing even when you're succeeding more than you would've expected. Even writing this piece, I've gone back and forth on if I'm doing better or worse than I would've expected. I do feel like the fog is clearing up some days, then I reflect on what I witnessed of my sister in her final days, causing me to well up with panic and fury that this was the fate she was confined to. 

One of the last things I told Alyssa was that I promised I'd work on this project that we talked about. I intend on keeping that promise. I hope that it will continue helping me in this life without my sister. Five months feels like an eternity and a day at the same time; a sensation I never understood until now.

TL

The Bittersweet Downside of Orkambi & CFTR Modulators

In less than a month, it will be the third anniversary of me starting what is arguably the best drug ever created for people with CF. That's a pretty grandiose statement, but CFTR modulators were an entirely new class of drugs in our arsenal of CF drugs. 

CFTR modulators are the drugs that "fix" (I use that term loosely here) the defect that causes cystic fibrosis. Let me digress for a second: I'm speaking personally here, but I would argue cystic fibrosis is more or less a collection of diseases as opposed to a single entity. The reason for this is that there are literally more than a thousand mutations. While some of those mutations may act similarly, the protein misfolding that occurs in the different mutations means that "fixing" that specific defect in each patients' case may be different than a patient with a different mutation. But even within the patients that have the same mutation, we still see variability in their clinical severity, which probably explains why Alyssa and I had such different experiences. My point in all this is that every single person with CF will have varying CFTR function because of many different reasons. What we do in the lab is trying to determine the drug that will work best for the patient. 

"Fixing" the defect essentially means improving CFTR function. A person without CF has good CFTR function, probably at least 50%. People with CF can have any percentage under that, and clinical severity is likely inversely consistent with CFTR function percentage. In sum, CF is a very complicated disease, or collection of diseases if you prefer, and every patient is different. 

My point with all of this is not to dispel any reason for hope. To me, the fact that we have modulators that can improve CFTR function, even with how complicated CF is, is a reason to be hopeful. The likes of Kalydeco and Orkambi, the advent of Symdeko, and the forthcoming triple combo therapies in the pipeline are concrete examples that it's reasonable to assume we may one day have drugs that improve CFTR function to the point where there is no issue with chloride transport in our cells, which would likely mean no clinical presentation of CF at all whatsoever – that unfortunately doesn't mean we'd have no residual scar tissue or fibrosis or bronchiectasis, but it'd mean slowed decline.

Which is why modulators are bittersweet. 


I'm not shy about my struggles with anxiety or depression. I speak openly about the life expectancy conversation with CF and I consider it to be one of my biggest goals to transform the conversation around death through my writings. When I started Orkambi, I knew it had the potential to affect my future, specifically physically, but I didn't really think it'd be life-changing in the way it has been mentally. 

With a chronic disease like CF, you come to accept that your health may fade. Maybe not fully accept, but you understand that it's likely in your future. You come to appreciate the days as they come and be thankful with your health when it's good. But with modulators, there is a new element of hope that's unlike anything else.

With modulators, you start to feel better, you start to realize the decline might not be that imminent. You start to wonder if your future is a little more secure than it has ever been. For me, I even start to feel a bit...normal – a word I desperately hate. 

If Orkambi can significantly slow my decline, it's not irrational to wonder if the next generation of modulators can stabilize my decline. Maybe the generation of modulators after that could even potentially reverse some of the decline of my lung function. This element of hope should be an overwhelmingly good thing.

But compounding a physical disease like CF with the paralyzing mental disease that is anxiety is a recipe for disaster.  


Living with a chronic, terminal disease means you're living on a contracted timescale. 

I've written posts where I've said that it's impossible for anybody to know of their future with any amount of certainty. That's true for everybody. There are unfortunate circumstances that can arise for literally anybody.

But it's different for people that suffer from chronic disease. If you know me personally (and hopefully this is also evident through my writings), I try not to live with bitterness in my heart. I have a really shitty disease that just so happens to be due to a mutation in my DNA. It's hard to not be pissed off about that at times. When I zoom out and realize that my life is the single chance I get at existence, there are times I'm pissed off that I wasn't given a chance to have a life free of constant worry. So I can admit I feel bitter at times. 

Orkambi is a reason to be hopeful that one day, I may have a normal existence, one free from worry that one exacerbation will ruin my lungs. I do have hope that modulators will extend my life significantly. I'm also so sad that Lyss never got that opportunity. She was never able to be excited about modulators because they don't work for post-transplant patients. I'm worried that until my dying days, I will have feel sorrow that my sister never had a chance to be hopeful about these medications for herself. 

I always want to be honest about my experiences. I understand that having CF has taught me a lot of valuable wisdom at a young age, and to be honest, CF has given me an outlet for creativity and a career. For those reasons, I'm grateful. The balance becomes difficult when I try to be the person that learns from his experiences but doesn't become too drawn into those experiences that nothing other than anxiety comes from them. When I write about these experiences, it does seem like I come out on the other side a bit better; I feel as if I've shed some of the extra mental burden and hopefully some good comes from it for those reading my pieces.


Some of my days are filled with a devastating existential dread where I have no interest in doing anything and feel like I will never feel fully fulfilled. Other days I'm hopeful and aspirational, dreaming of a world I'll someday hopefully make better through different avenues. 

I think what I hope people can get from this is that chronic disease is a nonstop physical and mental battle. There is an entire layer of life that most others don't recognize. I don't want to write to make people feel guilty or feel pity for me that I have to deal with this, it's more to be honest about my life and maybe others can become more empathetic through reading about it.

TL

The Letter

On the month milestone of Alyssa's death, we found a letter she wrote to us in her notes app on her iPhone. 

This letter became the last words I'd ever read from her. 

This letter has been haunting me for two and a half months, and I fear it will haunt me for the rest of my life.

It is lucid, soul-bearing, heart-wrenching, and unfinished. I've debated for the last couple months if I want to post it, out of respect for her and my parents. I've wondered if it would help me feel better if I posted it. Maybe I'd feel better because I'd have a chance to write about it, or maybe it would show people what Lyss was going through when contemplating the end of her life. Maybe, in the long run, I'd regret sharing it because then I'd never have any remaining bond between just me and my sister.

The letter also got me deeply thinking about the legacy we leave and what becomes of our relationships with others when we're gone.

In the midst of writing this, I've decided I'm going to post the paragraph she wrote to me, in parts, with my thoughts on those parts.


Tré..... I am so sorry you have to go through this. It’s not fair at all. You shouldn’t be only 23 years old and losing a sister and having to deal with all of that. You shouldn’t have to remember me, we should be sharing memories together for the rest of your life.

Well, this was devastating to read. Something Lyss and I probably always knew was that one of us would outlive the other. This feels callous to admit but, for the last five years, I've mentally prepared myself to bury my sister, that way I could be there for my parents in this tough time of grieving. I also knew the statistics on lung transplants and based on how healthy I am, seeing Alyssa die was something I've been shamefully preparing for for several years.

I know Alyssa knew I would find a way of continuing her legacy. She knew I'd be okay. But it breaks my heart that she had to be worried about me. She was literally in the middle of writing about her death and was worried about me losing her. That hurts so bad.

You are such a great kid and it’s been an honor watching you grow into such an amazing person and do such brilliant things in such a short time. It makes me feel so happy to have you as a best friend and brother. I couldn’t ask for someone better. I want you to continue doing great things and making a name for yourself, I know you will. I’m going to continue to watch over you.. I’m so sorry I won’t be there physically for you, but know you can always talk to me and I’ll always be right there. Anything you want of mine. It’s yours. You take what you want. 

I would be willing to bet that one of the personality traits my parents most wanted to teach us is humility. For the rest of my days, I will attribute any of my successes to the support my parents and sister provided me. I am so, so incredibly fortunate to have had their support over the years. I have never been told I couldn't achieve something (though I've definitely been gently pushed in other directions). I give myself some credit, but without my parents and Lyss, I can assuredly say I wouldn't have reached the position I'm in.

Less than two weeks after Alyssa died, I died my hair blonde. In April, I redesigned my blog, changed my logo, and promised myself I'd start writing more. For the month of May, I wrote daily about my life and experiences and called it "#31DaysOfTré." Since moving into my new house, I've finally started pursuing a bunch of creative ideas I've had for a while now.

I did all of those things in subtle acknowledgment of how I'm trying to live on for Lyss; to do things that take a bit of guts or that I wouldn't normally do and do it because she would at least admire the attempt.

Alyssa's support meant the world to me. She read and shared everything I wrote and she believed in me in everything I did. She was genuinely proud of me. In the months since she died, I've heard so many people tell me how she was so proud of me. It's so bittersweet to hear but I love hearing it. It reminds me to keep going when I'm struggling. How deeply she believed in me pushes me to pursue my ideas and hopes.

I’m sorry that some of your life has been spent with me in the hospital and I know that always hasn’t been fair. I never want attention drawn away from you. Gosh I can’t tell you how proud of you and the person you’ve become. It seriously makes me so happy and jealous at the same time. You have such a huge heart for everything you do. You put your mind to it. I’m so proud of the writer you are and what great things you’ve done in your life. It'll only get better.

I think this may have been the hardest part to read at first. Our relationship was different than every other sibling relationship. We had to worry about each other constantly while also be there for our parents. It hurt me to my core to know Lyss was sick or was hospitalized, especially when I was in college.

Living in Lexington was difficult. I felt that I was living this normal life, going out on the weekends, going to college, being healthy, while Lyss was dealing with the aftermath of a transplant in Houston without many friends or family, unable to work. The fact that, in her last days, she apologized to me for receiving attention for her health shook me to my core. When I read that for the first time, I was sobbing and trembling. I never knew that it bothered her that much when I visited her in the hospital.

I wish I told her this more when she was alive, but most of the reason I was so open and transparent about my CF was because of my admiration for her openness. Lyss had many scars on her chest and stomach, but she never apologized wearing bathing suits in the summer. She never complained about her lot in life, thought she struggled daily. I get told I'm strong all the time, but I feel like she was so much stronger than me. I'll forever remember her for her willingness to fight. I put my everything into everything I do because I want to live as much as I can in lifetime I have, something I feel like Lyss did.

I’m so sorry I won’t get to be at your wedding with you someday and share those crazy memories. I’ll be there in spirit though. Dancing around you guys with my awesome dance moves. This just isn’t fair you have to lose your sister at a young age. I never thought it would be so soon. I’m seriously so freaking proud of you. 

Damn. Alyssa loved weddings. She was so good at planning and decorating and designing. I would've recruited her and my mom to be my wedding planner. I'm sad she won't be able to do that because she would've loved that. 

I guess she did think about her future more than I realized. We tried not to discuss end-of-life stuff much. I wouldn't ask her because I didn't want to scare or hurt her, and maybe that's why she never brought it up with me. 

Duncan bear is so lucky to have you as a daddy. He loves you so much. Harley loves you so much too. So be sure to always remind him of his mommy and how much I love him. Dress him up and make his IG look cool as usual. He deserves to still have a sweet ass social media still haha. 

The end of the letter; clearly unfinished.

Weirdly poetic, I guess. It ends with her talking about our dogs; our children. We always teased each others' pups. I always called Harley "Second Place" because I joked Duncan was cuter; she always called Duncan "Big Head." 

It hurts but it feels like the perfect ending. Alyssa was a master of social media, a skill I've obviously tried to emulate, and she wanted me to still manage Harley's instagram. I can't imagine how painful it was for her to write those words, knowing she wasn't going to be around much longer.


I don't know how to write about death without it sometimes coming off callously or cold. The words I use sometimes feel dismissive of what happened to my sister. But I don't like tiptoeing around this. My life will forever be delineated by March 12th, 2018. I will always remember what events happened when I could tell Lyss about them versus which events I was immediately sad when remembering I couldn't tell her.

What becomes of our relationships when we die? We live in a digital era which means we have constant reminders of the deceased, we have messages that exist until we delete them, pictures and videos are always popping up in our feeds.

I feel like my relationship with Alyssa has changed a lot even since I said goodbye. I have written candidly about my struggles with suicidal ideation; I have written about CF; I have spoken at events. These are things I've done by channeling my despair into something productive because I know if I don't, I will self-destruct. I fear the day that I don't have passions or hobbies that I can manifest that sorrow into something else. 

This letter has become something entirely different than what Alyssa wrote. It has become the last remaining bit of Alyssa's life that feels new to me. I try not to read it that often so that I can hear her voice again when I do read it; each time it becomes more and more my voice. 

I try to put myself in her shoes. I try to imagine how I would handle writing a letter to the people I love most dearly in my final days. It's a terrifying thought, but considering this deeply existential concept allows me to try to understand what my sister went through. I feel like it's the least I can do after she went through all she went through.

My first birthday without Lyss

On the day I shared the piece I wrote about the one month mark of Alyssa's passing, I came home from work in a relatively good mood. Writing that piece was cathartic and I felt like some good came out of reflecting on the one month mark. Then once I came home, my parents told me that my dad found an unfinished letter Lyss had written us a couple of days before she basically became unconscious. It contained a lot and it was really painful to read. The quote below was particularly hard to read from my sister.

You shouldn't have to remember me, we should be sharing memories together for the rest of our lives... I want you to continue doing great things and making a name for yourself. I know you will.

For obvious reasons, no matter what the letter contained, it was going to be painful. These words were likely to be last words I'd ever read in her voice again. Writing is such a powerful medium for me because what I write will live long on after I eventually die. Reading Alyssa's words further escalated my guilt and my struggle. In Alyssa's last five weeks – and really, her last five years – she had to contemplate her life and what was going to come after her death for us. I now have this inescapable feeling that she is trapped in that mental state. I can't imagine how intensely painful it must've been for her to write those words. I have written a lot of painful words in my time, but I have never had to write a letter to my loved ones telling them my wishes for them after my death. 

Alyssa's words in this letter were lucid, well-written, and in her distinctive voice. I could hear her speaking the words to me, even though I can't clearly remember her voice right now. I could feel her presence. The process of making memories without her is difficult, agonizing, and full of guilt. I still feel guilty laughing, smiling, or even a general feeling of hope, though I fully know she would want me to be living life as best as I can in honor of her and bringing her along for the ride.

So how do I do that? How do I live life without the overwhelming sense that living life is somehow disrespectful to her? I don't have the answer to that. I've struggled with generalized anxiety for a while now, but all day yesterday, I had this widespread unease. I kept having feeling hopeful that I'd see her post a picture of us with a nice little message like she always does. I felt bad opening cards and gifts without her. I hated her not being there for dinner.

And yet, at different moments, I did feel a sort of comfort. I received a few nice messages that nearly brought me to tears. I went to dinner with my parents and a group of the guys. Maybe that will become a new tradition. I consider my guys to be as close to me as brothers, and even though they aren't blood brothers, maybe them being there was a way to fill the void of losing my sister. I feel that my experiences with CF and losing Lyss have helped me knit these tightly held relationships with so many people that can never be unwound. I consider that to be something to be thankful for. 

So many people loved Alyssa. Through their support through this difficult time, it's been clear to me how many people I have on my side. My birthday, although it felt incomplete without Lyss, reminded me that I have an army on my side to navigate this new life. Losing Lyss has felt like a post-apocalyptic hellscape where my parents and I have been climbing through the rubble. Making it through my birthday and feeling okay about it feels like a bit of a sunrise.

I've long wondered if it was narcissistic to want to be a writer. Writing is different than other forms of art. Aspiring to become a writer makes the assumption that people care about your words and what you have to say. I believe I have a unique voice and I hope to use that voice to encourage people to think about parts of life and life itself. I finally realized yesterday that my inclination to being open and writing about my experiences is unique enough that I can do positively with it.

I will continue to write in honor of my sister because she believed in me and my goals as a writer. Without Lyss, I wouldn't have the courage to write about what I'm going through. She said we should be making memories forever, and truthfully, I believe we will be, through the impression she left on my life and the way she lived her life forging the path ahead of me. That's the best gift she could've ever given me.

TL

When life becomes too much to bear

Every morning and night, I do what I call a "treatment." A treatment consists of inhaled medications – a short-acting bronchilator, a long-acting bronchodilator, a steroid for my sinuses, a mucolytic, a mucus thinner, an antibiotic – during chest physiotherapy, or the vest, that inflates and vibrates to help clear the thick mucus in my lungs. On top of this, I take a series of oral medications: vitamins, enzymes, a drug that helps liver function, an anti-inflammatory, a maintenance antibiotic, enzymes that break down food, and a CFTR modulator. I also have to exercise daily, ensure that I get enough sleep to help maintain my body's constant fight against infection, and be cognizant of my hydration levels, electrolyte levels, and even my glucose levels because I'm borderline diabetic (there's a form of CF-related diabetes).

I've been wanting to write about daily life about CF for a while, but admittedly, I wanted to do it in an educational way. Not this way. I've become so immersed in this daily regimen that it's been a long while since I realized how cumbersome and stressful it is.I'm not writing this to solicit pity or sympathy, but because writing is my catharsis and I'm sick and tired of this shit.


In my life, compliance isn't an option. Doing these treatments and exercising consistently are quite literally keeping me alive. If I were to succumb to the laziness that I so dearly want to succumb to and take a weekend or week or month to just relax and forgo my treatments, I would most certainly end up in the hospital. It is not hyperbolic to say that a short respite from my treatments could put me in the hospital with a severe infection. 

This reality is terrifying. It's true that life is fragile for everybody, but the reality of how fragile life is is an intimately known truth in my world. In my experience, the fragility of life isn't something most 23-year-olds think about. But living with CF and being a sibling of somebody whose been through end-stage CF and two double lung transplants has forced me to consider that fragility deeply. It's forced me to think deeply about the people whom I love and the goals I want to accomplish in my life. It's made me an existential person who is somehow simultaneously petrified of death and comforted by the idea that I just might know what could be the end of my life.


I wrote a poem last week that wasn't supposed to be a poem but ended up as one. I'll post it below, but it was about how living with CF is a complex paradox of hope and fear. 

Screen Shot 2017-08-29 at 8.24.59 PM.png

It was originally going to be a post like this one, but I started fidgeting with the formatting and I found some meaning in it, so it became a sort of poem about balancing hope and fear. When I started writing it, I was terrified of my future. By the end of it, I felt extremely hopeful. I hoped that that hope would stay longer than it did. Unfortunately, it didn't.


If I had it my way right now, I would cease all my treatments for a bit, just to see what life would be like as somebody without CF. I often joke about life with CF. I often discuss and write about how, without CF, I'm not sure I'm the emotionally complex and passionate person I am today. But I guess that's a facade; I would do anything and everything to annihilate this awful disease from myself, my sister, and everybody else who has the misfortunate of dealing with it.

Since I am in good health and relatively educated on nearly everything in regards to CF, I feel responsible to be the anchor of hope for everybody around me. I suppose this is a responsibility I've bestowed upon myself, but nonetheless, it's something that is a core part of my life and my character. At this moment, I can't find the fortitude to be that anchor. 

I'm so sick and tired of this of this godforsaken disease. It's stolen so much precious happiness and time from me and my loved ones that I'll never get back. 

When I write these posts, I usually try to find some bigger meaning or motivation for hope to inspire the reader and ultimately myself. But right now, life just feels like too much to bear. 

TL

May is CF Awareness Month

The Importance of Awareness

Since May is CF Awareness Month, I want to take the time to really hammer home how important awareness is. It may seem obvious why it's important for people to be aware of CF, but when I mean awareness, I mean when people know of me, but don't really know me that well, but are familiar with my life as a CF patient. 

I enjoy writing and I think I'm skilled at it. I'm also an explainer, so in just about everything I do, I try to give thorough answers. (Side note: a couple weeks ago, a buddy of mine asked how I choose books to read. The response he received was a novel, no pun intended.)

So when I write about CF, I write honestly and deeply. I explain everything in as detailed way as possible. I want to know why people think and do the things they do, so I want people to know why I think and do the things I do. I feel as though communicating through my writings is the best way to do that. Writing gives me time to think things through more clearly and put them more eloquently.

The Importance of Openness & Transparency

On a further point of why I try to be as thorough in my writings about CF is because of how crucial it is for us to be openness and transparent about our CF. Living with a chronic disease has affected our worldview intimately, so sometimes it's easy to forget that some people have never been around somebody with our life experiences.

If I'm open about my CF, I hope to create a culture of understanding where people can feel free to ask me whatever questions they want. I want people to understand CF, I want people to ask me the tough questions. It's nice to be talked to like an adult. Trust me, there isn't much that can be asked of an adult with CF that they haven't been asked or thought of before. 

If I'm transparent with people, especially regarding comments that can be misconstrued as offensive, I can teach somebody how to better engage in the conversation. 

CF Awareness Month

So for this month, I'm setting out to write more comprehensively about my life and what I go through. I'll explain medications, appointments, the psychology, the process our health goes through as it deteriorates, and many other points.

Please feel free to ask me any and every question you can come up with. I'm an open book. Comment on my statuses on Facebook or here if you want it to be public, or send me messages privately if you prefer. I have no preference either way. 

TL

 

We All Have a Fight and Why Empathy is Such an Invaluable Skill

I believe deeply that it's important to develop a good skill of empathizing with others, even though it can be painful sometimes. This piece is about how CF affects me so deeply. I wrote about how the internal fight is hard and that I put out an image of a confident fighter and sometimes that confidence is nothing more than a veneer. 

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The Overwhelming Specter in the Life of an Adult with a Chronic, Terminal Disease

Most kids growing up aren't worried about needles, or if they are, it's because of flu shots. Most kids don't know the word infection by 8 or 9. Most kids don't know what death is until they lose a grandparent or a pet. Most kids don't ever think about their lives being abruptly cut short. Most kids don't have CF.


Most teenagers may be self-deprecating, but most don't joke about their own demise to desensitize themselves to the fact that may not live long enough to graduate college. Most teenagers don't constantly worry about their lung functions. Most teenagers aren't embarrassed to cough in class for fear of being labeled as "sick." Most teenagers don't have to explain why they miss school so much or why their lungs work differently. Most teenagers don't have CF.


Most adults don't have to fear getting close with other people, just so that person doesn't have to learn the intricacies of CF. Most adults don't have to second guess becoming friends with somebody, just in case they may have to grieve you at a young age. Most adults don't have to question bringing a child into this world, because of the risk of that kid also having CF or that that kid may be raised without one of their parents. Most adults don't have CF.


The nasty reality of the world is that death is a foregone conclusion for every living being (as of now; of course with the advent of gene therapy and billionaires, this may not be the case forever, I suppose). 

Humans are animals, just as dogs, birds, sharks, and cows are all animals. But humans possess superior consciousness to all other animals; it's what allows us to cure disease, send spaceships cruising millions of miles away, and even contemplate our own existence.

For that reason, humans are more intimately interconnected to the prospect of the death of ours and our co-existors earthly bodies. We're able to envision our deaths; what may kill us, how our friends and family would process our death, and even if we have a soul that persists after our bodies go cold. We're able to worry about the people we care about. We can imagine how terrible we'd feel if we lost the people we care about. 

On this point is the point of this piece: how the anticipation of an early death can disproportionately hold a claim to how people with CF process the world around us. It's not a stretch to say that when someone doesn't get vaccinated then carries a particularly virulent strain of the flu in our space that our very lives may be in the balance. It's not crazy to wonder, even if our lungs our stronger than they have ever been, if a flare up of the delicately balanced microflora in our lungs may trigger a pneumatic exacerbation that could wreak havoc on our lungs and bring the grim reaper too close for comfort.


Life is short. Whether you live to 30 or 70, that's still shorter than the time we want. This isn't a pity piece. People with CF don't want pity; we want sympathy and empathy. We want people to realize that everybody has their trials and tribulations, because we have a pretty firm understanding of unseen trials and tribulations.

Death is a future reality for everybody. CF is just a ticket for us to think about death much younger than most people. 

But thinking about death so much requires one to consider its antithesis: life. It forces us to consider what we hold most dear and what we want to fight like hell for.

Death isn't so much anything, but rather simply the lack of life. 

It's important not to glorify the grim reaper. Glorification of death is what gives it its immense power over our lives. Death isn't a choice; the fear of death is. But fearing death is like fearing an injury. They're both inevitable. 


From my experiences of living with a cold-blooded disease that claims people far younger than they deserve, there's only a single solution to combat death: by living your best life.

TL

 

Pan-Resistant Bacteria & The Crisis It Could Bring

I read this downright frightening article the other day:

A superbug resistant to every available antibiotic in the U.S. kills Nevada woman

The article was reproduced on PBS with permission from STAT News where it was first published.

Here's the original article:

A Nevada Woman dies of a superbug resistant to every available antibiotic in the US

Why this is terrifying for everybody

Nearly a century ago, before the advent of antibiotics – Penicillin being the first true antibiotic – it was commonplace for people to die of bacterial infections. Bacterial infections can wreak havoc by themselves, but most bacterial infections are treatable with antibiotics. Until they become drug-resistant. Then they're no longer "bugs" – they become "superbugs." "Superbugs" is just a scary term for bugs that are difficult to fight with most antibiotics.

But until recently, it wasn't common, in the US at least, for people to culture pan-resistant bacteria. It's happened before, but the news that a woman died in September because of a bacteria that could fend off all drugs available in the US is alarming.

The video below gives a nice explanation of superbugs. The video does an excellent job of explaining how and why superbugs developed.

The video above has been provided by HYACINTH EMPINADO/STAT from statnews.com. It is specifically from the original site that posted the article. 

Why this is particularly terrifying to people with CF

Cystic fibrosis, as understood by people who are familiar with it, is a genetic disease that causes thicker mucus in the lungs. Due to this, as they understand, infection is more common.

To be clear, most patients with cystic fibrosis always culture numerous types of bacteria in their lungs and sinuses. Different external factors – missing treatments, missing medication, smoking cigarettes are all examples – can trigger an exacerbation, which is basically just the bacteria in the lungs that have already made a home flaring up and causing more harm than usual. 

Here's where things for doctors and patients become tricky.

Oftentimes, it's hard to determine which specific strain of bacteria is causing the exacerbation. There are two major types of bacteria – gram-negative and gram-positive – which have different cellular structures. For this reason, they're treated with different classes of antibiotics. Some antibiotics work by disrupting the materials that form the cellular walls of bacteria, others disrupt protein synthesis, among plenty of other mechanisms.

Unfortunately for all people, especially people with CF where lung infections are always a lingering possibility, bacteria evolve. Quickly. Usually much more quickly than we can develop drugs.

In today's research world, the funding just isn't there for new antibiotic development. The primary focus of CF fundraising is on other drug classes, specifically the most promising: CFTR modulators. I'll write about these in a different post. I also want to acknowledge that I believe that class to be the most crucial and deserving of the funding. 

Back to the topic of drug resistance: after trying all possible drugs and drug combinations, CF exacerbations usually progress to the point of no return. The lone remaining option for most CF patients who are eligible is a double lung transplant. The reality of transplants is that post-operative care is necessary for the rest of life and is basically like inheriting a separate, but in many ways equally as bad, condition.

What you can do to combat drug resistance

The most important thing anybody – people with CF and people without – can do is to follow the orders of the medications carefully and accurately. Don't miss doses, don't stop the treatment before the prescribed course is completed, don't pressure your doctor to prescribe antibiotics for viral infections, don't take antibiotics when you're unsure of the cause or when you've not been advised by a physician. 

An issue with drug resistance is the vast ignorance of patients. The truth is most people don't understand antibiotics; they don't understand why they're taking them, they don't understand how they work, and they're possible completely unaware of drug resistance. 

The responsibility is on both the patient and the doctor. The patient must do her part to understand the implications of their sickness and course of treatment; the doctor must thoroughly communicate the risks of not correctly following the course of treatment and why the specific course of treatment is even being applied.

In future posts, I'll write about CF care, normal care, healthcare in general, and more detailed scientific and medical articles. For now, I'll leave this post with an incredible demonstration of how bacteria evolve at a rapid rate.

In a creative stroke inspired by Hollywood wizardry, scientists from the Kishony Lab at HMS and Technion (www.technion.ac.il/en/) have designed a simple way to observe how bacteria move as they become impervious to drugs. The experiments are thought to provide the first large-scale glimpse of the maneuvers of bacteria as they encounter increasingly higher doses of antibiotics and adapt to survive-and thrive-in them. [Caption and video provided by Harvard Medical School.]

Everybody – CF patients, friends, and family – please take your medication as advised. It's vital for everybody.

TL