So much of how we evaluate success of various medications is based on objective efficacy. Has the patient’s FEV1 improved? What about BMI or sweat chloride? It goes without saying that efficacy is critical when looking at treatment routines and options, but I think one of the major things that’s often lost in evaluating different medications is how has the person qualitatively changed? What about Trikafta has changed my life or your own life?
In 2018 I participated in a Food and Drug Administration (FDA) patient panel where the goal was for patients (a handful of parents spoke, too) to talk about our hopes for future treatment options, and for the FDA to take our feedback into account when evaluating future potential therapies. One of the major themes discussed was the time it takes to perform daily cystic fibrosis maintenance. Another topic we talked about was how much CF weighs on us day to day even when we are fully compliant with our treatment routines.
Quality of Life is one of the buzz phrases that we love to talk about, but don’t adequately value when patients talk about it. In participating in the triple combo study, I had to fill out a patient quality of life form at most visits. It was refreshing that someone was consistently asking about my quality of life. Of course, questionnaires can only be so comprehensive, and yes, it was noted whenever data was publicly released from the trial, but it was quickly lost in the overarching discussion about the FDA’s approval. Quality of life is often overlooked when we look at the value of breakthrough meditations in favor of objectively measured clinic outcomes. So today, I am going to talk about subjective changes in my life.
The crippling fear of getting a cold, isn’t nearly as crippling
I am a magnet for the common cold. Maybe it’s the cross between having worked in high school sports for 6 years and now going to school myself, but if there’s a cold going around, you can damn well guarantee that it is going to find me. In fact, I’m just getting over one now! For as long as I can remember, I have feared cold viruses. Once my health began its slow decline in high school and then rapid decline near the end of college, the common cold, more times than not, would land me in interventional radiology for a PICC line and a few weeks of IV therapy. In extreme cases, any number of viruses would even land me in an inpatient stay at hotel hospital. I cannot emphasize enough how often a cold would spiral into a pulmonary exacerbation. I was looking at close to a 100% success rate in the years leading up to Trikafta. Now that I’m on an effective modulator, I brush off most colds like it’s nothing. Often, I don’t even need to adjust my treatment routine. I suffer the 10 days of a viral infection like the rest of the population, and then move on with my life. Only this past October did that not ring true, and it was because I was dealing with something far more severe than what usually amounts to the common cold (oh how I so wish I had swabbed). The confidence that I now have during cold and flu season compared to a few years ago can only be described as remarkable. Yes, I still take precautions – I wear my N95 mask on flights or in class if needed, I carry alcohol-based sanitizer and I regularly wash my hands. I don’t shake hands during cold and flu season and I certainly will not touch doorknobs or other commonly grabbed things unless I must. Ultimately, it’s enormously liberating to go through a cold without always facing the reality of heading into the hospital for further treatment whenever I come down with one.
Exercising is fun again!
This past week when I was home on break, I returned to my beloved OrangeTheroy Fitness routine. Sadly, we don’t have a studio in Hanover. The first night back held a significant running portion, where I ran 2.5 miles like it was nothing. Prior to then, I hadn’t run since it was warm outside (and that feels like it was forever ago), so you can absolutely say I was shocked when I looked at my stats after the class. In fact, over the 2 weeks I was home, I played hockey twice and went to OrangeTheory four times, which included a few sessions with this cold in tow (I know, public, sorry for exposing you to my cold, but maybe it’s payback for all the years I’ve been subjected to unnecessary exposure?). Exercising that often, let alone exercising with a virus in my sinuses, would have been out of the question even two years ago. For the first time since high school, I actually feel like I am physically fit. Maybe it’s a result of how often I am playing hockey at school, but the opportunity to exercise is hard to pass up now that I’m feeling good… and as Elle Woods would say, “exercise gives you endorphins; endorphins make you happy!”
Waking up in the morning isn’t hard anymore!
For my entire life I had resigned myself to the fact that I would never be a morning person. My sleep requirements continued to get greater and greater as I got older. At the peak of my disease severity (maybe 2014ish?) I required nine hours of sleep and even more hours of rest. It was so hard to start my day. If I didn’t immediately strap on my vest and start puffing on hypertonic in the minutes after waking up, I was in for a hell of a morning. In those days, I would easily fill up a quarter to a third of a plastic solo cup with mucus. When I would dive into a pulmonary exacerbation, my mucus production skyrocketed to filling up half of a solo cup during my morning treatments. I’m getting nauseas just thinking about it.
I became a morning person after a about a week or two of Trikafta. One of the first major changes in my life was my how little sleep I suddenly required. I was up with the sun most mornings. I actually had the chance to savor my coffee and get a bagel from town before needing to do treatments. Hell, I now have so much time in the morning I don’t know what to do with it. I am frequently one of the only people in the cafeteria for breakfast at school. My 5 year college reunion rolled around about 2 months after I started Trikafta and after a night of drinking (I know, I’m a bad trial participant, I broke the no excess drinking rule… sue me – it was one time and it was with college buddies) while everyone was nursing a hangover that Saturday morning, I was at Dunkies getting boxes of doughnuts for the boys! Locker room guy right here, every team needs one! Waking up and being able to breathe is one of my new life’s most simple pleasures. I enjoy my morning routine and my ability to get by on 6 or 7 hours of sleep. It’s amazing.
My social life has returned
My social life was among the first sacrifices I had made after college. Late nights, parties, trips, group dinners, dating, and spur of the moment plans all went out the window almost immediately. Of course, I worked very hard to get myself back into social commitments, but I never really experienced the young 20’s that most people talk about later in life. Looking back, I didn’t have the energy, and thus the desire to regularly put myself through long nights. It was very isolating. My social life came back with a roaring vengeance quickly after starting Trikafta. The sudden desire to do and see new things was so strong that it was nearly alarming. I mean hell, I moved to a new state and started school after only a few weeks of thinking about it. Back in December before the term came to an end for Christmas break, Darcy and I were talking about how much our lives had changed. We had just finished a week where every night we were doing something different. She was either out with one of the partner clubs, play hockey or at some social event while I was doing any number of things myself. It all culminated with a ski trip – something I NEVER thought I would do after I had massive hemoptysis at the top of a mountain in Jackson Hole back in high school. Darcy and I looked back over the week and concluded how far we’ve come since a few years ago when we started dating. Most days Darcy and I were together we were doing low stress activities with the occasional date night. Now we’re going full throttle 100% of the time. From hockey to skiing to the bar to house parties, It feels so different, having to social that is. The best part is… it’s a lot of fun.
Time
I suddenly feel like I have time in my day. In the past CF consumed so much of my life. The treatments, the rest, the feeding tube, the clinic, the insurance negotiations, the calls to the pharmacy, the sleep, the stress leading up to a procedure, the inpatient stays, all of it. Living day-to-day was exhausting and it was hard. Suddenly if feels like most of that has gone away. My treatment burden has decreased and I it almost feels like I can do whatever I want, whenever I want to – that is as long as I’m not building a spreadsheet for class. Time is our most precious commodity and it’s the very thing that chronic illness takes from all of us. Initially, it’s the time in a single day, then it’s the time we spend actively caring for ourselves instead of doing something we want (like being in the hospital), and then finally it’s the time taken off our lives once the disease progresses until the very end. Time is the single most important thing Trikafta has given me and that’s not something you will see in my PFT scores, sputum cultures, or BMI. Time gained is such an elusive statistic that it’s only worth it if we all take advantage of it. I wake up every day with the hunger to live the time I have suddenly gained.
