The Trikafta Experience

**Caleigh (Host):**
Welcome to our Fight to Breathe, a podcast rooted in resilience, community, and the power of storytelling. My name is Caleigh Haber, and I'm so glad you're here. I was born with cystic fibrosis, a progressive, genetic, rare disease that affects the lungs and digestive system. I've undergone two double lung transplants, spent years navigating the healthcare system, and I've lived through moments I wasn't sure I'd survive. But through it all, one thing has kept me going. Community. The fierce, brilliant and brave voices of others who know what it means to fight for every breath. This podcast was born from that fight.

Our Fight to Breathe is a space to elevate the voices of people living with chronic illness, disability, and rare disease. It's a space where we talk about the realities of what we face. The triumphs and the grief, the injustice and the joy, the medical complexities and the very human stories behind them. Each episode will feature guests from all corners of the CF and medical world, from patients and caregivers to healthcare professionals and advocates. Together, we'll explore not just what it means to survive, but what it means to truly live. My hope is that you'll walk away from each episode feeling seen, understood, and a little less alone. Whether you're navigating this life yourself or you're here to learn, listen, or support someone you love, this podcast is for you. Thank you for being here. Let's keep fighting together.

Today's episode is about trikafta, a drug that has radically changed the future for many people with cystic fibrosis. For years, this community's sole focus was survival. But now, for roughly 90% of individuals with CF, Trikafta has offered something new. More time. And with that time comes a complex, unexpected luxury. Figuring out how to actually live the years that were never promised.

In this episode, we're diving into what that means. We'll explore the life changing benefits of trikafta, the physical and emotional challenges, and the deeply personal realities of adjusting to a life on a medication often called a miracle, but which still comes with complications.

So what is trikafta?

Trikafta is a CFTR modulator therapy, a type of treatment that targets the underlining cause of cystic fibrosis rather than just the symptoms.

To understand that it helps to break CF down simply. Cystic fibrosis is caused by mutations in a gene that produces the CFTR protein. This protein is responsible for helping regulate salt and water movement in and out of cells. When it doesn't work properly, it leads to thick, sticky mucus that clogs the lungs, digestive system and other organs.

Older treatments focused on managing the symptoms of CF rather than the underlining cause. These included therapies like chest physiotherapy or airway clearance techniques to move mucus out of the lungs, inhaled and intravenous antibiotics to treat persistent lung infections and high calorie diets, enzyme supplements, or even feeding tubes to address malnutrition and digestive issues.

While these approaches remain important, they don't correct the faulty CFTR protein itself. That's where modulators like trikafta come in. By helping the protein function more effectively, they target the root of the disease rather than just treating its effects.

Trikafta combines three medications, alexacaftor, Tezacaftor, and ivacaftor. Together, they help the CFTR protein work better, improving lung function, reducing respiratory symptoms, and lowering the frequency of hospitalizations. For many, it also improves how other organs function, such as the pancreas, GI system and reproductive system. People often gain weight more easily, breathe better, and experience improved energy and overall quality of life.

Many doctors now say for the first time, “we're not just treating cf, we're managing it long term”.

While trikafta is not a cure, it has been a groundbreaking advancement, and because of it, life expectancy for people with CF has jumped significantly, with many now expected to live into their 60s and beyond.

Our mission with our Fight to Breathe is to offer a platform for people to voice their opinions in an accepting and nonjudgmental atmosphere. We want to foster a sense of belonging and support, offering insights, sharing experiences, and amplifying the voices within the cystic fibrosis community. Each episode will bring you closer to understanding the realities of living with CF while also highlighting the strength, resilience and hope that binds us all together.

Now, let's jump into the episode.

Today I'm excited to be joined by two incredible guests, Caleb and Tiffany Remington. Caleb lives with cystic fibrosis and Tiffany is his wife and caregiver. Together they share their journey with the world through their platform @ustheremingtons, offering a window into the highs, lows and everyday realities of life with cf.

Their honesty, vulnerability and humor have made them trusted voices in the CF community, and today they're here to talk with us about what it's really like to be beyond trikafta. The good, the hard, and the everything in between.

Welcome to the podcast. Tiffany and Caleb, it's so nice to see you. It's been forever it's been so long.

I'm really grateful to you both for coming on to share your perspective and experience with trikafta.

So just to start our listeners with an introduction of yourself, Caleb, can you tell a little bit about your diagnosis story with cystic fibrosis and the early years for you and your family?

**Caleb Remington (CF Person)**
Well, first off, congratulations on the podcast. Your setup is beautiful.

**Caleigh (Host):**
Thank you.

**Caleb Remington (CF Person)**
We're so proud of you. You guys are killing it abroad. We miss you dearly.

But, yeah, the. I was diagnosed at the age of one. My mom saw that I had a huge appetite, but I just wasn't growing. So her mother intuition kicked in. She took me to the doctor, and the doctor said, yeah, he is failure to thrive.

They did a sweat test back then because they didn't have genetic testing. So they determined I had cystic fibrosis. And so, yeah, it was pretty devastating for my parents to hear such news because they didn't know they were carriers. And there was no one in the family that had cystic fibrosis. So they reached out right away to the Cystic Fibrosis foundation, and they found a ton of support in just hearing from other people in the community.

And so from a very young age, we've been doing cystic fibrosis walks and bowl-a-thonas and everything we could possibly do to help raise money and awareness for the disease.

And so for me, I was a very fortunate patient with cf, where I didn't really feel the full effects until I was about 19 years old. So, I mean, I still had a cough. I would still. If I got sick, I was really sick. But I just chose to be super active and, you know, live outside. This was, like, right around the time when they told CF patients to, like, get outside instead of them, you know, being in a bubble and, like, protecting them. And so, yeah, I went full throttle.

I played the saxophone in seventh grade because I wanted to challenge my lungs. I ran cross country. I played golf. I was into hockey. So, like, every sport I could do that involved running, I did it.

So, yeah, I think my parents really just instilled nowadays it might be considered toxic positivity, but I think that was my survival mechanism where I could flip everything into a positive, no matter the situation, no matter what, how difficult things got. And so, yeah, they just instilled we got to live our life to the fullest and go day by day and not think too much into the future because, you know, we just got to enjoy the time we have now.

**Caleigh (Host):**
Thank you for sharing. And I just learned Something new about you. The saxophone. I had no clue. That's so funny. You should start playing again. I mean, it is really good for your lungs.

**Caleb Remington (CF Person)**
It was insane. Like, during band practice, I would hawk up the biggest loogies, so I always had a spit cup. People thought I, like, dipped in seventh grade, but I was my spit cup.

**Caleigh (Host):**
You shared some about your experience growing up and how you felt really fortunate with your health. And I was also the same way. I mean, you and I know each other, so we both know that we really went, like, hard on sports, and that was really good for our health. And you and I both didn't quite start feeling the effects of cystic fibrosis until it became progressive with our age and with, I think, a little bit with. Why am I losing the word for it? Puberty. That's the word with.

**Caleb Remington (CF Person)**
Yeah, for girls.

**Caleigh (Host):**
Yeah, with puberty. And for me, it was, like, a little bit earlier as a female and whatnot, but

Before gene modulators were even on the horizon. What did survival look like for you? Were you ever told about the survival of cystic fibrosis and the life expectancy being short than normal and how old were you and sort of. What was your reaction around that?

**Caleb Remington (CF Person)**
Maybe at an earlier age than most. I knew about the life expectancy and the seriousness of it just because I had a friend in kindergarten that had cystic fibrosis. His name was Johnny Whalen, and he passed away at the age of 11.

And so this was before they knew we. We should be six feet apart. We were best friends, and he was terribly sick. He'd only show up to kindergarten, like, for a week, and then he'd be gone for a month, and then he'd come back for a week. But, like, during that week, we were. I remember being in, like, the fire truck together. I remember going to his house and playing like we were best buds. And then. Yeah, like, when he wasn't going to make it, that's when I was, like, just. My whole world flipped upside down. I didn't understand fully just how serious the disease was until we lost Johnny.

So I've always had that. That, like, life expectancy, even though I knew I was, like, healthy and in a good spot.

**Caleigh (Host):**
That's interesting. I mean, that you just happened to know somebody and that you had that experience. It's really sad, but in a way, I guess it did open up your eyes to the severity of the disease and how it's really important to stick to your medical regimen and to do everything that you can to stay healthy.

And Tiffany, I know online too, you've talked about how open Caleb was when you guys began dating and how he even warned you of some of the, you know, lack thereof, opportunities and possibilities in life. What was your reaction around that? And how did you decide to pursue Caleb anyways?

**Tiffany (CF Spouse and Caregiver):**
Yeah, that's a really great question. I think, you know, at that time we were 24 years old when we met. I have done a fair share of dating around and I've also lived a pretty complex life up until then.

I think when we met, I already knew deep down what I needed emotionally and mentally in a long term relationship, let alone someone that I would want to marry and potentially like grow a family with. And when we met and our first conversations were about life and death, I'm like, hmm. That was immediately something that like, stood out because all the prior conversations that I've had with significant others and people that I casually dated, we barely penetrated the surface. And I think that really gave me just, yeah. Insight into something that I didn't even know existed or something that I knew I needed and wanted.

Yeah, it was, it was interesting because like, I feel like the first year we knew everything about each other, like at the deepest of our core level. But when it came to the frivolous of like, what's your favorite color? What do you like? All of those questions, we were like, where we didn't really know that much about one another and, and I think that's really what honestly like stood out to me. And I knew of cystic fibrosis. I had a friend whose mom had cystic fibrosis and we did some CF walks growing up. So I was privy to it, but I didn't understand the, the depth of cystic fibrosis and the condition in itself. So when our relationship kind of took a pretty serious turn, I feel like even within the first month I started doing research and yeah, and Caleb told me about the life expectancy and all that stuff. So it was really, it was really interesting. And I talked to my family and friends and they were hesitant of it too. And they're like, do you really want to pursue something that you truly do not have any idea what the future is going to look like? And I kind of just rested on the, yeah, the belief that, you know, whether if it's one year, five years, 10 years, I rather experience like that type of burning deep love and connection than, you know, with someone that I, that I would just, I feel like settle with, you know.

So, yeah, I think it was a lot. I Think there was a lot of fear, rightfully so. But I think also again, there was so much excitement and hope and a lot of passion that really just kind of drove through any of the fear.

**Caleigh (Host ):**
Sounds really similar to Brian and myself.

**Tiffany (CF Spouse and Caregiver) and Caleb Remington (CF Person):**
I feel like you guys can relate.

**Caleigh (Host ):**
Yeah, it's like you. You talk about things that most couples don't talk about sometimes, ever in their lives pretty early on. But that connection that it can foster is super intense. And people, either people that Caleb and I date, like we experience, people are either very receptive to it, sometimes people are receptive to it, but end up not being able to maybe carry that burden or that heaviness. And some people can, and some people make amazing partners who can be caregivers at the same time. And the relationship that I think individuals with CF have with their partners is really deep and really complex and has a lot of layers because it's not just romantic or just this or that. There's just so many things that go into it.

Caleb, what was your reaction when you first heard of gene modulators and trikafta?

And did it give you hope or did you also have any type of anxiety or were you skeptical at all as well?

**Caleb Remington (CF Person):**
Yeah, so my journey to trikafta was interesting. I knew about the G511D combination modulator back in 2010 during a Mauliola surf experience in. I want to say it was in Los Angeles, where or Malibu. There was a patient there that was on the modulator. So I kind of got to like pick his brain and understand like how it was helping him. And it wasn't like a major life changing drug I think at the time. But he said he just wasn't declining anymore. And so like, just staying stable was like a huge breakthrough. And then fast forward a few years.

Shortly after I met Tiffany, Carmen Reyes from CHLA reached out and asked if I wanted to get onto a clinical trial that was going to work for patients with one copy of Delta F508 and one copy of a nonsense mutation. And so I qualified. The trial actually got closed because the results weren't that great. And so I guess from like 2010 on, I like knew it was possible. I was like, if I can stay healthy long enough, there's a potential I could possibly be on this drug. It's like you have to be excited about it, but then drop all expectations because if I was just sitting there hoping and wishing and like, when is it going to come? When is it going to come? It may never have came. So I just sort of like in the back of my head, knew it was a possibility and was really excited.

And then once I got the news that this, that trikafta was available, it was extremely life changing. I wanted to get my hands on it right away.

**Caleigh (Host):**
And how long did it take you to actually start trikafta?

What was it like when you first heard that you were going to receive the medication? And can you tell us, is it a smooth process? Did you have any issues with accessing it? Like barriers with insurance or approvals or delays?

**Caleb Remington (CF Person):**
Yeah, so I was on a pretty steep decline in like 2019. My lung functions were like dipping into the low 60s on a regular basis. If I got sick, I think I hit in the 50s once.

And so yeah, it couldn't have came at a more perfect time for me because, yeah, just the, the mental capacity I was in with dating Tiffany, we got married, we were thinking about kids. Like all of this on top of not feeling good at all and you know, hacking up a lung every morning.

So I got the drug in January 4, 2020. I'll never forget the day we went on a hike and I was like, we got to the top of the mountain in San Diego and I took my first trikafta pills and just had so much gratitude. And all the people that have fought up until that point, all the people who lost their lives prior to taking the drug, you know, it's just so much work went into that pill. And so I just consumed all of that and just cried my eyes out.

But the two week, there was like a two week where the clearing they call it. And I was like, I don't know if it's going to happen to me. I don't really have any sinus issues. And like, I felt like my lungs were pretty clear. I filled up probably three or four water bottles full of mucus in two weeks. And it was unbelievable.

Like at first it was just raging migraines, but then like shortly after that two week clearing, we were living on Campland on the bay and I was doing wind sprints across the campground and I would run literally like a mile. I felt like I was running seven minute miles and I was not coughing. This was like the most insane feeling I've ever had where I was like, I could exert this amount of energy and then just like take a few deep breaths and get my resting heart rate back down to normal and like not a single cough. I was like, this is insane. This is absolutely insane. And so the though I didn't have any barriers I was on a really good insurance through my company. And so, yeah, once we got the. Obviously, you got to go through and get a prior authorization, but there was no hiccup with that.

And, yeah, we got the drug, thankfully, because, yeah. Accessibility. And I guess they did a lot of work with FDA and all of that to prove the cost. I know the cost is absolutely insane. Like, $330,000 for Trikafta a year?

**Caleigh (Host):**
$300,000 a year for trikafta for individuals living in the United States.

So it's amazing that you didn't have barriers. And I think it is something that a lot of people struggle with that, you know, we feel. We're very aware of, and hopefully those barriers go away as soon as possible.

But going back to your story, the first two weeks, you were saying you were coughing up tons of mucus and filling up water bottles. And as somebody who hasn't had this experience with gene modulators because I've had a double lung transplant, you know, hearing about those stories is pretty incredible. It's almost like you don't believe that they can actually be something that happens. Did you have any other symptoms besides coughing up mucus that you can share about your experience with trikafta?

**Caleb Remington (CF Person):**
That was really the biggest one. My digestive issues didn't go away or decrease in any way, because I know some people. Because you're taking it intravenously. It has helped, like, some people's pancreas and. But no, for me, it's. It was. I didn't. I. I've stayed the same amount of enzymes. The biggest thing was just my cough went away. And so, yeah, I would be driving down the road, you know, randomly three, four months later, just being like, I literally can't believe I haven't coughed in three months. Like, not a single cough.

Like, I go to the doctors, and they're like, can you cough up, like, a sputum culture to test? And I'm like, no, I literally can't. I can't cough mucus to spit in a cup. So they have to, like, jam a stick down my throat.

**Caleigh (Hoost):**
Yeah. For people that don't know it, they have to induce mucus production, which is not fun.

They have different techniques and tricks up their sleeve to try to make you basically, like, not vomit, but cough up mucus.

I can't imagine what was going through your head, Tiffany, and what was it like as the observer and as a life partner of somebody who was drastically transforming in a new, amazing, positive way, but also a way that you really didn't know what the longevity of it was. You didn't know what the future was, what was running through your head. It must have just been really, really equally busy up in your brain as well.

**Tiffany (CF Spouse and Caregiver):**
Yeah, I think, you know, I think because we were in this mindset together of like being cautiously optimistic about everything, we were kind of just. That's how we were approaching this new opportunity that trikafta was allowing us. And first of all, I also want to backtrack a little bit and just say how privileged we are to have access to it because, like, we were talking about earlier, that is not the case for everyone. And Caleb, you know, has been able to hold a pretty steady job all his 20s and into his 30s. And we know that plays a huge factor in insurance and care and accessibility. So just having the drug in our hand, like less than six months after it rolled out was nothing short of a miracle. And we just knew, yeah, just how privileged we were to be able to start the journey as soon as we could.

And yeah, I think the first two weeks when Caleb was describing like all of the mucus and the sputum that was just coming out and I'm just like visually watching this and I feel like I. Caleb, when it comes to his cough, because he's, you know, you know, he's coughed all of his life, he. It's almost become like. Like what? Like just your everyday. That's his normal. And it's like for me, over the years, I literally was like counting how many times he coughed a day and night. My level of like body awareness for him was so heightened. So to be able to see like trikafta work as quickly as it did was just mind blowing. I'm like, this isn't a placebo thing. Like, it's actually like happening. And yeah, after a month later, just to hear almost the clearness in his voice too, like he's always had, I feel like this, the CF rasp. And it was just interesting to notice even his voice changing because of just not coughing as much. And I think it gave me so much more relief. I also feel like I am just like overly empathetic and overly heightened on everything.

And I think I definitely carry the most amount of fear for the both of us. And yeah, I think it just, it brought me so much relief and just so much hope that, you know, the future that we just started dreaming for could be a reality and a possibility.

I think that, you know, as the years went on, we were just so mind blown, even just around the fact that he wasn't getting now, knock on wood, as sick as he normally does throughout, like, cold seasons and flu season and all of that. And we were just, yeah, just so overjoyed.

And now that we have kids, they're like walking germ bombs. And we were so nervous as to what life was going to look like with little, you know, germy, virus-y kids that we love so much. Like, what was that going to do for us? I think that was probably one of, at least for me, my biggest fears because as a caretaker, that's a lot of mental, physical, emotional load to carry as a parent and also as a partner.

So, yeah, we've been so, so, so blessed to be able to navigate that this past four years with, with our kids and seeing how Caleb's health withstands. Yeah, these, these crazy viruses that walk through our door. And it's been a, a huge part of the positive, the positivity of, like, trikafta and what that has afforded us.

**Caleb Remington (CF Person):**
So before kids or before trikafta, I, if I got any kind of runny nose, cough, sore throat, I, I'd almost immediately go on antibiotics. And now I've. I fight every single minus. If it's the flu, I'm done. That puts me on my butt. But like, common colds, no problem. I can, like, fight it over a week, maybe week and a half.

**Caleigh (Host):**
There's some commonalities between what you're saying and what I've experienced with transplant, because after my first transplant, I made my rounds calling people, you know, to talk to them and stuff for the first time. And that was instantly something that a lot of people told me was, oh, my gosh, your voice is different.

You guys talk about these amazing opportunities that you've had, you know, with running sprints and also having kids and be able to fight off viruses, which is. Anybody living with cystic fibrosis or a loved one of somebody with cystic fibrosis knows it's very common that as soon as we get sick with any sort of tiny symptom, you start antibiotics because we're unable to fight off small infections ourselves. And these small infections turn into large infections, which over time can scar our airways and decline our lung function. And sometimes we don't gain that lung function back. And so it's really important that we, you know, try to prevent as much as possible these viruses and bacterias.

But speaking about opportunities, Caleb, what new doors has trikafta opened in your life that once felt closed personally, professionally, or even physically?

**Caleb Remington (CF Person):**
I think personally the biggest door that's opened is just the possibility of being able to watch my kids grow up and get married someday. Like that to me was never, I never really went there because it was too scary of a place.

But yeah I, the like even just my conversations with my doctors has flipped from like survival mode to what can we do now so that Caleb when he's 65 is healthy.

That's a mind blowing perspective like that I've never experienced. It's always doom and gloom. But if when I went to the doctors and like we gotta get this bacteria, we got to get that bacteria, go on this cycle of antibiotics. And it was never about the future, not even about kids.

And so one, I'm glad the CF Foundation has shifted now that trikafta is around to hey, like starting a family is a very high potential for anyone with CF now that we have these modulators for the majority of, of patients. And so yeah that is the biggest one personally.

Professionally, I never started a retirement fund because I didn't think I would make it to retirement. Professionally it's, it obviously is good to be healthy. I don't have to take as many sick days. Yeah, I just have more energy throughout the day to get work done. And I have to communicate with my boss less about cystic fibrosis. Whereas before I had to be very open and honest and transparent with every little detail because otherwise they wouldn't fully understand the situation.

But I've kind of taken a backseat. A lot of people don't even know I have CF at the company. Whereas before it was like everyone knew. So that's been a different interesting turn of events.

**Caleigh (Host):**
And as your day to day medical regimen and routine changed at all, have you gotten time back? Are you still relying on the same treatments and therapies or have you been able to pause or even adjust your medical regimen?

**Caleb Remington (CF Person):**
Yeah, I've been able to pause a few things and decrease the amount of times that I do like pod hailers, Toby pod healers. But as I said before, my digestive system is the same so I still take eight pills with meals and four snacks. But yeah, everything on the like long respiratory side of things is more active as needed. So if I'm feeling sick then I start, you know, my Tobi pod healers right away I'll start azithromycin.

Yeah, everything's as needed.

**Caleigh (Host):**
I can't even imagine this world of cystic fibrosis anymore. I'm so far like on this other, in this other like atmosphere of transplant world that every time I, I hear and I learn more about people's lives nowadays, it's really shocking and the most positive way for people who qualify and benefit from gene modulators. It really is incredible.

Tiffany, have you noticed any changes in Caleb's energy or mood or just overall outlook in life?

**Tiffany (CF Spouse and Caregiver):**
Yeah, no, definitely. I will say trikafta and you can maybe chime in if you want to. Mentally, I think it did bring a little bit of anxiety I felt and I don't know if that's just also because we've become parents and children just naturally bring on anxiety, but I feel like that is a common symptom and side effect is just a little. Just more anxiousness.

But overall, mentally, I think it's been able to give us the opportunity to dream and hope. I think we were living the day by day and trying to be in the most presence of time that, you know, we didn't really think beyond or at least in our discussions together, our pillow talk, you know, we wouldn't really be talking too much into the future because that was just too much of the unknown. And now I think, you know, we're able to freely dream and hope for things like every average normal couple.

And again, I say that with so much privilege that we're able to do that because Caleb is in good health standings and trikafta has been so beyond helpful. I think your baseline now is around like mid-80s and it is.

We're no longer constantly. Yeah. Running the what ifs Scenarios in our heads and we're. And we're really allowing ourselves to. Yeah. Talk about things that excite us or, you know, having those conversations and trying to figure out what excites us. And yeah. Very new territory I think, for us, and for us as parents to really. Yeah. Have those, those conversations of like, what does the future look like?

And some just probably like right now we're kind of just like twaddling our thumbs and like kicking our feet like, okay, so where do we start? And I know where we're at. And, and we're just. Yeah, we're just kind of going with the flow now but with a lot less fear.

And yeah. I think it's also been able to help Caleb dig a little deeper too and allow himself to lean into like, uncomfortable feelings that maybe he's suppressed all of his life because he's been forced to live in this positive mindset just because that is, you know, truly what kind of got him through his whole life. And so, yeah, I think now that we're in this space and we feel safe enough to have the hard conversations of like, okay, what actually were the hard moments? Because now you are able to, like, feel that and feel that in its fullness rather than have to sugarcoat it because you're just like, I don't know what tomorrow brings. Does that make sense?

**Caleigh (Host):**
It totally makes sense. I think for me, I still don't do that because I've lived in survival mode for most of my life and I feel like now, even post transplant, I am, you know, healthy and I really can't complain.

I have same as you, like, GI issues and diabetes and different things. Of course, like, there's always complications and, and things that we struggle with.

But I'm always waiting for the other shoe to drop, even though maybe I'm waiting now a little longer instead of like, oh, it'll happen tonight. Maybe I'm like, it'll happen next week or, or in a month.

But we are conditioned so much to have every situation be positive because it's the only way to truly survive sometimes. Because without being positive or looking at things in an optimistic way, we can break. Or maybe we're scared of breaking, or not wanting to push forward and stay motivated in the fight because it's too real and it's hard and it's tough. But yeah, I mean, I at least commend you that you're like, processing things now.

Maybe I should do it. I don't know. I'm just trying to live day to day.

**Caleb Remington (CF Person):**
I haven't fully started, so you're not behind me.

**Tiffany (CF Spouse and Caregiver):**
One life, it's hard because. Right. I feel like, you know, this kind of goes back to the questions before of just like accessibility to if it's like a modulator or, you know, a transplant or anything, like you have to live and straddle this line of gratitude, but also this guilt.

And you know, for me, being a mental health advocate, from everything that I went through, I just want to make sure that everyone and everyone's story is like, everyone's allowed to feel their struggle and their grief in its fullness.

I feel like we in this world, in society, dismiss a lot. We. We do it to ourselves too. We dismiss our own pain and our own struggles because we're like, “we are so privileged, we are so grateful”. Like, there are so many other people out there who aren't, who aren't given this opportunity or who have it worse. And I think at the end of the day, while there is like such goodness in living in, like, positivity, it is, in my mind, detrimental if we're not able to really allow ourselves to feel the things that we're allowed to feel and that we're valid to feel because it is hard. Like, life is hard.

Everyone's struggles is their own struggles. And there's multiple truths that can exist. Right. Like, acknowledging your pain and your struggle can also coexist with having gratitude and celebrating your life.

And I think that's where the conversation between Caleb and I, I'm like, you don't. You don't have to always put on this strong armor. I want you to feel safe enough within yourself to feel like you can be vulnerable and that you can discuss these things and not sound like you're entitled or privileged or whatever, because those are still battles.

And, you know, your struggles are very valid. And I want that to just be more normalized.

**Caleigh (Host):**
We're all valid in our feelings, like you're saying, and it is good to process them and whatever way works for you. For me, it's always been writing and art. For other people, it's talking to a therapist. There's so many different ways.

But I do highly encourage people that are listening to take Tiffany's advice and. And process what you're going through or what you have been through and seek tools to move forward in life in a way that's positive, but also reality. Right. So that we have tools going into the future that we can take with us in the next battle.

So trikafta has dramatically changed the trajectory of your health. Do you ever feel like you're relearning your body again? Like you're living in a body that you have to get to know all over again and sort of understand all over again?

I feel like that every single day. I look in the mirror and I'm like, who is this? And then also my symptoms are just. I don't even know what my symptoms are sometimes with cf, I felt like I knew every single thing immediately. Whenever it happened, I could diagnose myself.

Do you feel the same way, or can you relate at all?

**Caleb Remington (CF Person):**
Yeah, I can definitely relate to some things. Maybe not all, but yeah, for me, the norm of not coughing has been. It's, like, hard to put into words that, you know, because I used to wake up and cough, I would literally count 200 times a day, and I would just, like, for an hour straight, cough up mucus and just spit it outside while I'm taking the dog for a walk. And so that to me has been one of the biggest life changing things.

**Caleigh (Host):**
Tiffany, how do you continue to support Caleb through the ups and downs of trikafta? Not just physically, but mentally as well.

And, and how do you also continue to be so positive about your guys future together?

Because it's not easy. I know for Bryan and myself personally. You know, I been basically raised to be a mother and taught that that was what we did as women and what we do and to be a stay at home mom and all of those things.

And I think my experience with health has definitely hindered my desire to do that because I am so safeguarded when it comes to bringing new people into this situation and also leaving Bryan possibly in the future and what that looks like for him.

But how do you make the decision to continue to have children or strive for goals that you have in life together?

**Tiffany (CF Spouse and Caregiver):**
Yeah, I think, you know, gosh, so many things I feel like as women, kind of like what you were saying, we are just natural caretakers. Um, I think that is just in our DNA.

I think with Caleb, you know, I was essentially, I'm a type A Virgo, so I like dive and I'm, I feel like Bryan is very similar in this fashion too where “we're like okay, spreadsheets, like Excels and numbers, and contacts, and this is the workflow of like how to get medication from point A to in our house”.

And I feel like, you know, when we started dating I was just trying to piece all that together and obviously that's all in Caleb's head, you know, when we first started dating. So trying to extract the logistics out of that was, it took some time and, and for him, he's been able to handle his health for the majority of the time in his adulthood on his own.

But when we, you know, married our two wives together, we did have to find a way to be able to communicate that and be on the same page.

So I think even prior to trikafta I was very much so more involved in medications and like making sure that he stayed up to compliance. And I became the mother

**Caleigh (Host):**

as any spouse has to do. Right. It's always like my biggest thing is “Take your enzymes”.

**Tiffany (CF Spouse and Caregiver):**
Exactly. And so, and that's hard.

I think that's, you know, something that you know, is not talked about enough either. Like as a spouse and as a supporter, I think there becomes this unspoken tension because as I'm sure I can't speak, speak of on, on behalf of you guys, but you know, as a cf patient who is independent.

You know, having someone come into your, you know, life and your system, it is. It's hard.

And I think, you know, there was many of times where it was a dance between being a partner instead of like a quote unquote mother, and also trying to have trust that, you know, Caleb was going to continue to, yeah, do things on his own.

And I think now post trikafta, I have also, like, let go of the reins a little bit and I'm like, okay, you know how this all works. I will tap in from time to time if things go awry. But I think now we've created a good enough process and system in place after almost 10 years that, like, we feel good about it.

But also, I'm not tripping out every two seconds of like, well, “you didn't do this, you didn't do this”. Because I feel like, you know, being an advocate in the CF community and having really close friendships like, like ours, Caleigh, I'm always like, you know, I just want the best for you because not everyone has this opportunity and not everyone has. Is able to access the care that we have. So I want to make sure that we're not taking advantage of that and I think that was a lot of our conversations and conflicts that have naturally just like, come up.

But yeah, I think trikafta has allowed, at least for me to kind of pull back a little bit and not be so. And not micromanage as much because I..

**Caleigh (Host):**
I mean, it is amazing how health can give our relationships the opportunity to flourish and transition into an actual partnership in a romantic life sense, rather than just a caregiver role sometimes. But that's just what's necessary. And I don't think either person wants to do it or either person wants to be the person who's reminding them or being reminded of something.

But, sometimes that's life and, you know, it's temporary and at least you guys have a good enough relationship where you do feel comfortable to remind Caleb to do something and Caleb can be perceptive to it and actually do it. And yeah, it's not so smooth, I'm sure.

**Tiffany (CF Spouse and Caregiver) and Caleb Remington (CF Person):**
But I have one story to add to that because Tiffany no longer is the micromanager.

Ever since Faye was about a year and a half, two years, she always saw Daddy get up before dinner and go into the office where I keep all my medicine. And so she was like, “where's Daddy?” Like, she always asked. And so Tiffany, for probably a year, “Daddy's going to get his medicine”. So she finally, she was at the point where she understood. And so now every time before dinner she goes, “daddy, you take your peels”

**Tiffany (CF Spouse and Caregiver):**before any meal

**Caleb Remington (CF Person):**
And so yeah, it's like the most adorable thing ever. And so now I got Faye to remind me.

**Caleigh (Host):**

That's so cute. I feel like that sentence alone could be like, I guess I could have kids... Nah, don't get enough. Love it for you guys though.

So Tiff, you are such a big advocate in life in general, not just for cystic fibrosis, but for mental health, for ivf, for so many things. And you share it a lot on your guys platform on at us, the Remingtons, which the community appreciates so much.

And it's been really, really cool to see you guys grow and see your story spread. It's been really nice as, as your friend to see that.

Do you have advice for people as to what we can do as a community to advocate for equality and access to medications both in the United States, but, but also worldwide?

**Tiffany (CF Spouse and Caregiver):**
I think the number one thing is knowing that everyone's story and everyone's voices matter. I think a lot of the times when it comes to change, we all think like we have to either fundraise x amount of dollars or we have to have a huge following or we have to have this like incredibly deep and like huge story to share.

When in actuality that's, that's not it at all. It's like just feeling comfortable enough and confident enough to share your story in whichever way that is, whether if that is through writing, whether if that's sharing on social media, whether if that's sharing your story with your local chapter at cystic fibrosis events. I feel like just, or just even sharing your story with your friends and your family and your circle.

I think that has so much potential to be able to change not only the narrative for cystic fibrosis in general, but just create this safe space where people feel like they are able to share and feel safe enough to share.

I think on a higher level we have to keep the pressure on from every angle, whether if that's like legislative, pharmaceutical or like grassroots. And like I said, that's sharing our stories, connecting with lawmakers, supporting local organizations.

I think, you know, for us specifically and Caleb, who's benefited from gene modulators, it's a responsibility to stand up and help bridge that gap and to continue to fight, raise the noise until everyone has access to like basic care.

I think there's a lot of fighters out there that aren't able to access the care, whether if that is because of a specific rare mutation geographically or just systemically. I think it is our responsibility as advocates to continue to create and raise that awareness that not everyone has the same access to care and that's not okay.

**Caleigh (Host):**
It was amazing. Answer.

Every voice and every story is unique and you never know who is going to hear that story and who might open up their, their heart and their want and feel motivated to share that to more people. Or maybe it's somebody that you don't know, but just happened to be a scientist or a doctor or a friend of somebody who's newly diagnosed.

I mean, you never know. The web goes so much further, I think, than any of us think or realize. And so sharing our stories is really important.

I do want to give a couple of facts about Trikafta quickly. Trikafta's list price in the United states is over 300,000 a year before insurance. As of 2024. Trikafta is approved in over 35 countries, but availability and pricing vary dramatically in many low and medium income countries. Trikafta is not yet approved, funded or available at all.

So yes, these stories matter and your voice matters and sharing the impact that it has on your life, Caleb and Tiffany, or listeners lives is really huge in like you said, raising up together and sharing and that's really beneficial to people all over the world who suffer from cystic fibrosis.

So thank you so much for talking on this podcast and every podcast, at the end of the show we ask questions from the community.

So these are questions that people in the community DM'd specifically for Caleb and Tiffany to hear their opinions and their thoughts.

So either of you guys can jump in and answer whoever wants, wants to.

The first question is, “what would you say to someone who's just about to start their journey on gene modulators?”.

**Caleb Remington (CF Person):**
I would say if you're just about to start your journey on gene modulators - prepare for the clearing. I heard that was a thing. I didn't realize it was a real thing. And so although I was like ecstatic about it. Yeah, the migraines were horrific. Yeah, it was, it was just a pretty brutal two weeks. But yeah, obviously there's a light at the end of the tunnel. You're gonna feel great. You're getting it all out.

Definitely there's, I mean this, if you already have access to the, to the modular, that's great. But yeah, for me like switching insurances has also brought up a ton of issues because you have to get a new prior authorization.

Definitely like now the CF Foundation. I don't know if any other chapters do this or clinics, but like you have to go to your three month checkup and you have to get specific blood work done to make sure that your bilirubin levels and other indicators are good so that you can stay on trikafta. I know I've heard some people say that they had to come off for a few months because their levels got too high.

Anxiety is definitely another symptom I've heard throughout the community where it's just heightened. I know they came out with an even newer modulator from Trikafta that is supposed to help with anxiety. So people who have suffered from pretty severe anxiety are switching to this newer version. I haven't quite looked into that to see if it would benefit me in any way.

But yeah, I guess for me the, the anxiety actually kind of comes from living longer and like being more of an individual and needing less support from others. And so it's a, it's, it's a drastic life change like physically, but then emotionally and mentally too, where you're like, I could go out and get a job now I feel good enough to contribute to society in whatever fashion that is. And so yeah, it's, it's like everyone has their own unique journey and so I just send you all the love in feeling better.

**Tiffany (CF Spouse and Caregiver):**
I'm going to piggyback off that.

So, and this is just more so of like observation on the community at large.

You know, when you start a gene, any, whether if it's a gene modulator or a new treatment or anything, keep a diary. Not everyone reacts the same way. Caleb is very fortunate that, you know, trikafta really worked well for him.

But you know, as Caleigh, you tried being on Trikafta for a little bit and I know that you had some complications with GI issues and all that stuff.

So like it's not, it might not be the treatment for you. So just be aware of that and be very honest with your medical team and maybe there's like different things that you can try out, but I think making sure that you are, yeah, just fully aware and heightened on like what you're feeling.

And obviously Caleb said yes. The two to four week of clearing is definitely rough, but like, if there's anything that persists beyond that, definitely reach out to, to your medical team and keep them in the loop about it because that is something to take note of and something to yeah. Take into consideration when it comes to your care.

**Caleigh (Host):**
Last question, “Many people in the CF community experience survivor's guilt, especially when others are still waiting for access. How do you both process those feelings and what helps you navigate them?”.

**Caleb Remington (CF Person):**
Yeah, survivor's guilt is a real thing. I've sort of. There's. There's so many angles to that.

The biggest one was I have a little bit of empathy because I watched, you know, two waves of the community get a modulator. And so I was on the semi rare where I, I watched the G511D work and then. Or Cambie, which if you had a double Delta F508 copy, then, you know, that was like 60% of the population in the US got access to this drug. So I was like that third wave. And so I kind of have this like, I wish that was me sense when I watched all a bunch of people I knew go on or Cambie and talk about how great it was.

And so I sort of have that same fight because I know what it's like to not have access to the drug. And so we still continue to do everything in our. With all the time and energy we have left after having two kids, you know, dedicate that to raising money and awareness for the disease. I, I talk with genetic counselors from time to time to give them my perspective on, on cf and just doing this podcast, like there's so many things that we can all still do to. To help fight for that last 10% and anyone globally who doesn't have access to this.

**Tiffany (CF Spouse and Caregiver):**
Yeah, I think we kind of touched on it prior to too.

You know, it is hard to celebrate when you know that again, not everyone has access to basic care at this, at this point, we do live on the fine line again of gratitude and guilt. I think that's just very human of us.

And especially like what Caleb was saying. He has empathy because he has been also on the other side in the weight.

And I think if anything, we use that as our fuel to our fire to really do the best that we can to advocate.

Caleb has been in a few clinical trials to help kind of move that needle forward. Obviously that is a big commitment for other people, but there's just so many other ways that you can continuously get involved, even if that's again, by sharing your story on social media in your small circle. I think doing those small things as often as you can is really how we really get the last 10% cross that finish line. And until we all have a cure. Finding local organizations, there's so many other wonderful cystic fibrosis support organizations that are out there, Emily's Entourage that specifically focuses on rare mutations for cystic fibrosis patients. So, like, yeah, just kind of expanding and expanding your knowledge and your research around that and, yeah, continuing to fan the fire in all different directions.

And know what your capacity is. You know, there are going to be some eras of your life where you're going to be able to really put your boots down on the ground and really move fast and furious. And there are going to be other points in your life where you do maybe have to backtrack a little bit because of health, because of family, because of, you know, just certain things that come up and just know that that's okay. And we're all doing our best to make sure that, yeah, everyone's stories heard and that everyone will have treatments that are appropriate to help them.

**Caleigh (Host):**
Well said. Thank you so much for coming on the podcast. I miss you guys. For everybody listening out there, just stay hopeful and stay motivated and try to keep yourself as healthy as possible so that when there is a cure, you're at your healthiest point and you can go live life.

To everyone listening, thank you for being part of our fight to breathe. This podcast exists because our stories matter. Whether you're thriving on trikafta, struggling with side effects, or still waiting for your breakthrough, you're not alone. We believe that every story has the power to inspire change. Share yours with us on social media. @FightItobreathe. Or email me [caleigh@fight2breathe.org let's keep advocating, supporting, and speaking up until next time, thank you for being part of this community. We breathe better together.

And a heartfelt thank you to the Cystic Fibrosis Foundation's Impact Grant for supporting this podcast.