Trikafta update

Today marks six months (180 days) on Trikafta for me! 🎉

Quick explanation for those unfamiliar with Trikafta: it’s the newest of four drugs called CFTR modulators. These medications are specific to cystic fibrosis and help correct the underlying defect in our cells rather than just treating symptoms.

I started Trikafta in November, near the end of a 2 week hospital stay. For some people, within hours of taking their first dose they noticed changes in their breathing or how their body was feeling.

I was NOT one of those people.

The changes I experienced were slower and more subtle. I began feeling like I was disappointing people when they’d ask how I was feeling because I didn’t have much to report.

My biggest “wow” moment happened when, after two months on T, I was sledding with my extended family and ran up a hill without thinking twice about it. I don’t run ever run, but especially not uphill! When I realized what I’d just done, I did it again and again.

Now, six months into this journey, I still can’t tell you when certain things happened since it’s been so gradual, but I can tell you that my body has indeed transformed.

For one, I don’t cough anymore. It’s the most bizarre thing! Something that has been so normal my entire life is now just gone!

I saw a roughly 8% increase in lung function, giving me my highest PFTs since 2014.

I rarely cough up any mucus. My treatment needs haven’t changed – I’m still doing just as many as I was previously, but I’m not getting a mouthful of mucus each time I cough. (CF is so glamorous, isn’t it?)

My digestion has changed significantly, and I’ve gone from needing to take 5 enzymes with meals to 2.

I’m not sure many people understand how painful CF can be on a daily basis. Lungs, joints, stomach, sinuses, overworked muscles — something is always hurting. But I now have more pain free days than I’ve had in years.

This isn’t even a complete list of the changes I’ve experienced, just the most significant ones. Most days, I still have a hard time wrapping my head around it all to be honest.

Every single day, I am amazed.
Every single day, I remain hopeful.
Every single day, I am grateful.

Whose body is this?

Life is funny.

Shortly after I posted about my amazing PFTs, my doctor called with some news. Turns out, the X-ray she ordered “just in case” showed that I have pneumonia in my left lung. I’d been experiencing some pain in a couple areas, but no other symptoms.

To say I was confused is an understatement.

Two things that I regularly stress are that 1) CF is insanely unpredictable and 2) numbers are only part of the bigger picture.

If we had just looked at those beautiful numbers, we would never have guessed that something was brewing. Because my doctor wanted to be thorough, we were able to catch this (hopefully early) and start treatment to take care of it before it turned into something much more scary.

I’m still blown away by the fact that I could have such great PFTs while also experiencing pneumonia. That leaves me feeling even more grateful for the effects of Trikafta which have likely kept me out of the hospital.

But it’s also incredibly unnerving to feel so disconnected from my body.

For so many years, I’ve been so in tune with every feeling, every single symptom. I’ve been able to tell the moment something seems off. But this time? I truly had no clue. I’m not having the same symptoms that would previously let me know, “Hey, girl, something’s going on in here.”

Learning this new Trikafta body might be even trickier than I thought. But I’m so grateful I have the chance to, and I feel extremely fortunate to have a whole team of people helping me learn how to navigate this new normal.

Mind: Officially Blown

Yesterday, my lung function was the highest I’ve seen since 2014!

I think I’m still processing that a bit.

PFT (3)

I’ve gained 8 percentage points since starting Trikafta in November. Many others have seen improvement far beyond 8% but I’m not complaining! I went into this experience with cautious optimism and absolutely zero expectations. I told many people that if I could simply maintain my level of health and quality of life, I’d be happy. But to see these improvements is a little mind blowing!

I’m not one who generally puts too much stock in numbers. I’ve talked before about how CF is a total numbers game, and sometimes it’s detrimental to obsess over them. Numbers are a helpful tool to measure what’s happening in the body, but they aren’t the whole picture.

Yesterday my doctor reminded me that for those of us with structural lung disease and decades of damage to our bodies, there may never be those HUGE improvements in numbers (although, to me, 8% is still pretty huge). We will always have lung disease. That’s not something to get discouraged about. This drug is also working in other ways, as evidenced by my improved digestion and clearer sinuses.

When Randy first entered my CF world, he asked if lung function was kind of like a cell phone battery – the higher the numbers, the longer the battery life. I told him it’s far more nuanced than that, but for someone who was just learning about CF, that was a decent analogy.

My whole life, I’ve known that battery will drain out. I’ve seen that scenario play out over and over as I’ve watched friends battle and ultimately lose their lives to this disease. It was never a matter of it, but a matter of when my battery would burn out.
When I saw those numbers yesterday, even though I know they’re only one piece of this complex CF puzzle, I couldn’t help but visualize that battery getting a little charge.

And it truly feels like years are being added onto my life.

We’re in this together

“How long have we been on Trikafta?”

Morgan asked this the other night and I’m still smiling about it.

Fighting CF is not something I do alone. I know this. I have friends, family, and care providers who are deeply invested in this battle. But I’m never more cognizant of how CF affects my loved ones than I am in moments like this.

“How long have we been on Trikafta?”

That one word was a reminder that my health affects her every bit as much as it affects me. She doesn’t feel the physical symptoms of this disease, but it has shaped her life in more ways than I probably even realize.

My fight is her fight. She shares in the celebration of my triumphs and the heartache of my challenges. My future and hers are intricately entwined.

She’s right – this isn’t just about me. It’s about us. We’re in this together.

We’ve started this thing where, when we notice the subtle (or sometimes not-so-subtle) ways that my body and health are changing – like when I run up a dang hill or swim the entire length of a pool — we look at each other and simultaneously whisper, “Trikafta!”

I feel so fortunate that I’m able to take this drug. It’s not just changing my life, it’s changing hers as well. I still really struggle with the idea that so many don’t have access or won’t benefit from it. But I’m overwhelmed with gratitude every time I see this little girl’s face light up because she knows her mama is feeling good.

Mom and Morgan

We’ve officially been on Trikafta for 72 days.

And we’ve expressed our gratitude out loud each and every one of those days.

Originally posted January 27, 2020

No longer confined to the sidelines

I’ve been on Trikafta for just over two months now, and I feel like it’s been a bit of a disappointment when I’ve said that my day-to-day life hasn’t changed that much. This drug is supposed to be “life changing” after all.

Well, yesterday we went sledding and at one point, my nephew was waiting for me at the top of the hill so we could sled down together. So I ran up the hill to meet him.

Did you catch that? I ran up a hill!

If you know me, you know that I don’t like to climb hills, even when I’m “hiking.” And I don’t run. Ever. For any reason. It’s just too painful.

But yesterday, I ran up that dang hill more times than I can count. I didn’t collapse or nearly vomit from coughing, and my lungs didn’t hurt or feel like they were seizing up. I felt fine!

Although at one point, I did sit down at the top of the hill and cry because I was hit with a startling realization. I wasn’t watching from the sidelines as I so often do. I was able to run and play with my kid for the first time that I can remember. Do you know how crazy that is?!

Last night as I was putting Morgan to bed, I felt tears on her cheek as I kissed her goodnight.

“Oh, no, honey. Why are you crying?” I asked.

“Because you ran up a hill,” she sobbed. Then she assured me, “they’re happy tears.”

This. Is. Life. Changing.