Sometimes difficult choices pay off: a Trikafta update

Originally shared to Instagram in a three part mini-series

Part One

“You don’t want to believe the drug that is saving your life is also kind of ruining it,” I said with tears in my eyes.

My doctor and I were 20 minutes into one of the more difficult discussions I’d had in recent memory.

“When I look at who I am today compared to who I was 2 years ago, it’s a completely different person. I don’t recognize myself.”

A few days prior to this appointment, I had promised myself that I’d be open and honest about the mental health struggles I’d been experiencing; I promised I’d finally admit how bad things were.

As a person prone to anxiety, it only made sense that it would escalate during the dumpster fire we call 2020. But it was more than that…

I’d begun experiencing some depression, severe brain fog, irritability, persistent insomnia that didn’t respond to sleep aids, and the worst anxiety I’d ever had. My daily level of stress and overwhelm had become nearly unbearable, and it was totally unwarranted (even given the circumstances of the world).

I’d written many of the symptoms off as purely situational, but I also recognized that some had begun before COVID, before 2020. They’d started very soon after I began taking Trikafta and had increased with time.

But, as I said, you don’t want to believe the drug that is saving your life is also kind of ruining it. So, I didn’t even let my mind go to that place…

Part Two

Why not try different/stronger sleep aids? Did you switch the am and pm doses? Drinking a lot of water helps reduce side effects; did you try that? Can’t you just take anxiety meds?

These are all things I’ve been asked, and they are all valid questions. However, for several reasons, my doctor and I feel strongly that my specific issues are linked directly to Trikafta and that adding medications (that can all have unpleasant side effects of their own) was not the right course of action.

I didn’t want to stop Trikafta – it’s been amazing in so many ways – so we decided to try reducing my dose. Straight from the clinic notes from my last visit, my doctor says: “We discussed the potential harm of reducing a drug that has been so beneficial with her lung disease. It will be up to her to decide how many side effects she is willing to endure in exchange for those pulmonary and hospital reduction benefits.”

Finding a balance has been the overarching theme of this whole experience for me, and it is indeed a delicate balance to maintain. My physical health is incredibly important, but so is my mental health.

I’ve waded through a sea of emotions since that day in clinic. I’ve felt mourning for the potential loss of something great. I’ve felt sadness. I’ve felt guilt. I’ve asked myself, “How can you do this when you’ve spent the last year and a half singing the praises of this drug?”

But here’s the thing: I’ve also felt incredibly empowered by this decision.

I am not ungrateful, in fact, I am bursting with gratitude for a supportive CF team and community, for the opportunity I’ve had to take this medication, and for the continued work happening to fine tune these drugs and find an even better treatment option (we’ve said all along that Trikafta is not the “end all be all” of CF treatment).

In the end, we each have to do what’s best for us, and I am confident that this has been a good choice for me.

Part Three

Okay friends, here’s where I tell you the good news. It’s been a month since I adjusted my Trikafta dose, and in that time, my mental health has improved dramatically!

Within just a few days of the change, I was sleeping much better. After months and months of barely sleeping, I cannot express how happy this made me! Turns out, sleep is kind of important to human health and survival. Who knew?

A couple weeks into this experiment, I told Randy, “I’ve felt more like myself the last few days than I have in a really long time.” To which he replied, “I can definitely tell!”

My general mood has improved. That soul-crushing anxiety has eased significantly. I’m able to focus on and actually complete my daily tasks. I’m not nearly as irritable or angry.

All of these things seem to verify that 1) my symptoms were indeed related to Trikafta and 2) lowering my dose was absolutely the right decision!

The downside is that I’ve experienced the return of some physical symptoms. We knew this was a possibility and I went into this situation knowing the potential outcome. However, at this point, even a half-dose of Trikafta seems to be more effective than previous modulators were for me. I’ll go back to CF clinic and also see my ENT in a month to get a better idea of how things look.

When I was in the middle of it all, I don’t think I truly realized the shape I was in. Looking back now, without such a dark cloud hanging over me, I can clearly see how much I was being affected mentally.

It feels great to be moving forward with the mental and emotional fortitude to roll with the punches of life once again. And maybe I’ll even throw a few punches myself (while wearing a feather boa, of course).

I don’t share this to discourage anyone. In fact, I hope that as you read this, you can feel the optimism and hopefulness I have for the future. I truly mean that. Things will only get better from here as we learn more about these therapies and how to improve them. The future is so dang bright, I can hardly stand it.

Mental Health Matters – Trikafta update

(Originally shared on 7/22/2021)

I had CF clinic today and here is the biggest takeaway: sometimes we have to do hard things and it’s okay to have mixed feelings about something.

I’ve talked a lot about the benefits I’ve experienced on Trikafta. I am endlessly grateful for the ways in which it has improved my life with CF. But at the same time my physical health has been improving, my mental health has been declining. Recently, that decline has been more rapid and noticeable, negatively affecting my daily life and relationships.

For a long time, I tried attributing some of the issues to stress or feeling anxious about life in the time of COVID. But I’ve also worried (and deep down, I think I knew) that it was at least partially related to Trikafta. The symptoms have become too much to ignore, so today — with the wise and loving guidance of my incredible doctor — I made the decision to lower my Trikafta dose.

I am not stopping entirely, and we aren’t absolutely certain that this change will minimize the negative side effects. There is also the chance that lowering my dose will mean I receive less benefit and some of my old physical symptoms may return.

But I feel like it’s worth a shot and I’m approaching this change with optimism and hope. I’ll admit, I cried a lot today. There are worries, heartache, and a little mourning happening right now along with hope for a better balance between my mental and physical health.

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.