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.

The stuff of nightmares

Early this morning, right before she awoke for the day, Morgan had a dream that I lost access to my medications. She then sent the following text from under her covers with tear-filled eyes.

“Momma, are you gonna lose all your meds?”

This is the fear we live with. I don’t just mean myself or my family, but also millions of others who are disabled or chronically ill and rely on expensive medications to stay alive.

While I currently have access to comprehensive healthcare and affordable prescription copays, I know firsthand that something as simple as a small change in personal circumstance, the whim of a health insurance company, or a faulty piece of legislation are all that stand between myself and a total healthcare crisis.

It’s exhausting.
It’s frustrating.
It’s sometimes terrifying.

This morning, after receiving her text, I called Morgan in to my bedroom to talk about things. We both expressed our thoughts, I offered reassurance, and we snuggled for a while. By the time we got out of my bed we were laughing, and I think that for a while at least, her worries have subsided.

I’ve said before that living with CF oftentimes feels like waiting for the other shoe to drop. Even when things are going well, uncertainty lurks in our minds and the minds of those who love us.

It is quite literally the stuff of nightmares.

My child’s knowledge of CF

(CW: talk of death)

Morgan was just six years old when she first asked, “Mama, can you die from CF?”

She has known the words “cystic fibrosis” for as long as she can remember. From the time she was a baby, she’s been immersed in the world of CF. She’s attended clinic appointments, stayed countless nights in the hospital with me, and seen me do treatments her entire life.

As a parent, I’ve tried to create a culture of openness in our home, which has included candid conversations about CF.

I’ve allowed her to guide the conversations, never giving more information than she asks for, but also being as honest and transparent as possible.

This has been helpful for her (as her anxiety is lessened when she knows exactly what is going on) but it has also led to some difficult conversations throughout the years. Like the one mentioned above.

“Can you die from CF?”

I believe that honest questions deserve honest answers, so at the tender age of 6, she and I had our first (age-appropriate) conversation about CF and the possibility death.

Because she knows she can come to me with any question or concern about my health, she is never left alone to wonder. While she understands that the realities of this disease can be harsh, she also knows that I am filled with hope.

Not only that, but she understands that I do everything within my power to ensure that I’ll be here as long as possible. She knows that pills, treatments, and hospital stays are all things I can do to stay healthy – so she cheers me on and holds me accountable.

CF adds a dimension to parenting that can be difficult and terrifying. The ways in which we choose to talk with our children about it will be as unique and individual as they are. Not all kids will benefit from the approach I’ve taken, and not every parent will be comfortable with that. I believe we intuitively know what’s best for our children.

For us, this approach has worked well. It’s what she has needed and explicitly thanked me for now that she is a bit older.

The hard things are always peppered with hope.
The scary moments are tempered by love.
And we face it all hand-in-hand.

Finally taking a breath

“This means so much to me,” I said to the sweet nurse administering my first dose of the COVID-19 vaccine back in January.

“Living through this pandemic as a high risk person has been… a bit stressful.” I chose my words carefully, not wanting to regurgitate my many personal frustrations onto her unsuspecting lap.

What I didn’t say was:

This has been so scary.

I absolutely hate the way we’ve been treating each other throughout this pandemic.

I’ve been shocked at the ease with which so many were willing to sacrifice the safety of the vulnerable for their own comfort and convenience.

I’ve been enraged by the politicization of medicine and science and human lives.

I’ve been saddened by the way this last year has forever changed some of my relationships.

I’ve been enraged and heartbroken by the massive amount of preventable loss we’ve experienced.

Those were the things I didn’t say, but as we talked a bit more, it became evident that I didn’t need to. She understood and shared some of her own frustrations and experiences with me.

For a moment, right before she gave me the injection, we looked each other in the eyes and smiled behind our masks (you don’t even need to see faces to feel genuine smiles; the eyes are always a tell).

“Well, maybe now you can take a breath, honey,” she said warmly.

• • • • • •

I was lying awake in bed last night and this conversation came flooding back into my mind.

When I hear or read people talking negatively about these vaccines or still debating the necessity of taking precautions (which, let’s face it, happens on the regular) I just remember this conversation and the peace it brought me. Her kindness and the moment of connection we shared made an exciting experience even more memorable.❤️