"Taking Brave Steps" by Elena Goodrow

Greetings my friends, I’ve had a lot on my mind recently. While I originally wasn’t planning on sharing my thoughts on this specific topic, I thought to myself… why not? I encourage you all to share your experiences with me, praising your honesty and vulnerability. I suppose that means I should do the same for myself. Practice being vulnerable, and know that this community will always love me for where I’m at. 

The Make-A-Wish foundation released a statement in late March announcing that cystic fibrosis is no longer a qualifying condition to receive a wish. The Make-A-Wish board of medical professionals will still review applications from CF patients, but they will only receive a wish if they deem the patient as having “additional complications or factors that make the current situation critical." They made this change because of all the recent advancements for CF, most notably the medication Trikafta. Because of all the advancements Triakfta has resulted in, the Make-A-Wish Foundation no longer deems CF to be “life-threatening” enough to automatically qualify for a wish. This timing feels incredible for me, as I just went on a Make-A-Wish trip last year. How crazy! I saved my Make-A-Wish trip until I was 17/18 years old because I wanted to wait until I was older because then I would be better equipped to pick the perfect wish for me. Plus those memories from the trip would be more solidified in my matured mind. Even though as a young child my family encouraged me to think about using my wish, they knew that I had the privilege to save it because I knew my health was stable enough. I’m so fortunate to have gotten to save my wish, and in 2022 my family went to Hawaii! Having that experience as an 18 year old young adult was so formative and magical. I’m glad I waited because it made my trip so impactful. That being said, I also think it’s wonderful when people use their Make-A-Wish as soon as they can. It’s such a valuable opportunity and I love that people can use it in a way that is best for them.

It feels unreal that a nationally recognized non-profit such as the Make-A-Wish foundation has made such a major change to their guidelines. To make things even crazier, that same week the CF Foundation announced the new life expectancy for CF patients is 56 years old. (This number and associated information are very complicated and that undertaking is for another day. But, the wonderful takeaway is that people with CF are presented with the previously unfounded possibility of living longer)

All of these things are incredible, and I’m so glad that CF fighters have new opportunities to live a longer, healthier life. However, these changes also feel scary for me. All of the sudden my life has been turned upside down. With CF you learn to expect the unexpected, you never know what the next day will bring. I’ve gotten so accustomed to this lifestyle that I can’t imagine a life where I feel confident that tomorrow will bring good health. This is my truth, but this doesn’t apply to every CF fighter out there. Yes there have been incredible advancements in our community, but not everyone is eligible for it. There are still those with genetic mutations that cannot take Trikafta or other modulators. There are still countries where these modulators aren’t even approved yet. Let’s say you are fortunate enough to be eligible for Trikafta, there is still not a guarantee you can afford it. The world of health insurance is brutal and can be unforgiving. I’m one of the lucky ones whose insurance covers Trikafta, and I’m forever grateful. However even if someone’s insurance covers 75% of the cost that can still be thousands of dollars out of their own pocket.  There are so many moving parts that revolve around access to this medication, I’m only dipping my toe into this whirlpool. The advancements made in these past several years are changing lives in the best ways, but there are still lives being left behind. There is still work to do and we can all keep fighting for ourselves and for each other.

In addition to all these major changes that have impacted me, there are also relatively smaller aspects of my life that Trikafta’s effects are seeping their way into. My doctor recently suggested to me that I look into trying to lose weight. I was absolutely floored when he said this. I’ve never heard those words my entire life. Since I was a baby I’ve been told I had no choice but to eat until I feel sick. I was told to eat whole sticks of butter, giant bowls of popcorn, piles of bacon or sausage, anything that I ate I would eat triple the amount than I wanted to. Same thing goes for salt, now my taste buds are so used to things being very salty that I can’t even taste salt unless it’s piled on. It’s been this way my whole life, and it feels like at the flip of a switch everything is different. Realistically, the effects of Trikafta have been unfolding for a couple of years now. I’ve known that my life will change, and that the CF world will never be the same. Yet it still feels like it’s come on so fast just within the past month. For the first time we are seeing the way Trikafta impacts the lives of people with CF after taking it consistently for a year and more. I’m so incredibly thankful for the people that created Trikafta, all the work and donations that went to that research is remarkable. I’m thrilled for the CF community and how some of our lives will never be the same. Still through all this joy, I feel the immense fear of change. CF doesn’t feel like it defines me anymore, it’s not something I mention to people because it rarely comes up anymore. Unless they notice my taking my medicines before I eat, I no longer sound like I’m struggling when I take deep breaths. I don’t have a thick layer of salt on my skin when I go on a hike with a friend. I don’t even use my vest much anymore, what is this reality?! It doesn’t feel real. Yet my reality is still so different to the reality of others that haven’t had access to Trikafta, that have taken it but seen no change, or those that came before us that contributed so much to help us get to where we are today. There are still people that have declining lung function, trouble with weight gain, who could never skip a physio session, those in advanced disease state, people being hospitalized frequently, transplant recipients, and SO much more. 

Let’s live in this joy and fear together. Taking brave steps through this new uncharted territory, our community at our side. No one gets left behind. We are in this together no matter how your CF story is unfolding.

Can anyone relate to how I’ve been feeling? How have massive changes within our world been impacting you? Both positively and negatively?