Orkambi isn't the top of the mountain.

Today was my first practice hike to prepare for my Xtreme hike in September. Today also just happens to be two years since I took my first dose of Orkambi, a medication that for the first time in my life, actually treated the underlying cause of my cystic fibrosis, rather than just tried to counteract the symptoms.

Celebrating two years of this life-changing medication
with a casual stroll through the woods

When I got up this morning, I was full of hope and excitement. I had it all planned. I was going to hike up that mountain and when I got to the top of the lookout and stopped for lunch, I would take my anniversary dose of Orkambi and soak up a moment that two years ago, I never dreamed I would be having. I even told one of my best friends that I might do a Facebook live and share the excitement with everyone on here. I've had an amazing two years thanks to Orkambi and had so much support through it all, it only feels right to share this milestone too.

Yeah, so here's the thing about having a chronic illness.... It doesn't give a damn about what you have planned.

Chronic illness, whether it's CF or any number of other ailments, doesn't care about your dreams or your milestones. It's relentless and no matter what, will always find ways to pull you back with every step you take forward.

Today, as soon as I started hiking, I knew something wasn't right. I couldn't breathe. at. all. The incline was far steeper than I anticipated and I was moving very slow. About 1/2 a mile in, after getting to a point that I couldn't take more than 15-20 steps without feeling like I was going to pass out from being so short of breath, I stopped and checked my O2 levels. It was 86%. Less than 88% is considered desaturating and is the requirement for needed supplemental oxygen. I have never needed oxygen for exercise before, so this shocked me. Actually, no... it terrified me. Seeing that number on the screen made my stomach sink. My health has declined since my hike last year but up until that moment, I felt confident I still had what it took to make it this year. Sitting there starting at my pulse ox though, I genuinely doubted if I was pushing myself too far. I mean seriously... 3 months ago I was getting evaluated for a double lung transplant and now I'm trying to climb a mountain?

Knowing that on this particular hike, the incline was going to get much worse for about a mile before it leveled out and I was already pushing an hour behind the group, I made the decision to turn around and adjust my plans. Our lead hike guide is a good friend and he helped me find another flatter trail that I could take so that I would meet up with the group at the end of the day. This modified trail meant I was only hiking around 7 miles while my friends hiked closer to 13. While I know that 7 miles is still a great effort and quite a workout for my lungs, I was angry. I was disappointed and I felt defeated.

I didn't make it to the top of the mountain today. I didn't get to have my amazing "aha moment" to the celebrate two great years I've had since starting Orkambi. As the day went on though, my disappointment turned into motivation because I realized that in a weird way, this was actually more fitting....

Orkambi isn't the top of the mountain. Orkambi isn't an aha moment.

Orkambi is definitely a reason to celebrate, but not the reason to celebrate. Not for me and certainly not for the thousands of individuals living with cystic fibrosis who aren't eligible to take it and are waiting for the next generation of therapy. Orkambi is just one step towards a future where I and so many others don't have to adjust their plans and adjust their lives because of this heartless monster of a disease. Remembering that is what gave me the strength to not just take another step today, but to take 16,400 more steps.

Today was really, really.. really tough. I don't know why my lungs were being such brats. Maybe it was just a bad day, maybe I'm more out of shape than I realized. Maybe my lungs really don't have it in them like they did last year... I'm not sure. But I'm going to keep training and see how things go on the next practice hike, adjusting as I need to until I get where I want to be. Even through the setbacks, I know I have to keep hiking. I have to keep trying. I have to keep moving forward. Just like we as a community have to keep moving forward.

Part of the amazing community here in Virginia! 

The last two years of my life have been ones that I never could've imagine for myself and these little pink pills are a big part of why. These pills exist because of the millions of dollars that have been fundraised for cystic fibrosis research over the last two decades. They are worth celebrating, but they aren't the top of the mountain that we are all aiming for and they aren't the moment that tens of thousands are desperately waiting to celebrate. That moment comes when every single person fighting this disease has a medication that treats them on a cellular level. That moment comes when we find a cure.

Please consider supporting my hike by making a donation today. Donations can be made securely online on my personal fundraising page, http://fightcf.cff.org/goto/jilliangoodwin-hike.

Thank you for listening and joining me on this very eventful day! Stay tuned for more updates!