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.

Because "not doing something" is not an option.

If you’re reading this, you probably know that when I was 3 months old, I was diagnosed with cystic fibrosis (and if not… well, now you do!). CF a life-threatening, genetic disease that primarily affects the lungs and digestive system of over 35,000 children and adults in America. CF is caused by a defective protein inside the cell, which prohibits salt from entering and exiting at a normal rate. This salt imbalance causes the body to produce abnormally thick, sticky mucus that clogs the airways of the lung and leads to life-threatening infections and irreversible lung damage. In addition, the thick mucus obstructs the pancreas from distributing digestive enzymes that allow the body to properly absorb food. Without these enzymes, most individuals with CF are incapable of digesting necessary nutrients from their meals. Cystic fibrosis can also cause a number of secondary conditions such as diabetes, liver disease, infertility, osteoporosis and more.

There is no cure for cystic fibrosis. When I was born, the life expectancy was just 20 years old. While it has since been raised to around 40, it is still a debilitating disease that significantly reduces quality of life and ultimately, is fatal for all patients. For the last 25 years, I have fought a daily battle against cystic fibrosis. I take over 20 medications a day and spend hours doing inhaled treatments and respiratory therapy to try and maintain my lung function. This spring, I began the process of getting evaluated for a double lung transplant, a last resort option for when my lungs are no longer capable of providing me the oxygen I need to survive.

During one of my transplant appointments in May, a psychologist asked me "What do you look forward to being able to do for the first time or do again?"