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?"

My Steps Towards Transplant: The Weeks Before

Yesterday, I started a series called My Steps Towards Transplant to share my story as I begin the process of getting a double lung transplant for my cystic fibrosis. In part one, we looked back on my decision to get a evaluation, which really began more than 8 years ago. I shared my experience on how I got to the point of making the decision to move forward with a transplant and how it was a relatively easy choice for me to make. You can read the full story here, My Steps Towards Transplant: The Decision to Evaluate.

Today, I want to talk about the weeks before my evaluation. The logistics or timeline, if you will, what steps I took to prepare and most importantly, how it felt.

My Steps Towards Transplant: The Decision to Evaluate

Four weeks ago, I officially began the evaluation process for a double lung transplant. But truthfully, my transplant experience started way before that. And it's going to continue far beyond that two-day evaluation. Today, I'm starting a blog series called My Steps Towards Transplant. This first week or two will chronicle my journey (so far) with transplant, from the first time I heard the words "double lung transplant" to the results of my evaluation. In the next few months/years, I'll continue to share my story as I move forward in this chapter of my life with cystic fibrosis.

I wanted to write this series for a few reasons. When I first publicly announced that I was being evaluated for transplant, I had an incredible outpouring of love and kindness. I started a public Facebook page, as a way to continue being an advocate and spread awareness about cystic fibrosis, but also to share my new transplant journey. I had over 500 likes in just two weeks and had dozens, if not hundreds of comments and messages. Family, friends and even strangers from all walks of life were getting in touch with me to say they were thinking of me and they loved me. I had so many people supporting me as I started this new process. Sadly, I haven't had a chance to update any of them on how it went, because immediately following my evaluation, I ended up getting a cold and was sick for several weeks... cystic fibrosis always has impeccable timing like that. I have chatted with family and some friends when I've seen them recently, but the majority of my friends don't know what happened, so that's why I'm writing now (p.s. here's a shameless plug to say please like my Facebook page, High Heels & Hospital Bills if you haven't yet :D ).

The second reason for this series is because in preparing for my transplant evaluation, I had questions.

What does CF "really look like?"

So it's Cystic Fibrosis Awareness Month and that means that Facebook and many other social media outlets are exploding with posts about CF. What it is, what it means, who has it, why we need funding. I personally have zero problem with talking about my cystic fibrosis for the rest of the months, but it is encouraging to see the community come together every year to bring much needed attention to this illness that affects us so deeply. I've noticed, however, a few posts, discussions and dare I say arguments about "what CF really looks like" and that it's not glamorous or pretty. I've seen this recently and on a number of occasions in the past.

Some think that the best way to bring awareness to is to show the graphic photos. Photos that bare witness to our most fragile state. Photos inside the hospital walls because most people will never have the chance to see something that raw. Others, take a different approach. They aren't as comfortable sharing that vulnerable side of their disease and they would rather post a picture where they look healthier, maybe with their make up and hair done or even a fun snapchat filter. Or maybe they don't want to be labeled as "sick" and they'd rather share the "normal", non-CF side of their lives as a way of showing the world that they're just like everyone else.

Well I, being who I am, have a few thoughts.

Ten years later.

Ten years ago today, I was admitted to the hospital. I was 15 years old and prior to that day, I had only been admitted to the hospital three times in my life. For someone with cystic fibrosis, this was actually pretty impressive. Growing up, I knew that I had CF but admittedly, I didn't know what it meant to be "sick."

I did my breathing treatments, took enzymes and vitamins and the occasional round of oral antibiotics. I had doctors appointments every few months. But between the fact that I was still very much a child and naive about my illness, my "doctor Mom" doing everything she could to keep me healthy and what I can only choke up to being "lucky," I hadn't yet experienced the harsh reality that many with cystic fibrosis face. Honestly, as a child, I barely remember even having CF. When I walked into the Pediatric Ward that Monday, I had no idea I was about to hit a breaking point. That Monday was a catalyst in my healthcare journey because for the first time in my life, I began to understand what cystic fibrosis really was. In some ways, I actually consider it to be my "first" admission because the illness I knew before was nothing compared to what I've known since.

I spent 23 days in the hospital that April. During which, I was diagnosed with CF related diabetes and a mycobacterium abscesses lung infection. I was started on insulin therapy and learned to give myself a shot with every meal. I also had a central line permanently placed in my chest, which I used for IV antibiotics, 3-4 times a day, every day, for the next 3.5 years. In a matter of days, everything I knew about my life changed.

I still believe the best is yet to come.... 2016 Great Strides Ambassador

And after all that we've been through

And after all we left in pieces 

I still believe our lives have just begun 

Cause now the past can be outrun

And I know you are the reason 

I still believe the best is yet to come

Has anyone ever told you "the best is yet to come"? Did you believe them? What was going on in your life when they said it?  I've always liked that phrase. I'm a fan of being optimistic and those words embodies positivity in every way. It says "even though things are great right now, they're still going to get better." The lyrics above are from a song by the band RED.

If I had to pick one song to describe the year 2015, it would be that one.

2015 was awesome. It seriously was. You can read about the first half of the year HERE. But to sum it up... I had the most amazing last semester including an internship, a executive board position in my sorority and a director position with ODU's Student Government Association.  I graduated with college with honors. I got a paid internship within a week in my field of study. I got the most adorable kitten in the world. And last, but certainly not least, I was able to start taking Orkambi, the first ever FDA approved medication to treat the underlying cause of cystic fibrosis for nearly 20,000 children and adults in America.

I wrote a blog about Orkambi when I first started treatment (read that one HERE), but per usual, I haven't written much since. Tomorrow will be six months since my first dose. People have asked how it's going and I have to be admit, I hesitant how I should answer. Don't get me wrong, I have definitely had positive results from this medication.... but I'm not going to lie and say that it's been some miracle cure-all.