Saying goodbye to my safe haven.

Last night, I had the incredible honor of dropping off more than 75 toys that The Junior League of Norfolk-Virginia Beach (JLNVB)  had collected for the Pediatric Ward of Naval Medical Center Portsmouth (NMCP). Ten volunteers from the league helped wrapped all of them plus another 75 or so, for the nurses to surprise the children who will unfortunately have to wake up in the hospital on Christmas morning.

After the excitement settled, my friends all headed home but I had to pick up a medicine refill so I went to the pharmacy. Sitting alone, I was looking at the pictures we took and the gravity of the situation hit me like a brick wall. I couldn't help but start crying right there in the middle of the waiting room.

I have been a patient of the CF Clinic at NMCP since I was six years old and was admitted to the that very pediatric ward more than 20 times in the last decade before transitioning to the adult ward. While I never had to spend Christmas in the hospital, some of the absolute worst days of my life have happened in that building.

10 Things Tuesday: Traveling with Cystic Fibrosis

Hello from Roma, Italia!

Last month, I boarded a plane to spend eight days in Rome. This isn't my first time traveling with cystic fibrosis and certainly won't be my last. And while I'm definitely not an expert, I've had my fair share of traveling oops-es over the years. As a result, I have learned a few tips to making your vacation both safe and enjoyable when dealing with a chronic illness.

For someone who has physical limitations or takes 20+ medications a day, the idea of travel can be terrifying. Being away from your routine, the extra walking, how on God's green earth you are going to pack and carry all of your medicine and supplies... It's no small feat, but that doesn't mean you can't still do it! You just need a little extra effort on the front end.

1. Forget crossing your Ts and dotting your Is... The most important letter is P! Plan, prepare and pack ahead of time. Like... way ahead of time. 

The most important aspects of traveling with a chronic illness are to plan ahead and be prepared. Honestly, most of the next nine tips are some variation of this. I'll get into more details as they pertain to certain issues such as carry-ons, accommodations, itineraries and travel buddies, but the very first step is to pack your bag.. and pack it early! I am literally probably the worst packer in the history of the mankind. If you have any doubt, ask my sister Jenny who during one trip, managed to repack my one full size and two carry-on size suitcases into just one full and one carry-on because she's a wizard like that. She will probably say otherwise... but I have gotten slightly better over the years. One thing I did in getting ready for my trip to Rome that significantly reduced my pre-trip anxiety was that I packed every single one of my medications and supplies five days before I boarded the plane, the earliest I ever have! Doing this so early ensured that I could take my time and make sure that I had everything I needed and it fit into my bags. It also gave me several days to mull it over and remember the extra items that I didn't think about the first run through. Far too many times have I packed the night before or even morning of a trip, and 12 hours later when I'm already on my way, remembered something else I needed. This time, I can say with complete confidence that I had every single thing I needed. Looking ahead at my next adventures, I'm going to make it a habit to at least pack these medical essentials well in advance, even if I still have some room for improvement on the whole packing-way-too-many-clothes-at-the-last-minute thing.

Dear Mom, thanks for... well, everything.

Mom,

Not long ago, I sat on my bedroom floor, hysterically crying. Everything was piling up and I honestly had no idea how I would get through the next few days... I was signed up to participate in an online virtual conference for adults with cystic fibrosis, called BreatheCon. I was suppose to go on a practice hike to train for my upcoming Xtreme Hike. And finally, I had my first follow up appointment with the transplant team at Duke University. Throw in a long work week, boy drama and the stress of being the lead planner on a volunteer event happening while I was out of town, I was done. I was past my breaking point. I felt overwhelmed and defeated. I didn't want to do any of it. I didn't want to attend BreatheCon, I didn't want to hike, I didn't want to go to Duke. I only wanted to crawl into bed and cry.

 
Yet, somehow, less than an hour later, I was logged into BreatheCon listening to one of the most inspiring panel discussions I've ever heard. 15 hours after that, I hiked 8 miles and was standing on top of a mountain. Another 48 hours later, I was walking out of my transplant appointment, feeling genuinely optimistic and motivated to keep pushing through the adversity that is living with cystic fibrosis.

It didn't just happen "somehow" though. It all happened because of you. Everything that's happened in the last 25 years, everything I've been able to do, is because of you.

I often get praised for being strong, resilient and accomplished despite my cystic fibrosis, but I didn't become any of those things by chance. I had the most incredible role model to show me how. Last weekend was a challenge and I got through it, but not by myself. I got through it, because of you.

So thank you, Mom. Thank you for everything. 

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.