Five years and a world of difference

“I had an increasing sense of dread about you, and now… it’s not there anymore.”

My doctor recently said this to me and it’s been bouncing around in my mind ever since. It’s something we talk about often — how bad things used to be. Even today, at clinic, it was brought up in conversation more than once.

“It’s so good to see you doing this well.”

“I remember when almost every clinic appointment ended with you being admitted.”

I remember those days, too. But sometimes I forget the gory details: how often I was in the hospital; the sharp downward trajectory of my lung function; the way that some days my body was so weak, simply walking from my couch to my kitchen sink felt like running a marathon; the countless hours of sleep lost to thoughts of whether I’d get to see my daughter grow up, or if I’d even be around for my next birthday.

Perhaps it’s not that I forget these things as much as I intentionally push them into the back of my mind. And when I do pull those memories out and dust them off, it’s almost as if I’m watching a movie or I’ve somehow tapped into someone else’s memories. It doesn’t seem like my life. It doesn’t feel real.

But that was my reality. Back to back hospitalizations had become the norm. I was so unstable that I didn’t even have a baseline. I lived from day to day, constantly monitoring my medical symptoms, carefully analyzing and planning every single thing I did, and setting strict limitations because the consequences of pushing myself too hard could be devastating. As careful as I tried to be, I felt so overwhelmed and constantly betrayed by my own body.

And always, always… I was afraid.

Five years ago (to this very day, actually) I was enrolled in the clinical trial for Orkambi. (For those of you who don’t know, Orkambi is the second of three drugs that treat the underlying cause of cystic fibrosis. All drugs before these simply treated the symptoms, like a band-aid, but these are designed to actually correct the malfunctioning protein that causes CF. In other words, there’s some crazy awesome and complex science at work here!)

It was a double-blind study, so neither my care team nor I knew if I was on drug or placebo. But from the very first dose, I had immediate and consistent side effects, so we were fairly certain that I was receiving the drug. We now know that many people experience what we in the CF community like to call the “Orkambi purge.” This consists of a variety of symptoms, including (but certainly not limited to): increased cough, fatigue, extreme shortness of breath, flu-like symptoms, and coughing up literally cups of old (sometimes hardened) mucus. My “purge” lasted several months but, compared to many others I’ve spoken with about this, my experience was rather mild. I wanted so badly to continue the trial, so I pushed through the side effects, keeping my fingers crossed that it’d be worth it.

Because of my involvement in the study, I was able to begin taking open label Orkambi before it was approved by the FDA and became available on the market. About a year into my Orkambi journey, I noticed something incredible. I hadn’t experienced a significant increase in lung function or weight, but I would get a cold and the strangest thing would happen… I’d get over it!

For the previous few years of my life, anytime I caught a virus of any kind, I would immediately start planning my hospital stay. Because that sickness would inevitably turn into a raging lung infection and, without fail, I would end up needing two weeks of heavy IV antibiotics to recover. But after being on Orkambi for a while, I went from needing 3-6 hospital stays a year to only going once or maybe twice in a years time. That in itself is completely life changing! That stability was more than I could have ever hoped for.

And now, five years later, I’ve survived a divorce, gone back to school, traveled a bit, celebrated my 31st birthday (which, at the age of 25, I thought I’d never see) and made about six million more memories with my child. I can do things now that I never would have been able to do before, like walk seven miles throughout downtown Boston or practice yoga almost daily. I can now make plans for the future with a fair amount of certainty that I’ll actually be there to see those things happen.

As I’ve been writing this, I’ve had two significant realizations that I want to share:

1) Orkambi was not the only change I made in my life at this time. I quit my job in order to focus on my health. I also made some big changes in my personal life, removing some toxic influences. Improving my mental and emotional health certainly had a positive affect on my physical health as well! It’s very likely that I wouldn’t have benefited from Orkambi as much if I hadn’t also made these changes.

2) I know that Orkambi isn’t a miracle drug for everyone. In fact, I know people who didn’t see any benefit, couldn’t tolerate it, or even got worse while taking it. Despite what we like to tell ourselves about our ability to control this disease, sometimes the things that happen to/for us are nothing more than dumb luck. I am incredibly lucky. I know this.

While it may sound like things are just peachy now, I have to be honest. Orkambi did not miraculously cure me. My health is still a full-time job. There are many days that I still struggle and am decidedly not okay. CF affects every single aspect of my life. It limits me in ways I wish it didn’t. My body and I will always be at war with this invisible monster. I still worry about not being here to raise my daughter, and I think about death far more than the average person (something I’d really like to write about, actually). I am in no way blind to the realities and ugliness of this disease.

Yet, where there used to be an “increasing sense of dread,” there is now expectation. And where there was once constant, almost crippling fear, there is now hope.

So, so much hope.

One more quick thing. Five years ago today, I was introduced to
Doctor Who by one of my study coordinators. I had to be observed
for several hours after taking my first dose of study drug, so we passed the time by
watching a few episodes. Over the years we have become good friends.
Well, today, she stopped by with a gift for me — a Dr. Who fleece blanket.
She had no idea it was exactly five years ago that our friendship and
my love for The Doctor began. This little serendipitous event has
made me happier than I can even explain. Life is so funny.   

4 thoughts on “Five years and a world of difference

  1. Love your story.

    There is so much that is forgotten and taken for granted in life. Here’s hoping there will be a reminder each day to be thankful for the wonderful basics in life without which none else would matter. Alive !


Leave a Reply

Fill in your details below or click an icon to log in: Logo

You are commenting using your account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s