By Olivia Davis
“Can’t you give me some indication of when it might arrive?” I pleaded with my doctor to tell me something - anything - about my application for early access to the new drug. “I’m sorry but that’s all I know right now. They said they would review your case during their next meeting, which could be next week, or if they don’t get to it then, next month.” I wanted to shake him and scream. Not one for confrontation, I settled for sarcasm. “Sure, take your time. It’s not like this is about anything important, like my survival.” He sat there looking uncomfortable. In general, doctors aren’t good with humor.
The first time I heard about the triple combination therapy was back in 2015, when Vertex Pharmaceuticals hinted that they were up to something really exciting. When the clinical trial started in 2018, I called every center that was recruiting patients to participate. No one would take me. I had the wrong genetic mutation for the trial, my lung function was too low, and they had patients of their own who they had already promised could sign up. I was crushed. I needed the drug more than ever. My lung function was at an all-time low - 41% - and I was on track to be listed for a double lung transplant if nothing changed. This drug was rumored to be a home run. The Barry Bonds of cystic fibrosis treatment. It was too early to release to the public, but I couldn’t wait any more. So in February of 2019, I started my petition for compassionate use of the life-saving drug. It was the last thing my doctors and I could think of that might help. I was already on a cocktail of antibiotics strong enough to kill a mammoth, and I was still being hospitalized every other month or so for my persistent lung infections.
Months went by of agonizing impatience. My paperwork funneled through hundreds of bureacracies, shuffling through the hands of one cog in the machine after another, and no one seemed to realize that the piece of paper in their “To-Do” pile held the fate of a human life.
To them, it was just another day in the office, but to me it was yet another week of untreated infection, eating away at my lung tissue. It was another night of sleeplessness and shortness of breath, another coughing fit, another Rorschach test of blood spat into the sink.
I woke up on a warm morning in September feeling chilled. I had gone out the night before against my better judgment, and I was paying the price for it now. Regretfully, I got out of the warm cocoon of my bed and walked to my medicine cabinet for the thermometer. 101.1 degrees Fahrenheit. This most likely meant being admitted for yet another hospitalization. I called to my fiancé in the other room. “Ali, what are you doing today?” She walked to where I was, took the thermometer from my hand and said: “Taking you to the hospital, I presume?” I looked down at the floor miserably. “I’m sorry,” I said. “I’m ruining your life. You should be out doing something fun today, not taking care of me again.” Tears started collecting in the corners of my eyes. “Olivia, there is nothing more important to me than taking care of you.” She said it mechanically like she has a million times before. I often feel like a burden in her life. The constant sickness, the canceling of plans, the hospital trips… how could she not be tired of it all?
Dutifully, she packed up a suitcase that wasn’t even fully unpacked from the last visit to CPMC, or our second home in San Francisco, as we sometimes called it. “Oh my girl, we are gonna get you the good drugs soon and you’ll start to feel better,” Ali said affectionately, but I only partly believed her. The drugs weren’t working as well as they used to, and lately, it seemed like I was in a hellish cycle of hospitalizations and fevers at home, without much “feeling better” in between.
We went in through the emergency room, and they were reliably slow to get me a room upstairs. Eventually, they wheelchaired me up to a room on the 7th floor and I collapsed onto the bed while people whizzed around the room, nurses taking my vital signs. Days went by like this - people buzzing around me, as I lay unmoving in my sweaty fever dream sheets.
On the 13th day of my hospitalization, my doctor came in dressed with the mandatory contact precautions - a plastic gown, mask, and gloves - with a smile on his face. It looked almost strained, like he had forgotten how to move his facial muscles in that way, stretched upward where they were usually taut and stern. “I have some good news for you.” “Really?” I sat up and looked expectantly at his clownish grin.
“Your paperwork was approved. You’re going to get the triple combination therapy.” I screamed involuntarily. “Oh my god! Really?? When?” “It’s getting shipped over to the hospital as we speak. You should have it in the next day or two.” I shot my fists up in the air in victory.
On the 14th day of my hospitalization, the resident doctor came in, holding the blue and yellow pill cartridges that held the drug I had been praying for the last year of my life. The plastic bag was marked with a sticker that said “Investigational Drug” in red capital letters. The nurse scanned them into the computer and put my first dose into a small plastic cup. I Facetimed Ali - she couldn’t miss this moment! I asked the nurse to take a video of me taking the pills so I could send it to my parents. “Here we go!” I giggled as I placed the pills under my tongue and swigged them back with water. Ali cheered for me through the phone and my nurse gave me a big hug. Now that I had the drug in my system, I was nervous. What if I had just worked so hard to get a drug that wasn’t going to work for me? What if it was too late to reverse the damage the infections had caused my lungs? What if I was let down yet again, by a new treatment? I didn’t have to wait long to have those fears quelled. Within hours of swallowing those yellow tablets, I was coughing out more mucus than ever. I was simultaneously disgusted by what was in my body and thrilled that I was getting it out.
I left the hospital the next day. As weeks went by, I was surprised by my ability to do things I wasn’t able to do before. I woke up two weeks after I got home, rolled out of bed to let the dog out, and walked to the coffee maker. I was pouring the grounds in the filter, whistling. I was whistling. I stopped and took stock of what was happening.
Just a month ago, waking up in the morning was a tortuous process of coughing fits, bracing myself against the kitchen counter with every shudder of my body’s forceful seal bark of a cough. But this morning was different. It was easy. I didn’t stop to cough or have to lay down on the couch immediately after I got up from bed.
I strolled into the kitchen to make coffee, without even thinking about it. Other things changed too. I noticed myself jogging to catch up to a friend on the street, and hiking with my dog Rizzo at a faster pace. I started singing in the car - no, belting - at the top of my lungs. I did it all without a thought, and that was the special part. All of a sudden, the negotiations with my body and the constant awareness of my jagged breath were gone. I was in the present moment, because I didn’t have the background dialogue in my head, monitoring my breath, my energy, what I was able to do and what I wasn’t. It felt like my body had been transported back in time to when I was healthy enough to “pass” as normal. My body adapted to my new state of health so quickly that it was almost like that whole miserable year of my life was a terrible dream. Was I ever really that sick?
These days I find myself worrying that I’m taking all of this for granted. I worry that I don’t appreciate the miracle of what I’m able to do, and what my body has conquered with the help of Trikafta (triple combo). I worry that the drug’s effect will wear off eventually, so I should do everything I want to do in life now, before it’s too late. I can’t just shake off the mentality I’ve mastered through my 25 years of living with cystic fibrosis. All my life I believed that I wouldn’t be here for long, so I learned to savor experiences; I treated every moment like it was precious, every good day sacred. I felt like a squirrel, stuffing as much food as possible into her bloated cheeks, in case it was in short supply later. Life felt like that. Like I was stuffing all of the possible goodness I could find into my pockets because I was scared that that was all I would ever get.
I remember as a child, I would look at my family and imagine the moments we were sharing as photographs I could look back on later. In particularly happy moments, I felt nostalgic. Like I was mourning that this moment would be gone soon, and nothing could ever be quite as good. I wanted to save all of these moments like acorns in my cheeks, but I knew I could never hold them all. I feel now that I can let them go, empty them out and let them spill out of me. I don’t have to hold them all, keep stock of all the good moments just in case, because I know that I have a lifetime of golden acorns left to find.
About the Author: Olivia is a singer, songwriter, aspiring author, and public speaker. She was diagnosed with cystic fibrosis at 17 months old. She lives in Berkeley, California with her fiancé and new puppy. She stays active in the CF community by speaking at CFF events, volunteering with the CF Peer Mentor program, and sharing her story on social media. You can find her on Instagram at @ohdavis.