Humbled Breaths

For breath is life, so if you breathe well you will live long on earth ~ Sanskrit Proverb

Tomorrow is Thanksgiving. A day we typically reflect on all we are grateful for. I give thanks every day for every breath, each step and for every moment, big or small, I am given. Right now though, it is difficult for me to adequately express the level of gratitude I feel presently in my heart. As I sit here, taking in the deepest breath, filling my little lungs with all the beautiful air I can, I then hold it steady. A smile spreads widely on my lips. I then slowly exhale…emptying my lungs slowly. I didn’t cough. I didn’t struggle. I didn’t feel the itchy, wheezy gasps that usually consume my lungs and throat. I am left speechless in this moment.

How is this happening? There is a new strength in my airways. In my workouts. In my voice. In my laugh. All I’ve ever known as “normal” is to cough, to constantly work hard to clear my lungs, to struggle to breathe, to feel wheezy at times accompanied by all this uninvited mucus just stealing my air. With my goal being to get it all out as much as I can. All the time. 24/7. Mornings have always been my most difficult. All that thick junk building up in my sleep and come morning, a fight waiting to get out all that I could. Even with all of my breathing treatments/ chest physiotherapy. Relentlessly time consuming and exhausting. Not anymore. I am so humbled as I write and share this with you.

I have cystic fibrosis (CF). That would have been perhaps helpful to have mentioned at the start. If you google CF, one definition you’ll find states “Cystic fibrosis is an inherited life- threatening disease that damages the lungs and digestive system. CF affects the cells that produce mucus, sweat and digestive juices. (yummy) It causes these fluids to become thick and sticky. ” This leads to severe respiratory and digestive issues, infections and sometimes, diabetes. Good times.

Now that you have read that lovely piece, I want to introduce to you Trikafta. Again, thank you google because sometimes it’s just easier. “Trikafta is a combination of three drugs that target the defective CFTR protein. It helps the protein made by the CFTR gene mutation function more effectively.” Trikafta is “for the treatment of CF in patients 12 years and older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene”. If you wish, research this even further for the medically technical scientific fun factor. Trikafta is, for the first time in my life, a triple combination genetic modulator that my specific mutations qualified for. I have been so anxiously (to put it lightly) awaiting for this drug! Not just for myself. For all of my friends living with CF who are eligible to be on it. The FDA approved it 6 months earlier than anticipated. It is an exciting and hopeful time in the CF community!

I am asking myself every day “How is this real life…is this a new life?” Nov 16, 2019. I took my first dose of Trikafta. In 12 days my life has drastically changed. Days 1-2 I purged. Immediately upon dosage. Dark green, to gray, to old blood, to thick mucus…all coming up. Disgusting junk that I didn’t know existed in my lungs. I had a runny nose, drainage in my sinuses and throat. Sore throat briefly. Some nausea. Soon what I coughed up went from dark green, to light green, to faded yellow, to white foamy, to clear. With hardly any effort, it came up in big amounts. Again, this occurred for me during the first two days. Suddenly, I could breath easier. I could feel air moving in my lungs in a way I have never felt. And I feel this now. Just..how?

As each day goes by my cough has become less and less. Nonexistent at times. Where is the mucus I’ve known forever? How can this be? My morning routine changed. I no longer wake up congested. Even during my therapies very little, if anything, is productive. I have tons of energy. My workouts feel at a new level. Easier. Stronger. I will write about those another time and try to hold back the tears. I want to sing, whistle, hum all the time! There is more pep in my step. I am breathing easier. I am breathing easier. I hardly have a cough. Those five words! I can breathe deep and not feel restricted as I once did. Someone just pinch me. It is unreal. Yet, it IS real.

Emotions have taken over several times as this is a lot to process. When I was approved for Trikafta after rounds of prior authorization denials from insurance, I cried. My heart leaped for joy followed by a sick feeling in the pit of my stomach. Guilt swept over me. Why guilt? I couldn’t help but think of my dear friends who have gone before me and will never experience this. Survivors guilt. Guilt for those who do not have access to this medication yet and need it now. It’s hard to NOT feel this way sometimes. I want this for all of my CF friends, and for those still waiting for a genetic modulator…we all deserve this. Each and every one of us.

Please understand. It’s not a cure. It will not reverse the years of damage to my lungs. It’s another form of treatment. I resume all of my current medical regime and exercise in addition to taking Trikafta. It has honestly increased my motivation to exercise. I have worked hard for 43 years to still have my original lungs. Now that I am fortunate beyond words to have this medication, I want to work ever harder to maintain and keep my lungs as strong as possible. I can still get sick easily, get infections, be on antibiotics as needed…CF doesn’t go away. But I feel now, for the first time, less fear for my future. I feel now a new beginning to the course of my life that I didn’t think was possible. I do not know how to comprehend this and what the outcome will be for me. But with each breath, my heart is overflowing with joy and gratitude. I give thanks above. My breaths are humbled with the hope of more days ahead, more time with my loved ones and more time to live and love with my entire being. Thank you, Trikafta. I am excited for this journey with you.