Today marks one year since I began taking the drug Trikafta, the new breakthrough cystic fibrosis therapy that targets and corrects the basic defect of the disease. What a year it has been!
I remember so clearly heading into clinic last October for a check up and to pick up my first month’s supply of the medication. The drug had not been FDA approved yet, but I was granted early access due to the severity of my disease. I had read the study results which were very hopeful, but had been warned by my doctor that these results were obtained by patients in better health than I was, so we were unsure if we could expect the same level of improvement for me. Still, there was hope in the air.
Now as I reflect on my experience a year later, I find it very hard to put it into words. I’m sure those of you who have asked me about it and listened to my incoherent ramblings would concur! My life has drastically changed. I still have cystic fibrosis. I still deal with certain symptoms of the disease and I have sustained lung damage over the years that will always limit me to some extent. Yet life has been breathed back into a body that was strained and declining. CF is a progressive disease, which means it gets worse over time. To experience not only a halt in the decline but a marked improvement and a reversal of symptoms is a truly remarkable and unexpected experience. I am incredibly grateful!
The very best part of my new life on Trikafta is the absence of a chronic cough and congestion. This has been the most life-changing aspect of being on Trikafta. Starting and ending each day without those intensely oppressive symptoms is an enormous gift. It means I can enjoy my mornings. It means I have significantly more energy. It means my body can maintain a healthy weight as I’m not burning hundreds of calories with intense coughing and constant illness. It means less systemic inflammation and no headaches or vomiting or back pain. It means freedom!
The second greatest gift is the absence of constant stress and worry. As time passed and I got sicker, as my lung function dropped, simple colds or viruses became major threats. I never knew how sick I might get or what the long-term damage would be when a “simple” illness came knocking at my door. And even on the healthiest of days, I never knew when I might have a coughing fit in public which (take my word for it), is a very unpleasant experience. One of the best things is simply being able to wake up each day and live my life without constant thoughts of my health and my disease. It’s a joy to be able to contemplate the future without fear or dread.
My life looks much more “normal” than it did a year ago. I can handle so many more tasks again. I get up and breeze through breathing treatments that take a fraction of the time they used to take. I can make breakfast, take Alena on a walk and to the park, come home and help with lunch, clean and do laundry or prep for my students while Alena naps and Lucas attends school online. I can take Lucas and Alena out in the afternoon and still have energy to make dinner, tutor, and get Alena to bed. I am exhausted every night. But it’s a tired that comes from a full day rather than from infection and illness. Feeling tired is so much better than feeling sick and tired!
There are other positives worth mentioning. I sleep better. When I wake up in the night, it’s because a warm, disheveled, toddler needs her mama, not because I can’t breathe or am uncomfortable. I don’t have nightmares about suffocating anymore. My posture has improved. We’re not spending hundreds of dollars on tissues! Everyone in the household is under less stress and strain. Some of Lucas’s stress-induced habits such as biting on toys or pencils have simply disappeared.
I still get out of breath with exertion. At last check, my lung function was 43%. A near 60% reduction in lung function, even without acute symptoms, will not go unnoticed. Pushing the stroller up a hill makes me winded. Cleaning, bending over repeatedly to pick up toys, running around like a crazy woman trying to get packed up and out the door for whatever activity I have planned, those things make me out of breath as well. But I recover quickly and move on without a problem.
My digestive system must have improved to some degree because my vitamin levels are far higher than they used to be. But I can’t perceive the improvement and still have daily symptoms to contend with. I also struggle with asthma. The same triggers are there–cold, damp weather, high humidity, times when my seasonal allergies flare up. I still culture the same two strains of bacteria that have been present in my lungs for some time. I still require more sleep than the average person (do you hear me, Alena??).
I wanted to talk about the timing of this medication which requires a diversion into the story of Alena. The years prior to Alena’s birth were challenging. I experienced health crisis after health crisis and watched my lung function drop from the upper 40s to the mid 30s. I fought harder than I’d ever fought to regain health and to find stability and I just couldn’t win. But in his time, God provided just enough healing and stability for me to become pregnant. He provided enough strength for me to carry Alena and usher her safely into the world. It was rough. It is the hardest thing I have ever done, to grow and nurture a baby within me when the challenges of my disease were so great. But God provided. He provided the strength to get through the intense first months with a newborn. At times it felt like I was just hanging on by a thread, but God kept me safe. He didn’t make it easy, but he sustained me. And now, as life has ramped up again with the 24/7 demands of caring for a busy toddler, I have Trikafta. If I try to imagine what life would be like right now if I was still as sick as I had gotten…it’s not a pretty picture. It seemed strange that we would have our second child 7.5 years after our first, when I was nearly 41 years old. But it was God’s perfect timing. Alena came when the help I needed was on its way.
In some ways it would have been nice to have had this medication earlier, to have sustained less lung damage, and to have experienced less pain and fear. But the struggles and difficulties have been used by God for good in my life. I have learned about trusting God in all circumstances. I’ve experienced the strength and beauty of community as friends and family have surrounded us with love and support. I’ve experienced the joy of unconditional love as Jaime never once wavered in his commitment to love me and sacrifice for me and our family when things got intense and his burdens were great. I’ve gained an empathy for those who suffer as a result of my own suffering. I’ve had the privilege to better understand and know my sister Sheri as I’ve experienced some of the struggles that marked her life. It has given me more respect and more gratitude for the beauty of her life and the strength she exhibited. It makes me feel connected to her even in her absence.
So many people have mentioned Sheri to me when they hear about the life-saving aspects of this medication, wondering if it’s hard that it wasn’t here in time for her and if I wish she were still alive and could benefit as well. Because I miss her so much, there will always be a part of me that wishes she was still with us. However, when I reflect on Sheri’s life, her story, her legacy, I conclude that I would not change it. The length of her life was ordained by God and his perfect plan for her was carried out in a beautiful, meaningful life. She had many challenges but her strength, her resilience, her faithfulness, and the beauty of her spirit resulted in a life of victory, not of tragedy.
Why it was God’s plan for me to benefit from this medication and not Sheri is a mystery that belongs to God and not to me. I can greatly rejoice in God’s goodness to me and not feel hurt or anger or guilt that it wasn’t for Sheri. I know God gave her just what she needed and he has done the same for me.
It’s time to wrap up this post, isn’t it! I want to end by saying thank you to all you wonderful people who have stuck by me in sickness. I’m so grateful the pendulum has swung back to a place of health. I am thankful that God gave me strength for the dark days of illness and struggle, and he gives me strength for today when life is much easier. My heart is filled with bright hope and so much gratitude!
Here are a few pictures of my loves. I hope they bring a smile to your face as they do to mine every day. Grace and peace to you all, my dear friends and family!