Hi faithful readers! Thanks so very much for sticking with me! I know I’ve mostly been absent from this blog in recent months. I have an explanation for that, and here it is…
I’ve started several posts but I haven’t been able to see them through. This post is my second attempt at an update regarding my health now that I’m on the new breakthrough CF medication called Trikafta. I’m having a hard time clearly communicating what has been a complex physical and emotional experience for me.
So. Where to begin?
August 31, 2019 was the first day of this journey. My doctor sent a message telling me I might be eligible to get early access to the new CF medication that was due to be FDA approved on or before March 19, 2020. Early access was being granted to some patients with low lung function. My application had not been submitted yet and the doctor was unsure if I would qualify if I was still nursing Alena. He wanted to check to see if I was willing to wean her if need be in order to get this medication early. I didn’t want to wean Alena. I didn’t want to surrender yet another positive part of my life to the demands of my disease. However, since the beginning of my pregnancy, through Alena’s birth and into the months that followed, I had been living under immense strain and my health situation felt precarious. I knew I could not turn down the opportunity to get early access to a medication that could improve my health and provide me with a measure of safety heading into the winter months where the threat of illness is at its worst. I wrote back and said if I was granted access, I would wean Alena if it was necessary.
October 4, 2019 was clinic day. My lung function was 29%. A bit disappointing! For some reason it had fallen five points after I gave birth to Alena. That didn’t make any sense to me, but regardless, it had settled there and wasn’t budging. I was just about to leave clinic when they received word that the clinic was approved for the early access program. They had me sign a consent to begin the program and said my individual participation should be decided within a week. The consent didn’t prohibit nursing but being that there are always unknowns with new medications, they suggested I wean Alena and I agreed to begin that process.
Early the next week I got a call saying that my case had been approved! We agreed that I would come in on Friday, October 18 to get the medication.
The weaning was not going well, at least not for me. I spent much of that week in tears. Alena was doing quite well with the bottles but I hadn’t taken away her favorite nursing times yet and my heart was broken. On Friday morning I woke up and knew I had to press harder about the issue. It just didn’t feel right. I emailed my doctor and asked if it would be possible to keep even a few nursing sessions. The doctor overseeing the early access program called me and we discussed the issue at length. In the end they agreed it should be safe to nurse part time. A huge weight was lifted and for the first time I started to feel real excitement about beginning the medication.
On October 18, 2019 I headed back to the clinic to do another breathing test and have an exam. My lung function was 29% again. Many people stuck their heads in the door to wish me the best as I started this journey and the excitement was palpable. āItās a new dawn,ā my doctor had said. I left there with medication in my hand and hope in my heart!
October 19, 2019. I took my very dose of Trikafta. It’s not an exaggeration to say that I felt different within 24 hours. My lungs felt less irritated and my coughing on Sunday morning seemed far less explosive. In fact I was able to nurse the baby back to sleep before my usual morning mayhem. Sunday morning and Monday I morning I coughed up my normal amount of mucus but it was looser and cleared more easily. Tuesday morning, just 72 hours after I started the medication, I coughed up only the tiniest bit of mucus, and on Wednesday, NONE. I’m not sure I can adequately explain all the ways that this one thing, alone, is life changing. Very few people other than my immediate family have witnessed what my mornings had become over the years, but I will tell you, it wasn’t pretty. In order to clear the congestion that pooled in my lungs overnight, I would spend several hours coughing. And not polite coughingāpersistent, gut-wrenching, vomit-inducing, violent coughing that would leave me bent over, sweating, and gasping for air. The amount of energy I spent doing this each morning was significant. Having that congestion meant constant infection, inflammation, and illness.
Over the next days and weeks I began to notice other changes. It felt good to breathe. I was getting the oxygen I needed and my lungs were calm. I still got out of breath doing “normal” things, but I would recover so much more quickly after exerting myself. I began to be able to do more–carry the baby up and down the stairs more easily. I could carry heavy baskets of laundry. I could push the stroller up a hill. I began to notice subtle changes in the way I was thinking. More often than not, it was, I can do this! rather than, how on earth will I do this? I stopped dreading physical activities. If I was up in the night with Alena, I didn’t worry about how I’d survive the next day. I started to feel strong enough and brave enough to do things I havenāt dared to use my limited energy on for the past few years, like running errands. When I was out and about on one such day, I suddenly realized I was speed walking. I had fallen back into an old habit that used to drive Jaime crazy! He would ask me to slow down all the time, until one day I asked him to slow down. That was a moment he said he would never forget.
One night, for kicks, I decided to try and carry Lucas up to bed on my back. I wanted to be reminded of the old days when I did that all the time. He looked a little surprised when I asked him if he wanted to but he climbed on and I carried him from the basement to his bedroom. It wasn’t too hard, even though I’m pretty sure he was a bit less than his current 55 lbs last time I did it. It felt good, oh so good to handle normal life again.
Since normal life was becoming more manageable, it seemed like a good idea to start exercising again. When I could, I took Alena out for walks, but it was hard to be consistent. It suddenly dawned me that I could probably work out in the morning again! I havenāt been able to for 10-15 years due to that incessant morning coughing. I decided to try it out the next day and was able to walk two miles in 30 minutes before Alena was up for the day. What a feeling!
Anxieties began to lift. I wasnāt worried about having coughing fits in public. Alena got a cold and a few days in, I realized I hadnāt been at all concerned about catching it myself. I stopped holding my breath if people got too close to me in public.
Hope for the future started tugging at my heart. I could more clearly imagine being not only present for my kids in the years to come, but also a participant in their activities and adventures. I found myself wondering if maybe, just maybe I might be able to hold my grandchild someday, or celebrate our 40th (or 50th!) anniversary. Is it possible? I donāt know. But maybe.
Amidst my joy and hope came some unexpected grief and sadness. As I began to feel better, I started to remember, really remember what my life used to be like. I remembered the feelings of strength and ability, and of freedom and vitality. I began to long for complete healing and I became afraid of getting a sense, for the first time ever, of what irreversible damage had been done in my body. As the grip of the disease loosened, I felt desperate to cast it off completely. I wanted to be free. I knew there was a limit to the improvement I would experience. This medication is not a cure and there is still dysfunction in my body. And nothing can erase the suffering this disease has caused and the physical and emotional toll it has taken. Not all losses can be regained, not physically or otherwise. I cannot go back to 100% lung function and I cannot go back to participate in all I have missed. I simultaneously felt intense gratitude for the blessing of significantly improved health and intense sadness for all that had been lost.
It also made me realize just how difficult things had become of late, and I felt overwhelmed by that. Since the beginning of my pregnancy with Alena until I began the medication, I had been struggling. My life had gotten very intense. Every day I was being pushed to the absolute limit of my health and strength. I had no reserve. I had only enough strength to (try to) handle the day to day demands on my body and no additional strength to process what was happening to me–how hard life was becoming and how this disease was seeking to ravage my body. It came into focus as I began to emerge from that place and I found I needed to face it and grieve it even while celebrating the improvements.
On Friday November 15, 2019, I headed back to the clinic to do another breathing test and have an exam to check my progress. I felt excited, anxious, worried, confident, and everything in between. I knew without a doubt I was doing so much better! But I didn’t know how my breathing test would look, and if it would show the gains that I felt. I wondered what the doctor would think and if he’d feel I was responding well.
First up was my breathing test. My FEV1 went from 29% to 39%! A 10 point gain, from 29 to 39, means I gained 34.5% function. In one month! That is significant! I was so excited. The rest of the exam went well and my doctor was pleased at the gains both in lung function and in strength and ability that were seen in such a short time period. The stories of my exercise and small, but meaningful physical feats were rewarded with a broad smile. We know from the study data that the greatest gains in lung function happen in the first weeks of beginning the drug, but he was hopeful that I might see another small bump as I gain more strength. And beyond that, there is hope for years and years of stability.
Obviously 39% lung function is nowhere near normal. I won’t be running any marathons or scaling any mountains, but with that much lung function and the absence of the most oppressive symptoms of the disease, I feel confident that I will thrive within the life Iāve been given. Itās truly a new dawn.
In this new reality, I will still be marked by the struggles of these last years. At times they will still cause me pain. But I wouldnāt change my story. I see Godās hand on every page. I have learned lessons in the darkness that could not have been learned in the light. I have seen God do miracles on my behalf. He has given me the strength and courage to do impossible things. He has protected and preserved me, sustained and upheld me. It has been his strength, his power, his provision that has brought me to this day. I am grateful to have labored hard for some of the greatest blessings in my life. These labors have been life giving and soul satisfying. And God has taken my pain and struggles and turned them into blessings for me over and over again.
As I become accustomed to an easier life, I pray I will never forget or forsake the lessons I learned in shadow. May I never mistake Godās strength for my own. He has given me strength for today and BRIGHT HOPE for tomorrow!
He reached down from on high and took hold of me; he drew me out of deep waters. He rescued me from my powerful enemy, from my foes, who were too strong for me. They confronted me in the day of my disaster, but the Lord was my support. He brought me out into a spacious place; he rescued me because he delighted in me. Psalm 18:16-19
