Happy New Year! I realize it’s March but it’s the first blog post of the new year and better late than never, right? Kind of my philosophy in life if I’m being honest. Anyway, I hope your 2020 is off to a great start!
The new year started off with more good news! Betsy has another follow up appointment with her pulmonary specialist on January 3. Her FEV1 was up again, this time to 43! That’s a 14 point gain and is nearly up to the same level as when Betsy started this blog. Nobody really new how much of an effect the Trikafta would have on her FEV1. There hadn’t been any patients in the test groups with starting FEV1 scores as low as Betsy’s. Needless to say she was thrilled. The doctor was even cautiously optimistic that she could still see more improvement, especially after she is able to resume consistent exercise.
Alena is a big fan of trikafta
Even more than the breathing test score itself, the change in Betsy’s day-to-day life is more than evident. Coughing for hours on end to start the day is a thing of the past. Betsy has been bound and determined to reclaim some of the household duties. She has implemented a new laundry system and, amazingly, we now retrieve clean clothes from our drawers rather than the laundry basket. Betsy and I are back to sharing the grocery shopping and the cooking! Last weekend, we had been toying with the idea of moving a desk up from the basement for Lucas to use. We’d get to it later, I thought. I had to run out briefly and in the 20 minutes that I was gone, she managed to carry up a desk from the basement and rearrange the furniture in the dining room. This is the kind of shenanigans Betsy would have attempted on a regular basis ten years ago. The “just do it” part of her personality had been suppressed for a long time out of necessity. No longer.
The desk. In its new home.
In other news, Alena continues to give us a run for our money! I can’t even believe I’m writing this but she will be one year old in just two weeks! She’s been trying to walk for about five months now. One of her favorite activities is grabbing the hands of whoever is closest and dragging them around the house! But just prior to her ten month birthday, she started taking solo steps and now she walks unassisted whenever the mood strikes. Alena is starting to talk as well. She says “mum” quite clearly and intentionally. Not sure where she got the British accent but there you go. She calls Lucas “baa” and if she wants to be in your arms she’ll say “up”. Nothing resembling “dadda” as yet. She calls the much beloved squirrels she sees out and about “pwa” and any human being, regardless of age, is “baby.” Alena’s sleep has been super irregular, but she’s awfully cute so we’ll call it even.
Our almost one year old!
Lucas is really enjoying second grade. He loves his teacher and has some of his best friends from school in his class. He’s a great big brother. He loves to make Alena laugh. He’s (almost) always willing to give her one of his toys or share the iPad. The other day I was trying to get dinner ready and had stuck her in the pack ‘n play. Alena found this arrangement to be suboptimal and was voicing her opinion. So Lucas climbed in with her and started to play with her. He’s also become a very big Star Wars fan over the last few months. He watched all eight movies in the lead up to the holidays just in time for us to see The Rise of Skywalker in the theater. Now he spends his free time making up his own Star Wars movies. I’m hoping one day he’ll sell the rights to his namesake George (Lucas) and use the proceeds to fund my retirement.
Well, only a short time until spring now! I’m looking forward to the warmer days and being outside. I’m also subconsciously preparing myself for all of the plans that Trikafta Betsy will undoubtedly have in mind for us to do. 😉 It’s going to be a great spring!
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. “It’s a new dawn,” 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 coughing—persistent, 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 haven’t 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 haven’t 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 wasn’t worried about having coughing fits in public. Alena got a cold and a few days in, I realized I hadn’t 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 don’t 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 I’ve been given. It’s 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 wouldn’t change my story. I see God’s 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 God’s 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
It’s amazing how a small thing like having a baby will limit your time for all non-essential activities, including blogging. It feels like just yesterday we were enjoying the last, long, warm days of summer. Hard to believe it will be November in two days. A lot has happened in the last couple of months.
At the park. In short sleeves. Must have been July.
Lucas has had a great start to second grade! He’s growing in different ways and learning a ton. We love his teacher this year. Lucas’s first grade teacher was great too but his teacher’s approach to homework this year is so much better for us. Last year we would get a packet on Friday which was due the following Thursday. We are just not the kind of people who are good about doing a little bit each night. We were constantly realizing it was Wednesday and we hadn’t started even the first page. It made for a lot of long, unpleasant nights. This year his teacher sends home one page of homework every night. It’s so much more manageable and Lucas has even realized he doesn’t hate math. Lucas’s three best friends from school all ended up in his class which has been great for him too. Earlier this week he programmed a video game in his technology class. A couple weeks ago his class went on a field trip to the botanical gardens. It’s been a great year so far!
“Dad, you don’t have to walk me to the bus stop anymore.”
Lucas has been playing a lot of soccer! He just finished the fall season with his school’s rec & ed team. He’s played with most of those boys since kindergarten and it’s been fun to be part of that community over the last three years. He also joined the travel club that I coach for this past August. It has been a really different experience for him but he’s learning a lot and has had fun wearing a real uniform and playing in a legit tournament (we took second place!). We finish up our fall season this weekend!
We play a lot of soccer.
Alena is seven months old which blows my mind. She has become so interactive recently. You forget how much and how quickly they change when your kids are almost eight years apart. I came home one day and she was sitting up all by herself. Now she’s trying to pull herself up on furniture. Crazy. Alena definitely seems to be an extrovert. She is energized when she is around people. She always has a smile for people, even strangers (Lucas would always hide his face…he still sort of does, haha).
At Trader Joe’s. On the lookout for new friends.
Alena is trying solid foods. So far the experiment has been met with mixed reactions. Alena loves her big brother. She is always excited when he gets home from school and she loves to sit on the floor and watch him play. Lucas loves to make her laugh!
Alena needs remarkably little sleep. I guess I am to blame for that. I tend to be more of a night owl and function pretty well on five or six hours. Alena sleeps more than that (don’t worry) but her naps have been inconsistent and recently she’s been more wakeful at night too. It feels like she just doesn’t want to miss out on anything. She’s growing and happy so I guess it’s not a huge problem (apart from the fact her parents are buying a lot more coffee than usual).
Who’s got time for sleep? (Photo taken at 4:21 AM)
One of the biggest developments of the past few months is that Betsy was approved to start taking a new drug aimed at the underlying cause of cystic fibrosis. There has been a lot of scientific breakthroughs over the last several years but until now, the new treatments didn’t work for people with Betsy’s genetic mutation. We knew that this new drug was in the pipeline but thought it would still be six to twelve months before it would be available to patients. Sometime in September, though, we got a call from the doctor saying she could apply to start the new drug early. She was approved in October! We had to frantically work out a few logistics, namely how to wean Alena. Initially, we were under the impression that Betsy would have to stop nursing the baby but thankfully after a few different consultations, we determined that reducing the feedings and supplementing with formula would be sufficient. Whew. Betsy has been on the new drug for less than two weeks but I don’t think using the words “life-changing” is an exaggeration. Her symptoms are greatly reduced. She doesn’t spend the first three hours of her day coughing. The other day, Betsy carried the baby and a basket of laundry up from the basement and didn’t feel overly winded. She is starting to exercise again. It’s remarkable. I am sure Betsy will write more about this but we are so thankful for this new development. It feels like we’ve gone back to a time when her disease seemed more like a footnote rather than the essay itself.
Living her best life.
For years we have been participating in the Cystic Fibrosis Foundation’s Great Strides campaign. The sole purpose is to raise funds for cystic fibrosis research. This new drug is a direct result of that research. We are so thankful for our good friends, Ross and Deb, who organize our Great Strides team every year. We are so thankful for everyone (many of you who are reading this) who has ever donated money to the Great Strides campaign. Every single dollar contributed has led to this medical breakthrough. We are so thankful for the scientists who are doing this important work. And it’s not done. The Cystic Fibrosis Foundation has recently unveiled a new research agenda to continue the development of treatments for every person with cystic fibrosis with an end goal of finding a cure for the disease. It actually feels like this could happen within Betsy’s lifetime.
Great Strides 2019
So that has been our fall! We are excited, tired, and thankful but above all, our hearts are full of hope.