Happy 2nd birthday to our baby girl, Alena! Although I’ll probably always call her my baby, she is certainly not that tiny baby who slept so sweetly in my arms anymore. She is full of personality, toddler-antics, and an alarming amount of energy and enthusiasm. I’m pretty sure I’m going to be tired for the rest of my life!
I’m writing this blog while having yet another cup of coffee because she decided to ring in her birthday by being up for half the night. Apparently it’s never too early to get the party started!
What shall I tell you about our girl at age two? Alena is usually very friendly and outgoing. If we go on a bike ride or a walk, she’ll say hi to anyone who passes by. Her middle name, Abigail, means source of joy, and sure enough, a hello from our bright-eyed girl has garnered a lot of warm smiles. Alena loves to play with other kids and knows the names of many of the children who live in our neighborhood. She loves to be outdoors and spends many happy hours at the parks near our home. She gets just as excited as her big brother when she sees squirrels, birds, deer, or any wild or domesticated creature that may wander by.
Alena is talkative and keeps us entertained with her words and phrases. She called Lucas “Wuwu” and herself “A-nana.” Jaime is either Daddy or “Dabu,” and I am usually Mommy these days. She speaks with a great deal of feeling and inflection. “Wait, Mommy!” “NO-NO Wuwu! A-nana’s turn!” “Are you doing, Dabu?” She asks to see Jaime and Lucas as soon as she wakes up in the morning and after her nap. She’ll be in for a shock once Lucas returns to school and Jaime returns to work!
Speaking of Lucas, Alena adores him. They have the occasional sibling argument which usually stems from Alena stealing all of Lucas’s toys and refusing to give them back, but usually they get along great. Lucas is Alena’s favorite playmate and she loves doing exactly what he is doing (no matter if she’s capable or not).
They are the sweetest of siblings and the best of friends.
Alena likes to do things together as a family. If I suggest a walk, she delivers Jaime’s and Lucas’s shoes to them and monitors their progress before pulling them out the door. Alena likes to get a laugh and Lucas is a willing party. A few of her favorites this year have been dumping her dinner bowl and doing a very convincing Cookie Monster impression. We have cleaned a LOT of food off the floor.
Alena is good at sharing for a two year old and when she’s not throwing her food on the floor, she likes to share it. She will walk all over the house searching for Lucas or Jaime in order to give them a soggy bite of whatever she’s eating. Lucas has gotten pretty good at pretending to take bites rather than brave her wrath by refusing.
I would love to tell you what Alena’s favorites are, but unlike her brother who has always had strong preferences, Alena changes her mind regularly. One day she’ll love mac and cheese and the next day she’ll refuse to touch it. For a while she loved the color green and constantly was asking for a green shirt. Before I had the chance to acquiesce she had moved on. Other short lived favorites that have gone the way of the dodo bird include Sesame Street, the Trolls Movie, the Madagascar theme song, and long walks in the stroller.
Here are a few of my favorite photos from this past year:
Alena is spunky little treasure and we can’t imagine our lives without her! We love her so very much. We are so thankful that she will forever be ours.
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!
Today Lucas is nine years old. Nine on the 9th! His golden birthday. Lucas was only two and a half when I started this blog. Where has the time gone?? This past year was a good one for Lucas, although it has been an odd year with the pandemic. Thankfully he has continued to grow and thrive, even in these extraordinary times. This morning we headed to Kensington Metro Park, one of Lucas’s favorite destinations, for a picnic. During the car ride, I told him that I write a blog post about him every year and asked what he’d like me to say. Here are the things about himself that he wanted to include:
Color: Yes, still orange!
Song: Butterfly by Twin Peaks
Top two animals: Cats and coyotes
Book: The Hobbit by J.R.R. Tolkien
Sport: Soccer, naturally! 🙂
Foods: Quesadillas and homemade breadsticks
Snack: Fruit snacks
T.V. show: Wild Kratts
Movie: The Hobbit: The Desolation of Smaug
Fictional Characters: BB8 from Star Wars, Aslan from The Chronicles of Narnia, and Gandalf from The Hobbit
Little Baby Bum. (This was a show that Alena used to watch. It has nursery rhymes and other learning songs geared towards babies and toddlers and Lucas HATED it. He is much happier now that Alena has switched to Sesame Street.)
Getting his nails clipped
Alena destroying the scenes he sets up when he plays
Lucas’s most enduring interest has been animals. He has amassed a large collection of animal figures over the years and he plays with them almost daily. This year he spent a lot of time doing “Who Would Win?” battles with various animals. He loves nature shows, Wild Kratts, and making us feel ignorant with this vast animal knowledge.
He is genuinely excited to see any animal in its natural habitat. He will yell, “Mom, come quick!” I’ll run into the other room expecting to see a bear in our yard, and he’ll excitedly point out the window at a squirrel (as if he hasn’t seen hundreds in his lifetime!). He loves the zoo but his favorite is to go for a walk in the woods and on trails at parks to look for critters and animal dens. His favorite bird is a blue heron and he was thrilled to find one in a pond just a few miles from our house, hunting for fish. We visit regularly now and he has named it Blue Fisher.
Lucas still greatly enjoys writing and drawing. One of his biggest laments when he found out he couldn’t go back to school this spring, was that he wouldn’t be able to retrieve his quiet time book, a blank book that he filled with writing and drawing during free time at school. Luckily I was able to find a similar book for him to use at home and he’s filled up two already. He also wrote his own Magic Tree House book (entitled The Deed of the Dodo) as a birthday gift for a friend this summer and learned to draw a bunch of Star Wars characters from a how-to book I found. He spent many happy hours making his own Star Wars encyclopedia on PowerPoint during the strictest days of our COVID quarantine.
Speaking of Star Wars, he got very interested in those movies this past fall. He watched the eight movies that were out before the holidays and then saw The Rise of Skywalker in the theater during his holiday break. After getting Star Wars figures for Christmas, he remade and performed all nine movies for us including some aspects of the original stories and adding his own details and variations. Once we were quarantined at home in March, he and Jaime built a plethora of the space ships and other vehicles by repurposing Lego bricks that Lucas had from other sets. I was impressed by what they came up with (and very happy not to have to buy a bunch of new sets).
He loves fantasy stories and fell in love with The Hobbit after reading it this summer. I suspect we may be treated to a Lucas remake of that epic tale at some point too.
Lucas enjoyed soccer again this year. In the fall he played for King Cobras, his school team, and Michigan Tigers which is the club where Jaime coaches. He got to have Coach Dad for both teams! He continued to play indoors over the winter but the spring season got canceled. This summer he has started up again with his Tigers team, however, it looks much different as he is only able to do training exercises with his team in a strict, socially distanced, outdoor environment. There are no player interactions, scrimmages or games but he is still thrilled to be back on the field!
And of course, now that he is a big brother, Lucas spends a lot of time with his little sister. It’s been fun to watch their relationship evolve from wide-eyed watching to all-out playing and tumbling around together. No one can make Alena squeal and laugh more than big brother! He reads to her, chases her around and wrestles with her, and keeps an eye on her for me if I need half a second to complete a task. He is very loving and attentive and only rarely gets mad at her when she steals his toys, drinks out of his water bottle, pushes her way into whatever he’s trying to do alone, or any other number of pesky little sister antics she pulls. She is lucky to have him!
All of these hobbies and activities are part of Lucas, but what we love most is his heart. He is sweet and considerate. He is loving and kind. Nearly every time we make a meal he likes, he tells us that it is the best we have ever made. He asks us how our day is going and listens to our response. He notices if we’re sad and tries to comfort us. He has already promised that when we get old he will stop by as often and he can and that he will take good care of us. He really is a gem and we love him so much!
To say it’s been a crazy few months would probably be an understatement. I was scrolling through the photos on my phone yesterday and this is what the days leading up to looked like:
February 29: Lucas slept over at my parents house. Betsy and I took Alena and went out for coffee.
March 1: We went to church in the morning and in the afternoon I took the kids out for a hike.
March 4: I took a lovely walk around central campus on my lunch break.
March 8: It was 60 degrees and Lucas’s soccer team had practice – outside!
March 10: I voted in the presidential primary.
March 11: I went with Lucas and his class on a field trip to the art museum.
Soccer practice on March 8!
It was all so normal. We were making plans to have a small party to celebrate Alena’s first birthday and brainstorming some ideas of fun things we might do over Lucas’s spring break. But March 12 ended up being the last day Lucas went to school. March 13 was the last day I went to work. I remember thinking on the first day of the lock down, “Oh, well, I guess we’ll be home for a few weeks and then things will go back to normal in April”. It’s June 29. Some things vaguely resemble our pre-coronavirus existence. Most things are still radically different.
Easter 2020: All dressed up with nowhere to go.
Our perspective on the COVID-19 pandemic has, predictably, been colored by the fact that Betsy is part of a particularly vulnerable population. We have been über cautious and I feel good about that decision. Even before cases started being reported locally, Betsy was starting to venture out less. We are pretty avid hand washers and usually have hand sanitizer when we go out but all of us were being even more diligent. My boss and I talked about the possibility of working from home for a time. Still, I never imagined how much our life was going to change over the next few months.
My basement work space/Lucas’s school computer/Zoom headquarters/laundry room.
The good news in all of this, is that we have all stayed healthy! (“PTL” as my mother-in-law would say)
We did the home school routine for two and a half months! I am personally very grateful for Betsy’s willingness to do the heavy lifting with respect to Lucas’s education. Being married to a teacher has its advantages. If it weren’t for her there is a pretty good possibility Lucas would be repeating the second grade next year. 😉 Lucas’s actual teacher did a great job organizing lessons and having video class meetings. On the last day of school, Lucas still considered second grade to be his favorite year of school.
Initially, Betsy was worried she wouldn’t be able to work at all since it was unsafe for her to see her tutoring students in her home office. But in the end, she was able to continue tutoring remotely using FaceTime. The parents of her students have been super supportive and willing to help out during the sessions when necessary! She works every day during my lunch break and a couple of evenings as well.
Since March I have only been going out to get groceries once a month. I leave early in the morning to try and get to stores before they got too crowded and I usually make stops at 5-6 different stores to make sure I can get everything we need and not give the impression that I am being a complete hoarder. And once I get home, I wipe everything down that needs to come into the house immediately and organize non-perishables that can stay in the garage for a few days. Maybe it is overkill, but we were not (and still are not) interested in taking any unnecessary risks. We’ve been extremely grateful for the friends and family that have been willing to grab us milk and eggs (and chocolate) in between my marathon grocery runs.
Quarantine crew – it’s a good thing we like each other.
The biggest thing is that we really did go into a pretty hardcore isolation. We would take walks around the block when we knew there wouldn’t be many people out and did a lot of playing in our backyard but that was really it for the better part of three months. We had a handful of visits from people who came to wave and say hello through our front windows. We have been grateful for the technology that has still allowed us to interact with so many people; what would we have done if this had happened 20 years ago before FaceTime and Zoom and Blue Jeans? In the last couple of weeks, we have started seeing other human beings (only outdoors and with plenty of social distancing) and it has been really nice. But I suspect it will still be quite some time before we step foot into someone else’s house or invite others into our home.
We got really good at ladder golf.
Lots of backyard soccer.
It has been hard to have many of our normal routines completely dismantled. There has been a loss of freedom. But it hasn’t felt hard to make these sacrifices for Betsy’s sake and for the sake of many others. And we are so grateful for those who have been doing the same! I don’t know what the future will hold. I have no idea if or when I will go back to working in the office. We have lots of questions about what Lucas will do about school in the fall. We have personal plans that are all kind of up in the air.
Real, live people!
All the way back in March, I was doing a Lenten inspired Instagram challenge and there were Bible verses for each day and you had to come up with a pictorial representation. The verse from Day 36 was Jeremiah 32:37-38. “I will bring them back to this place and let them live in safety. They will be my people and I will be their God.” It’s been good to reflect on God’s faithfulness and the promise of restoration, even amidst so much uncertainty, these last several months.
Baby girl is one year old today! She is such a gift. It’s been a challenging year in many ways, and also such a wonderful and memorable one! At the end of this first year we have a loving, proud big brother, a bright-eyed, energetic baby girl, and two extremely exhausted parents. But we wouldn’t trade our lives for any other!
Alena is growing and changing rapidly, so this post will be a small snapshot of this moment in time. What can I tell you about our girl? She is full of energy! This girl is always busy and rarely still. She is also full of personality! She has strong opinions and feels very free to share them. She seems to have big emotions like her brother–lots of happy squeals and laughter and cuddles and lots of loud protests. Buckle up!
Alena loves to be out and about. If she sees anyone getting coats and shoes on, she rushes over to the door. If we ask her if she wants to go outside, she starts clapping. On any given day if she’s fussy at home, she will cheer up outside or running errands. She likes to look at people and animals and likes to check out new places. She is usually pretty friendly and will even hold out her arms and go to a stranger on occasion. She currently loves squirrels and has lots of fun watching them run around in our yard. She laughs and squeals if they peak at her around a tree trunk. Lucas used to call them “mermals” and for now, Alena calls them “pwe”. She seems to feel that one syllable is enough for most objects. (I am “Mum”, Lucas is “Baa” and Jaime is “Gah.” The only two syllable word she currently employs is “baby.”)
Alena detests diaper changes and likes to make it as impossible for me as she can. As soon as I lay her down she begins twisting and turning and crawling away. If I manage to pin her down she lets me know loudly that she doesn’t approve.
Alena is a climber! She started climbing around eight months old and hasn’t slowed down. If I turn my back for a second she’ll be on the low window sills in our front room, on the top of the slide in the basement, half way out of the baby tub, or in some precarious position on the stairs. She even tries to climb out of her learning stool onto the kitchen counter.
Although “Mum” is still her favorite, Alena adores Lucas and Jaime. She tries to be as close to Lucas as possible and do whatever he is doing. She does her very best to keep up with him! She watches him intently and copies what he’s doing. Much to Jaime’s delight, after watching Lucas, she has figured out that soccer balls are for kicking and has already started her training.
Lucas is usually very tolerant of Alena grabbing for all his toys and pushing her way into his space. They have a special bond and she’s a lucky girl to have such a great big brother!
Alena sprints to the door when Jaime gets home from work each evening (or nowadays walks up the stairs from his basement work station). She loves to catch a ride up on his shoulders and likes to play games with him like pulling off his hat or knocking down the towers he builds. She also loves to cuddle up with Daddy at night.
Alena’s current favorites:
Food: Macaroni and Cheese
Book: This is a three way tie between Peek A Who?, First 100 Animals, and Who’s Wearing a Hat, is it the Orange Cat?
Color: She often chooses orange just like Lucas did! We’ll see if that sticks. It would be mighty convenient since we already have all things orange!
Animal: She loves squirrels, and much to Lucas’s delight, she also adores all his toy cats and any cat that pops up in a book.
Here are month by month photos from the year:
Even though a year has passed, having Alena still feels like a dream sometimes. Thankfully it’s a dream we won’t have to wake from! There isn’t much danger of actual dreaming anyway, as she barely lets us sleep. 😉
Happy birthday sweet baby girl! We all love you far more than words could ever express.
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.
It’s so hard to believe that 15 years has passed since you left us. Fifteen! How can that be. I can still hear your voice, as if I spoke with you yesterday. I can still see the contours of your face, feel the warmth of your hands, and hear the sound of your laughter. I easily picture your small frame and the way you walked (a purposeful walk!). I can see you standing by the sink on one leg like a flamingo, rinsing out your mouthpieces after a breathing treatment. I remember you in your blue robe sipping juice at the kitchen table with damp hair in the morning. I see you sitting at your sewing machine working on your quilt. Do you know, I never took that little piece of medical tape off your sewing machine? You must have put it there to mark a seam. It reminds me of you. And speaking of medical tape, I’ll admit, I’ve started wrapping gifts with it sometimes too now. I mean, why not?
I don’t dream of you as often as I used to. I suppose that is affected by the passage of time, and the fact that the intense grief is less constant now. But when I do, they’re always vivid, and I wake up feeling like we really talked. In my dreams, you’re always wearing a bright pink shirt. We sisters have been talking about our memories from the last months of your life. I remember the last time I saw you. I knew it would be the last, and it was so hard. You were laying on the couch, so still, and so tiny. In my memory you were wearing that pink shirt. I honestly don’t know if that’s accurate, but your pink shirt has become, to me, representative of you. Just like that shirt, you were bright. You lit up the room, and you were also so smart! You were vibrant and colorful. You were confident. You were fun.
You left an indelible mark on me. In fact your life had a tremendous impact on all our family, and on your friends and acquaintances too. You did such a good job living, keeping your humor, keeping your faith, staying you in the midst of your struggles. I know that’s not easy. In fact it overwhelms me to think of how much you suffered, especially at the end. Honestly, it scares me. Yet you lived and even died with grace and with purpose and with gratitude. You focused on the gifts and blessings and beauty around you and endured the pain and loss and grief without bitterness. You had so much strength and an incredible amount of courage. You lived well and you died well.
I wish I could sit down with you over beverage…coffee for me these days (I’m tired!). Maybe some Faygo for you. What would I tell you if we could chat? What would I say about these last 15 years? I’d want you to hear about the new medication I’m hoping to take soon for my CF. I hear it’s life-changing. I’m hopeful. I think you’d be so excited about it. How I wish you were here too and we could try it together.
I’d probably share some of my CF struggles. It would be nice to tell someone who truly understands. But I wouldn’t want to spend much time there. It’s not the biggest, most important part of my story these last years. I won’t allow that, because I learned from you, that it should never be. You never made cystic fibrosis your focus or your identity and I’m trying to follow your example.
I know I’d want to talk about my kids. First Lucas. I’d want you to know that he loves you. It is really the sweetest thing. He always includes you when he makes lists of family members or counts how many aunts he has or asks me about what it was like growing up. He told me when he gets to heaven he will be sure to find you. He wishes he knew you here on earth. He talks about you quite regularly. He comforts me when I’m sad because I miss you. He reminds me of you in some ways. He’s smart and very creative like you! He’s very loving. I think you’d get a kick out of the things that make him unique.
And Alena. There are times when I see your face in hers–an expression or a passing look. I’ll never forget the first time she smiled at me, a true “soulful” smile rather than a reflex. She looked up at me and her whole face broke into a grin and it felt like the sun had just burst out from behind the clouds. She is already vibrant, like you! She’s opinionated and feels free to “speak” her mind when she deems it necessary, also like you. She has a great laugh. She makes us work for it, but it’s so worth it! It’s bubbly likes yours. She doesn’t know you yet, but she will. We will tell her.
On my bed I have a basket full of supplies I might need for Alena in the night or early morning. It’s that basket you made, covered in blue fabric with cute little tulips. It’s a reminder of you within arms reach. I imagine maybe someday she’d like to have it.
I guess what I’m saying is that I see daily reminders of you–in my kids, in my house, in nature, in myself. Perhaps it’s because I’m always looking. It keeps you close to me and makes the distance between us feel a little less vast. But really you are here. Bits and pieces of you that you left along the way. Ways you touched our lives, memories we have, stories we tell our children about you, gifts you made, funny things you’d say, advice you’d give, things you wrote. Especially the example we had simply by watching you. All five of us sisters looked up to you (figuratively of course 🙂 ). You were a worthy line leader and an amazing example. I miss touching you and talking to you, yet you live on in my heart and I’ve brought you with me in my experiences. Because I loved you, and you loved me, and we are forever part of each other’s stories.
I miss you and love you so very much. We all do. How could we not? That will never change. And when we get to heaven, we’ll find you.
Sheri Leigh Van Bruggen~September 26, 1968-September 4, 2004
It’s that special day, Lucas’s birthday! I feel like I was just writing his seven year post. This past year has been quite the blur. It was a good one though. Here’s what makes our boy tick at age eight! I’ve sprinkled in some funny conversations we’ve had along the way:
Color: Still Orange!
Food: Chicken quesadillas
Song: Butterfly by Twin Peaks
Books: The Chronicles of Narnia by C.S. Lewis and the National Geographic Animal Encyclopedia for Kids
Animals: Cats closely followed by foxes
Sport: Soccer, soccer, soccer!
Going to bed (when do they grow out of this??)
Waking up (complicated by the aforementioned dislike)
Sweets (except for fruit and fruit snacks)
Picking up his toys (he regularly leaves death traps for us on the stairs)
Lucas is still into creative play and making his own movies, shows and games from beloved characters such as Lego or Chronicles of Narnia characters.
L: Mom, I made a new Lego 2 movie game! You get to be Emmett. The first challenge is to jump over the venomous red pants.
B: Venomous pants?!?
L: Yeah! They’re poison but they can’t kill you. They make you sick for two years which is better than dying. Although maybe not much better…
His movies, shows, and games always seem to have a great deal of action and peril. Never a dull moment in his imagination!
Right before Lucas turned seven, he got into VeggieTales and that was what interested him for much of the fall, even edging out DC Comics and Legos temporarily. The general veggie theme extended to his interest in my pregnancy and what fruit or vegetable size the baby was in utero that particular week. He even liked perusing the produce section at the local grocery stores to see what interesting fruits and veggies were available.
Right after Christmas (after asking for and receiving a lot of VeggieTales toys and movies), he lost interest and it became all animals all the time. He fished out his animal collection and built many different zoos which littered our floor for months and months.
Lucas’s zoo and the zookeepers
He also spent hours poring over his animal encyclopedia and made me feel good and ignorant with all his knowledge.
L: Mom, what do you like better? The African wild dog or a dhole?
B: Dhole? What’s a dhole?
L: Oh, you don’t know? It’s also a wild dog. It’s about the size of a margay.
B: ………..What’s a margay?
He started watching Planet Earth and as many spin-off series we could find. He loves David Attenborough and now narrates his own informational animal shows with a British accent.
The snow leopard is enjoying his meal in Lucas’s version of The Hunt
He and Jaime went on many nature walks in the area looking for animals and took many trips to various zoos. He used to love farms and wanted to be a farmer. Now he says he wants to be an animal scientist!
Soccer is still a big interest and he had a good year playing on the King Cobras team with his schoolmates. He and Jaime also attended many DCFC games, both the home games and any away games within a drive-able distance!
At a DCFC game with his friends
This year he continued his love of writing and filled several journals with his stories. He wrote his own Frog and Toad book, several Narnia-themed stories, some DC super hero adventures, and adventures involving his friends and family. At some point I introduced him to mechanical pencils because I got so tired of our pencil sharpener getting clogged. Now I’m so tired of picking up tiny pieces of lead off the floor.
Lucas likes a certain amount of structure and order in his life. He also loves to categorize things and make lists. He has lists of characters, lists of animals, lists of vegetables, lists of states, lists of license plates from different states,lists of projects he wants to do, lists of stories he wants to write.
J: What do you want to do right now, Lucas?
L: Let’s make a list of everyone we know in alphabetical order! I’m a little tired, so this will be a good, relaxing activity.
J: … … …
As you might expect, a huge part of Lucas’s year was my pregnancy and the birth of his sister Alena. He showed a ton of interest all through the pregnancy in how I was doing and where Alena was at in terms of growth and development. Since she’s been born he’s been a very loving and attentive big brother and he has earned her adoration. She watches him with wide eyes and gives him lots of smiles.
He’s very proud of her. And we’re very proud of him. Happy Birthday to our wonderful boy!