July 2021: We've got it

 After years of waiting, we finally have Trikafta in hand. After all that time, and given how expensive the drug is, I had expected the last step to be more of a hurdle, but instead it was totally seamless. Our doctor wrote the prescription, I signed some paperwork to enroll in Vertex's patient support program, they contacted me and found out what specialty pharmacy we wanted to use, and bam. A couple of days later the medicine showed up.

Lemon has been a champ about taking it so far. The main challenge is that it has to be taken with some kind of fat-containing food. At night, it's no problem, because he is about to do a tube feed so even if he doesn't eat much dinner, we know the formula will take care of it. In the morning, though, he does have to eat something, and for those of you who have been following along for any amount of time, you know that Lemon and "eating something" are not always concepts that go well together. But, we have found that he is usually willing to at least drink a glass of whole milk, so smooth sailing thus far. 

I have to say that at least up until now (we are still only on our second week) I haven't noticed any marked effects, either good or bad. I feel like the big results for us will come when Lemon goes back to in-person school in the fall. It is my greatest hope that the Trikafta helps him weather all the colds and other viruses that will undoubtedly be circulating in great abundance once everyone is back together again, so that he won't be constantly sick. So, we shall see.

Meanwhile, the summer has gotten off to a good start. The kids have had their first couple of weeks of camp, which have been pretty successful. I am definitely feeling good about the decision to just send them for half-days, that seems to be all the structure their little minds can handle just yet, and they like having their afternoons at home to bop around. With any luck, they will eventually warm up to the new (very nice, perfectly competent) sitter that we hired for the summer. 

Lemon is participating in a big study of the effect of Trikafta on kids. We had the first study visit the week before he started the drug, and I was pleased to find out that he was our hospital's very first research subject for the study. They hand-picked him because he has done so many other studies that they thought he was their best shot at having _their_ first attempt at this one very big, complicated study visit go well. And, he did not disappoint, he was amazingly cooperative, and since almost all the tests were familiar to him, he didn't balk at anything and we were "only" at the hospital for 4 hours, as opposed to the 7 projected in the study protocol. They took samples of pretty much every possible bodily fluid, did multiple different types of breathing tests, and trained us on a home spirometry device so that we could do even more breathing tests at home. We have to go back in another couple of weeks to do the next visit at one month after starting Trikafta, Unfortunately we won't get to see any of the data until the study is published, but Lemon is still very proud to be one of only 150 kids from around the country who are participating in this. 

To top off a generally excellent month, we were also treated to a visit from some cousins who live in Maryland and haven't seen in person since the summer Lime turned 2. It was wonderful to have them with us for a few days, the kids had an uproariously good time, and cried when their cousins left to continue their road trip. We can't wait to see them again, and to be reunited with everyone else that we haven't seen in far too long.

June 2021: On the cusp

 Amazingly, we have arrived at the last week of the school year. In just a few days, we will be done with K and 2, and on to 1 and 3. We went to the shoe store earlier this month as a very exciting family field trip (yes, COVID has adjusted our standards of what counts as an exciting family field trip) and I could not believe how big the kids' feet are now. Of course, they barely wore the shoes we got them in the fall since they have hardly left our property since that time, but that is a separate issue. In another week, they will be off to day camp every morning, which may be a bit of a shock to their systems (and ours). 

May was also CF awareness month. The annual Great Strides walk was virtual again this year, and for our team consisted of putting on our team T shirts and walking around the corner to pick up our weekly CSA share. But, the length of our walk should not be in any way construed as reflective of our commitment to the cause, which is as strong as ever. Thank you so much to everyone who donated to our team, and we are really looking forward to having the walk in person again next year. It's a great chance to connect with other CF families in the area, which we otherwise almost never get a chance to do, even without a global pandemic.

Lemon had a clinic visit last week, which was in person. I think since we are still spending so much time at home, the chance to be out of the house and the center of attention is still very exciting. He did a great job on his PFT's. It is amazing to see how much skill and control he has developed since he first started doing them. His lung function looks great and he's continuing to stick right on his growth curve so everyone was very pleased with him. His cultures came back clean as well, so that is great news.

I think we and many other households in the CF community are on pins and needles tonight because tomorrow is the day that the FDA is supposed to announce its decision on whether to approve Trikafta for ages 6-12. We have been waiting for this day for Lemon's entire life, and it is hard to believe that it is finally here. We have all the necessary labs, exams, and paperwork done, and Lemon's doctor is prepared to write the prescription for it the moment the FDA makes its decision. So, fingers crossed that the decision goes our way. I can't wait to see what the next chapter of Lemon's life will be like.

 

May 2021: A new adventure

This month, I took both kids for their first visit to the pediatrician since the before-times. Both are doing well, almost as though being home all the time and constantly grazing on tempting snacks has had some salutary effects. I am pleased to say that after scraping by at around the 10th percentile for most of his life, Lime has shot up to the 50th percentile. Lemon is still in around the 70th percentile for height and weight, which is great and will hopefully put him in a good position for (fingers crossed!) returning to school in the fall.

The major development for April is that I have made a big career move. After almost 6 years as a medical writer on a clinical research team, I have moved back to my roots as a biologist, taking on a big new role at a local biotech company. It was a tough decision but I was ready for a new challenge, and I feel like (fingers crossed!) the kids are now old enough and stable enough that I will be able to manage a more demanding schedule. The job change meant that I was able to take a couple of weeks off this month, my longest work-free break since being on maternity leave after Lime was born. It was a treat to be able to focus just on hiking, yard work, and house projects, and the time flew by much too quickly. My first day at the new job was today, so I can't really say much about it so far other than that my new email seems to be working and I filled out a W-4 form without too much difficulty. Hopefully I'll be able to provide a fuller picture next month.

In kid news, Zoom school continues to crank along. The kids are both tired of learning in our basement, but they are also learning so much. Every day now they come out with some new skill or fact that is a surprise to us--naming different types of clouds, going over the life cycle of a butterfly, adding 3-digit numbers in their heads to figure out how many points they need to buy things in their various computer games. And doing everything on Zoom did prepare them well for a new life experience that they both enjoyed tremendously, playing MineCraft online with their cousin who is from Brazil. So, they are certainly growing up prepared to be part of a global, online community. We also have them signed up for various local summer programs on the theory that they should probably relearn how to interact with their in-person peers before the next school year begins.

Last but not least, May is CF awareness month. As you know, we are hopefully (HOPEFULLY) just a month or so away from the FDA approving Trikafta for kids Lemon's age. The groundbreaking research that has led to the development of Trikafta would not have been possible without the CF Foundation, which has also advocated tirelessly for people with CF during the COVID-19 pandemic. Since the pandemic is still ongoing, the annual Great Strides walk will be virtual this year, and I hope you will consider making a donation to support our team. I must confess that with all the excitement of the new job I have not quite figured out how to get the Great Strides app to synch with Strava, so you’ll just have to take my word for it that I’ve walked (or run!) the requisite number of miles. 

Just click right here to make your way over to our team's fundraising page.

Thank you so much as always for your generous support!

April 2021

Welcome to spring, at last! I wasn't entirely convinced that we would make it through the winter, but we do seem to have come out the other side. Even here in Wisconsin, the bulbs are pushing up their first leaves, and we're having fun roaming around the garden determining which plants survived the winter. 

We have laid all the necessary medical groundwork for Lemon to being Trikafta. We did the necessary eye exam, which he passed with flying colors, and seemed pretty pleased about having the opportunity to show off his letter-recognition skills to the optometrist. After the eye appointment, we went over to the hospital to finally have blood drawn for the huge list of labs that had build up between the GI doctor and Lemon's regular CF doctor. Lemon handled himself like an absolute champ, sat as still as a statue for the phlebotomist without having to be told. The days when I had to hold him in my lap and restrain him for blood draws seem like a distant memory now. And, the blood work was all good news, liver function looks normal, vitamin levels normal, iron levels normal. So, we are in as good a position as we can be, and now we just have to wait for the FDA.

I spoke with the research coordinator at the CF center this week, and she let us know that the investigators in the hyperpolarized xenon study have decided to only let kids 9 or older participate in the second phase of the trial (ie the post-Trikafta phase). Lemon was delighted to hear this news, as he was not a fan of the MRI. He as less delighted to hear that I had eagerly agreed to enroll him in a different study that will follow his progress once he starts Trikafta. But, once I had reminded him how hilarious it would be that we would once again be sending his poop through the US Mail to help science, and that he didn't mind any of the other things he would be asked to do as part of the study, he came around pretty quickly.

Our little two-kid learning pod seems to be functioning fairly well. I think the kids, especially Lime, would love to have some more friends, but on the whole they have been handling being essentially together one on one 24/7 better than I could have hoped for. I think they are both pretty over Zoom school, but we keep reassuring them that the end of the school year is in sight. In a fit of optimism, I signed them up for a bunch of half-day summer camp programs, figuring that it would be good to gradually reintroduce them to civilized society before in-person school starts for them in the fall (fingers crossed!).  I do wonder what they will make of this year of their childhood in retrospect.

March 2021: Marching along

 February has been an exiting month all the way around. Weather-wise, we had a couple of weeks of real deep-freeze, when we essentially did not leave the house for two weeks, making the cabin fever of the pandemic that much more intense. Thankfully that stretch of weather is behind us now, and we are actually having a much-needed midwinter thaw at the moment. Of course it is still just March and this is Wisconsin, so no doubt there will be another wintery stretch yet, but these warmer temperatures do give hope that we've almost made it through.

There are some major changes in the works with our learning pod, as our school district made the decision to reopen for in-person instruction later this month. There will be both in-person and fully virtual options, and as far as we can tell it looks like families in our district are splitting about 2/3 in-person and 1/3 virtual. We have decided to keep our boys home for the rest of the school year. After talking to Lemon's doctor, it really seemed like since we are fortunate enough to be able to keep him home for now, it is the safest thing for him. And, to be honest, Lemon is perfectly happy at home for now. Lime, on the other hand, yearns for friends his own age, to see his teachers in person, etc. It is hard to keep him out of school, but we are trying to bear in mind that it is really just for 10 or so weeks until the end of the school year, and with any luck things will be normal-ish in the fall and they can both go back.

The other two families in our learning pod have made the totally reasonable decision to send their kids back to school, which will be a big change for our kids, who will miss having some other people around during the day. But, they got through last summer as just the two of them so no doubt they will manage this extra time as well. 

In CF news, Lemon had his second study visit for the hyperpolarized xenon study. This one went even more smoothly than the first. Lemon remembered everything he was supposed to do and flew through all the tests, even the tricky lung clearance index, so we got home hours before I had expected. He also had a video visit with his doctor this month (which turned into me having a phone call with the doctor when the video software failed). He's happy with how Lemon is doing, and we are set up to do an eye exam and some bloodwork later this month so that we will have all the boxes checked for the moment that Trikafta is approved. We have our next follow-up appointment in late May, and it is so exciting to think there's a chance Lemon could even be on Trikafta by the time that visit rolls around.

In vaccine news, Papa Bear is fully vaccinated thanks to his work at the VA hospital, and I was able to receive my first dose as the unpaid caregiver for someone with CF. And, Nona was able to get her first dose at last, so hopefully before too much longer we will be able to start doing stuff as an extended family again. In the mean time, we are doing all the things we have come up with to do over these many months, baking, making candy, building with Legos and other toys, art projects, origami, reading, and watching movies. As ever, we love and miss all of you and look forward to seeing you just as soon as we can. Stay safe and vigilant, and hopefully we will be on the other side of this soon.

February 2021: A little hope

 Hello everyone, and welcome to the new monthly blog format! I hope the start of 2021 has been good to you. We are making our way through the Wisconsin winter here, aided by lots of snow and baked treats. Our school district has remained 100% virtual, so our learning pod is continuing to run. The thrill of online school has definitely worn off for the kids, but they're doing their best under the circumstances and are still learning a lot of new things. Still, I can sense their eagerness to be back in the real world again. 

Of course we are very excited and eager for vaccines to become available to us. Papa Bear will get his second dose (through his work) on Wednesday of this week, but the rest of us are still waiting. Although people age 65 and over are now eligible in WI, Nona and Opa have to wait until their provider has doses available for them. Finger crossed that will happen sometime in the next month or so. As for me, well, I'm hoping for sometime in the spring or early summer. And no idea when anything would be available for the kids.

Late last month, Lemon did the first visit for the latest clinical study that he is involved in, the one that is testing the use of hyperpolarized xenon for lung imaging by MRI. This was by far the most involved study visit we have ever done--I had no idea exactly how involved it was going to be until the day got underway. We started off in the pulmonary function lab, where Lemon first did the usual pulmonary function test. He's quite used to it and got a couple of good measurements done really quickly. Then, we had to do the lung clearance index test, which is much trickier. Basically Lemon had to make a seal with his mouth around a thing that is kind of like the mouthpiece of a snorkel. Then, he had to breathe normally, without breaking the seal, for several minutes (while wearing a nose clip to make sure he was breathing only through his mouth). For a very fidgety 7-year-old, this is a big challenge. Honestly, I think it would be a little tricky even for a cooperative adult. So, that took numerous attempts to get a couple of good measurements, but we got there eventually.

Then, we walked across the hospital campus to the building where the MRI was. Due to COVID-19 restrictions, I couldn't actually go back to the MRI room with Lemon, so the nurses whisked him away and he had to handle

the whole thing himself. He got lots of compliments on his behavior, which is impressive especially given that each scan took almost an hour, and he had to breathe in the hyperpolarized xenon which made him feel a little dizzy. In another sign of his advancing maturity, he was offered his choice of either a truck or a Green Bay Packers scarf from the prize back, and he chose the scarf. Who is this child?!?

While we were at the hospital, I asked the doctor there if there was any update on the approval for Trikafta, since Vertex had signaled earlier that they were planning to file with the FDA before the end of 2020. The doctor said his latest information was that they had delayed the filing, and were now planning to put in the application in the second quarter of this year, which was very disappointing news. Imagine my surprise and delight, then, when Vertext announced just a few days later that they had filed, with an anticipated approval date of June 8 of this year! I am so excited, I can't believe that the big day is now so close. And I am so hopeful now that Lemon can be on this amazing medication before going back to school next fall, which will be huge for our peace of mind especially if there isn't a COVID vaccine available for kids yet.

So, all in all, I would say 2021 is off to a pretty good start, and I hope all these favorable trends continue. Like all of you, I am feeling very ready to put 2020 further and further into the rearview mirror. Let's see what February has in store.

Week 385: Into the future

 You guys, we made it through 2020. In retrospect, no idea how, although I did just empty out the 2lb bag of baking yeast that I bought at the outset of the pandemic, so there's one small clue there at least. In addition to the victory of arriving at 2021, I can also now report that we survived winter break. I didn't manage to do any themes or plan any kind of real daily schedule, but we muddled our way through. The kids had their haul of Christmas toys to play with, there was snow on the ground, we did many art projects and edible experiments, watch movies, and generally made the most of our extended time together. I also had a last minute project for work, just to keep things extra interesting. 

In other years, I've done kind of a year-in-review post, but let's face it, no one really wants to dwell on 2020 for a moment longer than absolutely necessary, so let's look forward instead in the hopes that 2021 will be a different sort of year. We are getting things off to a promising start in our household, with Papa Bear getting the COVID vaccine on Wednesday (through the VA Hospital where he works). I have no idea when "healthy people who work 100% from home and are under age 45" will come up on the priority list, but I'm guessing not for a while, and of course the vaccine for kids is a ways off yet. 

Later this month, Lemon is still scheduled to participate in his clinical study of imaging with hyperpolarized xenon. The COVID situation in Wisconsin has been better as of late (not great, but better) so I think I will take him to do that unless things take a turn for the worse in the next couple of weeks. Maybe someone on the study team will have some more information on when we can expect to get Trikafta, since there is a second "post-Trikafta" phase of the study. 

School in our little learning pod resumed today (YAY!!!!). I am incredibly grateful that we have this option for our kids, and it was kind of amazing to see them just pick right back up where they left off, doing their Zoom classes and running around in our back yard at recess. In four more days our school district will announce its decision regarding school reopening for the 3rd quarter of the school year. Even if they reopen, there will be a remote option, which is of course what our kids will be doing. I am cautiously optimistic that they will be back in person for the 2021-2022 school year, but that is still a long time from now.

One thing that has become clear to me from watching the kids over the winter break is that early childhood is well and truly over for both of them. With Lime losing his first tooth and learning how to read, he's beginning to make the transition from "little kid" to "kid." Lemon, meanwhile, is the living embodiment of boyhood, reading chapter books on his own, causing trouble, and so full of life.

Seeing all these changes in the kids has made me reflect on this blog, how it started out and where it is going. And so, dear readers, after over 7 years of weekly posts, I've made the difficult decision to step the frequency of the posts down to once per month, still on Mondays of course. When the kids were littler, things changed faster and the weekly cadence made sense. Now that they are bigger, the timescale has thankfully slowed down somewhat, giving their Mama Bear a bit of breathing room at last. I think the monthly posts will be a better fit for how they are now, and the pace of their lives. I hope you'll continue to follow our journey in this new format, it wouldn't be the same without you. 

Wishing you health, happiness and all the best in 2021, and see you all soon.