Monday, April 5, 2021

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.











Monday, March 1, 2021

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.







Monday, February 1, 2021

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.






Monday, January 4, 2021

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.  




Monday, December 28, 2020

Week 384: To the teeth

 I hope all of you are having a safe and happy holiday week. We are hunkered down here in our little corner of Madison, but just because we are hunkering doesn't mean we aren't having fun. The kids, for example, are pretty much having the time of their lives thanks to the generosity of everyone who gave them stuff this year. The number of things I have assembled from tiny individual plastic parts over the past few days is sort of mind-boggling. Also, my house seems to be littered with an absurd number of holiday-colored Hershey's Kisses wrappers. And, Lime managed to lose his first tooth on Christmas Day, while "secretly" consuming said Hershey's Kisses. But, did I mention the kids are happy? They are happy. And when you get down to it, that is what matters.

It is hard to believe that we have made it to this moment, the very last few days of the year 2020. What a year it has been. In stark contrast to what has been going on all around him, this has probably been the healthiest year of Lemon's life. He's grown tremendously, hasn't been sick a day since April, and has been able to almost forget that he isn't quite like everyone else. Being home all the time suits him just fine, he continues to be, in his favorite babysitters words, a "house cat." He likes being in his comfortable, familiar surroundings with all his stuff, and even now when leaving the property is a rare treat, he almost always asks if he can just stay home instead.

I am not sure what to expect from 2021, and perhaps it is best to just set expectations to a minimum and see what happens from there. I am still hoping that within the next few months, Trikafta will be approved for Lemon's age group, and then this state of health will just become his new normal. On the other hand, I am concerned that there most likely will not be a COVID vaccine for kids his age by the time fall comes around again, and we will be faced with some really difficult decisions about whether either of our kids goes back to school, or if not, what the alternate plan will be. 

But, all that lies in the future. For now, I just want to wish all of you loyal readers a happy new year. It feels good to bid 2020 farewell, and I hope to see all of you next year!








Monday, December 21, 2020

Week 383: Not quite there yet

 We are so close to the end of 2020. But, in true 2020 fashion, this year had at least 1 more trick up its sleeve for me--a very urgent end of year project! So, please enjoy these photos of the kids getting in some of their last 2020 kicks, and with any luck I will be able to provide you with a more cogent summary of the year next week!








Monday, December 14, 2020

Week 382: Let it snow

 It looks like we will be having a white holiday season here in Madison, with 7" of fresh snow falling over the weekend. The kids were of course beside themselves with joy at this transformation of their world. Luckily the new boots I ordered (since when do I have children with feet this big?) arrived just in the nick of time. With only 4 days of school left until winter break, let's just say that spirits are high among the younger generation. 

After weeks of watching Lemon's weight gradually tick downward, following our attempt at cutting back on the tube feeds, I decided we reached a point at which I was not comfortable going any lower, so we've added back DuoCal powder for now, to see if we can get things to stabilize. He is still eating more than he ever has before, but it is still clearly nowhere near enough to actually sustain him, and his intake didn't increase as much as I had hoped to fill the void left by the missing formula. Oh well. It was worth a shot. 

We, like everyone else, are so excited about the great vaccine news, it really is starting to feel like there is a way out of  this mess. We just need to continue to be patient, especially for the kids--it is sounding like a vaccine for them is still a bit of a ways off. Now I am wondering, will Lemon get Trikafta first, or the COVID-19 vaccine? No matter the order, I have high hopes for 2021!