November 2021: Cone-head Zombie

We have made it through another month of in-person school! As I expected as of last writing, Lime tested negative for COVID, phew. Aside from those few missed days, both kids have been there the entire time. I feel like we are finally settling in to a routine with going to school and work every day. A slightly chaotic routine, don't get me wrong, but a routine nonetheless. 

The kids both got their flu shots last week, so now we just begin what is hopefully the final wait until they can get vaccinated for COVID and we can all breathe a sigh of relief and let up on the levels of general paranoia ever so slightly. Maybe even triumphantly return to highly novel concepts like "extra curricular activities" and "swimming lessons" and "going inside friend's houses" and whatnot.

Lemon had his first post-Trikafta clinic visit last week. He is sticking right to his growth curve, despite having recently gone down to one (ONE) carton of formula per night. The amount and variety of foods he eats now would have shocked me five years ago, I would never have believed it possible. The dietitian couldn't believe it either, but said that if he keeps this up we might be able to get rid of the g-tube entirely next year. Lemon is very excited about that possibility, so hopefully that will incentivize him to keep up his progress. He also blew a new record on his pulmonary function tests, so it doesn't get much better than that from a lung perspective.

The only slight cloud in the sky, and it is slight, is that Lemon's liver enzymes went up pretty substantially after starting Trikafta. This is a known side effect of the drug, and his doctor didn't think that this amount of elevation was cause for immediate concern but is definitely something we will keep an eye on. So, we will redraw that at his next clinic visit and see how it is doing. In the mean time, we will keep on keeping on.

The kids had a lot of fun for Halloween yesterday. In a new parenting achievement, I hand-made their masks following instructions we found online. Lime was convinced that no one would be able to tell that the costumes were home made instead of store bought. I am flattered if not entirely convinced, but as long as they liked them I am more than satisfied!

 

October 2021: Wait it out

 This week, we have had the opportunity to experience one of the great dilemmas of in-person school during the time of COVID. On Friday, Lime came home with a slightly stuffy nose. By Saturday morning it was clear that he had a cold, or something that was presenting like a cold. There had been a few cases of COVID reported at the school during the week prior, none in either of the kids' classes, but still enough to be fresh in our minds. Luckily, we had a rapid antigen test on hand at home, so over much protestation I swabbed Lime's nose and conducted the test. For those who haven't used one yet, I highly recommend, it, it was fast and easy to do. Within 15 minutes, we had our result: no COVID. Yay!

Except that as of this morning, Lime was still very definitely having cold symptoms, and per the school's policy, anyone who has had symptoms within 48 hours needs to have a negative PCR test before returning. So, despite being 99.9% sure that Lime does not have COVID, we kept him home from school today and I took him over to the pharmacy to get a PCR test. With that bribe of a Happy Meal lunch in front of him, he made it clear that yes, he is entirely capable of holding stone still and not protesting while getting his nose swabbed. So, now we wait for the results, and Lime stays home. With any luck we will get them before the start of school on Wednesday and we can put this little episode behind us. I do wonder, though, how this is going to work in the winter, when it seems like at any given moment, at least 50% of all children have a runny nose or a cough of some kind. 

Other than that little drama, it has been a fairly uneventful month. I snuck out of town for a few days to compete in a marathon, my first one in two years, and it was such a joy to be out racing again. Lemon had his 3-month follow-up visit for the clinical study that he is doing, and luckily this visit was less intensive than the first couple, he "only" had to do the two different breathing tests and answer a barrage of questions. While we were there I saw the nurse-practitioner who used to oversee Lemon's care. She hadn't seen him since before the pandemic started and could not believe how much he has changed. He grew almost an inch just between July and now, never mind all that has happened since March of 2020 (remember those days?). Our next follow-up will be his 6-month visit, conveniently timed for the week between Christmas and New Years. At least he won't have to miss school?

Like many of you, we are eagerly awaiting some good news from the FDA on vaccinations for the kids, hopefully later this month. Once they are vaccinated I think we will all breathe a big collective sigh of relief, and then begin to think more seriously about reengaging with the rest of the world--that means you!

September 2021: In person

 Despite cancelling all of our summer travel plans, we managed to pack quite a bit of action into the last month of the summer. Lemon spent the mornings at a sports camp, which he said that he hated. Of course, every morning that I dropped him off there, he left me half-way down the path to the field to run and join the other boys. He learned a tremendous amount there in spite of himself--how to kick and throw a ball, how to have fun outdoors with other kids, how to just play a game for the fun of it without having there be a winner and a loser. So, I was really glad he did it, and hope that Lime can do something similar when he is old enough.

We also had a wonderful visit with Uncle Jared and Auntie Lauren, who traveled here from New York to see us. Their visit prompted us to do some nice things around town that we wouldn't have otherwise done, taking in a beer garden and a few other outdoor sights. Lemon and Lime were so thrilled to have a few new adults to lavish them with attention (and Pokemon cards and modeling clay).

While they were here, we also celebrated Lemon's 8th birthday. It is hard to believe that he is 8 now, a boy in full bloom, no longer even remotely a little kid. He is in fact enormous, growing so fast now that we decided to cut back to two cartons of formula a night, which I think is as little as he has ever gotten, even when he was just 2. So, I continue to hold out hope that one of these days he will become independent of the tube. He is continuing to eat a lot more, and to be much more willing to try new foods. He has made such tremendous progress since the feeding therapy days (how was that 4 years ago?). 

And now, both kids are back in school. In person, in classrooms, not in our basement. They haven't been to school since March of 2020, and Lime had never set foot in the elementary school where he "attended" kindergarten. They were both excited to go, especially Lime, who was eager for new friends and new experiences. So far, so good, and fingers crossed that with all the precautions our district is taking that they will be able to stay in person and stay healthy. The only minor hiccup was that Lemon's teacher forgot to give Lemon his enzymes before lunch on his first day. Honestly, if I had to single-handedly get 26 third graders to eat their lunches within a 15-minute period, I am sure that I wouldn't remember my own name at the end, never mind the medication that was thrust into my hand by some random hall monitor at 8am. She was very concerned and apologetic, and I'm sure they'll do much better this week as things settle into a routine. Luckily, for Lemon at least, missing an enzyme here or there really isn't an emergency, and I'm sure he didn't eat too much of his lunch anyhow, given that it usually takes him at least 15 minutes to settle down enough to even start eating.

So, we are, like all of you, hovering in this weird semi-pandemic state where things feel slightly more normal, but certainly not like they were in the Before Times. With two unvaccinated kids in the house, we are still being very selective and cautious about the things that we do. Luckily, the weather has continued to be good so we've been able to visit with lots of friends outdoors. Sadly, summer is winding down and who knows what this winter has in store for us. 

I hope all of you are keeping well, and that some day soon things will settle down enough for us to all see each other again.

August 2021: A month in

 Lemon has now been on Trikafta for over a month. Last week, I took him for his one-month follow up visit for the study of the effects of Trikafta in kids. We don't get to see the results for most of the tests, like the sweat test and bloodwork, because they are done at a centralized lab and the data is blinded. But, There are some things I get to see just due to the nature of the tests themselves. So, I can report to you that after a month on the drug, Lemon gained 2.5 pounds (without any Duocal in his tube feeds!) and best-ever results on his pulmonary function tests, with an FEV1 at 115% predicted, compared to his previous best of 100%. So, that is pretty amazing. It is always hard to tell with kids whether their lung function is actually improved or whether they just got better at doing the test, but either way I will take it. And I have to say, having watched Lemon do these tests for a couple of years now, I don't feel like his skill level has changed dramatically since say last month, so I think this is a real improvement and boy is it thrilling to see. We'll have to wait and see how things look at the 3-month follow-up in September.

One minor wrinkle is that we got a letter from our insurance company saying they will no longer pay for Lemon's formula or Duocal. I am guessing the addition of Trikafta prompted them to do a chart review, and they have decided that because the formula is available over-the-counter, it does not meet their conditions for coverage. We looked around but there aren't really any deals to be had on it so we are continuing to get it from the same home-health pharmacy as before, just paying for it with our own money now, and it is not cheap. It is sort of ironic that the insurance had no problem paying for the surgery to place the G-tube, and they will still pay for the bags, extensions, and the pump to deliver the formula. Just not the formula itself, which is ironic because all the other stuff will do Lemon exactly zero good unless there is formula to actually go into him. In any case, we are fortunate to be in a position where we can absorb the cost for now, and hope that as Trikafta continues to kick in and his appetite continues to improve, we can hopefully cut back on the amount of formula we are using.

The kids are continuing to enjoy their summer camps, despite being very good at identifying even the smallest imperfections to complain about. Lemon is doing a sports camp through the end of the summer, and I am hoping that despite himself he identifies at least one sport that he enjoys participating in. For his upcoming eighth (EIGHTH!!) birthday, we bought him a new bike which he really loves. He is a big accessories guy so he now also has a lock and a bell. He is so excited about it that he even wants to ride his bike to camp tomorrow for the first time, which warms my bike-commuting heart.

It is hard to believe how much of summer has gone by already. When summer started, it seemed like it would be infinitely long, and with so many childcare complexities to be worked out. Now, it's almost gone, and it feels like the beginning of the school year is right around the corner. Who knows what that will be like. But, I feel like we have done what we can to get the kids ready for it, and this strange version of early elementary school is the only one they will ever know. 

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.