Week 271: Here, there, everywhere

Fall travel madness is in full swing. Papa Bear and I are pinging around the country at an absurd pace. For instance, I got back from a trip to San Diego on Saturday morning (I am definitely getting to be too old to take red-eye flights) and am leaving this afternoon for Denver, hence the early blog post. The weekend was just barely long enough to go to a birthday party, and refresh the supply of food and clean clothes such that everyone will survive until I return.

Luckily, it seems like 10 days of Cayston was enough, and Lemon is in pretty good shape. That is, he only came down with a new cold yesterday, so odds are reasonably good that he will hold it together through my trip to Denver, and also my trip to Indianapolis, for which I leave on Friday morning (absurd). Fortunately, after I get back from Indianapolis, I will actually remain in one spot for several consecutive weeks and will have some ability to cope with any arising needs for contingency plans.

The most important news of the week actually comes to us from the New England Journal of Medicine, which this week published two back-to-back papers about Vertex's new triple-drug cocktails. These are the medicines that would treat the underlying cause of CF in people like Lemon, and the results from the Phase II trials look incredibly promising. You can see the abstracts of the articles here and here. Basically, after just one month on the drug, patients had substantial improvements in lung function.

It was always my dream that by the time Lemon was in elementary school, there would be a medicine that would treat the underlying cause of CF for people like him. I've heard rumblings in the CF community that Vertex might take one or both of these compounds for FDA approval as early as mid-2019 for adults, and then (hopefully) the next step would be for them to begin enrolling a pediatric trial. I'm pretty committed to having Lemon be a part of that trial, so fingers crossed that there is a study site that is close enough to us to make it feasible.

Thanks so much to all of you who have donated to our CF climbs, rides, and walks over the past few years--these amazing results are a direct result of your generosity, and give us so much hope for Lemon's future. More tomorrows!

Week 270: Even Though You're Old

On the way home from school today, Lemon informed me, "Ma, I love you, even though you're old." This leads me to believe that so far, my 40's have been a success.

In terms of other successes, Facebook reminded me that today is actually an important day in our history: two years ago today, Lemon was discharged from the hospital. And we haven't been back!!! Memory is funny, some things from two years ago I have a hard time remembering. But the hospitalizations? No. Those are seared into my mind. And I can't believe we've been out for two years. What a gift/our hard work has gotten us somewhere.

In terms of where we stand now, things seem relatively stable today (I know better than to speak in generalities that cover anything besides the present moment). We are on day 9 of Cayston, and Lemon's cough is minimal. I can't say that he's at true baseline, which is no cough, but I do feel reasonably comfortable saying that he is at "winter baseline," which is a little sniffle and a couple of coughs a day. We added in Flovent again, just to see if tamping down any inflammation that had arisen during a straight month of being sick could help at all. The answer on that so far is, who knows?

What I can say is that since we started Cayston, Lemon has packed back on 2.5 of the 5lb that he lost. His appetite has been good and I may or may not be sneaking in 8 scoops of DuoCal into the overnight feed instead of our prescribed 6. Since we're at "winter baseline" and we've got weight going in the right direction again, we're planning to stop the Cayston after tomorrow (10 days). I think the shortest we've done previously was 14 days, although our nurse practitioner did tell us that 10 would be reasonable if he seemed well at 10 days. All this is off-label anyhow so there aren't any firm rules. In this case, we have some pretty strong external pressure to stop at 10 days, which is that I'm going to be in 3 states other than Wisconsin in the next two weeks, and with me being out of town it is pretty tough to maintain the 3 times daily dosing that Cayston requires. So, cross your fingers for us, everyone.

In non-health news, we had a great visit this weekend with Uncle Jared and Auntie Lauren. We were having so much fun, in fact, that it appears that I completely neglected to take any pictures with them. It's a pretty kinetic situation when they're in the same place as the kids. They did bring the boys a couple of new toys, including a very popular bus that has been the subject of many heated ownership debates. I would tell you more about it, but I'll leave it to Lemon (and his teacher, who advised that kids should be encouraged to read words that they care about).

Week 269: Robot Stuck in Flower

We have just returned from Academic Success Night at Lemon's school, where we learned ways to support the teacher's efforts in the classroom in terms of teaching the kids to read. We learned that a) one of the kids who sits at Lemon's table at school is a girl, and b) like most girls, she appears to be a genius, in that she can draw a face with recognizable parts, and write her own name. We also learned about the book that the entire school is reading together, which is called The Wishing Tree. As part of reading that book, each kid was supposed to dictate a wish to their teacher, who wrote it on a strip of ribbon to be tied to a tree at school. Genius girl put down "To write." Lemon's wish, meanwhile, was "Robot stuck in flower." His teacher assures us that he is doing really well.

We also learned that we are supposed to be doing 20 minutes of reading per day with Lemon. So, our next big challenge is to somehow grow our day by 20 minutes. It may not sound like much time, but really, where is it going to come from? Lemon is only awake from 6am to 7pm, and he desperately needs all that sleep. He spends at least an hour every day doing therapy, and is at school from 8:30-3:30. And by the way, he doesn't really want to spend his time before dinner sitting still given that he's been cooped up in the classroom at school all day. It's a puzzle.

 On the health front, we finally decided that it was time for a round of Cayston. We were still hopeful late last week that we could avoid it, but then on Friday, after a few days of getting better, things started to get worse again. With Lemon being down 5 lb and coughing continuously for almost two weeks, we decided that it was time. Also, looking back over the past year or so, I can see that we end up doing a round of Cayston about every 3 months, and given that our last round was in June, we are about due. We started it on Sunday morning and I definitely feel like it was the right decision, the cough isn't gone yet but it's dried up almost completely and the threat of puking seems to have receded at least for now.

Of course, that brings us to our latest challenge, which is that Cayston is an antibiotic that has to be delivered using a special nebulizer, 3 times per day. We do once first thing in the morning, and once before dinner. And, once mid-day, of course, which is during school hours. So, I think the plan is that I am going to run over to the elementary school at lunch time and hustle Lemon off to a quiet spot somewhere to pump his lungs full of antibiotics, and then whisk myself and the equipment back home. Every day. For at least two weeks. Whatever it takes. At least the nebulizer is fast, and I knew what to write on my wish ribbon.

Week 268: Forty

You only get to have one last week of your 30's, so might as well make it a good one. Or, so the forces that orchestrate my life seem to feel. So, where we left off last Monday was that Lemon had come through one of those sort of 36-hour high fever and vomiting scenarios fairly intact, with some cough just due to generally being sick but not really a respiratory thing. All was well for a solid, I don't know, 12 hours thereafter.











Then, Lime brought a proper respiratory virus home from preschool. Both kids were pretty miserable. I mean even Lime coughed until he puked on at least one occasion, and was really not himself for several days. Papa Bear got it as well, and was also in an advanced state of Man Cold for several days. It hit poor Lemon really hard though. Honestly, this was the worst illness we've gone through in quite some time, probably since last winter I would guess. The shear intensity and frequency of the cough had to be seen to be believed. We backed down on tube feeds. Still puking. Backed down more. Still puking. Coughing and crying at night because he was so exhausted and just wanted to sleep. Coughing to the point of puking at the dinner table and then still wanting to eat because he was so hungry from not getting any formula for days on end. We were pretty much at the end of our chains. After an especially bad night on Thursday (like, really bad), it seemed totally clear that we needed to start Cayston first thing in the morning, so I got out all the stuff and sterilized it at 11pm so we'd be ready to go Friday morning.

And then, Friday morning, things seemed better. Not totally better, but just better enough that we decided to wait until mid-day, just to see. At mid-day, things still seemed incrementally better, so we decided to wait until evening. And then we decided to wait and see how the night went. Less coughing, no puking. Since then, little by little, we've crept our way almost all the way back to baseline. It's quite astonishing, really. I don't want to get ahead of myself, but it does seem like perhaps we made it through this one without antibiotics also. And Lemon only lost 5lb in the process. I'm trying not to think about how that took us an entire summer to put on, and it's gone and it's only the beginning of October.

Phew. I'm drained just writing about it. As a parent, all I want to do in those situations is trade places with Lemon. Let me do this for you, let me go through this so you don't have to. And that's just not an option. All I can do is try my best to help him and hope that I'm doing the right things, making the right decisions.

Anyhow. It's my birthday today. Papa Bear got me a lovely new running watch and a bottle of top-shelf bourbon (it's good to have a spouse who understands your tastes). The kids got me many wet leaves, each beautiful in its own unique way. It's been a good decade, full of unexpected twists and turns. When I started this decade, I'd just met a nice-seeming guy at a conference, I lived in Massachusetts, kids were something that a few of my friends had, and CF was something that I'd read about once in a genetics class. Curious to see what the next decade has in store.

Week 267: Full Tilt

Full tilt fall continues apace. This past week, I went to St Paul, MN for work for a few days, leaving Lemon and Lime in Papa Bear's capable hands. It seems that all went well on both ends. I had a great seeing my coworkers, who turn out to be actual physical beings, not just disembodied voices that I hear on the phone every day. The boys and Papa Bear ate Domino's Pizza, watched Star Wars, and did whatever other male bonding things there are to do when Mama's away.

Of course, when I returned on Friday and picked Lemon up from school, the first thing I noticed was that he was coughing again. Fantastic. He seemed perky enough, though, so we thought we'd just go with our standby strategy of trying to shake it out of him with extra therapy over the weekend. Friday night was fine, Saturday morning was fine, and then Saturday around mid-day he just collapsed, took a 3-hour nap, and woke up with a temperature of 101F. Yikes. Lots more coughing, and of course some puking Saturday night. Fun times. Sunday was somewhat better from a cough perspective, and by Sunday evening the fever had quit as well. Today he still has a little bit of a cough but no fever, and seems back to normal operating parameters otherwise. In an abundance of caution, since I did just hear a cough or two, I went into his room to turn down the speed on his feeding pump. Formula only counts if it stays on the inside, after all.


In reflecting over this recent series of little illnesses, here is what I think. Lemon gets a cough no matter what sort of virus he's picked up. Things that might just cause a random fever or malaise or stomach upset in everyone else give him a cough. Every time. Obviously it's still important to do the extra therapy to keep his lungs clear so that he doesn't develop a bacterial infection, but I need to stop thinking of all of these things like they're really respiratory per se. Once the virus is cleared and the fever is gone, the cough dries up. I guess that's comforting? I still need my magic dipstick test so I can tell right away what's going on, rather than waiting around for a few days to see if we're in "random virus" territory or someplace more sinister.

One little friendly reminder, speaking of sinister--flu season is fast upon us. I took Lime with me to Walgreens this weekend to do an errand so I got my flu shot while I was there. If you find yourself at your local purveyor if immunizations in the coming weeks, please get your flu shot too. It's good for you and those around you, especially the more delicate folks that you may come into contact with!