Week 202: To the coast

We made it. I'm still not sure exactly how, but nonetheless, here we are, soaking up sun, sand, and salt water on an Atlantic beach. I won't go into detail on the journey, which went about as well as could be expected. I prefer to focus on the result--we are all here having a good time, spending time with family that we haven't seen in far too long. All of our CF related plans seem to have worked out fairly well so far. The travel IV pole made it intact, the case of formula did not explode while in our checked luggage, TSA did not make me unpack my giant carry-on bag of medical supplies, and manual chest PT is going fine. Lemon's cough has actually pretty much disappeared since we left Madison, which makes me think it really was just allergies. So, we brought Cayston with us as a back-up but I think unless something changes pretty dramatically we won't use it.

I'm going to leave this one brief tonight, since I am on vacation after all (and Lime has decided that in order to maximize the vacation experience, he will now wake up _before_ the birds), and will provide a more in-depth summary of the whole trip once we're looking at it in the rear view mirror. Until then, happy Independence Day too everyone!

Week 201: On the floor

Lots of moving parts this past week as we launched into summer. On Tuesday, we had Lemon's first session at "food school." He loved it--continues to view it way more positively than summer camp. Each session starts off with 15 minutes of "gross motor" play to get the kids into the right frame of mind to sit still and receive eating therapy. Miss Rachelle had a technical term for it, but I believe the vernacular is "getting the ya-yas out." In any case, Lemon loved all the various toys, the ball pit, the giant foam blocks, and so forth. And the strategy was successful, in that he was then willing to sit still at the table for almost 45 minutes and consider the various things that Miss Rachelle was asking him to do. He was game for most of it, and seems eager to go back again tomorrow for another session. The purpose of the first session was mainly for Miss Rachelle to make sure that she had found the approach that would be the best fit for Lemon, and she thinks she has, so tomorrow we should be getting some take-home materials so that we can work on reinforcing some of the concepts at home.

On Wednesday, we had our big clinic visit, which took approximately forever. We started off with height and weight--good news on those fronts: Lemon has gained 2lb and grown an inch in the last 3 months. Not bad for a guy who eats literally nothing! He's doing so well, in fact, that our nutritionist thought it would be OK for us to go down a bit on the tube feeds in order to get him hungrier as we get further into feeding therapy. Then, it was off to pulmonary function testing, where the goal is to exhale as forcefully as possible into the mouthpiece of a machine that measures how much air you are blowing out. This was Lemon's first time doing it, so the main idea was just to have him try it and get used to the machine. He liked watching the candles on the computer screen go out as he blew into the tube. Unfortunately, I was unable to convince him that making a loud roaring sound is not exactly the same thing as exhaling as hard as possible. Still, it's a start, and the whole point of starting now is so he can learn how to do the test correctly--as you can imagine, the actual readings that you get from the test with a kid this young are of dubious accuracy.

After the PFTs, we met with our nurse practitioner. Generally speaking, she thought Lemon was doing great. She got to take a good look at him, since we are going through a strong "clothing optional" phase of development at the moment and he spent the entirety of the visit zipping around the exam room wearing nothing but his undies. She didn't really have any answers about the right approach for the cough he has right now. It's not what I would consider his baseline (or, at least, what I used to consider his baseline), which is no cough at all. But, it's certainly not what he usually is when he is sick, which is quite a lot of cough, and coughing in his sleep, which he's not doing at all right now. We did talk about whether or not we should just adopt the "usual" schedule for Cayston, which is 28 days on, 28 days off--our nurse practitioner didn't really have any guidance on that, she said I should do what I thought was best (because I'm the clinician?). Originally, it wasn't our plan to be on a schedule, but rather just to use Cayston as our intervention when he was sick. In practice, though, that has translated into the 28 on/28 off schedule almost exactly. By that schedule, we were on in March, off in April, on in May, and off in June. So, the big question Papa Bear and I have to decide in the next couple of days is whether we are going to be "on" for July.

Of course, the timing is really lousy because we're going on vacation next week, but on the other hand nothing about CF is convenient and I do feel like it is important to intervene aggressively now so that we preserve as much lung function as we possibly can. But on the _other_ other hand if there isn't a bacterial infection going on at the moment, there wouldn't really be much point to doing a month of inhaled antibiotics. We did do a throat culture at this visit so I'm waiting on the results from that--I suppose if we see anything besides normal flora there, that would be an indicator that would push me towards biting the bullet and doing the Cayston. We shall see. I'm not even going to get into the number of phone calls it took us to get our Cayston prescription refilled this week so that we'll be ready to do the Cayston if we decide to go that route--but it was a truly ludicrous number, an order of magnitude larger than the "1" you might expect given that this was a refill.

The other thing we did at this clinic visit was have some blood drawn to repeat Lemon's liver tests. As you may remember, we had them done late this past fall, when he had a PICC in, and the results were sky-high, presumably because he was really sick at the time. We repeated them a couple of months ago, and the levels were still high, but way less high than before. On Wednesday, the levels were ever so slightly less high than that, but still outside the normal range. So, our current plan of action there is to recheck again next month, and if they're still high after that, schedule a GI consult to get some thoughts. Since Lemon has never had liver tests inside the normal range in his life, I find it hard to imagine that next month will be that magic first time where they come back normal, but we shall see.

In the mean time, we are very excited to be gearing up for our summer vacation to the coast. It will be Lime's first time seeing the ocean, and I suspect he will love it. I've started the inevitable process of making mental lists of things, so many things, that we will need to bring with us. Packing is off to a promising start though, in so far as the portable IV pole that I ordered does appear to be small enough to fit in our largest suitcase. Filing that one under "things I never thought I would have to worry about when planning a trip."

Week 200: A peach

On Wednesday of this week, I slipped out of Madison for a 72 hour trip to Boston to revisit my pre-kid, pre-Madison life and some of my oldest and dearest friends. Life here is all-consuming, and I was surprised how easy it was to shed it for those few days and become, however briefly, my younger care-free self again. I can't thank Papa Bear enough for the opportunity to go. 72 hours isn't a lot of time for catching up with a lifetime's worth of friends, but it's enough to give a little oxygen to all those flickering flames and keep them burning until the next visit.

Meanwhile, back in real life, Lemon continues to cough. Just a little, and not at night. So, we still have no idea what's going on. Everyone else seems to be more or less over the summer cold that kicked off this round of everyone's favorite CF game, "Cold, allergies, or infection?" So, "cold" is seeming less likely, which leaves us with "allergies" or "infection." I'm not especially eager to leap into another round of Cayston, especially since if we start now, we will be doing Cayston while we're away on our big family summer vacation. Of course, if that's what it takes we'll do it, but I would like to be a little more confident that we aren't fighting allergies with antibiotics before we start. We have a clinic visit coming up on Wednesday (along with our first-ever pulmonary function tests!), so I'll see what our nurse practitioner thinks at that point.

Today, Lemon had his first day of summer camp. I'm glad to report that, in spite of the less-than-impressive phone conversation that I had with one of the camp staff last week, everything seems to have gone fine. I made up a printed sheet with all of Lemon's key medical instructions on it, gave a copy to his main teacher, and put extras in plastic bags in his lunch box and backpack. Lemon handled being dropped off at a new place with strangers very well--he was a little teary when I left, but report is that he quickly pulled himself together and had a great day. His teacher said that he was "a peach," and also noted (without my having said a word on the subject) that "he doesn't eat much." At least the camp fulfilled its main goal, which is that he was EXHAUSTED when he got home and promptly collapsed and slept for 2.5 hours. Victory!

Speaking of eating, tomorrow Lemon has the first of his weekly feeding therapy sessions. When we were setting this up, I had hoped to capitalize on what then seemed to be a bit of a trend towards increased willingness to eat. Of course, now that we're ready to start, the pendulum has swung firmly back in the other direction and we are once again in the land of approximately zero oral intake. Still, I'm hopeful that we'll make some progress. I've already heard Lemon imitating some of the language that he heard during his feeding evaluation, so intellectually he seems to be game. With any luck, his therapist will be able to convert some of that intellectual energy into practical results!

Today marked the official beginning of my next round of marathon training. I went out for my evening run as the last few summer rain showers were moving through, and they left some serious beauty in their wake--seems like an auspicious start!

Week 199: Heating up

Just because there weren't enough ambiguous coughs, sneezes, and other respiratory sounds in my house last week, all 3 of the males in residence decided to come down with a full-blown summer cold within 36 hours of my completing last week's post. We briefly talked about starting Cayston again, since things were sounding decidedly not good for a day or two, but we decided to try to ride it out instead, and I think that was the right call. At least I felt like I had two canaries in the coal mine this time: if Lime and Papa Bear will still symptomatic, there was no reason to think that Lemon's symptoms were caused by anything more than the cold. And, knock on wood, that appears to have been the case. All three of the boys seem to be more or less back to normal again, and just in time, since I'm not sure how many more consecutive weeks of respiratory symptoms I can handle.





Fortunately for my nerves, summer seems to be on the way. Lemon finished 3-year old preschool this past week, and summer camp starts next week. I'm interested to see how he responds to the challenge of being in a new place, with new teachers and new kids. Today I realized that I had never heard back from the program leader at the camp that I'd contacted about doing enzymes and whatnot, so I sent a follow-up email, and within a few hours he called me and said, "So, you have some medical concerns?" I rattled off something about medication with every meal, and oh by the way he has a G-tube and if it falls out it's urgent but not emergent, etc etc. "Uh, OK. Do you have this written down somewhere?" Luckily, I still have the sheet I made for Lemon's preschool teachers. So, hopefully everything will be fine. One good thing is that Lemon actually can advocate for himself now. He knows that he needs to take his enzymes, and will speak up if he doesn't see them. He also knows about his G-tube, and will say something if he notices anything wrong with it ("if" being the operative word here).



Summer on the way means that summer travel is fast upon us. It's hard to believe that our big family trip to the beach is just a few weeks away. Our plan is still to leave the vest at home, and do manual chest PT while we're away. In preparation for this, Lemon and I have started "practicing" every day, meaning that I do one full round of manual PT with him around mid-day. He resisted a little at first, especially since we had to figure out what positions worked best (he's grown ever so slightly since we last did this regularly, about 1.5 years ago). But, I think we've got it now and although he does protest a bit (because he is 3 and therefore must protest everything), I think he actually kind of enjoys it since it is 20 minutes of my completely undivided attention.

Summer is also birthday season in our house. I can scarcely believe it, but little Lime will be two years old in less than a month. I'm not ready. Just a few extra months of 20 months old, that's all I ask!

Week 198: Controlled experiment

As I've mentioned a couple of times so far this spring, one of the most frustrating things about dealing with CF is that you never know quite what it is that you are battling at any given moment. Way back when, let's say in late April, we were pretty sure Lemon had a cold. We increased PT, and after a while the cold mostly went away, but not completely. Was it seasonal allergies catching us at the end of a cold? Was it a bacterial infection that took advantage of the nice environment created by the cold? Was it just allergies start to finish? No way to know. But, on the advice of our clinic, we went ahead and started Cayston, and things got a bit better. So, it could be that there were some bacteria, and Cayston killed them, or it could be that it was just some residual junk from the cold and it would have cleared up, Cayston or no. Or, a bad patch of allergies that cleared up as whatever was blooming stopped blooming. We ended up doing 3 weeks of Cayston because the symptoms (whatever their cause) were still lingering after two weeks. After 3 weeks, things were 99% better. So we stopped Cayston.

Then, Lemon started coughing again after a couple of days. What now? A resurgence of bacteria that weren't killed by Cayston because we should have done the full month? Or more allergy symptoms brought on by the continuous pollen onslaught that is a Wisconsin spring? Another cold? Something about the cough reminded me about using Flovent to control inflammation in the lung. We still had the inhaler in the house, so we started it again, and within a day or two, the cough was pretty much gone. So, it seemed likely that it was allergies, not bacteria, at least at this point. Then, we had two back-to-back warm days, days that were warm enough that leaving Lemon to sleep in his room with the windows shut and the air purifier running was just not possible. So, with trepidation, we turned off the air purifier and opened the window. Note to future self who considers this option: this is not a good idea.

48 hours of sleeping with the window open and the air purifier off and we had itchy eyes, runny nose, sneezing, and coughing. Having done as close to a controlled experiment as one can do on one's own child, we sealed up the house, turned on the air conditioning, and turned on the air purifier again. Tremendous improvement. I think I learned two main lessons from this whole exercise. The first is that Lemon's allergies seem worse this year than last. They are no joke, and we need to be really vigilant about having the air purifier running at all times when he is asleep. And if that means running the air conditioning more often than I would like (which, honestly, is about never), so be it. Second is that Flonase doesn't seem to do enough for him when it comes to allergies, he seems to have allergy symptoms that are really in his lungs, not just in his nose and eyes, so when we think about increasing something to combat allergies, we should be thinking more about Flovent and less about Flonase (and we certainly should not be thinking about both, since that results in bloody noses).

In other news, the CF Foundation liked the first piece that I wrote for them (on the different classes of mutations that can cause CF), so at some unspecified point in the future, that content will appear on their website. I'll be sure to point you to it when it does. They've also asked me to develop two additional pieces for them, which I'll get started on over the coming weeks. I'm very excited that all of this is finally happening--it's been my goal to make this kind of contribution to the CF world since Lemon was diagnosed. There's also been some vague mention of the idea that I could interview CF researchers about their studies, which would be really fantastic--stay tuned!

Week 197: Duck to water

On Wednesday, we at long last had Lemon's feeding therapy evaluation. It had two parts. First, Lemon and I went into a room and had a "picnic" of foods that I'd brought from home, while two therapists watched through a pane of one-way glass. At the outset, I should say that Lemon was not fooled by the one-way glass for an instant. Pretty much as soon as we sat down, he looked at the "mirror" and said, "Is that a window? Is someone back there? I think there's another room over there." But, he wasn't really bothered by it, and ate a very typical snack: enzymes and one slice of orange pepper.

Next, one therapist went into the room with Lemon while I sat behind the one-way glass with the other therapist so that she could learn more about Lemon and his situation. Lemon had an absolute blast, because they were doing all kinds of silly things--blowing bubbles through a straw, making faces in a mirror, drawing on the mirror with ranch salad dressing, etc. He had the undivided attention of an engaging grown-up for about 45 minutes, and was pretty much in his element. Of course, the point of all this was to actually evaluate what his issues with eating actually are, but Lemon certainly would never have guessed that anything serious was going on.

What I learned is that, as I thought, there is nothing mechanically wrong that should prevent Lemon from eating. I'll get a more formal evaluation soon, but the things the therapist hit on were that Lemon doesn't feel like he's "good at" eating, and he doesn't like to do things he's not good at (true!). Also, since he's been tube fed, he really doesn't quite have the connections between feeling hungry, eating something, and feeling full worked out. It sounds like the broad strokes plan is to practice various things so that Lemon feels more confident about eating, and also to rework our tube feeding schedule/volumes somewhat to help Lemon establish the connection between hunger/eating/not feeling hungry anymore. Luckily we have a clinic visit coming up in a couple of weeks so that we can talk to our dietitian and get her input on how we can adjust the tube feedings without losing ground on nutrition.

In other news, Lemon learned how to ride a bike this weekend. Those of you who know me from my cycling past, will appreciate what a thrill this was for me. It really was amazing to watch. Two weeks ago, I took him out with his new two-wheeler and he could only ride at all if I held onto the seat the whole time and ran along behind him. Then, we were busy for a couple of weekends in a row and I didn't have the chance to practice with him. On Saturday, he hopped on the bike and almost immediately could ride short distances without help:

Then, today, after only one more day of practicing:

Week 196: Not a sprint

If CF prepares you for anything, it's being unafraid of the dentist. Lemon was very excited to go for his cleaning because there are toys in the waiting room, and prizes after. He walked right in to the exam room, and got on the table. He very capably advocated for which tool he wanted the hygienist to use to clean his teeth (traditional metal, please, none of this high-pressure water business), and told her when he needed breaks. It turns out eating food actually helps keep your teeth clean, so Lemon had some extra buildup on his teeth due to his preference for not eating, but otherwise everything was fine.

If only CF prepared one for cows. The day after our highly successful dentist visit, Lemon's school had a field trip to a local dairy farm. It started off well enough. Lemon was very excited about riding on the school bus. He was even moderately (if briefly) excited about seeing the baby calves. He was emphatically not interested in feeding any of them from a bottle. He was even more emphatically uninterested in the barn where the cows were hanging out. So emphatically, in fact, that we had to leave the barn and stand outside were we could see the more interesting aspects of the farm (tractors, dump trucks full of hay, front end loader, ATV). The milking parlor was also pretty much a non-starter. He did eat a piece of string cheese at the end of the visit, though, which was reassuring--I was worried that the whole experience might have put him off dairy permanently.

















On Saturday night, after the kids were asleep, I slipped out of the house and headed down to Rockford, IL, where I ran a marathon early Sunday morning. It was a great day for it and I set a new personal record, 4:07. I still aspire to get under 4:00 some day, but I put everything I had into the race on Sunday and I'm proud of the result. Honestly I'm looking forward to running a little less for the next couple of weeks and spending time on some much-needed upkeep around the house and the yard. But, don't worry, the next race is already on the schedule for October, so I won't be slacking off for too long.

At long last on Wednesday morning I'll be taking Lemon for his feeding evaluation. Eating has definitely been a bit up and down the last few weeks. Sometimes, he'll have a stretch of days where he eats "so much" (multiple bites multiple times a day) where I wonder if we still need the therapy. And then he'll have other days or weeks where I'm reminded that yes, we really do still need this. I think we're at the point where he's getting maybe 10-15% of his daily calories by mouth, which certainly feels like a big jump from the 0-5% we were getting for some of the winter, but it's not nearly at our goal of 50%.

After some discussion, we've decided to keep going with Cayston for one more week. We'd been hoping to stop after 14 days, but we knew we could only do that if Lemon was really 100% at baseline. And, he's just not. It's impossible to tell if it's still an infection, or if it's just allergies now, or what, which is frustrating. He's definitely much closer to baseline than when we started the Cayston, so I do think it was the right thing to do it. I just wish I knew when it would be OK to stop--with the high pollen counts right now, Lemon could be coughing from allergies for weeks (if past experience is any indication). Treatment is still such an inexact science. So, we'll just carry on for another week and then use our best judgement and hope that we got it right!