Week 218: The scamper

I am pleased to report that somehow, we survived a week without Papa Bear. By somehow, I mainly mean that I did nothing but work and take care of the kids and went to bed at 8:30 every night. I may also have eaten cereal for dinner every night, it's kind of a blur at this point. But, every treatment got done, every diaper got changed, and the trash got taken out. So, victory.

We did do one extra fun thing this week, just to keep things interesting. As long-time readers will

recall, we originally had two cats, Daphne and Denzel. Sadly, Denzel passed away about 18 months ago, and my life has just not been complete without an orange boy cat in it. For whatever reason (sleep deprivation?) I thought that this would be a good time to bring a couple of little scamps into our household, so on Wednesday I picked up two little rescue kittens to come join the circus here in our house. The kids and Papa Bear had picked out the name Donut for one of the cats, so naturally I decided the other one (my sweet orange boy) should be named Duncan. They are pure joy. And Lemon is so in love with them. I suspect they will grow up to be VERY tolerant cats, since he will, without warning, scoop them up by their mid-sections and tote them around, put them down his little slide, have them "jump" on his trampoline. Daphne is still getting used to the idea that there are some new residents in the house, but I imagine she'll come around eventually. She did eventually accept the kids, after all!

In other news, some of you may remember that a while ago I finally managed to worm my way into the Cystic Fibrosis Foundation to do some medical writing for them. I'm very pleased to announce that some of my content has now made it onto their website. Feeling the need to brush up on some basics about the CFTR protein? Maybe you're interested the different types of CFTR mutations? How about CFTR modulators? Check out these all-new sections of CFF's website. I'm glad to say that the Foundation has asked me to keep going and develop some content for them about "New Frontiers in CF Research," so watch this space.








On the health front, we are done with two weeks of Cayston. If you had asked me a couple of days ago, I would have told you that we would be stopping Cayston after 2 weeks, because Lemon seemed really well. Unfortunately, Lemon, Lime, and I all caught some other new cold, hard on the heels of whatever the kids had before. So, Lemon is once again coughing up a storm. I'm quite convinced that it's just the new virus and not anything bacterial, but it seems pretty dumb to take him off the antibiotic when he's actively sick. After making up a little bit of precious ground in the past week, we're once again cutting back on tube feeds, dealing with puking, etc. This does make me feel better about all the boluses I pumped into him last week when he was well, though--we need those precious ounces of body fat to help him fight off this new cold, and hopefully we'll only get pushed back to where we started from, weight-wise, and not further into the hole.

We did cross an important milestone this week: it has now been one full year since Lemon was discharged from his most recent hospitalization. We are definitely in a stronger position than we were a year a go, and we've learned some important lessons about managing Lemon's health that have helped us to achieve our hospital-free year. I think the key lessons for us have been the importance of nutrition, hydration, and inhaled antibiotics. A year ago, we were so focused on the respiratory symptoms whenever Lemon got sick, and were not as attuned to the nutrition/hydration angle. Last year's hospitalization really showed us what a difference even just staying hydrated during an illness can make for him. We also got away from our long-standing intervention plan of oral antibiotics, in favor of inhaled. I won't lie, inhaled antibiotics are a pain. Three treatments a day gets old for everyone really fast. I long for the simplicity of shoving a few mL of a liquid medicine through a G-tube twice a day. But, much as I long for it, that plan never worked for us. And if inhaled antibiotics ever seem like a big deal, I just remind myself what doing home IV's is like, and then I remember that inhaled isn't so bad. Whatever this winter brings, we are more prepared to face it than ever before, and this hospital-free year is an accomplishment that we can all be proud of.

Week 217: Off to the races

Full speed ahead as always around here. On Monday night, within just a couple of hours of my posting the blog, Lemon's cough took a marked turn for the worse, so we decided we had no choice but to start Cayston on Tuesday morning. It was definitely the right call, since he started perking up almost immediately, and I'm hoping that we'll be able to get away with a two-week course instead of a month (knock on wood). Fitting in 3 treatments a day with school and work and everything else is challenging to say the least. It will definitely be a relief for everyone to get back to twice a day.

On Friday, Lemon had his follow-up with the gastroenterologist to talk about his liver. The first thing we learned at that appointment is that Lemon has lost a pound and a half relative to his weight in JUNE. That's 4 months of not gaining a thing and instead losing ground. Partly because of when we tried cutting back on tube feeds when we began food school, and partly from him being at least somewhat sick pretty much non-stop since school started, which forces us to keep the overnight feed volumes low. Now that we have this latest cold more or less under control, we are in full-court-press mode to try and get weight back on. That means 3.5 cartons of formula at night plus 100mL bolus feeds morning and night. It's a lot of formula, syringes, and fuss, but we've got to make up some ground while we can, since winter hasn't even started yet.

The next thing we learned from the GI doc was that he wanted us to start on a new medication, usrodiol, that may slow down or prevent damage to Lemon's liver. We started the ursodiol today, and we'll re-run the liver panel when we do his annual labs in December to see if it's having any effect. It better be, because a one month supply costs us $90 out of pocket (for a generic medication!). 

When we got back from the appointment, I dashed over to our local Enterprise and picked up a rental car, and on Saturday morning I drove down to Des Moines, where I met up with an old buddy from Boston. We bummed around Des Moines on Saturday afternoon and were very favorably impressed by it. On Sunday, we ran the Des Moines marathon together, capping off the 2017 running season with a personal best for me, 4:05. I didn't quite manage to break the 4:00 barrier this time around, but I think it was by far the strongest marathon I've ever run, and I have high hopes for the 2018 season. In the mean time, I'm looking forward to the off season, where I can run a little less and spend a little more time on other pursuits. Many thanks to Papa Bear, Nona, and the rest of our crew for taking such good care of the maniacs while I was away!

I'll definitely kick off the off-season in style this week, because no sooner did I return from Des Moines this morning then Papa Bear took off for a week in Greece for a conference and some sight-seeing. I'm sure I'll be doing plenty of running around over the next week, although most of it will be chasing after the dream team, rather than endless laps of the neighborhoods of Madison!

Week 216: Mountain high

This week, I briefly escaped the midwest for a work trip to Denver. You will all be pleased to know (not that I had any doubt) that the boys managed to survive on their own without me for a few days. Papa Bear did an admirable job holding down the fort by himself, including carrying out 3 treatments a day for Lemon, who (along with Lime) has had quite a cough for the past 10 days or so. I, meanwhile, had two of the most amazing nights of sleep that I've had within recent memory, waking up not at all during the night and sleeping until the deluxe hour of 5 a.m. mountain time (aka 6 a.m. central time) every morning. I think some work was done as well, but I mainly remember those nights of sleep. Amazing stuff, I tell you.









We have been going back and forth all week about whether to start Lemon on a round of Cayston. He has had this pretty terrible sounding productive cough for over a week, after all--a fresh cold of some kind after he kicked the first cold of the school year so well. Fortunately, we have Lime to serve as our canary in the coal mine. Our thinking is that if Lime is also still coughing, and if both of them still seem relatively perky, there is no reason to leap to the conclusion that there is something bacterial going on in Lemon's lungs. So far, we haven't seen any of the other signs (puking, fever, very low energy) that we typically see when Lemon needs a round of antibiotics, and we're continuing to wait. It seemed like both kids went through the worst of it a couple of days ago, and have both been getting incrementally better the last 48 hours or so. Fingers crossed. We could really use a break from all these colds to work on some weight gain!

Progress with eating continues to be, shall we say, mixed. Lemon's therapist at food school continues to be very enthusiastic about his progress, which I suppose is her job. I have to say that we are not seeing overwhelming progress. I think we are in a marginally better position than we were at the beginning of the summer. But, we're still not anywhere close to my seemingly conservative goal of getting Lemon to eat say 200 calories a day by mouth. Today, I asked the therapist what she thought of one of our other goals, which is getting Lemon to swallow pills. I caught what I believe is called a "microexpression" that rushed across her face before she composed herself and said something about how that was an interesting idea. I think we're going to try anyway. I had been holding back because I was hoping that Lemon would make real inroads on learning how to eat, but I feel like as long as we're going to sit in a plateau with regard to that skill, maybe he could at least try to learn a different oral skill in  the meantime. The nutritionist at our clinic was very supportive of the idea, and I think it would be helpful to Lemon (and to us!) in the long term, because he's started to fight us on taking his enzymes in applesauce, so we really need another approach.

All that aside, we had a fun time celebrating my birthday yesterday. Nona made some delicious cakes, and we went on our annual apple and pumpkin picking adventure. I also got a great birthday gift, a steam mop/vacuum. It probably says something about my advanced age (or the number of people in my house who throw/drop food on the floor every day, either preceded or followed by rolling in the sandbox and then running into the house) that this is one of the greatest birthday gifts ever. Thanks to everyone who sent birthday wishes from near and far, it was wonderful to hear from all of you!

Week 215: In the cold

It is so hard to sit down and write a blog post on a night like this, when, to top off all the other various crises that are going on in the world right now, a horrific mass shooting has taken place in Las Vegas. The infinitesimally small daily struggles of our household barely feel worth writing about. Honestly, they become hard for me to concentrate on, never mind all of you out there on the internet who aren't daily participants in these little dramas. And yet, despite the demands on my attention from the outside world, I know that I can't take my eye off the ball here even for a moment, because the nature of CF is such that if you coast for even a day, you will pay the price. So, I once again acknowledge that I am writing this blog as a reasonably well-informed citizen of our country and of the world, but that I will remain true to its focus on the life of one family living with CF.







We had a clinic visit on Wednesday, which went well all things considered. Lemon did very well with his pulmonary function tests. He was able to produce consistent results this time, and was much better at controlling his breath as he blew into the machine. He just needs to figure out how to breathe out for longer, and then we will be able to get some real numbers on how his lungs are doing. Of course, the fact that we have to go to the pulmonary lab at the beginning of every visit just makes these check-ups even longer. I had to suppress a laugh when our nurse practitioner (who we didn't even see until we'd been in the clinic for over two hours) told us that our _next_ visit is going to be "epic" because at that one we'll have to do a chest x-ray and a blood draw also. Trust me, two hours in an exam room with Lemon is already a fairly epic experience.





We are still struggling with weight gain. We'd backed down on tube feeding over the summer in order to give Lemon space to be hungry, to maximize the chances that food school would actually get him to start eating things by mouth. Instead he lost weight, so eventually in late July or early August we decided to go back up to his usual tube feeding volume. Then school started and he got a cold, so we had to back down on the volume again to avoid all the vomiting. Then he managed to get over that cold without any antibiotics (!!!!) so we went back up on the volume. That got us to Wednesday, where we'd managed to get his weight back up to just under where he was in June. Then, of course, having been well for a week or so, both kids came down with a second cold, so now we're back down on volume again. Sigh.

On the plus side, we once again have a working refrigerator, which feels like nothing short of a miracle. Lemon is fairly obsessed with the fact that water and ice come out of the front of it. And, one has to imagine that some day, somehow, he will become as interested in its contents as his younger brother is.

I'm leaving on a quick trip to Denver tomorrow morning, so we shall see how the males of the household do for 72 hours unsupervised. Dominoes Pizza may be called upon. Luckily Lime is now sleeping as late as 4:45 on a regular basis, so Papa Bear should be just fine. By which I mean, send coffee and forgive him if he seems sort of dazed. He should be back to normal operating parameters again by Monday I would think.

Week 214: Conked out

I should have known better than to title last week's post "The unexpected." It just invites things. I suppose I could just as well have entitled this week's post "The foreshadowed," but my maternal grandmother was particularly partial to the phrase "conked out," and it's equally fitting.

So, here's the foreshadowing. Anyone who knows Lime well knows that one of the great sources of joy in his little life is the consumption of an afternoon Popsicle or three while sitting on the chairs in our front yard, watching various vehicles roll by our house. Because of his inordinate fondness for Popsicles, we noticed a disquieting trend. On some days, the Popsicles were not adequately frozen. At first, we attributed this to the fact that there are lots of people in and out of our house every day, some of whom may not be fastidious about closing the refrigerator and freezer. This list may include Lime himself, who will, in a desperate attempt to reach his Popsicles, pull back on the freezer door with such vigor and abandon that he ends up falling over backwards.

But, as the week wore on, it became increasingly clear that some more sinister force was at work. We reluctantly came to the conclusion that our refrigerator had, in my grandmother's words, conked out. This was of particular concern because at any given time, our refrigerator contains a value equivalent to several refrigerators' worth of prescription medication. So, one evening when the temperature in the cabinet had reach 48F and showed no signs of going back down, I called our reliable hero, Nona, who came over to pick up our stock of medications and perishable foods.  Just in the nick of time, too, as that evening marked the last 24 hours of our refrigerator's useful life.







I then spent a great deal of time on the internet, reading reviews, checking and re-checking measurements, searching for sales, performing comparisons to see which vendor could get us a new unit the most quickly, and so forth. In summary, we are living out of a cooler packed with ice on the kitchen floor, but with any luck on Thursday we will be back up and running with a new refrigerator that has the bonus feature of actually staying cold.

All this shenanigans has occupied what little remained of my available brain cells. In fact, I've been so distracted by the whole thing that I completely forgot to take Lemon to food school this afternoon. He has a clinic visit coming up on Wednesday, so I'd been devoting my mental energy towards not forgetting that, and making sure I was writing down all the questions I want to ask at the visit. And thus, food school fell through the cracks. There is now a weekly reminder set on my phone, and I am really hoping to have those neural pathways back starting next week.









I feel a little silly writing this whole post about a malfunctioning refrigerator right now. Yes, it's a pain, yes, it's disruptive, yes, it's money we weren't planning on spending in this way and at this time. But on the other hand, we are so fortunate that that is all it is. Unlike our fellow Americans in Puerto Rico, we have power, we have a functioning infrastructure, we can get ice, we can get more food, we can get more medicine, and we can get a new refrigerator. So, please consider sending a few bucks their way. There are a number of organizations, like this one, that are helping out there with relief efforts that could use your assistance. And hey, since tomorrow is a day ending in "y," maybe pick up the phone and let your senator know that you like having access to healthcare. Couldn't hurt.

Week 213: The unexpected

Parenting keeps you on your toes, no doubt about that. Just when you think you have something figured out or know what to expect, you discover that you were, in fact, wrong. Take, for example, what I had always imagined would be true about sleeping. I had a notion that I would perhaps not sleep ideally well while pregnant. I had also heard repeatedly that infants do not sleep at convenient times. And I had heard that sleeping through the night was not an event, but rather a process, and that the process would be considered to be well under way when the child performed the miraculous feat of sleeping from 11pm until 5am. But, in all the things I'd heard about sleeping, I did not hear messages about not sleeping when a child was say 2 years old. Or 4 years old for that matter. Perhaps the people who preceded me into parenthood realized that the survival of the species was contingent on them remaining silent on these points.

Well, I am now sufficiently sleep deprived to let you in on a secret from the land of having a two year old and a four year old: I have not slept until (not past; UNTIL) 5 a.m. more than 5 times since June. And it is starting to take its toll. To a very large extent, this state of affairs has been driven by Lime and his accursed molars, two of which remain lost but not forgotten somewhere in his upper jaw. He is gnawing and drooling as though his life depends on it, and still no teeth. These past few days, though, Lemon has realized that now there are things happening in our house at 4am, and being loathe to miss out on any action, has decided that he too must take part. I'm glad I have so many of the kids' favorite books memorized, as that makes it much easier to read them with my eyes closed...





Through all the exhaustion, though, the kids still find ways to make it worth it. For instance, today at food school, Lemon had a semi-accident (made it to the bathroom, but not quite soon enough or with enough control). So, when we got home, he had to change clothes. He's been able to get out of his own bottoms for a long time, but still kind of struggles with shirts. So, he slipped off the wet bottoms, refused my help with the shirt, and then, with the shirt off of his body but still stuck around his head like a turban, and without a trace of irony, he announced "See how grown up I am now?"

So, yes, my love is unconditional. I still find that whole scenario hilarious. But, just in case the kids are reading the blog, I will also emphasize that my love would still be unconditional, and I would still find them hilarious, if they slept until the decadently late hour of say 5:30. Also, I do not hesitate to point out that if they keep up their current routine after the clocks fall back an hour, they will have to start sleeping in the garage.

Week 212: On its head

To everyone's amazement, we made it through the first week of school. A big success from our first week is our new morning babysitter, who has rapidly become a favorite of the kids, taking them on all sorts of adventures in the wilds of our neighborhood before school. Predictably, this leads to some reluctance to actually leave for school, but we're working on that.

The first day of school for both kids was Wednesday. Somewhat to my surprise, Lemon was really hesitant to be dropped off, despite never having had a single difficult drop-off last year. He's been a little teary and clingy every morning so far. I was worried that Lime would actually be the harder one to drop off, being more of a Mama's boy by disposition than Lemon ever was.  And yet, on the first day, he saw his classroom, recognized it from orientation on the day before, and walked right in, leaving me standing in the hall in shock. And so it's been every morning since. I hear this is a common "second child" phenomenon, but whatever it is, I'll take it! At the end of the day, it's a bit of the reverse--Lime is exhausted and eager to come home, whereas Lemon, having found his footing, is having too much fun with his friends to leave.

Of course, every CF parent's big concern in the fall is the return to a classroom fill of disease-riddled vermin (ahem, I mean fellow scholars). We are apparently getting the school year off to a flying start, with both kids having come down with a cold after just 3 school days. Lemon was very sad on Saturday, full of congestion and general woe, but his spirits were better on Sunday and today, although he is of course coughing up a storm. We've gone up on PT and down on tube feeds in an attempt to maintain some sort of equilibrium and hopefully avoid a round of Cayston. Although, we haven't done any antibiotics at all since May, which is a great stretch for us.

At least this early cold has given us a good test case for how we will fit in 3 sessions of PT with our new weekday schedule. The answer, at least based on today's experience, is barely, but I think it's doable. Mondays are especially tricky because we now have "food school" on Mondays at 1, so fitting in PT between getting home from regular school at 12 and leaving for food school at 12:45 is tight, to say the least. It does seem like we are making progress at food school, though, so I'm committed to stick with it at least through the fall, with the hopes that maybe we could "graduate" in December (which is conveniently around the time that we will have completed the 20 visits that our insurance agreed to cover).

 I'm sure in another few weeks this schedule will feel normal and I will have forgotten about all previous schedules, but for the moment it really does feel like we turned our entire household on its head. Lemon decided to take this to a very literal level over the weekend by tripping on the sidewalk and somehow managing to land mostly on his nose and the left-hand side of his face by his eye, such that everyone I've seen with him today has been asking me what happened. At least at this point I'm used to accounting for a child with obvious facial injuries. With any luck, in the coming weeks the schedule will settle down, the cold will go away (please!!!), the scrapes will heal over, and we'll be on our way into fall.