Week 184: Write now

Here's a little good news as we head in to Rare Disease Day tomorrow. As many of you know, writing isn't just my hobby, it's my profession as well. Since Lemon was diagnosed with CF, I've been looking for a way to use my skills to benefit the CF community. So far, that's meant this blog. And a lot of emails to various people at the CF Foundation saying, "Hey, I'm a medical writer AND a CF mama. Let's do something." Finally, 3.5 years in, one of those emails landed in the right place, and I'm going to be developing some scientific content for the CF Foundation's website over the next few months. I'm so glad to have finally gotten this opportunity, and look forward to sharing the results with you in the coming months.

On the home front, things are pretty much business as usual, which, for us, is awesome. We are into our third week of Cayston so we are over the hump on this course and we're feeling cautiously optimistic that we may make it until spring without landing in the hospital again. I've finally made contact with the clinic in Milwaukee, so who knows, sometime before the end of the summer we may actually get seen there. The social worker that I spoke to there really impressed me with her knowledge of the disease and the steps she wanted to take to get us seen. She's presenting Lemon's case and what we want out of our consult to the doctors at their clinic's weekly team meeting, and she'll find out who might be the best person for us to see. Then, there's the little matter of paying for it, but I have a call in with patient accounts there so we'll see what they come up with. 

This week, Lemon went on his first ever school field trip. He and all his little friends rode on a school bus to a local pet store, where the store manager showed them lots of animals that they could touch and hold. Unsurprisingly, Lemon was about 100x more excited about the school bus than the animals. He kind of liked feeding the fish, but the rest of it (rabbit, guinea pig, puppy, parrot, snake, chameleon, gecko, star fish) didn't do much for him. He mainly wanted to know when we could go and ride on the bus again. To each his own. I went along on the trip as a chaperone--I'm guessing the only one with a spare G-tube button in her purse, but you never know. 

Week 183: Half way there

And, just like that, we are half-way through the 1/3 year. Lemon passed the half-way mark yesterday, and I feel like since we started Cayston and have CF at least vaguely under control, Lemon has actually had the chance to just be 3 and really figure out how to be a kid. Since we've started our current round of Cayston, he's managed to gain back 2 of the 2.8 pounds that he lost when he got sick again, which is amazing. It's clear from his activity level and feisty-ness that he's got lots of energy again, and he's plowing some of that energy into some big cognitive leaps.









I'm seeing this mostly with his social development. Up until now, Lemon mostly cared about himself, the main grown-ups in his life, his brother, and the cat. Other kids were at more or less the same level of interest as the cat--they moved around and made noise, which was interesting, but that was about as far as it went. Now, all of a sudden, he's figured out how much fun it is to play WITH other kids. On Saturday afternoon, it was freakishly warm, and I tried to convince Lemon to go on a special mother-son walk with me where he could ride his balance bike. I got a whole lot of nope until I suggested that we could go meet one of his little friends who lives down the street. Once he knew she was coming, too, he couldn't get his helmet on fast enough.











Lime is clearly moving from being 1 towards being 2. He definitely says fewer words than Lemon did at the same age, but his comprehension of spoken language, gesture, and facial expression amazes me every day. Lemon understood a lot of words at this age too--but mostly just the words about the things that interested him the most (truck, steam roller, helicopter, fire engine--the important stuff). Lime seems to know the word for just about every object or action he's ever seen. We FaceTimed with Uncle Jared this weekend on my phone, so Uncle Jared's face was about the size of a postage stamp. Lime was captivated anyhow, and Uncle Jared kept pointing to different non-nose parts of his face and asking Lime "Is this my nose?" Every time, Lime shook his head, until Uncle Jared finally pointed at the nose, at which point Lime nodded vigorously. And we're talking about a few pixels representing a guy that Lime probably barely remembers seeing in person. Crazy, I tell you.

















It's felt like such a gift to have a "regular week" again, although the price for this regular week is doing 3 treatments a day, day in and day out. Anything is better than being in the hospital, but even after just 10 days we're getting pretty sick of it, and it seems like the rate at which the little vials of Cayston disappear from the box in our refrigerator just isn't fast enough. Part of me is tempted to explore whether we could possibly do a 14-day course of this (analogous to a 14-day course of oral antibiotics) instead of 28 days. After all, we're using it off-label so who knows what the right length is for Lemon? I'm not going to try it this time, because I just want to make sure Lemon clears this thing and gets to spring without further incidents. But, I'll bring it up at our next clinic visit and see what kind of response I get. It's hard to believe, but we've been without a pulmonologist for a year now. We've made it this far, but I am really ready to have someone steering the ship again!

Week 182: Around again

And now, we know the answer. The question last week was, "Can Lemon bounce back from a non-respiratory virus without getting some kind of respiratory infection?" Spoiler alert: that would be a big fat N-O.

Lemon did manage to kick the fever pretty much as fast as Lime. By 24 hours in, his temperature was normal, and we kept him pumped full of Gatorade so he stayed well hydrated. Ultimately, though, that was not enough, and before we'd gotten much past mid-day on Tuesday he'd begun to cough again. And with the coughing, the inability to tolerate the nightly tube feeds. But, still, he wasn't coughing that much, relatively speaking, so we let things play out for another few days to see if just by chance, he could turn things around. We had our supply of Cayston on hand (it did, indeed, just show up on Wednesday without a fight), but we were loathe to start it because it's such a production for all involved. We still weren't convinced that there were bacteria there to fight--we had some hope that it was just a little bit of extra junk brought on by the virus, and if that were the case, the antibiotic wouldn't do anything, anyhow. And, Lemon seemed, all things considered, sort of OK--not exactly himself, but we've seen a lot worse at this point.

On Friday morning, I had Lemon hop on the scale. Just to review, at his check-up on Jan 27, he was 35.3 pounds, down from a high of 36.6. That's when we rejiggered his nutrition plan and increased the volume of the night feed. And that worked--in a week, he'd gained back the pound that he'd lost as was 36.8 lb when I weighed him just before this virus came to town. Friday: 34.0. That means 2.8 hard-won pounds got vaporized by this virus in just one week. I could tell he looked skinny, but man! The realization that we were losing so much ground on the nutrition front put us over the edge, and we started our next cycle of Cayston on Saturday morning.

The good news is that the Cayston seemed to kick in right away, and Lemon is feeling much better. He's tolerating his tube feeds again so with any luck over the next few weeks we'll at least fight our way back to where we used to be, nutrition-wise. I am grateful that it seems like after 3.5 years we've finally found a treatment that can work for Lemon and with any luck, keep him from being hospitalized quite as often. I'm grateful that, even though it seems distant now, spring is coming, and this cycle of Cayston will carry us through mid-March. If he can just sustain things on his own into April, we'll have made it through the winter.

I'm secretly hoping that we get at least one more snowfall before then, though--my Valentine's Day gift this year from Papa Bear was a deluxe new snow shovel (the man knows a his way to a woman's heart!). Lemon insisted that he needed a new shovel, too, which he selected himself. At the moment he's mainly been practicing by shoveling snow from the snow banks into the driveway, but I imagine with a fresh snowfall and some coaching I can get him to understand that there is a directionality to the process!

Week 181: Patriots

Often when I sit down at my desk to write the blog, I pause with my fingers over the keys on the keyboard, close my eyes, and think, "What happened this week?" Then, I'll remember something, and then think, "Wait, was that last week, or this week?" Often times, I'll go back and re-read last week's post to see when things actually happened.














This week was particularly foggy, because something highly unusual happened--I got sick. I had a cold that I caught from Lemon a couple of weeks ago (the one that almost got him admitted to the hospital way back in Week 178) and it's lingered on. I got mostly better but not totally. Then, on Wednesday, things suddenly got much worse, incredible congestion and headache etc. Thursday that progressed into a fever, chills, and inability to function in a standing position. Once Papa Bear got home from work, I got on the phone to my saintly parents, and a short while later, my dad (aka Opa) arrived to drive me to Urgent Care, since I didn't think it was safe for me to drive myself. After an hour or so of waiting in what I can only describe as a serious pit of contagion, I got seen by someone who confirmed my self-diagnosis of a bad sinus infection. A prescription for augmentin and Flonase later (where have I heard of that combination before?), and I was back home in bed, where I remained for most of Friday until the antibiotics kicked in.





And they kicked in just in the nick of time, because as soon as I became barely able to function again, first Lime and then Lemon came down with some sort of unrelated 24 hour kid virus, the main symptoms of which were a moderate fever and unbelievably intense whingy/clingy behavior which persisted from mid-day Saturday through Sunday evening. This illness gave us a chance to try out the hydration plan for Lemon that we'd developed with our nutritionist in October, so we dutifully pumped 50mL of Gatorade cut with water into his belly every hour on the hour while he was awake. He found the idea of pushing bright red liquid into his belly pretty entertaining and wanted to do the syringes himself. Drinking the stuff by mouth was, of course, of no interest whatsoever. In any event, both he and Lime are more or less back to their usual selves now, and hopefully (fingers crossed) we weathered that whole episode without setting off any kind of downward spiral, but only time will tell.  Ask me again next week, and if everything is still OK then, I will be feeling pretty good about it.









In other news, as promised, Lemon's pediatrician filed the form with our insurance company to request coverage for a consult at the clinic in Milwaukee. And, less than 48 hours later, the company rejected our request, saying that the services that we were requesting were available at our in-network provider. The thing is, they're not, really, but at least for the moment I think we're not going to fight this battle. We feel like we have a lot of future battles with our insurance company, and don't want to go to the mat for this one. Now, we're going to try something that, as everyone who's tried knows, is nearly impossible: we will contact the hospital in Milwaukee and find out how much it would actually cost to go there and have a consult with one of the docs.  This should be interesting.





I took Lemon to get his blood drawn earlier this week, and his pediatrician himself (?!?!?) called me up to let me know the results--his liver tests are much closer to normal now than they were in October. Still not totally normal, but he's actually never had results in the normal range on these tests. So, we'll retest in another few months and see where things are, and maybe at that hypothetical consult in Milwaukee we'll see if we can get someone else's opinion as to whether those slightly high numbers mean anything or not.



Finally, I called the specialty pharmacy to get a refill on our prescription for Cayston, so that we'll have it on hand should we need it. Given the amount of effort that it took to get the first installment, I had anticipated that this would be the first in a long series of calls. Instead, I got, "We could deliver it on Wednesday if that would work for you." I've become highly suspicious of things that seem too easy, but I'll take it!