Week 185: Plateau

I've always felt that with CF, we swing back and forth between focusing on pulmonary symptoms and focusing on nutrition. When Lemon was really young, those cycles were quick, almost dizzying. Now that he's older, the cycles have lengthened out. Sometimes, they get so long that we forget that we're even cycling. But, we still are.

Since we tried Cayston, I've gradually allowed myself to hope that we finally have a non-IV intervention plan that actually works. We're nearly done with our second 28-day cycle (thank heavens!), and Lemon is at respiratory baseline, no cough, full of energy. With spring around the corner, I am growing increasingly optimistic that we will make it through winter without another hospitalization. We know now to anticipate the spring allergies and not get spooked by them. We've got our air purifier and Zyrtec and Flonase ready to go.





In December, we really thought we had nutrition figured out. Lemon had hit 36lb. He was at 80th percentile for BMI. His blood levels of various vitamins and fats were in great shape. So, we started coasting. And I should have known better, because I know too well what happens when you start coasting with CF. It catches up with you. Guess how much Lemon weighs now, 3 months after that amazing weigh-in in December: 36lb; no net weight gain in 3 months. He lost a bunch when he was sick, then gained it back quickly, and that's about it. I'm quite sure he's gotten taller over the last couple of months, since his ankles seem to be sticking out of every pair of pants he owns. Taller but not heavier means BMI going down, and we can see it--there are lot more visible bones and muscles than there used to be. So, at our clinic visit next week (how can it be that 3 months have already gone by since our last visit?), we'll have to retool our nutrition plan again.

The other thing I've been wondering about a little bit is whether it is time to reconsider the idea of some kind of eating therapy for Lemon, because he is still soooo resistant to eating. We offer but don't push, we encourage, we praise, we present choices...and nothing. Tonight's dinner was about 15 minutes of drama for 2 bites consumed. Aside from a few bites of graham cracker or whatever at school snack, that was it by mouth for the day--and we would consider today a good day because he did eat a few bites of dinner. I feel like developmentally he's light-years ahead of where he was the last time we tried eating therapy, so maybe it's time to try again. With school letting out for the summer, maybe it would be a good time to focus on something different.

Just to keep things interesting, Lime has developed into an expert climber--a skill that Lemon never really honed to this degree. Lime will drag any kind of chair or stool around to his desired point of embarkation, climb on it, and head for the stars. Or the top of the dining room table. Or the kitchen counter. Or the top of his changing table. Or any other nearby surface that might hold something of interest, particularly something edible. He is also still waking up really, really early. This despite the fact that every night, as I put him in his crib, I say, "Good night, see you in the morning, not too early!" At this point I'm actually looking forward to the clocks springing forward. Assuming he stays anchored and the clock shifts around him, I can look forward to sleeping in until 5:30 sometime soon!

Oh, yeah, and happy birthday to Papa Bear--I guess that was this week, huh? ;-)

 

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!

Week 180: Ever vigilent

We have arrived at a day that I have been awaiting a long time: Lemon has been completely off antibiotics for two full weeks, for the first time since September. I contacted our nurse practitioner today and it's official, we're off hospitalization watch. If Lemon gets sick again, our intervention plan will be another round of Cayston. I'm told that we have a refill on our existing prescription, so tomorrow Papa Bear and I will get things in motion to get that refill into our hands so that we have it when we need it. We are currently accepting bets on how many hours of our time it will take to get everything in place to receive the refill. I'm so glad that we've finally found something that works for Lemon, after years of mucking around with oral antibiotics that, if I really think back on it, probably never actually cured him of anything in his life. But, it does drive home the reality that when he gets sick, we're signed up for a minimum of a month of three treatments a day--we can't even pretend anymore that just pushing another medicine through his tube twice a day will do anything. Still, if it keeps us out of the hospital and away from PICC lines and IV infusions, the extra time and energy of the inhaled antibiotic is 100% worth it.

On Friday, I took Lemon to see his regular pediatrician, who wants to follow him every 6 months. Honestly, I thought about cancelling the visit, because Lemon is actually well at the moment and I wasn't sure it was worth the infection risk to even bring him to a pediatrician's office at this time of year. But, I had an important agenda item: I needed someone with an MD to fill out the prior authorization form for our insurance company so that we can get our second opinion in Milwaukee. So, we went, and I'm really glad that we did. Lemon and Lime's new pediatrician is a genuinely kind, caring person. I immediately got more traction with him on the idea of a second opinion than I did with our clinic. The clinic's line has always been, "Oh, yes, that's a great idea, we really encourage that." Which has not, since I raised the idea, turned into a shred of actual support in terms of getting there. The pediatrician, on the other hand, upon hearing about the situation, said, "Oh yes. You need this. Let me take your copy of the form and the names of the doctors there." So, with any luck, we're one incremental step closer.

The other good thing about the visit is that we got the pediatrician to put some orders for bloodwork in the system for Lemon. As you may remember, back when he had the PICC in October, we drew labs and he had really high values for a number of liver enzymes. Our nurse practitioner attributed that to the fact that he was sick, and said we'd retest in March. But, since Lemon is healthy now, and who the heck knows what will be happening in March, I got the orders put in now so that I can take Lemon over to the hospital at our leisure to get blood draw, probably later this week. It's an extra trip to the hospital with all the attendant risks, but I really want to see what those numbers are when he's well. He's also been a little pale lately, even for his usual pasty white self, so we're also going to do a blood count and see if there's anything to be seen there or if it's just the natural pallor of a Wisconsin winter.

The one piece of bad news that we got at the visit is that Lemon has actually lost a pound over the last month. I think we'd become a little complacent about nutrition since his last clinic visit, because everything was looking so good--his vitamin levels were all in the normal range, and he was gaining weight like a champ. I think we mentally checked the box in our heads that said "Nutrition plan working." And, of course, as soon as you stop paying attention, things change. Lemon hasn't been eating much at all these last few weeks, and he's been feeling great so he's been EXTREMELY active. And that has had its inevitable result. So, we're going to try increasing his overnight feed from 3 to 3.5 cartons of formula and see where that puts us in a month or so. I feel like we're due for a recalibration of his nutrition plan anyhow--it's been nearly a year now since we had the feeding tube placed, and he's grown so much since then. So, now  that we (however briefly) have pulmonary under control, maybe we can focus our next clinic visit on that.

Oh, right, one more moment of hilarity from the pediatrician visit. The doctor checked Lemon's g-tube site and then asked me who was in charge of it. I gave him kind of a blank look. He clarified, "You know, which doctor is following up on it and doing the tube changes?" I had to bite my tongue not to laugh, and he did his best to hide his shock and dismay when I said that it was my understanding that I was in charge of it, and no medical person has looked at it or changed the tube since surgery decided they were done with us.

Out there in the world beyond this blog, it has been a big week for our country. I don't want you to think that I'm unaware or disengaged. But, I have made an editorial decision that this is a blog about the life of our family as it unfolds. Writing these weekly updates is therapeutic, relaxing, and a chance for me to try to view what's happening in our lives at arm's length. Honestly, at the current moment, it's a bit of an escape for me too, to take an hour and just think about what's happened in these four walls and look at all the cute pictures from the week. I hope that reading it is and will remain an escape for you, and that it will renew your energy and your commitment to fight for the causes that you hold dear.

Week 179: Marching forward

Want to know what happened in our house this week that was CF-related and unexpected? Nothing. Yup. No news to report. We've been off Cayston a week, and so far, so good. Still very much in one-day-at-a-time mode, but we'll take it.



Lime had his 18 month check up (18 months?), and it seems that he has left his little teapot (short and stout) phase behind him. He is still short, but now he is skinny. Given how much he moves around, I can hardly say I'm surprised. The doctor recommended a high calorie diet and some extra iron. Luckily I have some previous experience with those things. In the mean time, and not as much in the background as it would seem from the blog, Lime has continued to blossom into a real little person. He has his various passions, including his dedicated efforts to befriend our cat. In the past 24 hours, he has offered her such treasures as an olive, a red Lego, a piece of a donut, and a green block, placing each offering at her feet when she declines to take it from his hands.

On Saturday, I did what I thought needed to be done and ran from our house over towards the state capitol to join our edition of the Women's March. I have never participated in a protest before, but I felt like this time I had to go. One of the top issues on my mind is (no surprise) access to healthcare. It's pretty simple--kids like mine would die without access to healthcare. And, from my very fortunate position with so many resources at my disposal, I feel like that access is under attack. If the threat feels real and immediate to me, I can only imagine how much more real and immediate it feels to those who have fewer resources. And, let's be clear--chronic disease is not a rare thing. About 1 in 2 adults has a chronic disease of some kind. Even rare diseases are not rare things--1 in 10 people in the US has a rare disease. In summary, this issue affects everybody. Every single person in the US either has one of these diseases, or has a first-degree relative who has one. We need to work as a country to figure out a way forward on this.

There has been a lot of talk on the various interwebs over the last couple of days about what the march was, what it meant, what it will mean going forward. I've done a lot of reading, and I don't have any general answers. What I can say for certain is that I have the phone numbers of my three representatives in Congress programmed into my phone, and they have heard from me in the past weeks and will continue to hear from me on a regular basis with regard to this issue. Obviously, there are so many other issues, but I am realistic--I have the bandwidth for approximately 0.8 issues, which I am rounding up to 1. I am hoping that some of the other 3 million or so of you will be out there making calls about the other ones.

A side bonus of attending the march was that I managed to connect in person with one of my best CF mama friends. It's ridiculously hard for us to get together, because we're busy people with young kids, and our kids can't be around each other lest they pass germs back and forth. So, I think I'm not exaggerating when I say that we last got together for lunch in June. And, we promised that we'd have coffee together sometime before the mid-term elections. So that's something.