Week 236: Weighty

This week was monumental in that at long last, Lemon officially weighed in at over 40 lbs on the clinic scale (41 lbs to be precise). We'd been stuck at around 38 lbs for about 6 months, until at our last clinic visit I asked for ways to boost the calorie content of his formula--clearly the 1200 calories we were pumping into him overnight were no longer sufficient to bulk him up after the series of respiratory infections that he'd had earlier in the winter. We started adding about 150 calories worth of DuoCal (powdered, soluble calories, chemistry's best) to each overnight feed. And, voila, after 2 months of that plus not throwing up, a weight gain of almost 2.5 pounds. And, those two months happened to be January and February, which is downright crazy in that those have not been the most favorable months to have CF in Wisconsin in our past experience. Lemon is now back up to 64th percentile for BMI and has been holding steady on 70th for height. Woohoo!

The only downside of our clinic visit this time was that after our exciting weigh-in, we had to wait in our exam room for 40 minutes until the person from the pulmonary function lab came to get us for our test. This was frustrating, to say the least, given that Lemon is not particularly gifted at activities like "sit still and don't destroy everything." And, our appointment for the PFTs was at 8:00 a.m. We were there at 7:50 and in the room waiting right at 8. How they could be 40 minutes behind schedule for the first appointment of the day is sort of mysterious. By the time we eventually met with our dietician, at about 9:30, Lemon was completely wild with boredom, so it was hard for the dietician and I to have any kind of meaningful conversation. After the appointment, she sent me a message saying that the appointment seemed kind of chaotic, and was there anything I could suggest to improve things. I tried not to be snarky, but the basic message was, we had an appointment, I kept my end of it, where the heck were you guys? We had an even worse experience at our previous appointment back in December, so hopefully by the time we go back in May they will have gotten things figured out.

Lime actually had a pretty bad cold/cough this week, so much so that I kept him home from school on Thursday because he'd been up coughing all night on Wednesday night. I'm not sure there's anything more piteous than a 2 year old who is weeping and pointing at his face saying that his nose is "fluffy." In any case, it appears that this is a bug Lemon has had before, because miraculously he didn't catch it. Of course, he did catch something else, a vicious 24-hour stomach bug that struck first thing Friday morning. Luckily by Saturday morning he was pretty much back to normal operating parameters.

We had one nice bit of follow-up from the advocacy day last week: one of the state senators who received our thank-you note put it out on her social media pages. So, it seems like we really did connect with at least one person. Since the Wisconsin advocacy day was such a success, the national foundation asked if any of us wanted to join the national advocacy day in Washington D.C. this spring. Although I've kind of caught the advocacy bug now, my spring is already to crammed with other stuff to go this year. Watch this space in 2019!

Week 235: To the capitol

After a nice dinner last Monday night with my fellow advocates, I came home to practice my lines and get a good night's sleep before the big day. Since I was out for dinner, Papa Bear had a boys-only pizza party with Lemon and Lime, featuring delivery from Domino's. Lemon outdid himself and reportedly ate two pieces of pizza plus some of a cinnamon stick. I mention this only because it appears that this quantity of grease in one meal is sufficient to destabilize the delicate CF digestive system, which meant that I was up at 1 a.m. dealing with puke and starting laundry. So much for the good night's sleep, but I suppose it served as a good reminder of why I was going to the capitol to advocate.

On Tuesday morning I did something I haven't done in years, thanks to working from home--I got up, took a shower, put on professional-looking clothes, and caught a 7:21 bus downtown. After meeting up with the rest of the advocacy team for a quick breakfast, we walked across the street to the capitol building where we split up to go to our meetings. Each meeting had about 3-5 advocates, with a different set of advocates in each one of the meetings. It seemed like the organizers were trying for a mix of viewpoints in each meeting (care provider, family members, an adult patient with CF, etc), and they tried to make sure that each advocate met with his or her own representatives. I met with 8 legislators or their staffers over the course of the day, so it was packed. I had my show-and-tell bag in hand, and my rehearsed talking points in my mind. The meetings were each about 20-30 minutes long, so each of the advocates had time to talk and answer questions from the legislators. The fact that we had different people in each meeting was great, because it made the conversations fresh each time--we didn't know exactly what the other advocates in each meeting were going to say, and we had a chance to learn from each other as well as educate the legislators.

The legislators (or their staffers) that I had scheduled meetings with were Rep Terese Berceau (my state rep), Sen Fred Risser (my state senator), Sen Jon Erpenbach, Sen Alberta Darling, Rep Debra Kolste,  and Rep John Nygren. I also had an hour or so break in my schedule, so I sat in on a meeting of the Joint Committee on Finance, which was a good opportunity to see our state government at work. It was sort of fascinating watching the legislators make impassioned speeches about the subject at hand (something to do with Medicaid) even though the room had almost no spectators, and the other legislators who were there didn't even seem to necessarily be listening. I even overheard one of the legislators complaining about "all the political theater." It seems kind of mysterious to me, really. If they aren't performing for an audience (the room was nearly empty) and they aren't actually communicating with each other, what is actually going on? Who is the theater for? The good news, though, was that the legislators kept slipping in and out of the room between votes. The consultant that the CF Foundation had hired to help connect us with legislators was there sitting near me, so when she saw any legislators that she knew step out of the hearing room, she would grab me and make an introduction. So, I got some bonus hallway meetings with Sen Luther Olsen and Sen Leah Vukmir.

I was surprised to find how much I enjoyed the whole day, and how positive I felt after it. Politics
feels very polarized these days, and I had a hard time imagining how I would connect with some of the people on my schedule. I was impressed to find that, to a person, they were warm, friendly, and genuinely interested in what we had to say. They asked thoughtful questions, shared relevant personal experiences, and seemed to really value meeting with us. More than one of them remarked that one of their favorite aspects of their job was the fact that they could learn about such a diverse range of issues every day. This is apparently advocacy season here in Wisconsin, so we were by far not the only people there advocating for a cause that day.















Of course, all the meetings were made easier and more friendly by the fact that we had no specific policy asks this time around. This is not a budget year in Wisconsin (that happens on the odd-numbered years), so the purpose of these meetings was just to introduce ourselves and our cause to the legislators, with the understanding that we will be back next year with some more concrete goals in mind. Still, I felt really encouraged that it might be possible to find common ground even with people of a very different political stripe. It was certainly a great change of pace from calling the exhausted interns at my US senators' offices--even in this age of technology, there is a lot of value to an in-person meeting. I had also forgotten (or maybe never fully realized) how much important policy really is decided at the state level--these days, the national political scene seems to drown out the local, even if you are trying to pay attention.

I'm still tying up some loose ends from the day--we're supposed to send thank-you cards and emails to all the legislators that we met (not quite done with that yet), and send in a report to the CF Foundation about our experience (not quite done with that either). But, hey, I wrote a blog post about it, so that's a good start!

Week 234: Talking points

Tomorrow is Wisconsin's CF advocacy day at our state capitol. I've just gotten back from a dinner and training session with the other people who will be advocating with me tomorrow. There was one other CF parent (who bought me a much-needed Manhattan at the bar before the dinner started), a CF grandparent, and someone with a close family friend with CF. Also, to my surprise, pretty much the entire care team from our CF center was there--the nurses, the dietitian, our nurse-practicioner, the center director, and the social worker. And, on top of that, the staff from the adult CF center in Madison as well. So the provider-to-family-member ratio was pretty outrageous. There were also a couple of representatives from the advocacy team at the national CF Foundation office in Bethesda, MD, and a couple of local consultants that the CF Foundation hired to help them navigate the Wisconsin state legislature. 

Here's the deal. Tomorrow, we'll be headed to the state capitol for a series of meetings with legislators. I have 5 meetings, pretty much back-to-back for the whole morning, including meetings with both my state representative and state senator. The meetings are all in groups, so I'll be joined by various members of the group, including some of the people who help take care of Lemon.

We spent a good portion of tonight's dinner going over our talking points for tomorrow. Some of these meetings will likely be pretty brief, because the Wisconsin state legislature is trying to wrap up all its business in the next several weeks before they go into recess until next year. So, we may not have a lot of time to get our message across. As in, we might get say 5 minutes, while walking. Or we might get 30 minutes with a staffer. The big challenge will be to address talking point number 3 on our list: tell your personal story. How to convey how CF has impacted our lives, in maybe 2.5 minutes. I'm doing some practicing tonight. And also watching figure skating. 

In terms of legislative talking points, we have two main ones. The first is to thank our legislators for funding the Wisconsin Chronic Disease Program and Badgercare (Medicaid), which support more than 30% of people in Wisconsin with CF. The second talking point is to request an amendment to Assembly Bill 365, which is supposed to protect people with pre-existing conditions in Wisconsin. However, it only does so for people who have no gaps in their health insurance coverage. If you have a gap, under this bill, you might be at risk for higher premiums or cost-sharing provisions. It's a lot of information to spit out in a potentially short amount of time, so we will need to be on the ball.

In other CF news, just this afternoon the FDA approved Vertex's latest CF medicine, which will be sold under the name Symdeko. It treats the root cause of CF in patients who have either two copies of the F508del mutation (the most common CF mutation), or one copy of the F508del mutation and one copy of a list of about 20 mutations that were shown to be responsive to the medicine. Sadly, Lemon's mutation isn't on the list (although, as I scientist I feel like he should be on the list, since his mutation is genetically similar to several of the mutations that are on the list). In any case, this is tremendously good news for the CF community as a whole, and is evidence of the work that has been done with all of your generous donations to the CF Foundation. Even more importantly for our family, Vertex also announced recently that it has selected two more new CF medicines that it will advance to Phase III clinical trials--and these ones are the real deal for us--the medicines that will treat the underlying cause of CF for Lemon. We are waiting with baited breath for the results of those trials, given how good the Phase II results were. 

In the mean time, I will focus on the matter at hand--advocating to make sure that as these wonderful medicines become available, people with CF have the ability to access them! If you use "the Twitter" (yes, this is how it was described at tonight's meeting), follow the hashtag #CFAdvocacy tomorrow to see what we're up to at the state capitol--goofy photos with state legislators and all!

Week 233: Unconventional gifts

Lemon has continued to astonish me with his  interest in food. Whereas a week ago, he had a bit of a cold and was not really interested in eating, this week he has been feeling good and is continuing to push at his boundaries (in all respects, really, but we'll keep it positive and just focus on food). With no real prompting or pushing, this week he ate cornbread with butter, tomato soup, and a thin mint cookie, among other things. Perhaps most surprising was tonight at dinner, when I presented him with a plate of fairly safe choices (macaroni and cheese, salami, broccoli). He looked at his own plate, then looked at mine, and asked me for a cube of tofu from my stir-fry. I was skeptical but far be it from me to say no. He went on to eat 5 cubes.

He then remarked that the tofu was so delicious that I should make it every night, and that I should make a special point of making it for Valentine's Day. When I asked him why, he said that he wanted to give it to our neighbors as a Valentine's Day present. I pointed out that gifts are usually somewhat more durable and less coated in sauce--a nice box of chocolates, say, or some flowers. He looked at me as though I were totally naive to the ways of the world, and said, "No, not plain tofu, Ma. IN A BAG."  Of course.

I guess in summary I would say that I am so glad that we toughed it out at food school. I was very near pulling the plug on the whole thing a couple of months ago. Lemon was sick constantly, and it didn't seem to be helping at all. The only thing that kept me in it was the realization that once he starts kindergarten (which runs 8:30-3:30 here in Madison), we would not be able to do it anymore, so this year was our only shot. And that constraint kept me from making what would have been, in retrospect, a big mistake. Although, if we had quit, we would never have known that it _could_ have helped him, and the decision to quit would have seemed totally justified.

This theme is something that I feel like we come up against all the time in CF. We are trying something, and it doesn't seem to be working, or at least not working very well, and then we need to decide whether to keep trying it, or to stop and try something else, or to keep doing the thing that doesn't seem to be doing anything, just in case it is doing something, but then adding something else on top. It is a maddening way to try and make decisions, especially decisions with long-term implications for your kid's health. It's so rare to try something and get an immediate and clear read-out that it worked. I think that's happened to us twice, once with Cayston and once with ursodiol. Everything else has been much murkier.

CF advocacy day is a week from tomorrow, and planning seems to have kicked into high gear. I have a webinar to attend on Friday, and then next Monday night, an in-person training with the other volunteers. I'm really looking forward to it. After spending probably too much time these last weeks reading about the activities of our elected representatives on the internet, I'm so curious to meet a few of them in the flesh!

Week 232: Advocate

For the past, oh, I don't know, let's say a year, I've wanted to do something to add my voice to the public discussion on healthcare. Sure, I write the blog, and I've called my senators more than a few times, but it was always on my mind to try to do something more. Luckily, an opportunity was just dropped into my lap. Our local CF Foundation chapter invited me to go to the Wisconsin state capitol in a couple of weeks to meet with members of the state legislature to talk about issues important to families with CF. Even though I'd been hoping for just such an opportunity for a while, my first instinct was to decline--spending a whole day in an out of offices of people I don't know, talking to them? Ick. But, I managed to overcome my initial reluctance and signed on. So, that's forthcoming.

Other than that, pretty much business as usual here on the home front. Lemon continued to have a lingering but not terribly bad cough throughout the week. I fully intended to do an extra treatment with him during the week on several days, but sometimes it can be nearly impossible to fit in. On Mondays, he has food school, so we get home from regular school at 12, and have to leave again at 12:45 for food school, which gives a grand total of 45 minutes to get into the house, do a treatment that takes just over half an hour, re-locate all the outerwear, and leave the house again. It can be done but it isn't any fun for anyone. Then on a couple of other days, when our afternoon schedule was less hectic, he ended up being so tired that he took naps, and there is just no way I am going to wake up a child who is sleeping and desperately needs the rest to do an extra session in the vest. So, then it was Friday. Our schedule over the weekend was much more relaxed, so we were able to do 3 treatments both days, and I do really feel like that helped him to clear out the last bits of the cough, and he's basically back to baseline now. Would he have gotten there sooner if I'd managed to fit in more treatments during the week? Maybe. Do I feel guilty for not squeezing them in? Yes. But I try to be realistic.

Meanwhile, poor Lime has a couple of pretty sizable cold sores around his mouth. They don't trouble him too much except when it comes to eating ketchup. Which for him is a big problem, because he dips everything in ketchup. Not just the usual suspects like hot dogs and french fries, but also sandwiches, carrot sticks, apple slices, pieces of cheese, you name it. So, hopefully those will clear up soon.

As you may have heard, we're in the middle of a pretty bad flu season, which makes me nervous. Please be good to yourself and those around you. Get your flu shot if you haven't yet, wash your hands a lot, don't lick random surfaces while out in public spaces (yes, I live with preschoolers), and stay home if you're sick. While the flu may be an inconvenience to most of us, to vulnerable people like Lemon it could be an absolutely devastating illness, so please keep people like him in mind when you're deciding whether or not to go to work or send your kid to school. There are still another 12 or so weeks of flu season left, so please be vigilant!

Week 231: Kookaburra

This week, I would like to begin by describing a dream that I had one night this week. I don't usually remember my dreams, but this one was especially vivid. Here it is: I dreamed that Papa Bear and I were living all by ourselves in a small, modern apartment in Cambridge. Just the of us, no kids. The plot of the dream was as follows: I woke up (in my dream) after a full night's sleep, sat down at my table with a cup of hot coffee and read something. Also, it was silent. Completely silent. The end.

I have never really been good at interpreting dreams and I don't intend to start now.

What else is going on? Well, as predicted, our streak of good luck with colds has drawn to a close. A month of relatively good health in the middle of winter isn't bad, though, all things considered. This one is a weird one, though--when Lemon first got it, it came on fast and hard, from zero to a really severe cough that was making him cry in his sleep within 12 hours. And let me assure you as a CF parent, there is nothing more heartbreaking than hearing your poor kid cry in his sleep because coughing hurts. Papa Bear and I were sufficiently alarmed that we started talking about Cayston right away, but agreed that since he had no fever and that it had only been 12 hours, we were too seasoned in this fight to just jump straight to the intervention plan. I'm glad we didn't, because by 24 hours in, the cough had backed way off, no more crying, tolerating the full volume of his tube feed at night with no trouble. But it's lingering in a way that I don't necessarily like. He's still got a pretty frequent, loose-sounding cough. On the other hand, he is clearly feeling fine and the tube feeds are still staying down so we are just sticking with doing 3 vest treatments a day and watching.

Of course because he is not feeling 100% this week, eating has been much more of a struggle than last week. He did do one new thing, though, which is that we went to a friend's house for a play date and he actually ate snack there, which he'd never really done before. He usually gets thrown off by things not being exactly the same as what we have at home. But this time, even though the snacks were different, he sat down and actually ate stuff with his buddy. And, honestly, about the same volume of food as his buddy. I am cautiously optimistic that we've reached the two steps forward, one step back phase of this process, where we continue to make incremental but measurable progress. Now, to get that to translate into some more body weight. Sigh.

Lime has continued to wake up to pre-game with me at around 4 or 4:30 every morning, which is just awesome. He really likes fig bars, so my strategy is that when he wakes up, I grab a package of them, open it (it must be opened all the way, with the wrapper laid flat, otherwise he believes that he cannot get to the second piece), lay the opened package next to him on his bed, and tell him to eat it and go back to sleep. Which works in the sense that I get to be back in my bed from say 4:36-5:01. It's perfect. A few years ago there were lots of stories in the news about people who took Ambien or other sleeping pills and then would wake up and find their bed mysteriously littered with candy bar wrappers, which is pretty much the scene in Lime's bedroom right now. Oh well.

For those of you who are not interested in cat hilarity, you may want to stop reading here. Although, with the news cycle we've gone through in the past week, who couldn't use a little cat hilarity?

First, both Duncan and Donut figured out how to do this:

Next, we got this:

Here's where we are now:

Anyone have a metal canister with a screw top (preferably locking) lid to send me?

Week 230: Vigor

Lime (remember him?) hit the 2.5 year mark this week, and in honor of that occasion had a check-up. Lemon has never minded going to see the regular pediatrician, presumably because it is not nearly as bad or as time-consuming as a visit to the CF clinic. Lacking this frame of reference, and being 2.5, Lime acted as though he were being kidnapped. A small bribe in the form of M&M's went a long way towards soothing his troubled soul, though. At the check-up we learned that he is still, in the immortal words of his first pediatrician, "tiny but vigorous." However, he is incrementally less tiny than he use to be. Whereas at his second birthday he was in the 4th percentile, he has now jumped all the way up to the (wait for it) 11th. So, still fairly minuscule but now within the magic range of 5th-95th percentile that pediatricians find reassuring.







The only minor problem is that to get him there, I've been letting him drink whole milk all night long, to the tune of a pint a night. Well, it turns out that's not so advisable from a dental perspective. I switched him over to water, which he's taken to admirably well, except of course for the part where now he's waking up at 4 a.m. again to have pre-breakfast. One imagines there must be a point at which he will be able to consume enough calories during the day to sustain himself through the night, yes?

And then there is Lemon. I hardly want to write about it for fear of tempting the fates, but the purpose of the blog is to report the news, so here it is. Lemon is eating stuff. All kinds of stuff. Sometimes, even "a lot" of stuff. Double-digit numbers of bites of the same food. Randomly trying new things. Pasta with red sauce and Parmesan cheese ("This is really delicious, Ma."). Hamburger with ketchup. Fried cheese curds. Watermelon. Weird hummus that was not exactly the same as the hummus that we have at home. Chips with seeds in them. Crackers with cheese spread on them. It's honestly shocking.

So, what's going on? Probably several things. He's continuing to get older, and to become more aware of what his peers are up to. We've been working hard at food school, and he's acquired new skills and confidence when it comes to eating. He's been healthy for something like 3 consecutive weeks, which is the longest illness-free period we've had since school started, so maybe he's finally feeling truly 100%. I have one other idea, I have no idea how valid it is, but thanks to ursodiol, his liver is functioning normally for the first time in his life. A very common symptom of liver inflammation is: poor appetite. I do wonder if for a long time he has just not been feeling like eating because of that inflammation, and now that it's resolved, it's contributing to this new interest in actually eating things. As with so many other things about treating CF, we will never know for sure. In this particular case, though, I'm willing to live with some uncertainty.