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!