Week 240: See reverse

Technically, it is no longer winter. CF is not an illness that abides by such technicalities, however. So, of course, one more cold is winding its way through our house right now. Lemon succumbed first, then Papa Bear. Lime appears to have a mild "fluffy nose," but is in good spirits otherwise. Poor Lemon is coughing his little heart out. We've gone up on our daily vest treatments and down on our overnight feeds, and so far we may be hanging on.






Lemon  was totally exhausted this evening, and of course prefers to sleep with his head buried in a pillow, just the thing when you have a cold. There's no way to convince him to go to sleep propped up, so he was very sad and coughing/crying alternately, but once I held him propped up in my lap and got him to sleep, I stealthy rearranged his pillows and got him into the propped-up sleeping position without waking him. All is peaceful now, although I'm sure I'll have to go in and re-prop later on. Related--technologies for keeping sleeping 4-year-olds propped up? Anyone?


Other than this new cold, we've had a good week, capped by a really fun visit with Uncle Jared. Both kids are finally old enough not to be intimidated by the height and the beard, so they had a great time playing and demonstrating all the new skills they've acquired since they last saw him.




















We got our first official piece of mail this week from the local elementary school where Lemon will be starting kindergarten in the fall. And, even with just that one piece of mail, so many questions. It was an invitation to an orientation at the school, where we are to drop the kids off, let them play and have a snack, and then pick them up. But. Who will do his enzymes at snack time? Are there other kids with CF in our school district? Among his future classmates? Surprise, he has a G-tube, hopefully it won't choose to fall out while he's there...The invitation included a little survey to fill out about your kid, with a question at the bottom followed by a single blank line: "Is there anything else we should know about your child before we meet them?" I used the single blank line to write two ominous little words: "See reverse." I imagine I'll be getting a phone call at some point.

Week 239: 800 liters

This week marks two full years since Lemon had his G-tube placed. I vividly remember giving him a bath on the night before his surgery, looking at him, and thinking, "This is the last time I will wash this perfect little body before it has a weird hole where there shouldn't be one." Aside from that one memory, I barely remember him without the tube anymore, it's as if he's always had it.

It's hard to believe that when he had the tube placed, he was just 25.4 lb, or 6th percentile for his age. Now, he's 41 lb, and in the 70th percentile. He's also grown 7.5 inches in height in that time, going from the 30th percentile to the 70th. So, was it the right decision for him? Yes. I didn't like it at all at the time. Not at all. I wanted desperately for him to be as "normal" as possible. I wanted him to be able to eat and flourish like any other kid. I didn't want him to have anything that would make him visibly different. That obviously was not in the cards.

I remember reading somewhere when he was a baby that even if kids are picky eaters, they won't starve themselves to death. I believe that is almost correct, in that MOST or even NEARLY ALL kids won't starve themselves to death. Mine would have, without a doubt. Part of that is the fact that the deck was stacked against him in a way that he could not possibly understand. What two-year-old has to consume somewhere between 1500-2000 calories just to stay alive? There is no way evolution could have prepared his brain for that concept. And, he was sick all the time, so who can fault him for not wanting to eat, really?

One thing I had not anticipated two years ago is how dependent we would still be on the tube now. I guess I thought that we'd use the tube to supplement whatever he ate by mouth during the day, and use it to give medicines and whatnot. And that without the pressure to eat so many calories, that he would just come around and start eating on his own. Hah. Part of me thinks that having the tube to fall back on enables him to continue being so averse to eating orally. Surely if he felt hungrier he would eat more. On the other hand, I know that he has been hungry before, and that in no way has that been enough incentive to get him to eat. 

So here we are. We have a tall, well-nourished boy who is bursting with energy and intelligence. I try not to let the fact that he is literally comprised of maltodextrin, sodium caseinate, and soybean oil bother me too much. I still live in hope that some day he will learn to love to eat. When we started food school, I set as my goal that I would like him to eat enough calories by mouth every day that we could stop doing a bolus feed in the mornings. In other words, I would like to have confidence that my nearly 5-year-old child would eat a measly 150 calories by mouth every day. We're not there. I don't even see a clear path to being there, but then again we've been in lots of places on this journey where the path forward has not been obvious.

That seems typical of parenting generally, in a way. You face what seems like an insurmountable hurdle. Will they ever poop in a toilet? Will they ever sleep through the night? Will they ever learn to read? You make numerous and valiant attempts to move forward. And, ultimately, at some point, a small miracle occurs, the kid does whatever it is, and on you go towards the next hurdle. I try to be patient. I try to be supportive. I try not to think about when it might be that I will get an uninterrupted night's sleep in my own home. And I remember how grateful I am that we are stuck here instead of somewhere else.

Week 238: Party animals

We had not one, but two dinner parties this week. The first was the annual school pizza party featuring some musical entertainment. As was the case last year, it was way to exciting an environment for anyone to even consider the possibility of eating anything. It was also too daunting for Lemon to try to go down onto the dance floor with the other kids, that is, until Lime agreed to go with him. Then, they both made it as far as the rear edge of the dance floor before retreating to the top of the steps.

The second dinner party featured Indian food at our house, and some very special guests--Uncle Joel and Aunt Donna, whom we haven't seen for just over two years. They live in Maine, and back when we lived in Boston, we used to see them all the time. Now, not so much. This is the part of living in Wisconsin that is still hard--we are so far from so many people, and given the particulars of our travel situation, we just don't get to see people nearly as often as we would like. So, it is especially wonderful when people come to visit us, even if it's just for a few days. We started talking again about our idea of taking the kids to Maine in the summer to enjoy the beach and all the other fun things there. We'll definitely do it. Maybe in 2022.

If memory serves, slightly more food was eaten at the home dinner party, although this was definitely not a banner week for Lemon, eating-wise. He is clearly still working on some kind of cognitive development, it shows in every aspect of his behavior (sigh). His food school teacher said she could definitely see it at their session today. She's shifting focus now from challenging him with new foods, which he's become relatively proficient at, to focusing on eating more bites in a row of the same food. That is still a big hurdle. Nowadays we can almost always get him to take one bite of something (which is huge progress, let's not discount it). Even in this sort of eating dry spell that we're in, he did surprise me yesterday by eating, of all things, a slice of pickled daikon radish. But eating any sort of volume of anything at any given eating occasion? Not so much.

Spring is gradually starting to creep into the air here. It hasn't been exactly warm, but there is some hint around of what is to come. Or maybe I'm just imagining it as I stare at the pictures in the garden catalog that showed up a few days ago. But, the kids can clearly detect it, too, and we've started spending more time outside each day, for which the interior of our house is extremely grateful!

Week 237: Like a lion

At least we're getting a good start on the "like a lion" aspect of March, with several inches of snow on the ground and more continuing to fall as I write this. Lemon seems to have been inspired by this description of March, and is currently working something out. Who knows what. But whenever he is working something out, taking on the next developmental challenge or mastering the next skill, the first thing to go is eating. After so many good weeks in a row, stretches of consecutive days where he at maybe even hundreds of calories by mouth, we are back to the good old days of sitting down at the dinner table and declaring that we are not eating anything. Hooray for the familiar.

At least we've seen the other side of this now, and I can hope that, once Lemon has figured out whatever he's working on, we will get back to where we were, and maybe even move forward from there. We had food school today, which he enjoyed as always, although his food school teacher agreed with me that we're in a little bit of a regression right now. Even though it's only March, they're starting to work on their summer schedule and the schedule for next year. I took the forms. She thinks there are more things that could benefit him, maybe a group eating therapy session in the fall? I trust her judgement, and I also trust my own judgement that there is no way we can possibly manage the transition to full-day kindergarten, the transition to two school drop-offs and pick-ups every day, the daily burden of CF care, AND food school. Something's gotta give here. But what will it be? Instead of dwelling, let's focus on the fact that Lemon spontaneously (and correctly) used the verb "surround" today.

After a couple of pretty CF-intensive weeks, between the advocacy day and the clinic visit, it was nice to have CF take a relative back seat this week. Things will pick up again in that department in the coming few weeks, as I have a few more articles to write for the CF Foundation website, and a team web page to set up for our annual Great Strides walk (how can it be time for that again already)?

In other signs that it is March, Papa Bear's birthday was on Saturday. Cake decorations were selected by Lemon (blue) and Lime (white). As part of Papa Bear's birthday gift, I got everyone in the family a new Wisconsin Badgers T shirt. You will no doubt notice that there is no cute picture of all 4 of us in our shirts on this week's blog, mainly because someone (ahem, Lemon) detected that his new shirt was not in every way identical to his former Badgers shirt (now too small) and refused to wear the new one under any circumstances. Ah well.  Instead please enjoy this picture of a pair of what I'm pretty sure are sandhill cranes (thank you, Google) that flew over me as I was running this past weekend. They sure sounded like they thought spring was in the air!

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!