Week 280: In retrospect

Once again, I find myself writing the blog on the eve of a holiday. It has been a busy week, wrapping up another fun Christmas visit from Grandma and Grandpa. Of course, no truly fun experience can be completed without a quick trip to urgent care. In this case, Grandma caught one of our notorious Wisconsin winter viruses and was feeling badly enough to seek medical attention. Unfortunately, best medical advice for this particular illness was to remain hydrated and rest--at least she tested negative for flu. Unsurprisingly, said non-flu virus made a romp through the rest of the family, including Lemon, Lime, and Papa Bear, while Grandpa and I did our best to keep the operation afloat. Papa Bear and Lime are more or less over it at this point. Lemon seems to be feeling OK, but of course still has a really big cough that is preventing us from giving him the full volume on his tube feeds. So much for catching up on weight gain over the winter break, but at least we backed down quickly enough on the tube feeds that there hasn't been any puking.

Papa Bear and I haven't had a chance yet to get through all the photos from last week, and I figure many of you may be otherwise occupied later this evening, so in lieu of a more up-to-date spread, I thought it would be fun to take a look back at all of the new years that we've celebrated since this blog began. It's been quite a ride, and it's been a great joy and comfort to share it with all of you. Looking forward to whatever 2019 has in store!

2019:

2018:

2017:

2016:

2015:

2014:

Week 279: Jingle

I have begun to get the impression that some sort of holiday-related celebration is taking place tomorrow. Grandparents have arrived from New York, and our basement appears to have been converted into a satellite Amazon shipping facility. Since it seems likely that you, loyal readers, may also be taken up with preparations for whatever tomorrow has in store for us, I will keep it brief. Be good, drink a stiff bourbon, and hope for the best!

Week 278: Poop in a hat for science

I knew this wasn't going to be our greatest clinic visit ever, but even so, getting our numbers was a bit disheartening. We went into September with Lemon at the 95th percentile for BMI. As of last week, he'd lost a total of 6 pounds, and also grown taller. We have done this math in previous winters but I don't think the result has ever been so striking. He has fallen all the way down to the 43rd percentile for BMI (when the goal is always to stay above 50th). A lot of hard work, all torched in 3 months.










The "good" news, though, is as follows. First, his height velocity is undiminished, so even though he's lost a lot of weight, he has still had enough nutrition/reserves to keep growing upward. Second, his lung function (however reliable pulmonary function tests are in kindergarteners) is unchanged and at 100% of expected for his size. Third, we know what we need to do and we have the tools to do it (more calories in, through the tube). Fourth, as our nurse-practitioner pointed out, the fact that we had those reserves on board probably saved us from a hospital admission and IV antibiotics. So, it's not all grim news.

We're following up with another visit in 2 months to see if going back up to 3.5 cartons of formula per night from the 2.5 we had switched to is enough to get things moving in the right direction again. I suspect it will be, as long as we can actually do it every night. Luckily, winter vacation starts on Friday so that should really cut down on the number of viruses that Lemon is exposed to, and also should increase the opportunities he has to eat. I can't say that I'm shocked that he doesn't eat much lunch, given that the school lunch period is 15 minutes. But, if he's at home and can graze on home-made holiday treats all day long for two weeks, we may get somewhere.

We also found out that it was time for everyone's favorite part of the clinical study that Lemon is participating in: stool sample collection. Even better--they wanted another sample from the non-CF sibling. This is the first time that I've had to collect samples from two toilet trained individuals, who, I might add, are at peak scatological humor. It make take our household several weeks to recover from the hilarity of scientists asking me to place a plastic "hat" in our toilet to catch the precious specimens, followed by the process of scooping the right amount of the goods into a little vial and putting the vial in a bag, the bag in the container, the containers in the mailing envelope and, of course, the biggest question, "Will the mailman know it's poop?"

That excitement behind us, I also signed Lemon up to participate in two more tests for the study, a multiple breath washout test and a chest CT. I'm always glad to participate in whatever research we can, because this is the way forward for the whole CF community. For these tests, though, I'm also really curious about the results. These are tests we wouldn't normally have access to as part of routine care, but I hope they will give us a more complete picture than we've had before of what's going on in Lemon's lungs. That is, if I can convince him to lie still for 20 minutes in a CT scanner. Wish me luck!

Week 277: Perchance to dream

The nights are long at this time of year, so you would think everyone would be well rested. I think every parent of a kid with CF will tell you that a good night's rest is hard to come by. Lemon had to give up his nap when he started kindergarten. Yes, he was still napping at age 5. My totally non-random sample of other CF parents is pretty consistent--kids with CF need a lot of sleep. Anyhow, since he gave up his nap, we now have to wake him up every morning so we can do everything we need to do in time to start our day, which kills me. And that's after 11 hours of sleep. Today, there was no school, so Lemon was home all afternoon with his brother and the babysitter. And of any possible activity he could have chosen, what did he pick? A 2-hour nap. As in he voluntarily walked to his room, lay down, and didn't come out for 2 hours.

Here is another unexpected source of sleep disruption: the kids decided this weekend that they wanted to rearrange their bedrooms. Both of their beds had been against one wall of their rooms, and we rotated them 90 degrees so they were against the other walls. Presto, a whole fresh look on life. One small issue, which we hadn't thought about when we made this change: Lemon's pole and formula pump were to his left in the old configuration, and are on his right in the new configuration. So, last night, his formula pump kept alarming because he was rolling over on top of the tubing and cutting of the flow, something that almost never happened in our old configuration. Luckily, the only person who is woken up by the alarm is me (loose definition of "luckily"). We're trying again tonight, so fingers crossed that even though the pump doesn't wake him up, maybe it reprograms his subconscious somehow so that he'll start rolling in the other direction.

Unrelated to sleep, but there's another interesting thing that happened recently. We have a copay card for one of Lemon's medicines. Yay! We've had the same card, with the same numbers on it, for several years. Awesome! The pharmacy keeps those numbers on file so that the discount is applied every time that we fill that prescription. What could be better? Well, the company that makes the medicine sent us a letter, on a single 8.5*11 piece of regular paper, saying that, great news, they're making "improvements" to the copay plan to serve us better (ominous). No significant hints at why the new plan will be better, or anything like that, just that it is now better.

At the bottom, in an area about the size of the surface of a matchbook, are some numbers that look like the numbers on our card. I think nothing of it, because I assume they're just reprinting the numbers on our card so that we know the letter was intended for us or something. Luckily, Papa Bear has a more suspicious mind than I do, so he got out our old card and discovered that the only discernible "improvement" in the copay plan is that we now have some totally different numbers than the ones we used to have, even though there was nothing in the letter to the effect of "Hey, guess what, here are your new and improved numbers that you will need to use to get your new and improved benefits!" Being the crafty sort, Papa Bear excised this matchbook-sized area from the letter and affixed it to our old copay card with tape so he will have it when inevitably we are denied our copay benefit because our numbers no longer match what the drug company will have on file. New and improved, I tell you.

Week 276: For the duration

With Papa Bear safely back home from his recent trip to Copenhagen (yeah, don't cry too many tears on his behalf), we are FINALLY done with the 2018 travel season. We are all staying right here until sometime next year. It has been a rather dizzying fall but, somehow, we all made it through.

In health news, both kids seem to have contracted yet another cold. How many different respiratory viruses are there in Wisconsin? The answer appears to fall somewhere between "several" and "one trillion." Lemon did have a self-advocacy breakthrough on Friday, though. After a day of coughing (a lot, but by far not the worst we've ever had), I set up his formula as usual. He eyed the bag nervously as I hung it, and said "Mom, that looks like too much formula. I might throw up." So we skipped it. And do you know who did not puke on Friday night? Lemon. And guess who didn't have to wash any puke out of any sheets on Saturday? Me. Win all around. Except for weight gain, of course, which remains non-existent. But, hey, it's hard to win on all fronts at the same time, especially in the winter. I'll take a partial victory.

In other news, Vertex released some great data on a Phase III trial of their triple combination therapy (the one that would help Lemon). You can read more here. They saw big improvements in lung function of people with one or two copies of the common F508del mutation. They are still waiting for the results of a Phase III trial with their second triple combination before deciding which (or both) drug combinations to submit to the FDA, probably late next year. And then, hopefully, they will begin enrolling a pediatric trial, and hopefully we will be in it!

Speaking of being in it, I forgot to mention a couple of weeks ago something interesting that came up during our appointment with GI. The GI doc asked me if Lemon was eligible for any of the current Vertex drugs, and I said no, but that I was hoping to get him enrolled in a future trial of one of the triple combinations. And he said, "Oh, good thing we got his LFTs back in the normal range, then, and we'll have to keep an eye on that so he stays eligible." And you know, I've read the enrollment criteria for the trials many times, and I know that they require the liver values to be at or near normal, and somehow I had never connected the dots in my head that Lemon would not have been eligible before we got him on ursodiol, because his liver values would have been considered too high. Huh. Interesting that our CF clinic wasn't thinking about that. Anyhow, here's to having a variety of different specialists on the care team.

Somehow I've spent an inordinate amount of time this week planning for both Christmas and next summer. Coordinating the kids schedules, figuring out how Lemon can do a little 3-day art program over the holidays without my having to train anyone new on doing enzymes, trying to decide who will be doing what in the summer when almost any camp will take someone who is 5 and almost no camps will take someone who is 3...the party never stops!