Week 332: Seeing Red

Ah, another Christmas complete. Generally speaking, we had a lovely time. Grandma and Grandpa were in town from New York, many fabulous gifts were exchanged, too much delicious food was consumed, movies were watched, quality time with family was enjoyed by all. And yet, as always, there were a few little wrinkles.

First of all, we decided to start Cayston on Christmas Eve. I mean, what holiday season is complete without a course of strong antibiotics? It was definitely the right decision, though. Lemon was pretty sick, had been sick for a week with no real sign of improvement, and the cough began to improve pretty much immediately after we started. Fingers crossed we will be able to get away with a 10-day course this time, and have him back at "winter baseline" in time for when school resumes next week. Of course, we have lost yet more ground on nutrition that we now need to make up. Luckily he is well enough now to tolerate the regular volume of tube feeding overnight, so at least there's hope that we may at some point get back to where we once were.

In other minor health crises, Grandpa managed to cut himself pretty badly with a kitchen knife and went to the ER to seek medical attention (verdict: tetanus booster and prophylactic course of oral antibiotics). Grandma caught a virus from somewhere or another and unfortunately wasn't feeling that great for the latter half of their visit. At least the conjunctivitis didn't set in until they were safely back in New York (no, I am not making this up).





Then there was the slime. The boys got a set of new, weird Play-Doh like things as a gift. One of them was called Super Cloud, which is basically somewhere between slime and silly putty. At some point, Lime decided to smear a handful of it on Lemon's shirt. The babysitter, who was with them at the time, took the not entirely unreasonable step of taking off the shirt and tossing it directly into the washing machine, unbeknownst to me. Then, the next day, I decided to embark on one of my favorite thankless tasks, washing all the covers from the cushions on our couch, which get kind of grimy due to the number of forts that are built around here. So, I threw a load of cushion covers into the machine, ran it, and was thrilled to discover that they all came out covered in blotches of red slime; blotches that were impervious to isopropanol, acetone, dish soap, and, well, everything.  Fabulous. A call to the company resulted in such helpful comments as "It got on the couch? Really? I will have to have a specialist call you back." Because who would have thought that a gloppy product marketed to preschoolers could ever come into contact with a sofa?

In any event, upon noting that the product did seem to soften up in hotter water, I took a risk and ran the cushions covers through the washing machine again with warmer water, and that appeared to get rid of the splotches. Or, it spread the red slime in such a thin, even layer on our red cushion covers that it was not noticeable. Either way, our couch is cleaner, reassembled, and not permanently disfigured. And the Play-Doh company is sending an age-appropriate game as compensation for our troubles. They asked if I wanted a replacement Play-Doh product, to which I replied with a very emphatic "NO!"

So, that was our holidays in a nutshell. And, onward to 2020, where my greatest wish is for the pediatric approval for Trikafta to come through. Fingers crossed, everyone!

Week 331: Ho Ho Home

And just like that, the holiday week is upon us. Naturally, it would not be a holiday week without some manner of illness cropping up. Lime has had some kind of fairly assertive cough for, oh, I don't know, a week? It is hard to keep track of these things. Anyhow, we kind of thought perhaps it had skipped Lemon this time around, but no, it had not. He's had a pretty solid cough since Tuesday, and had to miss his class's winter show on Wednesday since he just was not well enough to go to school. Generally speaking, he is pretty feisty and does not like to miss things, so when he was lying inert in bed and could not be persuaded to go to school, even later in the day after I let him stay home for the morning, I knew things were serious.

He's been gradually recovering, although the cough has been pretty severe. For the first time in a long time, I had to actually stop the vest and nebulizer mid-treatment and let him take a break for a few minutes, because he was coughing so hard he couldn't tolerate it. I think/hope he is gradually starting to get better, even though he still doesn't really sound great. We'll give him another couple of days before deciding about Cayston. Because, of course, I'm thinking, "well, if we do have to do Cayston, Christmas vacation is an ideal time since he doesn't have school for two weeks." Because this is the calculus we do in CF households. And also why we don't travel in the winter.  We seem to pick up enough things without spending time in pressurized contagion tubes (aka airplanes).






As usual, Grandma and Grandpa braved the odds of modern air travel and made their way here from New York. We've been enjoying a quiet visit with them so far, taking in a few Star Wars movies, reading, playing with toys, etc. Meanwhile, Papa Bear and I have been gradually converting our office into an Amazon Prime distribution center, or at least that is what you would think given its current appearance. Our boys are very lucky to have so many exceptionally generous people in their lives. Thanks to a certain grinch in their family tree (ahem), they are at least aware that the gifts come from the people in their lives, and not some bearded fellow with a sleigh.

I hope all of you are enjoying whatever holiday you are celebrating, in whatever manner you are celebrating it. And, I hope you are steering clear of sources of contagion and practicing good hand hygiene! Because, really, nothing says holidays like the abundant use of hand sanitizer.

Week 330: Check it up

Another week, another unusual first--Lemon's first CT scan. We spent many happy hours at the hospital on Wednesday for our quarterly check-up. As usual, there were many people to see. First, the respiratory therapist who did the pulmonary function test. Then the social worker. And the nurse-practitioner. And the dietitian. And the other respiratory therapist. And the nurse who does the throat culture. And (surprise!) the phlebotomist, because unbeknownst to us we were doing liver labs. And the research coordinator. And the guy who ran the CT machine. So, it was a big day. But, here are the upshots.

I talked to the nurse-practitioner about Lemon's cough. I have felt like this fall we have spent precious little time at what I would consider "winter baseline." Most of the time, we've been hovering somewhere above it. And yet, we've only done Cayston once. So, I was starting to wonder if we were under-treating things. But, the nurse-practitioner told me that it's been a rotten fall for a lot of families, and that they have a lot of kids in the hospital right now as a result. And, she thinks we're doing fine. Despite all of the illnesses and associated reductions in tube feeds, Lemon has actually more or less held his ground weight-wise since September. Of course, he grew some so his BMI went down, but not by much. So, our instructions from a respiratory perspective are to keep on keeping on, so to speak. From a nutrition perspective, we are to add yet more DuoCal to our tube feeds, to see if we can actually gain some ground rather than just treading water. So, we've started that and will see where it takes us. 

Lemon was surprisingly calm about the surprise lab draw, and I'm glad to report that the results we got back were great, all of Lemon's liver function tests were more or less normal. We have a follow-up scheduled with GI for next month, so we'll see what the doctor wants us to do now. Lemon's throat culture also came back with no surprises, phew. 

The CT scan was actually really fun for Lemon. He liked that we got to go on an extended walk through the bowels of the hospital to get to the room where the machine was, and he was amazingly cooperative about holding his breath and staying still. I think he got a big kick out of talking to the technician through the intercom while the scans were running. And, luckily, the whole thing only took about 5 minutes. Unfortunately, the CF team doesn't get to see the results of the scan, since it is a research scan and not a diagnostic scan. The research radiologist will tell them if there are any unusual findings, and if there were, then we would have to get a diagnostic scan to follow up. So, that seems like kind of a waste to me (I mean after all, presumably the research scan is good enough to actually see/tell something, right?). But, anyhow, I suppose if all we learn is that the findings are not unusual, we do have one more tiny bit of information than we did previously.

Other than all that, we are now in the full headlong rush to winter break, where we alternately have fun and wonder what we will do with the kids with two full weeks off from school. I'm sure we'll come up with something, the only question is what...

Week 329: Finals

I know, I'm getting a bit old for this, but I have a final project for a class due tomorrow. So, I will keep things brief for this week. Overall, it has been a good one. I think I managed to teach Lemon's class something about genes. Honestly, even if they only remember the first point of my little presentation, which is that "jeans" and "genes" are two different things and that "genes" are your body's instructions that are made out of DNA, I will consider it a success. And, I had a fun time reading Lime's class a book about large numbers. So, all in all, a good week for spreading the nerd vibe among young people of impressionable ages.

We are gearing up for Lemon's clinic visit on Wednesday morning, which will include a chest CT as part of the clinical study that he has been a part of since he was born. It is in fact his last visit as part of that study, which is kind of an amazing milestone. I am looking forward to figuring out what other things we can participate in to contribute to CF research going forward. In the mean time, here are some pictures from the week,and back to my final project for me!

Week 328: Week of the week

Happy Thanksgiving, folks. Hope you had a good one. I am glad to report that we had a very nice one here in Wisconsin, just the four of us plus Nona and Opa. We were happy to just hang around and enjoy a few days off together, livened up with trips to see Frozen II and the aquarium in Milwaukee.

We also took advantage of the long weekend to get the boys ready for being "Explorer of the Week" (Lemon) and "Child of the Week" (Lime) at their respective schools. What possessed me to sign them both up for these honors on the same week escapes me right now. In any event, we printed photos, decorated posters, gathered objects to show the classes, etc. I also am going to Lemon's class on Wednesday to give a presentation on genetics. I will steer clear of CF genetics, of course, and focus on more benign and funny traits like tongue rolling. Still, I suppose it's good to plant the seeds of understanding now, so that when Lemon and his peers are ready to learn more about CF, they'll have the basics down.

Papa Bear and I also watched a new documentary ("Love, Antosha) about Anton Yelchin, an actor who was killed in a tragic accident in 2016. What many people did not know about him was that he had CF. It's not a major focus of the movie, mainly because he kept it so private, but it is still amazing to look at the body of work he produced and know what he must have had going on in the background to sustain it all. One sort of amazing fact that I gleaned from the movie was that Anton's parents somehow managed to keep it a secret from him that he had CF until quite late in his childhood. I really don't understand how they managed that, given the need for so many doctor's appointments and therapies. I also have to wonder (it wasn't discussed at all in the movie) how Anton felt about finding out so many years on that his parents had kept such a secret from him. He was very close with both is parents, and there was nothing in the latter portion of the movie to suggest that the revelation of that secret had had a negative impact on the relationship, but I find that difficult to imagine.







Health-wise things are OK, I think. Lemon still has a little bit of a residual cough, but seems to have plenty of energy and is tolerating his full tube feeds (knock on wood!!!) so we may be OK for now. We actually have our next clinic visit coming up a week from Wednesday, so we can see if they have any new winter insights for us. In the meanwhile, we will just try to stay vigilant and hopefully but a bit of the lost weight back on so that we have some kind of buffer going into the heart of winter.

Week 327: Bear hunting

I am sorry to report that the cold that began last week has stuck around. Lemon developed a pretty bad cough over the course of the week. "Luckily," school was closed on Thursday so we had him at home to do extra therapy, and then decided to keep him home on Friday also since he was feeling fairly miserable. Even with all the extra therapy every day through today, he hasn't quite managed to shake this thing off yet. Which of course means a week or so of essentially zero tube feedings, since he's been coughing too much to tolerate them. Which of course means that as of this morning's weigh-in, he's lost essentially all the weight we put on him in the summer. And December hasn't started yet, never mind the whole period encompassing January-March. So, I'm not feeling like we're in a great position, nutrition-wise, at the moment.

The silver lining, if you could call it that, is that I do think Lemon is starting to turn the corner on this without us needing to resort to antibiotics. I've been hesitant to start them for all the usual reasons, compounded by the fact that he was just on them within the last few weeks, and I don't like the idea of having two courses so close together. I mean, if he needs it he needs it, but I'd rather him not need it and I am hoping that in this case, he won't. We decided to defer the decision until Wednesday, which is the first day of the kids' Thanksgiving break. If he is still in a bad way then, we will start, since we will have 5 consecutive days at home where we won't have to do the whole running over to the school in the middle of the day to administer medications thing.







Lime, meanwhile, is largely over it although he still coughs occasionally. And I lost my voice yesterday, but fortunately it is more or less back, given the number of work phone calls I have tomorrow where it would be highly advantageous if I could actually speak.










Beyond all the health things, the big event of the past week was Lemon's school conference. I'm glad to say the behavioral issues that his teacher was concerned about at the beginning of the year have lessened. Not gone away entirely, of course, but at least are now within the bounds of what can be expected of a first-grade boy. Maybe just barely within the lower bound, for some things, but as they say in tennis, the lines are in.We still have a lot of room for improvement, and I am still committed to the idea of finding someone to work with Lemon on some of these things, just as soon as I have the time. Whatever his behavioral issues are, they have not interfered with his progress on reading, where he has already achieved the target reading level for the end of first grade, woohoo!

Week 326: Shiner

If the week doesn't involve a bad cold and a black eye, is it really a week? These are the questions one ponders as the second half of November commences. Lime is mostly over the cold now, still prone to occasional coughing fits but no longer sounding like an alien and more or less his usual demeanor, all for the low price of several low-sleep nights this past week. Of course, while he was feeling the most poorly, there was an incident that involved Lemon, Lime, and the corner of a table. Accounts as to the exact nature of the incident differ, but all parties agree that it involved the corner of Lime's eye and the corner of the table coming into contact with a certain degree of force. So, if you want to know why my child looks like he was in a bar fight, well, now you know as much as I do.

Although I'd held out hope that perhaps Lemon would escape this cold, since he didn't come down with it right away, it appears those hopes were in vain. He started sounding like an alien yesterday, and was pretty miserable when he got home from school today, coughing and exhausted. He had a mild fever this evening as well. So, I loaded him up with ibuprofen before bed and am hoping against hope that maybe he'll just throw this off in a day or two. Fingers crossed. It's tough knowing that the two possibilities for CF and a cold are either getting over it quickly or it kicking off some much more involved cycle of illness. We've been very lucky of late that we've been able to dodge that latter outcome more often than not, but that doesn't mean we've forgotten that it's out there as a possibility by any means.

I did finally hear back from our clinic on ranitidine. The response was that they are only changing to a different medication for patients who are on the liquid formulation, and for patients who take pills, like Lemon, they are staying the course unless the patient prefers to switch. So, I suppose we will stay the course for now. I am very reluctant to make any changes to Lemon's digestive regimen since it took us so long to get things working right in that department in the first place.




Other than that, I feel that this week provides a good sneak preview of the childcare situation for the coming weeks. Today, the nanny was sick. I fully anticipate that tomorrow Lemon might be home sick. Lime's school is closed Wednesday - Friday. Lemon's school is closed Thursday. And next week is Thanksgiving. A related question--since when does Christmas start immediately after Halloween? Is this a new phenomenon? I feel like for a majority of my life it had the decency to hold off until after Thanksgiving, but maybe things have changed. At least I am not alone in the household in wishing that fall would stick around a little longer--Lime insisted on listening to Halloween music before bed tonight!

Week 325: Snowvember

Who doesn't love a little Monday morning snowstorm in mid-November? As an added reminder that winter is upon us, Lime has his first serious cold of the year. He has a cough that reminds one of a wounded seal, and alternates between thinking that it is hilarious that he "sounds like an alien" and being full of woe and self-pity. I am in fact writing this post from his bed, as the latter two sentiments appear to be preventing him from falling asleep. So far (fingers crossed) Lemon seems unaffected and is sustaining his winter baseline of a few coughs and frequent throat clearing.



Of course, when our nanny showed up today, she had a bit of a cold, too. At one time in our lives, I probably would have sent her back home to avoid exposing Lemon, but a) see above re Lime, and b) given that Lemon spends every day in the disease incubator known as an elementary school, I can't get too worked up about him being around someone who has a cold but also understands basic hygiene.






In pharmacological news, some of you may have read that ranitidine (aka Zantac), a medication Lemon has been on for almost 6 years, has been recalled due to contamination with another chemical. We heard about this through the news, and our pharmacist gave Papa Bear a handout about it last time he picked up prescriptions. Who haven't we heard anything from? Our care team at the clinic, who ask me every single time I set foot in the building which medications Lemon is taking. I have a message pending with them to see if we should be switching to something else. Of course, we'd rather not mess with Lemon's digestion, which has been going along fairly well for the last couple of years. On the other hand, we'd rather not be giving him a contaminated medication, either.

Speaking of digestion, we may finally adjust Lemon's enzyme dose again. He has been doing a single 36,000 unit capsule with meals and snacks for ages, but at our last clinic visit our nurse-practitioner mentioned that he is big enough that two capsules is actually an appropriate dose for him. He's been mentioning stomach aches off and on over these past couple of weeks, and it occurred to us that maybe we really should be doing two capsules at least with larger meals. Lemon is very sensitive to pill burden, though, so we will have to sort of ease him into it gradually. He also does not like taking his 4 evening pills after dinner, so today we had a discussion about switching two of them (his vitamin and iron) to the morning. He was in favor of it so we will give it a try and see how it goes. I feel like there is so little he can have control over, it is important to honor his feelings in those few areas where we do have some wiggle room. His behavior has been a lot better of late and we'd like to do everything we can to keep it that way!

Week 324: Georgia on my mind

Yet another big week in our household, featuring Halloween followed by my quick trip down to Savannah, GA to run yet another marathon. I'm pleased to report that everything went well, thanks in no small part to Papa Bear who was both chief costume engineer and also managed to keep the household running smoothly in my absence.







Mother nature did try to throw a little bit of a curve ball into our Halloween by dumping 5" of snow on the ground in the preceding days. But, we Sconnies are made of pretty solid stock, and besides, the need for candy trumps cold weather any day of the week as far as certain residents of my household appear to believe.

We had hoped to just do 10 days of Cayston this time around, but I felt that at 10 days we were still not all the way back to Lemon's winter baseline, so we kept up through day 14. I'm feeling good now that that was the right call, because after 14 days we really are back at baseline, no more coughing at night. Hooray!

 I also got to see one of the cutest little moments played out in the nurse's office for several days last week. I would pull Lemon in to the office as he left the cafeteria, and during the second week of Cayston another little girl from Lemon's class was also stopping at the nurse's office after lunch to do an inhaler. Every day, he would wait for her to finish her inhaler and then the two of them would walk back to class together like little breathing buddies.I think it made him feel like the whole thing was so much more normal, since someone else from his class was doing it too.










I can also report that Savannah is a really cool city. I had high hopes for the trip after reading "Midnight in the Garden of Good and Evil" (recommend, if you haven't yet) and Savannah did not disappoint. It has amazing trees and parks, tons of history, a river, and a pretty darned nice marathon course if you're so inclined. I'm really glad I made the trip, and also really glad, after a few weeks of non-stop travel,not to be traveling again until 2020!