Week 336: Playing possum

Nothing quite gets one's blood going on a Monday morning like one's children calling excitedly "Mama! Come quick! Something furry! In the living room! I think it's a rat!" Upon further inspection, there was something furry. Most thankfully, it was not inside the living room, but rather visible through the living room window. And, it was not a rat, but an opossum. A really, really huge opossum. We watched it, it watched us. And then we went about our respective days.

Other than that, I am thankful to report that it has been a fairly uneventful week out here on the frozen tundra. We have continued along with our manual chest PT regimen, and have another two weeks or so remaining before the repeat chest X-ray. We are just fervently hoping that Lemon does not catch something else in the mean time. The viruses are flying fast and furious in Wisconsin at the moment, so much so that the children's hospital took the precaution of banning all visitors under the age of 12 from the inpatient floors. Not exactly a comforting feeling. But, the kids still have to go to school and such so we just try to emphasize good hygiene and hope for the best. Well, that and I handed Lemon's teacher a giant 4-pack of sanitizing wipes this morning before school. We'll refer to that as active hoping.

I got Lemon on the scale again this evening just to see where things are and he seems to be holding steady for now. Still not quite as heavy as he was at the end of August, but no longer as light as he was right after all the recent illnesses. I would love it if we could get a little bit more weight back on him, but honestly just holding steady is a pretty good accomplishment for the winter months. And, to be fair, we are holding steady at something like the 75th percentile for BMI so really not too shabby.

I'm feeling good that January is almost over, so only another couple of months of winter to go. Amazingly, of course, that means the complex coordination of summer plans has already begun, complete with signing up for camp, planning of vacations, etc. Hard to believe that in less than 6 months we will be back on the beach, and Lime will turn 5. Things do seem to move right along!

Week 335: Tenure

As you all know, I do try to keep this blog by and large about the little citrus folks, but every once in a while we larger people can intrude on the narrative. And, I feel like if there were ever a week that such an intrusion would be justified, it would be this week, where I can share the great news that Papa Bear is now officially a tenured professor! He worked incredibly hard for this and it was well-deserved (and a surprise to no one) but nonetheless a tremendous accomplishment and we are all very proud of him.



Of course, on the morning of the tenure announcement, where were Lemon and I? At Children's hospital, of course. Where else would we be, really? We had our annual follow-up with GI. As always, more questions than answers, but here is what we know. Lemon's liver ultrasound has findings that, in an adult, would be consistent with "mild fibrosis." Because the technology is so new, there is no accepted pediatric standard for the measurements. So, no one knows how to interpret it really. But, between that and the bloodwork the doctor believes that everything is normal-ish, for a kid with CF. So, we were told to basically stay the course, continue on our current dose of ursodiol, and repeat the ultrasound this coming summer, along with a repeat of the bloodwork. I am going to take the whole thing as a case of "no news is good news" and move along.

We have been continuing with our daily regimen of manual chest PT in addition to the vest in an effort to get the collapsed area of Lemon's lung to reinflate. I really wish we had some interim way to tell if it was doing anything. Or some way for me to know if I am doing the PT in the best possible spot. But, of course, no and no. So, we are doing our best and we will see where things stand next month. I am trying to line up a second doctor to look at the x-ray and give us an opinion on how to best move forward based on what we see there, so stay tuned on that front.

Other than that, we are just keeping our fingers crossed that we can avoid illnesses for a few weeks at least, so Lemon can get some more weight back on and so forth. Not going to school certainly helps. I feel as though there were just two weeks of vacation, and yet somehow Lemon only has school 3 days this week. I know, I know, I have turned into a curmudgeon. But I came by it honestly.

Week 334: Seen in June

Nothing like a little CF drama to start off a new year. So, as you loyal readers will recall, about a month or so ago Lemon had a chest CT as part of a research study that he is in. And, that, to my disappointment, the CT itself would not be shared with our clinical team, but rather that they would just be notified in the event of "significant findings." Well, guess what, there were significant findings. 

The significant finding is RML atelectasis, or an area of the right middle lobe of Lemon's lung that has collapsed. This is, of course, not great news. The recommended course of action is to do a full month of manual chest PT (focused on the right side) in addition to our regular airway clearance regimen to try to clear the collapsed area and get it to reinflate. We will get a chest X-ray after the month of extra therapy to see if that has happened. If the area does not reinflate, it may be that we will need to have a bronchoscopy to try and reinflate it. 

None of that is ideal, of course. What is far less ideal is that, after receiving the report from the research CT, the doctor who is the head of our CF center went back to the last X-ray that Lemon had done (IN JUNE, MIND YOU) and said that actually, he could see evidence of the area of collapsed lung there, too. This does seem to beg the question of what we are doing the annual X-rays for, if not to catch things like this early, when we have the best chance of intervening before lasting damage is done. As of now, the doctor estimated that we have a 50:50 shot at getting the area to reinflate. He also said something about how good it was that we caught it early. Early? We could have caught it 6 months ago, apparently, and we wouldn't have caught it until 6 months from now at our next scheduled X-ray (if then!) if I hadn't decided to sign Lemon up for that CT. So I hardly view this as an occasion for self-congratulation.

Suffice to say I am very disappointed by how this has all unfolded, and am definitely planning on getting an additional opinion once the next chest x-ray is done. Luckily a new pediatric pulmonologist has joined our CF center in the interim, so I will start there.

Meanwhile, we are following up with the GI doctor on Friday of this week. Now, of course, I am really wondering about the conflicting reports that I got on the results of our last liver ultrasound, which were either "normal" or "mild fibrosis" depending on who you ask. At this point, I am very interested to hear directly from the GI doctor.  

So, not really the start to 2020 that we were hoping for, but at least Lemon is feeling fine and has managed to gain back some of the weight he lost in December, so we are in a better position for whatever lies ahead.

 

Week 333: Decade

We have now entered a different decade than the one the kids were born in. Wild. Of course, in some ways it felt like the past two weeks were about 10 years long, so maybe I shouldn't be surprised.

The past week was sort of a blur of semi-working, semi-juggling the kids, and trying to maintain everyone's sanity for a week without any regularly-scheduled programming. We finished up Cayston after 10 days as hoped, which is great, an I am glad to report that Lemon's symptoms have not returned so I think we kicked this little flare-up. Of course, at a price. Lemon's weight is now down to where it was in August of 2018. Not exactly the kind of throw-back that one wants to see. Hopefully we can catch some kind of a break here the next few weeks so that we can bulk him up a bit again, given that it is only January, meaning February and March still need to be contended with.


After two full weeks with a near-complete absence of routine, we are struggling to get everyone back on track. In some sense, having the rigor of the CF routine always running in the background has been helpful, in at least giving some form to each day. Still, I realized this morning that I had basically forgotten how to pack lunches, and also couldn't recall what time Lemon's school lets out on Mondays, it being early release day. Luckily the babysitter remembered. And I managed to pick Lime at the correct time despite forgetting and then remembering in several consecutive 15-minute intervals. We'll get the hang of 2020 yet.

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...