Monday, December 4, 2017

Week 224: Zippy

Every now and again, maybe about 2 weeks out of every 52 it seems, we get a week where nothing much happens. And man are those the best weeks ever. Lemon and Lime both recovered from the cold they had over Thanksgiving and stayed well for the last 10 days or so. It's amazing what 10 days of good health will do for a couple of boys. By that I mean that their energy level is such that Papa Bear and I have serious concerns as to whether our house and worldly possessions will survive the onslaught. One afternoon this week when Lemon was being particularly "energetic," we at first wondered if something was wrong. It turns out, actually, that something was right--it had been a week or so since he'd last vomited, so he was finally feeling caught up on sleep and nutrition and boy did it show.

I'm glad Lemon was full of pep for his week as "child of the week" in his class at school. He got to wear a crown every day, and have a poster with pictures of his family hung in the classroom. On Wednesday, I went in and did a science demonstration for the kids, layering corn syrup, water, and olive oil in a drinking glass. I think it went well, although thus far Lemon has declined to recount the experience for anyone. After some prodding he is willing to concede that I was in his classroom, but that is about it.

Papa Bear was out of town for a couple of days this week, so at the kids' request I took them on a drive "at night time" (ie 4:45pm) to see the holiday lights around our neighborhood. Since they almost never leave the house when it's dark, this was a profoundly thrilling experience, which we topped off by picking up some take-out food from a restaurant, another cause for high excitement. I couldn't get any traction with Lemon on the chicken satay that I got for him, but weirdly he adored the cucumber salad that came with it. In other news, his relationship with food continues to mystify everyone.

Lime appears to be going through some sort of growth spurt, and I've temporarily conceded the point that he may take a sippy cup of milk to bed with him. The old me would have not been in favor of this idea at all. The new me, on the other hand, recognizes the inherent benefit implied by not getting up to pour a glass of milk at 3 a.m. He's almost up to 25 lb, so who knows, may be by his 30 month check-up in January he will have clawed his way out of the 4th percentile for weight. His little personality is getting more assertive by the day, and he and his brother seem to always want to be together, even when they struggle to maintain domestic harmony. But, being alone is boring. The cats agree!












Monday, November 27, 2017

Week 223: Gobble it up

As if to reaffirm our decision to avoid flying during the winter for the foreseeable future, both kids came down with yet another bad cough on Wednesday. This, and the associated puking (which followed a solid stretch of let's say 3 or 4 puke-free days), made us question the wisdom of continuing with our planned Thanksgiving trip to Milwaukee. But, the prospect of doing nothing except sit around  the house and cough for Thanksgiving seemed too grimly depressing, so we packed up everything we own and went ahead with the plan.




















Ultimately it was the right choice and I'm glad we did it. I couldn't honestly say that it was a relaxing time, but we had a lot of fun with Nona and Opa and the kids got to experience a lot of new things. We did a lot of swimming in the pool at the hotel, went to a museum and saw some dinosaurs, and went to an aquarium to see some fish. Lime was particularly impressed with all the tall buildings, which is a good reminder that he is really a country mouse, Milwaukee being the biggest city he can ever remember seeing.

The kids were both still pretty sick on Thursday, but by Friday they were starting to turn things around, and today both of them seem nearly recovered. I have to say the one thing I am the most grateful for this year is that I finally feel like we have some degree of control over Lemon's respiratory health. Don't get me wrong, this fall has been pretty much a non-stop series of respiratory viruses of varying intensity that have affected both kids (and their adult companions to varying degrees). But, Lemon has been able to throw off all but one with just increased airway clearance and no antibiotics. It still sucks that he is sick so much, but this is nothing like the bad old days when every time he got a runny nose, Papa Bear and I knew that within 6-8 weeks we would be talking about IV antibiotics. I can't tell you how good it feels to go into a winter and not feel completely and utterly defenseless. It's only November, of course, and there is still a lot of winter to go, but we have fought hard and won a number of rounds already, and any number of wins is more wins than we ever had before.


I wish that I were feeling more bullish on nutrition, our other constant battle ground. Eating is still not really a thing for Lemon. He enjoys food school and will do a lot of things in the room with his therapist, but we haven't seen much translation of those lessons into increased food consumption at home. And, right now we have no room at all to back down on the tube feedings. With all these illnesses and all the puking, we are still at 0 weight gain since June, and that's with us pumping as much formula as we can in through the tube whenever he's well enough to keep it down. I'm starting to feel like maybe the win here is just not losing any further ground until spring, which is kind of a depressing thought but it's good to be realistic I suppose.

In more uplifting news, Lemon is "person of the week" at his school this week so if you'll excuse me, I have to go research science demonstrations that are appropriate for the 4K set and take 15 min to execute...







Monday, November 20, 2017

Week 222: In the mind

There is an old Yiddish folk tale about a guy who feels like his house is too crowded and noisy, so he asks his rabbi for advice. The rabbi suggests that the man bring a goat into the house. Predictably, this does not improve matters, so the man goes back to the rabbi. Following a succession of visits to the rabbi, the man has brought basically every animal on his farm into his house. Finally, the rabbi suggests removing all the animals, at which time the man discovers that his home is actually a peaceful paradise.

Our version of this story goes something like, oh, man, having two preschoolers, one of whom has a chronic health condition, is really tough at times. How about if one of the kids randomly starts performing toilet activities not on the toilet? Better? No? How about giving one of those kids the ability to vomit on command? No? That didn't improve things either? How about if we just return to the status quo of two preschoolers, one with a chronic health condition, but no random toilet activities and no vomiting?

You guys, it is paradise on earth over here. I have not washed a puke-soaked thing in several days. What was our genius strategy? We told Lemon that if he kept puking, we could not go on our planned family trip to Milwaukee for Thanksgiving, which involves staying at a hotel with a swimming pool, and possibly also seeing dinosaur fossils at a museum. Like flipping a switch. I can tell that he feels much better, too. After all, throwing up is not pleasant and exhausting, plus which if you retain say zero of the nutrients you are supposed to be ingesting, you feel lousy. Now he is bright eyed and full of energy again. Fingers crossed, we are done with that whole scenario.

The one problem we were still having was with brushing his teeth, where the mere sight of his toothbrush would make him gag violently, and I could only brush about two or three strokes before I had to stop lest risking whatever dinner had made it in coming back out again. I mentioned this problem at food school today and his therapist suggested a new toothbrush. I was skeptical, because I thought it was really the physical sensation of the brush in his mouth that was causing the problem, but given that we are going to the dentist TOMORROW I was willing to try anything. So, after bath this evening, I loaded up the old tooth brush, got so far as about the end of Lemon's nose, and he started gagging. I then pulled a brand new tooth brush out of the drawer, and asked if he wanted to try that one instead. He said yes, so I got it out, and lo and behold, he didn't even flinch, and in fact insisted on taking a turn brushing his own teeth after I was done.

The mind is a powerful force. The young child's mind is both powerful and capricious. Ah well. With a sufficient supply of kittens, running, and bourbon perhaps we'll make it through.



Monday, November 13, 2017

Week 221: For real

The cough that both kids have had for more than a week is starting to gradually subside. We've been doing three treatments a day for basically two weeks running but (knock on wood) have not felt like we need to start another course of Cayston yet. We are still relying on Lime as our canary in the coal mine--if he's still coughing then the fact that Lemon is coughing too seems less troubling.
I don't usually dwell on this particular topic on the blog, but this cold has brought with it a lot of puking. Even Lime has coughed to that point a few times with this particular bug, and with Lemon every meal and tube feed has been fraught with peril. Ick. Lots of gross laundry, many, many outings with my new steam mop, etc. But, on the plus side, our issue from a few weeks ago of a certain someone performing bodily functions where they should not be performed seems to have stopped of its own accord. So, although there was gross laundry, there was a dearth of other gross messes to be cleaned, which felt like progress.

However. There is always a "however." However, it seems that all this puking from having a cold and a bad cough and swallowing a lot of mucus gave Lemon a new idea. Perhaps he found that being the potty bandit did not result in the amount or caliber of attention that he had in mind. He certainly noticed that if you puke, your parents tend to sort of rush around, act extremely sympathetic, cater to your needs and wants, etc etc. Those of you familiar with kid logic may see where this is going. We had a few puke incidents that came without the usual warning signs, but sometimes there are no warning signs so I didn't think anything of it. Then we had one last night at dinner that seemed really sort of weird, like everything was 100% fine one minute and 100% the opposite of fine the next, where I actually said to Papa Bear afterwards that I suspected Lemon had done it intentionally. But, we both rejected the idea, because really, who does that.

Then, there was this morning at 4 a.m., when Lemon started yelling "Hello!" very cheerfully from his room. He has a stoplight clock, and he knows that he is not supposed to wake us up when the light is red unless he needs help. We'd had a couple of mornings over the past week or two where he's done this "Hello!" routine at ungodly hours and been given a stern reminder about the whole red light concept. So, I stumbled out of bed and over to his room grouchy and annoyed and ready to give the whole red light speech again. But, when I opened his door, he said, with a big grin, "Look, I threw up!" Having no choice, I sprang into action with the whole cleaning routine, while talking to him in a neutral tone about what was going on. Was he feeling sick? No. Was he coughing a lot? No. What happened? He wanted to throw up, so he did. Of course, by this time, all the commotion had woken Lime up, so we all got to start our day, which I suspect was Lemon's objective all along. 

I sincerely hope this is a short phase. Or maybe just a one-off. Throwing up isn't fun, right? No amount of getting to sit on top of your grumpy mom and discuss the relative merits of various sports cars at 4:41 a.m. is worth that, is it? 

In less gross news, we are still trying to figure out a solution to our overpriced ursodiol problem. We transferred all of our prescriptions from CVS to the children's hospital, and at least there they are only charging us $60 a bottle for the stuff, rather than $90, but $60 is still quite a bit more than the $5 we would like to be paying given that it's a generic medication. We'd like to switch to pills, which we could just crush and give through Lemon's G tube, so I've been in communication with the GI doc's office about that. Well, actually, I have been locked in a MyChart messaging war with a nurse who, how shall I say this, DOES NOT KNOW HOW TO DO MATH. Like, the kind of math you would need to be able to do to divide the number of milligrams in a pill by two and compare the result with the number 170 milligrams to determine if it were a) the same number, b) a smaller number, or c) a larger number, and to make a therapeutic decision on the basis of that comparison. For the moment, I am giving up on this war, because I lack the time and energy. We will just pay for this month and next month's bottles of the compounded stuff, and then see how Lemon's liver enzymes look in December. If the ursodiol seems to be doing something (in which case we will continue it long-term), perhaps I will be able to find a way to actually speak to the doctor, who presumably understands both math and the concept of therapeutic range, and we can work out an actual solution.

A closing note from the "height of irony" department. Lemon's Pulmozyme prescription is filled by CVS specialty pharmacy. Theoretically, it is possible to get the prescription set up to auto-refill. I say theoretically, because Papa Bear is among the most intelligent and diligent of pharmacy customers, and for over a year he has been unable to achieve this despite innumerable attempts. Finally, today, he succeeded. And, tomorrow, he will have to call and transfer the prescription to our new pharmacy. Victory!

Monday, November 6, 2017

Week 220: Changing time

We have certainly come a long way from where we were a year ago. Halloween reminded us that at this time last year, we were doing home IVs, and Lemon in fact had to go trick-or-treating wearing a backpack containing his IV pump. This year, I kept alive the "trick" in trick-or-treat by bringing the kids to get their annual flu shots on Halloween morning. Both kids were very dramatic about the whole thing, wailing about the indignity and injustice of it until I said, "Hey, that lady is dressed in a pilot uniform. And she is offering you stickers." End tears, return to school.





We enjoyed a week of relative good health here before a new cold hit the dynamic duo on Friday into Saturday. Now we're back to having our house sound like some kind of sanatorium, and poor Lime has coughed himself so hoarse that he sounds like a little frog. Lemon's voice is unaffected but he too has been coughing up a storm, with all the attendant setbacks in terms of nutrition. At this point I feel like if we just hold our ground we'll be winning--the possibility of exceeding his June weight any time soon seems remote. The good news is that in spite of having this pretty bad virus both kids are in good spirits, enjoying the kittens, and generally having a good time. The bad news is that we've had to miss food school again, the third time in 6 weeks. I am really wondering whether it's even worth continuing to pursue it in the winter, or whether we'd be better served by just waiting for spring again. Winter is just not an eating season for Lemon.

We got an upsetting letter in the mail this week: as of January 1, our insurance will no longer cover the pharmacy that we have been using since we moved here (formerly Target, now CVS within Target). When you fill as many prescriptions per month as we do (see illustrative photo), the relationship with the pharmacy is an important one, and we'd invested 4 years in this one, figuring out who the good and bad pharmacists were, getting the "auto" refill system to work the way we wanted, negotiating the copay cards and specialty pharmacy and all the rest. We're switching to the pharmacy at our children's hospital, on the theory that perhaps a pharmacy affiliated with a hospital might be able to handle a complex prescription load. Well, that and the fact that they aren't Walgreens, the other choice now offered by our insurance.

Another loss this week: the cart that we've used for Lemon's vest and associated equipment since we started on the vest reached the end of its service life this week. The kids and I had a good time putting together its replacement (thank you, Amazon, for remembering exactly what we ordered last time). It's hard to believe that we are already two years into using the vest. I recently ordered Lemon the next size up on the vest itself, since he'd outgrown the old one. Being aware of his deeply conservative nature, I ordered him the exact same color, just in a bigger size. I thought he'd be excited when the new one came, but instead he refused to even try it on. He was only willing to try it on when I promised that it was just a test, and that he would still use the old one. He did not anticipate that I would be so sneaky as to swap the old one out for the indistinguishable new one when he wasn't looking. The depths that parenting pushes you to...





Monday, October 30, 2017

Week 219: Bandit

Sometimes when I sit down to write the blog, I leaf through the photos from the week to remind myself of what happened. A thoughtful review of this week's photos indicates that we basically played with kittens non-stop. Not a bad way to spend a week, when you get down to it. We had been keeping the kittens downstairs in our office, but this weekend we decided that arrangement was too inconvenient and that they were sufficiently oriented to their new living situation that they could have the run of the house. Poor Daphne is still not totally on board with this new development, particularly since Donut likes to take a swipe at her with his paw, or bite her tail when it twitches in his vicinity. But, she is coming around and is definitely not unduly stressed. Our plan of getting two young cats seems to have paid off in that they mostly play with each other and leave her alone. Mostly.

















Today marks the end of our third week of Cayston, and with Lemon back to baseline we're going to stop treatment now and see how he does. Given that it's only October I'm sure we'll have many weeks in the not-so-distant future to do three treatments a day, so if we can take a break from that grind for a few weeks now I'd just as soon do it. We've been continuing to hammer away at nutrition, and he's finally back up over 38lb, ie, we're once again where we were in June. With any luck maybe we'll get up to 39lb before the next cold hits.






















We did have one "interesting" issue this week, which is that starting in the latter half of the week, we started noticing the occasional appearance of puddles around the house. They were too big to be kitten-related, and at first we thought they might be related to our new refrigerator with its tempting water dispenser and the imprudent use of open-topped cups, but they kept showing up in places where there were no cups, and far from the refrigerator. We observed, gathered clues, interviewed witnesses and suspects. All of which led to one conclusion: Lemon had been intentionally peeing at random locations around the house. Seriously? What is this? I was on board to do diapers and potty training and accidents. But intentionally peeing around the house? In his brother's bed? Nope. He seems like pretty much his usual self otherwise, and most of the time still uses the bathroom like a regular person. So what the heck? At the moment our only strategy is to positively reinforce the potty and hope that this behavior goes back to from whence it came, but any suggestions would be welcome!








Monday, October 23, 2017

Week 218: The scamper




I am pleased to report that somehow, we survived a week without Papa Bear. By somehow, I mainly mean that I did nothing but work and take care of the kids and went to bed at 8:30 every night. I may also have eaten cereal for dinner every night, it's kind of a blur at this point. But, every treatment got done, every diaper got changed, and the trash got taken out. So, victory.

We did do one extra fun thing this week, just to keep things interesting. As long-time readers will
recall, we originally had two cats, Daphne and Denzel. Sadly, Denzel passed away about 18 months ago, and my life has just not been complete without an orange boy cat in it. For whatever reason (sleep deprivation?) I thought that this would be a good time to bring a couple of little scamps into our household, so on Wednesday I picked up two little rescue kittens to come join the circus here in our house. The kids and Papa Bear had picked out the name Donut for one of the cats, so naturally I decided the other one (my sweet orange boy) should be named Duncan. They are pure joy. And Lemon is so in love with them. I suspect they will grow up to be VERY tolerant cats, since he will, without warning, scoop them up by their mid-sections and tote them around, put them down his little slide, have them "jump" on his trampoline. Daphne is still getting used to the idea that there are some new residents in the house, but I imagine she'll come around eventually. She did eventually accept the kids, after all!

In other news, some of you may remember that a while ago I finally managed to worm my way into the Cystic Fibrosis Foundation to do some medical writing for them. I'm very pleased to announce that some of my content has now made it onto their website. Feeling the need to brush up on some basics about the CFTR protein? Maybe you're interested the different types of CFTR mutations? How about CFTR modulators? Check out these all-new sections of CFF's website. I'm glad to say that the Foundation has asked me to keep going and develop some content for them about "New Frontiers in CF Research," so watch this space.








On the health front, we are done with two weeks of Cayston. If you had asked me a couple of days ago, I would have told you that we would be stopping Cayston after 2 weeks, because Lemon seemed really well. Unfortunately, Lemon, Lime, and I all caught some other new cold, hard on the heels of whatever the kids had before. So, Lemon is once again coughing up a storm. I'm quite convinced that it's just the new virus and not anything bacterial, but it seems pretty dumb to take him off the antibiotic when he's actively sick. After making up a little bit of precious ground in the past week, we're once again cutting back on tube feeds, dealing with puking, etc. This does make me feel better about all the boluses I pumped into him last week when he was well, though--we need those precious ounces of body fat to help him fight off this new cold, and hopefully we'll only get pushed back to where we started from, weight-wise, and not further into the hole.

We did cross an important milestone this week: it has now been one full year since Lemon was discharged from his most recent hospitalization. We are definitely in a stronger position than we were a year a go, and we've learned some important lessons about managing Lemon's health that have helped us to achieve our hospital-free year. I think the key lessons for us have been the importance of nutrition, hydration, and inhaled antibiotics. A year ago, we were so focused on the respiratory symptoms whenever Lemon got sick, and were not as attuned to the nutrition/hydration angle. Last year's hospitalization really showed us what a difference even just staying hydrated during an illness can make for him. We also got away from our long-standing intervention plan of oral antibiotics, in favor of inhaled. I won't lie, inhaled antibiotics are a pain. Three treatments a day gets old for everyone really fast. I long for the simplicity of shoving a few mL of a liquid medicine through a G-tube twice a day. But, much as I long for it, that plan never worked for us. And if inhaled antibiotics ever seem like a big deal, I just remind myself what doing home IV's is like, and then I remember that inhaled isn't so bad. Whatever this winter brings, we are more prepared to face it than ever before, and this hospital-free year is an accomplishment that we can all be proud of.

Monday, October 16, 2017

Week 217: Off to the races

Full speed ahead as always around here. On Monday night, within just a couple of hours of my posting the blog, Lemon's cough took a marked turn for the worse, so we decided we had no choice but to start Cayston on Tuesday morning. It was definitely the right call, since he started perking up almost immediately, and I'm hoping that we'll be able to get away with a two-week course instead of a month (knock on wood). Fitting in 3 treatments a day with school and work and everything else is challenging to say the least. It will definitely be a relief for everyone to get back to twice a day.
On Friday, Lemon had his follow-up with the gastroenterologist to talk about his liver. The first thing we learned at that appointment is that Lemon has lost a pound and a half relative to his weight in JUNE. That's 4 months of not gaining a thing and instead losing ground. Partly because of when we tried cutting back on tube feeds when we began food school, and partly from him being at least somewhat sick pretty much non-stop since school started, which forces us to keep the overnight feed volumes low. Now that we have this latest cold more or less under control, we are in full-court-press mode to try and get weight back on. That means 3.5 cartons of formula at night plus 100mL bolus feeds morning and night. It's a lot of formula, syringes, and fuss, but we've got to make up some ground while we can, since winter hasn't even started yet.

The next thing we learned from the GI doc was that he wanted us to start on a new medication, usrodiol, that may slow down or prevent damage to Lemon's liver. We started the ursodiol today, and we'll re-run the liver panel when we do his annual labs in December to see if it's having any effect. It better be, because a one month supply costs us $90 out of pocket (for a generic medication!). 

When we got back from the appointment, I dashed over to our local Enterprise and picked up a rental car, and on Saturday morning I drove down to Des Moines, where I met up with an old buddy from Boston. We bummed around Des Moines on Saturday afternoon and were very favorably impressed by it. On Sunday, we ran the Des Moines marathon together, capping off the 2017 running season with a personal best for me, 4:05. I didn't quite manage to break the 4:00 barrier this time around, but I think it was by far the strongest marathon I've ever run, and I have high hopes for the 2018 season. In the mean time, I'm looking forward to the off season, where I can run a little less and spend a little more time on other pursuits. Many thanks to Papa Bear, Nona, and the rest of our crew for taking such good care of the maniacs while I was away!
I'll definitely kick off the off-season in style this week, because no sooner did I return from Des Moines this morning then Papa Bear took off for a week in Greece for a conference and some sight-seeing. I'm sure I'll be doing plenty of running around over the next week, although most of it will be chasing after the dream team, rather than endless laps of the neighborhoods of Madison!