Week 180: Ever vigilent

We have arrived at a day that I have been awaiting a long time: Lemon has been completely off antibiotics for two full weeks, for the first time since September. I contacted our nurse practitioner today and it's official, we're off hospitalization watch. If Lemon gets sick again, our intervention plan will be another round of Cayston. I'm told that we have a refill on our existing prescription, so tomorrow Papa Bear and I will get things in motion to get that refill into our hands so that we have it when we need it. We are currently accepting bets on how many hours of our time it will take to get everything in place to receive the refill. I'm so glad that we've finally found something that works for Lemon, after years of mucking around with oral antibiotics that, if I really think back on it, probably never actually cured him of anything in his life. But, it does drive home the reality that when he gets sick, we're signed up for a minimum of a month of three treatments a day--we can't even pretend anymore that just pushing another medicine through his tube twice a day will do anything. Still, if it keeps us out of the hospital and away from PICC lines and IV infusions, the extra time and energy of the inhaled antibiotic is 100% worth it.

On Friday, I took Lemon to see his regular pediatrician, who wants to follow him every 6 months. Honestly, I thought about cancelling the visit, because Lemon is actually well at the moment and I wasn't sure it was worth the infection risk to even bring him to a pediatrician's office at this time of year. But, I had an important agenda item: I needed someone with an MD to fill out the prior authorization form for our insurance company so that we can get our second opinion in Milwaukee. So, we went, and I'm really glad that we did. Lemon and Lime's new pediatrician is a genuinely kind, caring person. I immediately got more traction with him on the idea of a second opinion than I did with our clinic. The clinic's line has always been, "Oh, yes, that's a great idea, we really encourage that." Which has not, since I raised the idea, turned into a shred of actual support in terms of getting there. The pediatrician, on the other hand, upon hearing about the situation, said, "Oh yes. You need this. Let me take your copy of the form and the names of the doctors there." So, with any luck, we're one incremental step closer.

The other good thing about the visit is that we got the pediatrician to put some orders for bloodwork in the system for Lemon. As you may remember, back when he had the PICC in October, we drew labs and he had really high values for a number of liver enzymes. Our nurse practitioner attributed that to the fact that he was sick, and said we'd retest in March. But, since Lemon is healthy now, and who the heck knows what will be happening in March, I got the orders put in now so that I can take Lemon over to the hospital at our leisure to get blood draw, probably later this week. It's an extra trip to the hospital with all the attendant risks, but I really want to see what those numbers are when he's well. He's also been a little pale lately, even for his usual pasty white self, so we're also going to do a blood count and see if there's anything to be seen there or if it's just the natural pallor of a Wisconsin winter.

The one piece of bad news that we got at the visit is that Lemon has actually lost a pound over the last month. I think we'd become a little complacent about nutrition since his last clinic visit, because everything was looking so good--his vitamin levels were all in the normal range, and he was gaining weight like a champ. I think we mentally checked the box in our heads that said "Nutrition plan working." And, of course, as soon as you stop paying attention, things change. Lemon hasn't been eating much at all these last few weeks, and he's been feeling great so he's been EXTREMELY active. And that has had its inevitable result. So, we're going to try increasing his overnight feed from 3 to 3.5 cartons of formula and see where that puts us in a month or so. I feel like we're due for a recalibration of his nutrition plan anyhow--it's been nearly a year now since we had the feeding tube placed, and he's grown so much since then. So, now  that we (however briefly) have pulmonary under control, maybe we can focus our next clinic visit on that.

Oh, right, one more moment of hilarity from the pediatrician visit. The doctor checked Lemon's g-tube site and then asked me who was in charge of it. I gave him kind of a blank look. He clarified, "You know, which doctor is following up on it and doing the tube changes?" I had to bite my tongue not to laugh, and he did his best to hide his shock and dismay when I said that it was my understanding that I was in charge of it, and no medical person has looked at it or changed the tube since surgery decided they were done with us.

Out there in the world beyond this blog, it has been a big week for our country. I don't want you to think that I'm unaware or disengaged. But, I have made an editorial decision that this is a blog about the life of our family as it unfolds. Writing these weekly updates is therapeutic, relaxing, and a chance for me to try to view what's happening in our lives at arm's length. Honestly, at the current moment, it's a bit of an escape for me too, to take an hour and just think about what's happened in these four walls and look at all the cute pictures from the week. I hope that reading it is and will remain an escape for you, and that it will renew your energy and your commitment to fight for the causes that you hold dear.

Week 179: Marching forward

Want to know what happened in our house this week that was CF-related and unexpected? Nothing. Yup. No news to report. We've been off Cayston a week, and so far, so good. Still very much in one-day-at-a-time mode, but we'll take it.



Lime had his 18 month check up (18 months?), and it seems that he has left his little teapot (short and stout) phase behind him. He is still short, but now he is skinny. Given how much he moves around, I can hardly say I'm surprised. The doctor recommended a high calorie diet and some extra iron. Luckily I have some previous experience with those things. In the mean time, and not as much in the background as it would seem from the blog, Lime has continued to blossom into a real little person. He has his various passions, including his dedicated efforts to befriend our cat. In the past 24 hours, he has offered her such treasures as an olive, a red Lego, a piece of a donut, and a green block, placing each offering at her feet when she declines to take it from his hands.

On Saturday, I did what I thought needed to be done and ran from our house over towards the state capitol to join our edition of the Women's March. I have never participated in a protest before, but I felt like this time I had to go. One of the top issues on my mind is (no surprise) access to healthcare. It's pretty simple--kids like mine would die without access to healthcare. And, from my very fortunate position with so many resources at my disposal, I feel like that access is under attack. If the threat feels real and immediate to me, I can only imagine how much more real and immediate it feels to those who have fewer resources. And, let's be clear--chronic disease is not a rare thing. About 1 in 2 adults has a chronic disease of some kind. Even rare diseases are not rare things--1 in 10 people in the US has a rare disease. In summary, this issue affects everybody. Every single person in the US either has one of these diseases, or has a first-degree relative who has one. We need to work as a country to figure out a way forward on this.

There has been a lot of talk on the various interwebs over the last couple of days about what the march was, what it meant, what it will mean going forward. I've done a lot of reading, and I don't have any general answers. What I can say for certain is that I have the phone numbers of my three representatives in Congress programmed into my phone, and they have heard from me in the past weeks and will continue to hear from me on a regular basis with regard to this issue. Obviously, there are so many other issues, but I am realistic--I have the bandwidth for approximately 0.8 issues, which I am rounding up to 1. I am hoping that some of the other 3 million or so of you will be out there making calls about the other ones.

A side bonus of attending the march was that I managed to connect in person with one of my best CF mama friends. It's ridiculously hard for us to get together, because we're busy people with young kids, and our kids can't be around each other lest they pass germs back and forth. So, I think I'm not exaggerating when I say that we last got together for lunch in June. And, we promised that we'd have coffee together sometime before the mid-term elections. So that's something.

Week 178: Flying blind

Humans, by nature, look for patterns. No human more so, perhaps, than the parent of a kid with a chronic illness. When did I see this particular thing before? What happened after I saw it? What did I have to do to get things right again?

Longtime readers know our pattern: sneeze goes to cough goes to puking goes to antibiotics (oral if this is the first or second or third cycle in a while, IV if we've done a few circuits of the pattern already). So, there we stood last Monday, 3 weeks into our first one-month course of Cayston, when the sneezing started. Anyone want to guess what happened next? Coughing? Puking perhaps? Yes, and yes. And, to make things scarier, not only had we already done many, many, many courses of antibiotics, but we were ON an antibiotic--the antibiotic that was supposed to help us crush whatever bug has been lurking in Lemon's lungs since September.

On Wednesday, I have to say, things were looking far from good. Lemon was pasty white, coughing, miserable, fever of 101F, huddled on the couch staring with glassy eyes at the world around him. I'd been in touch with the clinic on Tuesday to say that he was getting sick again, and the nurse practitioner and I had begun to concoct a plan. We hoped perhaps the Cayston would keep things at bay long enough to give the hospital some lead time--we wanted to coordinate several procedures at once and getting all those people in a room at the same time involves juggling a lot of schedules. We were targeting admission the following Tuesday. On Wednesday, given how bad things were looking, I contacted the nurse practitioner again and said I didn't really see how we would make it until Tuesday given the trajectory we were on--one we've been on so many times before.

The NP called and emailed and did whatever other magic there was to do and somehow got everyone aligned.  We were to come in Thursday afternoon so he'd be all ready to go for the big day Friday. On Friday morning, we would have Lemon put under general anesthesia, get a PICC placed, and have a bronchoscopy done. After that, we'd spend at least 72 hours in-patient so that they could titrate the dose of IV tobramycin, a drug he's never had before, but given that IV Zosyn didn't kill whatever this is last time, they wanted to add something new. Once everything was titrated, we'd go home and finish out 2 weeks of IV's at home, Zosyn continuously for 22 hours a day plus a dose of tobramycin every day. If this sounds like a big, daunting plan, that's because it is. And this plan does not describe the logistics of staying employed, maintaining a household, taking care of not one but two children, etc. But, the nurse, Papa Bear, and I agreed--we've been fighting this since September, and we still don't actually know what it is. So it was time to find out and kill it once and for all.

This brings us to 5:30 Thursday morning, when Lemon bounded into our room and shouted, "Good morning! It is a great day!" Most parents are ill-equipped to receive surprising news at 5:30am, and we are no exception. We looked at Lemon. No cough. Good color. Lots of energy. What? THIS WAS NOT PART OF THE PATTERN.

Remaining in the state of disorientation that occurs when the predictable chain of events is derailed, we staggered around. We did his morning therapy. Had coffee. Had breakfast. Handed the kids off to the nanny. Did some work. Listened and watched and waited for a hint of what had been going on the day prior. And there was none. Just before mid-day, I called the clinic and told them what was going on. The nurse I spoke to (not our nurse practitioner) suggested that we come in and go through with the plan anyhow, because we had a plan, and Lemon had been sick for so long, and so forth. Papa Bear and I talked the whole thing through again, and I talked it through with my ace support team of fellow CF mamas. On the one hand, he has been so sick, for so long, and the hospital had managed to get everything set up perfectly for him, and if we canceled and he suddenly got worse again, we'd have to do everything described above but without the advantage of things being set up ideally. On the other hand, I absolutely did not want to subject Lemon to procedures and medications that he did not need. Ultimately, the other hand won out, and I called the clinic to say we were cancelling and would not be showing up for our admission that day.

I still have no idea if this was the right thing to do or not. What I can say for sure (or almost for sure) is this: what happened here is something new to us. Lemon got a respiratory virus while he was already on antibiotics. The virus made him really sick for 72 hours or so, and then he was able to clear it on his own. I am pretty confident of this because, shortly after deciding to bail on the hospitalization, I started to feel really lousy myself. Friday, Lemon went to school, and I hid in the basement nursing cough drops and burning through an entire box of tissues.

By Sunday, I was pretty much back to normal, and we decided to celebrate the fact that we had more or less survived a week without anything going catastrophically wrong by taking the kids out for donuts. Lemon had been asking to go to a restaurant for the last few days (lord knows why, not like he eats or anything) but we thought Dunkin Donuts might be up his alley in terms of degree of formality and menu. He selected a Green Bay Packers donut and was quite thrilled with the whole thing. So thrilled, in fact, that he was unable to sit still at all and at some stage decided to try to climb back up onto his chair from the floor where he'd been standing, using a more athletic and higher-velocity approach than is typically advised. This resulted in him flying back off the chair at a similarly high velocity, and colliding with the table forehead first, splitting open his left eyebrow.

Fortunately, that particular Dunkin Donuts is pretty close to our local urgent care clinic, and the incident occurred just a few minutes before they were scheduled to open. We hustled Lemon over there, only to have the doctor there tell us that it definitely needed stitches but that we would have to go to the ER at Children's Hospital because she had a hunch that Lemon might possibly need to be sedated to let anyone put stitches in a half-inch from his eye. So, Lemon and I dropped Lime and Papa Bear at home and headed over to Children's. When we got there, the guy at the front desk said, "Oh, hey, I remember you!" (comforting?). Of course, the doctors there looked at Lemon and decided that he definitely did not need stitches, but rather just some glue, which they put on and then sent us home. Sigh.

So, that was last week. Tomorrow, we give Lemon his last dose of Cayston. Then, we watch, and we wait. I wish I could say that I was concluding the blog post like this because it's a serial and it's always a good idea to end an episode on a cliff-hanger. But, in fact, that is just how our life is right now.

Week 177: Sneezles

By and large, what you read here is in my voice, but Lemon would like to share with you in his own words one of the things that is happening to him right now: he has the sneezles. A mild cold, a bit of a runny nose, and a fair amount of sneezing. He's feeling fine. In other words, the kind of thing that prompts me to react with "Oh no. Was that a cough or a sneeze that I just heard? Did I just heard him cough in his sleep, or was I dreaming? How many more days of Cayston do we have left? What if he starts coughing and we're still on Cayston? What if there are some sneezles left once the Cayston is gone?"

For months now, I've felt like I've been inhabiting a through-the-looking-glass world where at a superficial level, everything seems fine--Lemon is tall, heavy, pink, energetic, largely asymptomatic--yet my perception of it is that in any 24 hour period there's a significantly greater than zero chance that we'll end up back in the hospital. I suppose in a sense, I'm glad that this chapter will reach a conclusion sometime in the next two weeks. We have another 7 days of Cayston left after today, and if Lemon gets sick again within a week or two of that, we will go to the hospital. And, if he doesn't, I suppose we resume our regularly scheduled program of taking each winter day one at a time. I also suppose we'll initiate the process of getting our second 28-day supply of Cayston, because there are supposed to be 28 days off in between cycles, and experience teaches us that we will need all 28 of those days to execute the transaction.

For the past few months, we've been feeling pretty complacent about nutrition. Lemon was eating his usual diet of bits and pieces, but a little something a few times a day, and the feeding tube was taking care of the rest. He was gaining weight like a champ, and blood levels of all the nutritional markers we looked at were in the normal range. But, for the past two weeks or so, his eating has gone down to just about zero. As in, when I reflect over the time between Tuesday and now, I can list off-hand exactly what he's eaten: a grape, 1/4 of a turkey and cheese sandwich, a slice of apple, three bites of pepperoni, a handful of popcorn, a few sips of milk. Even by his standards, this is pretty bad. In past, I've noticed that his eating falls off when he's working on some piece of cognitive development, and once he's worked out whatever it is, he resumes his "normal" eating habits. So, I'm hoping that's what's going on here although this is a longer than usual dry spell--in the past, they've lasted a few days and we're going on a two weeks or more here.

One thing that's happening, whether it's a result of whatever advances in cognition are going on or as a result of our slavish adherence to the 3x/day PT sessions that are each made even longer by the Cayston administration, is that Lemon's getting bored of watching videos on YouTube. He knows what things he has to say in order to get us to pause the treatment ("My tummy hurts!" "I have to go potty!") and has been saying them with greater frequency. He also used to fight us about turning off the iPad when his therapy ended, and now he turns it off himself and jumps off the couch. I suppose I should have seen this day coming, but we were in such a nice space for a while with being able to leave him on his own during therapy and get some other things done (ie, showering). Recommendations for age-appropriate iPad games featuring trucks, anyone?

Week 176: Skilled labor

How much time does it really take to take care of a "child with special health care needs?" Thanks to some new research, we don't have to guess anymore. A paper that came out last month in the journal Pediatrics last month looked into this issue (the story was picked up by NPR--you can read their story here). The answer is a lot. For a kid with CF, this study found that parents provided 12.9 hours of care per week--one of the top 3 conditions of the 20 conditions they looked at. That's 12.9 hours of just care that focuses specifically on the child's medical needs, not all the other care that any kid needs. 12.9 hours of running the vest, delivering inhaled medicines, washing and sterilizing nebulizer parts, pouring formula into feeding bags, drawing up medication and vitamins into syringes, washing the syringes, and so on and so forth. Those 12.9 hours do not include activities that the study considered "coordinating healthcare outside the home," things like making appointments, contacting care providers, dealing with pharmacies, ordering supplies, and dealing with insurance companies. That's an additional 3.9 hours per week. In summary, this is why I have not returned your phone call/email/holiday card.

As a caregiver, I've gotten used to the amount of work I have to do to keep Lemon healthy, and it doesn't bother me too much anymore--I'm used to my share of those 12.9 hours being gone, my expectations have been reset. I'm doing those tasks at home, we have a routine, and I can multitask. What really bothers me is the 3.9 hours. Those to me feel like a complete and utter waste of time, every time. Those are the things where it feels like "the system" could just work so much more efficiently, so many more things could be automatic, and so many of the things that are supposedly already "automatic" could actually work in an automated way (why it takes so much effort to get refills of prescriptions that are supposed to automatically refill, I have no idea). It's validating to know that my experience is pretty average, or average for those of us who live at the extreme of this continuum, and I guess I'm glad to know that my efforts are one tiny part of the $35.7 billion worth of work that parents of kids with healthcare needs provide annually.

Cayston has definitely increased our care burden this month. We just finished our second week, and the good news is that Lemon has no cough as of now. We have two more weeks to go, and then our first 28-day course of Cayston will be done and we will see if it was any better than oral antibiotics at really reaching deep and killing whatever's been making him sick since September. Because Cayston has to be administered after a vest treatment in order to be maximally effective, we're locked into doing the vest 3 times per day at 30 minutes a pop. Then the Cayston itself has to run, that's another few minutes, and another few minutes to set everything up beforehand, and another few afterwards to take everything down, and wash the special Cayston nebulizer (you only get one of those, so you have to wash it after each use and then sterilize it once a day). So, at the moment, just the breathing treatments aspect of our lives is at over 14 hours per week. It is going to feel like a vacation to go back down to just 2 treatments a day!

This was the week that we were supposed to be in Boston. We are still sad about not going but it was definitely the right decision. Lemon is doing really well, and many people that we wanted to see in Boston were sick this past week, so had we gone and had Lemon managed not to catch something on the plane, we probably would have had to avoid many of the people that we were specifically traveling to see. We enjoyed a pretty quiet week around here, and even threw a little New Year's party with some friends in our neighborhood. I'd be lying if I said I wasn't nervous about the coming year, but I suppose we'll tackle it like we did the last one--one week at a time. Wishing all of you happiness, peace, and health in the year to come.