Week 171: No Turkey

Last week, we pushed our clinic to consider some additional therapies in the hopes of finally getting Lemon back to respiratory baseline after over two months of ups, downs, and various forms of antibiotics. We still haven't heard whether our insurance will cover the inhaled antibiotic Cayston for off-label use, but they did cover Flovent (a corticosteroid inhaler) without batting and eye. And, I'm glad they did--after 48 hours on it, Lemon was cough-free for the first time in ages. Of course, he's still on levofloxacin so the big challenge will be in 10 days or so when that ends, and we see if he can finally sustain his respiratory health on his own. Fingers crossed. At the moment, the plan is to stay on Flovent until spring, and we're still looking to get a second opinion with hopefully some additional new ideas (no progress on that yet, still waiting on insurance coverage). But, for the first time in a couple of months, I feel like we may be on the right track, or at least we've found the first step onto the right track. And I am very thankful for that.

Other than that, it was a fairly quiet week, in as much as having a one year old, a three year old, and a four-day holiday weekend can be quiet. We enjoyed a fast vegetarian Thanksgiving lunch with Nona and Opa, and then found various ways to keep the kids amused and out of trouble over the next three days. We went to the indoor farmer's market, the grocery store, the children's museum, and a donut shop. And we talked to friends and family scattered across this big country that we live in. I am very thankful for all of them, and their support. It is reassuring to see that even though we don't see each other in person quite as often any more, they are still out there waiting for us at the other end of the internet.

Lime is starting to learn lots of words. As befits his Wisconsin roots, the first vehicle name that he learned was "tractor," followed in rapid succession by "bus" and "dump truck." He knows what the horse, the cow, and the sheep say. And, in true Badger style, the two foods that he can clearly ask for by name are "milk" and "cheese." All other foods he still asks for by pointing at and grunting with such vigor that his whole head turns red. And when I say all other foods, I mean exactly that. I kid you not when I say that this child was in tears the other night because I decided to limit the number of roasted Brussels sprouts that I was willing to give him as a snack before dinner. I'm thankful to have this lovely little person who appreciates food in my life!

Week 170: Off label

This week, I went to Chicago for a night to attend a conference for work. This was my first night with zero children since October of 2014. So, how did it feel to be solo in a city with no one but other grown-ups to talk to? AMAZING. I will even forgive my colleagues for stretching our work dinner until after 11pm so that my blissful night of uninterrupted sleep was only 7 hours long. I'm hoping this marks the beginning of a happy tradition whereby I get out of dodge at least once or twice a year. But, now I'm back in the thick of dodge, and we're dodging, or trying to anyhow.

Here's where things stand. Lemon's been on oral levofloxacin since a week ago Friday to fight off the resurgence of the thing that we've been fighting for two months now. Ready for something startling? Oral levofloxacin didn't work the first time we tried it against whatever this is, and it isn't really doing the trick this time, either. It's pretty much done what it did before, which is to say that it has gotten Lemon to the point where he feels fine but is still coughing a bit. Not a cough that would make anyone who doesn't know the back story think anything at all was wrong with him. But, we all know the back story here. We know enough to grasp that if he isn't absolutely, 100% at respiratory baseline and we stop the antibiotics, whatever this is will just come roaring back within a few days. So, what now?

As you might imagine, I put this question pretty directly to our care team at the CF clinic, pointing out that doing the same thing over and over again and expecting a different result is, well, you know. I asked what ideas they had to break the cycle. They came back with a couple of things. First, they want to switch Lemon's acid reducing medication from Protonix back to Zantac, because a study was presented at the most recent CF conference that kids who take proton pump inhibitors (like Protonix) have more respiratory infections. I said I was happy to try that, but that they should not forget that Lemon was on Zantac for the first 2.5 years of his life and got just as many respiratory infections as he does now, so I hardly think that will do anything.

The other idea that they had is that maybe Lemon has reflux that is causing or contributing to his problems. They know that he has already had two GI imaging studies, neither of which has shown evidence of reflux. Of course, no evidence of reflux isn't proof that there isn't any, just that it wasn't seen. But, he's also never had any symptoms that would suggest that he does have reflux. In light of all that, I'm hardly eager to do the test they have in mind, which would involve placing a sensor in Lemon's esophagus for 24 hours to measure the pH in there. Oh yeah, they put the sensor in through your nose and then tape the tube to your face and connect it to a data recorder that's in a little pack that you have to carry around while the test is going. What could be better? So, no, not unless there's a really compelling reason.

And, that was pretty much the end of the idea list. I hadn't wanted to plant the ideas that I'd had, because I wanted to hear what the clinic folks would come up with. But, being unimpressed, I wrote back to them and asked about trying another course of Flovent, as we had done back in March in response to Lemon's lungs looking inflamed during the bronchoscopy. Or, how about trying inhaled antibiotics rather than oral since oral really doesn't seem to do the trick for us?  I just heard back today that we can move forward on the process of trying these options. Both the Flovent and the inhaled antibiotic that our clinic suggested (Cayston) will be off-label for Lemon. He doesn't have asthma (Flovent) and doesn't culture Pseudomonas and is less than 6 years old (Cayston). So, those are getting routed to our insurance to see what they will agree to.

In the mean time, we are going ahead with trying to get a second opinion in Milwaukee, but more obstacles there--that hospital is out of network for us. So, I have to file a pre-approval form (filled out not by the CF clinic, but by Lemon's PCP who has literally seen him once) and then the insurance company gets 15 business days to think about it and give us a decision. So, with the holidays coming up, I would be shocked if we get that second opinion before the door closes on 2016.

With all this going on in the background, I am glad that, following the great travel debacle of 2014, we resolved not to try to travel for Thanksgiving any more. I am still sad that we will not be in Boston surrounded by family for the holiday, but the disappointment of never having planned to go pales in comparison to the disappointment of planning to go and canceling. And, I'm hoping that soon we'll have something new to be thankful for--namely, a treatment plan for Lemon that actually works.

Week 169: Aftermath

So, some politics occurred since I last wrote. We all have opinions about this, and I have gone through a gamut of emotions about it. But this is not a blog about politics, and I'm not going to get into all that here. I'm just acknowledging that it happened, and stating the obvious: we, like everyone else in the country, are going to be watching closely to see what happens next. Now back to your regularly scheduled programming. I'm sorry to report that this is going to feel like a re-run.

On Wednesday (a week to the day after the PICC was pulled), Lemon had a runny nose. Doesn't sound like an emergency, but we have been down this road altogether too many times. Thursday he started coughing, and Friday we were on the phone to the clinic. Both the CF specialists were out of the office, and unsurprisingly the pulmonologist on call decided to stick to the letter of his treatment plan and give us yet another two-week course of levofloxacin. Because that has worked so well in the past (ie, it has never cured Lemon of anything in his entire life). He's been on it for a few days now and he is certainly better than he was when he started it, but as was the case last time it seems to be getting him to about 80% better and that's it.

In summary, we need a new plan. I don't know what the new plan should be, nor should I know because I'm not a pediatric pulmonologist and I don't have a full knowledge of what all the options are, with their attendant benefits and risks. What does worry me is the fact that I don't know where this new plan will come from. Sadly, I've grown increasingly convinced over the last few months that it is not going to come from our hospital.

When Lemon first got sick in September, I suggested to our team that we needed a fundamentally new plan. That seemed to fall on deaf ears, because instead we went on our all-too-familiar journey of a few failed courses of oral antibiotics followed by a round of IV Zosyn. Now, we're heading down the exact same path again, whether it's a repeat battle with something that wasn't killed by the Zosyn or whether it's something new (opinions differ). We have been down this path entirely too many times to continue going down it and expecting a different result at the end.

So, in the coming weeks we will be looking to get Lemon seen at another CF center. The logistics seem a bit daunting but I really feel like at this point we have no choice. Lemon's doctor, whom we really trusted, left our clinic in March. They haven't been able to recruit a replacement for him yet, so right now there are just two CF specialists at our center, and they work really closely together. That's good in a way, but it doesn't really leave room for a lot of diversity of opinion or new ways of thinking, which is what we really need at this point. 

With all this going on in the background, it was great to have some fun diversions. One of my long time cycling and running buddies from Boston came out to visit, and we ran the Madison marathon together. It was my first marathon since before I was pregnant with Lime. I'd been hoping to run a personal best, but between the disruptions to my training with Lemon's recent course of IVs and the incredible headwind we had to tackle at the end of the course, it wasn't quite in the cards. Still, I missed my personal best by less than 90 seconds, and placed in the top third of my division.  Not too shabby, and enough of the pain has worn off now that I can say that it was a great time and I'm looking forward more races in the future.

I know some of you wonder where I find the time to train. Honestly, I do it because I have to. I'm no good to myself or anyone else if I don't do this kind of stuff, and after nearly four decades on the planet I know myself well enough to understand that. Some (most) weeks, it's the only "fun" activity that I have time for, but keeping up with a training program and clicking through the miles on my calendar as the count-down to the race approaches keeps me focused on something besides the day-to-day drama in our household. Being an endurance athlete is good training for being a CF caregiver too, since in both cases it takes a lot of patience and perseverance to get anywhere at all. Here's hoping both Lemon and I settle into some kind of good winter maintenance pattern soon!

 

Week 168: On the eve

I'm a writer by profession and by hobby. I'm not often out of words. But, tonight, I just don't feel like I have that many left. Nor can I imagine that my loyal readership has much capacity left for consuming words found on the internet after the last few months. Tomorrow is finally election day, so with any luck the flood of online words will slow a little bit, and we can all take a moment to catch our collective breaths and square our shoulders to face whatever comes next.

On Wednesday, Lemon and I went to the clinic and got the PICC pulled. I had the idea that since we had the PICC in and could do blood draws totally painlessly, it would be a good idea to do the draw for his annual labs, since he was about due anyhow. That may or may not have been such a smart idea, since the tests came back today and revealed extremely high levels of a few liver enzymes. This is probably due to the fact that he was just really sick and on IV antibiotics.  Probably. But, now we have something new to sort of keep in the back of our minds for a month or two, until we repeat the tests. And of course, that means a needle stick, which is what I was trying to spare Lemon by having the labs drawn from the PICC in the first place. Sigh. At least the PICC is gone so we can go back to regular "two boy" baths, and the skin on Lemon's arm that was under the dressing can start to recover.

The last two nights have led me to believe that this whole clocks falling back business is specifically designed to torture the parents of young kids. Getting up at 4:15 is bad enough. But 3:15 is really for the birds. And by birds I mean nocturnal owl type things. Hopefully our guys (Lime in particular) will get this sorted sooner rather than later. Either that or I think Papa Bear is actually going to follow through on his threat to set up a Pack-n-Play in the basement.

I know this election has impacted all of us, and I know a lot of you who have more capacity than I do have been personally involved in getting out the vote. I know this not just because you have posted about it on social media, or told me about it in person. I know this because I live in a swing state and I have a land line and IT HAS BEEN RINGING OFF THE HOOK EVERY NIGHT FROM 5-9PM FOR THE LAST MONTH. I do thank you sincerely and earnestly for your efforts, and I am officially done with politics for the foreseeable future. I may take a tip from my grandmother and cancel my subscription to the New York Times for a while. All I can say with certainty about tomorrow is that I'm going to get up, take care of my kids, and go vote. Afterwards, I'm going to get my hair cut.  Because I haven't quite had the time to do that in the last 9 or so months, and whatever the future holds, I will be better equipped to handle it if I am less shaggy than I am now.

See you on the other side!

Week 167: Trick or treat

As you can probably tell, since I'm awake after 7pm, we are no longer doing our every 8 hours around the clock IV routine, which is a good thing. At our clinic appointment on Wednesday, the doctor and I agreed that Lemon had made a lot of progress (including gaining back some of the weight he'd lost), but wasn't quite back to where we wanted him to be in terms of respiratory symptoms. We decided that we had better be really sure we'd beaten back whatever was causing all these problems, and the best way to achieve that was another week of IV antibiotics. However, since Lemon was doing much better, the doctor felt that we could go down to every 12 hours instead of every 8, which has made a sort of amazing difference in my energy level. It's still a big to-do, and adds a lot of stress and a bunch of extra things to do every day, but is so much more manageable.

We're getting reevaluated again this coming Wednesday, and hopefully then they'll pull the PICC out and we'll be done with IV antibiotics. I say hopefully because Lemon is, I would say, 95% better. He still is coughing just a smidge more than what I would consider his baseline, and that cough sounds a bit more productive than I would ideally like. Of course, Lime is also coughing a bit and has a runny nose, as does pretty much every other human resident of Madison at this time of year. So, we'll see.

As an aside, running IV's at home makes you acutely aware of just how much plastic is used in modern medicine. The amount of garbage we're creating is just staggering. I have always thought of myself as an environmentally conscious person (recycling, composting, etc), but no number of LED light bulbs can possibly offset the amount of waste from even one course of IVs. I feel like I'm taking out the garbage twice as often right now as I normally do. It doesn't sound like much to write it down, but all those syringes, all that packaging, the gloves, the tubing...it just adds up somehow. And then there's still all the normal stuff, including one kid still in diapers and a disposable tube-feeding bag every night. Yeesh. Please be extra good about reusing things this week on my behalf! 

In the mean time, we are doing everything we can to get Lemon's life back to normal. He's back at school, with his PICC tucked away inside a long-sleeved shirt. He went trick-or-treating tonight while his evening infusion was running, with his IV pump tucked away in his backpack and tubing running from the backpack down the back of his shirt to his arm. In typical Lemon fashion, he was absolutely thrilled about wearing his Superman costume, and was delighted to go out with Papa Bear and collect candy. When he got back to the house, he and Lime were allowed to have a small sample of the loot. Lemon took about a half-dozen licks of a lollipop and ate one Milk Dud. Lime, on the other hand, took one bite of a Reese's peanut butter cup, gave me a look that clearly said, "This food has existed in the world for my entire life and you didn't tell me until now????" and stuffed the entire thing into his face. Good thing we didn't have very many trick-or-treaters at our door this year, so we have a bunch of extras.

So, that's a wrap on an October that was more exciting than I think any of us could have imagined. Fingers crossed for a more peaceful November!

Week 166: Unstable equilibrium

Having a kid with CF means living in a state of unstable equilibrium, like being in the car at the apex of a hill on a roller coaster. Even if at that moment on the apex you are sitting still, in only takes the slightest push in either direction to send you flying off down one side or the other. And last week's stomach bug was all the push we needed.

On Tuesday morning, Lemon started coughing again. I had a feeling this was a very bad sign, and contacted the clinic right away. We got a prescription for a further two weeks of augmentin, but the augmentin just was not up to the task of getting things back under control after we'd gone so far off course. Lemon was weakened by the stomach bug, and his throat was undoubtedly inflamed by all the vomiting he'd done. So, when he started coughing again, he started vomiting again, and it wasn't far from there to dehydration and a sort of downward spiral.

I called the clinic again on Thursday morning to try and get him seen. I spoke to a nurse at 9:45 who agreed that he probably should be seen.  She started scanning the schedule to see what appointments they had available. She found one and then said, "oh, but this one won't work, its at 10." Luckily, we live 2 miles from the hospital, so I said, "no problem, hold that spot." I hung up the phone, tossed Lemon in the car, and high-tailed it over. The doctor that we saw took one look at poor Lemon, huddled in my lap with dry lips, sunken eyes, and a big cough, and said "You're not going to like this, but I think we have to admit him." I wasn't surprised, since I agreed with that assessment--we needed a pretty serious intervention to get things turned around, both from a hydration perspective and to actually kick whatever's been plaguing Lemon since mid-September. He sent us home to pack up and get organized, and we were back at the hospital by 2 to get into our room.

They placed a peripheral IV right away. Some of you may remember from a few hospitalizations ago that it took the strength of 6 adult human beings to restrain Lemon enough to get a line started. This time, someone had the clever idea to use a technique I've only previously seen used on one of our cats while at the vet--they basically wound Lemon up in a bed sheet so that only his head and the relevant arm were exposed. Using this strategy, only 4 people were needed and the placement actually went pretty fast and smoothly. They got a fluid bolus into him right away and he perked up perceptibly. They also started IV Zosyn to start tackling whatever was in Lemon's lungs.

On Friday, Lemon had his 3rd PICC placed. This time, they did it under conscious sedation rather than general anesthesia. I got to stay with Lemon through the sedation, until things were pretty well underway, and I rejoined him as soon as the procedure was over. The hardest part of the whole thing was preventing him from eating or drinking until 1pm when the procedure started. We've often wondered what it would take for Lemon to be actually hungry--it turns out that taking in basically zero calories for a solid week and losing 10% of his body weight will do it.

We pretty much insisted that they let us go home on Saturday. Lemon was fully hydrated and doing much better overall. We've done home IVs before, so that gave the team at the hospital the confidence they needed to let us go. The only "small" hiccup that we've encountered being home is that the infusions, which are supposed to take 30 minutes each, are instead taking 60 minutes each. We've tried a new pump and new tubing with no improvement. Given that I'm administering these 3 times a day, at 6am, 2pm, and 10pm, the extra 30 minutes really matters. But, no one seems to have any idea what's causing the problem and their solution seems to be that since it's infusing and we're managing, we'll just ride it out. Easy for them to say, they aren't the ones saying up til 11:15 running one treatment and getting up at 5:30 to prepare for the next. We're being seen again on Wednesday morning and assuming Lemon is back to baseline, the PICC will come out then, so it's only another few days of this madness--which is a good thing!

Week 165: Go with your gut

This week got off to a promising enough start. Lemon came off antibiotics on Monday and it seems like the solid month of drugs might have put Something Sudden back in its place. Wednesday morning, Lime had his 15 month checkup--he's still short (6th percentile) and a little bit stout (12th percentile), but is sticking right on his growth curve and hitting all his milestones and whatnot. And, as the PA assures me at every appointment after she measures him, "Boys can grow until they're in college!" Or, he might just be short. Either way, it's OK by me.

I am now searching my memory of Thursday to see if there were any signs that, in retrospect, should have alerted me to the impending storm. I don't think so, but perhaps the overall absence of things to worry about should have clued me in. On Friday morning, Lemon vomited after his vest treatment. But, that happens from time to time and we didn't think too much of it, he seemed totally fine afterwards. The rest of Friday seemed pretty much OK, too.  Neither of the kids ate much dinner, which in retrospect was a small mercy. Because things became decidedly not normal after that.

There's no need for me to go into any further details. For those of you with kids, you know what having toddlers with a stomach bug is. For those of you who don't, let's just say my fondest wish right now is that I could somehow use two pairs of long-handled tongs to pick my house up from its foundation and dip it in a vat of boiling Lysol.

Thankfully, the storm seems to have largely passed. As is the nature of these things, symptoms progressed on a course from the top of the GI system to the bottom. Lime still has very little appetite, but whatever he does put in has been staying there long enough to make its exit through the traditional route, even if at a somewhat more rapid pace than usual. Lemon is getting a bit of nutrition through his tube (although we've temporarily stopped boluses and went down to one can a night over the weekend; we are, with fingers tightly crossed, attempting two cans tonight) plus whatever bits and pieces he eats during the day, which is currently less than usual, which isn't much to start with. He's also really struggling to tolerate his therapy--I have to imagine when your stomach is a little off kilter, being shaken violently for 30 minutes is not a soothing experience. We've definitely learned to take him seriously when he says he doesn't feel well during treatment, and are lowering the intensity of the shaking a bit until he's on more solid ground. Of course, we don't want to back off too far lest something decide to settle in his lungs, so it's a balancing act as usual.

All in all, this stomach bug is a totally normal thing, in its way. All kids get stomach bugs. Things are pretty much horribly gross until the thing runs its course. And then it's over. But it has reminded me how fragile Lemon's health is. The weight that we've worked so hard to put on him has absolutely melted away over the last few days. At least with the tube I can feel confident that we can put it back on again, and fairly quickly at that. But it is still a stark reminder of how far from well he really is--including this "regular kid" thing, he's been sick for 5 weeks with hardly a break. And it's only mid-October.