Week 345: Peanut butter pie

We have made it through week 2 of "safer at home." I cannot claim that we made it through with an over-abundance of grace or style, but here we are. The kids had some ups and downs this week as they tried to make sense of this strange new life of ours. They don't quite miss school, but they do definitely miss doing all the things they are used to doing.













Papa Bear and I are continuing to try to adapt as well. Having both of us working from home all the time is strange, and juggling the kids while trying to get any amount of work done is a big challenge. And, not only are we supposed to juggle them, but we are also now supposed to teach them. Or, at least, guide them through whatever materials their teachers have provided. They are both young enough that the online stuff just doesn't really work for them. There are a couple of apps that Lemon will dig into for a few minutes at a time. Lime is already kind of over screen time, and will ask repeatedly if it is time to stop watching videos yet. We may need to teach him to read. At least the weather is getting nicer.

From the CF perspective, things seem pretty good at the moment. Lemon's cough is almost completely gone, and since we're stuck at home anyhow we've been doing 3 treatments per day. I am still waiting to connect with the new doctor about our long term plan. He called me on Thursday but I missed the call, and of course I can't just call back, I have to wait til he calls again. I guess he prefers the phone to email, which I understand, but on the other hand the phone does require both parties to be available at the same time, which can be a challenge. Hopefully we'll connect soon, since we are almost at the end of Lemon's course of antibiotics, and we need to know what our next move is.








In the mean time, we're just doing what everyone else is doing, trying to maintain some kind of sense of normalcy in the face of all the fear and uncertainty around COVID-19. We're trying all the usual distractions, doing puzzles, reading books, watching movies, doing yard work, and of course baking. I made probably the most midwestern thing I have ever made in my life, a chocolate peanut butter pie from the Norske Nook cookbook. People, this pie contained both Cool Whip and chopped Reese's peanut butter cups. I may need some of you to come conduct an intervention when this is all over. On the other hand, Lemon absolutely adores it, so it looks like I may be making it again.

Week 344: Not home today

It feels like we are living in some kind of strange nightmare. I hope all of you are holding up OK and somehow adapting to this new reality in which we find ourselves. I know it's a struggle for all of us. 

Meanwhile, because a global pandemic on its own might not be sufficient excitement for a week, Lemon developed a terrible rash all over his legs and belly which we concluded was an allergic reaction to azithromycin, since that was the only thing that had changed in his life in recent days. We stopped the azithromycin, did a few days of Benedryl and hydrocortisone, and at least that is now back to normal.

Relatedly or not, Lemon had started coughing last Saturday.We were kind of keeping an eye on things while waiting to hear back from the new doctor with an updated care plan. Eventually we decided the cough needed intervention, and worked with the pulmonologist on call to get a prescription for augmentin, which should work against the bacteria that grew out from the bronch. We started that a few days ago and the cough has pretty much cleared up. I am still eager to hear what the new long-term plan is, but I suspect possibly the pulmonologists are a bit swamped right now. I also wonder in retrospect if at least some of the coughing wasn't due to the allergic reaction to azithromycin, since Lemon's biggest allergy symptom is always cough. I suppose we'll never know, and in any case we would have wanted to start antibiotics to kill the bacteria that were found during the bronch, so it probably doesn't matter. 

At least I can report that we all survived the first week of no school successfully. I'm not sure how much learning occurred, but we tried. I think our biggest success has been to have Lemon write a daily entry in a diary about his experiences during the pandemic. Learning apps have been a mixed bag overall, the kids like some of them but not others, and the amount of time they will do any one things is limited.

For better or worse (mostly better, I think) the thrill of screen time has totally worn off for Lime. Now, when we tell him it's time to watch videos on his iPad (while Lemon does therapy), Lime will start willingly enough but after about 10 minutes he wants to know if it is time to be done, and often will wander off and start doing something else. Like the rest of America, I have been baking a lot this week,so he's learned some important life skills such as how to whip egg whites to stiff peaks, and how to make a chocolate ganache. Hopefully these will come in useful someday when he needs to impress his friends.

Anyhow, again, my best wishes to all of you near and far. Stay home, stay healthy, and be well. 

Week 343: Like no other

Every week, I think, "Wow, what a week." But this time, for real, what a week. Most importantly, before I say anything else, please take every precaution to ensure your health and the health of others, especially those who are made more vulnerable by age and underlying health conditions. I know it's no fun, believe me when I say that all of us in the CF community are used to making these hard choices, but these measures are the only things that we as a group can do to protect ourselves from a serious virus against we have no immunity, no effective medicines, and no vaccine.

So, stepping back in time a few days to when one could still leave one's house, Lemon had a bronchoscopy on Thursday. He handled the whole thing remarkably well. We had to skip his overnight feed and stick to just clear liquids during the day, which was tough since he actually did get hungry (!). We were told to get to the hospital at 1:15, which we did, and then waited in the pre-op room for a solid 2 hrs before anyone came to do anything. By the time we'd signed the consent and gone through all the other preliminaries, it had been almost 2.5 hours between when we arrived and when he went back for the procedure. That's a long time to wait as an adult, never mind a 6-year-old, although he was happy about being allowed to watch a lot of TV.

The procedure itself was quick, and was performed by Lemon's new doctor. He was happy with how it went and said he got a good sample from the area of Lemon's lung that has been the focus of so much attention of late. He anticipated that it would take until Monday to get the results of the cultures back, and that he would call me once they came in with a treatment plan. Then I hung around at the hospital for a further 2 hours waiting for Lemon to wake up enough to be released, and we went home via McDonald's

where he got a much-deserved Happy Meal. I have never seen him eat so much so rapidly, I think. So, it is reassuring to know that he does sometimes get hungry.

In any case, after that, the world kind of came to a grinding halt. We managed to get ourselves vaguely organized before a series of announcements that school would be closed effective Wednesday, just kidding Tuesday, just kidding as of right now (ie Sunday). So, now we are scrambling to figure out how we are going to get work done, teach our kids something, stay healthy, and stay sane for however long this next phase of our lives is.









Just to sort of top things off, Lemon has a really bad cough. It started on Saturday, but has been progressing steadily since then. I spent all day glued to my phone hoping for the call from the doctor, but it never came. I'm guessing the clinical lab may be a bit overwhelmed and thus our culture results are delayed, but we really, really need them. With a cough like this I know we need to intervene soon. We have Cayston on hand, but if that isn't the best drug to fight whatever has been living down there in Lemon's lungs, I'd rather start with the right one. And soon.

Be well, everyone. We may not be seeing you for a while but we are thinking of all of you every day.

Week 342: Self-containment

Just now, Papa Bear reminded me that his birthday was this past week. True story! A lot has happened since then. Here are the updates. After some further discussion, we decided to postpone Uncle Jared's visit. At the time it seemed perhaps overly cautious, but from where I'm sitting today (and only a few days later) it seems like unquestionably the right decision. Coronavirus seems to be spreading rapidly, and we just cannot afford to take any extra risks with Lemon's health. I also have this nagging suspicion that Uncle Jared's visit will just be the first in a line of things that we end up postponing until the environment in the world seems safer, but one day and one week at a time.

We had our clinic visit on Wednesday. Lemon's PFT's and weight were both good, somehow he gained 2 lb since our last visit which surprised everybody, in a good way. Lemon also did a lung clearance index test, which was the last test for the research study he's been part of since he was born. Kind of a milestone to age out of that one at last.

The other thing that happened is that after the visit, I sent around some messages to the new doctor and the nurse practitioner basically demanding an action plan, and at last I think we finally have one. The new doctor finally responded that he is willing to take Lemon on as a patient (yay!) and also that he agrees with the nurse practitioner and the old doctor that we should do a bronchoscopy. But, to be clear, he favors it as a diagnostic procedure, to see what if anything is growing in the collapsed area of Lemon's lung, as opposed to a therapeutic procedure to clear out the area. Some incidental clearing out may happen as long as he is down there with a tube, but that isn't the primary purpose.




So, Lemon will be having a bronchoscopy on Thursday afternoon. When exactly? Who knows, because it would be far to easy to plan around it if we knew what time it will be happening. We don't get to find out that information until Wednesday. We haven't told Lemon about it yet, on the theory that it would just give him more time to worry about it. We will discuss it with him on Wednesday, maybe once we know more details about what exactly will be involved. The good news is that it will be done outpatient, just using conscious sedation, so we should (hopefully) be in and out of the hospital pretty quickly.  Then there will just be the anxious wait until the culture comes back.

Despite the worry over the procedure and the exposure risks involved in even setting foot in a hospital these days, I am really glad that at least, 9 months after this thing should have first been detected, we are finally doing something about it. Once we know what organism is growing down there, we can plan an appropriate antibiotic strategy to treat it, and with any luck avoid a round of IV antibiotics. I'm also glad that we are transitioning to the new doctor. I am sorry to part ways with the nurse practitioner, she is a very kind person and has taken good care of Lemon for all these years. But, having her as our primary care provider was never the plan, and I am feeling good about the choice to be working with a pulmonologist again. Fingers crossed!

Week 341: Get it together

After giving the matter extensive thought, I decided on Tuesday to reach out to the new doctor and ask him to take Lemon on as a patient, without the involvement of the nurse practitioner. I felt that the new doctor was the best fit for us, and he had a lot of ideas that I wanted to try to improve Lemon's care, both to address the immediate issue of the area of his lung that is collapsed, and for long-term preventative care. So, I sent the new doctor a message saying we wanted to switch over Lemon's care, and that we would like to begin the treatment plan (antibiotics etc) for the lung issue right away. And....nothing. Not a peep, other than the response from the nurse saying she was passing the message on to the doctor.

So, it has now been 9 months since the June x-ray, when this problem should have been detected, 2 months since the CT scan that drew it to everyone's attention, and 1 month since the repeat x-ray showing that our efforts with manual chest PT were unsuccessful. And we still have no treatment plan, and no conclusion about who will be leading Lemon's care going forward. This, my friends, is just awesome. Luckily (?), we have our regular quarterly clinic visit with the nurse practitioner scheduled for Wednesday, so maybe I will have more luck in person figuring out what is going on, who's talking to whom, and why nothing is happening. I am a relatively patient person, but I am about at the end of my patience with this situation.

The other thing we've been keeping our eye on is of course the corona virus situation. This has been on our minds a lot this week since Opa just returned from a trip to Italy, and Uncle Jared is supposed to come from New York on Friday for a visit. I think the odds that either of them has come into contact with the virus are vanishingly small. But, the consequences of Lemon getting the virus could be pretty grave. So, how does one weigh that? The benefit of the family reunion against the almost (but not quite) zero chance of something bad happening? It's a puzzle, and with new information coming out at a breakneck pace, it's a new puzzle every day. I would say I would ask our clinic for guidance but I'm honestly not too bullish on them at the moment (see above).