Week 98: Americana

This weekend, we attended our neighborhood's 4th of July parade.  It doesn't get much more "America" than this.

The parade was led by a fire truck--our local Engine 9 that Lemon and I have seen many (many) times, followed by a band comprised of anyone in our neighborhood with an instrument and the desire to participate.

 Tell me this baton-twirling lady isn't your hero!

 Most of the people in the parade were kids of various ages on their bikes, which they'd decorated with red, white, and blue streamers.

 The parade traveled a couple of blocks to our local park, where we got to take a look at the fire engine up close.

 The culmination of the festivities was a picnic and party, but by the time that all got started Lemon was so exhausted from the excitement that we had to go home for a 4 hour nap.

On Sunday night, Papa Bear and I completed our American experience (and also our annual soccer viewing requirements) by watching the World Cup final.  So, a very good weekend all around. 

This was also an amazing week for the CF community.  First and foremost, the FDA approved Orkambi, a two-drug combination that treats the root cause of CF in about 50% of patients.  We aren't in that 50%, but we are confident that our drugs are coming soon.  Our sincerest thanks as always to everyone who has donated to the CF Foundation on our behalf--your dollars make these breakthroughs possible!  Second, the results of the first gene therapy trial for CF were announced.  Basically the study found that delivering a normal copy of the gene that is mutated in CF patients directly to the lungs once a month for a year was able to slow the rate of decline in lung function in the treated patients as compared to the placebo-treated controls.  The results are certainly very encouraging, and hopefully will be even more dramatic once more efficient gene delivery technologies are used.  Yet another reason for us to end this week in a very hopeful mood!

Lime is continuing to tick along.  I'm heading towards 38 weeks pregnant at this point.  Lime has continued to pass all of his tests with flying colors, and we're starting to get a little bit serious about the logistics of his birth.  For instance, I have sitting next to me right now an empty tote bag that I fully intend to pack with things to take to the hospital...  

Week 97: Smallish

Today we had Lime's 36 week growth scan.  After four weeks of twice-weekly monitoring, this was the test that would really determine whether he was struggling in utero and would have to be delivered early.  So, of course, I didn't sleep much last night.  Papa Bear came with me to the appointment, and got to experience firsthand the joy that is the non-stress test.  As usual, Lime sailed through, completing his required number of accelerations well before the 20 minutes were up.  Then, it was off to ultrasound land.  As you might imagine, I was really not happy to hear the ultrasound tech say "Oh, I'm new here, I don't know how to do the Dopplers so we'll have to have someone else come in to take care of that."  In any event, she did eventually manage to take all the required measurements (with no help from Lime, who squirmed away at all the wrong times), and then had someone more experienced come in to do the Dopplers.  We had been told last week that we would be meeting with the doctor today to discuss the outcome of the test, but of course they only had one doctor on duty this afternoon, and lots of patients who needed their results read, so they sent a nurse to talk to us instead.

The long and the short of it is that, after all these tests, all this monitoring, and all this anxiety, they have come to the remarkable conclusion that Lime is, well, smallish (yes, that's the technical term).  He is sticking right on his growth curve and is now about 5lb 9oz--on track to be born at almost exactly the same size as his (also smallish) older brother.  His heart rate, blood flow and amniotic fluid and all that continue to look perfectly normal.  So, at least for now, induction is off the table, and as long as Lime continues to pass his non-stress tests and Dopplers each week, they are happy to just let the pregnancy run its natural course--which is all Lime and I ever wanted to begin with.  Phew.

Lemon is continuing to focus on just being an almost-two-year-old.  We are eagerly awaiting the arrival of his vest, which will replace the chest physical therapy that I'm still doing manually twice a day.  Between my lap shrinking and Lemon growing, we're really running out of room to do it the way that we have been for the last two years.  Our doctor prescribed the vest  (we're getting this one) at our last clinic visit back at the end of May, with the idea of having it in place before Lime's arrival.  But, it's a pretty pricey piece of equipment so it appears that the wheels of medical bureaucracy are turning even more slowly than usual.  The week after it was prescribed, I got a call from the manufacturer about their estimate of what our out of pocket costs would be, based on our insurance.  I told them the costs were fine, and they said in that case they would go ahead, and we should hear something in 10-15 business days.  I somehow thought that meant that in 10-15 business days, they'd be calling us to figure out when to deliver the vest to us, and give us our in-home training on how to use it.

We still hadn't heard anything as of this morning, so I gave them a call, and found out that they are still waiting for some medical records from our hospital, which they then pass on to the insurance company, and then the insurance company will determine whether they're covering the vest or not, and then once the insurance company has made up its mind, then we can talk about when we might actually receive the vest and get our training.  So, not exactly where I was hoping the process would be at this stage, with my due date just over 3 weeks away.  Oh, and of course, all of this is happening using good old fashioned paper and snail mail.  Apparently, even though Madison is home to Epic, one of the biggest companies in the electronic medical records space, actually using the electronic version of those records is still a thing of the future.  At this rate I'm not that optimistic that we'll
have the vest before Lime arrives, which is very frustrating because we've been talking to our clinic about making this happen for, oh, I don't know, 8 months?  Maybe we have unrealistic East Coast expectations for how a process should run, but even by Midwest standards this seems a bit ridiculous!   

Week 96: Non-stress

Lime has officially made it to 35 weeks gestation, which is great news.  It means that pretty much no matter when he comes now, he will be ready to enter the outside world with a minimum of complications, and that his entry can be facilitated by the midwives rather than by an OB.  He passed two more non-stress tests, one Thursday and one this afternoon (pro-tip: maternal consumption of chocolate chip cookies and iced coffee immediately prior to testing produces excellent results).  His Dopplers, which measure blood flow in the umbilical cord and in his brain also look excellent.  Next Monday, he has his 36-week growth scan to see where he stands, and based on that we'll begin a discussion about when he should make his grand entrance into the outside world.  Based on everything I've heard so far, the doctors here would like to see him delivered sometime around 38 or 39 weeks, although assuming he continues to do well I will advocate for waiting as long as possible, to see if he might decide to emerge of his own accord. 

Looking back on my pregnancy with Lemon, I find it sort of ironic that the pregnancy in which Papa Bear and I discovered that we were CF carriers was by far the less stressful of the two.  Of course we were shocked by that news, but once we'd absorbed it, we basically just marched ahead and luckily things were totally straightforward thereafter from a pregnancy and delivery perspective.  In contrast, this time around, the constant surveillance keeps Lime and his condition on my mind at all times, and I never sleep particularly well on the nights before his tests.  I try to trust my "mother's intuition" that he's doing fine, since he is a _very_ active little guy, but I still get nervous before each test.  Ah well.  I suspect the next couple of weeks are going to fly by, and he will be here before we know it.

Lemon has continued to absolutely flourish.  I would say he is growing like a weed, except that summer in Wisconsin has given me a whole new appreciation for how fast weeds actually grow--from tiny little sprouts to giant 3-foot-tall spiky behemoths in about no time at all, particularly if there's a good rainfall.  Luckily, Papa Bear requested a weed whacker as his Father's Day present this year.  For those of you who know Papa Bear personally, you may find this a bit surprising on any number of levels, but rest assured that Papa Bear is willing to let me use said instrument "whenever I want."  I spent a solid hour testing it out yesterday, and I have to say it's really fun.  Especially compared to pulling up weeds one at a time--I've determined that in my current state of pregnancy there's no way to accomplish that task comfortably.  Now at least our yard looks, if not manicured, at least lightly domesticated.

Week 95: Acceleration

Lime has continued to pass all his tests, which is great news.  He'll be measured again in a couple of weeks, and based on those measurements, the doctors will decide whether he needs to be delivered at 38 weeks, or whether he's doing well enough to remain in utero a bit longer.  I'm very much hoping for the latter, although I'm quite tired of being pregnant at this point.  Nonetheless, I'd like Lime to have the benefit of staying on the inside for as long as he wants.  But, if he has to come out at 38 weeks, that's only 3 weeks away!

So, preparation for his arrival has shifted into high gear.  I've taken up a second career as a furniture assembler, putting together a new changing table for Lime, and a little love-seat and a new big-boy bed for Lemon (with assistance from Opa).  I'm also scrambling to get one last painting project done before time runs out.  It's amazing how much rearranging and reconfiguring the arrival of one baby requires!

On Sunday I volunteered at the registration table for this year's CF Cycle for Life in Madison.  It felt very strange to be on "the other side of the desk" at a cycling event.  One of the many reasons I'm looking forward to not being pregnant anymore is the slim chance that I might find some time to get out and do a little riding again.  Hopefully it's true that once you learn how to ride a bike, you never forget, because I feel like it's been forever!

Week 94: The most of it

This week certainly wasn't what we'd planned or hoped for, but all in all it turned out fine.  Lime passed his first non-stress test on Thursday, and his second one today, so at the moment at least things appear stable on that front.  We have our first set of Dopplers to look at his blood flow on Thursday, so fingers crossed there.  In the mean time I am taking advantage of all these non-stress tests, which force me to lie still on my back in a quiet room with no one else for a half hour, to do something I haven't done since roughly the time that I started writing this blog: read a novel.  I can see why I used to do this a lot, it's great.  Something I aspire to get back to on a regular basis sometime in the next decade...

The schedule of appointments that I have to keep, plus the concern about Lime's growth, kept us here in Wisconsin and forced us to cancel our trip to a family reunion in Connecticut.  We were very disappointed to miss it but tried to make the most of it.  Papa Bear and I both took Friday off of work, with the intention of having a fun family day.  We did, although of course not as we had expected--Lemon decided to take an unprecedented 4-hour mid-day nap, which precluded the visit to a petting farm (with ice cream stand!) that Papa Bear and I had planned.  Instead, we took Lemon to the playground and he seemed totally fine with that as a substitute.  No ice cream, though!  We did enjoy a delicious dinner thanks to two of my aunts, who sent it over to us as a consolation for missing the big family dinner in Connecticut. 

On Saturday morning, we took Lemon to the busy downtown farmer's market to see Cows on the Concourse.  He had been talking about seeing the cows all morning, so we thought he'd be thrilled, but when we got to the actual cows, he looked at them with a disdainful expression for a few seconds and then hurried off to find some trucks to look at.

We had a much more successful animal encounter on Sunday, when we took Lemon to see the new Arctic Passage exhibit at our zoo.  He loved the polar bear and the seals, but most of all he loved the Tundra Buggy vehicle that was on display near them.  In addition to being huge and having giant wheels, it is surrounded by a bed of small gravel that can be picked up and dropped through a fence.  Pretty much heaven, if you asked Lemon.

So, all in all, not a bad week, really, except that Papa Bear and I knew what we were missing.  When we first moved here, we were a bit baffled by how infrequently people who live here travel to other parts of the US and the world.  Now, I think we're figuring it out--you may want to travel, but it is basically impossible to actually leave Wisconsin!

Week 93: Bigger and smaller

It seems that our uneventful stretch has drawn to a close--good thing we really enjoyed it while it lasted!

Papa Bear was away for most of this week, so Lemon and I have been enjoying an abundance of mother-son bonding time.  As a coping mechanism, I have adopted many of his daily strategies: frequent snacks, avoidance of any foods that require cooking, and declaring the day done at 6:30pm. 

We had probably our best visit ever at the CF clinic on Friday.  I was concerned because our appointment time was 4pm, which I thought for sure would mean a long wait.  Instead, they took us right at 4 on the dot, and since some of the folks at the clinic had already gone home for the day, we "only" saw the nurse, the doctor, the respiratory therapist, and the clinical study coordinator.  What a breeze!  Lemon is the fattest he's ever been, up to 35th percentile for BMI, which makes everyone very happy.  We are officially off of "hospitalization watch," meaning if Lemon gets sick again we can treat him at home with oral antibiotics.  And, he got fitted for his vest, which will soon replace the PT that I've been doing manually since he was tiny.  So all good news.

Then came today, when I had a 32-week ultrasound for Lime.  Unfortunately, the news there wasn't as good as we would have hoped.  He has asymmetric growth restriction, meaning that his abdominal circumference is disproportionately small compared to the rest of him, which is on the smaller side but still very much within the normal range.  Right now I have just been put on a very rigorous monitoring schedule, with twice weekly visits with various practitioners to track his growth and heart function.  I suppose there is never a good time to find out that something isn't quite right with a pregnancy, but this was particularly terrible timing since we were supposed to go on our last pre-baby trip this coming weekend to see all our extended family on the east coast.  We are terribly disappointed not to be making the trip but it's the only responsible choice for us right now.  The main thing we have to focus on is giving Lime the best chance we can at getting to full term at a good size, and that is what we are doing.  It is incredibly frustrating that we've tried twice in the last 6 months to get to the east coast to see family, and failed both times.  Hopefully the third time (whenever it is) will be the charm!

Week 92: Just regular

Sometimes, on a Monday morning, our nanny will ask me whether it's going to be a "regular" week.  Over the past few months, I've come to the conclusion that whether or not a week is regular is something that can only be assessed in retrospect.  And, I am delighted to report that the past week has been a regular week.  After so many irregular weeks in a row, having one regular week has felt like a vacation.




We kick off every morning with Pulmozyme.  Lemon's current favorite video to watch while doing this treatment is an animated film about a garbage truck who goes around the city collecting trash from dumpsters.  It begins with an overly hearty narrator saying, "Good morning, city.  The time is 5:30 a.m."  I really feel that I do not need to be reminded of this fact on a daily basis.  But, Lemon finds the video so transfixing that he sits as still as a statue during his treatment, so I feel that it is medically justified.  Then we move on to our first round of PT, with videos on diverse subjects including construction vehicles and trains.  After PT, we do breakfast, which begins with enzymes always features a high calorie shake, 2mL of special CF multivitamins, 1mL of extra vitamin D, 1.4mL of ranitidine, 2.5mL of cetirizine, and a probiotic.  Then Papa Bear and I head off for work, and Lemon has all sorts of fun with the nanny and Nona until I get home.

We've added a really fun ritual to the end of our day, where Lemon and I sit on some folding chairs at the end of our driveway, eat popsicles, blow bubbles, and remark on the steady stream of vehicles that drive past our house.  They come in a variety of models and colors, as it turns out.  Sometimes, they come in groups and can be counted.  Periodically, something truly spectacular like a tractor trailer goes by.  Eventually, Papa Bear appears either by bus or by bicycle, indicating that it is time to go inside to watch more videos, do another round of PT, and eat dinner accompanied by enzymes (of course), 1mL of iron, 2mL of vitamin D, a dose of glutathione, and 1.4mL of ranitidine.  Then there's nothing left but a bath, books, and bed.








This week, we also completed a feeding study with Lemon.  He's been in an observational study since he was born, looking at the effects of diet on outcomes in kids with CF.  Periodically, we have to record every single thing he eats for 72 hours, and we took advantage of the long weekend to get that done.  So, when you read the published study some day, just remember, this is how they knew how many miniature Goldfish crackers he ate!