Monday, January 25, 2016

Week 127: Off and on

This week, we're remembering Great Grandma Virginia, who passed away a few days ago.  I will never forget the first time I met her.  She eyed me critically from her position on the sofa, declared that I was tall, had good teeth and good child-bearing hips, and accepted me into the family immediately.  She always welcomed our visits, and never failed to deliver a few zinging one-liners when we were there.  One of my favorite memories of her involved a well-meaning neighbor of hers who Virginia often found annoying.  We were over for a visit when the phone rang.  Grandma Carol answered the phone, and it became obvious that she was speaking to the neighbor in question.  From across the room, at volume sufficient to be heard several blocks in either direction, Great Grandma Virginia (slightly hard of hearing) yelled, "I'm not home!" 

I love this picture of her and Lemon from a couple of years ago when we visited her, it totally captures the spirit of her interaction with him.  She was the last of our kids' great-grandparents, so she leaves behind a void in their lives, and ours, that can't be refilled.  We will always treasure our memories of her.  

Aside from this sad news, it's been a fairly normal week around the homestead.  We're continuing to experiment with ciproheptadine to try and figure out the best way to maximize its effects on Lemon's appetite.  Last week, we noticed that it had a really potent effect for the first several days that he was on it, but then the effect began to fade.  Our clinic had mentioned that a lot of kids cycle on and off of the drug in order to maintain its effects, but they hadn't commented on how long the cycles were.  Since it seemed like we were losing efficacy, I consulted the internet, and found out that there are a surprisingly large number of ways that people cycle this drug (and those were just the ones that I found).  One schedule that I saw mentioned several times was 5 days on, 2 days off.  That seemed to me to fit well with what we'd seen, several days of high efficacy followed by a taper, so we're going to try that schedule until our next clinic visit and see where it gets us.  We went out for our first family meal at a restaurant as a foursome, which was much less of a disaster than we'd feared.

In Lime news, he's figured out how to sit unsupported, and is pretty good at it although it certainly helps to have quick reflexes if you're serving as his support person while he's attempting it.

 I will try to avoid too many "time machine" photos, but in order to console the Patriots and their fans I feel compelled to post this side-by-side of Lemon and Lime showing their true colors during the playoffs.

A few other highlights from the week--some strumming,

 some fire-engine viewing,
 and some chilly winter walks.
For the first time in a very long time, I feel like our family had a regular week.  Some happy times, some sad, some lovely memories, some very early mornings, a lot of coffee.  Yes, of course, some treatments and medicines too, but I am so glad that at least for the last 7 days, CF has left us more or less alone to just be a little family in a regular house in a sleepy, wintery city in the Midwest.  I could do a few more weeks just like this. 

Monday, January 18, 2016

Week 126: Skin and appetite

I feel as though lately we've just been swinging back and forth on the pendulum that is CF, wrestling with digestion and weight gain for a while and making some small progress, then switching to respiratory issues as we try to battle another infection, then back to digestion again once the infection is gone.  We had our 3-week follow up visit on Wednesday of last week, to see how things have been going since the PICC came out.  The good (great) news is that Lemon has held his own in the respiratory category, so we're just sticking with his current treatment regimen and continuing our vigilant march through winter. 

The less great news is that we're really in trouble now on weight gain, with only 300g gained in the last 5 months.  For the first time, the clinic brought up the idea of placing a gastrostomy tube, which is a tube through the wall of the abdomen and into the stomach for delivering tube feedings.  Lots of kids with CF have them, but it's something I've been very much hoping to avoid.  As our last ditch attempt to get Lemon to actually put food into his mouth, rather than me having to pump it into him with a syringe, we're doing a trial of cyproheptadine.  Although first developed as an antihistamine, it's now used a lot for one of its side effects--it's an appetite stimulant.  We started it on Thursday.  Luckily it's a clear syrup, which Lemon objects to much less than the cloudy suspensions, because we have to give him 5mL 3 times a day.

The results have been immediate and dramatic.  Lemon has eaten more solid food in the last 5 days than in the entire month of December, I'm pretty sure.  He's comfortably sucking down two Scandishakes a day, whereas for the longest time now we've been struggling to get him to consume one.  So, fingers crossed that the drug will continue to work and he'll put on some decent weight before our next follow-up in a month. Then, we can put the idea of the G tube on the back burner again for a while.  I will of course do it if that is really what it takes.  We need Lemon to gain weight for his long-term growth and health.  I would just much rather solve the problem with food and eating if we possibly can, and I'm hoping this drug will help us get there.  We're also going to meet with a psychologist to talk about eating behavior, so hopefully with his chemically augmented willingness to eat plus some new strategies, we'll manage to stay on some kind of growth curve until his brain is no longer two years old.

Although the cyproheptadine was just one more new medication, it made me reflect once again on just how much medicine it seems to take for us just to maintain our "normal" these days.  I suppose maybe it's because the new drug is 3 times a day, so it adds yet one more event to our daily health calendar.  Our routine is now:

Morning: albuterol, vest, saline, Pulmozyme, probiotic, Zyrtec, ranitidine, cyproheptadine, multivitamin, vitamin D

Mid-day:  cyproheptadine

Evening: albuterol, vest, saline, Pulmozyme, Zyrtec, ranitidine, cyproheptadine, vitamin D, iron, nasal saline, Flonase

Papa Bear figured out that the routine totals 32.5mL of oral
medicines and vitamins a day at baseline.  It's quite a lot when you're barely a 12 kg human being.  Not surprisingly, I've been thinking a lot about teaching Lemon to swallow pills lately, but I think that's a challenge for another week, or at least for a day when the children make the collective decision to sleep past 4:15 a.m.

Monday, January 11, 2016

Week 125: Limeade

How is it possible that Lime is already 6 months old?  He had his 6-month checkup today, and continues to be short and stout, although somewhat less short and somewhat more stout--he did a lot of growing over the last two months and climbed up to the 25th percentile for weight and 7th for length, a gain of 5 percentage points in each category.  He had nothing but smiles for everyone we saw at the pediatrician's office (well, except for the nurse who gave him his shots, but he forgave her quickly).  And, I got to feel like the ultimate modern working mom, doing a conference call from the pediatrician's waiting room while entertaining Lime with toys and walking him around to look at things. 

I feel like Lime's babyhood is sailing by far too fast.  All the drama of the last two months has made the time fly, and I feel like I've hardly had enough time to relish him the way he deserves.  I say that, and yet I am within 20 feet of him at pretty much all times, and often much nearer than that.  But it's still not enough.  He is my last baby, and once he is not a baby anymore that will be it.  And I'm not quite ready.  I've started to gradually get rid of some of the baby gear, which is bittersweet.  I'm looking forward to sleeping more, it is true, but it will be slightly heart-breaking when no one in my household has those adorable wrist fat rolls anymore.

Oh Lime, always know that you were born second but are never second in my affections.   I know it took you too long to get your own blog post, and there will be lots of times in your life when you will feel like you are coming second.  It's never going to be true, and I hope you will manage to come away from the experience of your childhood a more empathetic and compassionate person after facing the challenges that you will face.  Your sunny little smile always brightens the room even when things look grim.  Our family wasn't complete without you, and we will treasure you in your own right always.

Monday, January 4, 2016

Week 124: Reentry

2016 has gotten off to a grand start.  The IV's seem to have worked (knock on wood!!!), and Lemon's health has been excellent all week.  We had a wonderful time in Boston reconnecting with many of our oldest and dearest friends, most of whom were meeting Lime for the first time.  He was in his usual fabulous mood, and had a big smile for everyone he saw. 

Traveling with two little kids is no joke, especially when one of them has CF.  All the gear makes travel really cumbersome, and the treatment schedule constrains us and definitely eats into the time we'd otherwise be spending with friends or doing fun stuff.  It also seems like an inordinately large amount of work to essentially replicate our home set-up somewhere else.  We stayed in a hotel room suite, which was essential since we need a refrigerator for refrigerated medications and a stove for sterilizing our nebulizer cups. There may or may not have been an incident involving the stove and a dish towel. If there was such an incident, Papa Bear assures us that it involved smoke, but no actual open flames.  I wasn't there at the time, so all I can say with confidence is that the hotel staff insisted that we turn our ventilation system up to maximum and that they revoked our dish towel privileges for a few days.

Given that we got home at 10 last night and both kids were up before 5, I will keep things short  this week.  It is hard to believe that it has been two years since we moved to Madison.  We've come a long way since then, but Boston is still "home" to me in many ways.  Our house is in Madison and the kids' lives are based here, so in that sense, Madison is home.  But, when I walk through the old neighborhoods and visit all my friends, I can feel the illusion that it might be possible to slip back into my old life.  I know that life is history now, but I do want the kids to have a sense of belonging in New England--it is Lemon's birthplace after all.  One thing that I still find strange about Wisconsin is how rarely people who live here travel outside the state, and I don't want that to be our kids' experience, in spite of how difficult it is to travel with them.  So, we learned some good lessons on this trip, and with any luck we'll have the chance to apply them on another trip sometime soon.