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. 

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.

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.

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.

Week 123: Love that dirty water

Never a dull moment around here. In the midst of our round of home IV antibiotics, Grandma Carol, Grandpa Dudley and Uncle Jared arrived from New York for Christmas. Grandma Carol and Grandpa Dudley had been planning to come for Christmas for a while, and we'd almost cancelled their visit when we were admitted to the hospital.  After we got discharged from the hospital, though, we figured we should just stick to the original plan.  Uncle Jared had some unused vacation time to burn before the year's end, and apparently felt like a full immersion in our chaotic household was just the thing to soothe his nerves at the end of the year.  All in all it worked out surprisingly well, given that we were running home IV's at the same time as all these people were in and out of our house. I'm especially glad that the grandparents made it out, since it was the first time they met Lime, and they hadn't seen Lemon in quite some time. Grandma Carol got right into the swing of things, watching videos with Lemon while he did his various treatments. 

On Wednesday, we went to the clinic for a follow up visit. Since Lemon seemed to be back at baseline, we crossed our fingers and had the PICC pulled. It was an uncomfortable procedure and Lemon handled it amazingly well. Now we're just keeping up with his normal maintenance routine and waiting to see if the IV antibiotics did the trick. We've also added Flonase to his daily schedule in the hopes that it will help keep his nose from running and break the runny nose - cough - antibiotics cycle that we have been on too many times. On Thursday, we celebrated Christmas a day early, before Grandma and Grandpa went back to New York. So many toys, so little time.

And, that more or less brings us to today, where somewhat against our better judgement but pulled along by the lure of friends we haven't seen in entirely too long, we drove off into the teeth of Winter Storm Goliath to fly back to Boston. The fact that I am posting this from a hotel room in our old neighborhood means that we made it. Don't ask me how. I will say that traveling with the vest was difficult in a way that none of us had anticipated. TSA and the airline didn't make so much as a peep about it.  Leave it to the two year old to find a way to make life more interesting.  The compressor for the vest packs into a rolling case about the same size and design as a regular carry-on, and while we were waiting at the gate before boarding, Lemon decided to stick his head between the two upright parts of the handle on the case and was temporarily stuck, much to his great dismay.  Hopefully the experience was traumatic enough that he won't make the same mistake again!

It is definitely weird to be walking around these old familiar streets after a year's absence. Our hotel is across the parking lot from our old pediatrician's office, where we received Lemon's CF diagnosis. Right after we arrived this afternoon, I ran across the street to CVS to get whole milk and high-calorie snacks, and had a vivid flashback of the hour we spent in the very same store, two and a half years ago, fighting with our insurance company to get Lemon's first prescription for enzymes filled. It's an odd lens through which we CF mamas view the world, that's for sure. Now I'm enjoying a coffee mug of white wine from our old local wine store (keepin' it classy!) and hoping that once the kids adapt to their new surroundings, their behavior will return to normal so that the friends we've traveled so far to visit won't deem us unfit parents. 

I note, without a shred of regret, that this is the last post for 2015.  It's been quite a year, and I won't be sorry to have this one in the rear view.  Let's see what 2016 has to offer--I wish all of you the very best of health and happiness in the year to come.

 

Week 122: PICCing up

It's been a week of a few more surprises, not the least of which is that I'm writing this post from home, at my own computer.

Surprise 1: I thought I knew what tired was.  I was wrong.
Surprise 2: I thought we had a complicated medication schedule before.  I was wrong.
Surprise 3: I thought I knew just how resilient and generally amazing Lemon is.  I was wrong.

On Tuesday of last week, we had a PICC placed.  After the rodeo-type scenario that was the IV placement, we decided to put the PICC in under general anesthesia, rather than just sedation.  Since Lemon was going to be down in interventional radiology anyhow, we took chest and abdominal X-rays, too.  It cracked me up that the radiologist, who was younger than I, referred to it as a "chest film" even though I'm quite sure he's never touched a physical piece of film during his time as a physician.  In any event, the PICC placement went very smoothly and the X-rays looked normal, so that was a win overall.  On Wednesday, the doctors evaluated Lemon and his home team, and decided that we could be discharged to finish up the IV's at home.  Hooray!  We were discharged Thursday afternoon.  By the end of the day Thursday, we'd received a giant bag of supplies from the home health pharmacy, and a visit from a nurse to teach us how to use them.   

Overall I am delighted to be home.  It is vastly better for the kids--they can live their normal lives with a minimum of disruption.  For Papa Bear and me, this existence really stretches the definition of normal, but I'm more than willing to stretch if it keeps my kids feeling like regular kids.  Here's what happens in our house these days (in order to keep this to a manageable length, I'll just focus on what happens between 5:30-6:30 a.m., and you can just use your imagination to fill in the 3 additional meals, 2 additional IV treatments at 8 hour increments, and 2 additional vest/nebulizer treatments that happen at other times of day, plus the usual shenanigans of caring for a 5-month-old infant and keeping a household running).




















5:30. Get up.  Wash hands and put on gloves.  Connect tubing to new syringe of antibiotic, load syringe and tubing into pump.  Prime two saline syringes and one heparin syringe.  Lay out other assorted IV supplies on tray.  Assemble two neb cups and masks.  Load with hypertonic saline and Pulmozyme.

5:45. Go into Lemon's room.  Get therapy cart out of closet, plug in power strip and compressor for vest.  Slip vest onto Lemon by opening one shoulder so as not to interfere with the tubes coming out of his right arm.  Sterilize connector on the PICC with an alcohol prep pad.  Flush line with one of the saline syringes.  Connect tube from antibiotic syringe to the connector.  Connect hoses to vest.  Administer two puffs of albuterol.  Start vest.  Connect neb cup with hypertonic saline to compressor and start compressor.  Check to make sure antibiotic is really running.

6:10.  Switch to Pulmozyme neb cup.  Express enthusiasm for the remarkable rendition of  "Wheels on the Bus" that we are watching on YouTube. 

6:20.  Power down vest and disconnect hoses.  Put on fresh gloves.  Disconnect tubing from the PICC.  Connect saline syringe to the PICC and administer the first flush, disconnect and then connect heparin syringe for the second flush.  Remove vest.  Gather up disposable debris and put in trash.  Remove gloves.  Take off vest.  Stow cart and various supplies in places where a two year old will not destroy them.

6:30.  Breakfast.  Prepare Scandishake.  Mix applesauce, probiotics, and enzymes.  Convince Lemon to ingest the aforementioned.  Load syringes with ranitidine and Zyrtec.  Convince Lemon to ingest the aforementioned.  Locate multivitamin and vitamin D solutions.  Convince Lemon to ingest the aforementioned.  Locate coffee cup.  Discover that coffee is cold.  Put in microwave for 33 seconds.  Ahhh.  Ready for the day to begin.

The good news is that Lemon does seem to be more or less back to baseline.  We have a follow-up appointment at the clinic on Wednesday, and my guess is that they will take the PICC out.  Then, we'll watch and wait.  Hopefully all this has been worth it, and whatever has been causing all Lemon's troubles will be gone, and we can return to our version of normal.  Although I wouldn't recommend this lifestyle as an overall fitness plan, it has had one noticeable side effect--two kids later, I could fit into my wedding dress today (if I still owned it, that is).  

Week 121: In

For a brief moment last week it looked like we might have kicked this bug. After adding hypertonic saline to our daily routine, Lemon was able to get back to his baseline. Unfortunately, once the oral antibiotic ended, the runny nose returned with the cough hard on its heels. I knew pretty much as soon as his nose started to run that we were going to end up being admitted, but we managed to hang on in a state of semi-denial until Sunday morning.  Then, knowing that an admission was inevitable, we decided to just head in rather than waiting at home for Lemon's symptoms to get any worse. I think that was the right call, especially since Sunday afternoon is a pretty quiet time at the hospital and we were able to get ourselves and all our stuff situated without too much trouble.  

It required the combined effort of 6 adults to get Lemon's IV placed, but after that trauma it hasn't seemed to bother him much. He is receiving Zosyn, a broad-spectrum antibiotic, which hopefully should kill whatever is causing his troubles. We're also doing 4 vest treatments per day in an effort to shake out whatever we can and help the antibiotics work. 

There are a lot of things that are unsurprising about this hospitalization so far: terrible food, constant interruptions by various staff members, blood sugar monitoring at 2am, etc. 

Some things have surprised me, though. One is the amount of attention it requires to keep a two-year-old from pulling out his IV by accident. Lemon has been amazingly good about not messing with it on purpose, but he moves around a lot and those little tubes seem to want to get twisted and snagged on every possible point of entanglement. And then there's the pole and the wire that plugs it in and the furniture and the toys and the other people in the room. Another thing that surprised me is that we're essentially using all our own gear and medicine here at the hospital. We brought our own vest and compressor, and all our own medicines and vitamins. Good thing, too, especially for the enzymes, otherwise we'd be asking Lemon to wait 45 min before each meal or snack while an individual enzyme capsule was carried in by passenger pigeon from Nebraska. 

Some other minor surprises include the fact that this room in a children's hospital doesn't have a changing table, which makes two kids in two different size diapers a real challenge, never mind the fact that they want us to save Lemon's diapers so they can measure his output.  Also, room service will not deliver coffee, and you can't even call until 6:30 for your non coffee to be delivered, which doesn't help much if the kids are up at 5:15.  Rest assured my little coffee pot from home is now installed in a spot befitting its importance in terms of my survival. 

None of this would be as big a deal as it is were it not for the fact that we're not allowed to leave the room, lest we spread whatever Lemon has. I'm told there's a nice kitchenette on the floor with a refrigerator and hot water and whatnot, but it's of no more use to me than my kitchen at home. There's also a nice activity room and family lounge that we saw on our way in and won't see again until we leave. 

So, that's our story. Lemon, Lime, and I in a hospital room together for the next 10-14 days, with our key support people and the ever changing roster of staff drifting in and out. We live so close to the hospital that I can see the foot of our street from our 5th floor window, and yet home sure seems pretty far away right now. 

* written on my phone, so no photos this week--hopefully next week I'll figure out a way to get the pictures up.