Monday, March 30, 2015

Week 84: Ukes in springtime

Thankfully everyone stayed healthy this week, so I think we've made real progress towards getting Lemon back on track with his weight.  He's been sucking down 2 Scandishakes per day (~1000 calories total) on top of the rest of his food.  We have our next clinic visit tomorrow so we'll get an official assessment then, but I'm optimistic that even if he isn't back to his all-time high of 10.1kg, he'll at least be on his way.

The nebulizer continues to go well.  I ended up ordering a new mask and nebulizer cup online (I got the PARI baby setup for those who care).  I was a little disappointed with our clinic, I have to say--after a day or two with the system they gave us, I sent them a message saying that the mask was too big and didn't stand up to steam sterilization, and asked if they could please send us something smaller and made of silicone.  In response, they sent me a package in the mail with a new set-up exactly like what they'd given us before, plus one additional mask that, while smaller than the first one, is also not steam sterilizable.  Humph.  Thank heavens for the internet, that allows patients to easily learn more about what's out there in terms of options!  Now I just have to find out whether I can exchange the compressor that they gave me for the one that is actually supposed to go with the PARI setup (which they do give to patients, btw, just for a different medication), and we can put this whole little adventure behind us.

This weekend we had a wonderful visit from Uncle Joel and Aunt Donna, who came to see us as part of their epic road trip from Maine to Wisconsin via Florida, Louisiana, Texas, Arizona, and Colorado.  We had a great time, featuring lots of books, walks to see our favorite sights, and of course, ukelele music.  Lemon had an absolute blast.  They left this morning and Lemon has been asking about them all afternoon. 

It was a nice break to have a full week of only normal and fun events after several weeks in a row dominated by various medical things.  Hopefully we can sustain our current pattern through the spring!

Monday, March 23, 2015

Week 83: Six impossible things before breakfast

Thanks to everyone for your concern about our collective health--a week later and I think we are all through the worst of it.  My head has cleared, Papa Bear's pneumonia has resolved, and Lemon is (knock on wood) no longer coughing.  He's still on antibiotics as a precaution but we're hoping the doctor will give the OK to take him off them in a few more days.

This week, we've focused on integrating Pulmozyme into our daily routine.  Pulmozyme is an enzyme (DNase, for those who care) that helps break up the thick mucus that CF patients have in their lungs.  It is given with a nebulizer, which makes the liquid medication into a fine mist that the patient can inhale.  It takes about 7 minutes to administer one dose of Pulmozyme.  So, our first challenge was to identify the 7 minutes in a day when Lemon was least likely to object to sitting very still.  We decided on first thing in the morning, when he's still sort of peaceful and half asleep.  So our new morning routine goes like this:

I wake up at 5:45, stumble into the bathroom and thoroughly wash my hands.  Then I go to the refrigerator and get a dose of Pulmozyme, which comes in single-dose plastic ampules.  Back to the bathroom, where the nebulizer parts are sitting waiting for me inside the steam sterilizer.  Assemble the parts of the nebulizer, and squirt in the medication.  It's now about 6, and I head into Lemon's room.  Without turning on any lights or doing anything that would wake him up further, I get him out of the crib, sit him on my lap, hold the mask of the nebulizer up to his face, and turn on the compressor.  Then we talk about the day ahead, sing songs, etc, until the treatment is done.

At around 6:10, we're heading out of Lemon's room and back to the bathroom, where I take the nebulizer apart and put it to soak in a bowl with hot water and soap.  Then off to the living room for chest PT.  Finally at around 6:35 we head into the kitchen, where I make a shake, get Lemon into his high chair, administer enzymes, two oral medications, two vitamins, and breakfast.  Once Lemon is going strong on breakfast, I head back to the bathroom one last time to rinse the nebulizer parts, toss them into the sterilizer, and get that running so that everything is nice and sterile and waiting for us the next morning.  By then it's almost 7, so I have a generous 12 minutes to eat breakfast, drink my coffee, brush my teeth, get dressed, and be ready to hand Lemon off to the nanny and leave for work at 7:30.  Except for mornings like this morning when I also had to shovel 4" of fresh snow off our driveway, and ended up wondering if there was a way that I could eat my breakfast and brush my teeth at the same time.

Lemon has taken surprisingly well to the whole nebulizer scenario.  He was curious and skeptical the first morning.  The second morning, as soon as I walked into his room, he said "Mask on!  Good boy."  The third morning, he said "Mask on!  Compressor on!  Steam!  No pulling!"  And it's been pretty much smooth sailing since then, with the exception of a minor technical hiccup involving the mask.  Although the mask the clinic gave us is very cute and kid-friendly, and they specifically told me to clean it in the steam sterilizer, the plastic it's made of isn't actually heat stable, so it sort of warped and changed color.  It is also too big for Lemon's little face, so they are sending us a smaller silicone one that should hopefully solve both problems. 

I feel like we're slowly regaining ground in terms of Lemon's weight, and we'll find out more at his next clinic visit a week from tomorrow.  His new favorite dessert certainly helps!

Monday, March 16, 2015

Week 82: Best week EVER

When I left you last week, Lemon was gradually improving from the cough that he'd developed the week prior.  We think it took longer than usual for the antibiotics to kick in because he was coughing to the point of vomiting on a semi-regular basis, so about half of the antibiotics he got during those first few days didn't stay around long.  Luckily, that tapered a bit as the week went on, and I'm sincerely hoping Friday was the last incident since after that one I was sorely tempted to drag our highchair down to the curb and leave it there rather than dealing with the cleanup.

Given that Lemon spent about a week coughing violently directly into our faces, perhaps it's no surprise that by mid-week this week Papa Bear and I weren't in perfect health anymore.  Both of us were tired and had some pretty standard-issue cold symptoms.  These continued to get worse and worse, to the point where I was coughing and blowing my nose like no tomorrow, and Papa Bear was the same plus a fever of 102F. 

Just as a precaution Papa Bear went to the urgent care clinic on Sunday morning, and good thing he did, because his "cold" had blossomed into bilateral bacterial pneumonia.  So, he came home on two antibiotics and strict no-contact orders.  He's basically been hiding in our bedroom ever since, emerging occasionally for sustenance and new boxes of tissues.  Luckily, I never developed a fever or pneumonia because given that I'm pregnant they probably would have wanted to admit me to the hospital.  So, on top of being sick and pregnant, I've been doing all of Papa Bear's share of Lemon's care as well as my own.  Luckily Nona has been very generous with her time so I can put my feet up for a few moments here and there.

In any event, because Lemon still had a residual cough at the end of last week, we decided to bring him in for an extra clinic visit this afternoon.  It confirmed our worst fears--he's back down to 9.5kg, meaning that we lost about 6 months worth of weight gain in 10 days, and he's now fallen off the lowest curve on the weight-for-age chart.  In other words, we are in crisis mode.

Our most important objective is to keep him from getting sick again any time in the near future.  He has absolutely no reserves left, and needs to stay well for long enough to gain the weight back.  So, Wednesday morning we will be starting him on Pulmozyme, an inhaled medication that helps to break up mucus in the lungs.  The idea is that if his mucus is thinner, perhaps it won't be quite as easy for bacteria to take root there every time he gets a cold.  I'll write more about this next week, once we've actually started and have a better idea of how we will work it into our lives.

We're also re-twiddling everything to do with his digestive system.  After trying Pertzye for a while and not really seeing any difference, we will be going back to Creon for his enzymes since those we can get from our regular pharmacy, and Lemon seemed to have an easier time swallowing those beads than the Pertzye beads.  We're switching from ranitidine to lansoprazole, a stronger acid-reducing medication, to try and get maximum efficacy from the enzymes.  We're going to double up on the probiotics for the rest of the time he's on the antibiotics to try and keep his gut populated enough to actually digest his food.

Of course, the most important variable is something we don't have control over--what Lemon decides to actually eat.  Before he got sick, he was drinking two Scandishakes per day (1200 cal total) on top of the rest of his food.  Now he's down to an average of less than one per day, and mostly just picking at everything else.  Hopefully as he continues to feel better, this will pick up again.  Since Papa Bear and I have the same thing, I can say from personal experience that it really does make you feel lousy and not at all like eating, so maybe as we all clear this we can get back to something resembling normal food consumption.

Oh, and thanks to Papa Bear's visit to urgent care, we now know what has caused us all this misery--everyone's favorite virus, RSV.  It's no joke, folks.


Monday, March 9, 2015

Week 81: And Losing It Again

After the great news about Lemon's weight gain at the pediatrician's appointment last Monday, we were brought back to earth by a week sent to remind us that CF is always there, lurking, waiting for a moment when you feel like you might have gained the slightest edge, to bring you back to earth.

On Tuesday, we celebrated Papa Bear's birthday with an appropriately nerdy birthday cake (for those who care, those are the branched chain amino acids).

When we got home, we found that Lemon had developed a bit of a runny nose (picked up at the pediatrician's office, a version of an illness our nanny had, or something else entirely, we'll never know).  Of course, we hoped against hope that THIS would be the cold that he managed to kick by himself without antibiotics.  Not that he's ever managed it before, mind you, but we keep hoping.  And, for the next couple of days, he seemed more or less fine.  His nose continued to run, but he continued to do all of his normal things as well, so we kept on with our normal routine.  By Thursday evening, he was coughing occasionally, but still in good spirits. 

Friday morning we had our 20-week ultrasound for Lime (where is the time going???).  He looks normal in all respects, including his intestines which were checked to see if he has any signs of meconium ileus.  He doesn't, which is great although it doesn't foreshadow things one way or another in terms of CF diagnosis--Lemon had normal looking intestines, too.  Anyhow, Lime was busy whirling around so much in utero that most of the "still" pictures of him came out as white blurs on a black background, and even the 3D reconstructions didn't come out well--nothing really worth sharing.  And, assuming all goes according to plan, that will be our last look at him before he arrives in the air-breathing world sometime in late July.  So, all of us will have to just live in suspense!

Friday afternoon, Lemon's cough seemed a bit worse and his appetite had basically disappeared, so we were starting to think that probably on Monday we'd call for antibiotics, but it didn't seem quite bad enough to call immediately.  The difference between Friday night and Saturday morning was stark, though.  The cough deepened quickly into something formidable--big, wet coughing fits that shook Lemon's entire body, plus a moderate fever and no energy whatsoever.  We called in first thing Saturday morning and had the antibiotics in hand by mid-day.  We spend most of the rest of the weekend (when we weren't sleeping or doing extra sessions of chest PT) like this:

It took a good 36 hours before we started to see any impact of the antibiotics, but by late Sunday afternoon there were glimmers of hope.  Lemon started talking a little bit again, and found the energy to stand on his own feet and walk around a little.  He even ate a container of tapioca pudding, his first real solid food since Thursday.  Today he was better still--definitely tired and fragile but coughing much less and eating a bit more.  Of course, that precious 250g that he gained to get over the 10kg hump is totally gone--I can see every rib and vertebra when I give him a bath.  So, we have a measly 3 weeks before our next weigh-in to try and get that weight back on, plus hopefully a bit more.  And so it goes. 

Antibiotics really do feel like a miracle to us--this was without a doubt the worst cough Lemon has ever had, and the antibiotics were able to get him straightened out in relatively short order.  We are so lucky (and grateful) to be fighting this disease with modern medicine to help us. 

Monday, March 2, 2015

Week 80: Gaining ground

Today we had Lemon's 18 month checkup with the regular pediatrician.  I wonder what parents of normal kids worry about the most when they go for one of these visits--maybe the shots?  For me, it's the scale.  I'm always worried about whether he gained weight or not, and how much.  Once I set him on the scale, I just stare at the display, hoping, waiting for the numbers to come up.  And today, we finally cracked the 10kg barrier that we've been close to for so long.  Not by much, 10.09kg in all, but we'll take it.  To achieve that, our little now-22lb human had to consume a minimum of 1200-1400 calories per day, which is enough to sustain many adult women who are five times his size.  So, we will keep on with the strategy we are using now, and see if we can keep the weight gain up for another month, when he'll be seen at the CF clinic again. 

He may be small, but Lemon did manage to impress the pediatrician nonetheless.  He demonstrated some of his vocabulary, including apparently advanced words like "strawberry" and "enzymes."  His best trick, though, came when the doctor asked if she could count his teeth.  He opened his mouth to let her look inside, and she started counting.  When she got to eight, she paused for breath, so he chimed in, crystal clear, "nine, TEN!"  She said, "Wow, that was spooky!  What else does he know?"

Other than that, this week has been pretty uneventful.  We're readjusting to our life here in Madison.  It's hard to believe we were in Hawaii just a week ago, it seems like another world.  We're settling back into our old routines, and getting ready for a bit of snow tomorrow.  Also, tomorrow is Papa Bear's birthday so we've made some modest preparations for that.  I'm a little concerned because the only thing Papa Bear requested for his birthday this year is a kitchen gadget.  Isn't mid-30's a little early for age-related cognitive changes?