Week 120: Compressed

As I mentioned last week, we introduced nebulized hypertonic saline to Lemon's treatment regimen, in the hopes that it will help him kick whatever it is that we've been treating with antibiotics for the last month.  When we got our training last week, the respiratory therapist said that each treatment should take about 20 minutes.  Well, we ran it at home and it took over 30 minutes.  We've had this issue before, with our nebulized treatments taking much longer than they should.  In fact, over the summer I was convinced that there was something wrong with the compressor that drives the nebulizer, and I exchanged our unit for a new one under warranty.  That didn't make much difference, and we just sort of came to accept that the treatment we were doing took longer than it was supposed to. 

That was all well and good when it was just one small (2.5mL) treatment, once a day.  But, adding two additional treatments at 4mL each and we could see that all this nebulizing was going to dramatically increase our daily treatment time.  And we don't have more time in the day, as previously discussed.  I sent a message to our clinic explaining that our treatments were still taking too long, and asking for their suggestions.  The message I got back said that the respiratory therapist forgot that we were doing our treatments at home (where did she imagine we were doing them, I wonder?), and had quoted us the time it would take with the equipment that they have at the hospital.  Their proposed solution was to just run the nebulizer for 20 minutes each time, knowing that the full treatment wouldn't be delivered.  But, they reasoned, Lemon is small and probably 2/3 of the dose would be enough.

I didn't find this answer very satisfying.  First of all, Lemon won't always be small, and he will be doing nebulized treatments for the rest of his life.  So we need a solution that will work both now and when he's bigger.  Second, why not give him the full dose of each medication?  If we're going to go to all the trouble of doing the treatments, I feel like we should do them right.  Finally, the number of nebulized treatments that he has to do will only go up, so we need to solve this problem now, not later.  It seemed like our clinic didn't have the answer I was looking for, so I took to the internet, and consulted some sources of unknown veracity, like, you know, blogs.  And, I discovered that there is a whole category of compressors out there that for some reason our clinic never told us about, ones that can generate similar amounts of pressure to what they showed us at the hospital.  Yes, they may not be covered by insurance.  But we found one for $270 and thought it was worth a try, so we ordered it.  Happy Hannukah, Lemon!



Here's a demonstration of the difference between the compressor the clinic gave us and the one we bought.  In the video, the nebulizer connected to the new compressor (Mobileaire) is on the left, and the old (Innospire) on the right.  My assistant's arm can be seen in the lower right.  Look at the difference in output (mist) coming out of each one.  Night and day, right?  Now, we can get all our treatments done in about half the time as with the old compressor. 

While I'm so glad that we were able to track down this solution, and are fortunate enough to have the resources to just buy it out of pocket, it's raised some questions for me.  Why is it that our clinic never even mentioned this category of compressor to us as a possibility?  Why did I have to use a combination of Google and looking at some of my favorite CF blogs to track this down?  Why will insurance companies cover a nebulized medication that costs $1000+ per month, but balk at $270 for the machine that will allow you to actually deliver the treatment in a time short enough that it's compatible with the patient living a vaguely normal life?

Anyhow, enough on compressed air.  Lemon came off antibiotics yesterday, so we're just keeping our fingers crossed that he stays healthy for the next few days/weeks and that we can avoid a hospitalization.  Not that there's ever a good time for a hospitalization, but with Grandpa Dudley and Grandma Carol coming to visit for Christmas, and our much anticipated trip back to the east coast the following week, this would be a particularly bad time.  Of course, we were not so foolish as to purchase any non-refunable tickets or hotels, but here's hoping that just for once we'll be able to execute a trip as planned!

Week 119: Salt water

Where are we?  I'd say about 80% of the way there, which is not quite far enough.  We started Lemon back on cefdinir (an antibiotic) a week ago, and it kicked in quickly.  It got his cough back down from many, many times an hour to just a few times a day.  We've done three sessions of the vest every day since last Sunday.  We've added back two doses a day of Zyrtec to try to help dry up his various secretions.  But, we are most definitely not at baseline.  That wet cough is still lurking in the background, just waiting for a session of the vest or a dry bite of food to bring it to out.  Lemon is feeling and acting fine, and yet things are not quite fine, and we are nervous about what will happen after this coming Sunday, when our second two-week cycle of cefdinir ends. 

I do not like the feeling of being a sitting duck, so I called the clinic this morning and we managed to get in for an extra visit today before our extra visit next week.  No surprise, Lemon has not gained any weight since last time, but he hasn't lost any either.  He'd seemed so well in the waiting room that I was starting to question whether it was really worth bringing him in today, but he was eating snack when the nurse practitioner was in the room with us so she heard him cough several times and agreed that we are definitely not at baseline and need to get there whether we do it outpatient or in the hospital.  She outlined three possible scenarios.  One was to admit him today, one was to see if he manages to return to baseline by next Tuesday and if so just go on our merry way, and the third was to admit him from clinic next Tuesday if he isn't at baseline.  We agreed that it seemed premature to admit him today, so our focus turned to how to increase the chances of option B over option C.


















The one thing we had left to try was hypertonic saline, an inhaled solution of salt water that acts as an irritant to increase coughing and mucous clearance, and also helps to add moisture to the thick sticky mucous in CF lungs, allowing it to be cleared more easily.  The nurse practitioner and our regular doctor had been hesitant to add it at this visit, because they weren't sure if we would be able to distinguish the increased cough that might result from adding hypertonic saline to our regimen from the increased cough that we're hearing as a result of this illness.  I said I'd rather take that chance and try it than get admitted to the hospital next week knowing that there was one more thing we could have tried at home.  If there's one thing I have expertise in at this point in my career as a CF mama, it is distinguishing between the many different types of coughs that a two year old can produce, so I think we'll be able to tell what's going on a week from now. 

Of course, this decision extended our "brief" sick visit by an hour, since we had to try the first hypertonic saline treatment their in the clinic with the respiratory therapist, to make sure that Lemon didn't have any adverse reactions.  The first step of the treatment is an albuterol inhaler, which relaxes the airways and makes it easier for the treatment to penetrate.  The next step is the hypertonic saline itself, which takes about 20 minutes to nebulize.  Luckily when we're at home we can do this at the same time as the vest, so it won't actually add more time to our treatment routine--just two more medications to manage and two more nebulizer cups per day to clean. 

Lemon tolerated the treatment just fine, and as a reward for being an outstanding patient he received a new green truck from the clinic's prize bag.  Of course, he was so thrilled by the prize that he couldn't eat his dinner, but that's a separate issue. 

We'll begin integrating the new treatment into our routine tomorrow afternoon, once the medications are ready at our pharmacy.  That will give us one chance to run through it with two adults in the house, before Papa Bear leaves for a quick trip to New York on Wednesday to see his side of the family, whom we haven't seen in entirely too long.

What else?  Ah, there is Lime.  Wonderful, chubby, cheerful, patient Lime.  Full of smiles and laughter, figuring out his hands, resistant to sleeping anywhere that Mama is not, resistant to tasting cereal when nursing might be a possibility.  Fascinated by his older brother, by the cats, and by anything with tiny plastic beads inside that make a little rattling noise.  On the one hand I feel like I spend so many of my waking hours (of which there are very many) with him, and on the other hand I feel like I have so little time to just focus on him and drink in the joy of his babyhood with everything else that is going on.  I decided a while ago that I would like him to have his very own episode of the blog, where he gets to take center stage at least for one week.  Unfortunately, it can't be this week, and it probably can't be next week either. I hope for his sake and Lemon's that a quiet week comes quickly and we can all share in the bliss of a communal dose of babyhood, without the shadow of CF being quite as ominous as it is today.

  

Week 118: Hazy shade of winter

Our adopted home state looks a little different since last our last update. 

In our house, we scarcely need the visual reminder since we have a persistent audio reminder in the form of Lemon's cough, which is back with a vengeance.  His last dose of antibiotics was on Wednesday of last week.  The runny nose started up again almost immediately, followed as it inevitably is by the cough--the worst one he's ever had.  We went up to 3 sessions/day of the vest on Sunday, and this morning first thing I was on the phone with the clinic to come up with a plan.  We're back on oral antibiotics for another two weeks and reintroducing Zyrtec, and if he doesn't stay well after that, we're looking at the possibility of an admission.  We've stared down these odds before, but that was in March with spring on the horizon, not in November which only inevitably leads to December.  We're trying to remain optimistic, but it is decidedly harder at this time of year.

Aside from that, Mrs. Lincoln, it's been another normal-ish week at our house.  Papa Bear left for a conference early Thursday morning, and Opa and Nona headed off to Chicago on Friday, so I was solo with the kids for a few days.  It was actually fun in its own way, if you consider living in a sea of scattered toys and used tissues whilst going to bed at 8:30 and arising for the day several hours before dawn to be fun.  We shoveled the driveway, went out for ice cream, went to the library, and did our weekly excursion to buy groceries and see fire trucks.  The main thing is to completely and utterly surrender any hope that you (the adult) will actually accomplish a single thing for yourself.  If you set your expectations to zero, you are much less likely to be disappointed.  I managed a five minute phone call with an old friend and it felt like a major victory.  We were all very glad when Papa Bear returned around mid-day Sunday.  Lemon was especially delighted with the little souvenir that Papa had picked up for him in Orlando.

Looking ahead to this week, I am certainly glad that we set expectations to zero and made no plans whatsoever for Thanksgiving, so at least we won't have to cancel them.  We're hoping that Lemon's cough responds to the antibiotics quickly, otherwise I'll be calling the clinic again Wednesday morning to see if we can get in for an appointment before the holiday.  I suppose if we have to be admitted I'd rather it be from the clinic on Wednesday than from the ER over the holiday weekend. 

In the spirit of Thanksgiving, I have to say that I am so thankful to be fighting this battle now, in 2015.  I can listen to this cough and worry and fret, but I am worrying and fretting about his suffering, about adding new treatments, and the unknowns of a possible hospitalization, not that I might be in imminent danger of losing my child.  I am so thankful that we have a wonderful care team that I trust, and that their efforts are guided by the research that was made possible by everyone who contributed their time, effort and money before Lemon was born.  I am thankful to have the family that I have, and the support of all our friends near and far.  I hope all of you have a wonderful Thanksgiving, and please eat an extra slice of pie for Lemon--he could use the calories! 

Week 117: Red alert

This was our week of many appointments.  First, Lemon had his clinic visit on Tuesday.  To no one's surprise, he had gained barely 100g since his last visit, for a rate of 3g/day when our target is 5-10g/day.  Of course this isn't ideal, but his doctor didn't seem overly alarmed.  His feeling was that at least Lemon hadn't lost any weight, between his "experimental phases" and being sick last week.  At least the antibiotics had worked quickly, and his cough was basically gone. 

We are trying a few things to help with weight gain.  First, we increased the dose of ranitidine (an acid reducing medication) that Lemon is on.  He started on that to help raise his intestinal pH so that his enzymes could work better, and we haven't gone up on the dose in over a year. So, maybe we can gain a little ground just by re-optimizing his digestion.  At his next appointment, he's due for an annual chest X-ray, so we're adding on an abdominal X-ray as well, to see if there is any stool built up in his intestines.  Constipation is a very common problem in CF, and even "regular" CF patients sometimes have some build-up.  If we see that on the X-ray, then we'll start him on Mirilax to try and clear it out, which will hopefully increase his appetite by making him feel less full.  As you may imagine, the idea of giving a laxative to a two -year-old fills me with joyful anticipation, but whatever it takes, right?  Finally, we're scheduling an appointment with a specialist at the clinic who may be able to give us some new strategies to encourage Lemon to eat a bit more.  I still hold out hope that since what we have is essentially a behavioral problem, there will be a behavioral solution, and that solution might be more immediate than "wait until he's old enough that the behavior changes."

On Wednesday I took Lime to his 4 month checkup.  He is officially a little teapot, short and stout, being in the 2nd percentile for height but the 20th for weight.  He's growing right on his curve and has all the skills a 4 month old should have (including his first front-to-back rolls).  So, we got a few shots and went home.  Done.  See you in 2 months.  I'm really not used to this!

On Friday, around mid-morning, my phone rang and it was the CF clinic calling.  I thought that was a bit odd, since they don't usually call except automated appointment reminders, and we didn't have any upcoming appointments.  When I answered the phone, it was one of the nurses and I could tell by the tone of her voice that the message wasn't positive.  My mind immediately leaped to the throat culture that was taken on Tuesday.  Had they found something that they didn't want to find?  The nurse asked me how Lemon was feeling, and whether he seemed lethargic.  I said he was doing fine and was pretty much the opposite of lethargic.  She said that was good, because there'd been a mistake and Lemon had been prescribed twice the dose of the antibiotic that he should have received.  Since he was feeling well, the basic message was "no harm, no foul, go down to one dose a day instead of the two we told you to do and everything will be OK."  Deeply reassuring. 

Also deeply reassuring was the fact that this mistake was caught, not by the clinic, but by our insurance company who declined to pay for the prescription since the dose was out of range.  The pharmacy was not thrilled about not being paid, and they called the clinic.  It's sad that this mistake was only caught when there was money on the line, and sad that in spite of an electronic health record with a specific intervention plan detailed in it, that the doctor on call could have made this mistake.  I am quite sure that the electronic health record system used by our hospital popped up an alarm when this prescription was entered, and that the doctor clicked away the alarm without really looking at it because there are a million alarms a day, most of them not meaningful, that he has to get through in order to actually do his job.  So, added to the infinitely long list of tasks for the parents of a kid with a chronic illness is double-checking the dose on prescriptions, especially those done by someone other than our regular doctor.

Just to give Friday the 13th a little added kick, we got the news that Lemon's doctor will be leaving Madison come spring.  This is pretty devastating news for us.  We've seen him at the CF clinic every 6-8 weeks since we moved here, and I have so much respect for his knowledge and approach. We will probably switch to his senior colleague at the clinic, who is also a great doctor, so we will be in good hands.  Still, I wasn't really prepared for this change and am feeling some trepidation about it.  We cling to whatever is familiar and constant, especially when trying to navigate something as difficult CF, and Lemon's doctor has been a constant presence since we moved here.  Mainly I wonder how we will find the bandwidth to navigate one more new thing, but I suppose we will find it somewhere.

At least I did get one reassuring piece of news on Friday--I got my teeth cleaned and everything was fine.  Follow up in 6 months.

Week 116: Shake, rattle, and roll

As of a week ago, Lemon had developed a bit of a cough.  He was mostly coughing at night, although sometimes during the day as well.  We upped his vest treatments to 3 times per day to see if that would shake the cough out of him, as it has for the previous two coughs he's had this fall.  This one seems to have been made of sterner stuff, and he was still coughing on Friday.  He seemed more or less his usual chipper self, but the cough was there and it sounded "juicy," as our nutritionist at the CF clinic describes these things.  So, after some hemming and hawing, I called the clinic to request antibiotics.  I wasn't eager to do it, since antibiotics come with their own problems and every use could increase the chance of developing resistance.  But, with a cough lingering for 5 days, it was time. 



We started the antibiotics on Friday evening, and as of today the cough is mostly gone.  So, now after a full week of 90 min/day in the vest plus 3.5 days of antibiotics, we seem to be back on top in terms of pulmonary stuff.  Of course, we still have 10 days of antibiotics to go and they have taken their usual toll on Lemon's delicate digestive system, resulting in really yucky poop and an incredibly painful diaper rash.  It's also thrown off our usual system of giving all of Lemon's vitamins and supplements, because the antibiotic which has to be given twice a day with food can't be given in the same meal as his multivitamin, or his iron, or his probiotic, which we're supposed to increase to twice a day when he's on antibiotics.  While it may sound like not a big deal (and in some sense it isn't), it means that nothing is happening at the time of day when it's supposed to, which just increases the chances that we will miss some doses of various things.  It's not the end of the world, but it's not exactly ideal, either.

While all this was humming along in the background, I attended our CF center's annual Family Day on Saturday (video available here).  I brought Lime with me, and he was definitely the star of the show.  I do not exaggerate when I say that I had a line of people next to me offering to hold him for me.  He was remarkably well behaved and took a long nap during the keynote presentation by Dr. Michael Boyle, Senior Vice President of Therapeutics Development at the Cystic Fibrosis Foundation.  There's a lot of great stuff coming through the pipeline right now, from Vertex as well as other companies, and I continue to be very hopeful that before Lemon hits his teens there will be one or more good drugs available to help him. 

Beyond getting an update on the state of new therapies, the real highlight of the day was getting a chance to catch up with other CF parents.  As all parents know, having young children can be very isolating.  Add to that having a young child with a rare disease, and add to that having a young child with a rare disease who specifically can never be in the same room as other people with the same disease, and you have a recipe for feeling like you're alone in the world.  The instant camaraderie, even with CF parents that I'd never met before, was a huge source of comfort, and we're all hoping that we'll be able to set up some sort of recurring gathering more frequently than once per year. 



















This week and next are likely to be rodeos--tomorrow is Lemon's clinic visit, Wednesday is Lime's 4 month (!!!) check up, and Friday I'm going to the dentist.  All during business hours of course, so who knows exactly when I'll actually do my job.  And then at the end of next week, I'll be flying solo as Papa Bear heads to a conference and Opa and Nona take some well-deserved R&R in the windy city.  And the week after that is Thanksgiving.  Let's do this, November!

Week 115: Whack-a-mole

It seems like child care problems are cropping up as fast as we can solve them these days.  So, let's focus first on what went right this week.  Our new Tuesday/Thursday babysitter started with Lime.  That worked out really well.  She showed up on time, played with him until he was worn out, and read a book while he was asleep.  Lemon is also really interested in her, and we're hoping she'll come one more afternoon per week when both kids are around, so that Lemon can play with her too.  Lemon continues to love his preschool, and we got his first school pictures this week.

On the less positive side, our morning nanny had a little health crisis on Tuesday morning while she was here with the kids.  Some combination of still recovering from the pneumonia plus probably a bit of hyperglycemia from diabetes that became hard to control while she was fighting pneumonia left her pretty unwell, so she had to bail in the middle of the morning on Tuesday morning.  Fortunately she was able to rest on Tuesday afternoon and get herself back together by Wednesday, but it gave us all quite a scare.  Wednesday should have been a normal day, except that Nona had a migraine couldn't watch the kids that afternoon.  And Friday she was out of town for a wedding.  So, we effectively had about 70% coverage for the week, and were scrambling once again.  Somehow we made it through but we were pretty wiped out going into the weekend.

With all the upheaval during the week, we didn't quite manage a Halloween costume for anyone. 

Fortunately, our kids are too young to know that they have the lamest parents ever.  We went to our neighborhood Halloween parade anyhow, and luckily no one mocked us for showing up in street clothes.  Lemon wasn't quite sure what was going on, but he was intrigued by all the people in funny outfits.  We tried to walk in the parade, but Lemon made us pull over so he could watch it instead.  Then, at the end, he sat down at a picnic bench and ate two entire pieces of pizza as though it was no big deal, even though we've been struggling to get any solid food at all into him this week.  The late night (extended by a two hour refusal to go to sleep once we got home) certainly "eased" the transition with the clocks falling back.

Our sleep issues continued into Sunday, when Lemon refused to nap until we got in the car to go to the park, at which point he promptly passed out and slept for an hour in the parking lot.  Luckily I'd learned from past experience and had brought both Papa Bear and the stroller, so Lime and I took a nice walk around the neighborhood while Lemon and Papa napped in the car.  Once he woke up, Lemon had a great time at the playground.

Our food struggles have continued as well, with one notable exception--on Sunday morning in desperation I bought a small bottle of ranch dressing, thinking Lemon might like things better with "dip."  He fell in love with the bottle as  soon as I took it off the shelf at the store, carried it by hand for the rest of our shopping trip, wept bitterly when I pried it out of his hand to pay for it, and showed it proudly to the firemen that we met in the store parking lot on our way out.  He has decided that this marvelous substance should not be relegated to the role of "dip," but should have a starring role in the meal, and be consumed with implements reflective of its status as a main course.  To quote another CF mama on the subject, "You take what you can get."





Oh, and Lemon has another cough, so we're up to 3x/day vest treatments until further notice.  Someday I have to imagine we will get to the point of solving problems faster than they crop up, or possibly even have a multi-day stretch in which no new problems emerge.  I'll be sure to let you know when that happens!

Week 114: One more player

I was spared the frustration of a disappointing weigh-in this past week because our clinic appointment was cancelled.  I still don't exactly know who cancelled it; all I can say with certainty is that it wasn't me.  I realized on Thursday morning that I hadn't received the usual barrage of appointment reminder emails and phone calls, so I called the clinic to make sure I had the right date and time on my calendar.  The receptionist told me that the appointment had been cancelled, since we'd "been seen earlier."  I am still really irritated and frustrated by this, especially since our written clinic-provided notes from that earlier visit specified that the visit we'd already had on the books was the next step in our follow-up plan.

But, once an appointment is cancelled, it's gone, so I scheduled us for the next available in a few weeks time.  Honestly the news will be the same as it would have been on Friday--at the moment we're doing well on pulmonary (two cold/runny nose/cough things defeated without resorting to antibiotics) and not doing so well on nutrition (visible ribs and spine, throwing rice on the floor, etc).  Having never been the parent of a "normal" two year old, Lemon usually looks "normal" to me, but we went on a little outing to the zoo with some of his friends from school and seeing him next to his classmates really drove home to me how skinny he is at the moment.  We'll just keep buying heavy cream by the quart and hoping that eventually Lemon acknowledges that we aren't trying to poison him at every meal.

Another development this week was that we decided to add one more member to our care team.  Our nanny's bout with pneumonia a few weeks ago was a stark reminder of the fact that we are constantly one illness or travel conflict away from a child-care emergency.  So, we hired a very capable-seeming college senior to come watch Lime while Lemon is at school.  She'll also come a few other odd afternoons per month to hang out with Nona and both kids so she and Lemon can get acquainted, and then she can serve as our back-up/pinch hitter when another member of the team goes down.  When I describe our child-care arrangements to people, they sound ridiculously complex.  Honestly, even to me, they sound ridiculously complex (7:30-12:30 every day: our indispensible nanny.  12:30-4:30 M/W/F: Nona and Opa, T/R 12:30-4: Lemon to preschool, Lime at home with our new babysitter).  Even with all these machinations, I have 43 hours per week of child care to work 40 billable hours for my job.  I can just make it if I work absolutely flat out all the time that Lime isn't nursing, and put in a couple of night or weekend hours on top.  So far, it's been worth it--I'm really enjoying my job and my new coworkers, but the schedule is a bit intense with every single minute accounted for.

Fall is in full swing out there in the sunlit world above my office.  We've been trying to take advantage of the last few warm days by spending as much time outside as we can.  I'm really sad that the farmer's market on our street ends on Saturday.  It's become an integral part of our family's routine.  In fact, Lime has never known a single Saturday in his life aside from the day after his birth that we didn't go.   We've been having a lot of fun raking leaves in our yard, and collecting interesting specimens to look at in more detail.

We also made the bold move of booking some winter travel.  I've learned through hard experience not to get excited about a trip out of Wisconsin until I've actually arrived at the destination, but we at least now own plane tickets to Boston for the week between Christmas and New Years.  So, Boston/New England friends, please stick around for the holidays so that we can see you.  Lime can't wait to meet you!