A Look Into Our Daily Routine

The CF team is one part of the core care facilitators for CF patients.  The patients themselves and their parents/caregivers or spouse/significant others are the other part of that core.  Compliance is huge for patients to be able to stay as healthy as they possibly can, to try to ward off as many bigger complications for as long as they can, and to try to live a long, full life.

Compliance can be challenging at all stages of a CF patient’s life.  One benefit I recently discovered is being able to get a glimpse into potential future battles we may have with Judith; I’m part of a Facebook group for CF parents, and the children’s ages vary greatly.  I’ll often read through posts about the challenges in dealing with teens, and file the tips away in the back of my mind in case we have the same problems in the future.  Of course there are challenges with kids Judith’s age as well, and believe me there are days when I want to pull my hair out trying to keep her compliant when she is trying her hardest to get out of doing a treatment!

One thing that helps keep us in compliance is sticking with a solid routine.  Our daily routine is strict, but also allows for wiggle room depending on what’s going on that day.  A general day for us looks like this:

Wake up, have a can of Pediasure in a straw cup, mixed with the day’s dose of Miralax
Random “get ready for the day” stuff that everyone does
Have breakfast with another can of Pediasure; take vitamin
Play time for about 60-90 minutes
Vest, Albuterol
Snack and more play time or other activities
Lunch with another can of Pediasure
Nap
Yet another can of Pediasure, then about 30 minutes of play time
Vest, Albuterol
Dinner
Bath, bedtime routine, bed

Most of that is nothing out of the ordinary for a preschooler, and in fact the majority of Judith’s day consists of very typical activities.  The differences are in the treatments.  We’re fortunate because she is not on as many medicines as a lot of other CF patients, so it significantly cuts back on treatment times.  However, The Vest alone takes a full hour out of each and every day, and that’s when she isn’t sick.  Sometimes it can be hard and even annoying trying to plan appointments and activities around her treatment times and accounting for them, but it’s something you get used to.  Now, when Judith’s on “sick plan,” things are even trickier.  Treatments will at least double, which means she’s spending a good 2 hours every day on her Vest, and taking puffs of her Albuterol every 4 hours (again, though, the time is significantly less than it is for some patients because we use the inhaler with her, and don’t nebulize it).  Antibiotics are also added, which can often be a good 15 minute battle depending on what form they come in.  If she has a pseudomonas infection, TOBI would add an additional 40 minutes (approximately) to the plan.  I also know that once we add hypertonic saline and pulmozyme to her daily meds, treatment times are going to go up even more.

It’s a lot, and it’s overwhelming, especially if she’s sick.  We’re hoping that by fostering good compliance strategies now, it will help with the compliance battles we may end up facing as she gets older.

Switching Up Routines

It's always hard for us when we have a major change to our routine, especially a routine that has been in place for a year!  It was our first day without physical therapy services, and I'll be honest: I was nervous about how Judith would react, and I wasn't sure what to do with myself since I was missing out on one of the rare opportunities for face-to-face adult interaction that I get in any given week (pathetic, I know, but such is the way of life during RSV season, especially when you don't have Synagis to back you up).  By 2:30, I was trying to figure out why it felt like the day was dragging, and I remembered it was because we wouldn't have PT today.  To celebrate, Judith decided to take a nap during the time when she was so accustomed to working hard.

We're almost a week into the breathing treatments, and we've had a good stretch of cooperation!  It helps that I discovered the goodness of Mickey Mouse Clubhouse on Disney Jr. every morning - that show is like baby/toddler/preschooler crack, and Judith is hooked after 2 episodes.  As far as kids' shows go, this one is pretty good and doesn't drive me nuts like some of them (::cough cough Barney cough cough::).  It gets us through the majority of the treatment, and I'll take any special distraction we can!  She's also adjusting nicely to the mask.  I know that for many kids, the mask is a huge issue.  However, the more she's using it, the more she's adjusting to it.  I'm always amazed at how quickly she adapts to things, and I attribute a lot of that to everything she's been through in her short life thus far.  Plus it helps that she's used to using her spacer twice a day, every day - that has a mask on it, and Judith knows our key words for her treatment, so I can turn those around and use them during neb treatments!

Granted, I'm typing all of this and painting a glorious picture of this calm, cooperative toddler, and that's not exactly the case.  She has her moments, and today she was extra wiggly during treatment time.  Even so, I'm getting the bulk of the TOBI into her lungs, and that's the important thing.

I feel bad for Buster & Lady.  They're not terrified of the nebulizer, but they certainly don't like it.  The machine is loud, there's vapors in the air, and Judith looks like a little dragon breathing the mist through the mask.  For some odd reason, cords, tubes, and wires freak Buster out, and he's paranoid to jump up or go near an area that he perceives as "obstructed" (realistically, he can easily clear said cord, tube, or wire, but chooses not to unless we coax him... a lot).  Lady has taken to plastering herself against me while I give Judith her treatment - I love the dog, but it's mildly annoying when she does this and sinks into the crevice between cushions on the couch, making it hard for me to move if I need to readjust to Judith's squirming.  I know this is an adjustment for them too, and I'm hoping they adapt, otherwise I'm going to hate to see how they react once Judith can get The Vest.

Breathing Treatments and EI

No guarantees that I can keep this post short - sorry!  I'll break it into 2 parts though, because a lot has happened in the past couple of days!


Breathing Treatments
I gave Judith her first breathing treatment Wednesday night.  That was an epic fail: Judith screamed through the entire treatment, and it was the type of fit she normally reserves for the doctors' offices.  And since she had herself so worked up, she puked all over the place once the treatment was done.  So not only did I have to worry about remembering what I needed to clean on the nebulizer, I had to clean up the vomit in the PnP and the Step & Play piano, plus change her and supervise her to make sure she didn't get into the dog water bowls.  Fun times.

Thursday morning started the same way: she was refusing her treatment, was fighting me, and threw the mask with medicine cup attached on the floor... twice.  Finally I had her sit on the couch (I was right next to her with a hand on her in case she decided to bolt) and tried holding the mask at her face instead of trying to strap it to her head.  Throw in a little singing, and she was able to complete the rest of the treatment.  I felt much more confident about things after seeing what would get her to respond, and I filed it in my mind for the next daytime treatment.

Thursday night, she slept through the entire treatment, which was my goal (it was my goal for Wednesday too, but she decided to wake up).  Score!

This morning, she did well!  Yes, she still shoved the mask away and wanted to play with it more, but we got the bulk of the treatment into her lungs since she allowed me to hold the mask against her face for longer periods.

Now let's hope that she continues to do that well for the remainder of the treatments!



Early Intervention
Judith's annual eval was yesterday afternoon, and I am very pleased with the results!  Judith actually performed the majority of the tasks they were looking for, save for a couple things!

An hour and a half later, the paperwork was signed and we had the results.  I can't remember what all of the specific numbers were (we should get the report in the mail soon), but I do remember that she's at a 16 month level in gross and fine motor, which means we're discharged from PT services!  She's somewhere around 16-17 months for cognition, but is lacking in communication and speech - she scored around 11 months in those areas.  As a result, she qualifies for speech services, and we will get those moving within a few weeks once we set up a time with the therapist and whatnot.

If you are unfamiliar with how EI performs these evaluations, they score the baby/toddler based off of their actual/chronological age, so for a preemie, it often looks like there's a bigger delay than what there really is.  That's the case with Judith's speech and communication: if we adjust her age, she's actually within the normal range and would not qualify for services.  But because they base it off her actual age, she qualifies.  I'll do anything to help her get caught up to where she needs to be, and if speech services will be the final boost, so be it!

I was just thrilled to hear how well she's doing in so many areas, and to hear that she's almost completely caught up!  At this rate, she will be caught up by age 2, and that makes me happy to know how far she's come in such a short time!

New Nebulizer & TOBI

Judith's nebulizer came earlier this evening - a relief for me, because I feel better starting her treatments ASAP!  I honestly didn't expect the semi-fiasco we went through to get the thing.  A bunch of phone calls and 3 medical supply companies later, we were finally set with the proper nebulizer and all of the supplies we needed.  I picked up the TOBI earlier in the day, and it's been sitting in the fridge ready to go.

The insurance companies did end up buying the neb straight out of the gate, instead of doing a rent-to-own type of deal.  To me, that's the most logical move for Judith since it's likely we'll be using it more often in the future and whatnot.  For once, the insurance company agreed and thought logically ha ha!

Her mask is super cute - it's a fish, and I think she'll like to look at it!  I'm hoping that she'll adjust to it quickly.  She generally does well with her inhaler and spacer, so we'll see how this goes.  Tonight should be easy though: I'm definitely waiting until she's conked out for the night before giving her the first treatment!  Tomorrow, on the other hand, should prove to be interesting.

I would like to take a moment to state how grateful I am that John's work provides the option of a good insurance plan, and that the United States government is, at the moment, providing funding for Medicaid.  I can still remember talking with the social worker at our second appointment after we got Judith's diagnosis: she said that no matter how great your primary insurance coverage is or even how much money you make, it's important to get your child with CF on Medicaid to help cover the cost of treatments and medicines.  That has stuck with me for the last year, but today is when reality really sank in.

The nurse coordinator did mention over the phone that the TOBI would cost a couple thousand dollars, and double checked that we have Judith on Medicaid.  I said yes, that we applied after we initially got the info and have had coverage for almost a year now.  So I had an idea that the cost of this drug would be high, but we shouldn't have a copay (or much of one if we did have to pay).  On the drive to CVS, I reminded myself to check out the original cost of the meds, something I actually do for most of the meds we get for her because I'm nosy like that.  I went back to the counter, and the pharmacist handed me the bag: a big, brown, paper bag instead of one of the normal small, white bags - I knew we'd be getting a lot of medicine, but I didn't quite expect the pack to be quite that large.  Anyway, I made my purchase, and on the walk out of the door to the car, I looked at the "before insurance" price on the patient info card:

$5,274.52

That's right, folks: that's a comma and 4 digits between the dollar sign and the decimal point!  Your eyes are not deceiving you!  And that's only for a 28 day supply of 56 vials (since she gets it twice a day).

This is why the social worker said CF patients need the extra coverage.

Now I'm waiting for the statement to come from our primary insurance showing the breakdown of the costs for the nebulizer.

Aw Crap

It's just one thing after another in our family right now.

This morning the nurse coordinator from the CF Clinic called me.  When the number popped up on my caller ID on my cell, I knew it was from Hershey, but I thought it was the nutritionist checking in per our conversation at our last visit.  Instead, I was getting a call about Judith's latest throat cultures.  Judith cultured positive for pseudomonas this time.

Oh shit.

I know this is a common occurrence in CF patients, but it's not really something you want to hear that a culture came back positive for.  They have the potential to cause a lot of problems, and they can be hard to treat.  But there are meds that can treat it, and how long she will need to be on them depends on how well she responds to them.

We did have the opportunity to participate in a study with Judith (provided she would actually qualify).  I'm all for participation in studies - heck, Judith is the result of a PCOS study!  However, right now, I decided to opt out and wait until she's a little older before enrolling her into studies.  Considering she's only about 13.5 months adjusted and we have the preemie issues we're still dealing with, I felt it was better to go with a drug that we know will work and has already been FDA approved.

2 scripts later and a few phone calls from the clinic to the pharmacy and medical supplier, we are set to get a nebulizer for Judith and a 28 day supply of TOBI (Tobramycin Inhalation Solution, USP) for round 1 of treatments.  CVS was able to order the meds, and I can get them tomorrow.  Her nebulizer should be delivered tomorrow as well, provided the insurance company approves it quickly.  After the 28 day cycle, she'll need to have another throat culture - if she tests negative, we can stop treatments, but if she tests positive we'll have to do another 28 day cycle and will keep repeating the process until she cultures negative.  Hopefully she'll culture negative in a cycle or 2.

Honestly, I've been bracing myself for the possibility of a nebulizer for a while now.  I know that we've been extremely lucky to have avoided it thus far: Judith's 16 months old, and between the preemie issues and the CF it's pretty damned amazing that she hasn't needed something like this sooner.

I can't wait to see what the total cost for the neb and the TOBI are going to be.

Can't You Do Something By The Book? Just Once?

I'm frustrated.  Really, really frustrated.  There are many times when I'm glad that Judith's different, and her feisty & stubborn personality is behind her doing things differently than the norm.  This is not one of those times.

Remember my post from Monday about Judith's test results?  Well, you can color me completely baffled right now.  I don't deny the results - they did 2 tests on that 1 sample, and everything's normal.  We know that.  But then why did the enzymes make such a drastic difference?  And why, if things are normal, have things reverted back to the way they were pre-enzymes?  We're on our 3rd poo today, it's been a production each time to get it out, the color is not normal, it's getting bulky, and the last one looked a little greasy again (John changed the second diaper, so I'm not sure how that one really looked).  Some of the changes this week, specifically Wednesday, I thought were a combination of being off the enzymes and teething.  Today, that's been blown out of the window.

Naturally, Judith waits until office hours are over at the clinic and the weekend starts.  Lovely.  They have a nurse line for evenings and weekends, but I don't want to call them about something that's not really an emergency.  My anxious self wants to be able to discuss this today and see what needs to be done, but the rational part of myself is saying there probably isn't a lot that they can do for her, other than run more test (on what, I don't know) to try to pinpoint the problem.

I need one of those signs with a circle on it that says "Bang head here."

Antibiotics are Done!

Fingers crossed that we can avoid respiratory infections for a while.  So far, so good - we finished the Cipro for Judith's sinus infection this morning, and the congestion and cough are gone.  We dropped her CPT and breathing treatments back to the normal schedule, and her appetite seems better.  Knock on wood, it looks like we avoided some of the antibiotic-related diarrhea this time as well.

There's still a long way to go before we get to the end of cold/flu/RSV season, but I'll take as many healthy days right now as we can get!

CF Clinic

I have a feeling this will get buried among Judith's birth story posts, but for those of you who are interested, I have an update from today's clinic appointment!

It was our first appointment with Judith's new pulmonologist, and I had good vibes about him from the beginning!  He came highly recommended to me from a friend, and I'm so glad we made the switch!  He was able to answer my questions as best as he could, and I finally feel like we are getting somewhere!

First things first, and I know this is what most people are looking for: Judith now weighs 16 lbs 15 oz, and is 27" long.  She gained about a half a pound in a month, and is back on the charts! She's in the 3% for both length and weight!  We'll find out what her head circumference is when we go for her 1 year well baby.

I didn't get to speak with the nutritionist today (she had to leave early), so I don't know when we'll have the green light to transition to whole milk and wean off of the Neosure.  I do know that we will have to supplement, and they want to get that going now so we can keep her caloric intake up.

Her chest x-ray from October looks good, they're still culturing/monitoring the bacteria that they know is present (it's not growing, and they don't want to treat it because studies done on treatment of this type of bacteria show that antibiotics don't really help, and they don't want to risk it becoming resistant), and we can try weaning her off of the Zantac.  Judith's doc isn't totally sure that she's outgrown the reflux since she still shows signs/symptoms of it, but he did say that if weaning off the Zantac doesn't work he'll switch her to something else to try to control it.

I got some better answers about her genetic screen too.  Right now, it would be pointless to repeat the test to try to find the 2nd mutation - we'd get the same results, so we'll have to wait until they expand the panel of mutations before we can retest.  Judith definitely does not have a nonsense mutation, and the lab panel showed that some of the proteins are functioning, hence why she is still pancreatic sufficient.  We have about 2 more years to go before we can feel more confident that she'll remain that way - pancreatic insufficiency usually turns up sometime in the first 3 years of life.  If she does remain sufficient, she will probably have a milder form of the disease, and we'll be able to concentrate on treating the lungs and respiratory system.  I'm keeping my fingers crossed that her pancreas continues to function like it should!

The only bad part of the appointment was Judith's cough.  When they put her on the antibiotics almost 2 weeks ago, her cough went away, but started to come back this past Sunday.  Her doc is putting her on another antibiotic and also prescribed steroid treatment to help her lungs and to try to get rid of the cough.  I am so grateful that we started the Synagis when we did - her doc said they haven't seen too many cases here yet, but the season is closing in fast, and the protection will help her.  I would be extra nervous about this cough if we hadn't started the Synagis earlier in the month.

I feel so much relief after this appointment!  We'll get through the antibiotics - it's possible we could be dealing with all sorts of stuff the whole winter, so all we can do is our best to keep her as healthy as possible, and treat whatever may come up.

Cold and Flu Season is Here...

... and we may be fighting off the first cold.  I noticed that Judith has been coughing more toward the end of last week, and after the big weather change Thursday into Friday, it sounded wet.  I called the clinic first thing, and they put her on an antibiotic immediately to help prevent anything else from taking over (they like to treat all CF patients aggressively to ward off any potential problems as soon as they can).  Poor peanut hasn't been herself - she fell asleep on her back twice on Friday, and didn't roll over to settle in like she normally does.  Her appetite has gone to pot, and she's not taking in as much as she was before.  Combined with some major teething, she's turning into a hot mess - not that I blame her.  :(

The first thing I thought of while making the call to Hershey was "Thank God we got her first Synagis shot into her a week and a half prior!"  Any exposure to RSV, and the vaccine will help prevent it from growing further, but I don't think it is RSV since she hasn't had any other symptoms so far.

Anyway, her pulmonologist put her on a Sulfa antibiotic to help with the cough.  Cue a small panic on my part - I'm allergic to all sulfas, so now I have to watch her closely to make sure she doesn't develop a rash.  So far, so good; my allergies didn't turn up until I took it 2 or 3 times, and since this is her first time (I'm assuming - I can't remember if she had any sulfas when she was in the NICU) I'm hoping we'll be good to go.  Trying to get her to take it is a completely different story.  We have a thicker oral injector the pharmacy gave us since she's getting a teaspoon twice a day.  It's a slow process, and we can only give her about 1mL at a time or else she won't take any of it, and even then she still spits some of it out.  Apparently medicinal grape flavor doesn't fool her.  Naturally, she manages to get her hands involved, smearing some of the antibiotic on them, and attempting to stick them in my mouth.  I'm probably overreacting, but I really don't want to run the risk of ingesting some of it and ending up with a bad rash for Thanksgiving.  If I didn't know better, I'd say she's trying to get revenge on me for forcing her to take the stuff.  Only 8 days more of this...

As for the teething front, I am happy to announce that there are some definite bubbles on Judith's gums!  I managed to peek inside her mouth yesterday, and there are some prime spots ready to come through soon, mostly on the upper left side of her mouth.  Oddly enough, it looks like she's cutting her upper canines first, but the other front teeth don't look like they're far behind.  I can see them lined up under the gums, and Judith is gnawing on anything and everything she can get her hands on.  I still hold with the stance that we're lucky in the teething department - so many moms that I talk to seem to have it much worse with their little ones, and I count my blessings that she seems to handle it relatively well (even though it still has to hurt a ton).


Yesterday was super exciting!  I got to meet another one of the wonderful ladies I talk to online, and she took Judith's 1 year photos for us!  She is super talented, and such a nice person to be around - I really hope that we can get together again sometime soon!  Once we get the link for the proofs, I'll post 1 or 2 so you can see the great job she does!

I know this is long already, but I have to share a funny story about the doggies (and frankly, I don't post about them much and feel guilty about that sometimes haha).  John had his laptop at my parents' house, and when we got back from pictures he was working on some church stuff.  He left the laptop running downstairs, and came up briefly to look at a present idea for Judith.  Little peanut needed a diaper change, so I had to run downstairs to get the wipes.  Buster felt the need to follow me down, and next thing I know he's barking at something.  Buster doesn't bark often (not like Lady, who will bark at a speck of dust floating by outside because it dared stray onto her turf), but when he does, it's usually because he doesn't like something (like a cat, another dog, or a rabbit).  I was upstairs, and his barking continued.  My mom asked who was barking, and I said it was Buster!  My grandmother couldn't figure out what he was barking at either; after a few more minutes of this, John finally called him and he ran upstairs.  A couple minutes later, he followed John downstairs, and began barking again.  This time we figured out what the issue was: Buster apparently didn't like the floating balls screen saver on John's laptop, and was barking at it!  He did this once before with a makeup model on one of my magazines, and it was just as funny then as it was yesterday!  He's so weird, but so entertaining at the same time!

CF Treatments

I'd like to do another educational post of sorts, and tell you about the treatments Judith needs to have daily for her CF.

Twice a day, at her 6 AM & 6 PM feeds, Judith receives 2 puffs of Albuterol from an inhaler.  This is to open up her airways so she can breathe easier.  So far, we have not had to use a nebulizer (knock on wood), but I know that there's always the possibility that she will need one in the future.  Judith doesn't really mind the inhaled meds, and will often talk to us through her spacer.

The fun part (and I use the word "fun" loosely) is her twice daily CPT treatments.  CPT stands for Chest Physiotherapy (some people will refer to it as Chest PT), and this is the act of moving the mucous from the small airways to the large airways so Judith can cough it up.  It also prevents the mucous from sitting in the lungs and festering, which would cause lung-damaging infections.  This is not a treatment that will go away over time; in fact, this is a treatment that must be done daily no matter what, and will have to increase in frequency whenever she's sick.

This therapy may be something you're familiar with, or may be something you think of when you hear "cystic fibrosis": it's the treatment where someone claps on the lungs with a cupped hand or a special percussor (kind of looks like an air hockey paddle, but with a concave middle on the underside).  It can be done manually, or it can be done through a special vest.  We have to do it manually for now, because Judith isn't nearly big enough for the vest!

So every day, twice a day, Judith and I go through her CPT.  There are 3 "zones" (that's what I like to think of them as) that get the treatment, front and back: the upper chest, the middle chest, and the lower chest.  In each of these "zones," there are 2 areas that need to be percussed: 1 on the left, and 1 on the right.  Judith has to lay or sit a certain way while each area is being treated.  Keeping her in the proper position is the trickiest part right now - she's way more interested in rolling around!  I'm waiting to see what's going to happen when she's mobile and can crawl away.  The whole process is going to remain interesting until she's old enough to understand things!

I know this wasn't the most interesting thing I've posted, so if you managed to make it through this you deserve a cupcake!