Quarterly Clinic Visit

Judith's big visit was last Tuesday, and it's the one where we do all of our annual labs: blood draws, chest x-ray, etc., along with the typical quarterly stuff like cultures. I was pleased at how well she handled the visit, particularly since she had a busy day at school in the morning! A quick nap in the car while we were traveling to clinic probably helped her mood, as did a good lunch at the "local" deli chain that we love.

I was glad to see that Judith finally gained some weight; she gained about a pound and a half, which is good because she didn't gain between January and the end of April. She's still nowhere close to where they want her to be since her BMI is around 19%, but it's a start. Part of the reason that this makes me nervous, despite her gains this quarter, is that when she gets sick again, she's not going to have a lot of reserves to fall back on. There are other factors at play besides the CF that I'm sure aren't helping the situation: her ADHD, and her inheriting John's freakishly high metabolism (which I would love to be able to bottle, because I'd make a really, really nice profit selling it!).

She gained a few fractions of an inch in height, and is up to about 40.8". So no huge growth spurt... yet!

I'm waiting for her final culture results to come back, and they should be in by tomorrow. We're going to do a stool study again, because her patterns are really whacked out even with the miralax, so we want to make sure her pancreas is still functioning properly or see if she needs to start taking some enzymes.

We are starting her on some nebulized meds, which I am actually very pleased to start. We decided to start with pulmozyme first, partly because hypertonic saline is super irritating (which I knew for a long time), but also because she's not used to nebulizing meds on a daily basis, so this will be an adjustment. Her albuterol is taken via inhaler and spacer, and we only used our neb once for a 28 day cycle of TOBI when she was about 16 months old. Pulmozyme is done once a day, and from what I understand it's a quicker neb, so it's a perfect way to get her started on the nebulizing routine. Since pulmozyme is a mucolytic, it will help thin and break down the super thick mucus in her lungs, and help her cough it up easier. By starting this, and starting it now, we're hoping that this will help cut down on the number of exacerbations she has this coming cold and flu season, and I'm hoping that this will help clear things out enough that, combined with the albuterol to open the airways, we can avoid steroids... or at least oral steroids!

And this brings me to the next finding from our annual visit: Judith's x-ray results. Up until now, every report has indicated that things looked perfectly normal; no signs of damage or mucus buildups. While this isn't totally unexpected, and I was already figuring something was going to start appearing after the number of exacerbations this past winter, Judith is now showing signs of possible damage, and is showing an increase in mucus that's building up and blocking the airways. The mucus buildup makes sense, because she's had a bit of a cough that appears a few times a day, and has been around for at least a month. We're still trying to figure out if some of it could be allergy related. Anyway, it's a bummer that we're now seeing signs of damage, and I was hoping we could go longer without that reality setting in. At the same time, I realize we're extremely lucky that we've gone this long without any signs of damage. So now that it's here, our goal for Judith has to be tweaked a bit; instead of working to maintain no damage, we're now trying to keep it from progressing if at all possible, because the less damage she has once the corrector drugs are available, the better.

So overall, results from the visit really aren't that bad! We have some routine changes to make, once CVS gets the pulmozyme to us, and our days are going to get slightly crazier, but we'll adjust quickly.

TOBI and Cayston

Both TOBI and Cayston are inhaled antibiotics that are used to treat CF patients who are infected with pseudomonas aeruginosa.  Cayston is the newer antibiotic that hit the market back in 2010, and even more recently there was a new form of TOBI released that comes in a podhaler which older CF patients can use instead of nebulizing TOBI.

Some patients who are colonized with pseudomonas or have recurring infections might alternate between TOBI and Cayston to try to eradicate the infections, or may do a plan with just TOBI that could be 1 month on TOBI and 1 month off TOBI, repeating it as necessary.  Like so many other aspects of this disease, that will vary on the individual and the course of action their CF team recommends.

We experienced our first 28 day cycle of TOBI when Judith was 16 months old and she had her first positive pseudomonas culture.  The nebulized treatment took us about 20-25 minutes to complete, twice a day.  You need to do treatments with inhaled antibiotics after airway clearance therapies, because you don’t want them to be coughed out of the lungs.  The whole point is to get them to stay in there and fight the bacteria, so it is crucial to do this as one of the last parts of your treatment time block.


Sources: http://www.cff.org/treatments/Therapies/Respiratory/TOBI/#
        http://www.cff.org/treatments/Therapies/Respiratory/Cayston/

Mucolytics

Mucolytics like Pulmozyme are used by CF patients to thin the mucus in the lungs, and make it easier to cough it out (cff.org).  The CFF website says:
    Pulmozyme acts like “scissors” in the mucus. It cuts up the DNA strands outside the cell that can make CF mucus thick and sticky. This DNA is from the white blood cells that work to fight lung infections. Even if a person with CF has not had a lung infection, the white blood cell DNA is still in the mucus (cff.org).

Like hypertonic saline and albuterol, this is another nebulized medicine that I often hear other CF parents talk about.  Judith is not on it yet (nor is she on hypertonic saline), so our experience with both this and hypertonic saline is limited.


Source: http://www.cff.org/treatments/Therapies/Respiratory/Pulmozyme/#What_does_Pulmozyme_do?

Hypertonic Saline

Many CF patients inhale hypertonic saline during their airway clearance time.  The purpose of the hypertonic saline is to draw more water into the airways to make it easier to cough out the mucus (cff.org).


Source: http://www.cff.org/treatments/Therapies/Respiratory/HypertonicSaline/

Bronchodilators

Bronchodilators, like Albuterol, are used by CF patients to help open the airways.  Many patients receive the medicines through their nebulizers, but some may use inhalers instead.  The theory is that by opening the airways, it could help the patient cough up the thick, sticky mucus that needs to be cleared out of the lungs (ncbi.nlm.nih.gov).


Source: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1305882/pdf/10911817.pdf

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.