Kicking CF's Butt

Things are finally (almost!) sorted out with insurances, and we received our Pulmozyme yesterday! Our medicaid provider is requiring a preauthorization for coverage of the prescription, which Dr. G. warned us might happen, but I was kind of expecting our primary provider to be the ones to push back. So instead of waiting an additional 7-10 days for that to go through, and then however many additional days until Walgreens could ship the meds to us, I decided to just pay the regular copay for this month, and get started.

We have 2 doses in, and so far, so good! Judith's biggest complaint is that it "smells funny" and that she doesn't "like the way it smells." Keep in mind that this is Judith's 2nd experience with nebulized medications, and she wouldn't remember doing TOBI because she was too young. Since she takes her albuterol via inhaler and spacer, the smell is slightly different. Honestly, though, I'm not sure if she's really smelling the pulmozyme, because I caught some of the mist coming out of the mask and it didn't smell funky to me, or if she's actually smelling the plastic from the mask and other parts. Regardless, she's not physically fighting me to try and not do it; verbally, through many protests, yes, but that's so typically Judith.

I'm eagerly waiting to see the difference this drug is going to make for her! After the exacerbations last cold/flu season, I'm really hoping that this will help her clear the crud out of her lungs easier.

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

Reculture and Miscellaneous Musings

Our days have been fairly busy lately, especially now that Judith is walking all over the place and getting into everything she possibly can.  She seriously wears me out big time - at least I'm getting a decent amount of exercise chasing after her!

Thursday and Friday of last week we enjoyed some warmer weather.  Friday's temperatures were high enough that I was able to break out one of the adorable smocked sun suits I purchased for Judith a couple months ago!  I decided to go with the Preppy Alligator pattern from Shrimp & Grits Kids:

 Removing all of her animals from the chair

Check out that ruffle butt!

Adorable detailing, and a bonus side-eye (she's getting rather good at that...)

On Saturday I packed Judith and the dogs into the car and drove to my parents' for a little visit!  The dogs were excited to go to their house again, but were apprehensive at first - they weren't sure about what was going on with my dad, and they finally warmed up and realized things are ok after an hour or 2.  I went with my mom to the salon to get haircuts, and Judith had a great time trying to de-shelve the brightly colored bottles of products and playing in the hair that was cut off of my mom's head and my head.

Sunday afternoon we caught a break.  John played in our local AGO chapter's member's recital, and got a ton of compliments on one of his arrangements!  Thanks to these people complimenting him, he got an extra dose of motivation to start contacting publishers to get some of his stuff out there (I've been encouraging him to do this for years, but I guess this was the push he needed to really take it seriously).  Since Judith decided naps are for quitters (that made for an interesting worship service), I stayed home with her so she could sleep and I could relax.

We'll skip over Monday since it was a boring day with minimal action.  Fast forward to today: I took Judith to Hershey for her reculture of her throat/sputum.  While we still have 3 more doses of TOBI to give her, I'm keeping my fingers crossed that the meds worked and the pseudomonas are gone.  We probably won't get the results until Monday.

I was alone with her for this appointment, and thankfully she didn't have an epic meltdown - she was by no means happy about having her throat swabbed, and melted down a little bit, but it didn't last as long as normal.  She even reached out to the nurse for her to hold her!  Of course after the nurse swabbed her, Judith gave her best "boo boo" face and cried, but when I reminded her that she wouldn't see Dr. Graff at this appointment she calmed down.

Speech therapy went well today!  Judith babbled more for her therapist, and we got a charge out of her carrying around a canister lid and laughing hysterically at Lady, who was closely trailing her - Lady naturally saw a disc shaped object and assumed it was a frisbee for her, and wanted to play with it but Judith wasn't giving it up!  I knew she was getting tired on top of it, because she was extra silly, but I was also impressed at how focused she was when playing with Lady!  Naturally Lady had a blast chasing after Judith, even though she didn't understand why Judith wouldn't just throw the lid for her.

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!