Monday, August 4, 2014

Cystic Fibrosis Management for a 4 Year Old

Hi friends! I recently did a Guest Post on Blissful and Domestic about "A Day in the Life of Her", basically detailing a typical day in my life.  If you're interested to read it you can find it HERE.

In other news, we just recently completed a CF Clinic so I thought I'd do my annual "CF Maintenance" post.  If you want to see past years you can find them here (Year 1/ Year 2/ Year 3).  This actually should be a pretty easy post as Charlie is on the least amount of medication at the moment in recent memory! Pretty awesome!

Charlie's Current Cystic Fibrosis Medications and Treatments
Gene Type: Delta F508 (∆F508) homozygous
Age: 3 years and 11 months
Weight: 41.8lbs (95th percentile, up 4lbs from a year ago)
Height: 42.5 inches (95th percentile, up 2.5 inches from a year ago)
Weight to Length Ratio: 77th percentile!
Bacteria Cultured: staph aureus, pseudomonas aeruginosa, Stenotrophomonas maltophilium

Breathing Treatments (2x per day)

3 puffs Albuterol
2 puffs Flovent
1 vial of Pulmozyme, nebulized, mornings only
20 minutes Vest physiotherapy


Periactin (appetite stimulant, 3 weeks on/ 1 week off)
Pepcid (reflux)
Vitamin D
Azithromycin (antibiotic, MWF)
Creon 1200 (digestive enzymes)

Um. That's it. WHOA.  This is totally crazy, just 4 months ago we had TOBI, Pulmicort (we now use the Flovent inhaler instead, so it's 2 less nebulizers per day), steroids, and IV antibiotics in addition to everything listed above.  Now we barely have to do anything, it's totally awesome.  We have so much more time and way fewer nebulizer pieces to wash.

Charlie is still incredibly picky and isn't very inclined to eat much so thank god for Pediasure 1.5, which he has twice per day (and even more so, thank god insurance pays for it).  Somehow he's still managing to eat enough that he's not losing weight although I have no idea how lol.  He hasn't gained much weight in the past 6 months but he's just such a big kid that it hasn't made much of a difference with his BMI.  He's just so big, I can always pick him out of his preschool class or camp group because his head pops above all the other kids' :)

Charlie has had negative cultures for over a year with no signs of Pseudomonas so they have taken us off TOBI and moved us back into the CF1 clinic (no MRSA/no pseudo/etc group of CF patients).  I'm completely shocked that he actually cultured negative for that long but quite pleasantly surprised!  Of course now that we've gotten the pseudo and staph eradicated he's testing positive for a new bug - Stenotrophomonas maltophilium.  This is apparently not uncommon for CF patients to culture and it hasn't been shown to cause a decline in lung function, etc so our Dr reccomended we leave it untreated at the moment.  If he shows signs of coughing or increasing infection we will start him on antibiotics but for now we'll just let it hang out.

Charlie is very compliant with his breathing treatments.  His vest has logged 511 hours of physiotherapy on it since it arrived August 2012.  That equals out to 2.2 treatments per day for the last 2 years!  It's more than 2 per day because when he's sick his treatments go up to 4X per day.

Notable changes in his CF for the past year

* One close call for hospitalization that was fixed with levaquine
* One close call for the ER due to an intestinal blockage
* Our first hospitalization
* A whole year Pseudomonas free
* Colonizing S. maltophilia
* Vertex results are in for the combo drug for F508 mutations.  This medication would work for Charlie's specific mutation, although unfortunately it doesn't seem to work as well as I hoped it would.  However, progress is progress and that makes me happy!

So that's it! I mean, does it get easier than that? We definitely have had a lucky few months- I hope I'm not jinxing it by writing that lol.  Fingers crossed he stays healthy!

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