CF Appointment 12.7.18

The best way to start the holiday season is to get a great report at my CF appointment!  I wasn't quite sure what to expect since I battled a sinus infection around Thanksgiving.  I was living on DayQuil and nasal spray, just so I could feel like my head wouldn't explode from the sinus pressure.  Thankfully I felt fine other than my sinuses being clogged up - never felt anything move into my lungs! Yay!! That's always my fear - got to keep it out of my lungs!  I'd say I'm not 100% done with sinus issues, but I can survive off the medicine.  Just need to keep some tissues with me in my spin class - for some reason my nose starts running in that class...haha.

As usual, the appointment started with PFTs:

                             12/7/18     8/22/18     11/17/17     11/1/16
FVC                      104%          99%          99%             99%          
FEV 1                     95%           90%          89%             89%  
FEV 25-75%          77%           68%          66%             69%

I'm up some since August and even up just a bit more than I usually am this time of year.  So, that's cool to see.  I'm currently off my Cayston cycle - I'm right at 2 weeks off.  So 2 weeks to go and I'll start back - Merry Christmas to me!

My doctor and I discussed Cayston and having Pseudomonas Aeruginosa (that's the bacteria the Cayston treats).  There is another drug, Tobi, that is used to fight PA.  It used to be in a nebulized formula, which I actually tried 10+ years ago.  It tasted awful - I couldn't handle I stopped.  Then I learned of Cayston and have been on that since.  Back to Tobi....there is a new formula that is used in an inhaler, the Tobi Podhaler.  It's putting a capsule in a device, the capsule is popped so a powdery substance is inhaled.  Its twice a day for 4 weeks, then 4 weeks off.

We discussed giving the Tobi Podhaler a try.  I won't start it in December since I'll be starting at Christmas - with travel and just overall craziness with the holidays I don't want to throw in a new medication.  So, I'll wait til the next cycle which will be in February.  Plus that will give me more time to get insurance approval and all that fun stuff.

We also discussed a trial that is starting for CFers with one copy of F508del and a minimal function mutation.  That would be me!!! I have F508del and 621+1G-->T.  So that's pretty exciting to see my mutation show up on a list of trials!!  It involves taking ivacaftor, tezacaftor and a new drug VX-445. You can read about it here: Trial Enrollment

My clinic was able to select a patient to participate in the trial.  While I would have been happy to participate, I wasn't selected.  Thankfully my lung functions are too high and I wouldn't be eligible.  While I'm bummed that I can't be a guinea pig, I'm more happy that I'm healthy.  The purpose of these studies is to determine if the drugs its important to use someone that can see improvements from the medication.  So, I'm anxiously awaiting the results of the study!!

That about sums up my appointment.  I produced some sputum but not sure that it would be enough, so they took a culture too.  Yuck!  Always hated that part when I was little.

Happy to being feeling well!!


  1. We have the EXACT same mutations! How funny, I was beginning to think I was the only one out there. So glad to learn about you and see you doing so well!


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