GREAT news!!!

I had my follow up appointment today at my CF clinic to see how I am doing on the Kalydeco after 4 weeks (well, really 3 weeks with the stopping/starting/stomach issues).

I had my PFT's done first....they were 83% last time (FEV1) and today they were 87%. I was happy that they went up....because for the last 2 years they haven't increased. I thought they might  go up farther but I am pretty happy with 87%.

After my appt I went to get my repeat sweat test done. The test took and hour and they promised they would have the results that afternoon.

My CF nurse called me at about 5:30 with the results of my test. My first test, before I started the Kalydeco, was 81. The sweat test is a very good indicator if a person has CF or not-it measures the amount of salt in your sweat.

The sweat test scale goes like this:

Above 60: you have CF,
40-60:you are borderline and it is likely that you have CF
Below 40: normal


Are you ready for my results??? Keep in mind they were 81 (as in an above 60, you have CF level) 3 weeks ago.

Today......my sweat test was 36!!!!  36!

This means that my body is not acting like it has CF. It is AMAZING. Kalydeco does work on R117H! They haven't tested it on this CF mutation, they thought it might work but it has not been approved by the FDA for the R117H mutation. I am living proof that it works!
My CF nurse told me today that I should have a normal life expectancy...and that eventually we will probably back off on the breathing treatments! She said that I should stay pancreatic sufficient because I already am. 

I think I am still in shock and this hasn't totally sunk in! The kids and I are headed to Seattle tomorrow for their Irish Dance competition-so I will check back in next week!