August 11th

Thought I would post an update as I had clinic on Thursday. Insurance is still shipping my med (knock on wood) - I am so relieved! I am supposed to get another shipment this next week. I get nervous about it until I have it in my hands.

My clinic appointment went really well! My FEV1 was up to 89% (was 84% before I started Kalydeco, 87% last time). Before I started Kalydeco it was going down a point or two each appointment-so this is great! My doctor said that my lung function is pretty high already-that there is not a lot of room for improvement! Of course-I would love to see it at 100+!
The biggest change in my PFT's were  my smaller airways (FEV25-75) - they were around 65% or so before starting Kalydeco-on Thursday my best was 85% - amazing!

My doctor also looked at my x-ray from 2 years ago and said my current x-ray showed improvement-which is awesome!! So all in all a good appointment.

We talked about insurance denying me Kalydeco now - and he said because I have been on it for 6 months, and because of the fact that it is really helping me (PFT's are higher, sweat test is now at a normal level, and X-rays show improvement) that the insurance companies would have a hard time getting away with denying me.

We will see though-it makes me nervous that they could just yank it away from me if they decide they don't want to pay for it anymore.

I am still rarely coughing-which is fantastic! I don't feel like I am coming down with a lung infection all of the time either! They have started (or are starting soon) a study on one of my mutations (R117H) to see if Kalydeco works on it :) Lets hope it goes quickly and is approved by the FDA soon!