Day 2

I took my 2nd dose of Kalydeco this morning just before 7am. I have had a bit of a headache but I'm not sure if that is due to the med (it was mentioned as a side affect) or because I just have a headache!

I have been coughing up more gunk - however I am also on doxycycline for a lung infection so I'm not sure which is helping more. I am hoping it is the Kalydeco...I think the next few days will determine that. I tend to be a bit of a pessimist.....mainly so I don't get disappointed! Probably not the healthiest way to look at things, but it works for me.

The true test to see if this medication is working will be in a month when I have repeat PFT's and a repeat sweat test. I am hoping to see a change in my numbers - which means Kalydeco is working! Then I will allow myself to really get excited about this new drug!

Here's a quick story of my diagnosis with CF - thought I should probably explain how/when I was diagnosed.

I was 21 and came down with walking pneumonia.....my doctor wanted to figure out why an otherwise healthy 21 year old came down with walking pneumonia...they gave me a TB test (which was negative) and then a Sweat Chloride test to test for CF. That test came back borderline (I think around 60 or so) and I was then given the genetic test (blood test) for CF. Talk about a shock-finding out you have CF at age 21!

I didn't start preventative treatment until I was around 32 or so....as I would get lung infections we would treat them with antibiotics as needed. Luckily I can still get by on oral meds and don't need to have IV meds. I switched CF Centers when I was about 32 and it was the best thing I could have done! I went from a large CF center to a much smaller center with a great doctor. I started on Advair, Pulmozyme, Hypertonic Saline, 3X week Zithromax...all of these preventative meds helped my lung function increase from 65% to 95% (FEV1). Right now my lung function (FEV1) is right around 84%. I found out I have an asthmatic component to my CF, so the advair is a huge help-I can really tell the difference if I forget a dose.

I am now 37 (almost 38) and am in relatively good health. I need to exercise more....I plan on starting to run again if I can talk myself into it :)