I am DF508 and 2184delA. I wanted to try Kalydeco for a number of reasons, most of which have been expressed by others on this forum. I called my ins abouta month ago and they offered coverage witha $45 co-pay. I then convinced my physcian to allow me to give it a go. He said that we should proceed very scientifically so that if the ins company pushed back on refills we would have a compelling case for a prior auth.(He also said he would not allow me to remain on it if I did not show "significant" changes in sweat chloride or other clinical outcomes-- which I am okay with) So I went in last week for a sweat chloride test and PFTs. I will attempt to describe my status-quo before and after.
<span style="text-decoration: underline;">Before:
PFT baseline in the 90s, hemoptysis every 2 weeks or so, thick lung junk that comes up following treatments, with airway clearance, after running, and in the morning.... Deep breath in the morning ALWAYS results in coughing fit (except for after a round of IVs), laughing has the same outcome
PFTs pre-Kalydeco: 91% (I had been 100% a month prior but I've had a couple bleeds and junkier lungs in the past few weeks so things were trending down it seemed)
Sweat chloride: 101
<span style="text-decoration: underline;">After:
I acknowledge that I'm only 4.5 days in (I've taken 8 doses)... but I do feel different. It could be placebo effect... but I don't think my mind can trick my body into doing the things I've noticed. After about two days I began noticing that I was bringing stuff up with MUCH less effort and all throughout the day, whereas before I would have times of day that were more productive (because of airway clearance, treatments, etc). Its almost like I would clear my throat and there would be mucus there. I have had decreasingly productive (and thinner mucus) in about that past 36 hours. Yesterday and today I woke up, took a deep breath, and was able to exhale withouta coughing fit. I run daily and did my usual 6 miles on the treadmill yesterday. (let me qualify this by saying I certainly don't think Kalydeco will be my "cure" and get me to carrier status, but I am hopeful it will at least decrease my sweat chloride concentration). I am usually caked in salt to the point that I can see it on my legs. After my run I didn't have time to shower (boys woke up) and I was playing outside. The sun hit my (now dry) legs and I didn't see salt. Take it with a grain of salt (ha) but I'm interested in seeing my new sweat test results when we re-test.
I'll share more in a couple weeks when the "placebo effect" wears off.
<span style="text-decoration: underline;">Before:
PFT baseline in the 90s, hemoptysis every 2 weeks or so, thick lung junk that comes up following treatments, with airway clearance, after running, and in the morning.... Deep breath in the morning ALWAYS results in coughing fit (except for after a round of IVs), laughing has the same outcome
PFTs pre-Kalydeco: 91% (I had been 100% a month prior but I've had a couple bleeds and junkier lungs in the past few weeks so things were trending down it seemed)
Sweat chloride: 101
<span style="text-decoration: underline;">After:
I acknowledge that I'm only 4.5 days in (I've taken 8 doses)... but I do feel different. It could be placebo effect... but I don't think my mind can trick my body into doing the things I've noticed. After about two days I began noticing that I was bringing stuff up with MUCH less effort and all throughout the day, whereas before I would have times of day that were more productive (because of airway clearance, treatments, etc). Its almost like I would clear my throat and there would be mucus there. I have had decreasingly productive (and thinner mucus) in about that past 36 hours. Yesterday and today I woke up, took a deep breath, and was able to exhale withouta coughing fit. I run daily and did my usual 6 miles on the treadmill yesterday. (let me qualify this by saying I certainly don't think Kalydeco will be my "cure" and get me to carrier status, but I am hopeful it will at least decrease my sweat chloride concentration). I am usually caked in salt to the point that I can see it on my legs. After my run I didn't have time to shower (boys woke up) and I was playing outside. The sun hit my (now dry) legs and I didn't see salt. Take it with a grain of salt (ha) but I'm interested in seeing my new sweat test results when we re-test.
I'll share more in a couple weeks when the "placebo effect" wears off.