Ok sooooo....tomorrow I will be receiving my first dose of Kalydeco / Ivacaftor / VX-770. Last week (Monday) I had my baseline tests done (loads of blood tests and my first sweat test since I was diagnosed back in 1981 when I was about 9 years old.) My sweat test was around about 109 so we will see what happens after I have been taking it a few months. It has been shown to consistently reduce PWCFs sweat tests to below the CF diagnosis line (60). Baseline blows were irritatingly high considering I need to prove to the SCG that I am benefiting from K after six months. FEV1 was 2.6, FVC was about 3.4 I think. FEV1 was the highest it's been in 8 months, I'm going to assume that is down to the nebbed Cayston, which I have been having my first dose of for the last 10 days - The docs have told me to stop that now so they can measure the benefit of the K on it's own for the first month or two.
I've been following this drug since 2009 when I first tried to get on the trial; and although it was all very promising (I tried to enrol in the trail but was a few weeks too late due to my genes being lost in my notes, grrr) I think the penny finally dropped when I read this article:
It was this bit which amazed me, the first Eureka moment for CF R&D?
"At first, she thought maybe she was just having a good week, or month.
Then, she started to bring up more mucus. “Actually, it felt like a
flash flood,” she said. “I would cough and it would just come flying out
of me, not to be too gross. It was just everywhere. It was much thicker
than it used to be. It was constant, all day long, and I thought, ‘Oh,
crap, I’m getting sick,’ but I never actually got sick.” “And as time went on, my amount of mucus decreased, and my cough
decreased. Then I started noticing bigger and bigger changes. I was
sleeping. I was able to maintain my blood sugars. I could basically eat
and not have to worry. I no longer needed any medication for
constipation. And the biggest thing for me was, that year was the first
year in my entire life that I never got sick.”
"Roe has a port in her chest for intravenous antibiotics. She hasn’t
used it since she started the trial in 2009. And more: before she
started on the trial, she had a “huge mucus plug” in her chest so bad
that she was considering surgery to remove part of her lung; now it
appears to be gone.
As she spoke to the Children’s Hospital cystic fibrosis team, she
mentioned that her FEV, a measure of her lung function, had been 62%.
Now it’s 81%."
The first months supply for me arrives tomorrow. We shall see...:-)