Miracle Drug

It has only been 4 days. 4 days.

Wow. As the saying goes I'm only 4 days in but I already feel loads better. Mucus is still coming out, but it's coming out easier and it doesn't overwhelm and exhaust like it used to. My bowels feel normal. They haven't been normal since I was 22. My blood sugars seem better; normally I get a crashing feeling of dropping energy levels if I leave big gaps (i.e. 2 hours) between meals. Haven't had that yet.

On Friday I went for a run at the gym and stuff was flying out of me so much that I had to keep pausing the running machine every few minutes to run to the toilet and bring up more mucus, it was gross but good! Easier to bring up. Did I have more energy in my legs for running? Or was it in my head?

In the morning I'm not overwhelmed by mucus in the back of the throat; I can talk to my misses first thing in the morning without worrying about having a coughing fit!

I went for night out with some friends in Manchester to celebrate the arrival of K (on Saturday) It was an epic night out where we tried to pretend we were 18 again and we all had a lot to drink (like you have to do, once in a while ;-)) This normally gives me a really bad CF hangover with low energy, really bad bowels, and double the mucus for the entire day (sometimes 2 days). There was hardly any mucus first thing in the morning! There was a bit more through the day but nothing major, I didn't even feel tired!

For the last four years I have been in a gradual decline, I think the running (have entered the Manchester 10K for the last four years for the CF Trust) and mountain biking has hidden that decline a bit, but after taking K for 4 days I have realised just how bad that decline has been. I couldn't talk for mucus first thing on the morning. The decline has now been taken away and I feel 5 years younger.

I am in awe. I keep tearing up. There aren't really words.

First Dose

Holly is unimpressed by K

OK, so first day in, I would say I do feel slightly better in subtle ways (please be aware that this is pure anecdotal evidence and it may be placebo, you have been warned ;-). First off, my nose is running clear occasionally; can I smell better or is it in my head? Second, I went down to the gym after my first dose to do some running and weights; and while running I wasn't overwhelmed by mucus like I usually am (sometimes have to pause the running machine and have to make a quick dash to the toilets) And later on in the day when I was putting O to bed and reading him a story, I wasn't overwhelmed by tiredness like I usually am, didn't seem to get that blood sugar crash feeling like I get in the evening and seemed to have more of a breath to read to him, possibly due to having less mucus. Like I said, early days, it could all be in my head, but I don't think so...

Victory for Kalydeco!

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:

http://commonhealth.wbur.org/2011/05/cystic-fibrosis

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...:-)