6 months on...sponsored by Kalydeco ;)

Had my 6 months kalydeco checkup last week at hotel wythenshawe. Lf was stable at fev1 2.75, weight had gone up to 67kg, probably my highest weight for the last 4 years. So all good, more of same really, which considering I've not had ivs or oral antibiotics in that 6 month period for the first time in I don't know how long, is pretty remarkable really. Six months on a lot has changed, I am now capable of physically doing a lot more in the day (i.e as a parent to two little terrors ;-) and I'm not as exhausted in the evening either.

   I talked to the doc about whether I was still culturing pseudo (I was) I also asked him about whether younger people on K would find it easier to get rid of tenacious bugs like pseudo and he said that the evidence so far was that pseudo was still very much there, just causing a lot less problems. I myself noticed that I was developing a bit of a cough in the morning without my usual nebs (I'd dropped off promixin for a couple of months) but when I went back on cayston there was an immediate improvement after a day or so. I have also switched from creon 25 to creon 40 and this has helped my guts, it has also helped me not have to take quite as many tablets with each meal, bonus! The nurses also mentioned the phase 3 trials for the combined vertex drugs for DF508 were starting soon so fingers crossed that provides a breakthrough for the majority of PWCF.

   And that's it really, I can now throw myself into crazy things like an all day snowboarding learning session, before Kalydeco this would have destroyed me, now it is still hard, but I am not on the edge of utter exhaustion from dropping blood sugars and Cf lungs like I would have been before. It's great stuff.
   Stop the press...sweat chloride test came back as 37, anything below 50 is classed as normal. My original (pre Kalydeco) score was 120!

   

Aye I My Eye at the MRI

Well, it's been a while! Sorry. And all that. I've basically been really busy with a new job and haven't had much time to sit and reflect until recently. This is wrong, because I should always find time to blog. And vent, and moan. Sorry. And all that. ;-)

What happened back in March was that I was working part time at my request (I was frustrated at the type of work I was getting) for the last 6 months (at my old job) until the week before Kalydeco arrived, then, out of the blue my Manchester-based boss seemed to ask me to go back full time to help out with a new iPad based mobilization; which was great, because it was the sort of work I wanted to get into, but big companies being what they are I wasn't allowed to switch back to full time as all the layers of Norwich based middle management got involved. What they allowed me to do was work part time with overtime thrown in to take me up to full time pay AS LONG AS I HAD A COST CODE. Which was a bit silly, but OK, as long as I had a cost code because surely there was enough work to last for 6 months? There was but the cost code only actually lasted 3 weeks...doh! anyway, after all this silliness I went to a job interview which I wasn't expecting to get, but thanks in no small part to Kalydeco I was able to speak better and really impressed them, did the follow up interview and got the job!

To give some background I have had a few interviews in October/November for other jobs but hadn't done very well because I had struggled to find my voice with the huge amount of mucus in back of my throat in the morning, I couldn't speak with authority and it really affected my confidence in interviews. So big thanks to special K!!!

The new job *is* hard work, but it's in a team and there's a decent buzz about the place, my boss is a decent guy and for the first time in 2 years I'm actually designing and building projects. I'm a developer again! I'm going to put this one down to Kalydeco because without it I don't think I'd have had the energy to come across well in the interview and also cope with the sudden increase in work hours.

I have now had the cataracts operation on my left eye. This is where they cut out your old cataracts riddled lens and place an artificial lens in your eye. Scary, but worth it because I can now see loads better. In fact the day after the (local anesthetic) operation the colours I could see from my left eye were so intense I felt like I was Dorothy waking up in Oz! I will probably need reading glasses for my left eye though now because the lens is long sighted. My right eye also has *some* cataracts but not nearly as bad as the left, so I will probably leave the right for a few years and see how I get on. People with CF and people with Myotonic Dystropy can get cataracts, no one's quite sure why, but I guess with me it was going to be inevitable with both genes combined!

And so, on the Kalydeco front; I am, embarrassingly, at the stage of not remembering just how tough my life was before. It's awesome stuff. My 6 month check up is due in a couple of weeks time so fingers crossed it's as good as if not better than the test before before.

1 and a half months post K

Quick update; just heard back from the doctor at hotel de la Wythenshawe and my sweat chloride has gone from 120 to 62! Lf is up by 15% as well. More importantly; I feel loads better! I'm running out of superlatives really, my lungs just feel so much better than they ever have, or at least were when I was much younger ;-).

Scores on the doors for my lung Function were 2.775 / 3.8; no significant change on what it was, but I'm fine really, I've gone from choking in mucus first thing in the morning to having hardly anything there first thing in the morning. Oh and I can run for 20 minutes down the gym without bringing *anything* up, craziness! that would never have happened before K, except maybe back when I was 15...

Kalydeco has given me normal lung function!

Went to outpatients yesterday. Well, it's not as high as I hoped, and more importantly, it's not as high as I feel, but the scores at the outpatients were:

FEV1 2.85
FVC 3.70

Which works out for my age and height as:

FEV1 81.9%
FVC 85.3%

It's official, my lung function has crawled slightly above normal range, (normal range being 80% to >100%)  This is my highest blow since early 2009. 2009 was probably my last really healthy year (in retrospect.) 3 weeks in and Kalydeco has been awesome for me, even the physio was amazed at the turnaround. I didn't even do a best of three on the spiro machine ;-)

To put this into some kind of perspective; my last blow pre-IVs in December was 65%. My blow post-IVs in December was 65%. I had been on 2 weeks of Ceftazedime and Tobramycin and had been going to the gym 3 times a week to try and get rid of as much mucus as I could. After all that time, drug money and effort my FEV1 was the same as it had been before I had started IVs and I was pretty deflated. I was also baffled as these IVs had usually worked for me in the past. I felt slightly better but the cough and mucus were still there. The implications were that my CF was getting worse. All I can say is thanks to the scientists (and the IPIG :-))!

A loves her onesie

And on the tenth day...

Tenth day update! The mucus has thinned so much it is almost gone. I can talk properly for the first time in 2 years. I was borderline with blood glucose levels and kept getting hypos if I didn't snack every hour or two, that problem appears to have gone. I am already thinking of my life in terms of before K and after K, it's staggering...

10 days into K and I did my first outdoor 5k run of the year and I was a full 3 minutes faster than any of my 5k runs last year. It's official, my exercise tolerance is loads better with Kalydeco ;-) I feel like I have another gear while running, I haven't really felt like I had another gear since my twenties, maybe my teens...

I also used to get headaches after the runs as well. No headache today! Was a bit rattly a few hours after the run though so I think it did loosen some teeny plugs though.

O likes his batman onesie

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...:-)