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...:-)

Headless Chicken

I've not blogged for a while although I probably should have; this is good therapy. I've basically been really busy with interviews which unfortunately have not been successful so far. I have also received a bit of a hammer blow regarding my long term health which has come as a bit of a shock to me and my wife. To be honest I'm still trying to get my head around it, will write more later.

It hasn't helped that at the moment the doctors (and I) are still baffled by a 20% drop in lung function that hasn't responded to IVs (IVs FEV1 = 2.35, FVC = 3.35) which hasn't really improved since the start (of the IVs, although FVC went up from 3.0). I also had a severe gastric DIOS blockage on Monday. You can tell the severe ones because they are the ones you throw up on. They are the one's you end up going to A&E for. Thankfully I avoided it but only by the skin of my teeth. Scary times...However there may be some good news in the new year which I am keeping everything crossed for.

That's it for now; I've got an extremely dull compulsory online exam to study for next Wednesday so I need to get cracking with the brainwash. Wish me luck ;-)

Random picture of Macc Forest ride

Confidence is a preference

Confidence. It's a funny thing isn't it? With it you can do anything, but without it even the simplest task can seem insurmountable. I say this because my confidence seems to be wobbling all over the place at the moment and I don't know why, it's such a weird thing. I wonder if I need to see a psychiatrist, maybe I'm still not over my dad's death, I wonder if in some ways I think was I was keeping going to keep *him* going, visiting him every weekend with the kids and dropping food round. Now he's gone I can do anything I want to at the weekend, but now I have loads of choice I don't know what to do...

I've recently switched to part time at work (they wanted me to work 1 week every month in Norwich, I balked at this and handed my notice in.) As a compromise I offered to work part time in order to brush up on my development skills (and look for work) while still keeping the IT systems (that only I know) going at work. At the time I was confident and felt this was the right thing for me to do, but fast forward a few weeks, throw in a couple of failed interviews, a gastric blockage, some arguments with the misses, and a depressed mother and I feel like I've hit rock bottom...or something close. I'm also in a funny situation where I'm made an attempt to sever my ties with work but I'm still there connected and also struggling to do all the work that I used to in half the time that I used to have.

I now have 2 days free and I thought this would be more fun, but it's not as much fun as I thought it would be! After dropping the kids off at school and sorting out the washing and ironing, looking for jobs, revising for jobs, and doing some shopping, there's not a lot of time left before it's time to pick up the kids again...gahhh! Stay at home mums/dads of the world, I salute you! It's certainly not as easy as I thought.it would be...

There's also a physical side to this confidence thing for me, my body's getting crapper- in the morning my mouth feels like it's glued together with mucus (attractive ;-)) - I need to get to the bottom of this as I can't really shine in interviews if I can't talk properly ;-) I don't know whether it's CF sinus related CF mucus related or MD related - we'll see.

Sorry for the moan. I think the plan of attack (on my two days off) has to be:

1) Spend 1 hour a day looking for jobs. No more.
2) Spend 1 hour a day revising my technical skills. Don't do more and get stressed, it's not worth it.
3) 1 hours exercise on my days off.
4) Get the electric guitar out and rediscover making music, or a least play D and E really fast.
5) That's two hours left to do jobs around the house and eat - should be enough, in theory ;-)

Urgh, I can't believe I made another todo list, they're the bane of my life ;-) Anyway, have a picture of me and the kids messing about on the beach in LA, bliss.

Gutwack

Talk about being born under a bad sign, I had an interview Monday afternoon and on the morning I started getting the tell tale signs (shooting gut pains) of the beginnings of a DIOS CF related gastric blockage. I only seem to get these about once a year but when I do they are horrible, painful things that I wouldn't wish on anyone (no, not even Thatcher ;-))  So far I've took 8 sachets of Movicol and 4 portions of gastro grafin, but still nothing!

It's my wedding anniversary today as well, CF has no sense of timing...

It's now Wednesday; I had been contemplating admitting myself into hospital to get this sorted but fortunately my bowels finally clicked into action this morning (and how, I've gone through nearly a full roll of toilet paper ;-) ) phew! It's completely ruined my anniversary though, son of a ....!

FEV1 was 2.3 have decided to go on IVs if I don't pick up next month...have got some orals to tide me over...

America: Hot in the City

Last Friday we got back from the fly / drive in America: 3 days in Vegas, 2 days in Palm Springs, followed by 1 week in Laguna Beach in the OC, California.

It was lovely, but Vegas and Palm Springs were much too much too hot for me and kids (40 degrees C or 108 degrees Fahrenheit in Palm Springs!!!) It was much cooler by the beach in Laguna though. The kids have been jet lagged for the last 3 days since they got back though, which has made the nighttime "interesting" ;-)

I'm in a hurry, I'll update later...(you lucky people ;-))

Itraconazole on the dole

So this last year I've been...

I have been having real problems shifting the mucus out from my narrow airways and what has been coming out has been hard and dry and causing me problems at night as my long suffering misses will attest. My LF has not responding to IVs either and I have lost 20% of my lung power over the last 4 years.

Anyway, I have been racking my brains trying to think of a way to treat this, and yesterday with my CF Team we came up with a plan that involved trying either Itraconazole, Hypertonic or nebulised Meropenem for a month to see which one improved my LF the most most. Itraconazole seemed a bit of a non starter because my IgE Aspergillus levels were fairly low. (there is no fungus in the mucus either, although there is Staph and Pseudo) When I got the email from my new boss the other week, one of the contributing factors to the decision was the thought of spending more time on my health trying to get to the bottom of just what the hell was going on in my lungs. Anyway, to cut a long story short, my gut instinct was to go with Itraconazole first for a month and, amazingly, within a couple of days; it's like flicking a switch, I feel loads better and the mucus is coming up much easier. Blows at the outpatients were FEV1 2.5 and FVC 3.5 so a marginal increase, but hopefully improving for my next visit in 6 weeks.

In other news I handed my notice in last Friday; it was nerve wracking - they wanted me to stay and think things over (but didn't offer me more money ;-)), but I'm still determined to change jobs - more for the excitement of a new challenge really. Having handed in my notice things became a bit clearer in my head; I realised I need to move to find a new challenge. As a developer, there is a real buzz to be had from solving problems and creating elegant solutions and in the last year at work there's been precious little of that happening. There *may* also be the option of working part time at my old place (20 hours a week) but we'll see - if it does happen it will only be a stop gap until I find something new. I realise my talk of taking some time off has now changed to finding a new challenge, but we'll see - in the short term it will be taking time off to concentrate on my health, but in the long term, hopefully there will be more intersting jobs out there, perhaps in the health sector, as I do have an interest in medical science and it would be great to help out in some small way.

For my next job I would like to have the working hours of a senior cat

Should I stay or should I go?

Seriously thinking of quitting my job and having a month off before looking for something new.

The new line manager has just sent me an Email saying:

"Hello J,
I am very keen for you on a monthly basis to spend time on-site here at Norwich. What I am suggesting that you spend a week a month on-site, does this work for you?"

I was thinking of replying along the lines of

"Dear line manager / corporate goon, I have 2 kids under 7 and my wife works full time as well, what do you think?"

So I am seriously weighing up my options, it's not as if the job pays well enough or is exciting enough / look good on my CV enough for it to be worth me missing out on my family for 1 week in 4. Having typed this I have just realised I am turning down staying at a hotel with a free meal and no kids screaming at me for 1 week every month, am I insane? ;-) 

I have some money saved so I will be able to cope for a few months without pay and I will be able to in inverted quotes "concentrate on my health" (whatever that means, probably sat on my arse watching films I taped from film four, sound good to me ;-)) But seriously, there's all this stuff with Kalydeco kicking off, I have lost 20% of my lung power in the last 4 years, so it may be interesting to see if I can reverse it / get to the bottom of it with my team, who knows? Need to decide by Friday though...

It is Oscars birthday tomorrow, he is 4. He is still obsessed with Pirates, is reception ready for him? And will he learn not to wet his pants on a regular basis before he starts next month?

Sods Law and the Roses

Sometimes CF can really kick you up the backside - like when you catch a cold on the last day of your IVs. When I went into the outpatients to do my scores I got no change at FEV1 2.3 /  FVC3.3 so it looks like the IVs were completely negated by the cold, gutted :-( So I'm back on 2 weeks of orals (Cipro and Doxycyline, again) to carry me over this chesty cold, and as much running as I can manage in this shitty weather.

Anyway, I did fit in a trip to see the Roses at Heaton park last week, which was brilliant. The Stone Roses are part of my misspent youth, they first broke through when I was about 16...pop music had become really bland at that point and when they first appeared on Top of The Pops (in the same week as The Happy Mondays) it was a seminal moment: finally here was something real after all that bland rubbish, and they were from Manchester as well, fantastic! We're the band any good? After a couple of glasses of cheap red wine in a comedy packed to the rafters metrolink who can tell? But the music and crowd were so happy and positive it seemed to carry everyone away on a nostalgic bubble of happiness that rarely actually happens at these type of concerts, and personally, I thought it was a amazing. :-)

Outpatients through the looking glass

Started my IVs today at outpatients as I promised myself last month. Unfortunately my lungs haven't improved enough (FEV1 2.45 / FVC 3.4) despite the lovely week in Kefalonia followed by a shit week in rain filled Manchester (actually thinking about it the two things probably cancelled each other out). Hopefully I can regain some of this LF with the IVs but I don't know. I feel like I haven't hit the heights in the house of lung in a long time now and like Slade I don't know why. Anymore.

I've looked back at some of my old posts I found this one back in late 2008 and I had an FEV1 of 3.1. Who was I then? I had an annoyingly good FEV1 despite not doing any real exercise. Since then I've taken up running, mountain biking, Steve Jobs has taken over the world, died, and Europe is doomed on a weekly basis. And despite all that time and effort into getting physically fitter I have lost 20% of my lung power and I have no idea why. My last roll of the dice is to join a gym and try and build some muscle; if that doesn't work then Vertex and the PCTs had better pull their finger out with Kalydeco because I'm fresh out of ideas on how to beat this thing called CF. The new pharmacist is really hot though ;-)

2008: Smug twat with good lung function;-)

The Politics of Kalydeco

Don't you just love politics? The first cure for cystic fibrosis, the first drug that targets the underlying cause of CF and I have no idea when and if it will be available in the UK. I am hopeful, but hope is all I have at the moment. The company that owns the drug (Vertex) wants to make as much profit as possible, the NHS wants to save money. The total CF budget for the UK is 100 Million. If Vertex charges what it does in America then it will cost 50 million to treat 5% of the people with CF who have the G551D gene and can benefit from Kalydeco. There is posturing on both sides and the months tick by. Meanwhile people on the transplant list are one bad cold away from intensive care. Please sign this if you can, if only to raise the profile of Kalydeco / vx770 :

http://epetitions.direct.gov.uk/petitions/34753

We're currently trying to gather together as many people as possible who have the G551D CF gene. Please leave a message if you know anyone in the UK who has G551D.

I have been on holiday to Kefalonia for a week and have not posted about it. For shame. It was ridiculously lovely and probably my favourite holiday for a long time. I think staying in a villa suits me much more than the battery farm approach of all-inclusive resorts I have been to with the family recently which were fine enough but looking back I was perhaps missing something I only get when we can do what we want when we want. Here's some photos...

Roger Red Hat: Funny Bugger

Gutted,

Just read this post from Rich's blog (One of the blogs of fellow CF people I follow in the sidebar on the left) and (to put it mildly) it doesn't look good. Rich was one of those "call a spade a spade" people who seemed to have a really ascerbic, black, (and quintessentially British), sense of humour with absolutely no regards to the politically correct. However, he was never rascist and quite often he was really bloody funny. He used to post on the CF Forum as Roger Red Hat. 

http://la-boca-de-la-cueva.blogspot.co.uk/

RIP mate and thanks for the nice post about City.

Run de la Mancunian

Ugh! Not my best ever run. It should have been good; a month ago my training was going well and I was all ready and meaning to step it up to that hard-to-reach next level of training, but then loads of jobs and things seemed to get in the way and before I knew it I was unprepared and actually feeling worse than I ever had in the second half of the race (my left knee - cruciate? In particular was really aching for the final couple of miles and it was only pride that stopped me walking the final km to the finish) Also it was Rachel's mate's 40th birthday on the Friday and although we'd planned not to drink, Rach had had a terrible week at work and before we knew it we were downing sambuca shots at 7PM and staying up till 2, not the best preperation for the race on Sunday...I love the atmosphere of the race though, the crowd are really great and signing up for the race has the effect of forcing me to go out running when I perhaps wouldn't bother if I didn't have anything to aim for. No more fundraising for a while though; I've done it for 4 years and I feel a bit bad that it's always the same people donating. I swear I'm going to carry on proper running after the 10K this year though...;-)

1 Hour 10, loser ;-)

59 minutes, totes jealous ;-)