Songs From The Id

Abby's talking head

2012-01-04T12:39:00.000-08:00

Sorry about my daughter completely taking over my blog. Normal service will be resumed soon, promise ;-) Abby is doing loads for her school journal, she's doing stuff I don't even know how to do

Abby's Christmas Photostory

2011-12-05T12:45:00.000-08:00

Abby put this together (with a little help from me) for her VLE school forum as part of her homework. Turns out she's better than me at video editing...!

The big 4-0

2011-12-01T04:41:00.000-08:00

It was my birthday last week. I am now officially very old ;-). It's slowly sinking in that to my kids I must seem really old - I can remember my mum and dad's 40th birthdays really clearly; I must have been about 8-9, I can remember at the time getting older seemed an almost infinite way off. It's now here :-/.

The weird thing is in many ways I feel healthier than I did in my twenties - I do more regular exercise, I drink much less and I get more sleep (when the kids allow ;-)) I've not been an inpatient at the CF Ward since I was 26. And for that I've got to thank my brilliant wife for getting me away from drink and excess and into fell walking and fitness when I met her in my twenties. God knows where I'd be if I was single and still trying to follow the Jim Morrison rock star model of excess; I'd probably weight about 8 stone and be falling asleep in my own urine in a pub somewhere. As it is that's at least another year off...;-)

Anyhow, I can't get too complacent because although my CF at the moment seems under control (touch wood about 10 times) I know myotonic (muscular) distrophy may raise it's ugly head at some point. I have two genetic diseases for the price of one ;-) Mytonic Dystrophy is a late onset disease but it varies considerably so there is no way of knowing just when and to what degree it will effect me, so far so good but I've recently noticed my neck seems to be aching a lot when I wear a bicycle helmet on long bike rides - and I'm also struggling to chew roast lamb and roast beef fast unless it's really tender - maybe I'm losing some muscle tone in my head / neck before anything else? Otherwise I seem to be OK (touches wood another 100 times) although I feel I should probably join a gym to maybe build up my muscles a bit more.

Smiabetic not diabetic

2011-11-18T01:49:00.000-08:00

So I met up with the Diabetes doctor yesterday and it turns out I'm not diabetic, yet! The 4 day continuous needle in belly test showed that I largely stay within the 4-7.5 range with only the occasional spike up into the 8.5 (usually after dinner/tea in the evening) but crucially, it doesn't stay there very long. She was even suspicious about whether I'd changed my diet for the test, and I definitely hadn't...she thinks my sugars may go out of control when I have an infection, which strikes me as particularly cruel; just when you need more carbs, your body lets you down by using less of them when it goes diabetic and you lose weight, talk about a viscious circle. Anyway, for the present I am just to keep an eye on things and maybe check when I am losing weight or have an infection.

I saw this RTA on the way in to hospital, (see link below, it was just outside the hospital) I think the lorry driver just didn't see him (has since been arrested on suspicion of driving without due care and attention) and he didn't stand a chance, just horrible. Life is so precious, this is such a waste. This is why I hate riding my bike on the roads, it only takes one person in a car/truck to not see you and you could be in serious trouble.

http://menmedia.co.uk/manchestereveningnews/news/s/1465540_video-medical-student-26-died-when-he-was-hit-by-lorry-while-cycling-to-work-at-wythenshawe-hospital

I'm moving offices today; a bunch of railtrack guys are joining us so I have to move my stuff upstair with the commoners ;-) rather than having an office to myself. I don't mind really, since my workmate left it's been a bit lonely in the office by myself. Particularly with the year I've had being in an office by myself has given me a bit too much time to think, especially with losing my dad this time last year.

Another day, another letter from the Queen ;-)

2011-11-10T03:58:00.000-08:00

Abby wrote and sent a postcard (of William and Kate, obviously ;-)) to the queen last week and got a reply from her lady in waiting! She's such a royalist ;-)

Her reading and writing is coming on leaps and bounds this year (Year 2 infants) She struggled last year and we were really worried she was falling behind the others in her class but this summer she's really made a breakthrough.

Diabetic Shmiabetic?

2011-11-04T04:12:00.000-07:00

Ok, this blog is getting soooo out of date that I really dont know where to start, so I'll update with some med news (from my annual) apparently my long term blood glucose (BG) levels, otherwise known as HbA1c levels, have been rising steadily since 2006; it has gone from 5.5% back then to 6.3% this year and so I was referred to the diabetic nurse for a four day strap on test. This is where you wear a tiny device strapped to your belly (it doesn't hurt but get's in the way a bit. Sometimes ;-) ) which measures your BG levels continuously to see how the BG peaks and troughs are and how high and low your blood sugars go. You also have to do finger prick test four times a day so they can cross reference the belly device results with the finger prick device results.

I hope I'm not diabetic, it would be a real pain in the bum. So far it looks like my blood sugar is relatively normal apart from a slight peak in the evening after dinner; 2 hours after a roast squash risotto tea I peaked at 8.5, otherwise I seemed to be between 4.5 and 6.5, but we'll see when I meet up with the doctors in a few weeks...

Bathroom Blues

2011-10-26T06:04:00.000-07:00

We have no bathroom at the moment. We noticed the wall behind the shower was showing signs of letting the damp in. We got the plumber to investigate and it turns out it was worse than it seemed. The water had seeped behind the tiles and eroded the brickwork. Because of the state of the bath as well we've decided to get the whole bathroom replaced and retiled, including more loose brickwork. I hate old houses...anyway, I suppose it'll be worth it when it's all done, just missing my early morning shower at the moment. I need it to wake up / do physio!

Anway, we're taking the kids down to London Village for a couple of days tomorrow so it'll be nice not to stress about the house for a bit. Abby and Rachel are going to see Wicked! I am a tiny bit jealous, even if it is a girls musical ;-) Otherwise we'll do all the touristy things like the London Bus Ride, Tower Bridge, etc. Should be good fun!

Wales Bike Ride in Pictures

2011-10-21T02:37:00.000-07:00

This was a very hard ride, despite the gorgeous day. I was nearly in tears with back / neck / leg pain at the end of the ride. More later.

Dear Darkness...;-)

2011-09-14T04:51:00.000-07:00

...Please could you see to it that I not catch this cold that the rest of my family have? After two weeks away, Oscar has caught another barking cough cold after only four days back at nursery. The last cold I caught gave me a rubbish night cough that is only just going after a full month including two weeks of mind numbing IVs and another two weeks of male PMT inducing orals (Cipro) to make sure it's fully gone. I'm just starting to get clear. Please.

Yours sincerely
-Mr Rubbish lung man

In other news, I went to see PJ Harvey (you have to see her live, she's brilliant!) last week and met up with my sis and a couple of my old band mates Matt and Pat before the gig. Was good to see them after so long and see they've not changed - even after a year or so we just picked up where we had left off. Good times, shame the gig was on a Thursday, but at least I didn't have a hangover the next day.

We're off to Newcastle this weekend for Rachel's Half Marathon (she's doing the great North Run) she's been doing great with the training, although the running has taken it's tole on her feet (her nails are falling off and there's been loads of blisters). Anyway should be interesting looking after two hyperactive kids in the Toon while she runs. The kids are going through an "arguing" phase at the moment so it may get a little fraught ;-) Hope the weather's good for Rachel's run.

End of the Ivs shower

2011-08-23T14:53:00.000-07:00

Well, I can't wait for this set of IVs to end and to have that lovely first proper shower without worrying about getting my cannula wet - I also haven't had the sense of smell for the last ten days possibly thanks to the Meropenem. And the runs. it's all good fun ;-) ...also; I've been thinking about this a while but I don't think the IVs are having as big a clearing effect on me as they used to, it's taken till the end of the 2 week course for the IVs to start to kick in on clearing my lungs...I swear it used to take 3 days before my lungs would go clear...why this is now I don't really know - I suspect it may be because my small airways are getting more knackered (losing their elasticity, otherwise known as COPD) so it is getting harder and harder for me to bring up the mucus from the small airways. I was also wondering whether it was down to the IV Tobramycin causing a problem in a similar way to the nebbed Tobi which caused my lungs to produce more sputum. Don't know. Maybe I need to chat to the doctors about trying something else...maybe a three week course instead of just two (shudder, hurl), or maybe with prednisolone?? anyway here's hoping the holiday air will clear my rubbish lungs just as much as the IVs...

Off to France tomorrow (Vendee) with the kids, via a car, ferry, and getting lost in France...hope the sense of smell returns in time to smell the sea, the sun and the French food ;-)

New Doctor + Missed Vein = Pain

2011-08-11T01:03:00.000-07:00

Well, I'm back on IVs after the oral antibiotics failed to do the trick. Got into the outpatients at 3 and it was mega busy, I couldn't even find a place to park. Unfortunately there was a new doctor (and trainee) that I saw for my appointment. I hate new doctors, it always takes me a while to get a rapport going with doctors (unless they're Connie Beecham, which has not happened yet, unfortunately ;-). Anyway, I / we decided it was time for IVs. The cannula itself is usually put in by the nurses, unfortunately this time it was put in by the trainee doctor, Nooooo! I could tell he was nervous when trying to find a vein to put the pink cannula in, true enough he missed first time and seemed to spend an interminable time poking around trying to find a vein, despite the fact that I told him exactly where my good vein was. And the area he tried was miles away. Grrr...anyway, second time lucky so not atrocious. If he'd failed a second time I would have asked for a nurse to do it instead.

Blows were down at FEV1 2.5/3.6 although I went for a half hour bike ride on Tuesday and didn't feel too bad, so a bit weird really. At my last clinic they said that the resistance vector of my pseudomonas to Ceftazadine had increased, so I decided its time to try one of the other IVs, this time Meropenum and Tobi. I have done it before (last time back in 2005) so hopefully it will be OK this time as well....I've also done my back in carrying Oscar. And I have another cold on the way. And society is collapsing. Good luck everyone ;-).

Manchester Riots

2011-08-10T02:19:00.001-07:00

"A mob's always made up of people, no matter what. Mr. Cunningham was part of a mob last night, but he was still a man. Every mob in every little Southern town is always made up of people you know--doesn't say much for them, does it?"

— Harper Lee (To Kill a Mockingbird)

I'm just so gutted about this really. I don't go into Manchester on nights out very much these days, (now I've got young kids to look after) but whenever I do I have always felt safe, and now that seems to have been taken away. I see some of these robbing clowns are even looting charity shops which help raise money for underprivileged kids. I consider myself a liberal socialist; I've worked in Salford and seen how depressing / deprived it is, I want to see these kids break the cycle of benefits + dropping out and make something of their lives. But this isn't political protest, they're not protesting against racism or for democracy. It's not even nihilism because to be a nihilist you have to reject the idea of "cool" logos on sportswear as well as society's rules. This is pathetic opportunist robbery and vandalism. I want these thugs to be tasered / immobolised before they damage any more of the city I love.

Amy Winehouse

2011-08-08T02:21:00.000-07:00

Whatever else you can say about Amy Winehouse; there is no doubting her talent. An incredible voice and a fantastic songwriter. I think we've lost the Billie Holiday of our generation.

Not really going to try to discuss her addictions/probable bulimia, but like Kurt Cobain before she was obviously a gifted but ultimately flawed and vulnerable person with a lot of demons to overcome. I think a lot of her fans (myself included) were mystified as to why Amy could not gain strength from this soaring talent (apparently she wrote Rehab within half an hour; she recorded Valerie in one take) and ditch the drug taking losers and sort herself out.

Russell Brand wrote a lovely tribute to her and addiction in general and I pretty much agree with every word. We also need to get away from this music industry idea that drugs are cool. The Beatles may have dabbled in drugs back in the sixties but it shouldn't be exaggerated; they were never high when they were in the studio. Amy Winehouse wrote and recorded Back to Black with Mark Ronson within a week when she was stone cold sober. Anyway. Back to the voice...

Camping it up

2011-07-28T07:32:00.000-07:00

Currently agonising about whether to go camping in the Lakes (Eskdale Valley) with the kids this weekend; part of the problem is all the extra stuff you have to remember to pack for the kids to keep them happy, it was a lot simpler in the old days! Ah well, I suppose if we don't go now we won't be going this year. I'm hoping to get back to an old campsite in Eskdale valley. I camped there with some of my mates back in the nineties when we were all a bit wilder; thankfully we've all calmed down a bit now ;-).

Me: "Alright Chaps, We’re thinking of going to Fisherground campsite this weekend, leaving early Friday because they don’t take bookings. This site has special memories for me because the first time I was there B threw a can of soup over me and V puked up all over his tent after too much ale. Give us a shout if you fancy coming."

V: "I was so ill that night! The soup was hilarious -I think most of it missed you and went over his car. Didn't he drive off after that with soup dripping down his car door after that?"

Me: "Yes, B dented his car skidding around the campsite and then we rescued a semi-conscious sheep at the roman fort that had fallen into a stream"

G: "Eskdale that was when we all jumped off that bridge into the stream? wouldn't dare do it now but I was hurdling it from the other side of the road pissed up "

V: "Checked that bridge out few years back, no way I would jump it know, we were crazy back then.
Can't believe you remembered that sheep!"

Can't start a fire without a spark...

2011-07-20T02:34:00.000-07:00

"I wake up in the morning, and I aint got much to say
I go to bed in the evening, feeling the same way
I aint nothing but tired, tired and bored with myself
Hey baby, I could use just a little help"

-Bruce Springsteen, Dancing in the Dark

God, I must be ill if I'm singing Springsteen in my head (I don't even like Springsteen, apart from his Glastonbury show the other year). I got a cold last week and it doesn't seem to be shifting...I keep waking up tired with my nose and lungs blocked up, it's a bad virus alright. Oscar has the same cold as well (think he caught it from nursery and gave it to me) and he's got a violent cough too, especially when we're all sat together eating...I've ordered some oral antibiotics from the hospital (they faxed them through to my GP) so hopefully these will do the trick, otherwise it could be time for some more IVs. At my last clinic they said (after some probing questions by me) that the resistance vector of my pseudo to Ceftazadine had increased, so it may be time to try one of the scary IVs, like Meropenum...here's hoping I don't need to...

My Dad's house is now under offer; so me and my sister (and HMRC ;-)) may be getting some money through some time soon. I don't know, I just feel so empty at the moment, I'd give all the money away if it'd bring my dad back. Although it's nice to know there's some security there with which to look after the kids in case anything happens.

Work is just not inspiring me at the moment, part of the problem is that the two mates who I was working with every day when I first started the IT developers job a couple of years ago have left, so I'm a billy no mates in the office on my own (there are some guys upstairs, but it's not the same as having someone to talk shit to all day ;-)) Part of the problem I think is that the HQ of the business is down in Norwich so although I get fired up when I go down there for meetings about business strategy and all the work were going to do, I am inevitably forgotten about and left to my own devices when I return back to Manchester and the work seems very slow in arriving at my desk. The business is also taking on a lot of contractors in Norwich (at great expense) to do jobs that I'm pretty sure I could do, especially if I was in a half-decent team. I think it may be time for a change; a new challenge, I'm starting to get jealous of my mates when they talk about how busy they are in their jobs...

The Polecat

2011-07-13T05:50:00.000-07:00

Abby: "Mummy, there's an animal under the shed, it's cute!" It certainly is.
The little critter has been nosing around underneath the shed for the last few days. I love seeing the world through kids eyes. I get so wrapped up in my own trivial little dramas that I forget how fascinating the world can be.

Centre Parks with the Willow Foundation

2011-06-04T08:19:00.000-07:00

Busy busy busy! my blog is so out of date I forgot to post about this holiday, and I'm off to Tunisia this weekend as well...anyway:
Last month we went to Centre Parks in Nottingham thanks to the lovely people at the Willow foundation. I did feel guilty because there is no way I am as ill as a lot of people who are granted their special day; but as my CF social worker said, I am fast approaching my 40th birthday, and after that I am no longer eligable, so it was a case of now or never this year really. I also thought I should give my kids a nice holiday just in case the shit hits the fan (whether through cf or work or whatever), and I'm not able to give them a decent holiday for a while. We had a great time, especially the kids, centre parks is taylor made for familes really, with lots of stuff for the kids to do. Seriously, I don't think they've slept so well before or since. I enjoyed it too, apart from the rowing which looks easy but is actually really hard ;-)

Work and other four letter words ;-)

2011-05-18T01:09:00.000-07:00

Work is really frustrating the hell out of me at the moment; I'm a software developer who works on mobile apps but basically I haven't got anything challenging to do at the moment; just the usual day to day mundane stuff. I think I need a change, but I'm not sure what to try next. I wish my dad was here, he was good with career advise. Maybe you guys can help? I will write more when I get a minute.

Anyway, here's some pics from the Manchester Run, I enjoyed it, the crowd was fantastic as usual, but I'm a bit disappointed in my time -1:07- that's no improvement on last year although I was on IVs up until the day before the race so maybe they tired me out? I certainly found it a struggle after the first 5K. Rachel ran a good race and finished in 58 minutes although she was disappointed too, we're never happy ;-)

Breakthrough! and Sods Law...

2011-05-06T06:39:00.000-07:00

As you may or may not know for the last two-three years I have decided to start doing more exercise in a bid to help improve/stabilise my lung function and to hopefully stop it declining between IVs.

This is now my 3rd year entering the Manchester Run and for the training I have been going for runs about 2-3 times a week without much enjoyment because I always got out of breath and had to stop every 2 minutes. But last week I discovered why: I had been breathing the wrong way (while I was running) I had been breathing the way I imagined athletes should breath -holding the stomach in and puffing out my chest, breathing through the top of my lungs (a bit like David Hasslehoff running across the beach in Baywatch ;-)) what happened was I would run for about 2 minutes then I would have to stop and catch my breath and start again.

Anyway, I read somewhere (embarrassing ;-)) that breathing through the diaphramatic or tummy breathing is more efficient for getting oxygen, and well, it seemed to work, all of a sudden, I had much more energy when I was running. Maybe now I can I can actually start to enjoy running and improve my running times?

Unfortunately, not at the moment because the entire family has caught a rubbish cold and I have had to go on IVs. I've also got a partial gastric blockage so running is too painful. There is also 9 days to go before the Manchester run, doh!

At the outpatients my FEV1 was 2.45. I was put on Ceftazadine, Tobtramycin IVs and Movicol for the blockage. Boo!...

Homage to Catalonia

2011-04-20T04:18:00.000-07:00

Well, here's a book by George Orwell I should definitely read, all about the Spanish Civil war; the socialists, the communists, the fascists, the anarcho-syndicalists et al. The birth and death of political idealism...looks like powerful stuff. If I ever get around to reading it that is ;-)

Orwell served as a private in the Spanish civil war and it was these experiences that inspired his later books Animal Farm and 1984. Here's a quote, what's perhaps surprising is how relevant it still is today:

"He had learned a hard lesson,especially about the new political Europe. Totalitarianism, the new creed of 'the streamlined men' of Fascism and Communism, was a new manifestation of Orwell's old Catholic enemy, the doctrine of Absolutism" ^[21] - "the ghost of Torquemada had arisen, imprisonment without trial, confessions extracted under torture with summary executions to follow." ^[22] "The essential fact about a totalitarian regime is that it has no laws. People are not punished for specific offences, but because they are considered to be politically or intellectually undesirable. What they have done or not done is irrelevant."

Manchester 10K run

2011-04-14T05:24:00.000-07:00

Oooer. Time is getting very close (5 weeks!) to the next Manchester 10K run. I was getting really stressed about things (mainly to do with my dad's house and the delays in getting it on the market as well as the usual crappy work stress) and it was affecting my sleep. So my misses told me to have a run at dinner to relive the stress. It worked, although I felt awful at times while I was running. Coughing, spitting, burping (get awful gas in the top of my stomach while running sometimes) but it was worth it; certainly sorted my stress levels out for the time being.

I've spoken to my sister about the house and I think we're going to get the house on the market by the end of the month; there's just some stuff to move from the house to the garage, to tidy it up before the photos, and a complicated potential-death-trap ceiling-rose light system to install. So as long as I don't get electrocuted, I think we'll be alright ;-)

http://connect.garmin.com/page/activity/activity.faces?activityId=78953233

My word, you do look Queer !

2011-04-08T04:19:00.000-07:00

I found an old 78 record belonging to my gran (In the olden days records would be played at 78 RPM rather than 45 RPM for singles and 33 RPM for albums) in my dad's record collection. I can't play it because I recently sold my record player, but I did find it on Youtube; it's from a set of funny monologues from the 1930s (originally performed on the radio or on stage in a vaudeville show) by Stanley Holloway and it has a particularly black sense of humour; it's hilarious ;-)

Grizedale Lakes Bike Ride in Pictures

2011-03-28T06:51:00.000-07:00

So the score for the weekend is Woody 1, CF 0. Although CF will come back you can be sure of that. Unfortunately, there's only one good picture from the Grizedale trail we did on the weekend. The other one is of V's helmet after he took a tumble; at least we know why it's important to wear helmets. Also, looking at this main picture I have decided I really should not stand next to MB in any more pictures, he's about 6'2" and he makes me look tiny. ;-)

I cannot tell you how hard this was at times, especially towards the end of the 8 hour ride when I felt semi-conscious going down some of the hills with my shoulders aching. But it was worth it; my lungs felt really clean at the end of the ride (was coughing a lot at the start and brought up some nasty looking plugs halfway round) and at the end of the day it's good fun (most of the time!). Whenever I was struggling to peddle up a hill I tried to focus on some of the CF peeps out there that have gone through/are going through incredible fights on a daily basis with CF (Tori, Poozie, Angel, Kirstie and all) and I try and gain strength from knowing whatever I go through is nothing in comparison.

Not so sure about the pubbing/clubbing in the evening, Bowness is a bit towny, and I really couldn't be bothered...I think drinking is overrated. There was a girl/girl fight on the dance floor in one of the clubs (bouncers thankfully intervened before it got too nasty) that really freaked me out. I hate what alcohol does to some people...

Patronised on Ebay!

2011-03-24T05:27:00.000-07:00

Kids these days; should I be annoyed? I wouldn't mind but the thing I was selling from my dad's house only sold for 99p, the damn fiddly postage and packing to France cost £17...

Edit: I've decided to forgive him as he has offered to pay some money for red nose day because he feels bad for getting such a cheap bargain. ;-)

"Thanks for the "wrong" barcode 8-D LOOOOOOOOOOOOL
==)> not "LY215357443.......68"
but "LY215357443.......GB

Hopefully i'm used to buy in UK ... so i'm used to translate your two last numbers into corresponding letters ;o))
==)> i think you should go to bed by now ;)"

End of ABs and the real work begins

2011-03-22T06:02:00.000-07:00

Sorry for this geek post ;-) Last week it was the end of oral antibiotics and my lungs felt better without feeling *deep down clean* the way they do with IVs, however if you compare these two races - then my all round fitness does seem to have improved since the start of the minging infection (March 8th was in the middle of the infection and I was coughing all through the ride, March 22nd is on a sunny day today 1 week after the ABs) so hopefully I'm on the road to recovery...

Back to Activities

Bramhall Loop

Road Cycling

Tue, Mar 22, 2011 12:09 PM

Bramhall Loop

Road Cycling

Tue, Mar 8, 2011 12:38 PM

Summary

Distance	7.52 km	7.21 km
Time	00:29:28	00:33:02
Avg Pace	03:55 min/km	04:34 min/km
Calories	225 C	310 C

Avg HR (bpm)	123 bpm	131 bpm
Max HR (bpm)	148 bpm	151 bpm
Avg HR (% of Max)	66 % of Max	71 % of Max

http://connect.garmin.com/activity/comparison?activityId=74473657&activityId2=71944384

Last week I visited the hospital outpatients and was seen by Proff Web, it was nice to have a catch up with the grand old duke of CF ;-) because I rarely see him in clinic these days (he's semi-retired I think, but certainly knows his stuff). In the end we didn't talk about the usual CF things like I would at a normal oupatients clinic, we just talked about the good old days when I first moved to the Adult CF Hospital and the progress of the VX-770 trial. I was a bit depressed about the potential cost of the drug, and whether NICE would be able to fund it, but I talked it out on the forum and now I feel a bit better about it. FEV was 2.65 and 3.2 which is a bit down; I'm hoping it's going to pick up as I pick up the exercise...

Ebay a go go!

2011-03-16T06:24:00.000-07:00

I've started to put some of my dad's stuff on Ebay, I don't think we'll get much for it all but it's nice to know somebody will be able to use some of the stuff; especially his electric riser/recliner chair which I really hope somebody disabled will be able to use. Seeing some of his old stuff doesn't half bring back memories; he used to play D&D (dungeons and dragons) in the sports and social club at Phillips Hazel Grove. It's got something of a geeky reputation now but back in the day (before computers) it was popular in a cult way, there was at least as many women as men playing it in the group, at it's peak there were about 16 in the D&D group (AORTA: Association of reasonably trustworthy adventurers ;-)). We played it as a family too; my dad had read The Hobbit and The Lord of the Rings to my sister and myself when we were children and it really fired our imaginations, with D&D my dad as Dungeon Master would create a world similar to that in Lord of the Rings and we as characters in that world would have to negotiate our way through dangerous adventures...memories...

I've also got my mates to help out demolishing a rotten wooden outhouse and tidying up the garden, unfortunately there was so much thick ivy branches growing in the garden that taking down the ivy has demolished the fence! So now we need a new fence. We'll get there, hopefully by the end of the month because I could really do with putting the (emptied) house on the market so I can relax and forget about it. I'm not expecting it to sell straight away but at least if it's on the market there's a chance!

My sister has been working on the interior, it's not gone smoothly! The idiot she got in to do the decorating ending up giving the curtains away to a charity, so now everyone can look into the house and see it's empty. He also threw out the recycling schedule so now I don't know when to put the bins out! And he didn't actually finish the decorating job!!! Some people...

Kids last month in pictures

2011-03-11T04:20:00.000-08:00

Dad's House

2011-03-09T07:48:00.000-08:00

Maybe his back garden has been a bit neglected over the last few years. I think I'm going to have to have a go at doing it up before we can sell the house...there is also all his stuff as well, just boxes and boxes of stuff that I don't know what to do with. Books, photos, letters, computers, electronics, crockery. Normally, I'd put it in my loft and think about what to do with it later, but I don't have a loft, we got it converted so Oscar could have a bedroom! Help...

Rough Week

2011-03-08T02:20:00.001-08:00

Well, after the good news of VX-770 I was brought crashing straight back down to earth with a virus that cut out the middle man (nose and throat) and went straight onto my chest. So for the last week boat loads of sputum has been bubbling out of my lungs at night like mount Vesuvius. I rang up the Hospital and got them to fax an antibiotic order through to my doctors with the minimum of fuss. They're very good. I'm on Rifampicin and Ciprofloxacin, so I'll need to watch the mood swings and nausea...

On Sunday I went for a bike ride to try and clear some of the gunk. It wasn't pretty ;-) My mate was going to come with me but he had to cancel at the last minute. Just as well really, he would have needed counseling if he'd seen what came out of my mouth ;-). I felt really ill during but it was worth it afterwards because I did feel a lot clearer. My heart felt a bit fluttery for a few hours afterwards though, maybe it was a side effect of the antibiotics...

http://connect.garmin.com/activity/71646577

Good, no, Great news Day!

2011-02-23T07:41:00.000-08:00

Hey, look I know this is only really good news for us G551D's so far; but Vertex are working on a correction for you Df508's as well, so fingers crossed. I'm especially hopeful that this will be of benefit for Rob from the CF forum who is fighting to get on the transplant list at the moment.

"Phase 3 Study of VX-770 Showed Profound and Sustained Improvements in Lung Function (FEV1) and Other Measures of Disease Among People With a Specific Type of Cystic Fibrosis

- Relative mean improvement in lung function of approximately 17% from baseline compared to placebo achieved by people treated with VX-770; mean absolute improvement from baseline of approximately 10.5% compared to placebo; both measures through 24 and 48 weeks -

- Significant improvements in all key secondary endpoints for VX-770; patients were 55% less likely to experience a pulmonary exacerbation, had significant reductions in sweat chloride and, on average, gained nearly 7 pounds -

- Discontinuations due to adverse events were less frequent among people treated with VX-770 -

- Data support Vertex plan to submit U.S. and European regulatory applications for approval in the second half of 2011 -"

http://investors.vrtx.com/releasedetail.cfm?ReleaseID=551869

Dreams of Dad

2011-02-08T04:55:00.000-08:00

Had another dream about Dad last night, Can't remember the details very well. He had been admitted to hospital but he hadn't died, he had recovered but not enough to go home so we were organising a day out for him I think; then the dream changed and we were on a weekend break somewhere, but I got lost and was trying to retrace my steps back to the holiday home we were all staying at. In the dream we (dad and me) had a long conversation but I can't remember what it was about, I think he was just saying wise dad-type things and I was was talking back thinking yeah, he was really wise ;-) In another dream a few weeks ago I met his spirit while walking along a beach, later on I met my Gran's spirit and I tried to get his spirit and my Gran's spirit to meet so they could talk to each other and move on. In yet another another dream a few weeks ago we were talking on the phone, he was still living in Caterham down south and I told him I loved him at the end of the phone call, think I woke up crying at the end of that dream.

Maybe this is to be expected? Obviously not having my Dad around anymore coupled with the swiftness of his passing has left me with an emptiness (subconsciously and consciously) that will take some time to figure out. I was talking to my sister about it and she said when someone close to you dies it leaves a gap in your soul and your soul has to grow and change shape. It's similar to what happens when you have children, you have to change to become more responsible when a child arrives to that you need to look after - when Abby first came home from the hospital after being born I remember I was so nervous, the in-laws brought a bottle of champagne and after a few sips I started to get little tipsy and I immediately started to panic thinking "oh no, I can't be tipsy while looking after a baby) . My sister's very cosmic, if you hadn't guessed. She's also lived and worked in Peru near Machu Pichu; I'm very jealous ;-). We're trying to sort out his estate at the moment but it's not easy, he was something of a hoarder so he's kept every single letter he received for the last ten years, not fun trying to sort through it all.

Anyway, in the conscious world ;-), it's two of our friends' 40th birthday this Saturday, it's music based fancy dress. I was originally going to go as one of the Beatles but R wanted to go as a duo so we're going as the White Stripes. Gutted, I wanted to be John Lennon for the night ;-) Ah well, at least the costumes will be easy. Think I'd better start working out as well...

Exercise, fitness and fainting

2011-01-28T07:19:00.000-08:00

So the snow has melted and with it the excuse to not exercise. Even though if it's still cold and it's hard work. For some CF reason my base (after a month of doing bugger all) fitness is a lot lower than everyone else in our bike club; so while they can get to the top of the hill and take nice pictures like this one, I'm at the bottom of the hill still cycling like a madman trying to catch up, grrrr! I will get there one day...

The lodger

2011-01-24T03:21:00.000-08:00

There is a new lodger in our house. She is called Holly and she is very furry. Her previous owners were going to send her to a cat's refuge because their son was allergic to her, so we have stepped in. So far so good; she likes being stroked, she likes being picked up and carried like a bloated-furry baby ;-) , she likes being combed, and she definitely likes begging for tins of tuna when I'm making Abby's sandwiches. Also; she really doesn't want to stay in the house for the first two weeks so keeps trying to escape whenever we open the front / back doors.

Because we don't know whether our kids will be allergic we have laid down some ground rules: no sleeping on beds, and Abby must wash her hands after stroking and picking up Holly. I do like having a cat around, they're good company, even if cleaning out litter trays is a bit of a pain...

Gallons of rubbing alcohol float down the A6...

2011-01-12T03:16:00.000-08:00

Well it was our turn to host the New Years eve party this year, so we bought a karaoke machine, loads of wine and beer, and made 3 huge currys (on new year's eve) to feed the screaming hordes. We dispatched our kids to the in-laws, but some of our mates were bringing their kids so we set up the Wi in the front room for them to play. Everyone enjoyed the party, especially the karaoke; I never knew our friends were such frustrated rock stars ;-) Was a good night, at least until someone found the absinthe. Anyway, on balance, having been on both sides now, I think it's much more relaxing going to someone else's party! It's too much of a stress trying to work out whether you've got enough food / drink for everyone. In the end we made too much, but that meant we had lots of leftover curry to eat for the next few days ;-)

The party was a bit controversial because two of our mates are in the process of getting divorced, and because they (G and N), are currently ignoring each other, this meant we could only invite one of them because if we invited N, then G would refuse to go, along with his mates. I'm trying not to get involved and just hoping they can patch things up for the kids really, (their kids are the same age as Abby and Oscar.) When did life become so complicated? Also someone (not me) decided to invite an old school friend of mine who I've not seen since I left school some 2o years ago! I was a bit worried at first that he might turn up mob handed or something, but in the end he arrived and was more nervous than me, stayed for a couple of beers and a brief chat about the old days, and left; so it was no big deal really. Sometimes I think you drift away from friends after school for a reason, and maybe some blasts from the past should be kept in the past. My sister is adamant she never wants to get in touch with anyone from her old school and I kind of know what she means...

I don't know, but as the years go by I seem to be enjoying alcohol less and less, maybe it's the hangover / comedown, or (since the kids have arrived) any gleeful piss up is tempered with the knowledge that I have to be awake the morning after to entertain the children, but I don't enjoy it half as much as I used to. Maybe my body's getting old ;-) By the end of new year's day I was ready to go to bed at 8PM...I'm wondering if this is a CF thing, but every time I drink heavily, the next morning my lungs have much thicker mucus to bring up, and in the case of major bender, it can take a couple of days for the sputum to return to it's normal, more manageable thickness. I can also get terrible guts the next day as well. Alcohol sucks! Never again will I get drunk... ;-)

Turn, Turn, Turn

2010-12-27T14:41:00.000-08:00

The old man requested this Judy Collins cover of Pete Seeger's song be played at his funeral about a month ago. Ah, he was well into his sixties folk :-).

His funeral was last Wednesday and leading up to it I was dreading the whole thing. In fact when we parked up behind the hearse outside the chapel and the funeral directors got the coffin out I was really paniky and freaked out, but halfway through the service I felt sad but somehow serene. If he was there in spirit then I think he was telling me it was OK to move on, let him go, and not to be too sad. I'm going to miss him loads though, even though he was house bound in recent years he was still a big part of our lives.

Dad

2010-12-16T14:19:00.000-08:00

My dad died peacefully on Tuesday 13th December, 4.30 PM. He was 65. It was only the week after his birthday and on his 65th birthday we all went round and he told us (my sister, my mum and I) how proud he was of all us and his four beautiful grandchildren; we talked about lots of things (including the Tibetan book of the dead, Greek legends and all sorts) and it's just such a shock that this would happen because he was really upbeat. We talked about what he would have for his Christmas meal. (He wanted trifle!) I'd just set him up with a new digital recording box for his birthday and he seemed really happy.

He went into hospital Saturday evening with breathing problems, when I rushed in to meet him at A&E (I'd been on a night out so had to get a train and R had stepped in to help him out and persuade him he needed an ambulance) he was struggling but he was also talking about the City game in the afternoon and how crap one of our strikers was. His breathing seemed to settle although he was on O2 he seemed to barely need it. R went to get him some clothes and turn his fire off and at about 11 I left him to get some sleep just before he was moved onto a ward. When I got home the hospital phoned and told me to come back straight away. Shortly after arriving on the ward he had had a massive aspiration into his lungs, and he just never regained consciousness. We kept hoping, but in the end he was just too weak to come back from this trauma. In a way I think it's what he would have wanted, he hated being in hospital, he didn't want to end up in a home, and he kept his independence to the end, which at times was a real battle for him.

My dad had Myotonic Dystrophy; it wasn't diagnosed until he was 50. it's a form of muscular dystrophy and it was complications from this that led to his death. He had had his ups and downs with depression in the last five years due to becoming more and more housebound as the illness began to restrict his mobility and limit his ability to eat. In fact this year I was giving him half my Scandishakes (he was on Ensures as well) to help him keep his weight up. We talked about tube feeds and neck operations but he was too old and stubborn, he didn't want anything like that.

His mind was so sharp, he was a clever guy, a funny guy and he loved life. I hope the ongoing genetic research can one day eliminate awful, horrible, unfair conditions like this.

Mum and dad got divorced when I was 18 but after it had all blown over they stayed good friends and for the last years she helped with his shopping every week and they'd have a good natter.

I'm going to miss him loads and I'm really sad but I think he'd rather we remembered the happy times and I think he enjoyed life; he had great friends, family. He had two great jobs. He was a genius at micro chips/silicon chips. With his jobs / holidays he got to travel the world, he visited every country in Europe and most states in America. he got to ride the pacific highway from LA to San Francisco. He lived in Manchester and London, in London he lived in Caterham in a lovely house near the south downs with fox cubs at the bottom of the garden.

Love you dad.

xxx

Broken

2010-12-12T13:41:00.000-08:00

My dad's currently fighting for his life in hospital, I was with him when he was admitted but he deteriorated after he moved wards. He's currently stable but he's unconscious and it doesn't look good. Going to get some sleep and say some prayers.

Love to all xxx

Impatient at the outpatients

2010-12-03T04:26:00.000-08:00

It's been a while. Sorry. It's not you, it's me. I've had a lot going on, and I've been unable to articulate the thoughts in my head with any degree of clarity. I can't guarantee clarity anytime soon either. But I can guarantee it will be pretentious ;-)

Dah, well, I've had been feeling pretty good about health, life and fitness for the last few months, but cometh the winter, cometh the downturn. It all started about three days before my outpatient appointment, I'd been thinking how lucky I was to avoid getting a cold what with the entire population of greater Manchester having coughs and colds, when I actually came down with a cold; for the first three days it was just a bog- standard nose/throat cold, but on the fourth day, when my cold symptoms started to ease, the virus started to move onto my chest and my rubbish CF lungs started responding in their usual way by producing the thickest, gunkiest gunk this side of the Exorcist.

By the time the outpatients appointment came it was a complete no-brainer as to whether I needed IVs, so my cannula line was fitted and I was putting the IV boxes in my car ready to get on the home-IVs bus. At least that would have been the case if my Ultrasound appointment hadn't overrun so long that by the time I'd ran upstairs (on an empty stomach, stressed, and unseen!) to the CF outpatients it was already too late to get started on IVs, Arghhh! Note to me myself and I: if I have two appointments at the same time, one an ultrasound and one a CF outpatients then definitely go to the CF outpatients first! Anyway, I can't stress enough how professional my CF team are; when I told them what had happened. They ordered me some food, got me a cup of tea and ordered my prescription ready for the next day. Also, whenever I go in and say I need IVs (admittedly not that often, touch wood ;-)) they always agree with my judgement and just get on with the whole process with as little fuss and stress as possible. Makes it a lot easier for me. Blows were down some (FEV1 2.55) as expected, but I could feel that anyway with the increase in sputum and night coughing. I was going to do some running and biking this week to help with my physio, but with all the snow and ice, it might not be the best idea...roll on thaw!

I also have some stressful things going on at work, with my mates, and with my family, but I am all moaned out, so it will have to wait till another day ;-)

Oh Bramitob: We are so disappointed....

2010-10-06T05:30:00.000-07:00

To give a bit of background, I started on Bramitob a week after I'd come back from holiday in Ibiza. I don't know about you, but holidays in hot countries always seem to have a good effect on my chest, and I usually feel clearer and dryer while I'm there; whether that's down to the dry air or the sea air I'm not sure; although the last time I blew a massive 3.3 it was straight after a week by the coast in Wales in the middle of the summer. Maybe the NHS should pay to send pwcf off for a week in spain every winter, it'd be cheaper than IVs and perhaps just as effective? Just don't tell the Tories, or the Daily Mail ;-) I also finished off an old course of Cipro I had after the holiday to make doubly sure my lungs were squeaky clean for the test.

Anyway, I went to the hospital to do the first neb of the trial with the physio (they do this just to confirm there is no sudden drop in FEV1 an hour after the first dose of Tobi) After a few blows, all was well, in fact halfway through I figured out my blow technique has been all wrong (I was blowing too hard) and thanks to that (relaxing and blowing slower) I was able to comfortably blow 2.9; great! so all in all there was no ill effects after the first dose.

For the first week all was well, apart from a slight sore throat and the Bramitobi tasting of cheese. I even went on a solo mountain bike ride (both my mates cried off with excuses) in the rain with no obvious problems. However; by the second week, I was starting to get both a tightness and a wheezy rattle. And also, there was something I couldn't quite put my finger on; even though my sputum was thin and white, it was as if the sheer volume of sputum I was producing was starting to increase, to the point where it was causing me breathing problems. My long-suffering misses even complained that I was breathing noisily or "weirdly" in the night (and I hadn't been drinking either ;-)) So after consulting with the phsiotherapist (and the ever knowledgeable Telamere, who was also doing a similar trial) I decided to stop it.

Gutted, it's meant to be a lot more effective than Promixin and can even reduce the amount of IVs you need. Ah well...

Son of Tobi: Bramitob

2010-09-14T14:38:00.000-07:00

Earlier this year I trialed Toni through the INeb with less than spectacular results (my chest got wheezy, gunky and crappy and I ended up on IVs) Was that down to the Tobi or was that down to a virus, the cold, icy winter and weight loss? I've done Colymycin nebs for many years now with no problems and I've done Dnase since the mid noughties.

Whatever anway, the CF Manchester team did suggest we retrial the Tobi with a different, isotonic brand to see if it that would have a better effect. I'm just starting a months trial of this. so far so good but it tastes like parmeggiano cheeses (or smelly socks); I'm not sure which. Or is it goat's milk? 3 days in and OK so far. I've decided it doesn't taste like Parmeggian cheese, it's more like milk that has been left out of the fridge too long...the physio said they'd made an attempt to improve on the taste of Tobi as well. I'm trialing the Bramitob in a Pari compressor nebuliser and it takes about 5 minutes. The only downside is I need to deliver it near a window to avoid exposing the family to concentrated Tobi mist, and it's freezing! ;-) In trials Tobi is rated to be much more effective than Colymycin in blitzing pseudomonas which is one of the reasons I'm quite keen to get back on with this.

To Be Continued...

Ibiza In Pictures

2010-09-07T08:49:00.000-07:00

Chicken Curry

2010-08-27T04:27:00.000-07:00

Well, as it stands, I'm not sure whether we're gonna make it to Ibiza this weekend. Oscar has got chickenpox three days before the flight so we'll just have to see...anyway in the meantime this is my submission to CF Talk's food issue:

Woody’s Quick Chicken Curry Recipe (Serves 2)

Ingredients:

Chicken Legs/Thighs (4-6 pieces, leave on the bone)
Onion (chopped)
Garlic (1-4 cloves to taste, chopped)
Ginger (small piece, peeled and chopped)
Chillies (1-4 to taste, chopped)
Tin Chopped Tomatoes
Whole Garam Masala mixture, (ground with a pestle and mortar or spice grinder)
Vegetable Oil
Rice

Instructions:

1. Add the oil to a non stick pan or non stick casserole dish.
2. Add the chicken pieces and lightly fry them for 2-3 minutes until they are golden (no need to cook through)
3. Take them out and put them in a bowl for later
4. Add the onions, fry for 2-3 minutes until golden brown
5. Add the freshly ground garam masala. The frying pan should be hot enough to heat the spices through so that the lovely flavour really comes bursting out.
6. Add the garlic, ginger, chillies. fry for a minute
7. Add the chicken back in
8. Add the chopped tomatoes, add water to taste (the more water you add the longer it will take to reduce down to a thick sauce)
9. Bring to a boil, put the lid on, turn the heat down and then simmer slowly with the lid on for about 30 minutes.
10. You can check the chicken is cooked through by skewering it with a knife down to the bone; if the juice runs clear, you’re sorted.
11. For the rice, get one cup full of rice, two cups of water should do for 2 people.
12. Add to a saucepan, bring to a boil and then simmer for about 10 minutes when the rice should be ready. Stir occasionally to make sure it doesn’t stick.

This is my basic student curry recipe and it probably saved me from malnutrian when I was a student. I like chicken on the bone because it has more flavour and doesn’t dry out like chopped chicken can. You can adapt it in loads of ways; if you don’t want chicken on the bone? Chop it up. Want it with lamb or beef, fish or prawn? No problem, just remember lamb or beef may need longer to get tender, and don’t over cook the fish. If you want to add mushrooms and peppers, go for it. Want to add yoghurt or chopped coriander at the end? Simple, but turn the heat off and stir the yoghurt in really gently. You can buy ground Garam Masala from the shops but it loses it’s flavour really quickly so I would always recommend the whole spice version. You can pick up the whole Garam Masala mix from most supermarkets these days and all Asian grocers sell it.

Who ate all the pies?

2010-07-28T14:53:00.000-07:00

I had my best hospital visit for a while back on Wednesday. Weight is back up to 10 stone 5; so I've put on half a stone since losing load after Christmas. Hurrah for Scandishakes! With the help of a Scandishake with breakfast nearly every morning, my weight has slowly crept back up again. My blows have stayed pretty steady at 2.7/3.9, my flight SATS have somehow got better since my last test a few years ago. The only thing to watch is the aspergillus levels which have risen a bit, and the glucose tolerance test, which shows my blood sugar does dip quite low 2 hours after the test.

A bunch of us went camping last week near Ullswater. The kids coped really well, it helped that they had loads of kids their age to play with. The weather wasn't great but it didn't rain and you can't ask for more than that. The cheap supermarket BBQ meat was pretty gross though, it was so bad none of us could eat it and the dogs that went past in the morning steered well clear as well ;-)

Proud Brother

2010-07-23T06:18:00.000-07:00

typical, my sister inherits the long legs and I get the short and stumpy ones ;-)

Last weekend my sister was finally given her BSc Hons in Geography. Me and my mum went to the graduation ceremony with her boyfriend, then we all went out for a drink and some noodles to celebrate! Happy times, even if the ceremony was a bit boring (A massive procession of students being clapped, and J was right at the end...)

Current Eating/Treatment Regime

2010-07-21T07:03:00.000-07:00

PWCF need to eat a lot to stay at their current weight. I think I'm lucky in that I don't have to eat as much as some, but still, in the last 6 months, I've somehow lost about half a stone without my appetite dropping or without eating any less so I have had to add a scandishake to the mix. I had a meeting with the dietician the other week at my annual and she suggested adding another scandishake at the end of tbe day.

Food / Med Diary (Week)

6.30AM Get up
6.45 Azithromycin and Flucloxacillin
6.50 do Dnase on INeb
7.00 Breakfast Scandishake with full-fat milk and cereal
7.00 Vitamins, uniphyllin, calcium chew
7.45 Drive to work, drop off kid(s), do some physio in car, spit into empty creon pot in car. (Timing is everything in this ;-))
8.30 Arrive at work.
8.30 Do Colymycin Neb in car at work
10.30 Apple, banana
11.00 2 Pork Pies
1.00PM Lunch (Sandwich, Mayonaise, Meat Filling, Crisps, Tango or Tea)
3.00 Chocolate bar (note: Boost is 300 calories!)
5PM Leave work, pick up kids.
6PM Dinner with kids (have to play it safe so kids have something to eat so we normally have chicken, brocolli, peas and mash, or bangers brocolli and mash, or Lloyd Grossman's Tomatoe Chicken Balti and Rice, or Spagetti Bolognaise or Pasta and Roast vegetables which the kids will actually eat.
8PM Kids in bed. And relax. Either go for a run/bike ride or get the wine out, depending on how healthy/lazy I'm feeling.
9-10PM Colymycin Neb

What an exciting life I lead. Need to try and squeeze in another Scandishake at the end of the day but at the moment it's a struggle, my tum needs a rest ;-)

Tumbles day

2010-07-09T15:17:00.000-07:00

A tumbles day is a day to forget, and I've just had one. Yesterday I decided to work from home to make it easier to drop off Abby today and sods law; one of the big bosses decided to pay a visit to the office to see me, so I missed him. He also dropped off a request list that I should have had in May for a dealine at the end of the THIS month. Grump, grump, grump! ;-) And to top it off Abby threw a mega strop at the cricket club on the way back from the school summer fair because we wouldn't buy her any more sweets. Does anyone have a manual on how to be a firm parent? Because I seem to be made of mush when it comes to discipline.....anyway, I've got the wine out and I'm sulking. With three hours of Glastonbury I taped from last week.

Turkey Break in Pictures

2010-06-21T14:26:00.000-07:00

Lost: The Bad

2010-06-03T05:29:00.000-07:00

OK, We've had the good, now this is the bad (I still liked it though) :

1. Episodes where nothing happens in the plot and you get a boring flash back. The interminable episodes of Hurley's flashbacks (The Dharma VW van episode "Tricia Tanaka Is Dead") and virtually any episode with Charlie's band in the flashback. Fast forward material!

2. Jack's a bit boring. Maybe it's a flaw in the script, but his over-earnest acting started to get annoying by the third season. Particularly when compared to Sawyer; he never really changed throughout the series whereas Sawyer had definite character development.

3. Kate's a bit of a lightweight as well. OK, we get it, it's a love triangle! Just make your choice freckles, and get on with the plot ;-)

4. Locke vs Jack / Fate vs Free will / Man of science vs Man of Faith. Interesting at first, but became a bit tedious towards the end.

5. Llana, the french woman, Alex, Walt and all the other under-developed characters that the writers obviously planned to do something with but ran out of episodes to do it in so wrote them off! Naughty, lazy writing boys! ;-)

6. * New! Claire abandoning Aaron then disappearing and then reappearing as a mad women. I just never bought it. Also, Kate deciding to adopt Aaron for 3 years forming an emotional attachment and then abandoning him to go back to the island. Also, what happened to the two kids in the tail section? Kids in general were given a raw deal by the script writers

Lost: The Good

2010-06-02T04:08:00.000-07:00

So LOST is over, what do I watch now? Well before I watch anything else I need to find some closure, before I can properly move on ;-). I have decided that Lost is, was, and always will be 50% Brilliant and 50% Bollox. So with that I'm going to list the things I liked and disliked about the series.

The Good

1. Episodes like "The Constant". Desmond flips through time desperately searching for his Lost love Penny like "The Time Travellors Wife" and finally contacts her. One of the best episodes IMHO.

2. "The Looking Glass." All the season finale's were exciting, but this closer for Season Three was so good it actually gave me heart palpitations for a few hours after I saw it. Poor Charlie; I'd spent most of the time being annoyed by his Mancunian-druggy-rocker character, so it was a total surprise how gutted I was when he sacrificed himself for Desmond.

3. Sawyers nicknames/one liners. Son of a bitch ;-)

http://www.youtube.com/watch?v=8WurBi2yQeI

4. Hurley. Dude ;-)

http://www.youtube.com/watch?v=HOk9pIbM-T4

http://www.youtube.com/watch?v=eZErJDc6978

5. Sawyer grows up, becomes a leader after Jack and Locke disappear, and settles down with Juliet in the 70s. And why not? ;-)

6. They killed Juliet. You bastards!

7. The ambiguous mysteries of the island. The thing about mysteries is that not knowing what they are is always better than the explanation. Explanations are always a bit of a let-down.

Lost: spinoff series...?

2010-05-24T06:28:00.000-07:00

No, I haven't seen the final episodes yet, so I'll thank you not to post any spoilers please!

In the meantime, how about a spinoff series with Sawyer?
:-)

Manchester Run

2010-05-21T14:18:00.000-07:00

On the day, I woke up really early, I was a bit nervous. I kept waking up too early, then forcing myself to go back to sleep where I would wake up dreaming of running, or being late for the run. Needless to say, I ended up having a rubbish nights sleep and then had so many butterflies that I found it hard to eat anything in the morning. Oscar was still a bit poorly (he'd been sick the day before, once in his cot and once in the car. R's in-laws had very kindly offered to look after the kids while me and R took part in the 10K run. So once we'd packed them off we were all set.

We arrived in the car park super-early; last year we were in the last (pink) wave and Manchester seemed packed at the later time, this time with us being in the early (white) wave Manchester seemed eerily empty. We went down to the start, got some free isotonic drinks and waited. The bit before the start is a bit rubbish, you just want to turn up and run really and you always get too cold waiting around in your shorts before the run, and if you take a fleece or a coat or something then you will be lumbered with carrying it for the run, which will be a bit of a pain. What they are now doing is encouraging you to take your old jumble clothes to keep you warm before the race and discard them on the side of the street to be collected by the scouts for charity when the race starts. Which sounds like a good idea, if only we had known ;-)

Anyway, after huddling up with the other runners trying to get warm like penguins in the Antarctic, we were off. R soon peeled away from me; I think I stayed with her for the first 1KM, but my chest was giving me jip so I basically eased off and ran at my own pace, which generally involved running for 10 minutes, then getting out of breath and walking for a bit, then starting again. If only I could figure out a way to run without stopping, I'd be sorted! Anyway, before I knew it, I had gone past the first 5K; halfway, and then the real work starts! Unfortunately, my muscles started to ache, and I started to feel a bit low on energy...but I basically gritted my teeth and slogged my way to the finish, thankfully my chest had more or less been sorted itself out by then after the first half of coughing, so I could concentrate on trying to ignore my aching legs.

When I got to the final 500M, I actually really wanted to stop and take a breath before a sprint finish, but by that time there were crowds all around watching and cheering as the runners came to the finish, so I then pushed myself through a horrible bit where my lungs, heart and stomach felt like they were all thumping in unison, screaming for me to stop, and I wanted to puke! There was a guy with a mike on the finish line, I kept thinking "don't talk to me, I feel like I'm going to throw up...." Thankfully he ignored me. If it seems like I'm moaning, I'm not; It was a slog, but at the same time it was a great experience, and the crowds were wonderful, always doing their best to cheer us on...if my body holds up, I'll be back next year :-)

For the last week before the 10K I had been feeling a bit poo because a cold / sore throat I'd caught from Abby/Oscar had moved onto my chest and it was making me produce loads more mucus at night; this is always a big pointer for me when my LF starts to slip, when I produce so much mucus that it builds up during the night and interrupts my sleep by making me cough. When I'm on top of my health I don't produce enough in the night to wake me up. Anyway, I had kept up with a little bit of running just to keep me ticking over and I'd even cheated by injecting some left over Ceftazadine for a 4 day period, just to try and keep the mucus at bay really. Thankfully my chest seems to be a lot better since the run. Although at the outpatients on Wednesday my FEV1 was an average 2.675 / 3.8; so I am on 2 weeks oral antibiotics Ciprofloxacin and Augmentin, and we'll see how it goes.

Lakes Bike Trail In Pictures

2010-05-10T03:05:00.000-07:00

IV League

2010-04-22T06:42:00.000-07:00

FEV1 2.8 FVC 3.8 sputum from last month showed Staph and Pseudo. Has the Staph come back? I've not been keeping up with the Flucloxacillin, maybe I should start taking it at night again. Otherwise the visit at the end of the IVs was good enough, even if Doctor R did annoy me a bit with his questions. I also brought up the VX-770 trial, and he said I was giving him news. Which was a bit disappointing, shouldn't he be telling his patients the news? Anyway, hopefully we'll hear how the trial goes soon.

Friday Tune (Best of the last decade part 3)

2010-04-16T04:09:00.000-07:00

Sod it, it's Friday, it's just a nice tune :-)

Well I'm into my second week of IVs, lungs seem **mostly** clear, so I decided to celebrate it with a half hour run. I felt good on the run and even had some energy left at the end to do a sprint finish, which is unusual for me.

But, (big but) about halfway through the run I developed a rattling wheeze. I sounded like Darth Vader. It was a wheeze that I could feel more on the inhale breath than the exhale. I've had it for the last few days more so in the afternoon / evening. And no amount of huffing/coughing would shift it. I''m wondering if this is asthma related, (possibly ABPA, I've had it in the past) does asthma effect you more on the inhale or the exhale? Anyway, I rang the hospital and they're gonna do an X-Ray next week when I go back and also do Aspegillus blood test so we'll see what happens...

Rantline

2010-04-13T03:13:00.000-07:00

I haven't swore on this blog much, coz it's a limited form of communication, isn't it? Yesterday I felt like it but it would have just turned out like "the fucking fuckers fucked, it can get fucked for fucks sake" or something. And all I really have is questions with no answers. And I feel like I've been hit by a stealth CF infection with added wheeze factor. Last summer I was 10 stone 10, now I've dropped down to 9 stone 12 which has completely mystified me; I have no idea how this happened. Can a stealth pseudomonas infection really rob me of so many calories? The doctor reckons it could be but I remain baffled...On the plus side my stomach is really flat...;-)

The other day at hossy my blows were down (FEV1 2.4, FVC 3.8) my weight was down 9 Stone 12 and I was pissed off. Since the start of this year I've been doing everthing right, started Tobi nebs, done loads of exercise (3 hour bike rides through the hills, training for the Manchester 10K) and yet despite all these efforts the LF and Weight is at it's lowest since 2005. I wonder what the hell CF wants from me, I've played by the rules and I get my arse kicked ;-)

Anyway, I'm back on IVs (Cefttazadine and Tobramycin) and Scandishakes. The IVs give me the runs so I'm having Immodium to slow my guts down, which impacts on my appetite, combine this with 500 calorie scandishakes poured over my cereal and my guts are churning and gurgling like Johnny Vegas's private sess pit after he's had a night on the curry and beer. Meh!

Dorset Break in Pictures

2010-04-09T06:57:00.001-07:00

Timecapsule - spot the PWCF ;-)

2010-03-19T06:24:00.000-07:00

There is a person with CF on this picture. He/she is 9. It's 1981 or 1982. I think (memory isn't what it used to be). He/She has just learnt how to add fractions. There is no creon, only pancrex. No ciprofloxacin. No nebulised antibiotics. He/she has just been diagnosed, and is trying to get his/her head around the fact that he has to take enzymes with low fat food, skimmed milk(!), no chocolate(!), flucloxacillin and do physio with postural drainage....those were the days :-)

Fear, Loathing, and Physio Time (!)

2010-03-09T04:30:00.000-08:00

Went to the all-new outpatients to do the hypertonic saline trial with Leslie (the physio) yesterday. I went in my lunch hour hoping it would only take an hour to complete; unfortunately it turned into a bit of an epic visit and I wasn't back in work till 4! Oops. Anyway, since the last trial of Tobi I've been having some problems with my chest; nothing I can put my finger on but I've been producing more mucus on a daily basis, and I've had a growing wheeze which (handily) means the mucus is harder to bring up. This all started at Christmas when I asked on the CF Forum about what was good for bringing up mucus plugs and the majority answer came back as hypertonic saline. Hypertonic saline is a high salt (7%) solution that works (in theory) by drawing more water into your lungs by osmosis, so if you have hard dry plugs or sputum that is in general hard to bring up then it should moisten these and make them easier to bring up.

We did the FEV1 blows before the Hypertonic trial (FEV1 2.75, FVC 3.75) which was down but not too bad, considering. We then nebbed some (preventative) ventolin followed by the hypertonic saline. We then did some physio to clear out whatever the hypertonic brought up. I've got to say, I found this a bit awkward as I actually hadn't done physio with anyone at Manchester since my last inpatients stay (10+ years ago). This meant I had to get over my reluctance of spitting in front of strangers, but the physio techniques she did -all variations on the active breathing cycle that Mary taught me ages ago- were very effective in getting mucus out of the small airways and into the large airways where they can be huffed up. It was a good refresher course in physio techniques; breathing shallow and deep but holding back a forceful huff or cough -which closes the airways- as long as possible. Over the years I'd simplified the technique in my head into slow deep tummy breaths followed by an almighty forceful huff. What she did was more subtle with breathe and hold followed by gentle and then faster exhalations with vibes to try and ease out the mucus. All good stuff, and (hopefully) it's improved my technique quite a lot. Over the past few years I've had very few problems (touch wood) so I'd gotten a bit slack on the physio front. Now I'm determined, for the sake of my long term health, to be more regular with my physio. We'll see how long it lasts ;-)

After the trial my FEV1 blows came back 2.45 which was down 10%, this is classed as a significant drop so Leslie referred it to Jen/Roly, which turned the visit into a full on consultation and they decided to step back from the hypertonic trial until they could get to the bottom of what was causing the wheeze and the possible current infection. I also asked about trialling Spiriva (I'd never tried it), but Roly said it was throwing one too many ingredients into the mix, as they want to figure out what's going on first, so I'm on two weeks oral antibiotics (Cipro and Rifamplicin) before the next appointment, so fingers crossed it all calms down by then.

Washed out at the car wash

2010-02-23T05:32:00.000-08:00

I haven't blogged much about Abby and Oscar recently so here goes. Abby is now halfway through her first year at infants school and is doing really well. She is going for swimming lessons on Wednesdays (doing great) and ballet on Saturdays and she's turning into a very confident young lady (chatterbox!) of 4. Last week she won a spelling competition at school and won a prize so we were well chuffed.

Oscar we've been a bit worried about. Ever since he started nursery he seemed to catch every bug and cold that was going, in particular one weekend when he seemed to come down with Croup (barking cough sound, and seemed to have difficulty breathing) but in the end it wasn't too bad. I've mentioned in the past that it did cross my mind that he might have CF; when we did IVF we discovered my wife is a DF508 carrier and this led us to suspect the worst, however we contacted Leeds LGI and they said all donors are checked for CF so that would seem to make it highly unlikely. He also came back negative with the heel prick test. In any case in the last few weeks he does seem to have had a run of good health and this seems to have helped him gain some weight and he seems loads better in himself. His talking is coming on leaps and bounds as well, he is getting very good at mimiking and picking up some of the things we are saying to him. He can still destroy a room in 45 seconds as well!

I recently started a training blog It's completely no frills, it's just for me to provide a record of the exercise I've done and hopefully when I step it up and do more, I might see some improvement in my lung function. My misses bought me a mountain bike for my birthday, and I've recently been joining my mates on off-road courses. They all started early last year so their fitness level is a lot better than mine, then again I do wonder if my fitness level is where it is because of my CF related lung function (78%) . Uphill biking is very hard work. On my first ride my heart was beating so fast I thought I would collapse and I could see spots in front of my eyes. I was sweating like a stuffed pig as well (attractive ;-)) But I am starting to feel a tiny little bit fitter now (on my fourth ride) so hopefully there will be some long term fitness benefits. It's good fun going downhill as well ;-)

Some pics of me and the kids washing the car. Abby wanted to do most of the hosing but Oscar was interested too. When Abby wasn't looking he managed to grab the hosepipe and soak Abby from head to toe leading to a change of clothing halfway through the car wash. Very funny but it meant the car wash took ages as Abby insisted on eveything stopping until she got changed. A true diva in the making. :-)

Utopia

2010-02-21T03:07:00.000-08:00

How fucking good is this? What a singer, sends shivers down my spine.

The real Lady G...

2010-02-19T02:58:00.000-08:00

One of my favourite bands Goldfrapp is bringing out a new album very soon! I'm very excited! I want to post the video for the song "Rocket" (like everything at the moment it's a bit of an 80s pastiche, but a fanastic tune) but it's not on YouTube yet, so I'm going to have to paste this instead...

The Sundays

2010-02-15T07:56:00.000-08:00

I had such a crush on the Sundays' singer when I was a student, I loved her voice. Further reading (and an excellent article) here. A very british band.

The New Outpatients

2010-02-09T01:57:00.000-08:00

First visit to the all new outpatients yesterday. This was for a Tobi trial with the Ineb. They have put the outpatients in the old Pearce Ward, so the outpatient rooms are the old in patient rooms, which is a bit odd, kind of brings the memories flooding back. The last time I was in there there was a communal kitchen / social room where you could hang out, watch videos and fix yourself breakfast (this was about 10 years ago before the cross-infection rules kicked in for everybody, not just Cepecia patients)

The Tobi trial went OK, I got a new lilac coloured filter with a bigger container for doing the Tobi nebs. Nice. Unfortunately, the Tobi takes about 4 minutes to dispense, and you have to do 2 lots of it, which is a bit rubbish because I can neb Colymycin and Ventolin in 50 seconds flat. DNase takes about 2 minutes. Apparently it's because the Tobi is a lot thicker.

The blows before the Tobi test were a disappointing FEV1 2.7 FVC 3.6. The blows are in that area where they're not that bad but not that good; so it leaves me in a bit of a no man's land. If they were down a lot (say 2.3-2.4) I would go straight onto IVs, and if they were up high (3.0-3.1) then I would be happy as a butchers dog. Meh. When I do the spirometry test I get a high pitched wheeze so I think the 10% loss of FEV1 over the last few months may be down to that, because my sputum seems to be completely under control with nebbed Colymycin and now Tobi. In fact the last time I had IVs I didn't *really* need them I just had them to see if they would improve my FEV1, which they didn't have a massive effect on.

Anyway, I asked the physio about trialing Hypertonic Saline, as I want to see if this can reach the parts that other nebulised solutions can't ;-) Roll on next month...

4, 8, 15, 16, 23, 42...

2010-01-27T01:43:00.000-08:00

The final season of Lost is just around the corner. I'm so excited! Well almost...I must be the only person still watching this! Hopefully this time we'll get some real answers as to what the hell's going on as well ;-)

Questions:

Who is Locke?
Who is Jacob?
Who is Richard Alpert and why does he never age?
What is the black smoke?
What happened to Claire?
Why is Jack's dead father on the Island and is he really dead?
Why spend 4 seasons trying to get off the island and then spend the fifth season getting back on?
Could the people in the past (70s!) change the future or has it already happened?
Does free will exist?
If I can't exercise my free will not to watch the final season does that prove free will doesn't exist?
Is Juliet hotter than Kate?

Last night I went for my first jog of the year. Just went for a run around the estate for 10 minutes after R had done her run. Didn't feel too bad, although the cold night air made my lungs burn. I made sure I took my seretide, ventolin, uniphyllin so that my lungs didn't seize up. Oscar has come down with yet another cold, he's not really full of it in his nose and throat, he's quite hot and he doesn't seem quite himself.

Last of the snow in pictures

2010-01-25T08:04:00.000-08:00

Some pics of going sledging in the snow last week before it all melted. We went to see some friends up in Ramsbottom (in the hills) in North Manchester and it was freezing cold ;-)

2009: Stop me if you've heard this one before...

2010-01-22T05:28:00.000-08:00

Bluffin' with her muffin? You'd have to have been deep in the amazon jungle to have escaped this song, it was everywhere in 2009. The greatest pop song of 2009? Up for debate obviously.

The thing with Lady Gaga now is that it is becoming impossible to talk about her songs without talking about her image in the media, and she undoubtedly makes good copy and sells plenty of papers. She probably can talk art/fashion bollocks, she can talk about Bowie, Madonna, Warhol and pop-art till the cows come home. Is she faking? Is it all a mask? Who cares? IMHO None of that really matters. All that matters is writing catchy pop tunes that people like to buy and maybe listen to with the volume turned up before they go out on a Friday night. And you can't really fake that.

p.s. I'm a 38 year old dad, surely I'm too old to like Lady Gaga? I'm going to be such an embarrassing dad ;-)

2009: Best songs last year, part 1...

2010-01-19T04:29:00.000-08:00

Well, as usual it's taken me a while to get around to listening to Florence and the Machine; but I like this album; there's some great tracks and there are signs she could go on to greater things. I like the way she fuses parts of dance and indie rock. She's definitely one of the more interesting female artists around at the moment. She's not my favourite female artist though, PJ Harvey is still in the lead in that race, with Goldfrapp coming up fast on the outside...now, if only I could get Abby into her, or Lady Gaga for that matter (I bought Abby Lady Gaga's album for Christmas but she was totally unimpressed, she prefers Disney musicals ;))

Gene Therapy Trials

2010-01-15T04:33:00.000-08:00

Been thinking a lot about Jess today, and the truth is, I can't really comprehend what CF is like for someone like Jess because CF is a completely different beast for me; it doesn't really impact on me that much. Sure, I take the same tablets, nebulisers, IVs, and do a small amount of physio and exercise... but CF doesn't really restrict me in the way that it does someone like Jess. While I was getting drunk, popping god knows what, and generally having a carefree time at university, Jess was on the transplant list and her life was very much on hold. It's taken me a long time to actually connect with the CF community, I think a lot of this is down to fear of what happens to others happening to me, I've been dimly aware of the more severe aspects of CF though my two stays in hospital when I was in my twenties; I met other people with CF who were having a much worse time of it than me and to be honest it scared the hell out of me; maybe because of that I never really looked at it in focus, I always kind of kept it at arms length. It's only in the last couple of years that I've begun to find some kind of acceptance and begun to seek more knowledge about CF and what it is, and how it affects others as well as myself. And what have I discovered? Well, it's tough, and the people that have CF are bloody tough. And an inspiration. RIP Jess, you are braver than I will ever be.

On a more positive note -because hopefully Gene Therapy can one day prevent the severe lung damage like what Jess has suffered- this VX-770 trial that I've just missed out on has opened up something of a rabbit hole for me which is going to take a while for me to sift through and figure out. I've just had a quick scoot around to see what's happening with gene therapy trials. I still don't understand how the CFTR works, but when I do I'll let you know...

VX-770
VX-770 is a drug being tested by Vertex Pharmaceuticals in people with cystic fibrosis who have at least one copy of the G551D mutation. The drug may actually be able to target the defect in the CFTR gene and restore its ability to open up chloride channels, thus allowing salt to flow in and out of the cells properly. Unlike gene therapy, VX-770 would not replace the defective gene. Rather, if successful, VX-770 would repair the problem in the existing gene. Patients who took the drug showed significant improvement in several key CF measures, including lung function, nasal potential difference measurements, and sweat chloride levels. The findings suggest that VX-770 improves function of the faulty CFTR protein. This is the first time that any potential therapy has improved the abnormal sweat chloride (salt) levels in a person with CF. Excessive sweat chloride is a key clinical indicator of cystic fibrosis.

VX-809
VX-809 is another drug being tested by Vertex Pharmaceuticals in people who have two copies of the ∆F508-CFTR mutation. It is similar to VX-770 in that it may be able to get salt flowing through the cells properly, but it works a little differently. If it works as researchers hope it will, VX-809 would open chloride channels by moving the CFTR protein to its proper place on the airway cell membrane.

Miglustat
Miglustat is a drug manufactured by Actelion Pharmaceuticals that is already in use to treat other conditions, but it is currently being studied for use in people with cystic fibrosis who have two copies of the ∆F508-CFTR mutation. The study is small scale -- consisting of only 15 participants -- but so far the results have been promising as Migulstat has been able to reverse the CFTR defect and restore normal activity to cells.

Ataluren
Ataluren, which was once called PTC124, is being studied by PTC Therapeutics as a possible cure for people with CF who have nonsense mutations. In nonsense mutations, a piece of “gibberish” code appears amidst the normal code in the CFTR gene. The nonsense code acts like a stop sign, preventing the cells from reading any code that occurs after it. Ataluren may be able to correct that problem by helping the cells to ignore the stop sign and keep reading the code that occurs after it, thus restoring normal function to the cells.

http://cysticfibrosis.about.com/od/cysticfibrosis101/f/cure.htm

Doh!

2010-01-12T02:50:00.001-08:00

Just missed out on the VX-770 trial. I asked my CF Team what my genotype was in September. Unfortunately it was buried in my notes somewhere so the team had to get a third party Gopher to find it from my notes. I got the letter back on Christmas Eve, probably missed out on the trial by a matter of days. C'est la vie...

From: J
Sent: 11 January 2010 15:00
To: DJC
Subject: VX-770 Trial

Hello ,

I am a 38 year old male with cystic fibrosis. I have recently learned that my CF Genotype’s are G551D / N1303K. I have been told that I may be eligible for the VX-770 medical trial. Would it be possible for you to give me any further details?

Kind Regards, J

From: DJC
Sent: 11 January 2010 17:22
To: J
Subject: RE: VX-770 Trial

Dear J

I am really sorry to disappoint you, but recruitment to the trial you mention closed just before Christmas as the drug company had exceded the numbers they needed.

On a brighter note, they are planning an interim analysis after everyone has completed 6 months of treatment, ie. early summer, and if things are looking good, they will be applying for a license to allow your clinical team to prescribe this agent. It's difficult to predict how long this will all take, but they will certainly be trying to get it through as fast as they can.

I'm sorry not to have more positive news for you personally

Best wishes, J

I'm not too disappointed by this really; in the long term this is good for the CF Community because we are actually getting to the stage where we may be able to take a gene-correcting drug that may stop things like pseudomonas getting established in our lungs, it may stop our pancreases becoming progressively more damaged, and it may prevent us developing diabetes. Fingers crossed!

Further reading here on the cystic fibrosis transmembrane conductance regulator (CFTR), and on G551D and N1303K. If anyone can explain to me in more detail how the CFTR works then please let me know ;-)

The boys from the NYPD choir were singing Galway Bay...

2009-12-24T15:15:00.000-08:00

It could only be this really, couldn't it? Best Christmas song of all time?

Happy Christmas to all and to all a good night!

Christmas Countdown: Maybe this Christmas

2009-12-24T06:12:00.000-08:00

Just heard this recenty on Radio 2. A beautiful song. Most famously used (I think) in montages on The OC and Gossip Girl. Joe should have covered this for his X-Factor single; he probably would have got a number one instead of Rage Against The Machine, although I shudder to think what Simon Cowell would have done to the song (Key Change, Choir, etc) ;-)

Christmas Countdown: 2000 miles

2009-12-23T06:07:00.000-08:00

I love Chrissie Hynde's voice, it's so warm and soothing, like a glass of mulled wine at Christmas...

Countdown to Christmas: Donner and Blitzen

2009-12-22T12:09:00.000-08:00

I'm going to post some of my favourite Christmas songs in the build up to Christmas! Starting with this from Manchester's own Badly Drawn Boy:

Snow and the last outpatients

2009-12-21T15:53:00.000-08:00

Went to the hossy last week. It was mainly for a checkup with the phsio about how I had gone on with the Tobi trial last month. To my grumpy annoyance the blows were the same (FEV1 2.85 / 3.8) but she said that was a good thing; the important thing in the first month of the Toni test is to make sure that the volume does not fall, as that would indicate some kind of allergic reaction (to the Tobi) was going on. She did notice I had a wheeze at the end of the spirometry blow, so we discussed me going back on Uniphyllin to help combat it. So anyway the plan is next month to ditch the crappy old Pari Neb and start on the Tobi trial through the iNeb next month. (on a one month on, one month off basis) By then the outpatients will have moved from the Heart area to the new outpatients above the new super Pearce ward. (where it is alleged you can watch Sky Sports and get foot massages...I should be so lucky ;-))
I've also got the notes back from the doctors telling me what my Genotype is: G551D/N1303K.

Other news, Oscar has been really poorly for the last week with a D&V tummy bug, which promptly spread to R, her in-laws, and finally, me, despite washing my hands continuously and rubbing with alchogel afterwards (I now have the hands of a 100 year old man!). Being ill I've not really eaten for 4 days and I feel like that skinny guy out of Twilight (only not as tall)

Anyway, on Sunday it properly snowed in Stockport for the first time in years, so we took Abbby sledging down the golf club, and she loved it. Later on though we nearly got snowed in at the in-laws house when the roads froze and all the cars going through the village couldn't get up the hill to get out. For a while it was like vehicle civilisation had ended and we were back to doing everything on foot! Me and R were serious about settling the kids down at the in-laws and doing a 6 mile snow hike back home in the dark to get supplies and then walk back in the morning, but by the time we went back down to the village, the temperature had risen slightly and the snow was beginning to melt. I was slightly disappointed, but only slightly, because my legs were already beginning to stiffen up after walking for 20 minutes in the snow, so god knows what they would have been like after 2 hours plus. Would have been romantic though, it's great to be outside at night in the snow, everything is so quiet because the snow absorbes all the sounds, and covers all the hard edges, I just love it.

Anyway, everyone seems to be on the mend, so bring on Christmas!

Oscar's imaginary CF!

2009-12-04T03:09:00.000-08:00

I really don't think our (18 month old) Oscar has got CF, btw, but some of the similarities are a bit disconcerting:

Chest infections. Oscar was on ventolin and (banana flavoured) Amoxicillin after a virus he picked up last week moved onto his chest and gave him a chest infection. By yesterday his breathing was really-really wheezy, laboured and clicky, you could tell there was loads of gunk down there, and he just wasn't himself, he would play for a minute or two, but then he would roll around on the floor screaming until he was picked up. (carrying a 21 pound toddler around all day is a bit of a strain on the back!) I phoned the emergency doctors and the nurse on the phone who filters the calls before giving you an emergency appointment was a real bitch; I tried to explain to her what the situation was but she kept interrupting and cross examining me like I was in court or something, I felt like saying "look I have CF and I know what a chest infection sounds like, if my chest was as bad as Oscars I would be checking myself into hospital for some IVs!" Anyway, we eventually got referred to a doctor who agreed he had the startings of a chest infection, we then had to dash across town to get to a 24 hour pharmacy in Fallowfield. We then gave him the ventolin and amoxicillin (with a baby spacer to breath the ventolin in with) and in the morning he seemed to be a lot better.
Constipation / blockages. Oscar was on lactulose for being blocked up, but then he seemed to get things under control. Then after another week or two he went and got really blocked again so he was put on Movicol, twice a day, decreasing to once a day when his bowels settled down.

Here's some pictures of me and Oscar clowning around in the supermarket on the day he had his chest infection. He was fine in the morning while we were shopping then he went downhill really fast in the afternoon. Then he was much better in the morning. Kids eh?

30 hours in Paris

2009-11-18T08:44:00.000-08:00

Urgh, this blog is a bit out of date, I clean forgot to post this blog last month about our short trip to Disneyland Paris with Abby and Oscar.

Last (Halloween) weekend we went for a very short trip to Paris staying at Disneyland Paris. We caught the FlyBe from Manchester in the early Friday afternoon to Paris Charles De Gaul. We then got a bus then a train to straight to the Eiffel Tower with kids in tow. Abby was quite keen to climb the tower, especially when she saw it all lit up at night. Problem was, it was much busier than we anticipated, so we then had to pursued her that it was much better to head to the Hotel in Disney Village.

Anyway we arrived late at night at the hotel and everyone went straight to bed.

Saturday: we then spent our one full day in Disneyland. It had a Disney Halloween pumpkin theme with lots of men dressed up as pumpkin men. Abby was very excited by all things Disney-princess-like, so we basically spent the whole day in the fantasia (Disney castle) part of Disneyland. Halfway through the day Abby got exhausted so we went back to the hotel for a rest before heading back for the Disney Halloween parade. Abby was very scared of the Halloween monsters and kept hiding behind me when they tried to talk to her ;-)

Sunday, we caught a taxi back to the airport and were back in Manchester late Sunday morning. Maybe we should have stayed longer but Abby may have found it too exhausting!

my top 10 albums of the decade...

2009-11-17T06:21:00.000-08:00

Is it really the end of the decade? It went so fast! Anyway, In no particular order:

(Edit - I've added some explanantions. Sorry for the pretensiousness ;-)

PJ Harvey – Stories from the City, Stories from the Sea
I am a massive PJ Harvey fan, and am completely incapabale of objectivity where she is concerned, but this is the fabulously positive album that all non-PJ fans should own.

Radiohead – In Rainbows
Their best album since the era defining albums of "The Bends" and "OK Computer" fromthe previous decade. For some reason this fact seemed to get lost amid the hype of the way it was sold. I really need to buy this album proper because owning the mp3s isn't really enough for me!

The Strokes – Is this It?
Quite simply redefined indie guitar rock for the rest of the decade. The deceptively simple blend of 2 guitars and bass blew everything else away even if it did sound a bit like Blondie and Television from the late seventies. And "Last Night" was *the* song of the summer of 2001.

The Killers – Hot Fuss
Just a really exciting indie / pop album from 2004-2005.

Yeah Yeah Yeahs – Fever to Tell
The best of the second wave of NY bands that appeared after the Strokes. And Karen O is hot as well ;-)

Elbow – The Seldom Seen Kid
Mancunian local boys finally come good after years of being second best. "One day like this" is one of the most positive songs ever.

The White Stripes - Elephant
Proof that if you have some decent songs then you don't really need anything other than a guitar, a voice and some drums.

Grandaddy – The Sophtware Slump
A great album with some heartbreakingly beautiful songs about the breakdown when technology meets nature. Could anybody else write a song about an alchoholic android (Ged!) and make it sound so sincere and tragic?

The Streets – A Grand don’t come for free
A rap concept album. Wtf!? But when it sounds this good...

Amy Winehouse – Back to Black
Such a shame that Amy Winehouse is now more famous now for being in the tabloids than for being a great singer songwriter. Check out her debut album "Frank" as well, there's some great songs on that as well.

Honourable mention:

Badly Drawn Boy - Hour of the Bewilderbeat
The hype around the Twisted Nerve record label and BDB reached such a fever pitch amongst the lo-fi cool kids (I wasn't one ;-)) in Manchester around 1998-1999, so much so that when this finally arrived in 2000, it seemed a bit of a dissappointment. In hindsight, I was wrong; it's a wonderful record. Such a shame his career hit the skids so soon afterwards.

I need this guy to be my personal trainer!

2009-11-07T06:51:00.000-08:00

Tobi, Tobi, Tobi, Tobi!

2009-11-04T01:00:00.000-08:00

Went to the clinic the other day for my nebulised Tobi trial with the physiotherapist. The trial is to take nebulised tobramycin for a month and see how things go, and hopefully see some LF improvement over the next month as my blows have been a bit rubbish recently. We did the test and she also rejigged my iNeb with her laptop from tidal flow to TIM (Target inhalation mode) this is better because it quarters the time needed to do the inhalation, and it turns out I will need this extra time with all the extra stuff I'll be inhaling next month. She asked me about my iNeb compliance before she brought up a flow chart telling her exactly how compliant I had been with my nebs over the last 12 months! It turns out my compliance was only 70%, I was well embarrassed, I felt like a naughty schoolboy!

I had heard that Tobi was inhaled once a day, but for the monthly trial I will be nebbing twice daily with an old compressor neb (like I used back in the 80s! Doh!) You take it twice a day and also you need to nebulise Ventolin before it to stop the possible bronchiectasis. that'll be fine in the evening when the kids are in bed, but I will struggle to fit that into my morning schedule, which consists of getting showered, doing a bit of physio (in the shower), getting breakfast, getting Oscar and Abby dressed and fed, and dropping either Oscar or Abby off at nursery / school before work. I think I'm going to have to use the iNeb for Dnase in the car on the way to work, which I don't really like doing as it's not safe and I could get pulled over. The physio also said I should neb Tobi in a ventilated room away from the kids. Hum. Looks like I'm getting up even earlier in the morning...

Anyway enough moaning! I actually felt quite loose in the chest after the trial tobi neb so hopefully I will see some real benefit when the month is over.

Boston in the Fall

2009-10-23T01:58:00.000-07:00

Well, me and R finally decided to go to Boston for a long weekend to celebrate our tenth wedding anniversary. The in-laws offered to look after Abby and Oscar for the weekend so we were all set. We took the afternoon flight to Boston via Heathrow, during the main flight there was a choice of films to watch, and because we don't usually get the chance to watch any films during the flight (because we're usually trying to keep the kids entertained) we both crammed as much as we could into the flight!) I watched Moon, The Last King Of Scotland and Che part 2. Of the 3 I'd recommend Moon if you like intelligent sci-fi, and The Last King of Scotland if you like to be terrified...Che part 2 was a bit boring for me; well acted but devoid of tension because I knew the ending. I was also left not really knowing who Che Guevara really was...
We arrived in Boston late on the Friday and caught a cab straight to the hotel where some champagne was waiting for us in the room, we then stumbled around looking for a restaurant (I wanted to try an Afghan restaurant around the corner but R fancied an Italian so we eventually found one inside another hotel after going round in circles for what seemed like a long time ;-)) and then we were truly knackered so we went straight to bed.

In the morning the view from the hotel looked a bit too much like Manchester; cloudy, grey and raining but we decided to make the most of it, went to the shopping centre next door to buy a couple of rain coats for the baseball and then set off on the freedom trail. The freedom trail is a redbrick trail that starts off in Boston common and takes in loads of historic sites relating to the American revolution and the historic break from Britain, and the shaping of the American government and the constitution and bill of rights. It was interesting but I felt a bit funny as a British tourist reading about the British army being vilified and the British parliament hated for their taxes! (I think the paper tax, a tax on all forms of paper was the last straw!) In the guide book the walk was supposed to take 90 minutes, but when we did it it took us most of the day, we did stop for lunch and go into some of the state museums though. Beware when ordering lunch in diners/restaurants; sometimes the portions are huge! Me and R ordered a couple of basic pasta dishes from an Italian diner and the plates that came with the meal were enough to feed a family of four; we would have been better off ordering one meal between the the two of us! It is true what they say about America, if you are there for a long period you would easily put on at least a stone...
When we got back to the hotel it was time for a quick shower before going to see the Boston Red Sox on the Metro; R had always wanted to go to an American baseball match since I can remember. The match was good, The away side (The Cleveland Indians) started off well but the Red Sox seemed to have a bit more quality and after a massive score with a home run with 3 other players on the bases in the 3rd period they always looked the likelier winner (if that makes sense to anyone let me know, I'm not sure I understood it ;-)) Baseball is much more of a social affair than the football we have here, there's lots of chatting through the match and a constant procession of boys carrying food above there heads shouting "Clam Chowder! I got Clam Chowder Right Here" and "Hot Dogs! Get your hot dogs here" So you can scoff your face through the match without having to get up! I've got to say the local clam chowder is worth trying especially if you need warming up on a cold night!
On the Sunday thankfully the weather was brightening up. We decided to book the whale watching at Boston harbour; it's a three hour round trip and apparently it can get a bit hairy if the sea is rough. Thankfully for us it was a beautiful day and there were plenty of humpback whales around. They are quite breathtaking to see close up. When they surface they do a really loud exhale creating loads of steam -they exhale something like 90% of the air from their lungs so they can take a deep breath before diving back down- humans exhale 25% to give you an idea of the super-FEV1 that a humpback whale must have ;-)
On the way back we had a wander though the City, I was going to buy R something nice from Tiffany's but we didn't find anything she really liked so we decided to console ourselves in the Cheers bar. As you might expect it is a bit touristy in there. On the last (half) day we got a quick coffee and bagel (or cwoffee and baygel as they say in Boston) and had a wander around Harvard trying to look intelligent (!) before a last meal in the Cheesecake factory (desert is literally 100s of different flavours of cheesecake) and then home. I've got to say I really like Boston, there's less to do than in New York, but the people seem friendlier, and there's more of an old colonial/British-Irish feel that made me feel really at home. We missed the kids terribly though!

Everyone's poorly except me and my monkey

2009-10-15T03:36:00.000-07:00

Everyone's poorly except me :-). Abby's got a stonker of a cold and has forgotten/is refusing to poo, this is giving her really bad stomach ache so we've put her on high fibre and Lactulose. We went to Blackpool to show her the illuminations last weekend but had to drive home early because she was in so much pain from needing to poo but refusing. I think it may be psychological as she's just started school and maybe it's all a bit intense for her; it's not like nursery where if she doesn't feel great/is tired she can take herself off for a snooze/some quiet time on the bean bags. Also, she's gone from being a big fish in a small pond to being a tiddler in a massive shouty lake! Oscar has just vomited the entire contents of his morning bottle of milk all over R, and he's had to take the day off nursery because he's just not himself. R went to the MRI to find out about her protein C deficiancy, they didn't really tell her anything she didn't know, except she should have a dose of heparin as well as wear the surgical stockings before long hall flights (oops! We just got back from Boston the other week!) As for me; well, I've discovered I'm allergic to Septrin (nasty rash on my ankles which seemed to be spreading up my legs, thankfully it stopped when I stopped taking the septrin.)

Anyway, I need to blog more but I'm too busy at work! Here's some 70s psychedelia from the Flaming Lips, haven't got any of their albums but I may buy this new one. I love the bassline!

Don't be concerned...

2009-09-30T03:05:00.000-07:00

If this song were a car it would be a convertible 1957 Thunderbird driving the ocean drive along the West Coast of America from Los Angeles to San Francisco. Ah, the optimism of the sixties. I love it!

Four Sleeps 'Til Boston...

2009-09-28T04:14:00.000-07:00

Only four sleeps till me and the misses go for a long weekend in Boston for our tenth anniversary! Boston in the fall. I'm torn; I'm excited, but it's the first time we've been away from the kids for longer than a night, so we'll miss them big time. It'll be strange being husband and wife rather that mum and dad for a few days. What the hell will we talk about? ;-)

We've been "mum" and "dad" for so long I've forgotten what we used to talk about before the rugrats arrived. Mealtimes will be strange as well without having to figure out what Abby will eat on the menu and quickly then wolf down our food before Oscar gets bored and attempts to swallow dive off the top of his high chair...:-)

Demolition Man

2009-09-17T01:32:00.000-07:00

Oscar is at that age (14 months) where he can trash a room in 1 minute if he's left alone. In the kitchen he pulls out all the pots and pans, all the cereal boxes (including once, when he covered himself in Ready Brek) and all the washing up powder. In Abby's room he pulls out all her clothes and all her toys. All good fun ;-)

Outpatients and the sun

2009-09-10T03:00:00.000-07:00

Hospital appointment yesterday; and for the first time in many weeks the sun came out! On my first spirometry I blew so hard that everything went black for a second and I got a massive headache ;-). On the second blow I hit FEV1 2.82 FVC 3.8. Hum. Blows were again a bit disappointing but I'm not producing much, I just feel slightly wheezy. It's now been a while since I broke the magic 3.0 level. To be fair Doctor J.H. noticed it was down for me and actually looked up the percentage (a first as they don't normally do that in Manchester CFU, except for the annual) and I had it confirmed as 78% -my score for last years annual was 83% so some way down really. In mitigation I have just come off the back of a cold, but my chest does seem to have cleared so I don't know why really. I asked the Doc about whether my pseudomonas had any resistance to Colymycin, but my last lab tests had all came back with no resistance (to Colymycin or Tobramycin) She did mention Tobi nebs and how they could be more effective (standard practise is to do one month of Tobi, one month of Colymycin; the downside is that there is a slight risk of hearing loss and there is more of a chance of pseudomonas developing resistance to Tobi than to Colymycin; although she said it was quite rare and she could "count on one hand" the number of patients whose bugs had developed resistance to Tobi. Anyway, I'm down for a Tobi trial with the physio when I go back in a months time, probably using the INeb with the new tidal flow (she said it can take 30 minutes to dispense if you use the old way! Nooooo!). I've also been put on two weeks of oral Septrin (Co-Trixomazole) and Rifampicin to see if it can clear anything left on my chest from the cold. I was warned that Septrin can cause a skin rash, but so far so good. (Last night I had a dream that I developed a rash all over my arms, but when I woke up there was nothing there. I hate having "obvious" dreams ;-))

I also asked what my two CF gene's were (they couldn't find it in my notes; they'll get it for next time) and what my CRP level was, apparently the last one was 5, and it was 6 when I had my last set of IVs in summer, although it can get as high as 40 when I have a really bad cold and chest infection. Otherwise I'm going to make an effort to get out and get some exercise on the unused mountain bike that's been in my shed/garage for the last ten years; this summer some of my mates have turned into mountain bike bores; seriously, the last time we went out for a drink it was *all* they talked about, I was not impressed! But I guess if you can't beat em you may as well join them, I'm not going to talk about bike magazines in the pub though ;-)

history

2009-09-09T06:13:00.000-07:00

I've been meaning to post something like this for a while...Kudos to Jo for reminding me.

I was diagnosed when I was 9 thanks mainly to the persistence of my mum and to the doctor who finally decided not to fob us off and referred me for an x-ray. From the x-ray they thought I had TB so they put me in an isolation ward for a month, when the TB test came back negative they did the allergy scratch test. Finally they did the sweat test, and on the second sweat test I was diagnosed. Looking back all the signs were there; every winter I would pick up a cold and it would stay on my chest for weeks or months, I would produce sputum by the bucketload. From the age of 7 I started to lose weight noticeably. When I was a toddler my mum frequently complained to the docs about my farts smelling wrong and I used to get the runs from time to time, presumably because of the amount of undigested fat in my system.

This was back in the 70s, and in those days no one had heard of CF, The yellow pancease tablets (Pancreatin? I honestly can't remember the product name) were rubbish, and the wisdom then was to go on a low fat diet (skimmed milk and no chocolate, is it any wonder I rebelled? ;-) I was also put on regular Flucloxacillin, which I more or less take to this day.

I think back in those days I had mainly Staphloccocus on my lungs. It wasn't until about 12-13 that I remember the word Pseudomonas being used regularly by the docs. I generally went into hospital for IVs about 3-4 times a year. The doctor would first give me oral antibiotics but when they invariably didn't work I was put on IVs through a cannula (the cause of many a trauma, although thankfully they always did the job in clearing my lungs) As a kid I can remember thinking/feeling that my health was sliding, but halfway through secondary school my health seemed to stabilise, I think this was down to a combination of Creon becoming available -which helped my weight gain, coupled with teenage testosterone kicking in which helped me with exercise (I should probably give the hospital credit here for the IV courses as well, although they weren't appreciated at the time as you can probably imagine). I seemed to get better at sports, took part in the 10K a couple of years running. When I was 16 the doctor let my mum do IVs at home. I have always done IVs at home since then. When I was 18 I was referred to the fledgling Manchester CF Unit, then in Monsall Hospital.

When I was 25 I got hospitalised a couple of times with major DIOS gastric blockages. The second time was quite scary as I was in for a month and it didn't seem to clear and surgery was mentioned. Thankfully it eventually cleared. The DIOS was caused partly because my pancreatic function was declining and had finally reached the point where Creon 10 was no longer working properly, I switched to Creon 25 and things gradually got better. I also started taking salt tablets, especially in the hot weather. Over the years I've become quite good at spotting the early signs (stomach pains) of DIOS and hit it with the Kleanprep early doors.

And that's it really. I'm now 37. I still have IVs about 3 times a year. I got married when I was 27 to a wonderful woman. We tried IVF for 5 years but it wasn't to be, so we decided to go through the donor insemination route and now have two beautiful kids, 1 and 4. Luckily my LF has stayed pretty stable throughout (it's about 80% atm). DNase made a big difference to me day to day in reducing the sheer volume of mucus produced, I also do Colymycin twice daily. I have no idea how I would do nebs without the Ineb, as it allows me to run after toddlers while inhaling. I think my health has been more stable when I have more weight on; I look back at some of the photos when I was a student and I look scarily thin, I feel like going up to my skinny-ass younger self and saying "eat some pork pies."

Cork and Kerry Mountains

2009-09-07T07:15:00.000-07:00

My mum and my sister have travelled the length and breadth of Ireland to build a picture of our family tree (On my mum's side, both grandparents were from Ireland). Apparently they've found quite a few relatives, I can't wait to hear the tales. Anyway, this gives me an excuse to post this song, which I love, I don't know why :-)

perspective

2009-08-25T06:26:00.000-07:00

I was feeling a bit rough over the weekend, but I'm now much better. Unfortunately, the rest of my family seems to be falling apart instead. Following her pulmonary embolism, R has been diagnosed with Protein C deficiency, this means she is prone to blood clots and needs to be careful with long haul flights, and avoid taking the contraceptive pill amongst other things. Fortunately, since she met me this hasn't been a problem (due to my tubes being blocked we don't need to use contraception) so I guess she's lucky she married me ;-). This has also bumped up our travel insurance for our proposed tenth wedding anniversary trip, much more than my CF does, who'd have thought?

Oscar is struggling to poo. For the last few weeks he has been painfully straining throughout the day, we'll check his nappy and find nothing in there, except the occasional pea-sized rock-hard stool. It sounds funny but the pain is sometimes so bad that he howls in pain, it's not fun to watch. We finally took him to the doctors last night and he has been put on lactulose.

On top of this my dad is getting quite poorly. He has a genetic muscular disease called Myotonic Dystrophy (MD). It's not very well known, the symptoms are similar to muscular dystrophy but they tend to present themselves at different stages of someones life, in my dad's case he didn't really have any symptoms and wasn't diagnosed until the age of 50. He's now 64. Because of this he does have weaker arms and legs, but he has been managing to get about his bungalow OK and he has been managing all the problems admirably well. The big problem for him at the moment is that the muscles in his throat are getting weaker so it getting harder for him to chew and swallow food, which is why he's turning to liquid foods more often. It also seems to be slowing his digestion down. He is now on supplements to stop him losing weight (Fortisip, BuildUp, Scandishake, all the things familiar to PWCF!) and he is
also taking lactulose to help move his digestion along (again, familiar to PWCF although I've never actually been prescibed it myself) We're going to organise another Occupational Therapist visit to see if there is anything that can be done to help. I just feel so helpless really, I wish there was something I could do to help repair his muscles and help him to enjoy his retirement, he's a very clever guy, he loves travelling, he's worked hard all his life, I just wish he could enjoy his retirement in the proper way.

I feel really gutted about what happened to Vicky from the CF Forum. She went into hospital about six months ago for a simple port operation. However that didn't go smoothly and after that it just seemed to be one horrible thing after another. The pain she must have gone through I can't even imagine, or maybe I'm just too scared to imagine. I also guess I feel relieved that my version of CF isn't as bad, and it makes me so angry that someone so young should have to go through something like that.

Feelin' Mingin'

2009-08-21T07:43:00.000-07:00

Robotic sounds mostly coming from my chest at the moment, especially when I'm asleep: Click, Whiirrrr, Click, Whirrr....Cough! Cough! Click, Whiirrrr, Click, Whirrr....

The cold virus from last week has moved onto my chest with a vengeance, and my daft CF lungs have responded in their usual fashion by creating pints and pints of goo. Bleuuurrr. Last night my head felt really woolly and I still feel a bit nauseus and spaced out; I also had a slight temperature. I don't think it's because I'm coming down with anything; it's just the sheer amount that I've been huffing through the day (which also makes me tense my neck and shoulders) that I've given myself a headache. I've put myself back on Uniphyllin to try and stop my chest getting tight, so hopefully things will improve in the next few days...I also can't use the Creon tubs as spit pots anymore because they've gone and changed the shape, so I'm using plastic cups in the car, which are minging because they're transparent. Moan, moan, moan....;-)

Edit: 2 Days Later. I've decided my symptoms are down to some kind of stomach bug, mainly due to the mild nausea and bloated feeling at night, coupled with aching limbs, especially at night. I'm also a lot more tired than usual, which all chimes with previous symptoms I've had a few years ago. ( No D&V this time though, thank god! )

Anyway, I heard this on the radio the other night. I don't know anything about early Fleetwood Mac apart from Rhiannon, but I thought this song was really beautiful. Stevie Nicks has a great voice. She's hot as well ;-)

Everyone should have a copy of this album...

2009-08-12T08:47:00.000-07:00

Has it really been 20 years since this (their eponymous debut album) first came out? It can't be! As had been said before, they were a guitar band that you could dance to, who looked cute. What more do you need? Apart from some more albums of course :-)

The Stones Roses peaked too soon, but their fusion of guitar based music and dance inspired many other bands such as Oasis, Blur and the Britpop movement of 1995.

NME's best British album of all time revealed

The Stone Roses' self-titled 1989 debut album has been voted the best British album of all time in a Sunday newspaper poll.

In 1989 the critical response was generally positive and today the album is widely considered to be one of the very best British albums released.

The Stone Roses' eponymous first album has been voted best album of the last 50 years by listeners of the BBC digital station, 6 Music.

Reni is considered by many to be the best drummer of his generation, and the "single most important drummer in UK indie circles"

Gonna add a link here to a guitar part I never learnt. Kids have it easy these days, at the time the only way to learn songs was to sit with your guitar forever stopping and starting a tape player to learn a riff by ear, this could take hours and even days. nowadays you've got ready made lessons on youtube!

Second cold in two weeks, grrrr!

2009-08-11T05:59:00.000-07:00

Nurseries are lovely places full of joy and laughter. I sometimes think that working in a nursery would be my ideal fun job, then I remember the high energy levels of groups of kids under the age of 5 and realise I wouldn't be able to take the pace. They are also hubs for the common cold amongst other infectious bugs; Abby and Oscar have both come down with yet another cold - Abby had a temperature on Friday and they both came down with colds over the weekend, and it looks like me and R have now caught them as well. Pass the First Defence...

In the last couple of weeks, both Abby and Oscar have had their birthdays. Oscar is now 1 and Abby is now 4. (photos to follow.) In September Abby will be leaving her nursery and starting infant school. This is both brilliant and a little sad for me because I feel like she is growing up faster than I can keep up; she'll also be leaving the aunties and some of the friends that she's been with for the last 3 years.

Oscar is now very close to walking, and has taken his first few (unsupported) steps. His favourite pastime is emptying the kitchen cupboards of all their contents, including the cereal cupboard, noooo !

Video Schmideo!

2009-07-29T04:24:00.000-07:00

Heh Heh, well I finally got my video for cftube looking the way I want, there's no stopping me now! I'm going to bore my relatives senseless with my holiday videos ;-)

Seeing the vid really makes me miss playing in the band; we split up a couple of years ago because we all had other things going on in our lives and possibly because of that we just lost momentum really. I think M (The singer) had a real gift for writing songs; the rest of us just added the sonic film-flam around his songs. Anyway; I am for hire, if only I can find a band that needs a guitarist...

Oink, Oink!

2009-07-21T03:38:00.000-07:00

It feels a bit like I'm surrounded by the tentacles of Swine Flu at the moment. There have been a few suspected cases at Abby and Oscar's nursery; and today there has been a couple of suspected cases at my workplace. The company policy is to send the people home (quarantined) until their symptoms subside. That's the idea anyway; this morning one guy turned up feeling unwell with a temperature, and stayed around for an hour checking the NHS Direct website before being sent home, doh! All this makes my working from home seem very desirable, so I'm going to agitate to get my laptop setup so I can work from home.

We (Abby, Oscar, and me) have got colds at the moment. Oscar's terrible case of imaginary CF (;-)) continues; he's got a chest infection at the moment, if I put my ear to his chest I can hear the phlegm bubbling up, poor guy. R took him to the doctors yesterday as he was quite fed up, just not himself, with a slight temp. He's been put on a 5-day course of Amoxycillin. Because it's for kids it comes as a lovely yellow banana-flavour medicine. He doesn't like it though, and he's taken to spitting it out onto my work shirts, which now have lovely yellow stains on them ;-) Abby and me aren't too bad, I've just got one of those almost-colds that doesn't have full blown symptoms yet. I've run out of Vicks First Defence though, I couldn't get any at the supermarket either, I think it's sold out with all the concerns over swine flu. I don't think we've got swine flu (touches wood) though, the doctor said that the symptoms of swine flu present themselves very quickly and are quite severe, with persistant high temp. Roll on the immunisation jabs I say...

parte dos del Menorca...

2009-07-16T08:05:00.000-07:00

Menorca came and went in a blur of sun, sea, sand, swimming pools, hyperactive children and more annoyingly hyperactive reps. We stayed at the Victoria Playa hotel, which was very child-friendly and did some really nice (half board) buffet food. The buffet was a bit bland but they did do some more adventurous traditional Menorcan dishes like tapas, lobster soup and a tripe casserole with chick peas and chorizo. Abby did OK with the food because there was a kids buffet section with chips, beans and ice cream. Oscar took a fancy to the french baguettes, they helped with his teething. I was eating like a king; a cooked breakfast every day with danish pastries, toast, something with chips for lunch and a full three course meal in the evening couretesy of the buffet. I swear a normal person would have put on half a stone after 6 days on that diet; I'd just gained 1 pound when I got back, Grrrr ;-)

The pictures below are from when we hired a car for a couple of days and went up El Toro, a mountain in the middle of the island with a church at the top. On a clear day you can see the whole of the island. from the top. We also went to the two main port towns on the island; Ciutadella and Mahon, they are both well worth visiting for their fantastic architecture. As for the kids, well, Oscar was a real handful (crawling everywhere, opening and closing doors, ripping up other peoples Heat magazine, putting our plane tickets down the hotel toilet, etc) and Abby had a great time, she's getting very confident in the water, diving underwater in the swimming pools and even ventured out quite far in the sea. She didn't quite get into the social side of things with the other children until the last couple of nights though. The only complaint I'd have is the rooms, they were a bit cramped with Oscar in a cot on one side of our bed and Abby in a single bed on the other side. This meant that we always woke up early because whoever woke up first would always wake everyone else up...

Well, ho hum back to work and I'm still ridiculously overworked doing two people's jobs. I'd complain if I wasn't so pathetically thankful of having a job in this recession (another friend of mine has just been made redundant and he's been working for the same company for 20 years+

Anyway, to cheer me up, here's is a little video of Abby's new favourite "wash up" song as she calls it (The Boy Does Nothing by Alicia Dixon), they play it a nursery at the end of the day when they're tidying up :-)

The water in Menorca don't taste like what it 'orta

2009-07-12T14:29:00.000-07:00

Here's some pics from Menorca. Menorca's lovely, I'd definitely go there again. A cheesy Judith Charmers-style write up will follow when and if I get some time ;-)

I'm melting!!!

2009-07-01T14:08:00.001-07:00

Phew! I can't cope with this humidity, it like living in a rain forest. Well it's 10 PM and I'm sat here with a sweat on, basically praying for some breeze to come along and cool me down so I can sleep tonight. Oscar's in the loft and I really feel sorry for him with it being the hottest room in the house; he's currently sleeping in just his nappy with a fan pointing at his cot. Bless him.

Went to the hossy today for the end of my IV course. remarkably the cannula stayed in despite all my (and Abby and Oscar's) efforts to make it fall out ;-). Blows are slightly less than what I would have hoped :( FEV1 2.9 FVC 3.85. I think I seem to get more out of the first week of IVs, although my chest is definitely clearer now. While the new doc went out to check my blood level results I had a look at my previous blows and apart from a magnificent 3.3/4.3 back in 2006 (post IVs and post 3 days in Wales by the sea), they've stayed pretty steady (from 2.9-3.0) when I'm well, can drop to 2.4 when I have an infection. Maybe it's wrong to obsess too much about FEV numbers, perhaps how we feel is more important ;-) Thank god for Immodium though! IVs always give me the runs these days, but taking two immodium tablets every day gave me surreally normal bowel movements while I was on IVs and even carried me through a mortal weekend on the lash in Blackpool. I'm starting to pay for it now though, now I've gone "cold turkey" on Immodium (this is an accurate analogy, because Loperamide is an opioid-receptor agonist, it basically dopes up the gut -like opium- to slow it down, luckily or unluckily depending on your point of view it doesn't cross the central nervous system like other opiates)...Oh yeah, the high para-protein levels of last month (I Googled it and it suggested scary complications with bone marrow) are probably spurious and nothing to worry about, and that is Proff Webb approved, so good news :-)

Right, it's panic stations because we're off to Menorca for a week of sun, sea and sand this weekend (maybe we should just stay here, it'll probably be colder over there) and I haven't even thought about all the things to get for packing. Some things I've done last week are:

Gone clubbing in Blackpool with a cannula in
Decided clubbing in Blackpool was pretty grim.
Played a golf course with a terrible hang-over and a tubigrip over my cannula
Lost over half a stone in sweat on the hottest day of the year on said course
Had a really bad round after I pulled a muscle in my arm and decided to never play golf again because it's poo.
Decided mountain biking and running are much better things to do than golf.
Lost my jacket bought for me by my misses in the hotel in Blackpool, I've phoned them up but they can't find it, waaahhh!
Vowed never to drink again because it turns me into a cretin the next day ;-)

I have seen them riding seaward on the waves

2009-06-23T03:24:00.000-07:00

I found this on the internet while searching for something entirely unconnected. It's my mum's favourite poem. She can never read it without choking up near the end. The weird thing is, she's now passed this onto me and I can't read it to the end without welling up. Parents eh?

"The love song of J. Alfred Prufrock: T.S. Eliot

S’io credesse che mia risposta fosse
A persona che mai tornasse al mondo,
Questa fiamma staria senza piu scosse.
Ma perciocche giammai di questo fondo
Non torno vivo alcun, s’i’odo il vero,
Senza tema d’infamia ti rispondo.

LET us go then, you and I
When the evening is spread out against the sky
Like a patient etherised upon a table;
Let us go, through certain half-deserted streets,
The muttering retreats
Of restless nights in one-night cheap hotels
And sawdust restaurants with oyster-shells:
Streets that follow like a tedious argument
Of insidious intent
To lead you to an overwhelming question …
Oh, do not ask, “What is it?”
Let us go and make our visit.

In the room the women come and go
Talking of Michelangelo.

The yellow fog that rubs its back upon the window-panes,
The yellow smoke that rubs its muzzle on the window-panes
Licked its tongue into the corners of the evening,
Lingered upon the pools that stand in drains,
Let fall upon its back the soot that falls from chimneys,
Slipped by the terrace, made a sudden leap,
And seeing that it was a soft October night,
Curled once about the house, and fell asleep.

And indeed there will be time
For the yellow smoke that slides along the street,
Rubbing its back upon the window-panes;
There will be time, there will be time
To prepare a face to meet the faces that you meet;
There will be time to murder and create,
And time for all the works and days of hands
That lift and drop a question on your plate;
Time for you and time for me,
And time yet for a hundred indecisions,
And for a hundred visions and revisions,
Before the taking of a toast and tea.

In the room the women come and go
Talking of Michelangelo.

And indeed there will be time
To wonder, “Do I dare?” and, “Do I dare?”
Time to turn back and descend the stair,
With a bald spot in the middle of my hair—
[They will say: “How his hair is growing thin!”]
My morning coat, my collar mounting firmly to the chin,
My necktie rich and modest, but asserted by a simple pin—
[They will say: “But how his arms and legs are thin!”]
Do I dare
Disturb the universe?
In a minute there is time
For decisions and revisions which a minute will reverse.

For I have known them all already, known them all:—
Have known the evenings, mornings, afternoons,
I have measured out my life with coffee spoons;
I know the voices dying with a dying fall
Beneath the music from a farther room.
So how should I presume?

And I have known the eyes already, known them all—
The eyes that fix you in a formulated phrase,
And when I am formulated, sprawling on a pin,
When I am pinned and wriggling on the wall,
Then how should I begin
To spit out all the butt-ends of my days and ways?
And how should I presume?

And I have known the arms already, known them all—
Arms that are braceleted and white and bare
[But in the lamplight, downed with light brown hair!]
It is perfume from a dress
That makes me so digress?
Arms that lie along a table, or wrap about a shawl.
And should I then presume?
And how should I begin?

Shall I say, I have gone at dusk through narrow streets
And watched the smoke that rises from the pipes
Of lonely men in shirt-sleeves, leaning out of windows?…

I should have been a pair of ragged claws
Scuttling across the floors of silent seas.

And the afternoon, the evening, sleeps so peacefully!
Smoothed by long fingers,
Asleep … tired … or it malingers,
Stretched on the floor, here beside you and me.
Should I, after tea and cakes and ices,
Have the strength to force the moment to its crisis?
But though I have wept and fasted, wept and prayed,
Though I have seen my head [grown slightly bald] brought in upon a platter,
I am no prophet—and here’s no great matter;
I have seen the moment of my greatness flicker,
And I have seen the eternal Footman hold my coat, and snicker,
And in short, I was afraid.

And would it have been worth it, after all,
After the cups, the marmalade, the tea,
Among the porcelain, among some talk of you and me,
Would it have been worth while,
To have bitten off the matter with a smile,
To have squeezed the universe into a ball
To roll it toward some overwhelming question,
To say: “I am Lazarus, come from the dead,
Come back to tell you all, I shall tell you all”—
If one, settling a pillow by her head,
Should say: “That is not what I meant at all.
That is not it, at all.”

And would it have been worth it, after all,
Would it have been worth while,
After the sunsets and the dooryards and the sprinkled streets,
After the novels, after the teacups, after the skirts that trail along the floor—
And this, and so much more?—
It is impossible to say just what I mean!
But as if a magic lantern threw the nerves in patterns on a screen:
Would it have been worth while
If one, settling a pillow or throwing off a shawl,
And turning toward the window, should say:
“That is not it at all,
That is not what I meant, at all.”

No! I am not Prince Hamlet, nor was meant to be;
Am an attendant lord, one that will do
To swell a progress, start a scene or two,
Advise the prince; no doubt, an easy tool,
Deferential, glad to be of use,
Politic, cautious, and meticulous;
Full of high sentence, but a bit obtuse;
At times, indeed, almost ridiculous—
Almost, at times, the Fool.

I grow old … I grow old …
I shall wear the bottoms of my trousers rolled.

Shall I part my hair behind? Do I dare to eat a peach?
I shall wear white flannel trousers, and walk upon the beach.
I have heard the mermaids singing, each to each.

I do not think that they will sing to me.

I have seen them riding seaward on the waves
Combing the white hair of the waves blown back
When the wind blows the water white and black.

We have lingered in the chambers of the sea
By sea-girls wreathed with seaweed red and brown
Till human voices wake us, and we drown."

IV league

2009-06-19T05:21:00.000-07:00

Outpatients on Wednesday. it was quite packed in the waiting room because the docs were away at a CF conference in France (Brest?) last week. I think I saw Gazza from the forum, I was going to say hello, but before I did he was whisked away into a side room. I never know what the etiquette is with other PWCF anyway; whether it is OK to chat with all the cross infection concerns these days. Anyway I was ushered in by a new CF specialist nurse (she's hot, but I didn't say that ;-)) FEV1 was relatively unchanged at 2.85 and FEVC was down a bit at 3.5, I had decided beforehand to go on home IVs barring some kind of miraculous turnaround in blows.

The doctor was new as well, is it just me or are the doctors getting younger and younger? I have to admit the first thing that runs through my head when I see a new doctor is "Oh god, can he/she fit a cannula?" I don't like being the guinea pig to some inept youth learning how to find a vein (Andy Jones was particularly bad for the first year I remember ;-)) I always get my IVs fitted on the big vein that runs at the back of my arms, I can do left or right, but obviously the left is more practical because I'm right handed. Other than that it was just a normal IV visit; cannula went in OK, after an initial scare when it stopped bleeding back. Standard procedure (for me) is to give me the first dose of Ceftazadine and Tobramycin, to check if there are any allergic reactions and then go round the back of the pharmacy to pick up the boxes of IVs. At that point I was going to ask the CF specialist nurse about Laura but then I decided against it; it would probably put her in an awkward position as she wouldn't be allowed to say anything due to patient confidentiality, and then I started to question why I wanted to know more in the first place. Anyway, before I knew it I was driving back with plastic bags and boxes of bottles, bungs, needles and syringes. I've put it all by the side of my bed. The Tobramycin dose has changed from 4ml twice a day to 7.5 ml once a day. I'm taking some kind of immodium variant as well because IVs seem to give me the runs these days.

I then discovered that I'd run out of needles for the Tobramycin finger-prick-test so I foolishly decided to do it with a normal green IV needle. I attempted it four times but the blood was not forthcoming so I had to ask the long-suffering R to do it for me. She didn't hold back, in fact it was a bit of a painful gusher, Ouch! No masochist, me ;-)

Out with the old, in with the new!

2009-06-16T05:41:00.000-07:00

We've had four good years together; she's been there in my hour of need, she told me all about this great new drug called Dnase. We went travelling together, to Croatia, California, and Shanghai, she's never let me down (apart from America where she decided to run out of batteries halfway through the trip damn it.) But now, our relationship has run it's course and the end is nigh (sniff.) It's time for me to replace my old Ineb. And try the younger and smoother model...

OK, I think I've took the analogy a bit too far ;-) Anyway, first impressions; the new model unfortunately looks exactly the same (no sleek redesign by some young idiot like Ben from the Apprentice), but the original jaw-rattling shake it did when you breath in (sometimes so fierce you would end up droping it if you weren't holding it) is now a lot gentler and quieter, the beep is different too. I love my new Ineb ;-)

Me and R went to see the Doves on Saturday in Delamere Forest. They were great, the sound was really good. The field in which they played was like a natural outdoor audiorium. We left just before the last song to avoid getting stuck in the car park on the way out. Not very rock and roll but when you get all night baby sitters you have to make the most of them ;-) In out defence we did get to hear the final encore "There goes the fear" as we were walking back along the woodland path. Magical. Another obscure fact: I saw these guys play back in New Years Eve 1999 at the Night and Day cafe (Manchester), on that night they were the unsigned backing band for Badly Drawn Boy and were most famous for their early 90s hit under the name Sub Sub.

Video and Moviemaker nightmare!

2009-06-11T06:03:00.000-07:00

I'm trying to edit together a video of my old band for the CF YouTube thinggummy and I can't seem to insert other pictures without buggering up the audio stream. Anyway, I've decided I don't really want to use the song after all, although it does pick up towards the end. We did do better songs but they aren't on the video, unfortunately. I'm on the right with the pink guitar by the way :-)