Keswick Lakes bike ride 2012

I think my age (hopefully not MD) may be catching up with me ;-), for the first hour of the bike I was fine, then on a down hill section my front wheel hit a rut and I went flying over the bars and landed quite heavily. After that I was quite shaky and after another hour my neck was in agony and I couldn't lift my head to ride my bike. We had a rest as the other 4 lads were all up for going back to the cottage with me. But I told them to carry on the ride because there was no point in them all missing out. Gutted but it was the right decision for me, my neck was in a bad way but taking the flat road back to Keswick gave me an easier time than the rocky hills would have. I'm going to have 2 weeks off the bike and then see how it goes. Other than that a good weekend, lungs were pretty clear (after the ABs last week) and we had some great weather as well...

The big 4-0

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.

Dad

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

perspective

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.