No Future?

So, yesterday was a pretty significant day in the Cystic Fibrosis community. Vertex pharmaceuticals in the USA announced very promising data from their phase three trials of a combination drug that will treat the genetic cause of Cystic Fibrosis directly. The data claims that if and when the drug should become available in the US and EU markets that it *could* offer suitable patients with the double delta DF508 gene mutation anywhere between a 6-10% (or maybe more) increase in their lung function and see a decrease in the amount of time spent in hospital as it seems to also reduce the amount of exacerbations of CF too.


People with the double DF508 gene mutation make up about 50% of the CF community.


What does this mean? Well, it's a bona fide scientific breakthrough as, since now, all medicines and treatments have been tailored towards treating the underlying symptoms of Cystic Fibrosis - this would be the first drug ever to attack the cause directly. The real world implications of this could be staggering indeed, young children born with CF could now take this drug and live a whole and normal life, requiring much less treatments and time in hospital and experiencing much less serious long term damage to their lungs, perhaps for some, none at all.


And yet, I'm not jumping around with happiness... I don't really feel anything about it. Should I?


Undoubtedly this is a major scientific breakthrough the likes of which have never been seen before and I'm glad that many people won't have to go through many of the horrible things that I did growing up. However, part of me feels angry too. There's a lot of people throwing the words 'miracle cure' around right now, people are elated with the news of the breakthrough, which is completely understandable. There's lots of hope and there's lots of optimism that this drug will lead to other drugs and new treatments and that this whole process could potentially be the first baby step towards an eventual cure for Cystic Fibrosis.


I guess, being the super dooper realist that I am I'm just deeply entrenched in cold, hard reality. If the FDA approves the drug in the US it will probably take between 8-12 months to do, then subsequent approval has to be sought in Europe too which will most likely take another year. Then there's the question of whether the already under strain budgets of the NHS can afford to purchase it. There's all kinds of cost quotes flying round right now, some say in the US it will cost anywhere between $160-250,000 dollars per course of treatment for each patient so it is likely to cost a shitload in the UK.


Another issue is one of what about the people who don't have the double DF508 gene mutation? They continue to wait and hope for a similar breakthrough as that's the problem with genetic diseases like CF - any breakthrough in treatments invariably denies another group within the community as it is just that complex to gain any ground on. Irritating bastard that it is.


My anger is for those of us who the drug offers very little for, the older people with CF who have struggled and fought their way into adulthood. Those of us close, or already on the transplant list, and yes, those of us who have already lost the battle.


Time is always against you when you have Cystic Fibrosis, and discoveries like this are too late for many. I can't help being angry about that. I'm not 100% sure if I qualify for this new treatment *should* it become available, my CF unit are busy attempting to categorise people's secondary mutations if they have them so I'm sure they will tell me once they have assessed the information. I'm more concerned that If I am viable then by the time the drug is approved and if the NHS can fund it what state will my health be in by then? 2-3 years is a lifetime away right now, despite me doing really well and busting my balls to stay that way with another transplant assessment coming up.


Don't get me wrong, I'm happy and recognize the massive importance of yesterday's breakthrough. I just wanted to share some thoughts about the harsh reality of how it will affect the CF community as a whole.


*throws gang sign with hands and struts away from laptop*

Fear Costs Life

This latest blog isn't going to be particularly in-depth about an aspect of living life with CF. It's more a statement about life and what life is really about. Deep huh? It's going to be one of those blogs that will probably come as a shock to people who read it (Andy has actual feelings - shock, horror!).

About three years ago, I was reeling from a break up with someone I was on and off with for about two years. At one point I decided to dip my toe in the realm of online dating which was overall a nightmare as in my opinion, most people who online date are completely emotionally damaged for the most part and batshit crazy.

Don't get me wrong, there are diamonds out there on the t'interwebs, but overall it's just a huge NO for me.

And that's what this blog is about: the person who walked into my life that is a diamond.

I didn't realise it at first, I took her and her feelings for granted and in all honesty didn't believe her intentions were true. You see, for all my confidence when it comes to life and women in general, pretty much every woman who has said all the nice things that we all want to hear has inevitably fallen short of proving them with their actions at some point so I've been hesitant to believe them.

That's not to say all my exes have been bad, just most of them have.

Also, when you throw into the mix societal pressures regarding gender roles in relationships, 'men must have a job' and 'you must be able to have kids' and so on (a lot of women are incredibly shallow and fickle these days), being an adult male with CF is quite troublesome at times and as a result I was simply too scared to believe my diamond was genuine. I mean why would any woman be seriously interested in a man who can't work anymore, can't naturally have children and most of all who would want to love/risk loving a man where there is a good chance that they will die at some point in their relationship/life together?

That is the absolute truth of the matter but what a fool I was for thinking that bullshit eh?

I believe fear is mankind's worst trait. Quite simply put it holds us back, it denies us so much in life, so many good things that many more people would experience if they just had the balls to acknowledge it, process it, and laugh in its face.

I know I like to sing and dance about being awesome all of the time but the brutal truth is that at times, I am just as guilty of being as weak and fearful as many people are these days.

To her absolute credit, my diamond stayed in my life and I now absolutely couldn't imagine it without her. She's never faltered once, doesn't care that I have Cystic Fibrosis or that I am unable to work anymore as a result and has been steadfast in showing me how much she loves me over and over again. She's been there for me when I've been seriously ill, happy, sad, grumpy the whole nine yards and we've also shared some really great times together too. In short, she's the only person who has ever truly made me feel like being 'me' is acceptable and when we spend time together it is as though the rest of the world and its problems just don't exist. She's been completely infallible.

That's a very special thing indeed. It's that thing which we read about and see in films and cultural narratives/discourses. And its that thing which many people search for their entire life but unfortunately never attain or find.

I've recently decided to face how I truly feel about her, and she knows this, because quite frankly and as much of a cliche it is life is just too bloody short and I'm bored of pretending and lying to myself, and most of all lying to her.

You don't take for granted what we have and regardless of the fact that if this particular part of my life has an happy ending or not as that is up in the air at the moment, then I know I won't ever take her or her feelings for granted ever again.

In short, what I want to say is don't be scared to love people, or BE loved by people. It's incredibly hard at times I agree as so many are not true to their words these days which I have experienced over and over again, so if you're lucky enough to find a diamond who is then grab it and cherish it. Your life will be enriched infinitely.

If my diamond is reading this then I just want to say thank you, for being you.

Oh, and to everybody else I'm not having an emotional breakdown or anything or turning into an emo.

Real talk.

Peace out.

To Know Me, Is To Understand Me. Maybe.

Wow. One blog in and I'm suddenly a combination of Oscar fucking Wilde (without the bum sex) and ol' Bill Shakespeare (god rest his soul).

So many thoughts and things to write about. Here's hoping I can keep this up and not fade into obscurity ey, because you know, internet fame is the shit these days, right?

I was inspired to write this blog as I recounted the countless times people often get the wrong impression of me which in all honesty I find hilarious.

If you know me 'in the real world' or even have added me on Faceachebook then you'll probably have thought I'm a cunt at one point or another due to the things I say and post. Which is great, because I am. Not in the negative, tossy, fuckwittery sense like most people are these days. I mean, let's be honest we all know a cunt don't we and their masses seem to be growing exponentially daily at an alarming rate, whereas genuine, decent people seem to be on the decline.

Maybe society is in a state of devolution. Behold the Cuntocalypse. Hmm.

Anyways, back to me.

I am brutally honest in all areas of my life. Always have been, always will be. I can hands down say that is one of the most beneficial things which has come with being born with a terminal illness such as Cystic Fibrosis. I am truly grateful for that outlook too. I just cannot be arsed lying or pandering to people, or their expectations of how they think we should all act and behave. The older I've gotten the less I've cared about offending people too. Life is just too bloody short for tolerating fuckwittery in my book.

Fact is, most people are full of shit and lie their arses off daily about the most inane and stupid things, and that kind of stuff boils my piss as I just don't see the point in it. There's even some cultural theory that backs this up (one of the reasons I fucking loved my degree).

In 1959, a sociologist called Erving Goffman wrote a book called The Presentation of  Self in Everyday Life in which he proposes that human beings use theatrical performance in their daily face-to-face social interactions.

Goffman proposes that we all have two distinct regions, a stage managed front and back region. For example, say a well-known celebrity projects the image of being a clean living, happily married man or woman and then they are revealed via the press to be a drug addled cheating fuckwit which is often the case for most of them. Ugh celebrities, definitely need to write a venomous blog about why I hate most of the pointless oxygen thieves at some point.

If you think about your own social interactions for a minute with people I guarantee you can think of occasions where this theory rings true. I mean, just look at how much women bitch about their mates or other women as a shining example, or how much you have to tolerate that cunt you work with who you secretly despise and wish dead on a daily basis. It is human nature.

I guess what I'm saying is that I have a truly unique perspective on the world and life in general. Even more so now I'm getting older with CF and face a very uncertain future. I rebel in the face of normal expectations, assumptions and just the general mind-numbing boringness of most people these days (boringness is totally a real word by the way).

I seem to have very little of a front or back region (sorry Erving mate). I don't have one of those switches in my head that stops you saying what you think. Mine seems to be permanently on 'speak your mind' mode. Often with offensive and hilarious results.

I love life, I love taking the piss out of myself, people and situations every day as it is true that a day without laughter is a day wasted. Inappropriate humour is the best kind of humour for me and pretty much most things are fair game to be poked fun at.

I'd also admit that my cunty demeanour is a test. If people can hack it and give as good as they get then they are pretty much in my awesome book. And those are the type of people I want in my life.

Those with spirt. And passion. And vitality. And most of all the sheer balls to say what they think.

I know so many people who have so much shit going on because they choose to surround themselves with shit, weak and dodgy people and I'm fucked if that is ever going to be me. I have an extremely low tolerance for bullshit, drama and just the general stuff that we all hear people bleating on about everyday and think, ''who fucking cares you stupid cuntnugget?''

So, in closing statement to the court, yes I'm a cunt your honour. I may well be the cuntiest cunt to ever cunt a cunt in all honesty. But, before judging, take some time and think, or attempt to understand that in the face of insurmountable odds I just want to enjoy life and embrace it. as we all should but so often so very few of us actually manage to.

That's not an excuse by the way, I more than welcome criticism and appreciate being told off sometimes as we all need it.

In short, I'm a good cunt and if you're like me then you're very welcome in my life.