VX-770

There has been some news surrounding CF and a tablet that can possibly help people with CF

Taken from the Daily Mail online (click here for the link)

The first drug to tackle the root cause of cystic fibrosis, rather than just the symptoms, could go on sale next year.
In trials, the twice-a-day pill dramatically improved the lung health of men and women with the debilitating condition.
They also put on weight and needed fewer antibiotics.
If trials on children are as successful, manufacturers Vertex Pharmaceuticals could apply later this year for permission to market the drug in Europe. Clearance is likely in 2012.
Britain's 8,000 adults and children with cystic fibrosis include Gordon Brown's four and a half-year-old son Fraser.
Cystic fibrosis is the UK’s most common life-threatening inherited condition and occurs when a genetic flaw produces a defective version of a protein key to the health of the lungs and digestive system.
Thick, sticky mucus clogs these organs, leading to recurrent chest infections and poor growth. Other symptoms include diabetes and infertility.
Although treatments have improved greatly in recent years, average life expectancy is under 40 and there is no cure.
In the trial, 161 people with cystic fibrosis were given either the new drug, which is known only as VX-770, or a dummy drug two times a day for a year.
At the outset, most of those taking part had just 60 per cent of the lung function of a healthy person - a figure that improved by almost 20 per cent by giving VX-770.
No other drug has produced such dramatic improvements, this week’s New Scientist reports, probably because they have tackled symptoms rather than the underlying cause, a defective protein in lung cells.
Patients also put on an average of half a stone in weight, suggesting the disease’s effects on digestion were eased, and were half as likely to need antibiotics for flare-ups.
Peter Mueller, Vertex’s chief scientific officer, said: ‘Treating the underlying cause of cystic fibrosis with VX-770 led to clinical improvements that were far beyond our expectations, providing support for an entirely new approach to the treatment of this disease.’

The Cystic Fibrosis Trust described the results as ‘very big news’ but cautioned that VX-770 would only be suitable for about 5 per cent of sufferers.
But a second drug, that is at an earlier stage in development, offers hope to another 75 per cent of patients.
Vertex is already testing a combination of the two drugs on patients and the first results are expected within months.


Now I'm not going to get excited, I'm not in that 5% and it's not a cure. I don't believe they will find a cure in my lifetime but I do believe treatments are improving all the time to increase average life expectancy. I have the common mutations of DF508 which I assume is the 75% they talk about, which they are running tests on next. But it's nice to know that in my lifetime there may be some people with CF who can take a tablet that deals with the cause of CF rather than the effects, there just isn't anything like that at the moment.
When I read that they have achieved a medication like this for my mutation and its available to take, that will be the day I have tears of joy in my eyes. Either way its great news for that 5%, it's not a cure but it's start!

A fellow blogger has a daughter with CF and she has done a great blog explaining more about the drug and how it works, click here to view

BBC Horizon - Miracle Cure? A Decade of the Human Genome

Back in April two people from the BBC (one of them was Dan Walker, a producer) came to see me about featuring in BBC's Horizon, regarding advances in cures for genetic diseases, as it is 10 years since they discovered the human genome. They stayed about an hour and videoed me whilst asking some questions (to see how I appear on film etc) and said they would get back to me. I knew they were seeing other people with CF as well and when they didn't get back to me I assumed they had picked someone else. It would have been nice to be informed of this or at least a thanks for me agreeing to see them but never mind, i'm sure they are busy people!

Anyway the programme is going to be on next Monday (25th October) on BBC 2 at 9pm. When they told me about it, it seemed really interesting so have a watch if you can! Here's a link for more information

http://www.bbc.co.uk/programmes/b00vm2d5

Organ Donation Awareness

I just wanted to blog about an event that is happening that some people might be interested in and is being organised by some of my friends!

Three lovely ladies have organised a ball to raise awareness of organ donation, they are only here today because they all have received a double lung transplant!

Here is the flyer for the event and a link to the facebook group, sadly I can't go as I could give them an infection and the same rule applies to anyone else with CF pre transplant, however anyone else is welcome to go, so if you fancy a trip to Glasgow then get your places booked!

For the facebook group click here

One of the three lovely ladies is Victoria who received her transplant quite recently. She has also been on TV lately with her boyfriend Gregg, to raise awareness of organ donation and CF of course. Here is the link to see them, skip to part 3! They were both great!! Remember they didn't know the questions in advance and were nervous!

How to represent CF?

Had outpatients today, my lung function is the same at 43% and my weight is 54.4kg. So all really stable which is a good thing. It annoys me when the doctors say my lung function is really good though, as I know its higher than it has been but it's not my best. So no I wouldn't say it was really good, I'd say it was OK, don't be celebrating quite yet...! Also, why can I not hit the 55kg mark for my weight??!! Weird!

Had my port flushed and some bloods taken from it to check my liver. I stopped taking voriconazole last week as I have been on it 3 months now, so now I am having a month off before I go back on it. My results from Bristol (to see which drugs would be most effective against the fungus on my lungs) have still not returned, it's been 6 weeks now so they are going to chase that up.

Some of the nurses saw me on TV! I hadn't told any of the staff but they still found me out! They said it was good anyway and asked how it had come about. I don't like to give too much away as I wouldn't like them to find this blog, I don't know why. Just wouldn't feel comfortable with it.

Most people said they liked my TV spot. I know it was abit short but I think they managed to get quite alot in, in two minutes. Some people with CF criticised it saying I didn't look ill enough and what was the point of it, that people would just think CF involved a few pills and physio and that was it, they were quite nasty and did apologise in the end. Then on the other side parents of children with CF were saying it was good that is showed me doing everyday things and was positive, one said they would have preferred to not have the average life expectancy bit.
So either way, however it was put across someone would not have being happy.

Firstly, I think it would be impossible to put across on TV how difficult it is to live with CF unless they followed you for months and had a TV programme just dedicated to CF. Even then I don't think it would be 100% successful. Some things just can not be put across, it's something you have to experience.
Secondly, I do not think the point of the programme was to make people feel sorry for me and donate money to the CF trust. If a person with CF had been on the programme that did need oxygen, was in a wheelchair etc, all that would have done was reinforce people's ideas of what a person with CF looks like. The majority of people with CF look perfectly healthy and normal even when very ill, I think that's what it showed.
What I think the two minutes achieved was to show abit about CF, what it involves, whats embarrassing about it (it was called embarrassing bodies after all) and like the doctor said (I didn't get there in time to record this bit), not all diseases can be cured and people have to learn to live with them, which is what I was doing in the footage.
People with CF need to remember, sputum, tablets, coughing and physio to them is perfectly normal. To healthy people watching, these things are not normal and therefore it has an impact on your average person.

Me on the TV!!

Here it is! Will comment tomorrow as don't have time now. I will say though, I do not usually have such a red and shiny face! There were loads of bright lights shining on me and it made me all warm! Also just ignore us laughing in the background!

Channel 4

Hello
Just to let you know I shall be on TV on Friday, Channel 4 9pm. I am going to be on Embarrassing Bodies, don't be put off by the name, they are doing sections of 'Living with ....' amongst the other stuff they do, so my section is living with CF. I hope it's ok and I don't let the side down, if I am honest I am dreading it!!

Jess on the News

Jo's Send Off

On Friday it was Jo's funeral, I wish I could have gone but it was a long way and I couldn't have gone on my own. I said a prayer for her at 11am which was the time of the service. Her friends on the CF forum (including me) put our money together to help give her a good send off. Jo loved cake (she ate a whole Victoria sponge every day to put weight on) and she loved Betty Boop, so here is what we sent her..... She would have loved it! We also sent her some flowers. I really miss her and still can't believe she is not here anymore, it's going to take some getting used toIn other news I have been busy... getting filmed! I'm not going to say anything else as it's not getting aired until Easter time, but watch this space!

Today we have been to look at five bungalows, yes five bungalows! We have seen one we LOVE! So watch this space too!

I'm starting to feel better I think, still getting tired easily and chest is still getting pains but it is starting to feel less tight. I don't think I have lost anymore weight but not put any on either, just keeping it steady now. I keep feeling lightheaded and like I can't see properly which is strange, I think it must be the voriconazole as it can cause visual disturbances. It feels like I'm in a daydream and not in my body, not good at times! I've been to the gym twice this week and walked Alfie so at least I am getting back to the exercising, it always goes on halt when I'm on my IV's as I never have the energy to go.

I am currently watching X Factor, so far I have voted three times for Olly. I knew he'd be in the final! Olly to win!!!! This is the first time I have watched the x-factor final in years, every year I'm always out! Yet another sign I'm getting old....!

Pick Me Up

Here is the article. Not sure if you will be able to read it but worth a go! Click here for a copy you can read properly! Its quite dramatic and cheesy but what did you expect?!

IV Time

I've given in and phoned the hospital to start some IV's, I hate feeling tired and been useless. I did nothing yesterday and haven't done much today really, I am not sleeping very well and my appetite is seriously not good. I weighed myself yesterday and I am now 55.3kg so I really do think I need some IV's before it starts becoming a problem. Therefore I am starting some IV's next Wednesday, which means I will be on them for the wedding we are going to. Not that happy about it, but what can you do? I'm still going to have a few drinks at the wedding, you have to celebrate!

The night out on Saturday as been cancelled, hardly anyone could go and both my friend and i don't feel great so we were going to drive back instead of stay over and leave abit earlier. So in the end my other friend cancelled it and we are going to rearrange it another night. So it has worked out for the best really. I'm just going to go out for a meal instead now, somewhere nice so I will actually eat something!

To give you an idea of what my eating habit is like at the moment I'll tell you what I have had today. It's quarter past 5 in the evening and so far I have had a small bowl of coco pops, a cup of hot chocolate, pack of chipsticks and 2 hob nobs. I have no inspiration of what to cook for tea, if it was just me and I didn't have Pete to think about, I probably wouldn't have any tea to be honest!

It turns out the mortgage advise we were given was incorrect, I knew it didn't make sense and thought I was the stupid one. Turns out the mortgage advisor is stupid and I am good for realising this! We can still get one but just not as much as she had told us.

I went to pick up the wedding photo package today, we have to pick 50 photographs out of 800!!! Best go anyway as I have to take Alfie to his training class, byeee! x

PS- My friend Tori who also has a blog and myself are going to be in Pick Me Up Magazine, the 13th November Issue which I think comes out next Thursday. So make sure you buy yourself a copy!