cystic fibrosis

cystic fibrosis research is improving all the time. hopefully in the next 5-10 years there will be a major breakthrough, probably through the gene therapy door, as <3 mentioned.

Put dead simply:

there's a gene that allows the pumping of certain ions into your lungs, which in turn draw water into the mucus that basically lines and protects your respiratory system. The water makes the mucus more runny, so that cells in your respiratory tract can hike it up to let it catch all the nasties that you breathe in-and then you tend to swallow it, taking all of the dust and crap and bacteria down into your stomach where it's rendered harmless.

In CF sufferers, the [CFTR] gene is deformed/mutated so it can't pump the ions in, therefore water can't get into the mucus.

As a result, the mucus stays all treacly, like tar instead of like runny snot , and it is very difficult for you to get it up and out of the lungs -so this heavy, thick, sticky mucus irritates the lungs, and it traps bacteria and whatnot, so it causes a cough and you're more susceptible to lung infections/bronchitis/pneumonia and whatnot.

Also, the mucus in your pancreatic duct gets all sticky, so digestion is harder, too.

So basically sufferers have a diminished lung capacity, and have to have daily physio so that they can hack up as much of the mucus as possible.

oh yeah, I know. I mentioned it slightly earlier.

A lot less people are on it than I would have expected, but I guess it's so short term, and so expensive..

That, and the NHS will be jiggered if it has to shell out for regular therapy for every CF patient. sad really.

Pah, most of these ethical whiners probably don't suffer from something so chronic and otherwise untreatable. I think the main problem that the scientists have, however, lies in the fact that they *don't actually know* how half of the processes involved in the isolation/uptake/whatever happen, nor have they been able to gauge the long term effects, if there are any.

That, and the NHS will be jiggered if it has to shell out for regular therapy for every CF patient. sad really.

True, but then a sufferer may not like the idea of a "non harmful" virus being in their lungs, in case that decides to mutate, plus the body may decide to try to fight virus antigens, which wouldn't be too good either.
The only option (that I can think of at the moment) at not having to shell out for regular therapy in future would be to put it in the gamets of an adult CF sufferer, but then there's the ethics again... And the unknown side effects.
It's unfair, really, isn't it...

Put dead simply:

there's a gene that allows the pumping of certain ions into your lungs, which in turn draw water into the mucus that basically lines and protects your respiratory system. The water makes the mucus more runny, so that cells in your respiratory tract can hike it up to let it catch all the nasties that you breathe in-and then you tend to swallow it, taking all of the dust and crap and bacteria down into your stomach where it's rendered harmless.

In CF sufferers, the [CFTR] gene is deformed/mutated so it can't pump the ions in, therefore water can't get into the mucus.

As a result, the mucus stays all treacly, like tar instead of like runny snot , and it is very difficult for you to get it up and out of the lungs -so this heavy, thick, sticky mucus irritates the lungs, and it traps bacteria and whatnot, so it causes a cough and you're more susceptible to lung infections/bronchitis/pneumonia and whatnot.

Also, the mucus in your pancreatic duct gets all sticky, so digestion is harder, too.

So basically sufferers have a diminished lung capacity, and have to have daily physio so that they can hack up as much of the mucus as possible.

Why not just use liposomes as vectors for the gene therapy and step past your 'virus mutation' fears.

hi all, just wondering if any of you out there have it, i do and would like to talk to other people who might have it? im in good health and 23,

I have a relative in Ireland with CF. Is there a higher incidence of CF in the Irish population than here?

I'm delighted that you are keeping well! I met a woman (whilst I was an in-patient at the Royal Brompton (Lung) Hospital) who was going to be the first candidate for gene therapy. She went on to have a baby daughter and her story was covered by the national press. It was a marvellous, inspiring story for CF sufferers and their families.

I have a friend who has it, and is going relatively strong at nineteen, but he's convinced he's going to die anyway, so he's very into drugs and alcohol. :| Which can't be fantastic, but he's not got the greatest outlook by all accounts, and is apparently enjoying his decadence "while he can".

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Do you have like..gene therapy and stuff?

Because the success rate for that, as far as I know, is shockingly low, much lower than using the virus as a vector, anyway. The CFTR gene just doesn't get to the nucleus on most occasions.

true, but if you are so anti-virus, which frankly are 'dangerous' for entirely different reasons than you gave, then you are left with markedly few alternatives.