I'm not very strong on this Topic.

Hey there,

It is very confusing, I get confused sometimes still and I've lived with it for 24 years!!

Yes, you are right, there is at present no cure for Cystic Fibrosis, they they are working hard to get one, it means basically changing the whole gene structure of your lungs and then also stopping it rejenerating those genes back in to CF ones.
CF effects almost everything, your lungs, liver, pancreas, van deferens and fallopian tubes. It is a faulty gene that screws up some doors that usually let chloride through. Because these doors don't work it means that mucus is thick and sticky clogging up everything that uses mucus. Very basic explanation there, about CF.

It's crazy that lung transplant is listed as a cure. It's not a cure, as it 1) doesn't fix all of the other problems associated with CF and 2) It is true transplanted lungs wont carry the CF gene, meaning they won't have the fault that causes all the sticky infection breeding ground. However, like the gene therapy issues they have had with rejeneration of bad genes. This happens after transplant too. Your genetic code is still there working away, meaning the transplanted lungs genes will eventually be replaced by your own.
Transplant is only given as a last hope measure, when your own lungs are so weak they can hardley support themseleves anymore.

I hope this helps, I probably haven't put things in the best way but I think I've covered everything almost. Any more questions, do ask.

All the best
Gem

Yeah it is all a little crazy huh?

I'm not so sure about other genetic diseases, I'd guess so. Especially the liver as that is faster to rejenerate than lung tissue. I think the lungs is still a slow process as once the CF genes are back in place, things will be different the second time around, as treatment is better, and you are more aware of infections, than you are in childhood. We all used to play together as kids, there were no cross-infection rules, meaning mutations of infections were made that thrive only in CF lungs. The longer you can avoid CF infections, the longer it will be to have any significant lung damage.

The good thing is-when I was born, my life expectancy was 5, I beat that. I beat the 12 year mark and the 21 year mark. I'm due to beat the 25 year mark in February. Up until a couple of years ago, the expectancy was 31, now it's 40 I think!! This is good news though, as it means by the time your little cousin reaches my age, life expectancy is probably going to be normal-at least 60! Even without a cure coming a long, this is where we are headed. Treatment is a lot better now than what it used to be. I think when I was diagnosed at 2, I'd just missed out on the previous dietry advice of eating nothing with fat in it, as enzymes weren't all that good.

Now treatment is there for mucus thinning, better antibiotics, enzymes. It's all just generally looking better all round. There is now Dornase Alfa, which is a nebuliser that breaks down certain DNA cells in the lungs which is what makes mucus about 10 x thicker for us. This is pretty new, and I'm sure people taking it before lung damage really is a problem, will help prevent infections in the younger generation.

I'm not saying there is nothing to worry about, I'm just saying there is now a lot less to worry about. Perhaps when your cousin gets to their 20's, which is usually when lung transplants are happening now, it might be that it isn't even thought about until a lot later on, as treatment is always advancing.


CF is still pretty young, in how long it's been known about, and even how recent the location of the genetic fault itself has been.

Gem

thats awful im sorry to hear that, just when you think things could start looking up. i have CF and was diagnosed at 6 weeks old im now 21, i havent had any transplants and am not on the list as of yet, i have quite alot of infections but my main issue is my stomach, my bowels and pancreas are really rubbish, and i suffer really bad with acid and am awating confermation of weather i need a laprascopic fundoplication operation. anyway i hope you are managing ok i pass my regards to your family
xxx