I'm optimistic about the vertex drugs, especially since my kids have the G551d mutation. Having said that, I dont get too excited these days, but if my kids are doing the vertex trial and report good results, I will become more excited about it.
The vertex drug actually does work at correcting the problem, and there are some on this forum who have participated in the ph2. trial and had fantastic results. If the ph3 is successful, I think that it will pretty much give cfer's with the genes it targets a fairly normal life and I would imagine a normal life span.
I'm working of the fact that most people with cf die from lung related issues, so if that can be corrected it would be fantastic. As far as the liver issues in cf go (the above post), quite a few pwcf have had successful liver transplants and continue to do well.
The lung issues are the most serious problem for the majority of cfer's. My oldest kids are nearly 30 and 28. If the vertex ph3 trial works as well as the ph2 and doesnt take too long to be accessible, then I actually believe that my kids will benefit by having a normal lifespan <img src="i/expressions/face-icon-small-smile.gif" border="0">