Race Against Time

Hopefully, you have been hearing some really exciting news regarding Cystic Fibrosis and medicine in general lately. Science is beyond amazing and even my pessimistic side that is adamant that I will never see a cure for CF in my lifetime is starting to fade. And I am certain that young children with CF will never understand what the disease was like for us older CFers and that is an amazing thing!

There is a part of me though that has to hold my breath or swallow my excitement. The newest medications for CF are still years away and even though these very well could stop CF progression in it's tracks I can't help, but worry these meds may come just a little too late. I have pseudomonas, I have scarring and I have a disease that has progressed a lot in my 33 years. I wonder if I will still have these lungs by the time these medications come along. I lost a few friends right before Orkambi became available and know even more that were transplanted before being able to try any of Vertex's meds. I really hope that I can at least try some of these third generation drugs and I hope that I will be around long enough to see them come to market.

Sometimes life with CF can make looking into the future a daunting task. Thinking how hard my lungs have worked and how hard this disease has worked towards destroying my body in the past 33 years it can make the future seem bleak at best. With all this scarring and bacteria how can I fight against CF long term? Despite doing everything my doctors ask of me plus more I still see my lungs struggle just to keep up with this ever growing army of bacteria. The future looks long and treacherous. Thinking long term is frightening and exhausting at the same time. Realistically, given what is available today I can't imagine keeping these lungs long enough to see old agr.

Hearing about these new medical advances in CF allows me to make mini goals to reach for along the way to old agee. They have become a mental rest stop along the way. Maybe I can fight this battle for 5 more years and then I will get these meds to help allieviate my exhausted body from this raging war. Yes, 5 years sounds so much easier than "forever" and I can wrap my mind around making it just 5 more years. It makes the future less exhausting, unachievable and scary. If I keep up this all consuming battle for just a few more year back up will arrive and the long term fight may be a bit more feasible.

There are never any guarantees for tomorrow and this is even more true when you have a progressive disease. I know I may not gain as much as healthier CFers from these medication, but more than anything I just want the chance to try. A chance to live to see old age even if these lungs don't improve. I can manage a pretty nice life with these scarred and battered lungs. For now, I am doing everything in my power to be here as long as possible and a goal of 5 years actually feels doable. So I wake up each morning and I swim and I do all my treatments and I take a few supplements on the side. And I feel I have enough reserves for the next few years. Let's hope Science can meet me half way!

Potential New Drug for CF

Okay, so if all goes well it won't be ready until 2017. That just means we have to keep ourselves alive and well for another 6 years. I can put up with that. It is nice to see so many drugs in the pipeline that will hopefully make our lives longer and more enjoyable.

The article can be found here.

Yay!

Yay!!

http://www.cff.org/aboutCFFoundation/NewsEvents/2011NewsArchive/6-9-Phase-2-Study-of-VX-770-and-VX-809-Results.cfm

VX news release (very detailed)

http://investors.vrtx.com/releasedetail.cfm?ReleaseID=583683


I am trying not to get too excited, but I think it is okay to get a little excited. BTW, sorry that I wasn't able to link it. You will have to copy and paste it.

Genes and Severity of Cystic Fibrosis

We all know that CFers seem to have different experiences with their disease. Looking at CF forums some Cfers receive lung transplants in their teens or die before their 18th birthday while others are in their 30s with PFTs in the 90s. Drs, scientist, and patients alike would love to know what other factors besides mutation type leads to these variations in severity. This article discusses just that. Although, it is still being researched it is great to know that the more we learn about the disease the better the prognosis will be for all of us.


Genes Tied to Severity of Cystic Fibrosis Identified - US News and World Report