65 Roses, NACFC & Hope

Originally Posted on www.brfcf.org on October 19, 2012

New York in the fall is like seeing a real Monet instead of a bathroom print. My eyes struggle to incorporate the red, orange, and yellow hillsides as they roll along the Hudson River. The colors undoubtedly reminded me of the passing of time. Time for us is typically marked by quarterly CF visits and rotating months of Cayston – markers that seem to sneak behind us like unwelcome knocks at the door.

Last year’s NACFC opening session was a flood of excitement. Vertex 770 was poised to become CF’s first control drug. It would only serve three percent of population but, it presented “the proof of concept” and the energy surrounding it was contagious and undeniable. At this year’s conference, it was the potential of the combination of 809 and Kalydeco that spurred enthusiasm. The results have been fabulous, and I am so happy for those friends and families whose lives may soon experience a change in ways I cannot allow myself to imagine.

I vividly remember the stark, cold walls of the clinic room three years ago. I think I was there. Was it really my life? It was Maylie’s second CF clinic appointment, and my heart was still weighed from her diagnosis. I was overwhelmed by all of the new information, yet still felt ignorant about CF. I remember holding my two girls when the nurse walked through the door and told me about Ataluren. She told that it was doing well in the trials; she told me 2012 would be an amazing year for individuals and families with nonsense mutations. I remember feeling so lucky.

Now I sit on a train pointing towards New York City. Three days have passed since NACFC, but I’m still trying to make sense of my thoughts and emotions. I spent the weekend deciding if my knee-jerk reaction to some of the blogs from individuals who attended the conference was warranted, or if I was simply overreacting to the over inflation of hope that I saw spattering the pages of Facebook.

Here were my published thoughts:

I find that hope drives me in so many areas of my life- at times it is the only thing I can hold onto as I look at my sweet May. I see myself as someone who believes in the impossible and I want to share that hope, that faith with others. I do have to say that I have read blogs and posts from other parents from NACFC 2012 and I find myself a little scared, maybe even slightly irritated as hope IS what we all hold onto. There was hope at the conference, but the over inflation of hope is a disservice to our community. Please do your own research. Call your clinic or the CFF and ask for reliable overviews of the data that was presented.

Twenty twelve has come and mostly gone, and a control for nonsense mutations is not around the corner. I am not a pessimist. CF researchers are doing amazing work. If I have learned anything in the past three years, it’s that humans are complex organisms and cystic fibrosis is a complicated disease. It is no simple feat to manipulate defective proteins so they function properly in living cells. We are getting closer each day, but progress is incremental.

My aforementioned post was intended to state that there is much hope in the world of cystic fibrosis, but it is important to live beyond hope. As a member of the CF community, I feel the need to be protective of all of our hearts as the risk in our community is so great. Don’t get me wrong, I am filled with hope for Maylie and I eagerly anticipate Maylie’s version of Kalydeco. However, my hope for May goes beyond a “CF cure.” Will she have the contagious smile and dedication of Emily Schaller? Will she make everyone laugh and be an amazing friend like Josh Mogren? Will her voice echo on stage like Rose Longue, and inspire all those who hear it? To me, hope for CF is more than just a control for the symptoms or a means to fix the basic defect, it is the desire to raise my daughter in the likes of all the amazing adults who have grown strong because of the hurdles and fears that CF has thrown at them. Each one of them have enriched my life beyond words and made the CF journey more manageable.

So here’s to hope for the future in whatever it may hold, and to being alive and present in what we have today.

 

Kat Porco