Saturday, July 18, 2015


There is a flurry of emotions whirling through my mind. I feel as if I am teeter tottering between every emotion that a human can possible feel throughout each and every day. Why all this emotional instability? The newest Vertex drug, Orkambi, is the culprit of all this emotional confusion.

The two years of utter free falling into a dark abyss from this disease made me wish with every fiber of my being for Orkambi to be approved. Every wish my heart held so dearly revolved around something, anything that could pull me up, even just a little, from this bottomless well. Now that Orakmbi is here I feel such a mix of emotions I feel I may go crazy with uncertainty.

The optimistic side of me is feeling excitement so strongly that I can feel it buzzing in my bones. The optimistic side of me wants to scream and shout and jump up and down that there is something, anything that may help me maintain the last 5 months of stability for the next several years. The optimistic side of me want to believe that this drug will work well for me and I will gain 4% lung function or more! I want to have more energy to chase and swim and bike with my daughter. I want to gain weight easily, and have fewer aches, and have more stability in my day to day life when it comes to health.

And then a voice, just as loud and adamant as my optimistic voice, comes bursting into my mind. The voice is my realistic (pessimistic?) side and it is reminding me that not ALL people experienced increased lung function and there were a few that experienced side effects that required them to stop the medication (mainly elevated liver levels). Some felt nothing and others had symptoms that were troublesome and even a bit scary like extreme shortness of breath.

To add icing to my troublesome cake I feel paralyzed with fear that my insurance, which has been reviewing my case for over a week now, will deny my ability to even try this medication.

So much of life with Cystic Fibrosis is luck. What mutations you have, how the disease presents itself, what bugs you catch and how much havoc they wreak on your body, how well you respond to meds and if you are allergic to them. Here is just another example of luck. I have heard this drug has been a life saving miracle for some people and for others it is a nuisance not worth their time. How I feel about this medication is anyone's guess and sheer luck just as most things with CF. And of course, luck will determine if my insurance will even cover this $259,000 a year medication.

I just hope that this time, lady luck is on my side!