Orkambi: A Life Sentence

I've lived my whole life like it has an early expiration date. Recently, a drug was FDA-approved that could change that. I'm grateful, but I'm also scared…

| 5 min read
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Amy Braid
Amy-Braid-Wedding

When you have cystic fibrosis you spend your life waiting for the ball to drop on your health. You live life as best as you can: you go to college, you get a job in your field, you get married, you have a social life -- maybe even a child or two. And in between all that living, you fit in hospital stays, IV antibiotics and daily therapies

But what happens when you are in your 30s, and you can no longer work because your health is too precarious? You have been evaluated for a double lung transplant (but deemed too healthy), and then you learn that Orkambi has been FDA-approved and could change all that?!

Well, for me, I was excited. Then I was scared. Then I was about a thousand other emotions rolled into one.

You may be wondering why I am scared and why I am not so totally overwhelmed with happiness that I can't think straight. You might also be thinking that I am ungrateful since I am not shouting from the rooftops. But I am grateful. I am also just scared of life, as crazy as that may sound.

Then

I don't remember when I became fully aware of CF and its consequences. I always knew my life was going to be shorter, but I don't think I ever truly THOUGHT about it. Not until about 15 or so years ago anyway, when I was roughly 20 years old. I began to think about my life expectancy when my life was opening up before me.

I went to college and immediately after graduating I got a job in my field. I dated here and there and had my own apartment. But I lived day to day, and month to month.

I never made long-term goals because for me, “long term” was never going to happen. The future was very uncertain.

I had always assumed I would never see 40, and 60 was never even a blip on my radar. To be honest, I thought I would be dead by 26. But here I am at 34, still alive and kicking.

I lived my whole life expecting to die at any point. Knowing that my life had an early expiration date that was gaining momentum as I aged, I put aside thoughts of ever having to bury my parents or my husband. Aging, and all that accompanies it, was the last thing on my mind. I always assumed that I would never see my niece and nephews graduate high school or my step-kids get married. And I definitely never thought about saving for a “real” retirement when I worked.

And Now

Orkambi is now making me face the possibility of ALL of those things. I may get the chance to grow old with my husband. I may get to see my niece and nephews graduate and get married and have babies of their own. I may get to dance with my step-son at his wedding. I may get to experience all the things that the elderly experience -- the good and the bad, like burying your parents and friends. I may have to start saving for a retirement -- in my mid-thirties!

So why then does all that make me scared when it should make me ecstatic?

When I think of my possible future because of this drug, I find myself thinking of it in terms of a cancer diagnosis. Hang on, don't scoff at me yet. When a seemingly healthy individual is given a diagnosis of terminal cancer, their whole life is flipped upside down. There is no longer the possibility of a long-term future. They need to live life day to day and hope that they can make it to any milestone. The thing is, I already did live my life that way. I've lived with a life-shortening disease, and now it may no longer be life-shortening. I'm suddenly faced with a future of endless possibilities. Both scenarios are scary. Both will change your life and flip it upside down.

When I think of my life on Orkambi and the possibilities it opens up, I am scared. But I'm also very excited to “live” my life again. I can see myself as an old woman, and it's crazy. Maybe I will be able to sit on the porch on rocking chairs with my husband one day and watch our grandkids playing in the yard...and maybe, just maybe, I won't be so scared anymore.

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Amy was diagnosed with cystic fibrosis in 1987 at the age of 6. She lives outside of Boston with her husband and two stepchildren. Amy earned her B.S. in interior design and her M.A. in history and has a passion for both. Although unable to work, she concentrates on maintaining her health and expanding her crafting skills. She also has her own blog about life with CF, Mastering the Art of Breathing.

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This site contains general information about cystic fibrosis, as well as personal insight from the CF community. Opinions and experiences shared by members of our community, including but not limited to people with CF and their families, belong solely to the blog post author and do not represent those of the Cystic Fibrosis Foundation, unless explicitly stated. In addition, the site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.