On Orkambi and Hope

Girl With a Balloon by Banksy

Girl With a Balloon by Banksy

When you live with illness your entire life, you get used to certain realities. With Cystic Fibrosis, the reality is this disease kills you. Thanks to research and medications funded by the Cystic Fibrosis Foundation, the life expectancy has risen substantially in my lifetime, but its still hovers at about 40 years old. Another reality is we don’t often go gently into that good night. Whether it’s respiratory failure, transplant complications, heart failure, or liver failure, end stages of Cystic Fibrosis are hardly forgiving.

Yesterday, my reality maybe changed. At the very least, it got put on hold. Around lunch time, my speciality pharmacy called to say my health insurance approved a prior authorization for the newly FDA-approved CF drug, Orkambi. In other words, my insurance agreed to foot the whopping $300,000 annual bill for the prescription. I nearly fainted at the news.

If you’re not part of the CF community, maybe you haven’t heard of Orkambi. I’m here to tell you, it’s a big deal. When I was born in 1988, they didn’t even know what or where the CF gene was. Now, 27 years later, Orkambi is the second drug to get at the underlying cause of CF. The first drug to treat the underlying cause was Kalydeco. While a breakthrough in CF research, Kalydeco was only successful for a rare mutation affecting about 4% of CF patients. Though Orkambi is not an all-encompassing drug (it does not work for all CF mutations), it does target the mutation found in almost half the CF population. Rather than just treating symptoms of the disease, Orkambi helps the defective CFTR protein function normally for those with two copies of the F508del mutation.

During drug trials, researchers noted a 40% decrease in lung exacerbations. It’s those tricky lung exacerbations that lead to inflammation and infections, decline in lung function, damage to the lungs, and ultimately, death for Cystic Fibrosis patients. A 40% decrease is life changing.

I’ll be starting Okrambi in the next two weeks and as I type this I can’t believe it’s real.

Orkambi is not a cure, regular daily treatments and clinic checkups are still required, and as mentioned, it is not effective for all mutations of CF. But cure or not, it feels like I’m getting a new lease on life. Having kids and actually sticking around to raise them feels less like a daydream. Growing older doesn’t sound as scary because maybe I won’t get sicker with each passing year. Maybe I’ll still be here when they cure this thing.

My life leading up to Orkambi has hardly been all doom and gloom. I have an incredible support base of family and friends. A beautiful and loving boyfriend and an even more beautiful and loving golden retriever. I’ve traveled, been on a few adventures. And on the health front, I’m doing well. My lung functions are up and I can run a full three miles without wheezing. All in all, I really, really like my life. But it’s hard to shake a weight as heavy as chronic illness. Like many people with CF, I struggle with anxiety and at times, depression. Some of my anxiety probably stems from my fear of infection, from my fear of getting worse. Every flu season I hold my breath and hope I can evade it. Every flight I flinch at any passenger sniffle. I worry so much about getting sick, sometimes I actually make myself sick with all the worrying.

With Orkambi and its possible 40% decrease in lung exacerbations, for the first time in a long time, I feel light, unburdened. I feel hopeful.

I’m not the only one feeling hopeful. Yesterday, when I called my parents with my Orkambi news I could practically hear their hearts bursting over the phone – and from 2000 miles away. The fight’s not over. CF is still scary, it’s still a killer, and I still have it. But I think my family is due at least one long sigh of relief.

Before I start planning my bright(er) future, there might be some side effects. Maybe even brutal ones. Some CFers call the first week on Orkambi “hell week” or “the purge.” Excessive cough accompanied by boatloads of mucus, bloody sputum, and at times, vomit, are all common experiences in the first weeks on this pill. And while I’m not particularly looking forward to any of that, I can’t shake this feeling of lightness.

Plus, I’ve been feeling crappy for going on 28 years – what’s a few weeks?

 

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On Standing With Planned Parenthood

PPIn her essay “The Alienable Rights of Women,” Roxane Gay writes: “I struggle to accept that my body is a legislative matter. The truth of this makes it difficult for me to breathe. I don’t feel like I have inalienable rights. I don’t feel free.”

Friday, when the House voted to defund Planned Parenthood, Gay’s words hit me like a punch in the gut. In 2015, are women really not free?

After the news of the vote broke, I turned to social media, perhaps naively, hoping it might help me make sense of things. Instead, I heard crickets. Every day, my newsfeed is littered with outrage over everything from Taylor Swift’s belly button to deflated footballs. But when it comes to the reproductive rights of women, apparently it’s just another day on the internet. Overwhelmed by the apathy, I looked to Twitter. Again, my optimism was squashed. #PlannedParenthood is not trending. But Saturday was #TalkLikeAPirateDay, in case you were wondering.

And so I’m stuck here questioning, where is the outrage now? I am choking on words writing this, aware that to some, I come off as an angry feminist. Am I angry? One hundred percent yes. I’m screaming, spitting angry. And yes, of course I believe in equal rights for women. Even more, I believe in safe, affordable, and accessible women’s health care. And due to recent events, I’m now a scared feminist. Do I think the Senate will pass this ridiculous attempt to hurl women (and America) back in history 50 years? No. I can’t think that or I might not ever get out of bed again. But I’m scared that in 2015 this subject is getting any mindshare at all. Enough mindshare to bring it to a vote but not enough to warrant a Facebook post.

I think the fact that we’re all not screaming at the top of our lungs is alarming. Does our online apathy explain how Planned Parenthood even got on the chopping block to begin with? Do we really not care, at least not that much, about women’s rights – about women’s health?

I’m certainly not the only person who stands with Planned Parenthood. 73% of Americans surveyed in August said they are in favor of federal funding for women’s health exams. And 54% said they backed federal dollars going to Planned Parenthood specifically for those services. And through a sea of brunch pics,  I have spotted a few pissed off peers online since Friday’s vote, thank goodness. But I’ve noticed some of those speaking up feel they have to explain why they need Planned Parenthood. They’re listing cancer screening, pap smears, breast exams. Don’t worry though – not abortion.

You may have seen this statistic thrown around: abortion only accounts for about 3% of Planned Parenthood services. That statistic has been scrutinized because while Planned Parenthood performs a lot of other vital services to millions of American women and men, some argue since the organization receives more than 10% of their revenue from abortion services, abortion is deeply tied to what they do. (Important to note, typically, federal funding doesn’t pay for those services, save for in the case of Medicaid with rape, incest, or to save the life of the mother.) But isn’t all this beside the point? I don’t know why we have to separate Planned Parenthood services from each other – label them “good services” (cancer screening, STI prevention) and “bad services” (abortion). We won the right to our bodies in 1973 with Roe vs. Wade, so whether you visited Planned Parenthood for a routine pap or an abortion shouldn’t be open to public forum and debate. Frankly, I’m tired of talking about it.

And maybe that’s the problem. Maybe we’re all tired of talking about it. Maybe it’s not apathy, maybe it’s exhaustion. Maybe so many of us have been battling for basic women’s reproductive and health care rights for so long, through each election, through each ill-informed candidate, that we’ve simply gone hoarse from all the talking?

But I have to believe we’re not done fighting yet, that we’re not done speaking up. Certainly, the denial of women’s rights incites more anger than some deflated footballs?

 

My Xtreme Hike Story

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You may have heard that living with Cystic Fibrosis (CF) feels like breathing through a straw. Unfortunately, this isn’t an exaggeration. It’s true and I’m here to tell you it’s really, really scary. But the thing about having CF, or knowing someone with CF, is that despite the scary times, there’s a lot of hope, too. And for good reason: in spite of our orphan disease status (only 30,000 people with Cystic Fibrosis in the United States to date), the CF community is a fiery one – they get shit done. Thanks to the tireless efforts of the Cystic Fibrosis Foundation and their volunteers, the life expectancy has more than doubled in my lifetime alone.

But let’s not get ahead of ourselves. The median age of survival is still only hovering around 40. I turned 27 this past March. Since I’m a millennial, that means I’m just barely an adult (seriously, I went to the dentist by myself for the very first time last week – no cavities Mom!). And while CF isn’t a childhood disease anymore – with just about half of us CFers over the age of 18 – this current median age of survival is a hard pill to swallow. And trust me, we’re used to taking pills.

So what can we do? Well, let’s revisit that hope thing I touched on earlier. While there certainly are some bad days with CF, I’ve been beyond lucky to have many, many more good days. So many good days in fact, that I’m healthy enough to participate with my boyfriend in this year’s Cystic Fibrosis Xtreme Hike Colorado (Saturday, September 12, 2015) to raise funds, awareness, and above all else, hope for the CF community. And I’d love your help doing all three things. Please take a minute to donate if you can, share on your social, whatever it may be – and help us kick CF to the curb. 40 is not enough. Not even close. It’s time for a cure.

All donations help – big or small. Click here to help me reach my goal!