Today, I took a step back in a slightly longer video (it clocks in at 4:43 if you can bear it) to talk about my amazing network of friends and family, and what Orkambi really means for me, and thousands of others with my mutation, in the long run. As always, thanks for watching!
Day 4 has so far been much better than Day 3 – thanks heavens. Coughing a lot but I can hack it…literally! A-yo. If you want to hear more about my mucus, watch the video above for the full update.
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?
Cystic Fibrosis is hardly a glamorous disease. Among the laundry list of CF symptoms, many of us struggle with the following (lovely) traits:
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Wheezing or shortness of breath
- Frequent greasy, bulky stools or difficulty with bowel movements
Told you…no glamour here.
Some people seem to think having a chronic illness should eliminate body image issues – like maybe chronically ill people can’t be bothered with something as superficial as cup size or cellulite. That may be true for some young people with CF, but my reality was this: dealing with life-threatening lung infections and painful intestinal blockages certainly took my mind off beach body season from time to time, but sick or not, I was still just a girl growing up in a body-obsessed society. And living with CF often feels like you’re not in control of your own body, let alone at peace with it. For a long time, I spent many more nights awake in fear that my crush might google CF and see “bulky stools” or “phlegm” in his search results than I did thinking about the gravity of my illness.
In high school, I dreaded summer. At least certain aspects of it – the public parts. Sure, it meant beach days and school vacation, but it also meant salt crystals on my skin, a mucus-riddled cough from the humidity, dehydration (and probably diarrhea), and worst of all – bikinis. Remember those bulky stools I was talking about earlier? Those are the result of a heavy mucus buildup in our CF bodies, which often causes malabsorption of food and poor weight gain. And while that means we tend to be skinny (as if that’s the end-all, be-all, right?) it also typically leads to GI issues, intestinal blockages, and in my case, a distended stomach – or what I’ve come to know as my pot belly. What’s worse, just above the pot belly marks a pretty sizable scar – a remnant of intestinal surgery from my infancy. I spent most of my life aged 14-24 trying to hide what seemed to me a deformed and embarrassing stomach.
When fall came back around, I happily packed away my two-piece but not my body issues. And while my bloated stomach was safely out of view, my fingers were not. I am part of a percentage of CF patients who exhibit clubbed fingers and toes. A benign symptom of CF, but an unnerving one nonetheless when you’re young – anything that made me different made me less attractive, or so I joined the millions of insecure young girls and women in believing. Though I’ve gone through this life with most people not noticing, or at least holding their tongues about it, you’d be surprised how many adults have no qualms asking me”what’s wrong” with my fingers and toes. Unlike my stomach, there isn’t much to do to hide misshapen appendages. So instead, as a teen I avoided nail salons at all costs, opting to keep my nails short and unpainted, in hopes they’d be mistaken for plain rather than ugly, or even better – ignored all together.
Another tricky thing to hide: a chronic cough. And once more, it’s hardly glamorous. While Nicole Kidman may look dainty as a daisy coughing up blood as the tuberculosis-infected Satine in Moulin Rouge, I assure you that’s not what it looks like in real life – at least not any time I’ve had the pleasure of experiencing it. And though I’m happy to report I cough up blood very rarely these days, I do cough up some pretty serious loogies. Big, green, monstrous phlegm balls that sometimes wind up sticking to the inner elbow of my shirt after a good cough sesh. Better than sticking in my lungs I suppose, but not ideal for a young woman just trying to get through the day without inducing vomit from unsuspecting passerby. And I’m not the only one who suffers from these mucus attacks. There have been casualties! When I was 21 and recovering from a particularly nasty bout of pneumonia, one of my visitors said something funny I guess because laughed – and shot a huge phlegm bullet onto my friend’s boyfriend. At least he was nice about it…
Even when I don’t have pneumonia, my cough is loud and often head-turning. Read: not cute. As a younger woman, I tried to stifle my cough all the time – praying to stave off the concerned stranger or the “cut down on the cigs” jokes. I chewed gum in glass, hoping it might curb my urge to cough. I popped cough suppressants before work (which by the way, is not encouraged at all with CF as we need to cough it up and out of the lungs). I went to bonfires, coveted any seat up-wind and crossed my fingers the breeze wouldn’t change. I hung out with smokers and pretended it didn’t bother me – almost always risking a lung infection or the very least a gnarly cough in the days to come. And I’d even get mad at myself if I had to let a cough escape somewhere public and quiet – in the library or at the movies.
Speaking of quiet, I used to miss days of school just so I could poop in peace. Fun fact: those bulky stools I keep bringing up also don’t smell that great if I miss my pancreatic enzymes. And these trips to the bathroom bucked the asinine urban legend teenaged boys so routinely believe and perpetuate: girls don’t poop! As if it wasn’t terrible enough being the only human girl to ever poop, if I missed too many enzymes, I might land in the hospital with an intestinal blockage – an experience that is not only painful but embarrassing. Try explaining to your teenaged friends what an enema is.
Sometimes I think the most difficult part about growing up with a rare disease, but not so rare body image issues, is that no one ever touched on any of this in CF clinic. Body image wasn’t really a factor. I loved my clinic and in no way am I faulting them – when I was born, CF was still a childhood illness and they were probably just trying to get us to adulthood. But I do hope that today’s young people with CF as well a the CF clinic staff are more educated about the possible body image issues accompanying this disease. Because I couldn’t help but feel deformed when a group of med students fawned over my clubbed fingers and exchanged geeked out “just like in our text book” looks before even so much as a “hello” to the insecure kid attached to the abnormality. And because I still can’t shake the judgement and confusion I was met with when I told one doctor in college that I had ceased taking the steroids prescribed for lung inflammation because the side effects (weight gain and acne) were too much to handle freshman year. While everyone around me was concerned about treatments, BMI, clubbing, and nutrition, it seemed they’d forgotten they weren’t just talking to a CF patient, they were talking to an adolescent girl.
I’m not an adolescent girl anymore, thank God. So is there a happy ending? Have I finally come to peace with my body, with what CF has done or will continue to do to it? No. And I’m not sure I ever will but I am making progress. As I’ve gotten older with this illness, I’ve stopped rolling my eyes at that “my scars tells a story” mantra and have actually kind of embraced it – or at least I’m only sometimes rolling my eyes these days. I now appreciate my body and am kind of amazed at its resilience. I’m still here! Even more remarkable, I’m feeling pretty good most days. Having a flat stomach is not nearly as important to me as being able to run 3 miles without wheezing. Plus, one piece bathing suits are back in style. And while you still may never find me in a nail salon (some insecurities are tough to kick), I’m happy to talk enemas any day of the week.
I go back and forth a lot when it comes to writing about Cystic Fibrosis (CF) – writing about my Cystic Fibrosis. Some days it feels so coded not just literally in my DNA but in my life experience as a whole that I couldn’t possibly share my thoughts with any one person, let alone an audience of anonymous internet readers. Other days I worry that writing about CF would put me in a box – forever stamped a sick girl who blogs about mucus. And no one wants to read about mucus. But it’s on days like today, as I sit down on my porch with a cup of tea and my MacBook, when I think, well, shit – I guess I’d probably read it.
The truth is, having a chronic disease is lonely. And when you’re living with a rare chronic disease with a high risk of cross-infection making it difficult to meet the few others like you face-to-face, the alienation can be tough to shake. Though I have an amazing support network of friends and family who constantly motivate me to stay healthy and happy, at the end of the day, none of them have CF. And when CF is freaking them out, they get to talk to other people in their exact same situation – other parents, other significant others, other siblings, other friends. They can meet for coffee and hold hands and say “man, this sucks.”
For the most part, my only interaction with someone like me is when I stumble across the occasional local newspaper story about a high school senior raising money for the CF walk. Or worse – the obituary of someone who’s lost their battle. And while any exposure to this orphan disease is more than welcome, Cystic Fibrosis isn’t all just fundraising and dying – at least that’s what I’m hoping.
“Solitude is fine but you need someone to tell that solitude is fine.”
― Honoré de Balzac
There are parts of this illness that I don’t want to think about, let alone scare my friends and family by saying them out loud. Maybe this stems from being a middle child – I always want everyone to be OK. But I think it also relates to the media’s portrayal of illness and our culture’s expectations and discomfort surrounding “the sick.” We want to be inspired by their bravery, by their indomitable spirit, by their thirst for the life despite the tragic brevity of their own. It seems the worst thing a sick person can be is angry or depressed.
So, here’s where I come back to the idea of writing about CF. But in the end this won’t be a “CF blog.” A little because of the difficulty and discomfort mentioned above, but mostly because I’ve got more on my mind than just my illness. Remember, there’s a whole lot that goes on between the fundraising and the dying parts. When I’m not thinking about CF, I’m thinking about a million other things I want to write about, things I want to do, places I want to see, snacks I want to eat after work. But I will include CF from time to time in these posts because I can’t shake the feeling that I’m not the only one disconnected by illness. Maybe someone else is perusing the internet like I have countless times, wondering how others with CF are getting by. Wondering if they’re happy, if they’re healthy, if they’re funny, if they’re angry, if they’re scared.
And if they stumble across any of my forthcoming blog posts, they’ll see at least for me, it’s often a combination of all the above.