Cf on the Big Screen

Great Divide

A contentious red hot debate is smoldering in the cf community over the movie Five Feet Apart, now in theaters. Cystic fibrosis (cf) is the central feature of this feature film. And it has polarized patients and their families in a way almost reminiscent of American politics. It’s been interesting, if not unsettling, to see sparks fly in the public arena among our relatively small population of individuals who normally just knuckle down fighting the good fight for their health with diligence and dignity. What’s got people so riled up? Why is the movie so controversial?

In one camp we have naysayers, a group of vocal high profile patient advocates (including Gunnar Esiason and friends). They’ve used their platforms to pan the movie from the start. My take is they legitimately feel disrespected, perhaps endangered, too, by the notion of a forbidden love between two star-crossed teenagers who both have cf. Their argument is Hollywood will not only romanticize the disease and capitalize on people suffering with chronic illness, but promote a dangerous disregard for infection control practices to the cf community. The critics resoundingly say, “no, this is not the real cf.” A media blitz this past week leading up to the opening resulted in a veritable PR war between outspoken critics and the film studio.

In camp two, other community members are abuzz with excitement and emotion. Fans post support for the arrival of the first ever mainstream production about cf. Above all, supporters value heightened awareness thanks to the film. They feel it will start and enhance conversations about what life is really like for people fighting cf. Of any inaccuracies, they’re likely to say, “folks, it’s only a movie.” And no matter what, this movie gives everyday people a clear, though narrow, window into the isolated and grueling world that is cf. What The Fault in Our Stars did to elevate the plight of a different patient population, this one’s for us.

We can’t forget about a final camp. Of course, people with a firsthand connection to cf are not the only moviegoers. Fans of romantic weepie dramas starring a heartthrob like Cole Sprouse of Riverdale popularity are bound to be at the theater. Plenty of young people will show up at Five Feet Apart for sheer entertainment, but they will leave with an enduring impression – probably their first – of what cf is all about.

Where We Stand

Our hearts guide Av and me to align mostly with the second camp of supporters. When the first trailer came out, she squealed “OMG, Cole Sprouse has cf!? This is 14. She has invited me to go with her (which is pretty sweet), so our plan is to watch the movie together as soon as the moment is right.

Av told me of her first positive Five Feet Apart effect. A school mate caught her popping pills when she was eating a snack during History class. He whispered, “why are you taking those?” She said, “because I need them.” He asked, “OMG, are you alright?” Her answer was, “All good. Ya know, ‘five feet apart.’” She gestured towards herself. His eyes popped, but then he nodded at her in awe with fresh understanding. We’ve got genuinely interested kids as well as those who’ve merely heard about the movie who now recognize the words cystic fibrosis. I’m grateful Av and other teenagers with cf can use their enlightenment as an ice breaker.

At first it seemed strange, random even, to imagine someone making a romance out of a decidedly unsexy medical condition like cf. But to know some of the backstory is helpful. Five Feet Apart is dedicated to the late Claire Wineland, a young woman who fought cf, a popular vlogger and advocate. She died last fall at 21. She was close friends with the movie’s filmmaker, actor and director Justin Baldoni, through an earlier making of a documentary about Claire’s real life. Justin was so inspired by her, he joined the ranks of cf allies to help carry on her legacy. In my view, Claire’s involvement in the early stages of the movie gives real credence to the idea of sharing an inside look at cf life with the wider world. It’s easy for me to stand behind a force like Claire and her enduring messages of positivity in the face of her own demise. It’s easy, although incredibly heartbreaking, to see her reflected in the female lead in the movie.

Myth-busting

I confess the reason Av and I already have a pretty good read on the plot and setting in Five Feet Apart is we already devoured the book. Between the book and trailers, we know basically what to expect from the film. We do understand where some of the trouble lies with the depiction of cf in a fictitious, romanticized, hospital-based storyline. While we and other insiders can distinguish what’s fact from what’s fiction based on firsthand knowledge and experience, how about folks in the third camp – the general public – who decide to take a chance on Five Feet Apart? This is what I would want them to know:

  1. Hospitals are not like a cruise ship or Melrose Place for cf patients. We used to call Av’s inpatient stays “hospital camp.” We would decorate her room with twinkling lights, artwork, and special pillowcases to seem as homey as possible. We brought in sketchbooks, games, and movies; and spoiled her with care packages. Ordered all the treats she wanted from room service. We got friendly with doting nurses, respiratory therapists, and other caregivers. At moments, she could feel like her hospital room, along with hidden attractions she explored during exercise with physical therapists, were a cherished haven away from home. That’s the extent to which we can relate to the characters’ hospital life in the movie.
    Here’s the real deal: patients who are admitted for treatment for cf would not be hanging out five feet, six feet (the actual rule), or any feet apart. They would instead be isolated in their rooms on contact precautions and probably never lay eyes on the living, breathing beings on the other sides of the walls. Five Feet Apart does a decent job presenting the premise of deadly cross-infection. I guess it’s plausible that stealthy teenagers with crushes might sneak around, but hospitals would never knowingly allow patients infected with all manner of cf bugs to roam freely in and out of newborn units, swimming pools, or multi-purpose rooms. Patients with cf don’t go to the hospital gym to work out together. It’s pure fantasy that a patient would get away with “renting out” his bed so friends from home could come and make out. Patients with cf don’t get to sit around under the door jambs of their hospital rooms shooting the breeze from across the corridor. Truly. Sadly. This is a Five Feet Apart reality check.
  2. Not all cf parents are messed up and absent. I’m sticking up for my cohort here. As far as I’m concerned, it would be highly irregular to see a cf parent click through the halls of the hospital in heels before jetting away for weeks on end without once visiting her terminal kid. Granted this story is told from the perspective of teenagers, who we know on rare occasions to be self-absorbed and annoyed with parental types. (Ha.) The parents in Five Feet Apart serve as convenient secondary figures. Their primary role is to add further grief to their children’s already fragile psyches because of their own heavy personal issues, including anxiety, grief, and marital problems. What I’ll say about this is, aside from a shared hyper-vigilance, cf parents are as fiercely individual and complicated as our children. We come from all backgrounds; we have different interests and occupations. We approach parenting children with chronic illness in our own unique ways. We try our best, but we disappoint sometimes. We are also more than likely wearing yoga pants as we are camping faithfully, night after night, by our children’s bedsides in the hospital. And we’ll be there still, even once those children morph into sleepy, sneaky, snarky teenagers.
  3. Sudden and scary health emergencies rarely happen without warning to people with cf. Spoiler alert. Someone in Five Feet Apart is going to die. (Because, of course.) We know it’s true that cf is a killer. Young people tragically pass away from the condition far too early. Claire Wineland is one of countless angels. But the typical way cf progresses is insidiously slowly, albeit with intermittent sharp declines. From what I know, patients suffering from end stage cf would be quite sick and quite immobile; they either succumb to failing lungs or must undergo lung transplantation, which carries its own inherent risks. Maybe the plotline will play better on screen than in the book, but I’ve never heard of someone with cf being well enough to cook dinner and hang out with friends within hours of collapsing and perishing. The loss in the movie feels like a so-called cf ghost story.
  4. Cf is a thief. This theme is 100 percent true. Cf steals time, time, and more time. Time and again. It steals precious hours and years. It steals school days. And trips. It steals freedom. And dreams. And hearts. Breath. And life. Yeah, Five Feet Apart nailed this one.

Av and I are going to see the movie. It remains to be seen whether we’ll give it two thumbs up, but we are hopeful. Strategically, we’ve decided to wait and try to go next week when the early rush will probably have quieted down at the theater. As usual, we’ll bring enzymes for snacks. Hand sanitizer, always. We’re bringing tissues. And something more. Because we know other curious souls with cf may be there, too, Av will have a mask to wear. If we suspect a close encounter with another person with cf, we won’t hesitate for a second. Av will need to move away surely and remain no less than six feet apart. It’s just another day in the real life with cf.

Hole in Her Safety Nets

On the eve of her big journey to the Netherlands, according to plan, I was spreading on the dining room table all the contents to be packed into Av’s therapy backpack, when it hit me like a brick: the refill she needed of Orkambi had never shown up. It was already after hours on Friday evening! I had been expecting that medication on Thursday morning! Since overnight packages tended to be reliable, I previously checked off both mental and written packing lists. What could possible have happened between Thursday and Friday to cause me to overlook something as essential as a missing Orkambi delivery?

Oh.

Over the last 18 hours, I had single-handedly juggled the end of my first week in a new office, the kids’ early release for the start of spring break, an eighth grade conference, a kindergarten conference, a high school parent meeting, and a Thai dinner out. That all happened successfully, yet no delivery.

I scurried to login to myChart, and shot a concerned message to our pharmacist who manages Av’s drug orders. I stated the predicament flat out, “Hello, I realize there’s nothing to do about this on a Friday night, but…”

Orkambi is one of the groundbreaking treatments known as a cftr modular, which to some extent corrects the genetic defect that causes cystic fibrosis. Since Av added it to her regimen after she turned 12, we give it credit for a boost in her lung function, and even more, for stabler health. The thing to understand about Orkambi is, it’s a deluxe, precision medicine exclusively for select cf patients; its annual price tag could buy you a decent house in a small town. Unfortunately, you can’t just pick this stuff up at CVS.

We were in a pickle. She was in a pickle. For all the well-laid preparations, she would have to go to the Netherlands with a gaping hole in her safety nets. I could continue flipping out, I could seethe, or stress, or I could:

  1. Exhale and let Av know about the missing medication. When I told her, she launched into a rendition of a Scout song she made up a few years back on a camping trip. The refrain goes, “Iiiifff we die we die together…” I joined the chorus, which made us giggle for about half a minute. I then asked her, assuming she would be traveling with what little Orkambi she had on hand, whether she thought it better to take the regular dose for five days and quit cold turkey or halve the dose and spread it out over ten days, almost the length of the trip. She would decisively opt for the latter.
  2. Dial up the pediatric pulmonologist on call at the hospital over Friday night to ask if there was any shot whatsoever in hell of acquiring an Orkambi refill from a specialty pharmacy before 10:30 a.m. on Saturday morning? The answer: highly unlikely, but he would check first thing and get back to me. We discussed the dosing plan (he agreed with Av) and he reassured me she would survive this, too.
  3. Exhaust my limited courses of action, and then put the problem in perspective and accept the change in circumstance. Av traveling without enough Orkambi is pretty much a nightmare come true, but it could be even worse. Missing digestive enzymes, for instance, would be a far more painful prospect. So there. The whole trip is one giant leap of faith anyway.

What lesson, aside from the dark side of procrastination, was I reminded of on the eve of Av’s trip? (All you cf parents out there: order those refills early and often!) No matter what, we can’t let our foe get the better of us. We must balance the endless string of challenges cf presents us with enough levity for our own sanity. Take it from my gal. When she boarded the plane, she had a brave smile on her face, maybe a tear in her eye, my kiss on her cheek, and a ridiculously adorable campfire song in her heart… “Iiiifff we die we die together…

Traveling Heavy with Cf

We’ve got traveling on our minds. Among other exciting trips our family has planned this year, Av herself soon embarks on an adventure to the Netherlands over spring break with her granddad (aka Opa) and cousin. While she’s a seasoned traveler, this is her first time going away anywhere for any length of time without me coming along. And there’s only one way she can pack: heavy. I’ll try the translation in Dutch: dat is zwaar. You see, with cf, I know of no humanly possible way to travel light.

We are destined to pack large. Our dog weighs 70 pounds and growing. Lou still needs a booster seat. Phil usually wants to bring along a canoe or a chainsaw or something. Since Av is a cellist, we’ve figured out precisely how to wedge the cello case into the back of our small sportwagon without crushing the bridge, which leaves room for…not much of anything else. On our big road trips, I’m typically the one in charge of packing up the whole cf show. Having over a decade of practice, and given an uninterrupted hour or two or half a day, I can expertly compress every element Av needs for her cf care into just three pieces: a 66-quart storage bin, a 15-pound roller duffel, and a cooler. These vessels take up merely three-quarters of the rear cargo hold. Who cares about clothes anyway?

In truth, the heaviest baggage when traveling with cf is not so much the luggage as the forethought and fortitude required. The dream would be to send my gal off to bike along the canals of Holland carefree. Visiting her Dutch relatives with nothing more than a sketchbook in hand and a passport in her pocket. The reality is, traveling and cf aren’t that compatible. If she ever wants to go to away, whether on a sleepover, to a sleepaway camp, or to college for that matter, the predicament is how to depart from the regular home routine of cf therapies. Seems like we always scramble for pills and a modified treatment schedule because, as well prepared as we try to be, spontaneity and cf still conflict. The central problem boils down to: cf never stays home.

Last time in Amsterdam

Of course, cf also never takes vacation. And so, off it must go as Av’s constant companion. If you’re curious about how much daily responsibility she’s going to have over the course of her Netherlands stay, check out the “quick” care tracker she and I drafted together. It may be a hassle for her to fit in so much therapy while traveling, but at least it will come second nature.

Av’s first solo voyage is bound to be not only her own rite of passage, but ours. Can you hear the echoes of “time to let go, let go, let go…?” Indeed, we’ve no choice but to trust in her abilities to care for herself. We trust she’ll return home to us in good, if not good enough, health. Should cf so happen to prevail and she gets sick, well, I’m sure we’ve got extra treatments for that. What’s most important at this juncture is her embracing an awesome adventure she can cherish for a lifetime. The world beckons to our intrepid young traveler. We are wishing her not only a goede reis, but blessings on her journey.

My Messages for the Newly Diagnosed

I recently connected with a local mom who, like me, has two beautiful daughters. Her baby girl was born with cf. She invited me as an “experienced” cf mom; along with a social worker and respiratory therapist from our center, and staff person from our chapter; to join in an evening gathering at her home to talk about what life is like with cf in the presence of her closest extended family and friends.

The purpose of the evening was to inform the inner circle about how best they could support this family and their little daughter. But the benefits of the conversation were exponential. I can’t think of a smarter, more inspired idea than calling in your reinforcements during those early days of cf parenting. Those are the days when you’re still fretting over your baby’s every gurgle and wondering what warrants calling the doctor. Those are the weepy days of wondering when or if you’ll ever feel (back to) normal. Frankly, you’re wondering if anyone else can adequately learn how to care for your child with so many complex medical needs. You’re seriously doubting you’ll ever get another date alone with your spouse.

This family welcomed me warmly into their home on an icy winter night. We shared in food and wine before settling comfortably in the living room to talk. We gazed upon the baby who kicked and cooed happily on a blanket on the floor. While we can predict certain things about cf, we can’t know what’s in store for this child, what challenges her future holds. Only one thing’s for sure. The goodwill of the exceptional people who gathered together in that living room is sure to carry her through.

Here are the thoughts I shared with them:

  1. You can still be the parent, or family member, or friend you are meant to be. You just add on an extra layer for cf. Think of cf like a blanket, albeit sometimes a wet and scratchy one! My advice is to stay true to your parenting style. Hold fast to your family traditions. Keep the pastimes you love in your life. Keep traveling. You just have to bring along the blanket.
  2. Your child can still be the person she is meant to be! Remember, cf is now a manageable condition people survive with thanks to advances in treatment and care. Grown-ups with cf often say their disease does not define them, but is only a part of them. Give your child any opportunity to pursue her dreams. She just has to bring along the blanket. For a long while, you’ll be there to help her carry it.
  3. There is no denying cf requires a lot of extra time, work, emotional and physical energy, organization, money, and more. These factors will become your new normal. Be ready. Because before you know it, you’ll be cooking nebulizer stew every night for at least the next eighteen years. And you’ll get so good, you could do it with your eyes closed. Guaranteed. You’ve got this!
  4. Cf will absolutely change any old easy-going ideas you used to have about germs and cleanliness, and illness and medication. You will panic every time you hear about a sick classmate or cousin. You will never again forgo a flu vaccination. You will never leave home without having enzymes and hand sanitizer in your possession. Never ever. Welcome to the hyper-vigilant club.
  5. Cf will change you forever. It brings the whole family plenty of heartache, but also silver linings, and maybe even a greater purpose. While much of cf feels uncontrollable, remember the parts you own. You can:
    1. Establish a good routine early-on for your child and your family;
    2. Make adjustments as you learn. Once you know better, do better; and
    3. Jump into cf events to feel like you are actively helping your child.