Two good reasons to see Five Feet Apart

We went to the late show on a Thursday at Chapel Hill’s most deluxe theater. Not crowded, so no concerns about sitting too close to other moviegoers. No need to mask up (just for a photo opp!). From the first moments of the film, Av and I were transported. A compelling cast of actors had us hooked. The familiar hospital world captivated us. The slow-burning tender-love-and-care under the most difficult of circumstances touched us. Look, we’re partial. But I’m sufficiently convinced Five Feet Apart is a tribute, not a transgression. Skip the book and see the movie for:

  1. A snapshot of the treatment burden in cystic fibrosis (cf). The movie treats us to instructive, technicolor scenes of how most people with cf must care for themselves everyday. The depiction reflects not only inpatient lives but home lives of so many real cf fighters. Av and I are familiar, if not intimate, with those carts of pill bottles, treatment schedules, nebulizers, airway clearance, calories galore, feeding tubes, coughing, spitting up globs of mucus, shortness of breath, research trials, long-term IVs, surgery, scars, oxygen. Frankly, I’m fascinated by the notion of cf care as a central feature in a major motion picture. While the movie is no documentary, it’s more technical than your average flick. And as we easily anticipated every twist of the neb cup and rhythm of the regimen, we also realized this stuff might not intrigue everyone. Case in point: a pair of girls sitting near us in the theater who seemed way more interested in the texts on their phones than in the medical minutia of cf.
  2. A glimpse of the emotional toll cf takes. The movie weaves throughout its story the central characters’ reckoning with their own mortality. Seems unreal, right, to think seventeen-year-olds must grapple with what it will be like to die; how little time they’ve got left; or how to cope with survivor’s guilt. Sadly, for so many cf fighters, this is a part of life. Add on more layers for cf’s thievery of freedoms, which most healthy people take for granted: the freedom to leave (the hospital), to love (the one you cannot be with), and to live as fully you desire. This film gives us a striking portrayal of the sadness and anxiousness behind such tremendous sacrifices. I was deeply moved by it; anyone with a pulse will leave the theater counting their blessings.

Av’s and my final recommendation: watch Five Feet Apart. Learn it, feel it, and join in the conversation.

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.

Common Cold

Not runny
Not drippy
Not sneezy
Just pressure bout to explode my head

All I want to do is

Saw through the top of my skull
Tilt open
Start scooping the goop
Just scoop and scoop and
Remove all the goop with an ice cream scoop
Into the bowl

And next I need a

Butter knife
To sliver off polyps
Like butta
One and five and ten and
Plop go those nuisances
Into the bowl

Not finished

Slice straight down the middle of my nose
Open up wide like a door
Drill precise little holes in left cheek
Holes on right
Use a straw to
Suction and slurp and
Squirt the gunk
Into the bowl

Cement
Mixed
Pour and coat and
Coat and pour the cement
So smooth so
Polyps don’t grow up-in-here no more

This is an old-school
ENT-style
Cf freestyle
Dee out

Life, interrupted

Life

My excuse for taking an extended break from the blog is we were preoccupied the whole summer with traveling in between regular work and play and preparations for the girls’ upcoming school transitions. On two big car journeys (out West and up North, respectively) we covered thousands of miles to traverse 28 states in the course of visiting 5 national parks, a number of scenic state parks, and our favorite coastline. While cf accompanied us as usual along every leg and stop of the way, I’m grateful to say it stayed quiet enough in the backseat for us to enjoy plenty of unfettered driving, exploring, trekking, sightseeing, camping, packing, and family time. Yep, our summer was bright and busy and fun. It was a bucket checklist kinda summer.

Life is grand

Now as we welcome fall routine, Lou is settled comfortably, growing and thriving in first grade. Av has found her tribe at an exceptional public magnet arts high school. Our days demand a lot, but mostly, everything is just swell.

Interrupted

Everything was well. I was beginning the long exhale of having both daughters finally back in school when cf wreaked havoc once more.

Av is dealing with an adverse drug reaction, a complication surely landing high on the list of alarming setbacks patients with chronic illness and their parents must face. Its onset comes fraught with questions, concerns, and regrets. At the heart of the matter is: my child’s acute physical pain and safety; potential long-term effects from a course of medicine that was only supposed to help, not hurt; and a steadily shrinking list of available treatment options for the future.

We’re convinced the culprit was a heavy-hitting two-week course of oral antibiotics, previously tolerated, but which has left Av saddled with mind-numbing, debilitating tendinitis of the wrists and knees. Since when did a kid treated for a lung infection wind up in wrist splints and knee braces? This is a reckoning with risks versus benefits.

Life interrupted by tendinitis means her participation in JV tennis and orchestra programs halt; a relatively fit 14 year old takes the elevator instead of stairs; and she struggles in first quarter of high school to keep pace with note taking, essay writing, art projects, and a CPR unit. All because of her medical treatment. It’s frustrating and disheartening to stand by knowing she’s in pain as she tries to sleep, write, draw, play cello, play sports, catch the bus, do therapy, and otherwise move normally about her life. It seems so unfair.

Yet when have we ever known cf to be just, considerate, or discriminate.

I hope eventually we conclude, this ill-timed adverse drug reaction rudely interrupts her regularly scheduled cf life to bring lots of extra strength and resilience.’

Hats

My remarks during the opening ceremony of our cf walk yesterday…

Happy Great Strides day, everyone! This looks and feels just like a big birthday party! We are here to celebrate Great Strides with all our CF fighters…including my daughter. Instead of wearing a party hat, I think I’m going to stick to my old well-worn Aviary hat that says ‘cure cf.’

This morning, I actually want to talk with you some more about hats. Like many of you, I wear many hats. I wear the hat of daughter, sister, wife, and friend. I also wear the hat of mom to my two daughters, Av and Lou. I wear the hat of a CF mom for Av. I wear the hat of her caregiver. And now that Av is a teenager, I am proud to say I wear the hat of her partner. We have reached the incredible milestone where she and I now share in many of the responsibilities of her care.

As for her care, it’s never a party. It’s no fun for her to be constantly fighting aggressive infections. It’s no fun watching your daughter’s disease progress with every passing year.

But there is some good news, too, in the world of CF. Av would tell you, since she started taking one of the breakthrough treatments called Orkambi, she is breathing a little bit easier. It’s the CF Foundation that drove the research and development behind Orkambi in large thanks to fundraisers just like this one. Breathing easier is a gift. And that’s the best reason I can think of to be celebrating on Great Strides day.

Let’s circle back for a few more moments to hats! One of the most meaningful hats I wear is the hat of an advocate. I used to think being an advocate meant going to speak with our congressman about CF, and so we did that. Later I learned that being an advocate also meant standing up and speaking up for my daughter when she was sick in a hospital bed. I’ve done that.

But do you know what else makes an advocate? An advocate wears the hat of a walker. The hat of an awareness raiser. The hat of a fundraiser. An advocate is a champion who raises his or her voice for a cause she or he believes in. Do you know what that makes you? Because you are standing here today celebrating Great Strides with me and my daughter, you, too, are a CF advocate. Together we are amplifying the call for even better treatments for our loved ones, who valiantly fight cystic fibrosis, and one day, for their cure.

Fellow advocates, to borrow a line I hear often from our walk director: thank you for all you do! Hats off to you and happy Great Strides!

 

Appearances Can Be Deceiving

I won’t forget a time, years back, when I recoiled at the notion my daughter would always be sick. We were at a routine cf clinic visit. After the doctor had seen her, one of the therapists waltzed into the exam room and greeted Av by saying sympathetically, “I hear you’re sick.” Geez, really. Did she have to use that word? I wished this person hadn’t planted a single seed of concern in my young gal’s psyche. Could she not see how very well she looked?

Granted, Av happened to be coping with a little extra cough that day. The doc was putting her on more antibiotics. But despite some pesky symtoms, she hadn’t slowed down a bit. She was keeping up with school and activities as usual. She wasn’t home in bed with a fever or something. She would take her medicine and be good as new within days. Sick? Av was not living life like a sick girl. I simply could not equate a little increase in cough with the state of her being sick. Also…denial ain’t just a river in Egypt. I guess even a cf mom is susceptible to falling down the invisible illness trap.

When both my daughters were about four years old, they were obsessed with reading a thin paperback storybook about The Berenstain Bears, which still remains dogeared on our bookshelf. You Berenstain fans may know the cubs (along with Papa Bear) always learn morals, and in this particular story, Mama teaches “that appearances can be deceiving.” She explains, “It’s just a grown-up way of saying that things aren’t always what they look like.” I admit, for me; as a grown-up, parent, and caregiver, all three; she makes a pretty ominous point pertaining to chronic illness. One of the most confounding aspects of cf is remembering that inside the beautiful facade, a war rages on. Some small battles are won from time to time, while others are lost forever to the triple threat we know as persistent infection, insidious inflammation, and permanent scarring.

What does sick with cystic fibrosis really look like? Sometimes sick shows its cards with its hallmark thunderous, juicy, body-quaking coughing fits, accompanied by globs of sticky mucus. At other times, sick is subtle, sneaky even. Sick is a breath a touch too crackly and constricted; a complexion a mite more translucent and sallow; a voice puffing from deeper within the windpipes. Sick is tired from snoring and waking up already fatigued. Sick is throbbing pain to the head, the tummy, and chest. Sick summons up anxieties about time. What time it is going to take her to fight yet another battle. What time she will lose to the chronic illness lurking inside.

Appearances can sure be deceiving. Cf fooled me once, but no more. While the battles continue to ravage lungs, pancreas, and sinus on the inside, if you happen to blink, you might think you’re only seeing a picture of health.

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.

Superlatives

When I spoke about our family’s cf story to a group of community members at a Great Strides kick-off meeting, I decided to chronicle more than thirteen years by focusing in on the big moments: the life-altering moments, the critical moments, the best, the worst, the scariest, the most hopeful. Here are excerpts from my talk, presenting our cf story in superlatives.

Most shocking: Reminiscent of seeing the Challenger tragedy on television when I was in sixth grade, or learning about Princess Di’s death over shortwave radio when I was in Peace Corps: in early 2004, during an ordinary drive from home to office, one of the most indelible, unexpected moment of my life occurred. I was pregnant at the time, expecting my first baby! I received a call from a doctor I’d met only the week before. I figured she was making a routine call to share the results of my amnio. I was partly right. She had results, just not routine.

I can’t say for sure whether we were overly-cautious or overly-curious first-time parents when we elected to do amniocentesis. The procedure gives information about a baby’s DNA via the amniotic fluid. It all started for me with an abnormal prenatal test result (moms may remember the triple test). We followed up with a diagnostic ultrasound in which echogenic bowel appeared. With echogenic bowel, the baby’s intestines appear white on ultrasound when they should not, as if solid, like bone. Since Down Syndrome is one condition commonly associated with echogenic bowel we decided to proceed with the next, more invasive step, amniocentesis, despite the inherent risks the procedure carries. We hoped to rule out Down Syndrome, and we would. Little did we know, but echogenic bowel is a marker of other genetic conditions, too, and the doctor, wisely, had ordered additional tests of the amniotic fluid.

Fast forward to that fateful phone conversation with the doctor. The only part I remember quite clearly was the delivery of the news, very reluctantly over the phone, that the baby would have cystic fibrosis. I think we were both were speechless for a moment. I stumbled on a question to the effect of whether the baby would be affected. She would be okay, right? When the doctor hedged, my heart pounded. Her answer was, “She will be affected. And at this point, we can’t know for certain how she’ll do.”

I floated into my office in a daze to do immediately the last thing newly diagnosed parents should: Google cystic fibrosis. Because the truth was, I knew nothing about it. I had blown off the offered prenatal maternal screening. I had no idea a distant cousin of mine has cf. I had no inkling until that very morning I was a carrier. There I sat on my computer in a cubicle facing the shocking prospect that the baby I was carrying was not only going to be born with a serious medical condition, but she would most likely die from it. I retreated to the closed-door storeroom at work where I could cry freely to my mom over the phone. I was reeling and processing the shock with her. I remember exclaiming, “The baby may only live to be my age!” Because the median life expectancy at the time in early 2004 was my very age: 31.

Fortunately, predicted median life expectancy for cf has almost continued tracking my age. (I may feel older these days, but the prospect is still far too young…) Unfortunately, the day our baby was diagnosed in utero, I could not reach Phil right away. He was working in another time zone, in Africa, I believe, and FaceTime and Skype weren’t so prevalent back then. Once I got word to him, he booked the first possible flights home for us to be together. That was the beginning, literally and figuratively, of our cf journey.

It didn’t take long for us to feel grateful we knew about the diagnosis from the very start. We were as prepared for it as we could be at birth. Fortunately, Av did not have to deal with the complication known as meconium ileus (as the echogenic bowel might have suggested) or surgery. And she did not fail to thrive. What she had was a relatively normal and well newborn experience, with the exception of starting enzyme beads in dabs of applesauce before every feeding – usually offered to her on her dad’s pinkie finger – as of day three of her life.

Most risky: We don’t often throw caution to the wind, but we also don’t do the bubble. It’s not conducive to the travelers’ lifestyle! A few years back, Av went swimming head-to-toe in a scummy and stagnant green pond during a weekend visit to her best friend’s grandparents’ farm. We knew that activity was risky, but… priceless fun. And she paid the price with an exacerbation and extra meds quickly following suit. Coincidence or scum? We’ll never know. C’est la cf vie. Yet it was not the most risky time. We, her parents, take credit for that.

When Av was in relatively good health at the tender age of one year old, with enzymes still her only medication, we moved, with something of a blessing from her cf doctor back then (a man who also didn’t believe in bubbles), to live in Ghana, in West Africa, for an opportunity in Phil’s work in public health.

Our adaptable little toddler had a blast! Her days involved fresh pineapple juice, dancing to drums, swimming pools, and even the chance to observe elephants up close and personal. For us, we got a pretty serious blast of cf parent stress. Because once we arrived in Ghana, Av quickly got sick. It would eventually, dramatically, land us with her in a sweltering, rat-infested (no exaggeration) African hospital to start her very first IV. Our British pediatrician pulled Phil aside and asked him basically what the hell we were thinking in wittingly bringing a young child with cf to Ghana. Granted, the doctor had been practicing in Africa for thirty years, and her context for cf was still the old one, of cf as an elementary-age childhood disease. But we got the gist. We knew by then living outside the United States, particularly in the tropics in a place with such limited healthcare, was never going to work, and would probably never be feasible for Av.

Did she come down with her first lung infection in Africa earlier than she would have in America? We’ll never know for sure. What I know is the only answer was to cut our African adventure short, just shy of her second birthday. We headed home where she would be admitted for her first official tune-up, in a much better hospital. This was the beginning for her and for us of coping with persistent and progressive lung infections, reduced lung function, and the accompanying cycle of treatments and hospital stays.

Most empowering: The Cf Foundation offers us an excellent platform for doing political constituency advocacy. Many of us have sent letters to our Congressmen or called about issues affecting our community, especially this past year. About five years ago, when we were living in Metro DC, we took Av to spend an unforgettable day going to the Hill to meet with staffers for our Maryland representatives at the time. We had the guidance of a staff person from the Cf Foundation’s advocacy office. This was our chance to touch on Av’s cf story in short bullet points to help drive home high-level talking points in support of what we still want and need: accessible, affordable healthcare. We invited the congressmen to join the congressional cf caucus to help further the interests of the cf community. I’m glad to say our representatives in Maryland were already on board with Obamacare, the cf caucus, and all the rest at the time. I could not say the same for elected North Carolina officials today (but this is another conversation…).   

Capitol Hill filtered

I thought what we did on Capitol Hill, in a nutshell, was advocacy. But not long thereafter, I got another revelation about the true meaning of what it is to be a patient-parent advocate.

Av has always had exceptional care at each one of her three cf centers, except for this once…

She was inpatient having already had a bronch and starting a tune-up in advance of sinus surgery we were anticipating and planning for. We got caught up, seemingly, in a power play between the attending pulmonologist in the hospital (who was not her regular cf doctor) and her ENT. The point of conflict was: the pulmonologist decided in a vacuum of medical charts to cancel sinus surgery (or postpone by about a month) with consulting with or informing us, the parents. In our view, she threw Av’s entire plan of care under the bus. Whether or not she was right from a medical standpoint, we felt frustrated and betrayed by this particular provider. 

On the next rounds, in Av’s hospital room, surrounded by a dozen observing medical students, the attending physician heard from us unequivocally about her failure to provide patient-family-centered care, the practice in which, quite simply, patients and families get a say in decisions about their care. It was another indelible moment for me: standing up next to my child’s hospital bed to speak up for her when she could not stand up for herself. Not only was she was sick in bed, she was also still too young to do it. All I can hope is that that encounter opened eyes (as it did for us) and made an impression on those dozen doctors-in-training who witnessed it that July day in that cramped hospital room. They are, no doubt, in charge of patients today, and I hope they’ll do better.

Most distressing: Some of Av’s most baffling cf-related ailments have really driven home the meaning of the phrase “what doesn’t kill you makes you stronger.”

Like the time she had a terrible adverse reaction and appeared to have been in a fist fight with an antifungal medication. Black eyes, busted lip, the works. The drug definitely won the round!

Like the time I received an emergency call from her best friend, breathless and anxious, to say please drop everything and race to the neighborhood park pronto with antacid. It had been the very first time Av and her bud were allowed to bike to the playground by themselves. By the time I got there, my gal was writhing on the ground, moaning, and gripping her tummy and chest in pain. Her friend a true champ by her side (thankfully with that cell phone). Bikes strewn on the ground. Concerned onlookers staring from a respectful distance. What a scene. Av chugged Mylanta straight from the bottle and then I carried her home to ride out the pain in a bath.

Like the Thanksgiving we spent with Phil’s family when we had to plod through a snowstorm in the middle of the night to take Av to the ER because of the worst tummy pains yet. 

Episodes like those have rattled us and moved us. None of us ever wants to see our courageous, resilient children suffer. We are combating cf because we want more than anything to alleviate our children’s pain.

Most uplifting: I’ve had the privilege in recent months to connect with a couple of moms of newly diagnosed babies. One is from a local family who welcomed a lovely little girl. And the other is a long ago grad school acquaintance of ours living out-of-state, who remembered about Av, and reached out to us when her son was born recently with cf.

That mom has been struggling this month through her baby’s very first cold and cough. She’s been concerned enough with his lingering symptoms the doubt has started creeping in. She’s wondering whether the bright messages of hope and optimism communicated by the cf care team when the baby was first born were not totally overblown. Basically, this mom, this couple, is still finding footing in a new reality and they are feeling duped in the face of cf.

I encouraged her to believe this: believe the hope conveyed by the care team. She has every reason to be optimistic about her little son’s future, just as I am still for Av, despite having already coped with our fair share of distressing and discouraging situations. More challenges are sure to come, knowing cf. But I could reassure her, not only do I know how fierce we cf families and our children are, I also know we have extremely promising groundbreaking treatments in our back pockets.

Since Av started on one of those groundbreaking precision treatments more than a year ago, her lung function, and other functions, not only improved some, but stabilized. She – and we – can breathe again! She’s remained so stable, in fact, that at the moment, typical middle school drama dominates our lives more than health concerns! Some unfortunate eighth grade friend troubles managed to overshadow her third sinus surgery this past fall. Funny to say, but I think we’ll take it? Essentially, Av is navigating a completely normal stage of life, just as she should be at 13, of self-discovery; of learning her way in the world; of learning how to take care of her relationships and her own health. She makes mistakes (don’t we all), but I see her flourishing in so many respects.

I firmly believe: cf is just another layer. It is a heavy layer, sometimes dragging us down like a wet blanket, yes. But it’s one of our layers: always present, always challenging us, and enlightening us. Cf is one layer in our family’s rich, rewarding, no-regrets life journey.