Mucus, Mortality and Music from Connecticut
Diet Cherry Coke is one of my two guilty pleasures. The other one I acquired in 2002, just a few days after my daughter was diagnosed with cystic fibrosis at the age of five months.
A friendly neighbor dropped by to offer help and good wishes. She made us a gift of a book about CF she had acquired at a yard sale or something. It was one of those cheapo-looking paperbacks, bearing the title Alex: The Life of a Child.
Thanking our neighbor for her kindness, I nevertheless thought to myself: Good grief. Disease lit. The very last thing I need right now.
Just before tossing this little pink book onto the household pile of unread volumes, I noticed the name of the author: Frank Deford. Could it be?, I wondered. The Frank Deford? It was 2002 and, by then, I had been an NPR fan for decades. And in 2002 every NPR fan knew the voice of Morning Edition sports commentator Frank Deford.
Unlike other sportswriters, including his colleagues at Sports Illustrated, Deford always seemed to have athletics in its proper perspective – ubiquitous, yes, but of little inherent value except insofar as it shed insights into the human condition and eternal suffering. I especially liked it when Deford did one of his “sports curmudgeon” editions of his public radio commentary.
I devoured Alex and in the process figured out why Frank Deford always had sports in perspective. He and his wife Carol endured the death of their daughter Alex, in 1980, at the age of eight, from CF. It was a very compelling read.
Not only did I resolve to be the kind of CF dad that Deford was – compassionate, doting, an incisive chronicler of his daughter’s tragic odyssey, a tireless and passionate defender of her interests both publicly and privately – I also picked up the habit of reading any other accounts of life with cystic fibrosis I could find.
Turns out it’s quite a subgenre. My most recent adventures in it include Salt in My Soul: An Unfinished Life by the late Mallory Smith, Gina Frangello’s 2014 novel A Life in Men (about the poignant European hijinks of a young American woman with CF), and even the novelized version of the 2019 movie Five Feet Apart about a couple of hospitalized CF teens who defy the onerous CF infection control protocols and fall in love. (A better title for that one might have been Impatient Inpatients: An adolescent love story.) I just started Elizabeth Scarboro’s My Foreign Cities, about loving someone with end-stage CF.
All this reading has made me wonder: What’s so special about Connecticut? I ask because my two favorite examples of CF disease lit are both compelling accounts of trying to live with the disease in the Nutmeg State.
Deford is the Jane Austen of the genre. A patrician, Princeton-educated writer, he lived with his former fashion model of a spouse in a wealthy Connecticut suburb. Alex had the best CF care available anywhere, dying nevertheless.
Connecticut’s other noteworthy CF author is Jay Gironimi. He is the Raymond Carver of CF disease lit.
Born in 1983 to a working class family – his mother was employed for nearly 30 years at a drugstore chain – Gironimi wrote a book that eschews poignancy in favor of practical wisdom about how to survive and thrive with CF when your parents get divorced, when your health insurance runs out, when you’re working as the night auditor at a hotel, or when you’d rather be practicing with your heavy metal band. (In Gironomi’s case, the ensemble is called All Hallows Evil.)
His 2013 book is Can’t Eat, Can’t Breathe, And Other Ways Cystic Fibrosis Has F#$%*D Me. Judging by the number of F-bombs Gironimi cheerfully drops into his prose, I assume the use of “F#$%*D” on the cover is a rare concession to mass media sensibilities, though it makes the title awfully difficult to pronounce.
I discovered Gironimi via his prominent role, as the sole voice directly from the CF community, in the two-hour cystic fibrosis edition of This Podcast Will Kill You. The co-hosts, epidemiologists/mixologists Erin Welsh and Erin Ullmann Updyke, chose Gironomi for precisely the same reason I admire him: He hates the idea of being a poster child for CF.
In this respect, Gironimi is the first CF author whose take on the disease aligns almost perfectly with that of my daughter. They are both entirely willing to talk about CF when prompted, but would vastly prefer to be known in the world for other skills and accomplishments. (Gironimi, for example, wrote and performed a song for This Podcast Will Kill You.)
To which you might well reply: Duh! Who wants to be famous for the sticky mucus in their lungs, or the fetid and runny signs of pancreatic insufficiency?
Fair enough, but for me it took a bit of work to understand, and make peace with, this perspective. I read Deford’s book and was inspired to emulate him. Since then, if I’ve learned anything about CF, it is that to thrive with this disease is to summon the kind of fortitude and resilience that is sorely lacking in most quarters. In his remarkable 2004 New Yorker article about cystic fibrosis, called “The Bell Curve,” Atul Gawande identified three qualities – focus, aggressiveness, and inventiveness – that account for CF-related success, in patients, caregivers, and researchers alike. I wanted the world to see me deploy these very qualities in service of my daughter and hoped she would feel likewise about cultivating and thereafter broadcasting such qualities.
In fact, my daughter personifies those attributes, much better than her dad does. And when pressed, she will allow me to use her image and a bit of her story to raise money for the Cystic Fibrosis Foundation (CFF) or, more recently, to agitate against the usurious prices of certain CF pharmaceuticals. But she laughs about it, and rolls her eyes. Which is why I, in turn, just loved it when Gironimi, during his interview with the Erins on This Podcast Will Kill You, had the audacity to make light of the CFF’s eternal and somewhat cloying “65 Roses” marketing effort (spun out of a cute CF kid, years ago, bringing his mother to tears by innocently mangling the odd name of the disease).
Everything about Can’t Eat, Can’t Breathe is delightfully to the same effect.
“I’ve grown comfortable in the old jalopy,” Gironimi writes of his CF-challenged body. “However, I do not want anyone to mistake that comfort for bravery. I feel like bravery implies that you had a choice and you took the courageous path. I don’t have a choice. If I did, you can be damn sure that CF would not be that choice. I’m sure it’s possible to face these challenges with bravery and grace, but I never cared to try it myself.
“I’ve also never viewed it as a test. I know some people do. Those people may actually be brave. I am not one of those people. I’ve learned from CF but I’m sure that the world could have found another way to teach me how to take pills.”
What a refreshing break from the fame-seeking professional CF patients out there. I refer to people like Gunnar Esaison (the blogging, self-promoting son of a famous NFL quarterback), Emily Kramer-Golinkoff (whose family started a foundation to raise money for beating rare CF mutations), the late Claire Wineland (who gave inspirational TED talks wearing her nasal canula), and Mary Frey (who sells merchandise and makes earnest videos about CF that cheerfully align with the spiritual convictions of her minister-husband). Other than the fact that some of them have drunk too liberally of the Big Pharma Kool-Aid, there is nothing wrong with any of these folks and the many others like them in the CF community. But when it comes to the silent majority of people living with CF, I suspect Jay Gironimi’s voice is the more representative one.
Hence my reverence for an anecdote appearing in Gironimi’s chapter entitled “The Fun of Failure.” It involves a time when young Jay is trying to pack in the calories so as to gain some weight (a perennial challenge for the pancreatically insufficient). One evening saw him swilling down copuious amounts of a disgusting slurry consisting mainly of weight-gain powder, and then reporting for work at his hotel-based night auditor job.
What follows is a detailed account of an ordeal that eventually caused the hotel to pay a plumber $240 to get the toilet to which Gironimi bolted running again. He compared the experience to giving birth to a “slightly premature child,” although managing to drop a pen down the toilet during the episode also contributed to the need to ring up the plumber.
Another CF author might have told a similar story, though perhaps less floridly, but would surely have included an inspiring denouement. Not Gironimi. “Luckily, the bathroom was in a public area and could easily be blamed on a guest,” he confesses by way of conclusion. “It turns out that in the battle of mind over matter, matter will eventually win – particularly if it is fecal.”
Compare that to my favorite anecdote from Deford’s book.
Toward the end of her life, Alex endured a series of painful procedures to fix her frequently collapsing lungs. As what was apparently the very last of such ordeals approached, Alex balked and panicked in the procedure room but her dad was there to hold her hand and calm her down. But just as the medical team was about to begin, Alex once more insisted on a pause. It turns out that her usually stoic dad could not quite maintain his composure as he watched his sweet little girl prepare to endure more suffering, so he had started to cry a little. Alex just wanted a second to wipe away the tear she had noticed rolling down her father’s cheek.
“Oh my little Daddy, I’m so sorry,” she whispered.
The two veteran nurses in the room just lost it at that point, Deford reported. I have to admit I start to tear up every time I read or even think of that story myself. For a CF disease lit afficionado like me, there is plenty of room to cry with Deford and laugh with Gironimi.
To some degree, the difference between Alex: The Life of a Child and Can’t Eat, Can’t Breathe is the difference between being born with CF in Connecticut back in 1972 and the same story unfolding just 11 years later. Alex Deford lived and died at a time when essentially nothing was known about how to treat CF; some of what they did in those days actually harmed rather than helped. Gironimi gets to joke about “smoking Pulmozyme in the bathroom,” referring to nebulizing the mucus-thinning drug that came along in the 1990s – the first in a long series of life-extending CF therapies that have been rolling out ever since. Indeed, since Gironimi is heterozygous for the most common CF mutation (508Fdelta) he is presumably eligible for Trikafta, approved last year and hailed by many as the biggest game-changer of them all.
Ultimately, though the stories diverge and the authors’ voices are markedly different, in one key sense these two classics of CF disease lit converge.
Fairly early in the story of Alex, Deford offers four specific nuggets of advice for CF parents when their kid is in the hospital. Since the first three are jokey throwaways (there’s always something wrong with the elevators; memorize your Social Security number; the hospital billing department will inevitably screw up), it’s obviously the last item on the list that Deford really intended to stick.
“You have to use one word all the time,” Deford wrote. “That word is ‘why.’ . . . No matter what anybody in a hospital tells you – and especially if it involves your child – ask them why. . . . and if that doesn’t obtain satisfaction, ask why again. You will never regret it.”
Gironimi makes a very similar point, but since he’s the one with the CF he gets there from the patient perspective. “It’s much easier to think everyone knows what’s happening with you than to actually explain it,” he writes. “This is what makes screaming ‘You don’t understand!’ seem like a good idea. Often the reason people don’t understand is because you didn’t tell them.”
“Think about how often we just abort communications when it becomes too much of a struggle,” adds Gironimi, confessing that he does not know how to explain what it feels like to have lungs full of nasty, sticky mucus. He also admits that although he knows how to fix computers, he has trouble explaining technology to others. He worries about what this means for situations where there is much more at stake than a broken laptop.
“The answer to this problem is questions,” he concludes. “Ask questions. Don’t ask with any agenda other than to start a conversation. If a person wants to answer it, they will. If they do, all you have to do is listen. If not, try asking another one. If they tell you to f*#k off, then f*#k off for a little while. You can come back and try again later.”
So I raise my glass of Diet Cherry Coke in tribute to these two remarkable Connecticut authors. Neither they, nor I, nor my daughter would ever claim there is anything good about cystic fibrosis. But the stories are good and, so far, these two are my favorites.