The Stones Applaud -- But I Don't

If you're the parent of a little kid with cystic fibrosis -- and I am -- you can't help but root around for stories of how people have confronted this miserable, life-threatening chronic illness, and when you read those stories you cannot help but feel grateful and inspired. Today the life expectancy of someone with CF is approaching 40 years -- whereas, had I been born with the disease, back in 1958, chances are I would not have survived long enough to see kindergarten. My beautiful little kid with CF is going to outlive me by many, many years -- and, make no mistake, this will happen in no small part because generations of families who came before us endured heartbreak and agony that we will be spared.


One such family is that of Ed and Patricia Mullin -- who lost not one but two daughters to cystic fibrosis. The elder of their two CF offspring, Teresa Ann Mullin, died in 1991 at the sorrowfully young age of 22. But Teresa was a persistent young woman, and an articulate one. Not content merely to struggle against the ravages of CF, Teresa managed to get herself into -- and out of, as in 'graduate from' -- not one but two very prestigious and competitive academic citadels: Phillips Exeter Academy and Harvard. It was while Teresa was studying in Cambridge, and beginning a career in journalism, that she got the idea of writing her autobiography, The Stones Applaud.


Teresa's parents are likewise remarkably persistent folks, and so they soldiered for 16 years to get The Stones Applaud published. Finally, Providence House Publishers, a speciality publishing house in Tennessee whose web site makes clear its overtly Christian purposes, brought out Teresa's autobiography last year.


It is my regretful and reluctantly stated opinion that Mr. and Mrs. Mullin have done the memory of their beautiful daughter -- both of their beautiful daughters, in fact -- a disservice by publishing The Stones Applaud so many years after Teresa's death, at least in the form in which it has appeared.

The world of cystic fibrosis has experienced a sea change since Teresa graduated from Harvard in 1990. Virtually every word of her autobiography is seriously out of date, and for anyone to conclude that what happened to Teresa Mullin is anything like what happens to a CF child today is to be profoundly misled. And yet, The Stones Applaud makes only one tiny reference, in a terribly inconspicuous place, to this important reality. Specifically, on the back of the title page -- you know the place, it's where they put the ISBN number and other publishers' minutia that only librarians and booksellers read -- the Mullin family makes the following deliberately understated disclosure:

In the years since Teresa penned The Stones Applaud, medical treatments and knowledge of cystic fibrosis have changed, so some of the things she experienced are no longer part of the lives of those with the disease today. This is the story as she lived it and her personal perspectives of life with cystic fibrosis; while it is factual, some of the names of individuals have been changed.


As you read through The Stones Applaud, it seems like every other minute she is checking into some hospital somewhere for an extended stay, at which she does stuff with the blessing of her caregivers that medicine now knows is dangerous for a cystic fibrosis patient. She is constantly frolicking with fellow CF patients, and the sad truth is that this probably contributed to her premature death. What kills CF people, by and large, is the effect of opportunistic bacteria to which non-CF folks are impervious -- bacteria that CF patients can and do spread to one another if, say, they hang out together in close quarters when not feeling well. And hanging out with other CFers in close quarters while not feeling well is a fair summary of each and every hospital stay Teresa Ann Mullin experienced.


The other seriously misleading aspect of this book is more subtle in character.

Before Teresa packs off to New England for prep school, she suffers under the care of a pulmonologist in Pennsylvania whose approach to CF care, at least as described in the book, can be summarized by calling her an egotistic disciplinarian. This physician keeps Teresa out of grade school for the better part of two academic years, until the family travels to Boston on other business and Teresa is checked out by the renowned Dr. Harry Shwachman.

Dr. Shwachman immediately promises his new patient a better life and introduces her to the notion that CF is best confronted, by both its caregivers and its patients, with creativity and passion. "Shwachman taught me to nurture a curiosity toward medical matters," writes Teresa. "At first I felt burdened with any new information . . . . But Shwachman helped me see that I needed to rely on my own knowledge and good judgment if I hoped to gain any measure of independence."

This is a key insight. But what Teresa didn't write, and what her parents failed to disclose, is that the story of CF is replete with doctors like Harry Shwachman -- from Dr. Dorothy Anderson, the chain-smoking pathologist at Columbia Medical School who first identified the disease in 1938, to Dr. Warren Warwick of the University of Minnesota, whose restless and daredevil take on the disease has not only endeared him to zillions of CF patients but has served as a much-heralded example of how healthcare in general ought to be approached. Dr. Atul Gawande, the surgeon and author, made Dr. Warwick a central character in "The Bell Curve," his 2004 New Yorker article about rebelling against medical mediocrity. In slightly altered form, this account now appears as a chapter in Gawande's 2007 book Better: A Surgeon's Notes on Performance.


If you care about CF, put down The Stones Applaud and read Gawande's book. Then scrounge around for a copy of Cystic Fibrosis in the 20th Century, edited by Dr. Carl Doershuk and published in 2001. There you can read about CF hero after CF hero -- like Dr. Paul di Sant'Agnese, the guy who figured out (during a New York City heat wave in 1952, before everyone had air conditioning) that CF could be diagnosed by measuring salt content in sweat, or Dr. LeRoy Matthews, who pioneered the physical therapy that has prolonged the life of so many CF patients, or Dr. Philip Farrell, who has successfully championed the absolute necessity of screening babies at birth for CF. (It is worth noting that Teresa Mullin was not diagnosed with CF until she was four years old, and thus she lost four critical years of treatment. This surely contributed to her death in early adulthood.) Yes, Dr. Shwachman is in there too -- my point is that he was not really unique but part of a proud tradition, one that continues to spread.


Why has it spread? Because the Cystic Fibrosis Foundation, under the leadership of some other CF heroes -- Dr. Robert Beall, the Foundation's president; Dr. Preston Campbell, the Foundation's medical director; Dr. Bruce Marshall, the Foundation's director of clinical affairs; and Leslie Hazle, R.N., the Foundation's director of patient education -- has become a tireless champion of what is known in medicine generally as "patient- and family-centered care." This is simply the radical notion that the real experts on CF (or any other condition requiring medical care) are the people who live with it. In practical terms, such an approach to CF means rejecting the very kind of assembly-line, authoritarian care that Teresa Mullin so forcefully condemns in The Stones Applaud.


What an honor to her memory it would be for her family to claim, rightfully, a sliver of credit for helping to move things in that direction. Instead, they simply allow their late daughter to perpetuate, inadvertently, an untruth, ironically by attributing an outdated sentiment to the late Dr. Shwachman. "Though he'd helped found the National Cystic Fibrosis Research Foundation in 1955," Teresa wrote in 1990, "he often talked about how the modern Cystic Fibrosis Foundation had become a politicized big business he no longer recognized."


He would not recognize it today. If you care about curing cystic fibrosis, you should write the Foundation a check.

There is one CF hero, other than Dr. Schwachman, that Teresa recognizes in her book -- but she damns him with faint praise indeed. No human being on Planet Earth has worked more assiduously to reduce CF to a curious medical footnote than sportswriter, novelist and NPR Morning Edition personality Frank Deford. Deford's book Alex: The Life of a Child, about his daughter's death from CF in 1980 at the agonizingly young age of eight, is powerful enought to stand on its own as compelling first-person literature that even people indifferent to CF ought to read. Here's what Teresa Mullin had to say about the work of her fellow CF author:


Although my family and I have great respect for Deford and the work he has done to teach the public about cystic fibrosis, I felt alienated by a book that focused more on Alex's death than her life. Instead of celebrating the short life of a fighter and calling for stepped-up medical progress, Deford cloaked Alex's story with a father's grief.


After you've put down Teresa Mullin's book, after you have read Atul Gawande's book, and after you have read Carl Doershuk's book, go see for yourself whether Frank Deford did nothing but wallow in a father's understandably boundless grief. As a CF dad myself, my favorite part of Deford's book is his list of "four basic things you must understand" about hospitals. One, two and four are, respectively, that you need to know your social security number, that the billing department will screw up but blame you, and that the elevators take forever. No. 3 deserves quotation verbatim:


You have to use one word all the time. That word is "why?" No matter what anybody in a hospital tells you -- and especially if it involves your child -- ask them why. Most doctors have a good bedside manner, but then, they're the bosses. When you confront them, especially standing up, eye to eye, they are not all that commanding; they are not any good at dissembling then, or even hedging. Very few people dare to ask doctors why. You'd be surprised; most always you can tell when a doctor is gilding the lily, or plain just doesn't know. Even Alex could spot the phonies by the time she was only six or seven. So ask why, and if that doesn't obtain satisfaction, ask why again. You will never regret it.


I've lived by those words ever since I read them. Surely they resonate fully with what Teresa Mullin was trying to say -- or thought she was trying to say. And maybe it's because she criticized a fellow CF author so unfairly that I feel comfortable about saying that, even though she died so sadly, I wish Teresa Mullin's book were not in circulation.


Teresa Mullin was not beneath using her chronic illness to help her get admitted to ultra-competitive schools like Exeter and Harvard. She didn't shrink from the fame and attention one gets by literally becoming a poster child for her disease. She didn't mind getting to meet celebrities like Susan Lucci (the seemingly ageless Erica Kane from the soap opera All My Children) or Joan Rivers. She apparently never had to worry about who was paying for all those hospital stays -- or, for that matter, who was paying for Exeter and Harvard. Her promoters today hail her as a young woman of insight and wisdom. And, yet, she manages to write this, in one of her concluding paragraphs:

We will not live in a civilized society until we tend to the disenfranchised among us. In this time when women, people of color, the gay and lesbian community, and the disabled all continue to struggle for basic civil rights, there is another, uncounted minority. The chronically ill, especially those of us with life-threatening illnesses, have spent too many years on the fringes of American society. No matter how content we have been in our own lives, almost all of us in this country have grown up shunned and discouraged. The enlightened among us have learned to hate the word "concern" as a euphemism for discrimination.


Teresa, I respectfully disagree. CF is awful, but it also happens to be, by and large, a disease of white people. Compared to people with other diseases, CF patients have good access to health care, and they have a well-endowed and persuasive foundation that is working day and night, and paying pharmaceutical companies tons of money to work day and night, to cure the disease. They will succeed. Meanwhile, it's hard to think of someone who studied at one of the nation's most elite prep schools, and then went on to study at America's most elite university, as among the "shunned and discouraged." You just died too young, and I mourn your loss. But I still didn't like your book.


[Pictured at the beginning of this post: My daughter using The Vest, a physical therapy device invented by Dr. Warren Warwick. I got to meet Dr. Warwick in 2005. It was like meeting Elvis.]

Vermont Saved From Dangerous N1303K License Plate Combo

Once upon a time, the owner of this blog celebrated his exotic cystic fibrosis mutation -- N1303K -- by acquiring a New Hampshire license plate with that deliciously obscure combination of numbers and letters upon it. I figured that, one of these days, I would cause some geneticist to drive right off the interstate at the sight of such an esoteric assertion of mutational militancy.

That, as far as I know, never happened. But the exercise was worth it nonetheless, if only because of the way the clerk reacted at the Department of Motor Vehicles in Concord when I arrived to submit my vanity plate application. She admitted to being curious about what N1303K was all about. When I explained that it was all part and parcel of my daughter having cystic fibrosis, and our family's commitment to beating the disease on every front, this kindly gal practically burst into tears.

Then I moved to Vermont and had to re-register my car in a new state. The contrasting stories from each state of what it is like to try to acquire a license plate with N1303K on it offer, I think, some real insight into how New Hampshire and Vermont differ from one another. And, as is so often the case these days, the longstanding assumption that Vermont is the groovy state and New Hampshire is the soul-less backwater doesn't really hold up to scrutiny.

N1303K is a completely impermissible license plate in the Green Mountain State.

This seemed obvious from the get-go, upon checking the web site of the Vermont Department of Motor Vehicles. It turns out that, however harmless such things are in New Hampshire, Vermont does not allow vanity plates that have both letters on them in combination with more than two numerals. I was resigned to this limitation, until I was driving along the highway one day and observed Vermont license plate X1951. I wondered if, perhaps, I had misinterpreted the applicable rules as they are imparted on the Vermont DMV web site.

So I filled out an application for a Vermont vanity plate reading N1303K. In due course, the Vermont DMV returned my $35 check along with my application -- with the word "denied" handwritten in red next to my request for N1303K. There was no indication that anyone had read or considered the earnest letter I had attached, explaining the whole CF thing asking the DMV to waive any requirement that would preclude such a combination.

This hardly seemed appropriate for a kindly and cute state like Vermont. It wasn't the rejection that smarted -- it was the lack of a nice note from some friendly DMV official apologizing for being unable to grant what was, obviously, a request of a thoroughly benign nature.

So, I decided to press on. Some quick legal research revealed that Vermont's statute governing administrative procedure contains an interesting provision designed to force state agencies to respond to citizens who think some rule or another is stupid or unnecessary or harmful. The statute says that anyone can ask an agency to change one of its rules -- and, within 30 days, the agency must either begin a rulemaking proceeding to grant the requested change, or explain in writing why it will not do so.

Invoking this requirement, in late December I wrote a terribly polite and respectful letter to Commissioner Bonnie L. Rutledge of the Department of Motor Vehicles. I explained the whole situation and asked that she either change the offending rule or waive it so I could have my N1303K license plate.

Commissioner Rutledge obviously didn't consider herself bound by the 30-day response deadline. But she did reply -- and here is her February 4 letter (postmarked 11 days later, on February 15) verbatim and in its entirety:

Dear [upstart citizen desiring N1303K license plate]:

This is in response to your request that this Department either grant a waiver from the administrative rule provision limiting the numbers that may appear on vanity plates to two or, amending the rule to do so.

Department rules adopted through the procedure of the Administrative Procedure Act have the force and effect of law. The rule in question does not contain any provisions that would authorize exemptions to be issued. For that reason, a waiver is not an option.

With respect to the amendment of the rule to delete the prohibition against three or more numerals, I believe that the rule serves the purpose of avoiding confusion between vanity plates and plates of the regular issue.

For that reason, your request for the Department to take action to amend the rule is denied.

Very truly yours,

Bonnie L. Rutledge

Commissioner

Well! I stand corrected and rebuked! Only an unreasonable and misguided citizen would fail to see how rational, even laudable, a government objective is avoiding "confusion" between vanity plates and ones issued routinely. If that kind of "confusion" were allowed to take root -- well, Vermont might become too much like New Hampshire, where major pileups have been known to snarl interstate highways when motorists are distracted by the bewilderment that arises out of being unable to distinguish, at a glance, between plates people paid extra for and plates that merited only the routine registration fee.

Not only that, but something close to chaos reigns inside the New Hampshire State Prison, where inmates working in the license plate shop have been known to riot at the prospect of being confused by whether any particular plate they must stamp out is a vanity plate or a plate "of the regular issue." Inmates thrive on certainty.

And of course there are the oppressed employees of the Department of Motor Vehicles in Concord itself. That poor clerk who took my application for my N13o3K plate was so confused by my request, and the resulting knowledge about cystic fibrosis that I forced upon her in an effort to resolve her confusion, that I expect she up and quit her job the moment I left the premises.

Indeed, one can see that by persisting with such an unreasonable request in Montpelier I engendered so much confusion in poor old Commissioner Rutledge that I immobilized her. Not only did she miss her 30-day deadline, but she also (I infer) was so bewitched, bothered and bewildered by having to deal with the whole thing that she let the letter sit on her desk for 11 days before she was able to bring herself to mail it on February 15.

License plates have a funny history in both states. New Hampshire once lost a big case before the U.S. Supreme Court, which held that First Amendment liberties are inconsistent with the kind of compelled speech that arises out of forcing people to promote the slogan "live free or die" on the front and back of their cars. Vermont has found itself embroiled in litigation over having deemed certain combinations of letters too offensive to be issued. So you can see how combining too many numbers with letters would threaten the very stability of the political fabric in the state that is so confusion-averse that it doesn't dare produce plates with official government slogans on them.

You win, Commissioner Rutledge. Thanks for considering my request and responding so thoughtfully. You are the kind of public servant who personifies the qualities that distinguish Vermont from those big, ugly impersonal states and their confusing license plates.

RIP VilLa NM; Bye-Bye Birth Canal

Here is how the Times Herald-Record of Middletown, New York reported the fiery, February 5 demise of the 2007 Record House that has been described as "this generation's Glass House:"

BETHEL — A hilltop home that was featured in the New York Times was destroyed tonight by a roaring, smoky fire with blue and orange flames.No one was hurt in the blaze, which started just before 9 p.m. The owner of the house off Burr Road, Leo Tsimmer, was in New York City at the time — it was his second home. By the time firefighters made it up the icy, winding road that led to the home, designed by a Dutch architect, the home with steel beams was ablaze.“There was nothing we could do to save it,” said Kenoza Lake assistant fire chief George Slater.

Clearly, the Times Herald-Record and Architectural Record have little in common beyond both having now featured this project, quite spectacularly, on its pages. The "Dutch architect" referenced in the former is Ben van Berkel, nameplate principal, along with his partner Caroline Bos, of the Amsterdam-based architecture firm UNStudio. van Berkel's newly incinerated VilLa NM -- the obscure name and typography having no publicly provided explanation -- was UNStudio's first project in the U.S. Leo Tsimmer, it turns out, is a Russian emigre who managed to rise from the ashes of the Soviet Union with lots of money and the kind of what-the-hell approach to life that allowed him to commission a second home that was more gotcha than dacha.

On their web site, the architects describe the project as

a box-like volume [that] bifurcates into two separate volumes; one seamlessly following the northern slope; the other lifted above the hill creating a covered parking space and generating a split-level internal organization. The volumetric transition is generated by a set of five parallel walls that rotate along a horizontal axis from vertical to horizontal. The ruled surface maintaining this transition is repeated five times in the building. From inside the huge window strips from floor to ceiling allow a fluid continuity between interior and landscape. From the exterior the reflective glass seams to become one with its surroundings.

Exterior photos make clear the project's homage to Philip Johnson's Glass House (and the Farnsworth House by Ludwig Mies van der Rohe, from which Johnson stole the idea) as well as UN Studio's own Mobius House, built a decade ago in the Netherlands.

But there's a twist. What made VilLa NM remarkable, it seems, was its dazzlingly white interior that was comforting and cozy where Mr. Johnson and Dr. Farnsworth found themselves on public display. New York Times architecture critic Nicolai Ouroussoff, compared the staircase at the heart of the structure to a birth canal. You can't get much more intimate than that.

Ouroussoff loved the place. "In a society driven by rampant careerism and painfully averse to risk — especially the risk of being labeled gauche — the house is a monument to what can happen when you let go of inhibitions," Ouroussoff wrote in his October review of the project. "It proves that the slickness of the computer age does not necessarily have to lead us to a world sanitized of sensual charms."

Okay. But Nicolai Ouroussoff is no Ada Louise Huxtable, who created the paper's tradition of architectural criticism. Like his snootish immediate predecessor, the late Herbert Muschamp, but lacking Muschamp's flair for invoking big aesthetic ideas, Ouroussoff has no interest in placing architecture in its social context, so that the real people who read the Times on the subway might get some sense of why they should give a damn about some Russian oligarch's high-gloss vacation home. That was Huxtable's genius -- in proving, with every column, that architecture mattered. The Muschamps and the Ouroussoffs of the world are more intent on proving that they matter.

And so you get the phenomenon of what appears to have been a certifiable artistic treasure being spectacularly destroyed -- with the local newspaper having no sense of the project's significance or even knowledge of the name of the world-class architect who designed it. In a sense, VilLa NM never really existed in the first place.

To be fair, some of the problem is inherent in the nature of residential architecture -- private places, by definition. Huxtable earned her chops by railing about big fiascos like the demolition of the neoclassical Charles Follen McKim masterpiece, Pennsylvania Station, in favor of the banal Madison Square Garden Center.

But there was a time when custom-designed residential architecture was thought to be, if not a template, than at least a laudable example that builders for those of lesser means could follow. The Case Study Houses, commissioned beginning in 1945 by the now-defunct Art & Architecture magazine, showcased important architects like Richard Neutra and Charles and Ray Eames. More importantly, the Case Study Houses literally put contemporary architecture on the map in the U.S. right at the point of the postwar boom -- proving to the general public, at least for a while, that a successful house did not have to imitate some arbitrarily selected historical precedent. Baack on this coast, tour Frank Lloyd Wright's Zimmerman House in Manchester, N.H. and see for yourself how one designer's original vision spawned a million imitations as suburban sprawl overspread America.

VilLa NM is unlikely to have many imitators among today's residential developments. By all accounts, its demise was spectacular and thus more memorable, to the locals in Sullivan County, than its construction. But since UNStudio's ill-fated first U.S. building stood in, of all places, Bethel, New York, the fabled home of the Woodstock Festival, it's too bad that the unusual building materials, when going up in smoke, didn't create a little Purple Haze to go with those blue and orange flames.

20 Seconds of Joy -- But Who Pays?

"I don’t want to die, I want to live. I’m pretty good at running away, and this is my escape!” So says Karina Hollekim in 20 Seconds of Joy, the hour-long documentary about her life, and near death, as a BASE jumper. Unfortunately, even those who are good at running away could not possibly escape fast enough from this tedious yet offensive film.


Inexplicably, 20 Seconds of Joy won the award for best film on mountain sports, plus a people's choice award, at the 32nd annual Banff Mountain Film Festival. The festival bills itself as an opportunity to "experience the adventure of climbing, mountain expeditions, remote cultures, and the world’s last great wild places." And if you don't happen to be hanging around the Canadian Rockies in the autumn, a selection of films and film excerpts from the festival tours North America each year.



So it was that I found myself at the Lebanon Opera House on February 8, in the company of a theatre-full of trust-fund hippies from Dartmouth whose idea of being at one with Planet Earth is the kind of resource-intensive, self-indulgent outdoor sport that is epitomized by BASE jumping. BASE, by the way, is an acronym for the kinds of perches from which these people leap -- buildings, antennae, spans (as in bridges or arches) and earth (i.e., cliffs or other natural formations). Don't these people have an exam to study for?



For five long years, documentary filmmaker Jens Hoffman followed Hollekim around from leap to leap and recorded her tiresome, hackneyed justifications for such reckless thrill-seeking. The film even stoops as low as to show an interview with Hollekim's mother, who suffered a debilitating brain injury when the family car got into an accident when Karina was just a little kid. Is Karina avenging her mother's tragedy? Fleeing from it? Subconsciously reenacting it?

We never find out, because this film has nothing to say beyond the obvious. Five minutes into the hour, it is perfectly obvious that this story is going to end badly, given the ceaseless repetition of the notion that death and crippling injury perpetually stalk BASE jumpers. Hollekim brashly states, over and over again, that she accepts her fate without fear or regret.

Thus, at approximately 45 minutes: Splat!

Ironically, Hollekim doesn't cripple herself by jumping off a building, an antenna, a span or some piece of earth. She leaps out of an airplane over Switzerland and, while the movie certainly has footage of the mishap, there is never really an explanation of precisely how Hollekim's fate caught up with her. Instead, we watch as she undergoes surgery after surgery, and endures month after month of rehab, in order to regain the use of her shattered legs.

By the end of the film, it is not clear whether she will succeed. I personally was indifferent to the medical outcome, obsessing instead over the question of who is paying what was obviously hundreds and thousands of dollars in medical expenses necessary for the repair of more than 25 open fractures. Like other European countries, Norway has a system of universal healthcare, which means that Norwegians with real jobs, in dangerous places like mines and mills, are paying for all those operations and all that rehabilitation. These people will likely labor for entire lifetimes without ever standing at any of the breathtakingly beautiful precipices one sees in 20 Seconds of Joy -- and if they ever got to such a place, leaping would likely be the last thing on their minds.

Likewise, leaping was probably the last thing on the minds of all those World Trade Center office workers as they arrived at their cubicles on the sunny morning of September 11, 2001. Maybe you have to have had the actual experience of standing atop one of those towers, on an observation platform deliberately set back from the edge to discourage vertigo, to understand viscerally what it must have taken to have forced all those involuntary BASE jumps one sees in footage of that horrible day. Maybe an ability to imagine what that must have felt like -- choosing free-fall over inferno a thousand feet above Greenwich Street -- is all it takes to dismiss Katrina Hollekim as an irksome character and any movie about her a waste of celluloid.

I get it that people like Katrina Hollekim are out there -- folks whose greatest joy lies in confronting and overcoming what would be, to anyone, the most fearsome of possibilities. To those people I say: Next time, try standing in front of an advancing tank in an effort to stop tyranny or genocide. Offer yourself up to a medical experiment calculated to cure the heretofore uncurable. Or rescue a child from a burning building.

Or go land on the moon like Neil Armstrong did. I learned today that Neil Armstrong never grants interviews to discuss his experiences as the first person to stand upon a different celestial object than Earth. Would that Katrina Hollekim had cultivated such laudable reticence. Even 20 seconds of silence would be better than 20 Seconds of Joy.