My old blog, www.kreis.coop, is gone (replaced by this new and improved one) . . . so here are some greatest hits from the past (mainly for archival purposes): A first person account of an epic concert by NIH Director Francis Collins, an architetural rant and rave about the best building in Vermont, and a public letter to my daughter.
Greetings from Nashville! The sense of celebration and joy here is palpable, and I have to admit I have gotten caught up in it.
As I write, the 33rd North American Cystic Fibrosis Conference (NACFC) is wrapping up here in Music City – it is the third time I have had the privilege of stepping out of my usual professional milieu to immerse myself in medicine, microbiology, genetics, and other cool scientific stuff in my capacity as a CF dad.
Before I tell you about why all 5,000 conference attendees are delirious, let me explain why this dad’s heart is bursting in particular.
While here, I have heard so much about both of my kids, Rose and Felix. Rose, of course, is the object of so much affection and respect as she takes up her new life as an adult who is thriving as a person with CF. Here’s what I said about Rose to the room full of newly minted CF caregivers who got a dose of my advice: “Every day I marvel at all this great kid has been able to accomplish – not in spite of being born with cystic fibrosis but, I contend, because of it.”
Those are the earnest thoughts of an adoring dad. But when Lynn Feenan, the nurse who held Rose as a failure-to-thrive baby almost eighteen years ago, speak so lovingly of my daughter my heart wants to burst. So, too, of Dennis Stokes, the pulmonologist who took care of Rose as a toddler and preschooler, or Greg Sawicki, the director of the pediatric CF care center in Boston that is now keeping Rose healthy.
They know Rose is a CF phenomenon – not just because her last FEV1 (the key measure of her lung health) was 104 percent, literally better than perfect – but because they know that this scholar-equestrian is going places.
But here’s the thing: Felix, as he nears his 14th birthday, is also a CF luminary. The advocacy staff at the CF Foundation raves about him, now that he has visited Capitol Hill two years in a row as a part of the CFF’s annual teenager lobbying assault. Let me know if you want to see the picture of Felix explaining to Pat Leahy, the senior member of the whole U.S. Senate, why the CFF’s policy agenda is so important and so right!
I’ve been to Capitol Hill with Felix. Though he hesitates to admit he loves his sister, you should hear how the CFF talking points, and testimony about what life with CF is really like, come pouring out of him so earnestly and persuasively. How gratifying to hear that the advocacy team at the CFF remembers how formidable he is.
So I am one very proud and happy dad. And, on top of that, I do admit to taking delight in the thing that everyone else is celebrating as well.
Was there a dry eye in the house when all 5,000 of us watched as Francis Collins – appropriately enough for Nashville -- picked up his guitar and belted out two songs? I don’t know. I was too busy crying myself to notice what other people were doing. You might know that Collins is the nation’s top medical scientist in his capacity as director of the National Institutes of Health (NIH). But did you also know that, 30 years ago, Collins co-led the team that first pinpointed the genetic cause of cystic fibrosis? That puts him right in the pantheon of CF heroes, right up there with Dorothy Anderson (who first identified CF as a disease, in 1938), sportswriter Frank Deford (the Gold Star CF dad who brought the disease to the nation’s attention), and a bunch of others whom I can tell you about if you have an hour or two.
Collins sang “Dare to Dream” – which he wrote – to celebrate the approval of Trikafta, which is the so-called “triple combo” drug, approved last week by the FDA, that will mean 90 percent of people with CF will have a drug available to them that treats the basic cellular problem at the root of cystic fibrosis. That’s what is causing all the delirium here in Nashville. But what really got to ME was hearing Collins do his rendition of the love song from the teenage CF heartthrob movie “Five Feet Apart,” called “Don’t Give Up on Me.” I lost it, not because I am a fan of the movie (though I secretly admit I am) but because the message Collins was delivering with this particular tune.
Because here’s the bittersweet reality. Rose isn’t part of the 90 percent. She has so-called “rare and nonsense” mutations. Trikafta (and the other modulator drugs developed by Vertex Pharmaceuticals with the CFF’s help) will apparently not help her.
So when CF hero Francis Collins sings about not giving up on someone, he’s talking about Rose. He’s reaffirming the promise that was also front and center at this year’s NACFC that nobody will be left behind to suffer with the life-shortening and literally breath-taking consensuences of cystic fibrosis while the 90 percent dance in the streets.
I believe Francis Collins. I believe Cam McLoud, chair of the CFF Board of Trustees and Gold Star CF mom. I got to meet Cam at a reception; she knows all about Rose now. I believe Mike Boyle, who is taking the reins as the Foundation’s new CEO just as it is committing an additional $500 million – that’s half a billion dollars – to the “Path to a Cure” Initiative, whose purpose is to deliver the goods for every CF family.
There’s a cool purple brochure all about Path to a Cure; I grabbed a stack of them. (Want one? Ask and I will put one in the mail for you.) Inside is an elaborate diagram of the path; towards the bottom is a box that reads: “Some tricky stuff to be figured out here.” Even though I am the biggest cynic and crank in the realm, I am all-in convinced that this “tricky stuff” will indeed be figured out. I’m asking you to be all-in too on this, not as a matter of faith but because the work is already under way . . . the data, about the progress already made, can be extrapolated to “CF = cured forever.”
I especially like the way Bill Skatch put it. He’s the CFF’s senior vice president of research affairs and (as disclosed at the first plenary session) a hang-gliding daredevil. Discussing the CFF’s work on rare and nonsense mutations, he deadpanned: “This is an exciting time for nonsense.”
In our family, the rare and nonsense mutation initiative could be called, with greater precision, the N1303K and 621+1G>T initiative. The first mutation is rare, the second one is nonsense, and both belong to Rose. We are thus so invested in this moon-shot effort. I have my own moon-shot to announce. I would like everyone I know to make a donation to my CFF Annual Fund appeal this year, between now and the end of 2019. All of my Facebook friends, all of my Twitter followers, everyone who has ever had a conversation with me in person, if we talked about kids or if I mentioned CF. I don’t care how much you donate – indeed, a small amount would be especially welcome because small gestures are so powerful.
“We must not abandon the ten percent of people for whom [the triple combo] drugs will not provide benefit,” Collins told NPR from Nashville. “And everybody who is working on cystic fibrosis is one hundred percent behind that sentiment. And the good news is there is now a lot of momentum to get to that place as well and ultimately to develop not just a drug therapy, but a cure.”
Carmelita Hinton, the educational visionary who managed to acquire two adjacent farms in Putney, Vermont and turn them in 1935 into the first coeducational boarding school in New England, left an interesting and inspiring document in her wake.
Issued in 1954, Hinton’s “Fundamental Beliefs of The Putney School” is a list of eight aphorisms that she hoped would continue to guide what remains, to this day, a working farm and a thriving community of high schoolers and their teachers. Three of those beliefs are relevant to the central assertion of this essay, which concerns a garage door recently installed, in outrageous fashion, at The Putney School.
A garage door is a fine thing, except when it is used to desecrate what is among the best buildings constructed in Vermont in at least the past 50 years. The building in question is The Putney School’s Michael S. Currier Center for the Performing Arts, opened in 2004 and designed by the well-known and highly decorated Somerville, Massachusetts architect Charles Rose.
It is tempting, but ultimately simplistic, to observe that the Currier Center defies all of the usual notions about how a place like Elm Lea Farm, since becoming the heart of Putney School, should look. Clad in green clapboard, featuring a multi-folded roof amply adorned with sod, the Currier Center talks more to the forest behind it than to its nearest visible neighbors, the white former farmhouse or the big red dairy barn. The Currier Center will not inspire a Ben and Jerry’s flavor. In other words, it does not contribute to the theme-park image of Vermont that is otherwise so prevalent, even in Putney.
But like its neighbors from the original farm, and unlike many contemporary buildings designed by famous architects, the Currier Center is designed so that all four elevations matter. I am especially fond of what might plausibly be called the rear façade, which shows off the roof to those driving or walking by on a campus access road, paying unmistakeable homage to the landscape from which this educational institution springs.
It is here that someone at the Putney School has seen fit to replace an elegant sliding glass divider with what appears to be a garage door that features faux woodwork and appears to have been purchased on sale from Home Depot.
Hinton’s second Fundamental Belief was “[t]o learn to appreciate and participate in the creative arts, where man gives expression to his struggle for communication of his inner life and for beauty, and to grant those arts great prestige.” (The Putney School has opted not to update Hinton’s outdated use of non-inclusive language; I have done likewise.) You can’t remain true to that particular fundamental belief and get away with slapping a cheapo garage door onto the back of a Charles Rose building. The assembly hall at the Currier Center is known as Calder Auditorium, in tribute to the Alexander Calder mobile that hangs there. After some well-meaning students once decided as part of a work detail to spiff up the Calder by painting it a different color, the school scrambled to correct this artistic error. Yes, the garage door, like the sliding glass opening it replaced, opens onto the building’s (otherwise unobtrusive) loading dock. But this is still the equivalent of retrofitting the mobile with a string of paper clips in hopes of making it work better.
Since the Currier Center houses not just performance but also the visual arts, Charles Rose took particular care to have his design communicate something to people who are learning the fundamentals of drawing. The interior is essentially an elaborate set of statements about perspective, with various lines converging upon distant vanishing points.
This is especially true of the main lobby and entry hall the building, which offers a visual joke of sorts. The space runs the length of the building from front to back and is designed so that the floor, ceiling and walls appear to converge at a distance much farther away than is actually the case. It is a reminder that artists can and often must create the illusion of depth. Rose’s visual pun has now been ruined by the inside of that hideous garage door, whose aluminum tracks on the ceiling make this clueless retrofit even more egregious on the inside than it is on the outside.
A bit of polite nosing around (in my quasi-undercover capacity as the dad of a Putney senior) revealed a plausible backstory to this desecration. A reliable report from a source who will remain anonymous suggests that the original glass door failed and would have required a custom replacement at an estimated pricetag of $50,000. And let’s just say that The Putney School is not as well-endowed as the Philips Exeter Academy in New Hampshire, which would never effect such a clumsy repair on its great architectural masterwork (i.e., the best building in New England, the school library designed by the late Louis Kahn).
But what about Hinton’s third Fundamental Belief? I refer to her call “[t]o believe in manual labor, be glad to do one’s share of it and proud of the skills learned in the doing.” Reverting to the sexist syntax of Hinton’s time, one man’s $50,000 custom door is another man’s opportunity to teach some Putney students how to fabricate something that would save the integrity of the Charles Rose design.
The lack of such an initiative suggests, I think, an underlying hostility within the Putney School to hosting and stewarding such a distinguished building. The roof has leaked so reliably since the Currier Center was commissioned that you’d think Frank Lloyd Wright himself had designed it. The auditorium is usually dark and gloomy, making it ill-suited according to some for serving as the principal gathering space of this peppy (not preppy) academic community. The names of donors appear everywhere, which is not in keeping with the zeitgeist of the institution that reveres simplicity and has not otherwise opted for expensive starchitecture.
More importantly, the web site of Charles Rose Architects offers the following boast: “How we arrived at a contemporary building with folded roofs, bold forms, vibrant interior colors, and high marks for environmental sensitivity is a story about the importance of consensus-building. Adding considerable complexity to the project was the large group of trustees, faculty, and students who all wanted a say in the building’s design and who had strong emotional ties to the campus.” Apparently this is another example of how history is written by the victorious; many people at the Putney School who lived through the design of the Currier Center apparently do not feel the process was as friendly and inclusive as its architect claims. As a result, hurt feelings linger – and hurt feelings can beget ugly garage doors.
None of this would matter much if the garage door were an isolated act of architectural hostility. But this is neither the first nor, apparently, the last or least of the insults heaped upon the Currier Center. It started with the crooked drainpipe the school installed several years ago, marring the simplicity and elegance of the front façade and campus-facing entrance plaza. Where does it end? Well, another Charles – the Prince of Wales – once famously described a new wing added to the National Gallery in London as “a monstrous carbuncle on the face of a much-loved and elegant friend.” The Putney School has its own monstrous carbuncle on the drawing board – a black-box theater that will grow out the back of the Currier Center like a Stage 4 tumor.
Now we see that the garage door added to the back of the Currier Center was the equivalent of a precancerous lesion on the skin of the building. Yes, the school will save money, gaining a needed new facility without having to add the parking and bathrooms and other expensive, ancillary things that a freestanding theater would require. But the Currier Center, as a work of architecture, will effectively be dead.
Maybe Carmelita Hinton would have been fine with the way this is going down. Though her husband patented the original jungle gym, nothing else about her publicly known story suggests an affinity for great or risky architecture. But the last of Hinton’s Fundamental Beliefs was this: “To wish to live adventurously though not recklessly, willing to take risks, if need be, for moral growth, so that one definitively progresses along the long slow road toward achieving a civilization worthy of its name.”
What kind of civilization is the Putney School conjuring when all of these assaults on the artistic vision that is the Michael S. Currier Center are happening without a peep of protest or comment on campus? It was a risk to build such a thing on the campus of a farm that metamorphosed into a boarding school, and now the school has a moral responsibility to curate and to steward its creation. What has happened to the Currier Center since its commissioning is a reflection not of moral growth but its opposite.
Let me be clear: I love the Putney School — as I have since I first visited the campus in the 1980s. I will love it still, no matter what the school does to its Charles Rose masterwork. My daughter has received a fabulous education there, and I was delighted when Head of School Emily Jones had this to say about her recently: “She has done so well here, and learned to push us around in just the ways I want kids to learn to push institutions around.” In that spirit, Putney deserves to be pushed around for its stewardship of the Currier Center, so that it doesn’t produce graduates who have learned to be passive occupants of buildings, oblivious to what architecture does to us.
Dear Rose:
You are about to turn 17 years old. As I think about that, I realize how happy I am to be your father. It is such joy to have watched you become who you are: a scholar, a skilled equestrienne, a person of insight and wit, a caring friend to others, and – yes – a young woman who lives with cystic fibrosis.
We have come so far from the days of Rose the failure-to-thrive baby, literally saved from starvation at five months of age when a medical resident thought to give you a sweat test. You are most assuredly not failing to thrive now.
But there is a part of me that is very sad as your birthday approaches.
You are doing a good job of taking care of yourself – and, as you know, it is no easy thing because your CF is with you all the time, usually claiming well over an hour every day for physical therapy, inhaled medication, and all kinds of other annoyances. There have been challenges and setbacks – and, you probably remember, during one such time over the summer you said: “I’m doing the best I can, Dad, but let’s face it – I’m gonna die of this eventually.”
My heart ached to hear you say that. “You’re wrong,” I blurted out, in the moment. I still think so. Here is why.
This is not a matter of blind faith or hope. It’s a matter of science – and, in particular, of extrapolating the measurable scientific progress that has been achieved, not just over the 70 years since CF was first identified but within your relatively brief lifetime itself.
Not long after you were diagnosed, we found out that you have unusual CF mutations: the one known as 621 + 1G > T on your mother’s side and N1303K on your dad’s. (Most CFers, as you know, have at least one copy of a mutation known as 508Fdelta). Back in 2002, this didn’t matter much; the latest breakthroughs were Pulmozyme and Tobi – drugs that help ward off the effects of CF on your lungs.
Then the Cystic Fibrosis Foundation (CFF) and its partners in the pharmaceutical industry hit paydirt – in the form of three so-called “modulator” drugs (Kalydeco, Orkambi and Symdeko) that treat the underlying cause of the disease: the failure of a particular protein to do its job in each of your cells, to allow chloride (and thus salt water) to pass in and out of those cells. Unfortunately for us, those modulator drugs have so far helped mostly just people with at least one copy of the 508Fdelta mutation. You, it turns out, are something of a poster child for what has become known in the CF world as “rare and nonsense” mutations. And in your lifetime, cutting-edge CF care turns on what specific CF mutations are in your personal genome.
A piece of good news is that the CFF’s drug development partnerships – referred to by the Foundation as “venture philanthropy” – were not just a breakthrough when it comes to how a disease-fighting charity can meet its goals. With respect to the modulators, it was literally paydirt: In 2014, the CFF cashed out its rights to future royalty payments from the drugs and pocketed a cool $3.3 billion.
What is the CFF doing with all that money? In large part, the Foundation is figuring out how to cure CF in people with rare and nonsense mutations. That’s you. In particular, three years ago, the CF Foundation did something truly radical. It took matters into its own hands.
It turns out that what venture philanthropy did for drug development the CFF Therapeutics Lab does for research breakthroughs at earlier stages in the process. I am talking about things like genetically altered stem cells, or chemical compounds that can suppress the effect of nonsense mutations, or new assays (systems for testing the effects of new chemical compounds) other innovations that fall under the category of “intellectual property.” When that intellectual property belongs to the CF Foundation, because it was created at the Foundation’s own lab, the CFF can act on those breakthroughs with the kind of speed and efficiency you need as a person with CF.
Well, the CFF figured it could just give it away – to drug companies and other enterprises willing to bring these innovations to market – if the Foundation opened its own lab and developed the intellectual property itself. Thus was created the CFF Therapeutics Lab in Lexington, Massachusetts.
Earlier this year, soon after finding out that this CFFT Lab is ground zero for doing something about people with nonsense and rare mutations, I discovered that every fall the CFF does a tour of the facility for key people in the CF community. I don’t really think of myself as such a key person, but when the invitation came I did not hesitate. I just put the date on my calendar.
Boy am I glad I did that. My visit to the CFFT Lab on October 1 was like a free and effortless trip to the summit of Mount Everest. Much of what I saw was unfathomable – what do I know about microbiology or genetics, after all -- but everything about the vista was breathtaking. Even though I skipped out of math when calculus set in years ago, and figured that English majors didn’t need college-level microbiology, the benefits of the technology being employed at the CFFT Lab were obvious. For example, there’s a $400,000 machine that uses robotics to process minute quantities of chemical compounds. When you can test 10,000 such compounds a week – “high throughput screening” is the catch-phrase – success is inevitable for the same reason that 10,000 monkeys typing will eventually produce a Shakespeare play. Likewise, just a couple of benches away, there is genetic sequencing technology that can plow through the human genome many times faster than the 15 years it took to map the genome for the first time. This will pave the way for using gene editing, or gene therapy, to restore the effective functioning of that defective cellular protein. Just a few feet away from all that is a whole lab that is using tissue from CF lungs (swapped out via lung transplants) to figure out if implanting healthy “stem cells” – or removing lung cells, repairing them, and replacing them – will turn CF lungs into healthy lungs.
Those are just some highlights I was able to grasp as a non-scientist. As a liberal artist and CF dad, what really blew me away was the people who work in the CFFT Lab. My new favorite friend at the CFFT Lab is Dr. Hillary Valley, a 2006 graduate of Concord High School – which is just a few feet away from my office in New Hampshire’s capital. You and I, Rose, were already five years into our shared CF odyssey when Hillary was heading off for her freshman year at the University of Wisconsin – and now she’s a PhD microbiologist who works to cure CF via gene editing. Like the other young scientists whom she works alongside at the lab in Lexington, she was drawn to the CF Foundation because of the resources, the energy, and the creativity the organization brings to its endeavors. Likewise there was Dr. Jed Mahoney, who leads the stem cell biology group at the CFFT Lab. Jed went all rapturous as he was showing off color images of lung cells he’s been messing with. Like his colleague Hillary, he was lured away from other endeavors and onto the payroll of the CFFT Lab because he resonates with the Foundation’s energy, passion and the commitment. Who doesn’t want to play for the team that is headed to the World Series?
When you ask these young scientists why they have dedicated themselves to curing cystic fibrosis they always say: “It’s the community.” They’re talking about us, the CF families. I think we owe them everything we can do to prove they made the right choice for the right reasons, because there are any number of other things these talented PhDs could be doing with their scientific acumen.
We owe others as well. I owe people like the sportswriter Frank Deford, who held his little girl’s hand as she died of CF in 1980 – and then spent the rest of his own life helping to lead the CF Foundation. You, I think, owe people like Rosemary Quigley. She made it to 33 before dying of cystic fibrosis in 2004.
Maybe you had a life like Rosemary’s in mind when you mused that you will inevitably die of CF – she, after all, graduated from law school, clerked for a federal appeals judge in Maine, and became an accomplished medical ethicist. But consider what Rosemary herself wrote about her situation not too long before her death: “I am discouraged by people who confer admiration on my most basic accomplishments; I seek supporters who never think I have done too much, who will push me further for as long as possible. I focus my spirit to outlast my withering body.”
Reading stuff like that, as the dad of a little girl with CF, I assumed that when you got old enough to understand your situation fully you would see it as I see it. I thought you would want to lean into this CF thing, not because you are lucky to have this disease – it is most assuredly not a blessing – but because fate has tethered us to one of the noblest and most successful human endeavors in history.
When you made clear that you didn’t automatically embrace my boundless and evidence-based optimism, I felt miserable for a few days. Then I sought help. I’ve made some very smart friends in the CF community, among them my fellow members of the board of our chapter of the CF Foundation. I reached out for their advice.
One board colleague, a young woman with CF who is just a few years older than you, said she likes feeling connected to “the big march to victory” but, she added, on her own behalf as well as yours: “A lot of times we are just tired.”
Another board colleague – a mother as well as a daughter – had this recommendation: “Continue to relay to her your excitement about research and opportunity and leave room for her to share that she needs to view this skeptically and be prepared for it not to work. You are a dad whose own biology contributed to her having this disease, so you need to believe that science and your efforts to aid it will have an impact. She is a kid who knows that despite science she will continue to suffer serious health setbacks and that her life may be cut short by CF. Your experiences of this disease are different and that is just the way it is going to be.”
Okay. It’s easier to be a militant CF heterozygote like me, with N1303K on his vanity license plate and the CFF pin on his lapel, than it is to live with CF every day. I get that. But I beg you to see what I see – a trajectory of scientific progress that means you are likely to outlive your dad by many years and ultimately die of something other than cystic fibrosis. Yes you will get tired. But when you are tired, we will lift you up. Not just me, but all of the people, living or dead, whose work and thoughts I have mentioned here.
Please try live your life with the understanding that you might not have to die of this disease. It has been my good fortune to learn about and sometimes to meet so many remarkable people who have been connected to cystic fibrosis. But none have been as remarkable as you -- and I so look forward to many, many more of your birthdays.
Love, Your Dad