I'm someone who maintains a personal roster of heroes. Hanging in my office are portraits of Eleanor Roosevelt and Louis Brandeis. At home, on my wall of family photos, is the Robert Shetterly portrait (part of his "Americans Who Tell the Truth" series) of Pete Seeger. And as a cystic fibrosis dad, I'm especially keen on maintaining a roster of CF heroes.
Who's on the list? Dorothy Hansine Anderson, the brilliant physician whose victimization by career-thwarting sexist pigs led do her being the person who identified and named the disease in 1938. Frank Deford, the sportswriter and Gold Star CF dad. Medical ethicist Rosemary Quigley, who died, way too young, of CF at age 33. Warren Warwick, the pulmonologist who invented the physical therapy machine my daughter uses. Lynn Feenan, the brave and brilliant nurse-coordinator of the CF center at Dartmouth-Hitchcock Medical Center. Alicia Casey, the pulmonologist who nurtured my daughter through her teenage years. My daughter Rose herself. And several others, too many to name here.
It's well past time to add Paul Quinton to the list.
According to the official biography on his school's web site, Dr. Quinton holds the Nancy Olmsted Chair in Pediatric Pulmonology at U.C. San Diego and is a professor of biomedical sciences at U.C. Riverside. More notably, he "discovered that the basic defect in the CF sweat duct was due to anion impermeability and not defective anion exchange."
Even to my eyes -- I'm an English major, a lawyer, and a reluctant convert to amateur microbiology and genetics -- this is a big deal. An anion is simply a negatively charged ion. The fact that chloride ions are simply unable to pass through the cell wall is literally the essence of cystic fibrosis. Thus, again according to his official biography, Dr. Quinton's discovery is "considered to be one of the major advances in understanding the basic CF defect."
Dr. Quinton also has cystic fibrosis himself -- a fact he not the least bit shy about disclosing. It clearly informs his world view and explains perfectly why Dr. Quinton eschews the cheerleader persona that is typical of other grey eminences in the fields of CF research and treatment. Yes, there is much to cheer about in the empire of the Cystic Fibrosis Foundation and its outlying principalities (i.e., the research institutions it funds, the care centers it accredits, and the pharmaceutical companies with which it co-invests).
In fact, Dr. Quinton does not hesitate to speak his mind, with anyone, when the subject turns to all of the Faustian bargains that have enabled CF care to come so far. He's particularly outraged by the prices charged by Vertex Pharmaceuticals for its breakthrough 'modulator' drugs -- Kalydeco, Orkambi, Symdeco and, of course, Trikafta -- the latest one, approval of which in 2019 means that, for the first time, there is an effective treatment available for 90 percent of CF patients, one that marks the first success in fighting the underlying cause of the disease at the cellular level. This success is a direct outgrowth of Dr. Quinton's big discovery (and, of course, discoveries made by lots of other smart people as well).
Here's a great example from last year that I just came across. One of the British tabloids rang him up, at the height of the struggle in the U.K. over modulator prices. Dr. Quinton did not hold back when the paper asked his opinion of the Boston-based Big Pharma firm whose stock prices have soared along with its CF-related successes.
"I'm very pleased with my work with Vertex and that it helped them develop these drugs," he told the Daily Express. "But I'm ashamed of what has become of my work and the avarice that it has generated."
I reached out myself to Paul Quinton at about the time he gave that interview to the tabloid -- and what strikes me is how fully his public statements comport with what he told me in our private discussions. This is a functional definition of speaking truth to power.
Sadly and ironically, the fact that Dr. Quinton has CF has played a role in marginalizing him. For much of his career as a creative seeker of CF-related scientific insight, Dr. Quinton was able to fly around and present his work (and his opinions) at conferences, symposia, meetings, even CF-related social events. But no more, thanks to the rigid infection control guidelines adopted several years ago by the CF Foundation. They allow only 1 person with CF at any indoor event, and that honor is typically reserved for someone who will tell a poignant yet grateful story that will move and inspire doctors and donors alike.
That doesn't leave much room for the CF patient who has managed to persist well into his eighth decade, who is personally responsible for advancing the science of CF treatment as an outright cure draws ever closer, but who doesn't hesitate to point out who's getting rich as a result. But Paul Quinton is the Diogenes of the CF community . . . and he deserves his share of speaking engagements, awards, and gratitude.
Oh, and did I mention Dr. Quinton is a horseman? I happen to be the dad of a young CF patient who aspires to compete in the Olympics as a dressage athlete. When I told Dr. Quinton that my daughter is an avid rider, he was delighted. In fact, he pronounced horseback riding as the perfect exercise for CF patients because of the virtual chest physical therapy such a rider gets. Now there's a CF breakthrough I can endorse for sure!