Sixty Fibrosis: Thoughts About July 24, 2018

Frankly, turning 60 – as I do on July 24, 2018 – does not feel like a happy occasion.

There’s nothing wrong with feeling old.  But at 60 you really do have to shed a self-image built around ingenuousness – the notion that you’re perpetually up-and-coming,  a source of new energy and ideas, a person whose best days are still in the future.  I am sorry to lose that.

When I turned 50, I felt relief.  I was so sure my second half-century would be the easier one, thanks to the solid foundation I had built in the first.  How completely wrong I was!  As recently as last week, I found myself throwing some of my most cherished ideals and hopes over the rail, in sorrow.

Where do you go at such a juncture?  I go to my heroes.

In this particular case, I went to Washington, D.C. where, on June 27, I got to listen to a panel discussion among four of them – the chief executive officer of the Cystic Fibrosis Foundation and three of his key people.  The topic was the latest scientific progress in the treatment of CF.

If you’re rolling your eyes – especially because you’ve probably heard similar sentiments from me before – please be reassured.  I still have not shed my cynical world view, the perspective that makes me inherently skeptical about big, mainstream organizations like this one.

I’ve been struggling with that perspective ever since the day in 2002 when the doctors told me that my baby daughter had cystic fibrosis.  That baby daughter will be 17 in a few months, and she’s also a hero.  An accomplished equestrienne and scholar, she makes having CF look like it’s just another part of the program, even when it lands you in solitary confinement for a month at a hospital far from home, as happened last year.

Rose’s brother Felix is also a hero.  He traveled with me to Washington last month to lobby on behalf of the CF Foundation.  Before we left, we stopped by New Hampshire Public Radio for an interview.  He calmly described his ambition to become a major league ballplayer, pointed out that major leaguers make lots of money, and rattled off that as a result he’d be donating at least half of his salary to the CF Foundation.  (You can hear the results of this interview about halfway through the July 20 edition of Word of Mouth.)

Felix also noted how much CF affects all of Rose’s family, and noted that until he turned 10 he had failed to grasp that not every sister of every kid lives with CF and puts up with its demands.  I’d call that the heroism of the routine – because, in our family, Rose’s CF regimen is referred to as her “routine.”

Who does not want to feel a part of something bigger than ourselves?  That’s what we get via our involvement with the CF Foundation.

Cystic fibrosis was discovered and named in 1938 by Dorothy Hansine Andersen, the chain-smoking, free-living lesbian pathologist from Columbia-Presbyterian (and graduate of St. Johnsbury Academy in Vermont).  Before Andersen, some babies were born with salty skin and didn’t live long – it was a mystery.

That mystery has morphed and blossomed into the greatest story in medicine.  I love feeling connected to people like Paul di Sant’Agnese (who invested the test for CF), Leroy Matthews (who essentially invented Rose’s “routine”), Warren Warwick (who invented the “vest” machine Rose uses for physical therapy), Francis Collins (who led the team that identified the CF gene, and now runs the National Institutes of Health), Frank Deford (sportswriter and gold-star CF dad), Rosemary Quigley (medical ethicist and CF patient), and others I can name if you’re interested.

It’s the greatest story in medicine for the reasons cited by Atul Gawande in his 2004 New Yorker article about CF, called “The Bell Curve.”  Among other things, the essay describes Dr. Warwick at work, with one teenage patient in particular – 17-year old Janelle.  Here’s a key sample:

“Let’s look at the numbers,” he said to me, ignoring Janelle. He went to a little blackboard he had on the wall. It appeared to be well used. “A person’s daily risk of getting a bad lung illness with CF is 0.5 per cent.” He wrote the number down. Janelle rolled her eyes. She began tapping her foot. “The daily risk of getting a bad lung illness with CF plus treatment is 0.05 per cent,” he went on, and he wrote that number down. “So when you experiment you’re looking at the difference between a 99.95-per-cent chance of staying well and a 99.5-per-cent chance of staying well. Seems hardly any difference, right? On any given day, you have basically a one-hundred-per-cent chance of being well. But”—he paused and took a step toward me—“it is a big difference.” He chalked out the calculations. “Sum it up over a year, and it is the difference between an eighty-three-per-cent chance of making it through 2004 without getting sick and only a sixteen-per-cent chance.”

He turned to Janelle. “How do you stay well all your life? How do you become a geriatric patient?” he asked her. Her foot finally stopped tapping. “I can’t promise you anything. I can only tell you the odds.”

In this short speech was the core of Warwick’s world view. He believed that excellence came from seeing, on a daily basis, the difference between being 99.5-per-cent successful and being 99.95-per-cent successful. Many activities are like that, of course: catching fly balls, manufacturing microchips, delivering overnight packages. Medicine’s only distinction is that lives are lost in those slim margins.

Exactly.

Dr. Warwick died in 2016, but his salient qualities – identified in the Gawande article as focus, aggressiveness, and inventiveness – live on in the CF doctors and researchers I listened to in June.  Much of what they described had to do with the CF Foundation’s “nonsense and rare mutations” initiative.

For entirely selfish reasons, this is of the keenest importance to me.  Rose has nonsense and rare mutations.  This means the miracle CF drugs you may have been reading about – Kalydeco, Orkambi, Symdeco – do not help Rose.  That’s true of, at most, 10 percent of CF patients – a minority of people within what is already an orphan disease.

Now maybe you can see why I am inspired by people who care deeply about the difference between 99.5 percent success and 99.95 percent.  These are human beings, afflicted with the same flaws, ambitions and self-delusions that hobble all of us.  But their struggle is among the noblest ones I have seen, and it one in which each of my two kids is also playing a meaningful part.  It leaves me convinced, at age 60, that each of them will attend my funeral, and I will have to go to neither of theirs.

So . . . if you want to send me Facebook greatings on the occasion of a milestone birthday, please do so.  But if you really want a piece of this – if you want to do something that will make me feel like this milestone is worthy of celebration – go here and join me in supporting the CF Foundation.  Even a token contribution will inspire me.

Apologies to the CF Foundation for stealing their “65 Roses” meme, and varying it a bit.  My kids will cringe, and you might laugh, but July 24, 2018 is definitely “60 Fibrosis” day for me.