AUTHOR: Richard Barager | POSTED: 12/10/10 10:20 PM
CATEGORIES: Literary Prescriptions, The Literary Doctor
Every so often there are cases that challenge our understanding of what constitutes futile medical care. Cases that remind us of the tremendous ability human beings have to adjust their capacities to the reality of their impairments. Sometimes majestically so.
There is no more inspiring example of this than the story of Jean-Dominique Bauby, the editor-in-chief of Elle magazine who in 1995 suffered a stroke that resulted in what is known as “locked-in syndrome”—a brainstem vascular occlusion of the pons resulting in quadriplegia and speechlessness, but with the retention of normal consciousness and cognitive ability. In other words, complete awareness and comprehension of being unable to move or talk. Pure terror.
Most patients die soon after the occurrence of this devastating event. But not always. Some live for months or even years in this immured state, at great financial cost to society. One cannot help but wonder in such cases if the cost justifies the benefit? Surely locked-in syndrome meets the definition of futile care: cases with so little hope of improving that continued measures of life-sustaining support are deemed futile and therefore abandoned in favor of palliation. Modern hospice care, with the proper use of analgesia and sedation, can make the embrace of death for victims of locked-in syndrome no more traumatic than slipping off into a drugged slumber from which they never awaken.
Interestingly enough, the literary world gave voice to this pitiful syndrome long before the medical world did. Plum and Posner didn’t coin its name (Posner, J.B., Saper, C.B., Schiff, N.D., and Plum, F., The diagnosis of stupor and coma. 4th ed. 2007: Oxford University Press.) until 1966; Alexander Dumas wrote of it in 1820, in The Count of Monte Cristo. A character in the story, Monsieur Noirtier de Villefort, suffered from this precise condition, and was described by Dumas as “a corpse with living eyes”—as apt and succinct a description of a patient with locked-in syndrome as any modern day neurologist could ever hope to articulate.
But what of Jean-Dominique Bauby? What became of his locked-in syndrome? It turns out that Bauby, due to the peculiarities of his particular stroke, was left with movement in one part of his body, and one part only: his left eyelid, a slightly incomplete variant of locked-in syndrome. And what did Monsieur Bauby choose to do with this pathophysiologic oddity? Unbelievably, he “dictated” his memoir—by blinking.
“It is a simple enough system. You read off the alphabet . . . until, with a blink of my eye, I stop you at the letter to be noted. The maneuver is repeated for the letters that follow, so that fairly soon you have a whole word.”
In this way, he laboriously blinked out his memoir, working hours a day for nearly a year in response to a scribe who meticulously called out letters ordered by their frequency of use. It took him on average two minutes per word to produce a book that fearlessly chronicled his every indignity.
“One day . . . I can find it amusing, in my 45th year, to be cleaned up and turned over, to have my bottom wiped and swaddled like a newborn’s. I even derive a guilty pleasure from this total lapse into infancy. But the next day, the same procedure seems to me unbearably sad, and a tear rolls down through the lather a nurse’s aide spreads over my cheeks.”
One of the more astounding revelations of this excruciating account was that shortly before his stroke, Bauby had reread The Count of Monte Cristo.
Jean-Dominique Bauby died two days after The Diving Bell and the Butterfly was published. The diving bell was meant to represent the prison of his body, and the butterfly his trapped mind fluttering inside it. His story is testimony of the majestic agony of the afflicted—and of the penumbra of medical futility, which even for a corpse with living eyes swirls beyond the palings of our certitude.