I have previously written in this space about the remarkable case of Jean-Dominique Bauby, the editor-in-chief of Elle magazine who in 1995 suffered what is known as “locked-in syndrome”—a stroke resulting in quadriplegia and speechlessness, but with preservation of consciousness and intellect. http://richardbarager.com/literary-prescriptions/a-corpse-with-living-eyes/

Bauby chose to deal with this incredibly cruel medical circumstance by writing a book—The Diving Bell and the Butterfly—that he “dictated” by blinking out a code with his left eyelid to a bedside scribe.

I recently came across an article about another patient with locked-in syndrome who chose to deal with her circumstance in quite a different, though no less admirable, way.

The case occurred in Belgium, which along with the Netherlands and Luxembourg are the only three countries in the world to date to legalize euthanasia. The United States has three states—Oregon, Washington, and Montana—that allow physician-assisted death (the prescription to a patient of Nembutal, modern-day Hemlock that the patient then self-administers), but not physician-conducted, active euthanasia. Here’s an excerpt from the journal reporting the case, Transplant International. http://onlinelibrary.wiley.com/doi/10.1111/j.1432-2277.2008.00701.x/full

“A 44-year-old woman was suffering from locked-in syndrome after brain stem vascular accident [4]. She was fully conscious and communicated only with eyelid movement. She spontaneously breathed through a tracheostomy and was fed through a gastrostomy. After 4 years of this assisted life, she asked for physician-assisted suicide according to the Belgian law. She was examined by a psychiatrist and a neuropsychologist who excluded depression and confirmed preserved cognitive function, and by an independent palliative care physician who confirmed dismal medical prognosis and the patient’s willingness to die. Euthanasia was granted and scheduled for a month later, as required by the Belgian law.

The day before the euthanasia, the patient expressed her will of after-death organ donation. The ethical and legal possibility of combination of the two separate processes, physician-assisted suicide and after-death organ donation was then considered and agreed by the institutional ethical committee president.

The intravenous euthanasia procedure was performed according to the regular protocol, in the presence of the patient’s husband, in a room adjacent to the operative room. The patient was in her regular hospital bed. No member of the transplant team was present during the euthanasia. When the patient’s death was declared by three independent physicians after 10 min of absence of cardiac activity, her cadaver was placed on the operative table. The liver and both kidneys were harvested and transplanted according to the regular Eurotransplant organ allocation rules for after-death organ donation [3].

Currently, more than 1 year later, all three recipients are enjoying a normal graft function.

This case of two separate requests, first euthanasia and second, organ donation after death, demonstrates that organ harvesting after euthanasia may be considered and accepted from ethical, legal and practical viewpoints in countries where euthanasia is legally accepted. This possibility may increase the number of transplantable organs and may also provide some comfort to the donor and his (her) family, considering that the termination of the patient’s life may somehow help other human beings in need for organ transplantation.”

The issue is this: is the linkage of organ donation to euthanasia unethical because it gives society (in the form of patients on the waiting list for organs and transplant centers wanting to do transplants) a stake in the deaths of those with terminal conditions? Or, for patients who want euthanasia in countries that allow it, is voluntary organ donation following euthanasia a life-ending but life-affirming altruistic act that allows another human being to benefit from the Gift of Life? And, if you believe the latter, does it then follow that patients in Oregon, Washington, and Montana who avail themselves of “the Nembutal” should be permitted to voluntarily donate their organs as well?

As committee chairman, I will be posing these fascinating ethical questions at my local hospital’s next Bioethics Committee meeting, but there’s no reason to wait until then for the debate to begin.

What do you think?

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.