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Locked-in’s Challenge to Autonomy

By Michael Cook

Four patients with locked-in syndrome have communicated that they are happy as long as they receive adequate care at home.

The craze for Marvel superheroes encourages us to think that merely being human is too easy. We need to exceed our limitations by adding superpowers – breathing underwater, eternal youth, colossal strength, regeneration, flying, spinning spider webs and so on. Of course, that’s just comic book stuff, but the same dynamic is at work in the Olympic goal of going “faster, higher, stronger”.

It’s a facet of the homage we pay to “autonomy”, the key value of contemporary bioethics. If our autonomy is diminished, we are diminished as human beings. Our happiness is deemed to be proportionate to our autonomy.

But medicine offers the competing narrative that less might sometimes be more.

Nothing illustrates this better than that rare condition, locked-in syndrome (LIS). Most people first learned of it after reading the international bestseller The Diving Bell and the Butterfly, or watching the film of the same name.

Jean-Dominique Bauby, the editor of the French edition of Elle, suffered a massive brain stem stroke while driving. When he woke up he was completely paralysed, apart from the upper eyelid of his left eye. Yet within 2 years he had written his book, which exudes a remarkable joie de vivre. He composed it by blinking when an assistant said a letter. Even in translation his prose was dazzling:

[M]y mind takes flight like a butterfly. There is so much to do. You can wander off in space or in time, set out for Tierra del Fuego or for King Midas’s court. You can visit the woman you love, slide down beside her and stroke her still-sleeping face. You can build castles in Spain, steal the Golden Fleece, discover Atlantis, realize your childhood dreams and adult ambitions.

And Bauby’s condition, terrible as it may seem, is not the worst way of experiencing LIS. He seems to have had “classic LIS” and retained some eye movement. Other patients retain some voluntary movement. But then there is “total LIS” in which the patient is immobile, fully conscious and unable to communicate.

Until now, perhaps. A paper published recently in PLOS Biology by Swiss scientists describes a computer interface that can decipher their thoughts. Four patients with total LIS were able to respond “yes” or “no” merely by thinking the answers. A non-invasive brain–computer interface detected their responses by measuring changes in blood oxygen levels in the brain.

Here is the interesting part. Contrary to expectations, the question “Are you happy?” resulted in a consistent “yes” response from the four patients. The lead author, Prof Niels Birbaumer of the Wyss Center, added:

We were initially surprised at the positive responses when we questioned the four completely locked-in patients about their quality of life. All four had accepted artificial ventilation in order to sustain their life, when breathing became impossible; thus, in a sense, they had already chosen to live. What we observed was that as long as they received satisfactory care at home, they found their quality of life acceptable.

This study marks a big step forward in communicating with people with LIS, but it is not the first to report that such patients are satisfied with life. A 2003 study in the Journal of Head Trauma Rehabilitation about Americans with LIS found that half were happy and half were occasionally depressed. The authors commented: “Clinicians may not appreciate that quality of life often equates with social, rather than physical interaction, and that the will to live is strong”.

Clearly, it is possible for people to reach deep within themselves and find happiness even with the most severe disability imaginable – if they are supported by family and friends. What is important is not to project our own fear of total disability onto the patient. As the authors of the American study wrote:

[We} call into question the assumption among some health care providers and policy makers that severe disability is intolerable. This prejudice is not inconsequential. Biased clinicians provide less aggressive medical treatment and/or influence family and friends (in ways not appropriate to the situation).

In a sense, the experience of LIS patients raises important questions about autonomy. What if we don’t need autonomy to be happy? What if all we need is love?

Michael Cook is editor of BioEdge, an online bioethics newsletter.