Locked in or locked out?
Can you be happy when you are completely paralyzed? When you can think and feel, but can only blink your eyes? Surprisingly, most people with the locked-in syndrome can be happy. What is devastating is when you are locked out of society.
The Intouchables: Locked-In Syndrome (LIS)
Many people have seen the movie Intouchables, about the friendship between Philippe, a millionaire left quadriplegic as a result of an accident, and his new caregiver: the rude, uneducated, and street-smart Driss. The millionaire’s friends warn him: “Be careful. These street guys have no pity.” Philippe replies, “That’s exactly it. That’s what I want. No pity.” I loved the movie. I am sure you did too. But I am also concerned about how Philippe is categorized by many people. He is seen as a remarkable exception to the rule, a rare person who finds life worth living despite his severe disability. What strength, how inspiring! I strongly disagree, on the basis of my studies of the Locked-In Syndrome (LIS). In the classic form of this condition people are completely paralyzed and can move only their eyes, but they do have intact cognition.
Quality of life is not related to the degree of disability
Make no mistake, I do think Philippe is strong, but I do not think he is an exception. Most people with LIS are fundamentally happy and find their life worth living. Over the last 12 years I have had the opportunity to interview over 30 people with chronic conditions of quadriplegia and LIS about their quality of life, their use of assistive technology, and – recently – about their inclusion in society. How do you interview people with locked-in syndrome? One way is to use the alphabet system. For a demonstration, see the video: Communication with a person with the Locked-in Syndrome (LIS) (Nijboer 2014). Most of the people I interviewed rated their quality of life as a 7 or an 8 on a scale from 1 to 10. Some even rated it as 9. The very few who rated their quality of life as poor were often people whose medical condition was still shifting. My findings are compatible with, larger studies (Bruno et al., 2015). And quality of life is not related to the degree of disability. I interviewed one person who had made a miraculous recovery from LIS but subsequently showed many signs of depression. Whereas another person who could only move his lower lip and eyes said life had never been better!
Healthy people do all sorts of things for you that you don’t want
Whenever I mention this at scientific conferences, public lectures, or birthday parties, people look at me as if I have just said the earth is flat. They just can’t believe me, as the finding is so counterintuitive. The response is usually: “Well, if I’m ever in that locked-in state, you can pull out the plug”. In fact, there are many organizations who fight hard to ensure that these patients have a right to die. It is true that many who are in the acute phase of the locked-in syndrome do want to die, but as I said above, in the long run most people with LIS value their lives. Yet some feel almost pressurized into considering euthanasia. They are afraid that people might be too eager to “help” them die, just because they pity them. Read Disability and Physician-Assisted Suicide (Batavia, 1997). As many LIS patients have experienced, healthy people are sometimes so helpful they do all sorts of things for you that you don’t want.
What is difficult and depressing is to be locked out from society
Good intentions can go a bit wrong. Mrs. UBA, a woman with late-stage ALS who can get about in a wheelchair but cannot speak, was helped across the road by a passer-by. He just unfortunately neglected to ask if she actually wanted to cross. A funny anecdote. But some anecdotes are not so funny. Good intentions can really go wrong. Mr. PR, a man with late-stage ALS and in a locked-in state, divorced his wife because she wanted him to get euthanasia, whereas he wanted to live. She now won’t let him see his twin daughters because he is so “selfish”. Mr. JK, also locked in, told me he had lost a friend who cannot bear to see JK continuing with this “undignified life and being such a burden to his wife”. For the record, Mr. JK is the CEO of a company he runs with his wife and employs over 20 people despite his disability. Moreover, his other friends regularly pick him up for a drive in the sidecar of one of the bikes at his motorbike club. So he feels strongly that his life is full of dignity. Studies have repeatedly shown that people can learn to live with being locked in. What is difficult and depressing is to be locked out from society. “What hurts is that people turn their back on me in the bar, because they don’t know what to say to a person like me”, said Mr. DVH, who has been locked in for over 20 years. Mr. WT talks about “spastic reactions” from society: “First it made me angry, now it makes me sad”.
People underestimate the quality of life of people with LIS
What am I trying to say here? It’s very simple. Life can be enjoyable even with disability or LIS. Healthy people, family members, and doctors consistently underestimate the quality of life of people with LIS. And these people therefore risk being continually reminded that life with this condition is not worth living. This even applies to apparently well-meaning remarks such as “it is so remarkable and brave that you want to go on living”. Some people feel pressurized into considering euthanasia.
'I really want to live in a world where disability is not the exception, but the norm'
Let’s get this straight: it’s not that I’m against euthanasia: I wholeheartedly believe we have the absolute right to decide when enough is enough. However, I wish we could fight as hard to enable life as we do to enable death. I wish people with LIS would not be locked out of society. In a spot-on Ted talk, Stella Young (yes, disabled) hits the nail on the head: “I want to live in a world where we don't have such low expectations of disabled people that we are congratulated for getting out of bed and remembering our own names in the morning. I really want to live in a world where disability is not the exception, but the norm” (Young, 2014)