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Locked-In Syndrome and the Horror of Being Trapped in Your Own Body

As people with degenerative disorders slowly lose control of their bodies, it's common to feel powerless, depressed, and even suicidal.

by Paul Willis
Nov 17 2015, 3:00pm

Photo via Flickr user D. Sharon Pruitt

In January 2014, Chris Harmon called his best friend John Filek over to his home in the quiet Minneapolis suburb of Burnsville. The friends had known each other for over 20 years, ever since Filek had answered Harmon's ad for a personal care assistant. The role required a knowledge of sign language, and Filek, having been raised by deaf parents, was fluent.

"I still remember walking up the ramp of his foster family's home and seeing him sitting on the couch," Filek told me. "We just fell into talking about our favorite TV shows, geeking out talking about our childhood memories of Star Trek and Star Wars."

Harmon had a rare neurological condition known as spinocerebellar ataxia (SCA), a disease of the nervous system that causes breakdown between the spinal column and the cerebellum, the region of the brain that coordinates movement. Harmon's cerebellum was slowly closing off for him. In practical terms, that meant a gradual retreat from his body.

He was diagnosed as a seven-year-old and when Filek first met him, he was confined to a wheelchair but still able to eat normally and breath for himself. His foster family nicknamed him "The Admiral," a tribute both to his obsession with Star Trek and his strong character.

On the cold January day that Filek went to see him, Harmon's willfulness was in evidence once again. Unbeknown to Filek, Harmon had come to a profound decision. His condition had drastically deteriorated to the point that, at age 43, he was now deaf, mute, and nearly blind. He had lost the use of his legs, relied on a ventilator to breath, a tube inserted into his stomach to eat, and enjoyed only very limited control of his hands and arms (he could rub his mouth with a napkin, but could not hold a spoon).

"He had told me that he was interviewing a new nurse and he needed me to interpret," Filek remembers. "I arrived at the same time as this 'nurse' and we were riding up in the elevator together and I said, 'Oh, I heard you were interested in working with Chris?' And she said, 'I think you're confused.'"

The "nurse," it turned out, was actually an official from a nearby hospice, who had come to the apartment to discuss the possibility of Harmon entering hospice care so that he could be taken off his ventilator. Harmon's lungs were too weak to function on their own, meaning he would effectively be ending his life. All this came to light as Filek was interpreting.

"I had to stay professional," he told me. "But in that moment I felt my body go cold."

When the meeting was over and the official had left, Filek leaned over the arm of his best friend's wheelchair and looked Harmon in the eye. "OK," Harmon told him. "You can start yelling at me."

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Depression in people with severe physical disabilities is still poorly understood. Of the little research that has been done, most suggests that depression is no more prevalent among this group than in the general population. Yet common sense demands that someone experiencing the kind of physical limitations Harmon had suffered would be more prone to depression than an able-bodied person, a view that's reflected in the treatment of degenerative diseases.

"Our clinical bias is that people should be depressed and, in fact, treating people with antidepressants is one of out first lines of therapies," said Dr. Dale Lange, medical director at Hospital for Special Surgery in New York, a specialist center for the treatment of Amyotrophic Lateral Sclerosis (ALS, the disease suffered by renowned physicist Stephen Hawking).

According to research, about one-third of those with ALS are treated with antidepressants. Dr. Lange acknowledges that this widespread use can make diagnosis more difficult since the meds help mask the depressive symptoms. Diagnosis can be complicated further by the degenerative illness itself, which, in the case of ALS, also known as Lou Gehrig's Disease, can sometimes present symptoms that look like depression to the untrained eye.

For example, research shows that about half of ALS patients have a pathological basis of easily crying and poor emotional control. Known as pseudobulbar affect, this neurological condition is also common in multiple sclerosis patients and stroke and brain injury victims and has nothing to do with depression. On top of all this, Dr. Lange says there's the broader problem of how these degenerative disorders affect communication.

"We don't have the same ability to look into people's lives in the way that we do with a normal, functioning, physically non-disabled person."

"Isolation feeds depression like mulch feeds plants. The antidote is human connection." — Dr. Dan Gottlieb

"Solitude," Harmon wrote in 2010, in his first-ever email to me. "It is a place where humans are not meant to dwell. I have gone to great lengths in my efforts to avoid the confinement of that place."

I'd first found out about Chris after reading an interview he had given to local media about a children's book he self-published. At the time I was trying to write a book myself, which was going to be a collection of interviews with people who had experienced extreme solitude, and it occurred to me that the condition of being severely disabled was one where this was an inevitable side-effect.

Harmon and I started talking regularly on Skype, and during our video calls, he repeatedly compared his condition to being trapped inside a glass bubble. In the title of his memoir, The Diving Bell and the Butterfly, Jean-Dominique Bauby offered two further metaphors that captured, like Harmon's glass bubble, the claustrophobia of his condition as well as pointing to the possibility of escape. Bauby was the editor-in-chief of Elle magazine in Paris until, in the mid-90s, a massive stroke plunged him into a state of near-total paralysis while leaving his cognitive function completely in tact, a rare medical condition known as "locked-in syndrome." The journalist's only way to communicate was by blinking his left eye, the method he used to dictate his memoir.

In Bauby's haunting, at times dreamlike account of his experiences, the butterfly is both a representation of the fragility of life and of freedom. The only freedom left to Bauby was the freedom to let his imagination wander at will. He explains this at one point in connection with his sense memory: "For pleasure, I have to turn to the vivid memory of tastes and smells, an inexhaustible reservoir of sensations. Once I was master of recycling leftovers. Now I cultivate the art of simmering memories."

Harmon's disability was not as severe as Bauby's: He still retained limited use of his arms, though his fine motor skills had disappeared and his movements were jerky and inaccurate. He could mouth words well enough to be lip-read and, although he was legally blind, he could make out hand signals as long as they were no more than a few inches from his face and so long as the interpreter wore black to provide a good contrast with the hands.

I had seen this in our Skype conversations, which transported me to his Burnsville apartment. When Harmon answered the call, the set-up was always the same: The camera revealed Harmon in profile, directing his conversation to the interpreter, who sat away to the left lip-reading. His face lolled to one side but if he said something funny or noteworthy, he would wait for the interpreter to translate it then snap his head round to offer me a smile. His ventilator, hissing rhythmically in the background, sounded like faraway applause.

During these conversations, Harmon talked frankly about how his disease had pushed him to the brink of suicide.

Sue Hagen, Patient Services Director for the National Ataxia Foundation, says that depression among ataxia patients is most common following an initial diagnosis and again each time the patient has to contend with a milestone in their loss of function.

"Each new normal is a time when depression can come along," Hagen told me. "For example, if they find themselves falling over a lot and they realize they're not going to be able to walk any more."

Harmon told me he had first contemplated ending his life in his early 20s. He had caught pneumonia, and the damage to his lungs forced him onto the ventilator and feeding tube. After a year, he decided he couldn't face the prospect of spending the rest of his life like this and wrote a farewell letter to his family, which he asked Filek to read.

What stopped him going through with it was the out-of-the-blue appearance of a childhood friend, who convinced him there were things worth living for. Afterward, the two started dating. She was blind and communicated with Harmon by taking his hand and drawing letters on his chest. When he felt the outlines of the letters, he wrote back in response. It was a painfully slow way of communicating, but Harmon seemed happy.

"Isolation feeds depression like mulch feeds plants," said Dr. Dan Gottlieb, a New Jersey-based psychologist, who was left paralyzed from the chest down following a near-fatal car crash in the late 70s. "The antidote is human connection. We live and die by the quality of our relationships."

Dr. Lange agreed, telling me that one of the biggest determinants in staving off depression among the physically disabled is the support network around them—friends and family, but also, he argues, the state.

"If there is governmental support that helps you play a role in mainstream society that makes a huge difference," Dr. Lange said.

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The children's book Harmon wrote was called The Treasures of the Shadows. It was about a little girl who discovers an old, abandoned house where figures from history come to life. I understood the story to be about the saving grace of imagination and it seemed significant that Harmon had composed it in his head at a time when he was even more isolated than normal: Minnesota state had taken away his interpreter service and it took a year of court battles to get it back.

In the meantime, he lay on his back in a care home in St. Paul, watching light dance on a crystal his mother had hung above his head. It was the only stimulus he had, and when a staff member stole it he was left to count to himself all day, into the hundreds and thousands, falling asleep, then waking up and resuming the count where he had left off. He told me he grew angry and withdrawn. He fantasized about hurting his family, yet clung to their visits "like a drowning man grabbing for a life ring."

In the accounts I've read of locked-in syndrome, anger is a common thread. In The Diving Bell and the Butterfly, for example, Bauby wrote: "I maintain a level of resentment and anger, neither too much or too little, just as a pressure cooker has a safety valve to keep it from exploding."

According to Filek, Harmon had frequent bouts of anger in the time leading up to his meeting with the hospice staff. He began picking fights with the health agencies that were providing him nursing care and interpreter services and got himself a reputation for being difficult. He also became increasingly demanding of his caregivers, insisting they memorize long lists of protocol and reducing some of them to tears.

"It was terrible," said Filek, who continued working as his interpreter. "I was at war with myself trying to respect his wants and desires and his need for order. But sometimes it was hard not to feel like a slave."

I had noted Harmon's tendency for micro-management when I went to visit him in the fall of 2010. I had assumed it was because his condition left him so much at the mercy of others that he became so obsessive about the few things he could control in his life. During that three-day visit, as in the video calls, he was mostly voiced by Kara McCoy, an ebullient Midwesterner, who affected a jaunty bounce in her voice whenever speaking on his behalf.

A screenshot of one of the author's video calls with Chris Harmon

On the final night of my stay, Filek had taken over the interpreting duties and we had gone to the Mall of America to meet McCoy and her family. Christmas was still two months off, but the mall was already decked out in festive chintz. We moved down walkways saturated in white light, past The Gap and Target, H&M and Walgreens, arriving eventually at a cavernous hallway where the tracks of a roller coaster reached nearly to the latticed steel beams of the ceiling a few hundred feet above.

"You must be ready to catch us if we fall out," I told Harmon after volunteering to accompany McCoy's daughter and two nieces on to the roller coaster.

"I look forward to it," he said, his words voiced by Filek. Walking away, I realized how Harmon's interpreters unwittingly masked who he really was: Voiced by McCoy, he was lighter, bouncier; voiced by Filek, he was darker, sometimes sarcastic. I'd never heard what Harmon's own voice sounded like.

"It's possible that some day I will be completely blind or completely paralyzed. I want to capture life while I can." — Chris Harmon

About two years after Dr. Gottlieb lost the use of his legs, he suffered a second kind of paralysis: a deep depression that left him frozen and hopeless.

"It was worse than grief," he told me. "I was completely self-absorbed."

Over time, he recovered, and along the way—in a process he calls post-traumatic growth—he began to discover who he really was.

"I was comfortable in my skin for the first time in my life. I knew I was the world's nightmare, but I was happy."

Some of this happiness came from living with such a strong sense of his own mortality, he says, which forced on him a heightened awareness of life's beauty.

"When you hold death nearby, life becomes precious," he explained.

On my final morning in Burnsville—the last time I saw Harmon—we took a stroll around a lake near to his apartment. The weather was fine and by the water's tranquil edge we caught the sun's warm glow. Above us light filtered through the leaves on a maple tree, illuminating the detail of their veins and the sheen of their translucent skin.

"It's possible that some day I will be completely blind or completely paralyzed," Harmon told me. "I want to capture life while I can."

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Just 38 hours before Harmon was scheduled to be taken off his ventilator, Filek engineered a meeting between Harmon and his stepfather. Harmon had sworn Filek to secrecy about his decision, afraid that if his family found out they would try to stop him, but Filek says Harmon was a good sport about seeing his stepfather. Near the end of their meeting, just before his stepfather was about to return home, Harmon finally told him about his plan. Filek arranged for Harmon's mother, from whom he'd become estranged in the time leading up to his death, to be told as well.

His family respected his wishes and on February 18, 2014, the day he was scheduled to have his ventilator removed, they came with him to the hospice.

"It was very surreal," said Filek. "So much of it was like any other day. You know, get him into his wheelchair, get him out of his wheelchair. Put him in the bed. He would say, 'I want this. Can you move the pillow? Can you fluff it?'"

With his family at the foot of his bed and his favorite movie, a Shirley Temple film, playing on the TV, Harmon was administered morphine to keep him calm while the doctors removed the ventilator.

"You could visibly see him sort of sinking into a sleep and it was almost like he was at the other end of a tunnel when his last words came." But while Filek stayed by his head, interpreting for him until the last, there was no need to voice those final words: Filek's name repeated twice. When he got home later that day, he found a final message waiting in his inbox. It was a tongue-in-cheek transfer of power, referencing the two old friends' mutual love of Star Trek.

"The world needs a new admiral," the message began. "I believe that you have the mind and heart to bare that title and all the responsibility that comes with it. Godspeed and clear horizons, Admiral John Lee Filek. Hailing frequencies closed."

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