Life on the inside: The locked-in patient who escaped

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Jake Haendel spent months trapped in his body, silent and unmoving but fully conscious. Most people never emerge from ‘locked-in syndrome’ but Haendel did and his account, as told by Josh Wilbur in The Guardian, provides some fascinating neurological, patient-care and psychological insights.

Herewith an edited version of Wilbur’s account. The full version can be accessed on The Guardian, see details below article, along with further images.

Jake Haendel was a hard-partying chef from a sleepy region of Massachusetts. When he was 28, his heroin addiction resulted in catastrophic brain damage and very nearly killed him. In a matter of months, Jake’s existence became reduced to a voice in his head.

After culinary school, Jake took a job as a chef at a local country club. At 25, Jake tried heroin for the first time, with a co-worker (narcotics are notoriously prevalent in American kitchens). By the summer of 2013, Jake was struggling to find prescription opioids. For months, he had been fending off the symptoms of opioid withdrawal, which he likened to “a severe case of the flu with an added feeling of impending doom”. Heroin offered a euphoric high, staving off the intense nausea and shaking chills of withdrawal.

Despite his worsening addiction, Jake married his girlfriend, Ellen, in late 2016. Early in their relationship, Ellen found out [that he was using heroin] and within months, the marriage was falling apart. In May 2017, Ellen noticed that he was talking funnily, his words slurred and off-pitch. “What’s up with your voice?” she asked him repeatedly.

On 21 May, a highway patrol officer stopped Jake on his way to work. He was driving erratically, speeding and swerving between lanes. That morning, he had followed his normal routine, smoking heroin before brushing his teeth. It was also normal for him to smoke, or “freebase” heroin while driving. As the officer approached his car, Jake could feel that something was different in his body. He needed to conceal the baggie of heroin, which lay visible in the open centre console, but he couldn’t reach over and close the compartment. His arms flailed uselessly against the dashboard. The police arrested him for possession of a controlled substance.

Jake made bail, but could hardly walk out of the station. In the next two days, his condition deteriorated and, on 24 May, his wife called an ambulance to their home. He stumbled to the front door, leaning on the walls to support himself. The medical responders thought he might be having a stroke, so he was rushed to hospital. Brain scans showed an unmistakable imaging pattern: profound, bilateral damage to the white matter, the bundles of nerve fibres that facilitate communication between different regions of the brain.

He was diagnosed with toxic progressive leukoencephalopathy, also known as “chasing the dragon syndrome”, usually caused by inhaling the fumes from heroin heated on aluminium foil. An unknown toxin, probably something in the substance that had been added to the heroin to make it go further, was wreaking havoc in Jake’s brain. There was no known cure or treatment, so he was sent home with a store of palliative medications.

Through the summer and autumn, Jake’s symptoms worsened. His muscles grew weak and his limbs became contorted. At home, he fell over frequently and had trouble swallowing. He couldn’t eat solid food and his speech became increasingly unintelligible.

In November, Jake was admitted to hospital and transferred to the neuroscience intensive care unit, where he was put on a ventilator and feeding tube. He suffered autonomic storms – a frightening constellation of symptoms sometimes seen following brain injuries. During a storm, the nervous system is in an overactive, disturbed state. Blood pressure rises, the body sweats profusely and spasms violently, breathing becomes rapid and shallow, and the heart might beat more than 200 times a minute. Jake would storm for four, eight, 12 hours at a time. “It was agonising to watch,” his father, a plainspoken man in his early 60s, told me.

Jake was fighting for his life. He was scared, confused, sometimes hallucinating. Damage to the myelin, the protective sheaths surrounding nerve cells in the brain, progressed until he had no motor control, and could neither speak nor direct his eye movements. For the most part, he understood what was happening, but could not communicate. He could hear comments from nurses and doctors who believed him to be irreversibly brain damaged. Jake recalls an ER doctor observing him like a specimen to be dissected. “Oh, geez, this guy’s so contracted,” the doctor said, hovering inches above Jake’s face. “It put me into more pain just hearing him talk about me like that,” Jake told me. “Like I wasn’t there.”

Eventually, the storms lessened in severity, and he was moved to a nursing home. After a while he was offered palliative care at home, which is generally given to those with terminal illness. His father was told Jake was expected to die within weeks.

To outside observers, Jake exhibited no signs of awareness or cognition. “Is he in there?” his wife and father would ask the doctors. No one knew for sure. An electroencephalogram (EEG) of his brain showed disrupted patterns of neural activity, indicating severe cerebral dysfunction. “Jake was pretty much like a houseplant,” his father told me.

They had no way of knowing Jake was conscious. In medical terms, he was “locked in”: his senses were intact, but he had no way of communicating. I could do nothing except listen and I could only see the direct area in front of me, based on how the staff would position me in bed,” Jake later wrote. The disease had attacked the cables carrying information through his brain and into his muscles, but had spared the areas that enable conscious processing, so he was fully alert to the horror of his situation. He struggled to make sense of this new reality, unable to communicate, and terrified at the prospect of this isolation being permanent.

Throughout, Jake maintained a clear sense of himself. He felt every jolt, twinge and spasm of pain. “I couldn’t tell anyone if my mouth was dry, if I was hungry, or if I had an itch that needed to be scratched,” he wrote later.

He was in constant pain, and was afraid of dying – but, worse than that, he feared being trapped in his body for ever.

For months, there was nothing for Jake to do but listen to himself think. His condition mirrored that of French journalist Jean-Dominique Bauby, who published a memoir in 1997 about his experience of locked-in syndrome, written by a transcriber interpreting blinks of Bauby’s left eyelid. The title, The Diving Bell and the Butterfly, conjures the image of his body as a sinking tomb with an oxygen hookup, his mind a fluttering creature trapped inside. In 2007, the book was made into an award-winning film.

Since that time, medical experts have invented ways of communicating with locked-in patients (including a groundbreaking “brain-reading device”). They’ve also gained a deeper understanding of locked-in patients’ mental states, with studies showing that a surprising number report a positive quality of life. For his part, Bauby struggled to find meaning in such a distressing experience. His memoir is an astonishing portrait of a shipwrecked mind. “Not only was I exiled, paralysed, mute, half deaf, deprived of all pleasures, and reduced to the existence of a jellyfish,” Bauby wrote, “but I was also horrible to behold.”

Back at home, Jake’s world shrank to the space of his low-ceilinged room. After a few weeks in bed, he hit on a kind of internal back-and-forth, which became key to his survival. “Two voices, both my own,” as he later described his often-frenzied inner dialogue.

More than anyone, Ellen felt certain that he was fully conscious. She had an ability to look into his eyes and understand what he needed. He described her intuitions as “telepathic”. According to Steven Laureys, a Belgian neurologist and expert on locked-in syndrome, “It has been shown that more than half of the time it was the family and not the physician who first realised that the patient was aware.” Medical professionals, however, do caution that family members “see what they wish to see”.

A psychologist would later tell Jake that his sustained awareness was a “gift and a curse”. “I wanted so badly to tell everyone what I was thinking,” Jake said. He endured a tremendous amount of guilt that he, a drug addict, had put his family through a nightmarish ordeal, and that the state had to foot an extraordinarily expensive medical bill likely costing millions of dollars.

After six months, Jake had lived longer than the state had expected he would, and could no longer receive at-home palliative care. Medical staff still had no idea if he was conscious, but his vital signs were stable enough that he could be moved. He was admitted to Massachusetts general hospital in Boston for re-evaluation in May 2018.

In the days following his readmission, Jake began to feel increasingly hopeful about surviving and even recovering.

In late June, he noticed that he could exert very limited control over his eye gaze, just enough to shift his vision up and down. “I thought to myself: ‘This is new,’” Jake said. Control of eye gaze can be the first stage in recovery of non-verbal communication – but at first it was inconsistent, so although staff noticed the flickers of motion in his eyes, they still couldn’t know for certain if he was consciously directing them. “It was incredibly discouraging to hear from the doctors, over and over, ‘It’s involuntary movement’. There were times when I felt like I was hysterically crying on the inside,” Jake told me.

On 4 July 2018, Jake had a breakthrough. That night, from the hospital’s 22nd floor, Jake could hear, but not see, the Independence Day fireworks over the Charles River. “I thought to myself: ‘I’m gonna see those things again,’” he said.

The next day, Jake’s primary care doctor noticed a very slight movement in his right wrist. He darted to the bedside. “Do that again if you can,” his doctor said. “Move your wrist.”

Jake suddenly found that he didn’t have to think about it; his wrist simply moved. The movement was minor, but it was a sign that his body was waking up. His doctors were shocked. The joy Jake felt was “indescribable”.

Within days, he managed to blink in response to questions. After a week, he was transferred to the brain injury unit at the Spaulding rehabilitation hospital across town. Spaulding is an impressive facility in a sleek, modern building, regularly ranked as one of the US’s best hospitals.

Michelle Braley, a speech therapist at Spaulding, was surprised to be working with a patient previously considered terminally ill. “When I read his chart, I remember thinking, ‘What’s this guy doing here?’ I had never seen a case as dire who became a candidate for rehab.”

Braley helped Jake learn to communicate non-verbally, starting with a simple letter board. As Jake gained greater control over his gaze, Braley brought him a device called the MegaBee, a tablet that allows patients to use eye movements to pick letters and phrases, which then show up on a screen.

Once he could communicate, hospital staff could evaluate his progress. “I did a cognitive assessment to see if there was impairment as a result of the leukoencephalopathy,” Braley said. “It was at that point that I realised that Jake knew exactly what was going on.” Again, the staff were stunned. They had suspected he was aware to some degree, but Jake could answer every question – about his condition, about his past – clearly.

How the brain repairs itself following traumatic injury or progressive disease remains mysterious. In recent decades, though, scientists have learned much more about how new neural circuits are formed, and how different areas of the brain are “recruited” to recover lost function.

I asked Seth Herman, a brain injury specialist at Spaulding, how it was possible for someone like Jake to recover. He cited the brain’s ability to transfer functions to different areas. “The brain wants to heal, to change itself and form new neural pathways,” he said. “Repetition is key, and Jake was willing to put in the work.”

A team of physical and occupational therapists spent weeks manipulating Jake’s muscles and using casts to realign his limbs and improve his range of movement. Gains were modest, but significant. The autonomic storms subsided over time. Jake grew stronger.

Jake left Spaulding in September 2018, and continued his rehabilitation at Western Massachusetts hospital.

Locked-in syndrome is rare – estimates say there are only a few thousand in the US at any one time. Most sufferers are victims of stroke or traumatic brain injury, and very few regain significant motor function.

Jake is one of few to emerge from a locked-in state, and doctors describe his recovery as “remarkable” and “unique”. Although MRI scans continue to show signs of damage to his brain’s white matter, he has recovered the power of speech, and hopes to walk again soon.

After meeting Jake, I asked Jake’s uncle, a radiologist, if he had a theory about how Jake got better. “On a superficial level, he got the shit scared out of him and decided he didn’t want to die,” he said. “Neurologically, I have no explanation. Perhaps there’s functioning at the molecular level that we simply can’t detect on an MRI. Maybe it had something to do with who Jake is.”

Jake is adamant that his condition improved because of a mental breakthrough – a shift in his mindset after months of being locked in. “I reached a point where I was like, ‘Fuck this, I’m going to recover.’ I thought about nothing else for weeks,” he told me.

Since the mid-20th century, there has been ongoing debate about the role the mind plays in healing. Jake Haendel – who, for six months, felt like a ghost in a broken machine – remains convinced that he managed to think himself better.

At the end of one of our meetings, Jake asked me a curious hypothetical question. “Would you rather be able to walk without a mind, or think without a body?” Jake spoke in a rush before I could formulate an answer: “I would choose my mind over my body. Even after being locked in, I would still choose my mind.”

 

Full The Guardian report along with images (open access)

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