A terrifying story of locked-in syndrome and recovery: Guardian “Long Read”

This story is important because it explains what people think when they are victims of “locked-in syndrome,” Very few people have survived to tell the story. This patient had Stage 4 toxic progressive leukoencephalopathy, which is thought to be usually fatal. The story is written up in The Guardian. The patient himself wrote a piece that was posted on rarediseases.org and quoted below. The patient also has his own website, jhaendelrecovery.com. This post, however, is not so much about the disorder, nor about its cause– it is about a person who recovered enough from “locked-in syndrome” to tell about his experience with the disorder.

Jake Haendel, now aged 32, developed locked-in syndrome gradually, starting in April-May of 2017. His “timeline” states that he began to have locked-in syndrome on December 20, 2017. He was immobile and unresponsive for a long time. He began to show signs of recovery on July 4, 2018– at least to the extent that his doctors realized that he was conscious.

” Doctors thought I was disconnected from all reality, but little did they know I was conscious and cognitively intact. I could hear everything. It was sheer terror!” (rare reflections: Jacob’s story on rare diseases dot org.)

At first, he lost the coordination in his arms and legs, then he developed weakness, and finally paralysis. He lost his speech, then his eye movements, and finally, his ability to breathe on his own. He spent many months completely immobile. In fact, after six months, he stabilized to the point where he was no longer thought to be dying.

His symptoms became noticeable to his wife in early May of 2017. He was arrested while driving to work because a patrolman noticed he was driving erratically. He made bail, but could barely walk.

Three days later, his wife called an ambulance and he was admitted to the hospital. At first, the tentative diagnosis was a stroke. An MRI scan, however, showed “profound, bilateral damage to the white matter.” He was given a diagnosis of toxic progressive leukoencephalopathy– essentially a death sentence. There is no known treatment and patients with this condition usually die within a few months at most.

He was sent home after the initial hospitalization and his family was told that he would continue to deteriorate until a supervening infection carried him off. Instead, he gradually stabilized.

While he was locked-in (or “locked-down”) he remained fully conscious. He could not move his eyes, and they remained open because that’s what happens when you are completely paralyzed: your eye muscles keep your eyes closed until you lose control of them; then they open. When people die, their eyes remain open and gradually glaze over. Jake was given eye drops to keep his corneas from drying out.

So, through his open eyes, he could see part of what was in front of him. He couldn’t move his eyes, so he only saw a part of what went on. He could hear everything that was said around him, whether it was intended for him or not. He was, he said, grateful to people who talked to him, read books to him, or sang to him.

Otherwise, he was bored stiff or profoundly depressed– at times, both. Sometimes people played the television, but he couldn’t always see it because it wasn’t necessarily in his field of view. He could hear it, which was sometimes good– but at other times it was even more boring or annoying. Sometimes, the TV was left on all night– then he could “watch” the late-night TV preachers, over and over again.

He spent hours at a time imagining his own funeral. He counted the seconds up to a thousand, over and over again. He went through terminal boredom. He valued, more than anything, just knowing what time it was– but the clock on the wall was just out of his view. His thoughts can only be imagined, imperfectly. Reading the piece in The Guardian might give you some idea; reading his own personal story doesn’t help much. The only way to really understand what he suffered is to go through something similar yourself.

After several months, it became clear to the doctors that he wasn’t going to die. His payments for at-home medical care expired after six months– he had lived too long. He was readmitted to the hospital for re-evaluation.

In June 2018, he realized that he could exert limited control over his eye movements– mostly just up and down. The medical staff noticed that his eyes were moving, but they couldn’t tell if it was voluntary or just a reflex. On July 5, a doctor noticed that he could move his left (or is it right?– two sites disagree) wrist on command. He was elated.

Gradually, further voluntary muscle control developed. He developed COVID-19 while in a nursing home, survived, and began to improve more quickly. Now he can breathe on his own, talk, eat, move his arms and legs, and has the ambition to eventually be able to walk again.

He has given a history of what he thought while he was immobilized and the emotions he went through. He believes that his personality has changed. His voice is certainly different. The personality he now has is optimistic and cheerful. He has gotten over the chronic depression that he suffered before he became ill.

He even started his own website, which became active on July 4, 2020. He has YouTube videos and a Twitter account. He says that the experience has changed him profoundly, although it is unclear whether the disease or the experience of disease caused the change.

In his case, his acute progressive multifocal leukoencephalopathy was caused by smoking heroin on aluminum foil. This is known as “chasing the dragon.” The toxin may be the heated heroin or a contaminant in the drug, a substance added to the heroin by the dealer. He started using opiates– Oxycontin– when he was in high school. He began to use heroin when the oxycodone became too hard to get or too expensive.

Heroin is actually cheaper than prescription opiates on the black market. It is also more pure, which makes it easier to get high. Heroin has become so cheap that dealers now add black-market fentanyl (50-100 times as strong) to their product in order to increase its value. Sometimes people overdose on heroin because it contains too much fentanyl, and they’re not used to it.

People who use heroin don’t always inject it. Pure heroin can be readily “smoked” (actually just vaporized with heat) or “snorted” (inhaled into the nostrils.) In olden days, most street-level heroin was only 5-10% pure, so it had to be injected directly into a vein to have much effect. Over the years, the price of heroin has dropped so dramatically that it is available virtually pure for a “reasonable” price.

Of course, “reasonable” is a relative term. To use a word that has been popularized by anti-climate change activists, the word reasonable ignores the “externalities” of heroin use. An “externality” is the damage associated with use that is not included in the price. In climate change, the “externality” is the accumulation of carbon dioxide and methane in the air that is not included in the purchase price of oil. In heroin use, the “externality” is addiction and associated medical disorders.

In this case, the medical disorder is toxic progressive leukoencephalopathy, the destruction of the white matter of the brain. White matter is the wiring between brain and other nerve cells; it is white because it contains a lot of fat that is used as insulation between the “wires.”

In this condition, the wiring between nerve cells is progressively destroyed. The ability to think and be conscious is preserved, but the ability to move is lost. In severe cases, all muscular actions are completely destroyed. The patient cannot blink, move the eyes, or swallow.

Severe toxic leukoencephalopathy due to smoking heroin is thought to be irreversible and fatal. Other toxic substances can also cause leukoencephalopathy, including overdoses of several medicines like methotrexate and metronidazole.

PS There’s a high rate of new coronavirus cases in Fresno County, California (where I am now): for 11/26, a total of 3,700 cases per 100,000 (37,000 out of just under a million people) and 477 deaths, with 328 new cases– per Johns Hopkins daily tracking web site.  Most of the mild cases are not being counted because people are confused as to where to get tested. (Roughly eight times as many cases have occurred as have been counted, according to the Centers for Disease Control.)

Over Thanksgiving, a [redacted] appointee on the Pennsylvania appeals court threw out he-who-must-not-be-named’s case there, saying, “Calling an election unfair does not make it so.” Just today, federal Attorney General William Barr admitted that no evidence of fraud that could have swayed the election has been found.

Reported from 11/26, a holiday: 103,116 new cases and 1,178 deaths– after earlier this week reporting 2,200 deaths in one day (New York Times.)

Reported on 12/1 for 11/30: 167,759 new cases and 1,265 deaths. The change over the last 14 days: a 3% increase in cases and a 28% increase in deaths.