Man, 35, left ‘brain dead and totally paralysed’ by severe locked-in syndrome shares incredible recovery
A MAN labelled “brain dead” has make an incredible recovery from an extreme form of locked-in syndrome that rendered him totally paralysed.
In 2017, Jacob Haendel was living a normal life as a head chef when his voice suddenly became high-pitched and he started losing his balance.
In the space of just a few weeks, his life was turned upside down after he was diagnosed with acute toxic progressive leukoencephalopathy – a neurological disorder where brain’s white matter is damaged by a toxic substance.
This in turn progressed into locked-in syndrome, which forced his body to slowly shut down.
An extreme form of locked-in syndrome is a condition in which a patient is aware but can’t move or communicate verbally due to complete paralysis.
It can be caused by a brain trauma, and infection, and exposure to toxins.
It’s not known exactly how Jacob developed the condition, though he says consuming certain chemicals “wilfully and otherwise” during his work as a chef may have been a factor.
Jacob, from Boston, Massachusetts, ended up paralysed, unable to talk or blink with a few months and could hear staff deliberating over switching off machines.
But after months of being trapped in his body, Jacob was suddenly able to move his wrist and blink again.
He went on to make a remarkable recovery and can now speak and move once again.
He’s the first ever person to recover from stage 4 acute toxic progressive leukoencephalopathy, he says.
Jacob, 35, said: “Everything has changed after what happened – my outlook, my feelings.
“Everything is much more positive in all regards.
“I now appreciate the little things in life – even being able to actually hold a cup of water!”
At 27 years old, Jacob started noticing strange symptoms.
Jacob said: “I felt kind of weird one day. It started with my voice – it became high-pitched, and people started pointing it out.
“My balance was a little off too. I could still walk, but I was holding onto walls in my house.”
He brushed off the symptoms at first, but things worsened.
“I started swerving when I was driving, and that led me to walk into the hospital one day,” he said.
‘Trapped’
In Worcester, Massachusetts, doctors initially suspected Jacob had had a stroke.
But tests revealed it was far worse than anyone could have imagined.
“Eight doctors were standing in my room, and I knew right away it was bad news,” Jacob said.
“The doctor sat on the edge of my bed and told me I had a very rare, terminal, progressive brain illness.”
The disease, known as acute toxic progressive leukoencephalopathy, is a degenerative condition caused by the inhalation of toxins.
Jacob’s life unravelled quickly.
He was told he would lose the ability to walk within a month, become wheelchair-bound soon after, and lose the ability to speak within a couple of months.
The illness would cause him to “likely slip into a coma and die,” he was told.
“By month five, I was at stage four, which they said no one ever recovers from,” he said.
“They told me I would enter into a coma and pass away.”
One of the most horrific parts was when I had an itch. I couldn’t signal anyone, and the weight of a sheet on my skin would hurt
Jacob Haendel
As the disease progressed, Jacob experienced unimaginable pain.
“It was severe chronic pain-autonomic storming, where your body’s systems just go haywire,” he said.
“I had swings of 106-degree fevers, and my heart was working like I was running a marathon.”
Three months in, Jacob became completely paralysed.
“One of the most horrific parts was when I had an itch,” he said.
“I couldn’t signal anyone, and the weight of a sheet on my skin would hurt.
“It’s like being trapped – your brain is totally intact, but you can’t communicate with anyone.”
Unable to move or speak, he could hear everything around him but had no way to let people know he was still there.
Jacob said: “One time I overheard the nurses talking, and one of them said, ‘I have to tell you about this really awkward hookup I had last night!’ “It was the funniest thing I had heard in months, and I was almost laughing inside.
“But then one of them said, ‘don’t worry about talking in front of him, he’s brain dead’.”
Jacob then began to wonder if he had in fact died, and he was hallucinating.
“It was so strange and horrible,” he said.
“They were discussing withdrawing life support. I was in so much pain, I was ready to go anyway,” he says.
Remarkable recovery
Jacob remained locked in for 10 long months.
But a breakthrough came when medical staff noticed a slight movement in Jacob’s wrist – something he didn’t even realise he had done.
“They told me to do it again, and that was my one shot, I focused everything I could on moving my wrist.”
Once doctors were aware Jacob was responding, they focused their attention on helping him to regain more movement.
And he learned how to communicate using slow blinks, which led to him being able to use a letter-board, where he could spell out words and eventually regain some form of communication.
The first words he learnt to say was “I love you” to his family.
Jacob was transferred to Spaulding Rehabilitation Hospital, where he spent three months relearning basic functions.
He was then moved to Western Massachusetts Hospital, where he stayed for 15 months.
What is locked-in syndrome?
Locked-in syndrome is a rare neurological disorder that causes complete paralysis of all voluntary muscles except for the ones controlling eye movements.
People with locked-in syndrome are conscious, alert and have their usual cognitive abilities, but they can’t make facial expressions, speak or move.
There are three main forms of locked-in syndrome:
- The classical form: People have total immobility but can move their eyes up and down, blink, hear and are able to think and reason
- The incomplete form: This is like the classical form except people can have some sensation and movement functions in certain areas of the body
- The total immobility form: People have complete body paralysis and loss of eye movement, but have their normal cognitive abilities
Locked-in syndrome is very rare, but it more commonly affects people aged 30 to 50.
It is caused by damage to a specific part of your brainstem called the pons.
Several specific conditions and situations can damage your pons, causing locked-in syndrome. The most common cause is ischemic or hemorrhagic stroke.
Other less common causes of damage to your pons that can lead to locked-in syndrome include:
- Infection in certain portions of your brain
- Tumours or masses on your pons or brainstem
- Demyelination, which is the loss of the protective insulation (myelin) that surrounds nerve cells
- Certain conditions, such as amyotrophic lateral sclerosis (ALS) and Guillain-Barre syndrome
- Injury to your pons
- Substance misuse
Source: Cleveland Clinic
“I started regaining movement in my limbs, and I worked on vowel sounds,” he said.
“I never imagined I’d recover like this.”
He gained his ability to walk with the help of surgeries and physical therapy and by December 2020, he had moved back home with 24 hour care.
He’s the only known person to recover in such a way from stage four acute toxic progressive leukoencephalopathy, and and possibly one of the only people to recover from locked-in syndrome.
In 2022, a man suffering with locked-in syndrome was able to ‘speak’ again after being fitted with a brain implant device that enables him to control a keyboard.
Despite his incredible progress, Jacob still faces daily challenges.
“I’m still learning how to walk with a cane, and my voice isn’t what it used to be,” he says.
In 2021, Jacob co-founded a mobile app, Ahoi, designed to help people with disabilities navigate accessibility challenges when out and about.
Jacob said: “Before this happened to me I was always a fun, outgoing guy, but deep down, I was sad and depressed.
“Now, even with all the challenges, I appreciate the little things.
“But it’s still been a strange and traumatic period, it will take a long time to get over.”