Eight Stories of Locked-In Syndrome
Imagine losing control over everything. You can't move on your own. You can't scratch an itch. And worse still, you can't tell anyone around you that you have an itch. You can feel pain, hunger, loneliness, and fear, but you can't react to those sensations. You are totally aware of your surroundings, but you can't communicate your feelings or desires, or even your basic needs. The term for this horror is locked-in syndrome. Someone with locked-in syndrome suffers from paralysis of all voluntary muscles except for those that move the eyes. This can be caused by neurological disease such as ALS, strokes, injuries to the brain stem, or drug overdose. The term locked-in syndrome has only been in use since the 1960s. Before that, people who suffered such traumatic injuries generally died from them, or else they were considered to be brain damaged. Here are the stories of eight people who have lived this nightmare.
One of the dangers of locked in syndrome is the possibility of misdiagnosis. No one knows how many people lived for years and died unable to tell anyone around them that they were conscious and aware. Julia Tavalaro was a 27-year-old housewife and mother in 1967 when she suffered multiple strokes that left her completely paralyzed. Doctors thought she was brain dead. Tavalaro was sent to a custodial institution where she lived for six years without anyone knowing she was aware of her surroundings. She was fed through a tube and cared for physically, but had no real interaction with her caretakers. In 1973, a speech therapist noticed Tavalaro's eyes moving in reaction to her words. Afterward, Tavalaro was given physical therapy and an opportunity to communicate, first by a letter board, then later by operating a computer by tapping a switch with her cheek. She also learned to control her wheelchair by head movements. Tavalaro wrote a book about her experiences, Look Up For Yes, and became a renowned poet. She died in 2003, at the age of 68.
New Zealander Nick Chisholm was 23 when he had a rugby accident in 2000. A resulting series of strokes left him paralyzed and unable to communicate. For the first three months, he couldn't even open his eyes. During this time, he was fully conscious and heard medical personnel discussing his imminent death and asking his mother if she wanted to remove life support. After several months, his mother and girlfriend convinced doctors that he was aware and thinking. Chisholm could move his eyes, and communicated by staring at letters on a letter board. His story is told in his own words, with medical explanations added.
Words can't describe the situation I have been left in—but this is as close as I can get it: an extremely horrific experience that I wouldn't wish on my worst enemy.
When you're like this (despite having 24 hour care) it's an incredibly lonely existence at times. It's amazing how much time I have to think about things now since the accident. There's heaps of thoughts that I don't bother even expressing.
Chisholm writes about contemplating suicide, although he didn't have the ability to carry it out. He has since regained some movement, and can pronounce some words. Chisholm is working toward a full recovery.
Bob Veilette was a Connecticut journalist and an accomplished jazz pianist in 2006 when a stroke left him with locked-in syndrome. After six months in a hospital, his family elected to care for him at home instead of putting him in a nursing home. That decision meant that Connecticut's Medicaid program would not cover the costs of his care, even though a nursing home would have been much more expensive. Veilette joined a movement to reform the system, although the changes they are working for will not benefit him personally for years. Private fundraisers support the family, but Veilette has had no speech or occupational therapy, and only limited physical therapy due to expenses. Veilette communicates with a letter board held by an assistant. He had no luck with a computer that tracks eye movements, because fluctuations in the size of his pupils upset the tracking.
31-year-old Catherine O'Leary suffered from hiccups for three years before the cause was discovered to be a brain tumor. During surgery to remove the tumor, a series of strokes left her completely paralyzed. She communicates by blinking; one blink for yes, two blinks for no. Eight months after the surgery, she can move her facial muscles somewhat, but still cannot speak due to a tracheotomy that allows her to breathe. Her brother is working to raise enough money to send her to America for medical treatment.
French journalist Jean-Dominique Bauby was 43 when he suffered a massive stroke in 1995. He was left with only the ability to blink his left eye. Bauby died less than two years later, but managed to write his memoir by waiting for an assistant to recite the French alphabet. He would blink when the right letter was pronounced. Using this method, he had to construct and edit each sentence and chapter in his head. The resulting book was The Diving Bell and The Butterfly (Le scaphandre et le papillon). Bauby died of pneumonia only ten days after the book was published. It was made into a film released in 2007.
Air Force veteran Gary Griffin is immobile due to ALS (Lou Gehrig's disease). He uses a device called the NeuroSwitch to control a computer and communicate with his family. Sensors are attached to the skin over a patient's muscles and signals are sent to an interface that translates the slightest muscle contractions into usable code. The company's website says even muscles that are considered non-functional may be used.
Advances in assistive technology gives new hope to those with disabilities, including locked-in syndrome. Johnny Ray suffered a brain stem stroke in 1997 that left him unable to move. In 1998, he volunteered for an experimental procedure developed by by Philip Kennedy, Roy Bakay, and the team at the Neural Interfacing Research Institute. Sensors were implanted in Ray's brain that allowed him to move a cursor on a computer with his thoughts.
"We simply make a hole in the skull right above the ear, near the back end of the motor cortex, secure our electrodes and other hardware to the bone so they don't migrate, and wait for a signal," Bakay says. The implant is an intriguing hybrid of electronics and biology - it physically melds with brain tissue.
After implantation, the research team calibrated the interface by asking ray to think about certain movements, and the software was programmed to respond to such signals. Ray used the cursor to spell words and even generate musical tones on a computer.
Dr. Kennedy is taking that technology a step further for his latest patient. Erik Ramsey was seriously injured in a car wreck in 1999 when he was 16 years old. A blood clot lodged in his brain stem and caused a stroke. Erik lost all voluntary muscle function, except for the ability to move his eyes up and down. He learned to communicate with his family using a letter board. In 2001, he inexplicably lost the ability to spell when he was hospitalized for pneumonia. Since then, he communicates only by rolling his eyes up for yes, and down for no. In 2004, Dr. Kennedy and his team implanted sensors in Ramsey's brain that can convert thoughts into speech. So far, he can think of the movements that produce vowel sounds and the computer recreates those sounds. The team hopes to program the interface to achieve consonant sounds within a year.
Links to resources on locked-in syndrome.
This article was inspired by a post at Metafilter.