Locked In Part I

“Locked In Syndrome,” professor Schmid lectured, “occurs when an injury to the brainstem, specifically the ventral pons, results in the patient being perfectly conscious and aware, able to feel everything from the smallest, most nagging itch to the most severe pain, yet is completely unable to move, or even breathe, on his own. It is a horrible fate indeed. The French have a most descriptive term for it, ‘maladie de l’emmuré vivant ‘ or walled in alive disease.

“If you compare Locked in Syndrome to a persistent vegetative state, you will find that in a vegetative state the upper portions of the brain, the parts that make us thinking humans, are destroyed; while the brainstem, the structure that channels motor nerve impulses and regulates our autonomic functions like respiration is preserved. In Locked in Syndrome, however, the opposite occurs. The cognitive and sensory regions are undamaged, while the ability to move any muscle from the mid face down is almost completely destroyed. The patient is left a quadriplegic, and is not able to swallow or even make a facial expression.

“There is however, due to a quirk of neuroanatomy, one part of the body they may be able to move to a small extent. The eye, but only vertically, up and down, not laterally, or side to side.”

As I took notes of Schmid’s lecture, I tried to imagine what a patient suffering from Locked In Syndrome must feel and think about while trapped in a completely immobile body, feeling every thing, hearing every spoken or whispered word at his bedside, only being able to blink an eye lid, and to perhaps move his eye up and down.. It was too horrible to imagine. My stomach actually began to feel queasy, and I was glad I was going into a specialty when I would not have to deal with such a heartbreaking scenario.

“Here’s something you don’t see everyday Rob,” Paul, the neurology resident, told me, “ we have a patient with LIS, Locked In Syndrome, who needs a respiratory consult. You ever hear of it?”

“Yea, way back in school though. I’ve never seen it clinically. Whats the history?”

“Mr. J is a 48 year old white male who suffered a pontine CVA (cerebro vascular accident, or stroke) He is trached (has a tracheostomy tube for breathing) and on a ventilator in ICU 8. Nothing else remarkable in the history, he was apparently healthy right up until he collapsed at work two weeks ago. He regained consciousness eight days post CVA, at least thats when we noticed him blinking when someone mentioned his name. He may have been awake previously, but he is totally paralyzed so no one noticed.”

“Can he use both eyes?”

“No, he can use his left eye only. For some reason, many LIS patients can only use their left eyes. We are still trying to figure out why that happens. Sometimes a few small facial muscles come back too, but that usually takes months.”

“How does he communicate?”

“You have to speak very slowly. Trying to blink a lot seems to take a lot out of him, so we use eye movement instead. He can move his eye vertically only, but its delayed, like the circuitry is somehow slowed down. Wait five to ten seconds to see the his eye move after you ask the question. A vertical movement means yes, if he doesn’t move his eye, the answer is no. Be sure to use plenty of eye drops for lubrication, and to paper tape his eyelids closed when you finish.”

I didn’t want to see this patient and mentally had to force myself to review his chart and talk with his nurse. For some indefinable reason, I wanted to walk out of the ICU and not come back. My stomach was queasy just thinking about going in there. I have seen and helped to treat patients with many different diseases, and I have come to realize that my greatest dread is to be paralyzed, totally dependent upon others for everything, and this poor unfortunate man’s disease was the epitome of all my secret fears.

I got myself together emotionally, took a deep breath, and walked into Mr J’s room. He was totally still, on his back in the bed, tilted up at about a forty five degree angle, the Semi Fowler’s Position. The ventilator hissed softly as the pressure rhythmically forced warm humidified air into his chest. The monitor over his bed glowed in squiggly lines and numbers of various colors, revealing the story of Mr J’s new existence; his heartbeat, oxygen level, and central venous pressure, all tracing their bright lines against the dark screen. The only movement in the room was his chest rising slightly as the machine pumped in each breath. His eyelids were taped shut.

I put my hand on his forearm and introduced myself in a soft voice, telling him I was here to check his lungs. I had no idea if he could hear me or not, because his skeletal and facial muscles were totally immobile.

“Mr J, I’m gonna remove the tape from your eyes and put in some fluid.” I removed the tape, opened each lid and administered a few drops of lubricant. “If you want to talk just look up ok?”

I looked closely at his left eye. After a few seconds the pupil moved vertically upward.
 

Note:

The editor of the French version of Elle Magazine, journalist Jean Bauby, suffered a massive CVA  in December 1995, and when he awoke 20 days later he found that his body was almost completely paralyzed – he could control only his left eyelid. He had Locked In Syndrome. By blinking this eye, he dictated a letter at a time and in this way he wrote his memoir which was published in March 1997, called The Diving Bell and the Butterfly. Bauby died of pneumonia a few days after his memoir was published. The 2008 film The Diving Bell and the Butterfly is a screen adaptation of Bauby’s memoir. It’s now on cable TV.

<p style="margin-bottom: 0in; font-style: normal;”>I discovered Bauby’s book and movie as I was researching Locked in Syndrome while writing about my experiences with Mr J. Parts II and III to come soon.