A Case:
A man in his 40's came to the hospital for weakness on one half of his body. In the ER, he vomited several times and fell to the ground unconscious. He never woke up. A CT scan showed an enormous stroke that had swelled, compressing the other half of his brain against the skull and squeezing his brainstem, the site of his most basic respiratory function, to the point of uselessness. His physical exam indicated not just coma, but flat-out brain death. There were several tasks left to do-
- Confirm it with an "apnea test"- measuring the CO2 content of the arterial blood and waiting until it reached a high enough level that it would induce a breath in any living human. No breath= no life.
- Contact his family
- Contact the organ donation network
I performed the apnea test, the first I had ever done. I punctured the artery in his wrist and sent multiple samples of blood to the lab. He didn't breathe, as expected. Diagnosis made, death pronounced. This is where things got difficult.
His wife was still living in Asia. She had no phone. His son was in contact, but was 9 years old. His cousin lived in NYC and our social worker found her. She was devastated. They were each the other's entire American support system.
We kept his body alive for days while organ donation tried frantically to get in touch with his wife. Their efforts failed. The decision was made to remove ventilatory support and allow his body to die. All organs would be left to fail and be buried without airtight consent for donation from the next of kin. I called his cousin and through an interpreter asked her to come in.
The interpreter was not available when she arrived. I had explained to her in past days that his brain was dead, and she had understood, but she was still crying profusely when she arrived. I could only comfort her with body language and a brief hug. I asked her to step out and I took out the breathing tube. I invited her in to be with him. I stayed to watch his body die while it was monitored.
I realize that sounds morbid, but it is a rare opportunity to watch someone die without having to run around like mad gathering cardiac meds, shouting orders, diagnosing a rhythm disturbance and hooking up the paddles. The dying process is usually fought, not observed.
After 5 minutes, his blood oxygen levels were low, but not as low as I had expected. His heart was beating normally. He lifted up his arms.
He lifted up his arms!?
Why on earth did this corpse just lift up his arms? Why am I gently struggling to keep a dead man's arms on the bed? Was my exam wrong? Was the apnea test poorly performed? It couldn't have been wrong, he still hasn't taken a breath! His brainstem is gone. What is his cousin thinking right now? I can't talk to her! I think this is a spinal reflex, but how do I explain that in sign language? I can't call for a translator and leave her here alone with a moving corpse! Oh God, she looks horrified. She's looking at me. Stay calm, act as if this is expected. Good, she's relaxing. He's relaxing too. Everyone's relaxing. His oxygen is way down, his heart's going to crap out any... there it goes. 45 beats per minute. 25. Fibrillation and pulselessness. Declared dead again. Another hug. She looks at me and tearfully says "good doctor." Cousin understands. I hope.
I never saw her again.
This was my introduction to the Lazarus sign, one of the many quirks of the human nervous system. It is in fact a spinal reflex. A brain dead human body can sit up from bed, lift its arms, withdraw from pain, and scare the hell out of everyone by grabbing at its neck.
The quirks of the nervous system are what drew me to neurology in the first place, but damn if they don't make medicine difficult. They're cruel. The head that turns to a sudden noise, the eyes that fleetingly track an object- they seem intended to instill doubt. The tricks and reflexes speak to family members, indicating to them from across the room that things can't possibly be as bad as the doctors say. It's cool air blown on the glowing embers of denial that so many unfortunate families harbor when their relative is near the end of meaningful existence.
Another patient of mine was septic. Her blood was infected, she had a terrible liver. Every organ system was failing. She was comatose and her eyes did something rare called "ping-pong gaze." Without considering its meaning for a patient, it is quite amusing, because it does in fact look like the eyes are following a ping pong game. It's a highly unnatural movement for one to make when there is in fact a complete absence of ping pong tables in the vicinity. People who exhibit it are almost uniformly doomed. Only one recorded patient has ever had any meaningful recovery after ping pong gaze. She spent about 36 hours ponging away, then died, as expected.
Her daughter was convinced that her mother was simply looking around the room, getting familiar with her surroundings, right up until the moment she went out in the electrical blaze of glory I described in my first medical diary.
If states as clear-cut as brain death and coma can fool us, what chance do we have up against vegetative state? It's better than coma, but worse than minimally conscious. The difference between coma and vegetative state is the arousal center of the brain is intact with vegetative state, but the sensory processing areas are nonfunctional. They are awake but not aware. Comatose patients cannot be woken up at all.
Vegetative patients go to sleep and wake up, their eyes "look" around the room, they can occasionally withdraw to pain. But there is no awareness. They won't blink if you flick your finger near their face, they won't turn their head if you're yelling in their ear. Families don't know what to do with vegetative states. The wakefulness causes confusion, denial, guilt, and opens rifts when families need them least.
Prognosis is any of these situations (except brain death) is a tough game that needs to be both played and deferred. When someone enters a coma, they are in a relatively unstable state. After about 2-4 weeks, comatose patients have almost invariably followed one of the following paths:
-deteriorated and died
-recovered some degree of awareness
-progressed to the vegetative state, the dreaded middle ground, which can last days, weeks, or indefinitely.
It's nearly impossible to tell which of these tracks an individual patient is going to follow. This is one of the things that militate against a family's withdrawal of care in the early stages of acute illness- the very real possibility of some recovery. Any loved one would understandably cling to any hope offered and a doctor often cannot say for sure that a comatose person will never wake up while they are still actively ill. We don't mean to create false hope, but our expressions of uncertainty often do, and the signs being produced by a loved one's damaged nervous system make an accurate appraisal and reasonable plan that much more difficult. Most people want honesty about prognosis above all else, even if that honesty will disappoint them terribly. The problem arises because families often expect honesty to mean something definitive, something blunt, something that will take away the suffering that goes hand and hand with uncertainty. I can rarely provide that.
What I can tell you is that as time goes on the chances of recovering awareness from vegetative state decline and after one month the chance is nearly zero. There are anecdotal reports of people who miraculously "wake up" after years in a coma, but they certainly weren't comatose to begin with (remember, coma resolves after 2-4 weeks) and probably weren't even vegetative. Many of them are "minimally conscious," a state that is rare and not well understood.
There isn't a moral to be drawn from these stories and descriptions. If you really need one, it's that all improvements in medical care have their price, and prognostic anguish is one of them. In a sense, these states are consequences of improved means of life support, an outgrowth of our power to extend life- "iatrogenic" (doctor-caused) in every sense of the word. Confusion reigns because many of these terms, like the technology, are relatively new and their definitions have not penetrated the national consciousness.
Education outside the "it's happening to my loved one RIGHT NOW" sphere is vital and it just hasn't happened. People still frequently use coma, brain death, and vegetative state interchangeably. I firmly believe that you cannot make adequate end of life plans for yourself without understanding these terms first and the basics of prognosis, and making sure your health care proxy is prepared for the games your nervous system might play.
Even after the fact, the most critical element in encouraging peace of mind and mediating ensuing negotiations over end-of-life care among family members is the amount of time that doctors spend explaining these issues, which is generally not something doctors are fond of doing. The fuzziness of prognosis makes it even more uncomfortable for us. The good ones will admit their ignorance (a theme I will keep returning to in future diaries), even in the face of overwhelming pressure for a concrete prediction. I want everyone to keep this in mind. The decisions are not as simple as resuscitate vs. do not resuscitate, withdraw care immediately if comatose vs. continue care indefinitely. These are complex, intensely personal decisions and should be amended as you age, as your general health improves or deteriorates, as your attitudes toward acceptable amounts of disability change.
I'll leave you with one final anecdote; a sad case that I think was managed well:
An 88 year old man was brought to the ER because he was "acting funny." A CT scan showed a subdural hematoma, a large amount blood around the outside of his brain. It was surgically evacuated, but a repeat scan showed a mild subarachnoid hemorrhage (SAH), a far more dangerous type of bleed that was a consequence of the surgery. He had not wanted aggressive medical care at the end of his life, but his family agreed that he could be treated effectively, possibly even back to his baseline. He was transferred to the Neuro ICU. He suffered no stroke, the most feared complication of SAH, but he was not waking back up. He stayed in the ICU, he picked up an infection from his ventilator, and slipped even deeper into coma. The pneumonia was treated, but he did not wake up. We could not tell why. In the middle of our workup, the surgically implanted shunt in his head became infected. He started the downward spiral, but there was still a chance that everything was reversible with aggressive enough intervention. His family decided enough was enough. They withdrew care in accordance with his wishes. After a good struggle to get the man back, we failed. His family mercifully recognized that, and they were flexible enough to put a stop to our interventions. He died peacefully.