04/24/16
TK Life

The Sad & Tragic ‘Life’ of TK

The Sad & Tragic ‘Life’ of TK

By Edison McDaniels, MD | surgeonwriter.com | nonfiction

Keywords: nonfiction, brain death, coma, miracle

TK Life

†††

This one is gonna be hard folks. Fair warning.

Recently, after reading my series on the dubious claims of miraculous survival after brain death, see The Hand of God?, one of the readers of this blog brought the following sad and tragic case to my attention. Here is the link for it: Long Survival Following Bacterial Meningitis-Associated Brain Destruction, but I have included a summary below.

The facts are these. A previously healthy four year old boy complains of a headache and within twenty-four hours lies comatose in an intensive care unit. In fact, on arrival in the ER, he has fixed and dilated pupils—a terribly ominous, but reversible, sign of brain stress and injury. A lumbar puncture (spinal tap) is performed and the recovered spinal fluid is cloudy (another ominous sign). Cultures of that fluid would ultimately grow H influenzae type b. This kid is suffering from a fulminant form of meningitis.

This is every parent’s nightmare scenario. He’s immediately treated with high dose, highly potent antibiotics, but it’s too late. He requires intubation and is placed on a ventilator, never to come off of it. And, apparently with remarkable rapidity, he develops a malignant form of increased intracranial pressure—we are told the increased pressure is sufficient enough to spilt the child’s skull sutures. Such high pressure would be fatal in any previously healthy person over the age of 12-18 months. It would push the brain out the bottom of the skull. This is, in fact, the most catastrophic event in neurosurgery, universally fatal. Game over.

Despite evidence of EEG inactivity, his family refuses to withdraw life support. The ventilator is continued, as are the remarkable and constant efforts to maintain the child’s body otherwise. He is treated aggressively for fluid and electrolyte imbalances. His blood pressure is supported with medication. His hormones, cortisol and others, are replaced. He requires lifelong cortisol replacement, as well as intermittent treatment for excessive urine output. All of these functions are normally coordinated by a functioning brain.

He never regains consciousness. His numerous infections over the years are treated with antibiotics. He is given fluids by gastric tube, as well as daily tube feedings. He neither acknowledges anyone’s presence, nor complains. By all the evidence, his care is exemplary.

His body dies twenty years after his brain. Of pneumonia after his mother finally decides it is enough and denies him yet another round of antibiotics.

Unfortunately, the story does not end with his death. An autopsy is done, limited to the brain only. The head is small, microcephalic is the term. This is understandable, since without the impetus of a growing brain pushing outward, the skull has no impetus to grow. It becomes unusually thick instead, since it still has blood flow.

The outer portion of the brain, along with its outer coverings, are calcified and stone-like. It requires a saw to cut through them.

The inner portion of the brain is gelatinous and soft. A mucousy scum of sorts, totally devoid of any recognizable brain structure or anatomy. In fact, the crud of the infection is still present since there was no blood supply to bring the body’s scavenger cells to the scene. Normally, such debris would have been carted off and replaced with scar.

It’s clear this is a dead brain, one utterly and completely devoid of any and all neurologic function.

But is this a case of miraculous survival after brain death?

There is no question—in this high tech era—a body can ‘live’ (that is, be kept alive) without the brain, but what exactly is the definition of life?

This case is remarkable for the length of survival (twenty years maintained on artificial life support puts this in a class by itself) and the heroic (and no doubt expensive) care required to allow that survival.

But to what end? He had failed every test of brain function. This child was truly brain dead and was never going to wake-up.

Such survival must be quite exceptional, in that very few individuals have the resources to pay and care for such a damaged patient. As indicated in the report, constant attention was necessary to maintain an acceptable homeostasis (internal melieu or physiologic environment) in which the surviving body could go on. It’s remarkable his care givers (family, doctors, nurses, aides, etc.) were able to keep him going for so long. Remarkable all the more that his family kept up the effort.

And ethically dubious.

He was perhaps ‘living’ in the biological sense, but was this individual ‘alive’ in the usual and humanistic sense of the term? Perhaps this is the ‘natural’ history of death when one intervenes to play God and keep the biological entity going once the soulful entity has departed.

This is not so much a miraculous survival after brain death as it is a failure on the part of the physicians involved to step up to the plate and guide this family—compassionately, with grace and dignity—through the dying process. It’s a testament to how far technology has advanced, and the growing gap between the possible and the ethical.

This isn’t life, people. It’s a sad and grotesque mismeasure of biology. It’s a frog prep, and as any biology major knows, we destroy those at the end of the day. Pity nobody had as much compassion for this child.

04/10/16
HandOfGodP4

The Hand of God? IV

HandOfGodP4

Part IV: The Hand of God?—The Will To Die

By Edison McDaniels, MD | surgeonwriter.com
You might want to read the other articles in this nonfiction series first. [Click Here]

Keywords: nonfiction, brain death, coma, miracle

The will to live is strong in us all. Every physician with more than a handful of years under their belt has stories of patients who defied the odds and thrived.

The twenty-seven year old woman I met as a surgical intern who was operated emergently for a burst ectopic pregnancy and turned out to have widely metastatic hepatoblastoma with a ruptured liver. She should have bled out that day, or died many times over in the months that ensued, but lived long enough—over a year—to appear as matron of honor at her best friend’s wedding. She looked positively radiant when I saw her the week of that wedding, a week before her own death.

The seven year old boy who ran through a plate glass window on his way back to the pool after going to the bathroom, eviscerating himself. He also tore his kidney off it’s vein and ruptured his spleen. He came into the trauma unit with no heart rate and bleeding clear saline—a result of the massive IV fluids given him in the field by the paramedics, who, as luck would have it, lived in the firehouse next door to his home. They were on scene in less than a minute. That boy spent a month in a coma before he walked out of the hospital.

A nineteen year old youth who survived five years and seven operations on his highly malignant brain tumor, a form of cancer that usually kills in months, before finally succumbing within twenty-four hours after he announced to all of us—with complete lucidity—that he was done.

Sometimes the odds go the other way, that is, not in the patient’s favor. I treated a young woman once, just eighteen, who presented with a mild head injury and a broken femur (the thigh bone). She was crying in the ER, and I made it my mission to comfort her. I told her she’d be fine, how she had only a minor concussion and a broken leg. “The leg is serious,” I said, “but not life threatening. You’ll be fine.”

Whereupon she looked me dead in the eyes and told me otherwise. She was miserable in her pain and through a clenched jaw said “I’ll never leave this hospital alive.” Those were her exact words and, so long as I myself draw breath, I will never forget her eyes. Dark and sunken, with a melancholy character I had never seen before and expect never to see again. They were dead eyes I suppose, though I didn’t know it at the time.

She was wrong in her words. She did leave that hospital alive. But she was correct in her sentiments. Before her thigh bone could be fixed, she developed something call fat emboli syndrome. Her lungs failed as a result and we transferred her to another hospital, one where they could do something called extracorporeal membrane oxygenation, which is a fancy way of saying they bypassed her lungs to oxygenate her blood. It’s like a lung bypass machine. But it didn’t work. She was young and healthy and had everything going for her but died fifteen days after our discussion in the ER.

Her death was no miracle, to be sure it was a tragedy. Tragedies are common in medicine, a dime a dozen. Luck of the draw. One person gets an abscessed tooth and is cured by pulling it, another develops bacterial meningitis for no apparent reason and is never the same again. Shit happens. People get sick. People die.

But one case in particular made me think otherwise. That occasionally, just occasionally, there’s another hand at work. The case made me question much of what I had witnessed over the years. I can’t say what I observed that night was a miracle, although it may have been. It certainly was a Believe It or Not moment though. I always knew the will to survive was strong, that a person with a strong mind and a weak body can sometimes push beyond the natural boundaries of life. But does it work the other way round as well?

Can a person with a strong mind will themselves to die?

I was working in a trauma center in Minnesota at the time. I was ever on call and that night was no exception. I remember it was after midnight when she came in. An elderly lady, I’ll call her Mabel. She was eighty-five and still lived at home with her husband of sixty plus years. Henry was his name. At ninety-three, he was more frail than she. He looked his age, she less so. They were both spry though. No walkers, no memory aids.

She had awakened thirsty and gone to the kitchen to get a glass of water. Not uncommon apparently, except when she didn’t return to bed her husband went looking for her. He found her on the kitchen floor, where she sat with a mild look of befuddlement about her. “I can’t seem to move my left leg,” she told her husband.

Henry called an ambulance and before long Mabel was sitting on a gurney in my ER with Henry ever present at her side. He wouldn’t leave her, even when she went for a CT scan. He insisted on helping her use the toilet, and when she had the slightest difficulty or hesitation answering my questions, he filled in. They were cute together, and obviously still very much in love.

Her problem wasn’t remotely life threatening. A very small hemorrhage, a form of stroke, on the right side of her brain along the midline. Too small to warrant surgery. In fact, I had to look twice to see any hint of blood at all. It was really more a case for the neurologists, the doctors who care for nonoperative strokes. I made a few phone calls, got the neurologists involved, and prepared to make my exit.

“She’ll be alright then doc?” Henry asked.

“Right as rain,” I said, or something like it. I assured the both of them it wasn’t remotely life-threatening. Her blood pressure and other vitals were normal and there was nothing of concern on the few lab tests we’d run that night. She was about as healthy an eighty-five year old woman as I’d ever seen or expect to see. “I hope I’m as healthy as she is when I’m eighty-five,” I said.

“Thanks doc, you’ve made my day. I can’t lose her. Without her, I’d be nothing but an old fool. She’s keeps my lights on.”

On the gurney, his wife actually blushed. Married over sixty years and they still touched each other like that. Down deep, where the air is rare and the fire is hot. Something my mom used to say.

I smiled through my fatigue and spoke in a voice a bit too colloquial, as I have a tendency to do at times. “You ain’t gotta worry about nothing tonight, mister. It seems somebody’s looking out for you two.”

Henry seemed to visibly relax. “Ain’t that the truth. Praise God, we’ve had a good life, Mabel and I. Nothing to complain about.”

“Nothing at all,” Mabel seconded.

Exiting the cubicle, I saw them kiss out of the corner of my vision.

An hour later, I was back in the ER. I’ve forgotten why after all these years. Something mundane but necessary. It was two in the morning and if it could have kept until morning I’d have been sleeping. It wasn’t surgery though, that I would have remembered.

I passed the cubicle with Henry and Mabel. Henry was seated on a chair beside the gurney; he might have been sleeping. Mabel smiled as I passed.

I was in the back somewhere, maybe talking to a patient, when I heard the overhead call for a code blue in the ER. That’s a cardiac arrest, an all hands onboard sort of evolution. I was close, so I heeded it.

I stepped into the hallway and saw the commotion at the other end of the emergency room. As I got closer I realized it was Mabel’s cubicle. Oh no, I thought, wondering if maybe she was having a seizure. A small crowd was already gathering.

That crowd wasn’t assembled around the gurney though. They knelt on the floor. They knelt over Henry.

An orderly was doing CPR while one of the ER docs was giving instructions to the nurses. Somebody brought over a crash cart. I watched as they opened Henry’s shirt and put leads across his front. The monitor was a mess of indecipherable squiggles.

“V tach!” the ER doc announced. “Paddles now. Charge to 400!”

He put the paddles to Henry’s chest, said “Clear!” and triggered the pulse.

I realized in that moment that Mabel was witnessing all of this. Her eyes were wide and pensive.

Henry jumped slightly when the charge was triggered. There was no animation behind it, a quick lift of his chest off the floor and back again, an inch or two at most.

“Again!” the ER doc shouted.

The look on Mabel’s face was now less pensive and more horror. “No, no please.”

Henry jumped again. The monitor had gone from squiggles to flat line. Not an improvement.

“Resume CPR. Recharge. Get me some epi and bicarb.”

“Please stop,” Mabel said.

The ER doc either didn’t hear her, didn’t realized who she was, or didn’t care. But I knew who she was, who Henry was, and—most importantly—who they were to each other.

I put a hand on the ER doctor’s shoulder. “It’s enough,” I said.

He looked up, perhaps only then realizing the man’s wife was present in the midst of all this horror. He called it. Henry was dead. It was just after 2:00 am.

I stuck around to speak with Mabel. I guess I tried to comfort her. She seemed…distant. Perhaps that was an effect of her stroke, not sure. They had no family members, none that came that night anyway, and I didn’t want her to be alone. I spent about half an hour with her, not nearly long enough as it turned out, before a chaplain arrived to take over.

“Thank you doctor,” she said, “for your kindness to Henry and me.”

I remember thinking how composed she was all things considered. “He was a good man. I’m sorry.”

“He liked you. He said as much.”

I thanked her again and said my goodbyes. I told her I would visit her upstairs later, though that may have just been lip service. Physicians are too busy for social visits to patient’s rooms, or so we tell ourselves. I had been growing antsy just waiting for the chaplain.

I never saw her alive again. She died before the sun rose that morning. She had been among the healthiest eighty-five year olds I had every seen. Her injuries were not remotely life-threatening. And she was dead.

An autopsy failed to disclose any particular cause of death. A little heart disease and of course the small stroke. Neither of these had killed her.

I believe she willed her own death, that she simply lost the will to live after her husband died in front of her.

A miracle? A hand of God moment? I don’t know. You decide. I’ve made my piece with it. Thanks for reading.

Crucible Cover

A surgeon struggles to save the life of the little boy on his operating table in 1951. He will be tested. Severely. 99 cents on Kindle.

04/3/16
HandOfGodP3

The Hand of God? III

HandOfGodP3

Part III: The Hand of God?—Brain Death, Coma, & The Will To Live

By Edison McDaniels, MD | surgeonwriter.com
You might want to read the other articles in this nonfiction series first. [Click Here]

Keywords: nonfiction, brain death, coma, persistent vegetative state, vigilant coma, miracle

If you google ‘brain death miracle’ you will get something on the order of 340,000 hits. That’s mind boggling.

Can it really be that a person without a functioning brain long enough to be declared brain dead can return to the world of the living and defy all the experts? And not once but over and over and over again?

Before we conjure images of a world populated by The Walking Dead, we need to ask ourselves this simple question:

What is more likely, a patient recovering from full on brain death, or a patient recovering who was never brain dead in the first place?

That’s right folks. These patients, every last one of them, were never brain dead in the first place.

To put it simply, dead is dead. A person who is truly brain dead is legally, clinically, and in every other sense imaginable, dead.

So what about these miracles?

The answer, of course, is coma. To the neurologically unsophisticated, coma can look a lot like brain death. Coma, at least as a first approximation, can be thought of as a substantial loss of the ability to respond to outward (and in some cases inward) stimuli. The key word here is substantial, as in significant but not total. In the presence of coma, there is life. And where there is life, there is—oftentimes—hope.

A patient in coma looks to be sleeping, at least to the uninitiated.  They will respond to some stimuli and not others. Some patients in coma do recover significantly, others do not. As the length of coma lingers, patients often develop stereotypic responses. One of them is eye opening. Loved ones and caregivers may even note a sort of sleep wake cycle—that is, periods when the patient seems predictably more awake than at other times.

Such cycles can confuse families, and the uninitiated care givers. It gives the illusion of recovery, that the patient is waking up and if only enough time is granted a substantial recovery is possible. This situation is variously termed ‘vigilant coma,’ ‘persistent vegetative state,’ or less precisely, simply ‘coma.’ While a very few patients do recover to a significant extent from this condition, the vast majority never will. And the longer the vigilant coma persists, the less likely recovery is to occur.

The informed reader will point to a few prominent cases in the lexicon wherein recovery appears to have occurred after many years. That’s the trouble—or the hope—with vigilant coma. It’s life, and, as mentioned above, where there’s life there’s hope. That hope is small, minuscule even, in most cases of prolonged coma.

But without life—in brain death, to be clear—there is no hope of recovery. Death is death.

The mistake that places a comatose patient among the dead for even a brief time has many sources. One of the more common is that of the sedated patient, or even the pharmacologically paralyzed patient, who is declared brain dead by the neurologically unsophisticated medical professional. Occasionally a patient may appear to meet all of the  clinical bedside criteria—no eye movement, no response to pain, no attempt to breath on an apnea test—because of hypothermia. This usually happens in very cold climates. Sometimes the temperature is normal but the apnea test isn’t carried on long enough for the carbon dioxide to rise sufficiently (carbon dioxide, not oxygen, controls the impetus to breathe—a believe it or not fact worthy of Ripley).

Remember, in all of this, the important thing is function. What is the brain doing or not doing? The brain is an organ like any other, with a so-called end organ function. The end organ function of the kidneys is to produce urine. The heart circulates blood. The lungs exchange oxygen and carbon dioxide. The end organ function of the brain is to ultimately coordinate every outward response a human produces (except for those coming from the GI tract, a fascinating fact in itself) and a myriad of inward responses as well (mundane things like coordinating walking and raising and lowering blood pressure, heart rate, and breathing, as well as deciding when it is socially acceptable to deficate or urinate).

If the kidneys fail, there is no urine—and no excretion of waste products. Without dialysis, the patient will die. If the heart fails, there is no pumping of blood. Just as often, the heart fails to pump effectively, which results in congestive heart failure. If the lungs fail, the patient suffocates, or suffers from oxygen hunger as in a bad pneumonia.

If the brain fails, the outward responses it coordinates cease. However, because the functions of the brain are compartmentalized, when it fails (as in a stroke) usually only some of the outward responses cease (for example, the patient stops talking—aphasia, or stops moving on the left side—hemiplegia, or develops blindness—amaurosis, or can’t coordinate movements and looks clumsy—ataxia, or…you get the idea).

If the brain fails to such an extent that most of these outward functions cease (we might call this partial or congestive brain failure in an analogy to congestive heart failure), the patient is in a coma.

But what about when the brain fails so massively that all outward—and inward—responses cease?

That is brain death folks, and it’s irreversible. Death by another name is still death.

One more thing, and it follows naturally from the above but may not be intuitive to the lay reader. If all outward and inward responses to stimuli fail in brain death, the body itself must fail eventually. Why? Because like an airliner deprived of its pilots, there is no master at the helm.

Such a plane may keep flying initially, but sooner or later (I’m guessing sooner) it will encounter an updraft or downdraft it doesn’t know what to do with. Hell, the plane doesn’t even know it’s flying. It has no awareness—and no ability to respond to its surroundings. And, as the second law of thermodynamics tells us, it will tend toward entropy and chaos. So it falls out of the sky.

A body without a brain is just as clueless. The heart may keep beating, but it has no awareness it is doing so. It can’t measure blood pressure, let alone adjust it up or down to the needs of the moment. The kidneys will still filter the blood and produce urine, but without the brain to tell them how much potassium to excrete or how much sodium to hang on to, their mission is doomed to failure. Even the lungs, seemingly nothing more than a bellows, will begin to collapse on itself without the brain to instruct it to take a deeper breath than normal several times a minute to clear any building secretions that might otherwise clog up its vital passages. You do this and swallow the result hundreds of times a day; when was the last time you swallowed? Did you think about swallowing? Were you even conscious of the fact you swallowed? Of course not. But your brain was, and is. Your brain keeps track of such things and a million other little tidbits you can’t consciously be bothered with as well.

A body without a functioning brain is a system tending toward chaos and will fail in a matter of days, a week maybe. It might last a little longer with close attention from both the medical and nursing staff attending to things like hydration, careful positioning to help the lungs, and drugs to maintain blood pressure etc., but not much longer. A few days at most. Certainly not months or years.

But a body in a vigilant coma—partial brain failure—can go on for years. Just google Karen Ann Quinlan. No hand of God miracle there, unfortunately.

Next time at Neurosurgery101—TheBlog: The most amazing thing—a miracle maybe, a Ripley’s Believe It or Not moment for sure—I have seen in twenty plus years as a brain surgeon.

Not One Among Them Whole, A Novel of Gettysburg, by Edison McDaniels

Not One Among Them Whole, A Novel of the surgeons at Gettysburg, by Edison McDaniels. Kindle & paperback now available.

03/27/16
HandOfGodP2

The Hand of God? II

HandOfGodP2

 

Part II: The Hand of God?—Looking at Brain Death

By Edison McDaniels, MD | surgeonwriter.com
You might want to read the first article in this nonfiction series first. [Click Here]

Keywords: nonfiction, brain death, coma, CT, MRI, miracle

Recently, I proposed a heart-breaking scenario, a badly injured man, surrounded by loved ones, apparently brain dead—who lives to defy his physicians.

Do such miracles really happen? Can a person really come back from brain death?

As a neurosurgeon, I have been involved in too many brain death scenarios to count. Let me say at the outset I have never encountered a hand of God miracle—not around the issue of brain death anyway.

That said, I have seen several patients I didn’t expect to survive—let alone thrive—do both. But that’s not the same thing. These patients were badly injured, but not brain dead. Some were in a coma, but that’s not the same as brain death. As a brain surgeon, I can tell you unequivocally it’s not possible to foretell how well a badly injured brain will recover. I’ve been surprised by this more than a few times. People I expect to do well don’t, and people I expect won’t do well do. 

It’s a matter of pathophysiology—we can often see the injury on our tests, but not how that injury will play out functionally with the rest of the brain. It’s a matter of function. We can’t see physiology in action on a CT or MRI. Note: we can see physiology in action on a PET scan, but these are used more for research than clinical decision making and not widely available in most hospital settings.

That’s fundamentally different however from brain death. In brain death, we aren’t looking at physiology but function—or rather the lack thereof.

Here’s a truism. The best test of how somebody is doing neurologically (that is, how their brain is functioning) is not a CT scan, MRI, or EEG. The best test to see how somebody is doing neurologically is to observe how they actually behave. That’s right. Forego all the expensive state of the art tests and actually observe the patient.

Do they respond to their own name?

Are they moving their arms and legs? Either spontaneously or to stimulus (such as the prick of a needle to draw blood)?

Do they speak, or attempt to speak?

Do they open their eyes?

Do they gum the breathing tube with their mouths?

Do their pupils respond to light?

Do they move when the sedation is removed?

Do they attempt to breathe on their own when the ventilator is turned off?

If a patient does any of these things, their brain may still be badly injured but they aren’t brain dead. And if they aren’t brain dead, it is extraordinarily difficult to foretell how far somebody will progress along the road to recovery (though if the injury is severe enough to be asking these questions it is unlikely they will recover to be the exact person they were before the injury).

But this still doesn’t help us with brain death. A patient who does none of the things listed above is not necessarily brain dead. Indeed, most of the time such a patient is not brain dead, but in a coma. Coma is not brain death.

So how do we, as physicians, know when a patient is truly brain dead and not just comatose?

The definition of brain death is an irreversible cessation of all functions of the brain, including the brainstem (which houses the most primitive brain functions necessary to support life: heart rate, maintenance of blood pressure, and control of breathing). It must be noted that a patient determined to be brain dead is legally and clinically dead.

But, practically speaking, when is a person brain dead?

Angio Blood flow

Answering that question can be surprisingly difficult for the neurologically unsophisticated. It turns out there are, in fact, several ways in which to show brain death. The summation of these will provide unequivocal evidence of brain death. All of these have to do with function and not just physiology:

  1. A cerebral angiogram that shows no blood flow to the brain. It is not possible for the brain to function in the absence of blood flow. This test is unequivocal.
  2. A CT of the brain with IV contrast that shows no blood flow to the brain. This test is unequivocal.
  3. An EEG that is flat line. The functions of the brain are transmitted via electrical pulses, which can be captured on EEG. In the absence of these pulses, there is no function and the brain is dead.
  4. The absence of all of these on exam: no pupillary light reflex; no movement or response of any sort to stimulation (such as grimacing or an increase in heart rate to a painful stimulus); no eye movement when the middle ear is stimulated with ice water (this is a severely uncomfortable stimulus in the living); no corneal response; no attempt at breathing when the ventilator is turned off (this is referred to as the apnea test).

For the apnea test to confirm brain death, the CO2 must rise above a certain threshold since, oddly, it is actually the level of CO2 and not the level of oxygen that stimulates breathing (this is why swimmers who hyperventilate and blow off CO2 may drown when the oxygen level falls before the CO2 level builds enough to force a breath; they pass out and drown when the rising CO2 causes them to inhale water).

Angio-no blood flow

In addition to the above, the patient must not be cold (hypothermia may simulate death), and there can be no sedating medications or paralytic drugs on board—a condition which is not always met in ICU patients who are frequently sedated and paralyzed to facilitate ventilation.

The law usually requires, and hospital policies usually demand, that two separate brain death exams be repeated six or more hours apart to confirm the results before they become official.

In my experience, and that of every neurologically sophisticated physician I have ever discussed this with, if the above conditions are confirmed, recovery is not possible and is never seen. So how then do we explain the all too common cases of so-called recovery from brain death?

That’s next time at Neurosurgery101—TheBlog.

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