A Gift

As I hovered over the isolette, just when evening had begun to feel like the middle of the night, I wondered why the task had befallen me to damn the soft, warm, fresh life in my hands. Grasping the coiled tubing, I pressed its blunt silver tip to the infant’s head.

"Throw the switch," I said.

From across the narrow bed, the nurse nodded.

Suddenly the infant’s skull glowed, the focused light beam finding no resistance to its path. I slid the transilluminator over the scalp, side-to-side, front to back. Except for the stark, blood-red sprig of an occasional vessel, there was nothing but homogeneous, yellow-pink haze from above the eyes to the base of the skull, from ear to ear.

So it was true. The ultrasound at the outside hospital had glimpsed a dreadful mistake of nature, a freak mishap.

The nurse shook her head. She had seen it before, too.

"You can turn off the light," I said.

I slipped a pacifier into the baby’s mouth. She sucked. She grasped my index finger, wrapping her translucent little fingers, tiny nails and all, around mine. Still too new to have a first name, she peered at me through narrow eyelid slits, as if to query who dared disturb her tranquil sleep.

One could still hope for a rim of cerebral cortex, no matter how thin, one which might be missed by transillumination. That would mean hydrocephalus -- something at least treatable. Any other cause for her large head would offer a slightly better prognosis. But I could not convince myself to hope.

When I left the NICU, memories flooded my weary mind from a distant hospital and much forgotten time. They rushed to the surface from beneath the sediment of swift river time that long ago carried me deep into life’s choices. Now the smell of the hand soap, the lemon-yellow gowns, pulsations and bleeps of monitors, even the snap and swoosh of the automatic doors as I left the nursery, reminded me of years spent caring for sick babies in the wee hours.

As I passed the empty cafeteria, the few workers seemed as dreary as the people in Van Gogh’s "Night Cafe." I recalled how I used to stand at the grill where I trained as a resident. Midnight was last call. Getting that grilled cheese with a slice of bacon seemed to give me the energy I thought I needed to survive the night. Now I regret the bacon.

Downstairs, entombed in the catacombs beneath the hospital, I finally arrived at the radiology viewing room. As soon as I snapped the baby’s films onto the one lit view box, the MRI confirmed my suspicions: no higher brain. Midbrain, pons, medulla, even a rudimentary diencephalon and vestige of frontal cortex showed up white in the dark void; however, no cerebellum, no basal ganglia, no cerebral hemispheres. Like a vast gray ocean, the abundant space the "thinking brain" should have occupied was filled instead with fluid.

On the long walk back to the NICU, I wondered how things had gone so dreadfully wrong. I could envision primordial cells dividing and migrating, totally dependent on genes and chemical signals to guide them. Then the growth messages stopped. Early in the pregnancy, perhaps even when the parents were planning the baby’s room or cuddling in bed and giggling over its first kicks, developmental order succumbed to chaos. Most fetuses with severe brain malformations spontaneously abort, but at a tertiary hospital, one has to expect to see the ones that don’t. The real question is how most infants all over the world for millennia have turned out so well.

The next day, one of the nurses noted brief jerking of the arms and legs. Reconsulted over the possibility of seizures, I returned to the nursery. Phenobarbital had been given. The examination revealed brisk deep tendon reflexes, but no seizure activity. I suggested an EEG. The irony struck me: not enough brain to be a human being in any real sense, but enough to mount a seizure.

On the way out, I mentally glimpsed the parents in the delivery room. They must have been told something was wrong, but were they prepared for the seriousness? Sure, in medical school I had seen grotesque specimens floating in formaldehyde-filled jars. I had studied photographs of malformed fetuses in books. I had even taken care of anencephalics, who lacked a skull overlying the empty brain cavity. It was easy to detach from them -- they looked subhuman. But this infant had clasped my finger. Whatever developmental monstrosity lurked beneath the skull, below the neck was a beautiful baby girl ready to live.

Two days later, I received a call to participate in a conference with the baby’s parents. The mother, still recovering from her Caesarian section, was well enough to travel to our hospital. I arrived early to find the parents at the bedside in the nursery holding the infant like the other proud parents, who were surrounded by family or friends. They showed no signs of the gravity I felt.

We convened in a nearby small, utilitarian conference room. The social worker, a nurse, and the neonatologist flanked the parents as they sat at the table.

The mother looked plain. Her dyed brown hair was very gray at the roots -- probably too much of a burden to maintain as the delivery day drew nearer. Fatigue registered in her demeanor. The husband was a large man, whose forearms and shoulders suggested a manual laborer. His features were coarse and his hair was streaked with gray.

The neonatologist introduced me and asked that I speak to the diagnosis and prognosis. I’ve learned the hardest thing is to start. From among all the possibilities that jump at you under the pressure of the moment choosing the right words -- kind, sympathetic, yet accurate words -- is the most difficult. Everyone’s eyes were on me. I felt a twinge of the feeling I used to get as an intern when I had to tell parents in the middle of the night that their child had died.

"Your baby has hydranencephaly."

The father began to take notes. "Could you spell that?" he asked calmly, as though he were at a board meeting.

His wife watched him scrawl the letters on a small yellow pad.

"That means the brain did not form normally, I’m sorry to say. The part that maintains heart rate, blood pressure, and respiration is okay, so the baby still breathes, sucks, and swallows."

"Isn’t that what normal babies do?" the mother asked. Her face was expressionless.

"Yes -- ." I paused. Before I knew it, I had sighed. "I guess what I’m trying to say is that’s all the baby is capable of. What’s missing are the parts that form thoughts, memories, personality -- the brain that sees, hears, talks, interacts, or loves -- all the things we take for granted. What normal babies do in the first few months of life doesn’t require much brain." I made a helpless gesture.

They looked incredulous.

I held up one film from the MRI scan overhead in front of the ceiling light and explained what they were looking at. Both parents fell silent.

"Why did this happen?" the father asked.

"No one knows for sure. During brain development, blood vessels that would have nourished the upper brain never formed or closed off. Many genes control brain development -- we’re only starting to understand that -- and something could have gone wrong with them. Or an infection is sometimes the cause."

They looked over at me. It was that look. I knew she would begin racking her brain for the one incident during the pregnancy that might have caused the anomaly.

"Whatever the reason, it’s not your fault. There’s nothing you could have done differently."

Secretly I wondered what role parental age could have played. Nature can be cruel to older parents.

"How long will she live?" the father asked.

"Odds are, not more than a couple of months." I raised my eyebrows and cocked my head to the side. "Some children do survive for years -- I’ve seen it happen -- but that’s rare."

"So what can we expect?"

"Well, she may develop seizures. The baby’s EEG -- that’s a brain wave test -- showed lots of abnormal electrical activity that can cause seizures. Normally the higher brain would suppress that, but there is no higher brain. Muscle tone also will increase until the arms and legs become fixed in position. Her head may become much larger, too. The children often die of infection, or the brain seems to run out of some vital energy."

"Would it help to drain fluid from the brain?" the father asked.

"Putting in a shunt to drain the fluid might help cosmetically as the head expands, but it won’t prevent the inevitable."

The neonatologist spoke up. There was a hint of urgency in her voice. "We need to make some important decisions for the baby. We need to know what your wishes are. Do you want us to put a breathing tube into her throat if she stops breathing? Do you want us to put a feeding tube into her stomach if she doesn’t eat? Do you want us to resuscitate her if her heart stops or her blood pressure falls?"

"Would she live longer if we did that?" the father asked.

"Yes, but be careful what you wish for," the neonatologist said. "You may prolong her life, but you will have to feed her through a stomach tube. She will need a hole in her throat, called a tracheostomy, where a breathing machine will be attached. She will need constant care because she won’t be able to care for herself."

"Will she grow?"

"She’ll grow, but not at the normal rate."

"So all her other organs are normal."

"Yes."

Oddly, he seemed relieved. Did he not understand?

"Can you tell if she will feel pain?" Everyone turned to the mother, who broke her long silence. Then they looked back at me.

"She will withdraw from pain, but only as a reflex. She will cry out of hunger, maybe even move to a bright light or loud sound, but she will be blind, deaf, and completely vegetative."

The couple was quiet for a few long moments.

"I know it must be difficult," I said. "She looks so normal on the outside except for her large head."

The mother nodded. This time her eyes were moist. "We’ve been trying so hard to have this baby," she said.

"It’s a lot to hear all at once. I’m sure this isn’t what you expected," I added.

Her husband looked up. "Well, of course, we want her around as long as possible. She’s our baby. She’s a gift from heaven."

We all must have glanced down at the same time. What I felt in the pit of my stomach was the same pang I felt on every such occasion. I had to greet perfect strangers as the messenger of devastating news. It never got any easier. No matter how badly I felt, it must have been so much worse for the parents.

When I peered up again, the whole medical team still seemed uncomfortable.

The couple looked at each other. Tears rolled down the mother’s cheeks. I got the feeling this was their last stand. From the chart, I knew how many previous attempts to have a child were unsuccessful.

"A word of caution," I added. "It is much easier not to go with the breathing tube in the first place than to remove it. If the baby survives, you’ll be committed. This isn’t going to be like brain death where you can remove the tube later at will."

The mother studied me. That point registered. Her face contorted and her mouth drew up, exposing the gaps between her side teeth. "I don’t want her to live like this."

"Are you in agreement?" the neonatologist asked the husband. "We are talking about a do-not-resuscitate order."

He met her gaze without answering. His demeanor said no, but when he viewed his wife’s agonized face, slowly he pursed his lips and placed his hand on her shoulder. "Okay." A long pause. "Whatever she wants."

"You can talk this over between yourselves, if you’d like," the neonatologist said. "We’ll leave the room."

The mother shook her head adamantly. "I don’t want any breathing machine or anything like that." Her voice was strong and resolute. Then she began to sob.

After a moment, the social worker asked, "Do you have the moral support of family and friends?"

She nodded. "My family lives here."

"Good."

When the meeting disbanded, I left the NICU to see other hospital consults. It wasn’t a day I was glad to be a child neurologist. I’m used to being called upon to give unpleasant news and to try and explain what doesn’t make sense. Was it my getting older that made things bother me more? Without knowing it, had I attended one too many of these terrible sessions?

Or did life just seem more precious?

Michael R. Pranzatelli, M.D.

Pediatrics

Third Place, Prose