The resident was unprepared to get called on this offhand comment. “Um, well, I was under the impression that anemia is often seen after delivery, right?” She looked at the fellow for support, but the fellow was scribbling notes on her rounding sheet and leaving the teaching to Van Marter.
“Yes, it is common to see a low blood count in the NICU,” Dr. Van Marter answered the resident. “But when do we typically see anemia? It’s not on the second day of life, is it?”
“Around a week?” The resident looked like she was guessing.
“Yes. So you would recommend . . .”
“Repeating the blood count?”
“This afternoon.”
“Absolutely.”
Van Marter was puzzled. She had watched Larissa’s delivery and had been the one to perform the intubation. The colleague who had covered the NICU overnight had reported nothing out of the ordinary when she’d signed out the patients to Van Marter that morning. Larissa’s first few hours had been relatively smooth—at least as smooth as could be expected for a baby born at twenty-six weeks.
But Larissa’s blood count was too low. Van Marter made a note to watch out for the results of the repeat blood test after lunch.
“So,” Van Marter said, turning to the medical student in the group and giving a wink to the resident she had just finished questioning. “What are the possible causes of a low blood count?”
“Well,” said the medical student, stalling. “ABO incompatibility could cause a low blood count.” The student looked like he was trying to conjure up a page of his textbook in his head. The pockets of the short white lab coat that needed a cleaning were stuffed with papers and reference books.
“Yes, it could. How common is ABO incompatibility?”
The medical student shook his head.
“Zero point six percent,” said Van Marter, indicating the rarity of this condition in which the mother’s immune system attacks the baby’s red blood cells. “And do you know what percentage of babies have a low blood count as a consequence?” The student again shook his head. “A very, very small percentage.” Van Marter enunciated each word. “Any other thoughts?”
“It could be caused by bleeding.”
“Excellent. What type of bleeding?”
“Blood loss at delivery?”
“Yes; where else?”
“Internal bleeding.”
“Uh-huh, and where? Where do premature babies bleed?”
“Internally?” The medical student apparently hadn’t read this section of his text, and he stuffed a bony hand into the pocket of his slacks.
“Okay . . . How about studying the causes of neonatal anemia and reporting back to the team tomorrow at morning rounds? I guess we know what you need to read about tonight.”
The group moved to the next baby.
With less difficulty than she’d had the previous evening, Kelly got into her wheelchair and we rolled to the NICU. We now knew that when we passed into the alcove between the locked outside door to the NICU and the door that opened into the vast and busy space, we were expected to stop and wash our hands, which we did.
Maneuvering the wheelchair to Larissa’s station, I couldn’t help noticing that the space that had been blocked by the blue privacy partition the previous evening was now empty.
Overnight, Larissa had been moved off the open table and into a Plexiglas-enclosed incubator. She lay propped on her side, snuggled between rolled-up blankets and moored by the semirigid ventilator tubing.
I rolled my wife’s wheelchair up to the incubator, and Kelly readily unlatched the two portholes and reached in to touch our daughter. We pressed our faces to the plastic and watched Larissa. Kelly gently rubbed Larissa’s hand and smiled when Larissa reacted to the touch.
“She had a good night.” The nurse smiled at us when she said this.
“Did you have a good night?” I asked Larissa.
“Oh, that’s good.” Kelly did not seem entirely ready to believe it.
Later, as we were leaving the NICU, Dr. Van Marter peeled off from the group she was huddled with, caught up with us, and introduced herself. She offered her congratulations on the birth of our daughter and told us she was optimistic because Larissa’s first day had gone well.
“Thanks so much for everything,” Kelly said. She was looking tired and a bit pale. I started to turn the wheelchair toward the door.
“You know”—Van Marter interrupted my motion with her voice—“her blood count was a little low this morning, and I repeated it and it’s still low, so I’m going to get an ultrasound just to be sure.”
“An ultrasound of what?” Kelly asked.
“Of her head,” Van Marter said without sounding too concerned. “It’s probably nothing, and usually we don’t get an ultrasound of the head until babies are three days old, but why not? Let’s go ahead and get it now.”
“Sounds good,” I said with a smile, but suddenly I felt beads of perspiration and heard a roaring sound in my ears.
“I’m here until tomorrow,” Van Marter said, “so we can touch base later.” She walked off. I pushed Kelly’s wheelchair out of the NICU.
“What’s the ultrasound for?” Kelly asked. “What are they looking for?”
“Bleeding. An intraventricular hemorrhage. They call it an IVH.”
“Do you think she has that?”
“I doubt it,” I lied. I could see no reason to worry Kelly with another issue that was out of our control. “I’m sure Van Marter is just being careful.”
Kelly wanted to take a nap, so after getting her settled in bed, I headed for the library. Four stories above the Brigham and Women’s lobby, tucked behind an inpatient ward, the library served a lot of purposes—a meeting space for residents, a place to check e-mail and, when time allowed, catch an hour of sleep on one of the comfortable, if slightly dingy, couches. I swiped my hospital ID to open the door and then sat at a computer terminal between a young doctor writing a scientific paper, his notes strewn haphazardly around him, and a medical student intently engaged in an instant-messaging exchange. Around me, tired residents scanned patients’ labs, checked e-mail, or looked for cheap flights to vacation spots.
I was there to do research on intraventricular hemorrhages. I searched for studies that showed how premature babies who’d had severe hemorrhages near birth did in childhood. There were only two studies, and neither was encouraging: a small number of children suffered only mild disabilities, but most were profoundly affected—with mental retardation, seizures, cerebral palsy, and other disorders.
The brain is composed of billions of cells that make trillions of connections with one another. At the highest level is the cortex of the brain, which communicates with the body by sending signals down the spinal cord.
Think back to your high school science classes and remember the picture of the brain: two hemispheres encasing a pair of fluid-filled cavities, called ventricles. The fluid in the cavities, which is indistinguishable from water to the naked eye, is cerebrospinal fluid. It bathes and cushions the brain by constantly circulating through a sequence of narrow channels that connect the ventricles to the space surrounding the brain and the spinal cord.
Along the ceiling of each ventricle lies a fragile network of blood vessels called the germinal matrix. This immature matrix tends to bleed after a premature birth, spilling blood into the fluid-filled ventricles. Once blood accumulates, in the ventricle or anywhere else, it clots.
Scanty bleeding is clinically meaningless, as the drops of spilled blood are reabsorbed over time. In fact, minor bleeds are so common that if every full-term baby had an ultrasound of the head, up to 10 percent of them would be found to have evidence of bleeding—a testament to the irrelevance of a truly small hemorrhage.
Significant bleeding into the ventricles is an entirel
y different matter. A large clot can clog one of the narrow channels and obstruct the flow of fluid, causing pressure in the ventricle to build. When pressure inside a ventricle increases, the ventricle expands, strangulating the blood supply to the brain cells that line the ventricle, slowly killing them. Injury—to brain cells, to the spinal cord, even to the cells that insulate the neurons—interrupts the connections that are central to the brain’s function, and broken connections result in impairment of movement, thought, and behavior.
Significant bleeding can also occur within the brain tissue itself, and an increasing amount of blood compresses the surrounding brain tissue, killing nearby brain cells and leading to permanent disability.
Hemorrhage in the brain is diagnosed with an ultrasound. The dishwasher-size machine, just like the one used to view the fetus during pregnancy, is wheeled to the bedside, and the technician snakes a handheld probe into the incubator. The probe is positioned against the newborn’s head; ultrasonic waves are sent inside the skull, and the sound waves that bounce back are analyzed by computer and then transformed into an image that’s displayed on the screen.
Later that night, after Karen, Hannah, and Grace had visited and while Kelly was watching an Ally McBeal rerun on television, I went to the NICU to inquire about the ultrasound.
I found Dr. Van Marter discussing some lab values with one of the fellows. It was late, and the NICU was quiet; the lights were dimmed, and a smaller staff tended to the babies overnight. The neonatologist saw me and motioned for me to wait while she finished her discussion, and then she came over to me. “Let’s go someplace quiet,” she said, touching my arm to lead me out of the NICU.
She took me down a hallway to a conference room, where we sat alone at the end of a long table.
“I know Larissa lost some blood in the operating room,” she began in a balanced tone. “So I wasn’t too surprised when her hematocrit was low. But I expected it to come up when we transfused her.”
“I didn’t know she was transfused—” I began.
“She was.” Van Marter silenced me, then returned to her topic. “The blood count came up, but not as much as it should have. You probably know that the brain is the most common place that premature babies bleed, which is why I ordered the ultrasound study.” When a significant intraventricular hemorrhage occurs, red blood cells pour out of tiny vessels and accumulate in the ventricular fluid; lab tests then show a lower number of red blood cells in the circulatory system.
Van Marter looked down, and then looked up at me. “The ultrasound showed a bleed. A very serious bleed. Though not the worst I’ve ever seen, Larissa’s hemorrhage is on the more severe end of the spectrum.”
On an ultrasound image, fluid in the ventricle appears as a dark half-moon within the uniform gray of the surrounding brain tissue. Blood appears as a shocking white cloud within the black of the ventricle or against the gray background of the brain tissue.
The ultrasound confirmed Dr. Van Marter’s diagnosis and one of my worst nightmares. The initial scan showed that blood had flooded the ventricles, threatening to raise the brain’s internal pressure and compress the healthy brain tissue. It also showed blood in the tissue surrounding the ventricle on the left side of Larissa’s brain, as well as in the cerebellum, the small sea-urchin-shaped portion of brain that juts out from underneath the two hemispheres at the back of the head. The cerebellum is the coordinator of the brain; it organizes and synchronizes much of the brain’s thoughts and functions, and hemorrhage here is usually a catastrophic event.
Last, the ultrasound showed that the brain had begun to swell—fluid had built up in the left ventricle, and the ventricle was expanding into the space that ought to be occupied by the left hemisphere of the brain. There was even evidence that the membrane that marks the midline of the brain was shifting toward the right, meaning the whole left brain was swelling out of its natural space and encroaching on the right side of the brain in an effort to accommodate the increasing size of the left ventricle.
The radiologist who’d looked at the images and dictated the report described what essentially amounted to an explosion of blood in Larissa’s head. We were seeing the beginning of a cascade of events inside the skull whose outcome was unknown.
A day after her birth it was clear that the term unscathed had been taken off the table. After my library session, I knew the consequences of Larissa’s ultrasound findings: she would likely have catastrophic movement impairment similar to severe cerebral palsy and affecting all four limbs. It was unlikely that she’d have normal intellectual function. Her early life would be marked by neurosurgery to relieve increasing pressure in her brain and orthopedic surgery to relieve the consequences of muscles unable to relax. Body functions and developmental milestones most of us take for granted—swallowing, seeing, hearing, sitting up, learning to speak—might be achieved slowly, or not at all.
Because of the newborn brain’s plasticity—meaning its remarkable ability to reassign tasks from an injured area of the brain to a healthy one—we had no idea where on the continuum Larissa would fall. An adult with Larissa’s ultrasound findings would certainly die or be severely and permanently disabled, and either outcome was a real possibility for Larissa. However, unlike adults, newborns have robust neuroplasticity—brain plasticity—so Larissa’s uninjured brain cells might eventually compensate for the brain tissue that the ultrasound showed was now a bloody morass.
Meanwhile, her immediate survival was in no way guaranteed, particularly given the evidence that bleeding on the left side of her head was causing compression of her brain. Dr. Van Marter explained that Larissa would have daily ultrasounds to track the progress of her hemorrhage.
“We will have to see whether the hemorrhage stabilizes or gets worse over the next few days,” she said. “If she bleeds more, there may be some hard choices to make on Monday or Tuesday.” Then she added, “But if the bleeding continues, Larissa may make those choices herself.”
I followed the pediatrician’s train of thought. “If there is a decision to be made, is it mine and Kelly’s to make?”
“It will depend,” she answered. “Let’s take it one day at a time and see what Larissa tells us.”
In a daze I walked back to Kelly’s room. I passed a colleague who asked me how Larissa was doing; I walked by without acknowledging his presence. I felt numb—not sad, or anyway not despondent, but unable to process, unable to consider what was happening. It was all I could do to make it back to Kelly’s room.
“What did she say?” Kelly asked. She had been waiting for my return. I wasn’t sure how long I’d been gone.
I shook my head. Kelly started to cry as I sat down on the bed next to her.
Chapter 3. Gifted Hands
The holiday season was a busy time for the chief of the largest NICU in New England. Dr. Steve Ringer was tall, not lean, and wore a thick beard, making this Jewish physician from the Boston suburbs ideal for the role he played every December: Santa. One December morning found him handing out gifts at the Department of Neurology’s holiday party as children climbed on his lap and whispered in his ear about toys.
Five floors above, a woman who had been hospitalized for three weeks due to concern that she would deliver her baby prematurely called her nurse and explained that she felt like she needed to pass a bowel movement. Because that sensation is not dissimilar from what a woman may feel right before her baby is born, the nurse panicked, told the patient she needed to wait until a doctor examined her, and raced back to the nursing station to call for help.
The young resident physician who arrived didn’t recognize this sign of trouble, and she allowed the patient to proceed to the toilet, where she unceremoniously delivered a vigorous three-and-a-half-pound baby boy into the bowl.
A few minutes later, Santa’s pager went off. Ringer raced upstairs in full red regalia and arrived just in time to se
e the little boy fished out of the toilet. As the shocked mother dropped back onto the toilet seat, Ringer helped dry the baby and told her he was pleased to report that the newborn was doing well. Soon the neonatology team caught up with their leader, bundled the new arrival into an incubator, and whisked him off to be evaluated. After congratulating the mother and assuring her that her baby looked very healthy, Ringer returned to the party.
Ringer was, in some ways, an unlikely leader of the NICU. He had grown up in the Boston suburb of Newton, not far from where he currently lived. He had watched his father, an internal medicine doctor, spend long days seeing one patient after another. It seemed to Ringer that although physicians occupied a place of distinction and respect in the community, their actual work was somewhat boring. In a community where Harvard Medical School cast a long shadow, Ringer thought that science—with its potential for lifesaving discoveries—was a more interesting destination than the exam room and so he had immersed himself in laboratory work from an early age.
Ringer went to college at Brandeis (fifteen minutes from Newton), where the laboratory opened up to him. He got a taste for experimenting in biology and chemistry and came up with a plan: he would become a physician scientist.
With his wife, Ellie, Ringer moved to Cleveland, where he went to medical school and got a doctorate in biochemistry. “I spent a lot of time shining lasers through solutions to measure the mass of large molecules of DNA,” he recalled. He decided on pediatrics and started his residency at Case Western Reserve.
Residency was a time of false starts. His experience in the clinic treating runny noses and earaches confirmed his suspicion about general practice: it was boring. In order to avoid the boredom, he needed to subspecialize. The question was, What subspecialty should he choose?
Because of his interest in research, he thought that clinical genetics would be his future, but a month-long rotation piecing together complex diagnoses from the constellation of unusual clinical findings found him bored yet again. A friend offered some advice: “When you find yourself browsing shelves in the medical library, pay attention to the journals and articles that hold your attention.”
Fragile Beginnings Page 4
