At
nine o’clock on July 28th last year, Wendy Nissley carried her
two-year-old daughter, Lacy, into O.R. 12 at Johns Hopkins Hospital to
have half of her brain removed. Lacy suffers from a rare malformation
of the brain, known as hemimegalencephaly, in which one hemisphere
grows larger than the other. The condition causes seizures, and Lacy
was having so many—up to forty in a day—that, at an age when other
toddlers were trying out sentences, she could produce only a few
language-like sounds. As long as Lacy’s malformed right hemisphere was
attached to the rest of her brain, it would prevent her left hemisphere
from functioning normally. So Lacy’s parents had brought her to Johns
Hopkins for a hemispherectomy, which is probably the most radical
procedure in neurosurgery.
Wendy laid her daughter on the
operating table. Because Lacy was so small, it took the
anesthesiologist almost ninety minutes to insert her intravenous lines.
George Jallo, the attending neurosurgeon, spent a long time arranging
her head on gel padding and then drew “Cut here” markings on her shaved
scalp. The rest of Lacy’s head, including her face, was covered with a
sterile drape. Jallo made one long cut across the top of her head from
the front to the back, and another at right angles to the first, which
started midway along it and stopped just in front of her right ear. He
folded back the scalp and made small holes in her skull with a power
drill, outlining a rough semicircle. Then he used the drill to connect
the dots and removed a portion of the skull. He cut another T in the
dura, a thin, leathery membrane covering the brain. Gently, he peeled
back two large flaps.
By half past one, Jallo and a
resident had already removed the right frontal lobe. David Lieberman,
the pediatric neurologist who had examined Lacy when she first came to
Johns Hopkins, looked on, shaking his head in wonderment. “It’s so
open,” he said, turning to me. “Normally, with brain surgery, you make
a hole about this big”—he curled his thumb and index finger into a
circle.
After removing the frontal lobe, Jallo embarked
on the parietal lobe. In case complications put a sudden stop to the
surgery, it was important to take out the seizure hot spots first,
gradually working through the hemisphere in descending order of
priority: after the parietal lobe would come a small section of the
occipital lobe, then the temporal lobe, then the rest of the occipital.
Finally, Jallo would cut the corpus callosum, the bundle of fibres that
connect the two hemispheres of the brain. The surgeons slowly worked
around each side of the parietal lobe, making tiny pinches in the brain
with electric cauterizing forceps. There was a slight smell of burning
in the bright, noisy operating room. As the cut became deeper and
wider, the tissue on either side browned and blackened, and the lobe
started to move back and forth. At the bottom of the parietal wedge,
the clean white of nerve fibres was visible; as the lobe was severed,
they came apart like string cheese. A surgical technician bent toward
Jallo with a small plastic bowl in his hands. Jallo picked the lobe out
of the skull—it was the size of an infant’s fist—and dropped it into
the container.
As she led me out of the O.R., Eileen
Vining, the attending neurologist, said, “Did you see how rigid it was?
Normal brain sags in your hands.” Vining talked quickly, moving from
one complicated idea to the next, punctuating each with “O.K.?” and an
expectant nod. She had been in and out of the operating room all
morning, and now she was off to find the Nissleys and tell them how
Lacy was doing.
Four hours later, Vining took me back
into the O.R. Lacy’s right hemisphere was gone, and her cranium looked
like a wide, uneven bowl. I could see the deep cavity where the frontal
and parietal lobes had been, and the white-pink color inside the base
of the skull. In the middle of the remaining brain was a shallow mound
where Jallo had left a layer of nerve fibres to protect the ventricle,
a fluid-filled pocket that cushions the brain and the spinal cord. The
white matter there was now gray-black. Jallo and his resident lightly
touched their forceps to it, and the cauterizers fizzed, sealing the
brain to prevent microhemorrhages. Hemorrhaging is a constant concern
in brain surgery, and at one point in the operation Jallo decided to
leave in a small piece of the right occipital lobe which threatened to
bleed dangerously. Jallo glanced at Vining and Lieberman, and the
doctors stretched forward to look at the severed corpus callosum. Over
and over, the surgical technician poured in saline, and Jallo and his
resident drew it out again with a loud suction pump.
When
he had finished removing brain tissue, Jallo tipped in small packets of
Surgicel, a feathery white substance that helps blood to clot. It
melted onto the surface of the brain. “That was good. There was not a
lot of bleeding,” Vining said. “You never know what you are going to
get until you open it up. Sometimes you just go in there and you hold
your breath and pray.”
In the final hour, Jallo sutured
Lacy’s dura, which had shrunk slightly from dehydration, and filled the
right side of her head with saline. The technician then brought the
missing section of the skull back to Jallo, carrying it in a wide right
angle over surgical carts rather than risk moving it over the floor.
Jallo reattached it, using four tiny dissolvable plates made of a
sugarlike substance. He then closed the scalp incision with a staple
gun, leaving seventy-eight aluminum staples in Lacy’s skin. The
hemispherectomy had taken nine hours. The resident bandaged Lacy’s
head, gently turned her onto her right side, and stuck a piece of tape
on her head that said “This side up.”
I
first met the Nissleys three months before Lacy’s surgery, at their
home in Palmyra, Pennsylvania. As I drove up, I could see Lacy’s
father, Mike, dangling a leg over the edge of the front porch. He
looked like a surfer, with a goatee beard and gold-lens Oakleys pushed
back on his head. Wendy sat on a chair near the front door, and Lacy’s
four-year-old sister, Lily, flitted around the adults. Lacy, blond like
her mother, lay across Wendy’s lap. “She had a seizure just before you
got here, so she’s sleeping now,” Wendy said. They invited me inside.
Lacy
had her first seizure on October 13, 2003, when she was four and a half
months old. Wendy had been doing laundry, and when she turned around
she saw that Lacy’s lips were blue and that she was shaking. She called
911, but by the time the ambulance arrived the seizures had ended. In
the following month, the family went to the hospital many times, but,
on each occasion, by the time they got there the spell had ended and
there didn’t seem to be anything wrong. The doctors took an MRI but saw
nothing unexpected, and they suggested that Lacy might have acid reflux
or sleep apnea, or could even be doing it on purpose, out of anger.
“But she wasn’t angry,” Mike said. “She was sitting there happy, and
all of a sudden it hit her like a train.” One doctor put her on
phenobarbital, increasing the dose each time there was another seizure.
“It made her lethargic, like she was drunk,” Wendy said. That November,
Lacy had a terrible attack. “Her arm went up in the air,” Mike said,
throwing his left arm straight up, stiff. “Her lips went blue. She was
having them in a row.” By the time the ambulance came, the seizures had
subsided, but the attendants advised the Nissleys to get in their car
and take Lacy to Johns Hopkins anyway. The drive took two hours, and,
just as Mike and Wendy walked over to the triage nurse, Lacy began
another cluster of seizures. The nurse said, “We’ve got to get you a
room.”
At Johns Hopkins, David Lieberman ordered another
MRI. The scan seemed normal, and after a week of further testing Lacy
was sent home without a clear diagnosis. Lieberman, in consultation
with Eileen Vining and the Johns Hopkins epilepsy group, tried to
control the convulsions with medication. Lacy was weaned off the
phenobarbital and put on Dilantin and Ativan. They tried Diastat,
Topamax, and, later, Tegretol and Keppra. But the seizures continued.
When they hit, Mike and Wendy would lay Lacy on the floor and talk to
her. “Every couple of minutes, she would moan and groan like she’s in
pain,” Wendy told me. Mike nodded. “They said that seizures don’t
hurt,” he said. “I don’t know that I believe that.” As we spoke, Lacy
sat up a few times and turned to look at me. When I waved and said,
“Hi,” she said, “Ba.” Twice, she smiled; once she looked completely
terrified. Each time, she turned her shaky head toward her mother and
nestled down.
In April, 2004, Lacy returned to Johns
Hopkins for four days of continuous EEG monitoring, and again in
November for another MRI. The scan showed that she had an enlarged
right hemisphere. The bulge on the right made the midline between the
two lobes bow slightly toward the smaller side, and there was a strange
fogginess on the right side of the scan, whereas the left was clear.
Lieberman told the Nissleys about hemimegalencephaly. “We had a
diagnosis, finally, and we were happy with that,” Mike said. “It’s not
sleep apnea. It’s not acid reflux. It is something. We didn’t know if
she was going to die.” Diana Pillas, who coördinates counselling at the
Johns Hopkins epilepsy center, told Mike and Wendy about various
possible treatments. They could try a ketogenic diet—which is ninety
per cent fat and has been shown to stop convulsions in some
children—but Lacy would be restricted to a special liquid formula for
two years. She also told them about hemispherectomies. “I couldn’t
believe it. I didn’t know people could do that and be alive,” Mike
said. “The first question I had was ‘Well, what do they put in there?’ ”
After
the initial shock, Mike and Wendy decided that the surgery was the best
option. Lacy’s seizures had hindered her development to the point where
she couldn’t walk unless she was holding someone’s hand, and she hadn’t
learned how to chew. The seizures were also becoming more frequent, and
they knew that children with her condition tended to live much shorter
lives than their peers. The Nissleys were concerned about the costs,
though. Mike worked as a butcher at a local supermarket, and Wendy, a
doctor’s receptionist, had had to quit working in order to look after
Lacy; no day-care program would take her. Johns Hopkins put them in
touch with another family whose child had had the procedure, and who
told the Nissleys about the financial aid that was available. They
finally made a date.
I bumped into Mike and Wendy as I
came out of the O.R. with Vining. They were smiling but tense. “We were
really stressed,” Mike said later. “We were all wondering if it was the
right thing to do. No one said it, but we all knew what we were
thinking.”
Neurosurgery
began with trepanation, the prehistoric practice of scraping, sawing,
or boring a hole in the skull. Trepanned skulls, some as old as ten
thousand years, have been unearthed in Europe, Africa, Asia, and the
Pacific Islands. Knowledge about the brain’s anatomy has accumulated
from the time of Galen, in the second century, but neurosurgery was not
recognized as a distinct specialty until the early twentieth century.
Since then, neurosurgeons have saved many brains by selectively
destroying some of their tissue. They’ve injected brains with pure
alcohol, electrocuted them, and inserted wire loops and metal probes
and wiggled them about. They have frozen brain tissue, and they have
burned it. But no brain surgery is as dramatic as a hemispherectomy. “A
hemispherectomy is the opposite of everything you are taught in
neurosurgery,” Jallo told me. “You are told throughout your residency
training to preserve the brain, get what you have to get, do your work,
and leave, but with this you have to take out everything along the way.”
The
first recorded hemispherectomy was performed, in 1888, on a dog by
Friedrich Goltz, a prominent German physiologist. (Apparently, the
post-op animal exhibited the same personality and a minimal reduction
in intelligence.) In humans, the operation was pioneered by Walter
Dandy, a Johns Hopkins neurosurgeon, who, in 1923, performed his first
hemispherectomy on a patient with an aggressive brain tumor in the
right hemisphere. The patient lived for three and a half years, until
the cancer invaded the remaining hemisphere. Dandy performed another
four hemispherectomies, all for patients who had brain cancer in their
right hemispheres. In 1938, the Canadian neurosurgeon Kenneth McKenzie
performed a hemispherectomy on a child suffering from seizures that
could not be controlled with drugs. The operation was a success, and
hemispherectomies came to be more popular as a treatment for chronic
seizures than for cancer. But eventually the operation fell out of
favor. Doctors found that, around ten years after the surgery, some
patients became paralyzed or comatose, and sometimes died. This was
caused by a buildup of cerebrospinal fluid, a saline-like substance
that cushions the brain. The fluid is produced at the rate of about one
teaspoon per minute, and in normal brains it is reabsorbed—the total
supply of fluid renews itself every five hours. But in some cases the
trauma of the operation seemed to prevent this, and the resultant
pressure could distort the skull and push the remaining brain to one
side, a condition known as hydrocephalus.
The
hemispherectomy’s resurgence in popularity is largely the work of John
Freeman, a pediatric neurologist who has been at Johns Hopkins nearly
his entire career. Tall and charmingly gruff, Freeman is seventy-three
and semi-retired, though when I visited him before Lacy’s operation he
was still putting in forty hours a week in his cluttered office.
“Semi-retired for a workaholic,” he said. Freeman saw a few
hemispherectomies while on a training fellowship at Columbia, and
started ordering them for patients at Johns Hopkins in the early
nineteen-seventies. By then, hydrocephalus could be detected early,
with scans, and the excess fluid drained to other parts of the body,
where it was reabsorbed naturally. In the early nineteen-eighties, he
proposed the treatment, for the first time, for a patient with a
diseased left hemisphere—a thirteen-year-old girl who suffered such
uncontrollable seizures that she had spent several months in the
intensive-care unit. The prospect of removing a left hemisphere—rather
than a right one, as had been the case in previous operations—was
daunting. It is generally thought that language is situated in the left
half of the brain, and no one could say for certain what the effect of
removing that hemisphere would be. “We worried about that,” Freeman
said. “What would your life be like if you couldn’t talk, and you
couldn’t understand, and you were just there and aware?” While a number
of the hospital’s faculty approved the operation, an outraged senior
physician vetoed it. But the only alternative, Freeman observed, was
for the girl to remain in intensive care for the rest of her life, so
the following week, when the doctor in question was away, Freeman went
ahead and arranged the operation. “Can you imagine doing a
hemispherectomy without the assurance that the patient would talk?” he
asked. The operation was a success, and the girl recovered the ability
to speak. “Now,” he said, “I can tell the families: they all talk.”
If
Freeman revived the practice of hemispherectomies, their leading
practitioner has been Ben Carson, who joined Johns Hopkins in 1984 and,
at thirty-two, became the youngest head of pediatric neurosurgery in
the nation. A star in his field, Carson has written a book about his
transformation from angry teen-ager in Detroit to renowned
neurosurgeon. He did his first hemispherectomy a year after coming to
Johns Hopkins. The Washington Post wrote a
story about it, and soon the hospital was getting calls from doctors
all over the country saying that they had patients with intractable
seizures whom they wanted to send. Carson has now performed more than a
hundred hemispherectomies. One of his oldest patients had the surgery
in his thirties.
Carson’s office, like Freeman’s, is
strewn with files, books, and X-rays, and there is an abundance of
photographs, cards, honorary Ph.D.s, and gifts—a hanging plate painted
with poetry, a framed sampler embroidered with “Dr. Carson,” and, on
the windowsill, a statuette of a handsome black man in a white doctor’s
coat who looks exactly like Carson. In person, Carson is mild, tired,
and friendly. He told me that when he started doing hemispherectomies
he would reach in and slice out an entire hemisphere at once. But he
modified this practice after one patient went into a coma for a month.
Carson believes that the patient’s brain stem was moved about
excessively during the procedure. “It’s too much like what I’m doing
right now,” he said, grappling with a plastic model of the brain in an
effort to pop out a recalcitrant lobe, which eventually shot off into
the mess on the floor. I asked him Mike’s question, about all that
space left by the missing lobes. In the past, he said, doctors worried
about this and tried to anchor the remaining brain by stitching it to
the dura. They would put all kinds of things in the cranial cavity—one
surgeon used sterile Ping-Pong balls. But, as Carson did more
hemispherectomies, he realized that the brain’s own drip of
cerebrospinal fluid could adequately fill the cavity. Sometimes the
remaining brain moves during the weeks following the surgery, but
usually by less than an inch. “It doesn’t seem to be a problem,” he
said. Much of Carson’s method is intuitive. “You develop a feel for the
brain,” he said. “Normal brain feels like a very soft boiled egg. A bad
brain feels like a mushy apple.”
Freeman and Carson
haven’t merely pioneered the surgery; with their colleagues at Johns
Hopkins, they have undertaken research into the effects of
hemispherectomy on behavior, language, and psychology. “We as a group
here in pediatric epilepsy have changed the way children are handled,”
Freeman told me. “It’s not just the surgery; it’s the network and the
contact. It’s the best thing I’ve ever done, and I don’t even do them.”
Freeman and Carson are in the process of handing the practice over to
Eileen Vining and George Jallo. Each doctor somehow resembles his
successor. Freeman and Vining are obsessive clinical researchers and
vigorous wranglers of surgeons, doctors, and patients. Jallo, like
Carson, has a quiet intensity and the gentlest of handshakes. When I
asked the surgeons how it’s possible for people with half a brain to
live, let alone have a life, each of them spoke about plasticity,
flexibility, redundancy, and potential, and then they smiled and said
the same thing: “We don’t really know.”
“After
I got this car, I noticed so many CR-Vs,” Christina Santhouse said. “I
guess you notice them after you get one.” Christina was driving a nice,
steady five miles per hour above the speed limit on the way to the
Bristol Pike Lanes bowling alley, in Croydon, Pennsylvania. She
recently turned eighteen and got her license on the second try. Her
family outfitted her Honda CR-V with an extra-long rearview mirror,
additional side mirrors, a knob on the wheel for steering, and a rod
that brings control of the indicator over to the right side. As an
eight-year-old, Christina played soccer, swam, and did karate. Then she
contracted Rasmussen’s encephalitis, a little-understood condition that
causes chronic inflammation of the brain. One day at the Jersey Shore,
her foot started twitching, and within a few months, as the right side
of her brain deteriorated, she was having hundreds of seizures a day.
Christina became Johns Hopkins hemispherectomy case No. 30—her surgery
took fourteen hours, one of the longest operations Carson has
performed. The alterations to Christina’s car are necessary, because
she has impaired motor function on her left side. (Each hemisphere of
the brain primarily controls the opposite side of the body.) She also
lost sight on the left side of each eye, and now wears prism glasses
that bring the left field of vision over to the center of the eye. When
I met her, she had taken her S.A.T.s and just finished high school,
coming in seventy-sixth in a class of two hundred and twenty-five. Last
fall, Christina was a freshman at College Misericordia, in Dallas,
Pennsylvania, where she’s studying speech pathology.
When
we pulled into the parking lot of the bowling alley, she parked in the
same spot that she always used. She wore jeans, a college hoodie, pink
nail polish, and Skechers sneakers. A small brace beneath her jeans
kept her left leg straight, though she still had a slight limp, and her
left arm was partly paralyzed. “I can do this”—she shrugged her left
shoulder, moving it and the arm around. “I can’t do this”—she twinkled
the fingers of her right hand at me. Christina booked us a lane and we
sat down to put on our bowling shoes. She was on her high-school
varsity bowling team for four years, and in her final year she was
captain. She pulled a leather glove onto her right hand with her teeth
and then insisted that I go first.
I knocked down eight
pins, and Christina knocked down six. “I get really nervous and my arm
gets stiff,” she said. “Also, I haven’t practiced for a month. And the
finger holes in my ball are cold.” In the next round, I knocked down
seven pins, and Christina bowled a strike. As the game continued, my
score lagged, while Christina bowled spare after strike after spare.
Her ball weighed fourteen pounds. I’m at least seven inches taller, so
I started out with a sixteen-pound ball. After almost losing it on the
first backswing, I changed to a fourteen-pound ball. As my elbow began
to ache, I moved to a twelve, then a ten. Christina doesn’t have the
balance necessary to run and throw the ball, so she positions herself
at the bottom of the lane, crouching slightly and swinging it like an
underhand discus. I tried bowling like Christina. She coached me on
where to stand and what to aim for, but my first ball, deprived of the
momentum that comes from a running walk to the edge of the lane,
dropped straight into the gutter. The second time, I swung harder and
my ball rolled just a little farther before it hit the gutter. On the
third try, I knocked down one pin. Christina explained that you have to
work out which side you’re stronger on, and step to that side as you
swing. Her final score was a hundred and seventy.
Christina
is a spectacular example of how well children can do after a
hemispherectomy, but she is no outlier. Many children who have had
hemispherectomies at Johns Hopkins are in high school, and one, a
college student, is on the dean’s list. The families of these children
can barely believe the transformation, and not so long ago neurologists
and neurosurgeons found it hard to believe as well. I asked Jallo if he
remembered his first hemispherectomy. “Yes and no,” he said. “I don’t
remember the patient. It was more of a ‘Wow.’ I was a resident in
training and I assisted in one of the operations. I didn’t realize you
could take out that much brain tissue and have someone be so functional
and useful in society. What amazes me is that, if someone all of a
sudden strokes out half of the brain, more likely than not they are not
going to survive. Yet a lot of these people develop their seizures when
they’re very young, or in utero, and when you take out half of their
brain in one sitting it’s as if they weren’t touched.”
There
are wide variations in recovery, and any brain surgery carries grave
risks. Many factors affect how well a patient does—age at the time of a
condition’s onset, age at the time of the operation, the nature of the
condition itself, and the determination of parents and caregivers to
maintain an intense schedule of therapy before and afterward. Possibly
the greatest danger is posed by the brain’s veins and arteries, which
are so numerous and so wildly, individually arranged that they are
impossible to map and very hard to control. Excessive bleeding can send
patients into shock and then into comas from which they never return,
or it can wipe out most brain function. The other conflicting challenge
of the surgery is the necessity of making sure that enough tissue is
removed. Freeman once saw a small boy who made good progress for six
months following a hemispherectomy, after which he began to
deteriorate. His doctors discovered that they had left a small piece of
the excised hemisphere in the child’s head. It was, said Freeman, no
larger than the top joint of his thumb. But the electricity from that
piece of neural tissue was enough to compromise the remaining
hemisphere. The boy had another operation, a “redo,” as the doctors at
Johns Hopkins informally call it, in which the bad piece of brain was
removed. After that, he had no more seizures.
The brain’s
remarkable capacity for recovery has long fascinated scientists.
Bradley Schlaggar, a pediatric neurologist and a professor at
Washington University in St. Louis, told me about an experiment that he
conducted for his Ph.D. He transplanted the visual cortex from an
embryonic rat’s brain into the brain of a newborn rat, placing it in
the spot occupied by the somatosensory cortex, which is responsible for
such bodily sensations as pressure and temperature. Once the second rat
had grown up, Schlaggar took a look at its brain and discovered that
the transplanted chunk of visual cortex was functioning as a
somatosensory cortex. Such a basic architectural feature of the brain
was thought to be entirely hardwired, but Schlaggar showed that brain
tissue could reprogram itself to serve different purposes. In another
experiment, Leah Krubitzer, a professor of psychology at the University
of California, removed large pieces of the brains of newborn
marsupials. Once the marsupials became adult, she examined the brains
again and found that they had organized themselves in such a way that
the visual, auditory, and other somatosensory areas were all in the
same relative positions that they would occupy in a normal brain, but
they were smaller, commensurate with the total space available.
Schlaggar
said that, in the mid-eighties, researchers invoked plasticity to
explain the brain’s ability to compensate for sudden damage—as when a
stroke victim relearns to walk or talk. Now, Schlaggar said, the
concept is used to describe a far more basic operation of the brain,
including how it develops from childhood to adulthood, and even how an
adult brain changes when a new skill is learned: “The idea is that when
you talk about plasticity you are talking about every bit of learning
that we do.” For a long time, the assumptions that neuroscientists made
about what was going on in a child’s brain were based on how the adult
brain worked. But now it seems that the brain’s internal configuration
depends on the age of the brain. If an eighteen-year-old’s brain
sustained some kind of damage, Schlaggar explained, it would occur in
neural terrain that is organized differently from, say, that of a
two-year-old, even if the injury was in the same spot. Children with
damage to a particular area of the brain often suffer losses that are
different from those sustained by an adult who experiences the same
trauma.
When Schlaggar lectures on plasticity, he shows
slides of the construction of the St. Louis Arch. “As the structure
goes up, the relationship between the scaffolding and the leading edge
of the two sides of the arch change as they rise up to meet in the
middle,” he said. “The interaction between the scaffold and the
emerging mature structure is dynamic.” The ever changing scaffolding of
the developing brain, Schlaggar said, means that the functional
organization of the brains of children performing a task can look quite
different from that of adults engaged in the same task. If you show
seven-year-olds and thirty-year-olds a word and ask them to generate a
verb for that word, the two groups can do so with equal accuracy and in
the same amount of time, but they use slightly different regions of the
brain to arrive at their answers. Children, Schlaggar explained, use
extra regions of the brain during their development which, like
scaffolding that falls away, are no longer needed when the brain’s
architecture is mature.
Although the plasticity of
Christina’s brain in the years following surgery has enabled her to
live a remarkably normal life, other aspects of her experience have
been hard. When we were alone in the car, Christina told me about years
of social difficulties. “I didn’t tell many people at high school, but
it kind of got out,” she said. She didn’t make friends until junior
high. A few years after the operation, one of her mother’s relatives
phoned to say that there was a photograph of Christina in The National Enquirer.
Before going to Misericordia, Christina hoped for a fresh start, but
when she spent an introductory night there her roommate showed her a
photocopied article about her hemispherectomy. A psychology professor
had handed them out to her class.
After bowling,
Christina drove me to her home and I spoke to her mother, Lynne, and
her grandmother Mary Lou. We looked at photographs taken the day of the
hemispherectomy, and we watched a video of Christina at Johns Hopkins
before the surgery. She is sitting on a bed, and her foot begins to
twitch. Within seconds, the twitches multiply and cascade through her
body, and she falls back onto the bed completely helpless. In another
video, Carson strides from the O.R. after Christina’s hemispherectomy.
He looks utterly invigorated. Lynne, waiting outside, clasps his hands
and then crumples before him in tears.
The
day after Lacy’s surgery, Jallo came by early to visit her. She was
moving her right arm and leg weakly, and her color was good. The next
day, she started to run a fever, and then she had the worst seizures
she’s ever had—three in a row, each ten minutes long. This was not
unusual—most practitioners think it’s caused by the trauma of the
operation—but it wasn’t good. The doctors adjusted her medication, and
over the next few days she improved. When I phoned Mike, he said that,
four days after the surgery, she had been out of bed a few times to be
held in her mother’s lap. “She’s not doing much of anything on the left
side,” he said. The left side of Lacy’s body had always been weak,
because it was controlled by the damaged right hemisphere. With that
hemisphere gone, Lacy would need a year’s intensive therapy to remind
her brain of the existence of her left arm and leg. Three weeks after
the surgery, Lacy’s staples were removed and she was released from the
hospital.
On a wet October day more than two months after
the operation, I drove back to Palmyra. As I pulled up to the house, I
could see two small heads at the front window. Lily and Lacy were
standing side by side on the couch, peering out. The doctors had told
Mike and Wendy that it could take Lacy six to twelve months to get back
to where she had been before the operation, but since July she had made
extraordinary progress. “We thought she’d be a blob for a few months,”
Mike said. “But once she started eating and got her strength back she
was straight at it.”
We sat again in the living room.
Lacy was pale and skinny, and her white-blond hair was an inch long.
The scar underneath was barely visible. She was in constant motion,
crawling and walking about on her knees. Sometimes, when she crawled,
she dragged the knuckles of her left hand on the floor. She babbled and
squealed, and she said “Yeah” when I asked her how she was. She helped
herself to a bottle of milk and drank from it while she idled on her
knees. She surfed around the furniture and stood at an activity table
in front of me. When I held out my right hand to her, she wobbled for a
second and then grabbed it with her left hand. She swung her right hand
around, placed it on top, paused, and was off again. Then she sat with
her back to me. She kept looking over her right shoulder to see if I
was still there, then looking away. “She’s really silly all the time,”
Mike said.
A month after the operation, Lacy began seeing
a physical therapist, an occupational therapist, and a speech therapist
on a regular schedule. The speech therapist would talk with her face
right up against Lacy’s, and after a few sessions Lacy began moving her
mouth to mimic the therapist. She learned some sign language, and used
the sign for “more” a lot at the dinner table, where she could now eat
the same food as her family. She still had to take the anti-convulsants
Tegretol and Keppra twice a day, and the doctors thought that it would
be a year before she could stop. But there had been no more seizures,
and, the week before I visited the Nissleys, Lacy had taken three steps
by herself for the first time.
By early December, Lacy
was walking everywhere, and in January the family returned to Johns
Hopkins for a scheduled checkup. In Jallo’s report, he wrote that Lacy
had done beautifully. She had had no seizures, and she was babbling and
walking independently. She was “a happy child.” Lacy still showed a
clear preference for her right side, but he wrote that he hoped for an
improvement in tone and a loosening up on the left side.
For
two months after that, Lacy walked and played, but in March she had a
setback. Wendy put her down for an afternoon nap, and about fifteen
minutes later she had a seizure. Wendy said, “She just woke up,
instantly, with her eyes bulging out.” The seizure lasted a few
seconds, and afterward Lacy rolled over and went to sleep. She woke up
happy. Everything was fine for a few days, but then she had another
seizure, again about fifteen minutes after she had fallen asleep. “We
were shocked,” Wendy said. “Lacy went about seven months without any
seizures. It is disappointing.” The seizures increased steadily—after
about a month, there were a few every day, each lasting five or six
seconds. According to Wendy, Lacy didn’t appear particularly distressed
by them, though occasionally they made her more tired. “We are not sure
what the next step will be,” Wendy said, but she and Mike remained
optimistic. For Lacy’s third birthday, they had a big cookout in a
rented pavilion at a local park beside a lake.
When Wendy
drove Lacy back to Johns Hopkins, Vining was thrilled with her general
progress. Developmentally, she said, Lacy was fulfilling expectations,
and even the recurrence of the seizures wasn’t unduly alarming. “They
are very mild,” she said. There were no signs of them on the EEG and
they lasted, in total, less than a minute per day. Vining speculated
that Lacy might have outgrown the dosage of one of her anti-seizure
medications, and she increased it. After a few weeks, however, there
was no improvement; the seizures were lasting longer, and had begun to
show up on the EEG. Vining switched Lacy to a different
anti-convulsant, Trileptal. Another possible cause of the problem was
the tiny nub of occipital lobe that Jallo had left in Lacy’s brain
during the operation. I asked Vining whether Lacy would ever need a
“redo” to remove that final piece of the right hemisphere. She
explained that only time would tell. Vining was hopeful that the new
drug would solve the problem. If it didn’t, she said, they would have
to think seriously about going in again. 