Hemis' lead Whole Lives After Surgery

By Nancy Shulins, The Associated Press

BALTIMORE - Miranda Francisco stands at the center of a crowded conference room. All eyes and all cameras are on the fidgety 6-year-old.

For a few self-conscious seconds, she stares mutely back. Then, with a giggle, she whispers, “Humpty-Dumty-sat-on-a-wall. Humpty-Dumpty-had-a-great-fall.

Had she quoted Shakespeare rather than a child’s nursery rhyme, the performance by the little girl from Denver couldn’t have been more dazzling to her audience of doctors, nurses, parents and reporters.

Half of Maranda’s brain, her dominant left hemisphere, the half that is verbal and analytical, is preserved in glass jars in a pathologist’s freezer, removed two years ago in a feat of surgery known as hemispherectomy.

It is rare surgery for rare neurological disease: progressive seizure disorders that affect only one hemisphere of the brain and don’t respond to any other treatment. Such disorders can be the result of a stroke, a congenital abnormality, a tumor, perhaps even a virus.

Maranda, along with seven other “hemis” and their families, gathered recently at Johns Hopkins Hospital for four days of mutual support and testing. It is the first of several planned reunions of these premier patients, who may one day lead to better understanding of these ailments.

But beyond the mystery of the disease looms a greater mystery: that of the cure. How can a surgeon remove half the human brain yet excise none of the human spirit?

The 10-hour operation that took half of Maranda’s brain has given her two years without seizures. Now a Kindergartner who swims and takes dancing lessons, who prefers purple to green, who strains at her mother’s hand like a puppy on too short leash, Maranda thinks the clouds on flight from Denver looked like “big puffy pillows.” And says so.

Julie Klingelhofer, 20, of Baltimore, smiles knowingly as she watches Maranda’s performance. Four years ago, Julie lost the left frontal, parietal, occipital and temporal lobes of her brain. She did not lose her love of children.

Today, Julie Klingelhofer has a certificate of competence from a community college, a steady boyfriend named Wayne, and an IQ of 77, a full 10 points higher than it was prior to her surgery. She also has a job in a day-care center, a job she would have been unable to do before her hemispherectomy.

Six-year-old Jennie McCameron of Kokomo, Ind., underwent the removal of the right side of her brain, the side that governs art and music awareness, the realm of insight and imagination.

Less than a year later, over breakfast, Jennie put down her goblet of orange juice to sing: “I’m a little teapot, abort and stout…”

A few minutes later, her mind took an insightful turn. “Everyday in every way,” she said solemnly, “I’m get better and better.”

Which is not just a reflection of her mother’s belief in positive thinking; it’s also a medical assessment the doctors can endorse - for Jennie, and for the other hemis who came back in the hospital’s Children’s Center.

For every question the doctors can answer, more crop up that they can’t - about the neurological disorders that crippled these children; about the process by which

the remaining portions of their brains have taken over

functions lost to surgery or disease; about the consequences

that may one day result from in operation their

neurologist, Dr. John Freeman, describes as “horrendous.”

Beyond that, the questions broaden; How can half of the brain be lost without any discernible loss of its byproducts – generosity, humor, insight, and optimism? How can a human being with half a brain still worry, plan, invent, imagine and reason?

How is it that 8-year-old Beth Usher of Storrs, Conn., can lose her left hemisphere, yet retain her large repertoire of knock-knock jokes?

Shortly after Beth regained consciousness, her father be as quizzing her about People and places. Brian Usher didn’t get very far, “Dad,’ Beth interrupted. with a trace of impatience, “I remember everything.’

For that, as for much of what has happened within the skulls of these eight pioneers, there are as yet no explanations, only theories. For now, the answer offered by Diana Waguespack, mother of a 3-year-old hemi named Lisa, must suffice.

“Perhaps,” she suggests, “miracles really do happen.

The closest science can come to a synonym for

"miracle" in this case is "plasticity." It describes the

phenomenon by which certain neurological functions

can migrate from one hemisphere to the other.

Dr. Ben Carson, chief of pediatric neurosurgery at the

Children’s Center, has performed most of the recent

hemispherectomies done at Johns Hopkins. "Plasticity

is something pediatric neurologists have known about

for a long time," Carson says, "But no one knows the

mechanism by which it occurs."

Scientists do know, however, that young brains are

more "Plastic" than old ones, which is why

hemispherectomies are best done early in life,

By 1985, when Maranda Francisco’s parents brought

her to Baltimore, Johns Hopkins surgeons had done five

hemispherectomies on seizure disorder patients. Such

disorders, like the surgery they prompt, are exceedingly

rare.

A healthy infant, Maranda was 18 months old when she

suffered her first seizure. Her parents, Lou and Terry

Francisco, tried every treatment the baffled doctors

suggested. Nothing helped.

By the time Maranda was 31/2, "I was counting 130

seizures a day," recalls Terry.

The diagnosis, when it finally came, sounded hopeless.

Neurologists in Chicago and Los Angeles agreed that

Miranda was suffering from Rasmussen's encephalitis.

Little more is known about it today than in 1955, the year

Dr. Theodore Rasmussen, a Montreal neurologist, first

identified it.

The left side of Maranda's brain began to shrink and

harden, triggering a storm of seizures that crippled the

right side f her body. Within months, she could no

longer walk, sit, or bold up her head.

Because Maranda's illness had attacked her dominant

left hemisphere, containing her speech center among

other functions, the prospect of surgery was frightening.

Her Denver neurologist consulted numerous specialists

before he and her parents decided to send her to Johns

Hopkins.

Freeman was strongly in favor of surgery for Maran

da. "I said, 'It's a progressive disease, she will continue

to have seizures, and chances are, she won't speak

anyway,' " he recalls.

One August morning in 1985, Carson, the softspoken

neurosurgeon, got up early. For two hours, he read the

Book of Proverbs. Then he headed for the operating

room.

Rock music played softly In the background as Carson

divided the connections between brain and stem,

thalamus and basal ganglia.

He pinpointed the major bleeding points overlaying

the corpus collosum, the cable of 250 million connecting

fibers that joins the hemispheres, and disconnected the

inflow and outflow to Maranda's left brain. He identified

the major blood vessels.

Then be tackled the delicate task of removing the

damaged tissue of Maranda's left frontal, parietal, occipital,

and temporal lobes. The thalamus, which relays

messages from the body's sensory organs to the cortex,

and the basal ganglia, the region it the base of the brain

that controls thinking, voluntary movement and

memory learning, were left intact.

Carson and Freeman had explained to Terry and Lou

Francisco that removal of the left side of Maranda's

brain would wipe out the right half of her visual field in

both eyes and cause paralysis on her right side. The doc

tors also prepared the Franciscos for the devastating

possibility that their daughter might never talk again.

But nobody was prepared for what happened as

Maranda was wheeled out of the operating room. With

her eyes swollen shut and her lips puffed out beyond

recognition, Maranda said: "I love you, Mommy and

Daddy."

Her speech had already migrated to the right side of

her brain.

Man with Head Injury Makes Dazzling Sculptures - Bill Scanlon

ROCKY MOUNTAIN NEWS, January 1999

LONGMONT, Colo. When a propeller sliced out chunks of Ivan Schlutz's skull, it left behind a gift the slowspeaking man shares with the art world.

Surgery and partial paralysis gave way to physical therapy, arts and crafts classes and ultimately, an extraordinary talent.

Ivan Schlutz, who lost 40 percent of his skull and much of his ability to cipher and spell in 1991, emerged with an astonishing ability to sculpt images of the West.

His bronze statues of American Indians and cowboys are featured in the latest issue of Art of the West magazine.

"The accident helped," said Schlutz, a former pilot who was hurt at the Longmont airport while fixing an aircraft engine.

Tests at the Mayo Clinic found he could read and do math at a fifthgrade level, he said. If given more time he can understand more complex reading and math.

Then, they gave him the art tests. "I scored perfect on both of them," said Schlutz.

"Something happened. Right where I was hit on the head, that's where the part of the brain is that controls some art ability."

No one at the Charles Dana Foundation for brain research had heard of a case in which a brain

injury left someone with greater artistic talent than before.

But press officer Barbara Rich noted that epileptics who’ve had parts of their brains removed have learned to use what's left in surprisingly effective ways.

"The brain is a marvelous instrument that can rejuvenate itself, "said

Rich.

Schlutz still suffers terrible headaches, and has to take daily therapy classes.

His art work sells in the $3,000 to $4,000 range at three Front Range galleries.

"I’m surprised he has gotten so good," said Schlutz’s wife, Dina. "Up until recently, it had just been a very expensive hobby.

"It's really been a blessing. At first, his sculpting was a quality of life thing he could feel he was useful. But he's really turned it around to produce something that is marketable."

The complex process of turning a clay image into a bronze statue can cost $2,000. Schlutz got early help from Fritz White, one of the top living western sculptors.

"I think of the idea in my head, then I spend about a third of the time working it out on a chalkboard, getting all the proportions and compositions," Schultz said.

In his 3,000squarefoot studio in the barn of his father's farm, Schlutz manipulated his latest creation: a Mandan Indian crouched in a buffalo dance, his head and shield covered with buffalo hides.

Schlutz feels a spiritual connection with the Mandans and their perseverance.

"The buffalo dance never failed," he said. "Sometimes it lasted months, but it never failed. Because they never quit until the buffalo came."