Lives That Are All but Whole on Half a Brain : Rare Surgery Dramatically Aids Victims of Progressive Seizure Disorders
Maranda Francisco stands at the center of a crowded conference room. All eyes and all cameras are on the fidgety 6-year-old, from the tips of her light-brown curls to the toes of her new party shoes.
For a few self-conscious seconds, she stares back. Then, with a throat-clearing giggle, on a single intake of breath: “Humpty-Dumpty-sat-on-a-wall,” she whispers. “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 heroic 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 that don’t respond to any other treatment. Such disorders can be the result of a stroke, a congenital abnormality, a low-grade tumor, perhaps even a virus.
Maranda, along with seven other “hemis” and their families, is back 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 an exuberant puppy on too short a leash, Maranda thinks the clouds on her flight from Denver looked like “big, puffy pillows.” And says so.
Julie Klingelhofer, 20, of Baltimore, poised and ladylike in her summery print dress, smiles knowingly as she watches Maranda’s performance.
Love of Children
Four years ago, Julie lost the left frontal, parietal, occipital and temporal lobes of her brain. She did not lose her love of children or her desire to work with them.
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 before her radical surgery.
She also has a job in a day-care center, a job she would have been unable to hold before her 1983 hemispherectomy. “I walk more normally now,” she says, glancing shyly down at her white heels. “I’m also more confident.”
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 puts down her goblet of orange juice to sing: “I’m a little teapot, short and stout.”
A few minutes later, her mind, minus its right hemisphere, takes an insightful turn. “Every day, in every way,” she says solemnly, “I’m getting 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 are back at the hospital’s Children’s Center.
The agenda for their four-day visit includes a full slate of medical and mental tests, as well as a picnic, a pizza party and a news briefing.
The news briefing is, by necessity, brief.
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, healthy portions of their brains have taken over functions lost to surgery or disease; about the consequences that may one day result from an 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?
Memory Survives Coma
Beth’s memories survived not just the loss of brain tissue, but also the 32 days that she spent in a coma, the result of some brain stem swelling that occurred in response to the trauma of surgery.
Shortly after Beth regained consciousness, her father began quizzing her about people and places from her past. 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 definitive explanations, only theories. For now, anyway, 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. Several others have been done at medical centers in Canada, Germany and South Africa as well as elsewhere in the United States.
“Plasticity is something pediatric neurologists have known about for a long time,” Carson says. “But no one knows the mechanism by which it occurs.”
Carson thinks a drop in the baseline of certain neurotransmitters, the chemical communicators within the brain, may trigger a corresponding change in cell function.
While no one knows for certain how plasticity occurs, scientists do know 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. In a lifetime of medical practice, many neurologists never encounter a single case such as Maranda’s, which explains why it took so long for doctors to put a name to her frightening symptoms. The name they were searching for was Rasmussen’s encephalitis, an inflammation of one side of the brain, a disease so rare that it can’t be found in medical textbooks.
For Maranda, Baltimore was the last stop on a medical odyssey that had already taken her from Denver to Chicago to Los Angeles.
A healthy infant, she was just 18 months old when she suffered her first seizure. As the seizures became more frequent, baffled doctors proposed remedies ranging from narcotics to black coffee to aloe vera, and her parents, Lou and Terry Francisco, tried every one. Nothing helped.
Terry Francisco, a high school dropout, orphan and teen-age runaway who had parlayed grit and athletic prowess into seven gold medals in state and regional archery competition during her 20s, was not one to give up easily.
“The doctors would put Maranda on a new drug, and I would go research it,” she says. When admission to one medical library hinged on having an MD, Terry put on a white coat and passed herself off as an out-of-town intern.
By the time Maranda was 3 1/2, “I was counting 130 seizures a day,” recalls Terry. “On top of that, she would twitch and tremble, with tremors that seemed like earthquakes.”
Drug After Drug
Doctors tried drug after drug--drugs that made Maranda hyperactive, drugs that made her lethargic, drugs that made her drool, says her mother, “like a foaming dog.”
“She started wetting the bed. She had seizures in her sleep. They put her on one drug, and she didn’t know who I was. At one point, she lost so much weight that you couldn’t tell there was a child under the sheets.”
The diagnosis, when it finally came, sounded hopeless. Neurologists in Chicago and Los Angeles agreed that Maranda 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.
Dr. Freeman thinks it could be the work of a virus, “although it runs counter to all that we know in virology. Nobody’s cultured it, grown it or isolated it. We’ve not found the virus,” he says, “only footprints that look like a virus.”
If the virus has proven uncommonly elusive, however, its footprints are certainly real enough. The left side of Maranda’s brain began to shrink and harden, triggering a relentless storm of seizures that crippled the right side of her body, robbing her, bit by bit, of all her developmental progress.
Choking at Meals
Within months, she could no longer walk, sit, hold up her head. With each seizure, her right arm and leg would jerk out of control. She couldn’t get through a meal without choking.
Because Maranda’s illness had attacked her dominant left hemisphere, containing her speech center among other functions, the prospect of surgery was especially frightening. UCLA Medical Center declined to operate.
Maranda’s Denver neurologist consulted numerous specialists before he and her parents decided to send her to Johns Hopkins. There, doctors began a lengthy debate about whether 4-year-old Maranda would undergo a left hemispherectomy.
For Freeman, Rasmussen’s was an old, familiar enemy. While on the faculty of Stanford University School of Medicine in 1968, he had been part of a team of doctors who’d decided to perform a hemispherectomy on a young seizure-disorder patient. While doctors had been unable to remove the entire diseased hemisphere, the patient had improved as a result of even limited surgery.
‘Doing No Harm’
Now, nearly two decades later, Freeman was strongly in favor of surgery for Maranda. “I said, ‘It’s a progressive disease, she will continue to have seizures, and chances are she won’t speak anyway,’ ” he recalls. “The philosophy with all these patients has been that if you select candidates properly, the worst thing that can happen with surgery will be no worse than the worst that can happen without surgery.” It’s the most basic tenet of medicine: First, do no harm.
Still, “we batted it around for a long time, and we talked extensively with the parents,” recalls Carson, the soft-spoken neurosurgeon.
Finally, they reached a consensus.
One hot August morning in 1985, Carson got up early. For two hours, he read the Book of Proverbs. Then he got in his car and headed for the operating room.
Rock music played softly in the background as Carson delicately 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 join the hemispheres, and disconnected the inflow and outflow to Maranda’s left brain. He identified the major blood vessels.
“First, you must know where they are,” Carson says. “They are distorted by disease sometimes, but you can still figure out relatively quickly which is which. Then, you must disconnect them in the proper sequence. One feeds the right hemisphere, the other feeds the left. If you disconnect the wrong ones, the ballgame’s over.”
In Extra Innings
The ballgame went into extra innings as Carson tackled the delicate task of sectioning and removing the hard, 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 at the base of the brain that controls thinking, voluntary movement, and memory learning, were left intact. Carson covered her raw, exposed brain stem with moist sheets of gauze soaked in thrombin, a clotting factor, to inhibit post-operative bleeding.
He then filled the cavity with saline solution to cushion the remaining hemisphere until Maranda’s body could produce the cerebrospinal fluid that would eventually fill up the space.
In the days before surgery, 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. It also would cause paralysis on her right side, although her right arm and leg had already become crippled by her disease.
The doctors went to great lengths to prepare the Franciscos for the more devastating possibility that their daughter might never talk again.
Terry Francisco, who had already lost one baby from a previous marriage to sudden infant death syndrome and another to miscarriage, did her best to prepare for the possibility of losing her only surviving child to surgery.
As it turned out, nobody was prepared for what actually happened as Maranda was wheeled out of the operating room after her 10-hour ordeal.
With her eyes swollen shut and her lips puffed out beyond recognition, Maranda said: “I love you, Mommy and Daddy.”
Those six words told her doctors something no CAT scanner could: Maranda’s speech had already migrated to the right side of her brain.
Three days later, she was walking by herself. Within a month, Terry says, “she was getting on elevators and disappearing. I had to go look for her.”
Home a Year Later
After two months in Baltimore, Maranda was flown back to Children’s Hospital in Denver to continue her recovery. Finally, three days before Halloween, she went home to the bedroom she hadn’t slept in for a year.
“It took me a week to convince her that this, not the hospital, was her home,” Terry says. “She was still on antibiotics, and she was still vomiting every day. But her spirits were fine. We never lost her again.”
Maranda still walks with a limp, although therapy has helped to minimize it. In the two years since she left Johns Hopkins she has grown 9 inches, learned how to jump rope, and become something of a local celebrity.
She also has appeared on national television, on a telethon to raise money for children’s hospitals. Among those who tuned in was Anne McCameron, whose daughter was about to undergo similar surgery in Baltimore.
Jennie McCameron, whose wide dark eyes and solemn expression echo her mother’s, had been a healthy infant. Her normal development came to a halt at 2, when she suffered her first seizure.
Brain scans showed irregularities in her right hemisphere. But none of the many specialists who had examined her had been able to pinpoint the cause of the seizures that seemed to zero in on the left side of her body.
Jennie spent three years in and out of hospitals in Indianapolis, Cincinnati and Cleveland.
Fed Through a Tube
By the time she turned 4, she could no longer crawl, hold her head up or eat without choking. She had to be fed through a tube. Daily steroid injections had bloated her body. And day after day, month after month, her left foot never stopped twitching--a sign of the electrical storms raging within her brain.
“Each time I kissed her good night I would wonder--is she going to be there when I wake up?” her mother recalls. “We had her in special ed, we took her to the zoo, we took her everywhere. She could still talk, she still laughed. But the sparkle had gone from her eyes.”
By April of 1986, Anne McCameron was counting 100 seizures a day, and Jennie was back at the Cleveland Clinic, where the mother of another young patient happened to show Anne a newspaper account of Maranda Francisco’s remarkable surgery.
“I called Terry, and she told me Maranda was doing fantastic. I called Dr. Freeman, and he said bring her to Baltimore.”
They arrived to discover that Freeman had already penciled Jennie in for surgery. “Just the thought of cutting open her head and taking out half her brain was terrifying,” Anne says.
“But it had been such a weird coincidence, us finding out about Maranda. It had to be fate. I told myself, this is just meant to be.”
‘Just Meant to Be’
Any lingering doubts disappeared when she turned on the TV to watch Maranda’s telethon appearance. “There was Maranda, with her little curly hair, clapping. Jennie said, ‘Don’t cry, Mommy. It’s all right.’ ”
Midway through Jennie’s surgery, her mother suddenly asked to be taken to the operating room. “I remember going into the waiting area at the back of the operating-room suites and asking to see the part they had removed. The nurse brought us three little jars. I looked at them and thought, ‘It’s all in those jars. All her problems are in those three little jars. Now, we can throw them away and start over.’ ”
The McCamerons were waiting as Jennie was wheeled out of surgery. Wordlessly, they lifted the sheet to stare down at her left foot.
What they saw made both parents weep.
For the first time in three years, the twitching had stopped.