'What Would I Be Like With Half A Brain?'
For Two Girls, Reunion Of Hemispherectomy Survivors Offers
A Close-up Look At What Might Have Been
By Mandy Stadtmiller
It was a happy, tragic reunion. One girl with half a brain healthy. The other with a whole brain half-diseased. And the best of friends.
Last September, Kaci Caves, then 13, and Amber Ramirez, then 12, met at Johns Hopkins Children's Center in Baltimore. Each girl suffered from a severe form of epilepsy and came to learn more about a rare surgery that could end the seizing, the need for high levels of disorienting medication and the progressive brain deterioration caused by their disease. The surgery is the most radical kind of operation imaginable: removal of half a brain, or a hemispherectomy.
They understood that the dangers of surgery were great: damage to speech and language, paralysis of the right hand, loss of peripheral vision, a limp in the right leg. But then, there were the risks if surgery were not done: progressive retardation, loss of speech, loss of motor skills.
Ten months later, the two were united again. Kaci had decided to have the surgery. Amber did not.
In the middle of a reunion last month of young hemispherectomy survivors, Amber watched Kaci.
"What would I be like," she wondered, "with half a brain?" And Kaci watched Amber, remembering what it was like when she still had seizures.
The girls have Rasmussen's encephalitis, a degenerative disease of the brain that causes daily, almost constant seizures. And daily, constant fear.
"Rasmussen's is an uncommon progressive viral-like disease which eats away one side of the brain like a Pac-Man," according to John M. Freeman, director of the pediatric epilepsy center at Hopkins. "It starts in one hemisphere and it eats its way through that side but never goes over to the other side. As it damages tissue, it causes these uncontrollable seizures and loss of function on the opposite side of the body."
Doctors estimate only 1,000 people in the world have the disease, which if left untreated can lead to mental retardation and paralysis. The cause is unknown, but doctors believe it might be an immune system reaction against one's own brain. While patients first try drugs to quiet the seizures, often the only option is surgery to remove the diseased half of the brain.
The 6-to-10-hour procedure involves opening a large portion of the skull to expose the brain. From there, the gray matter (or thinking part of the brain) is removed from one side to rid the brain of the cells from which seizures arise.
Such a surgery is possible because the brain has two distinct hemispheres. As a child grows, each side becomes more responsible for specific tasks -- such as language or artistic abilities. When one side is damaged, the other often compensates and fulfills those duties. And the younger this happens, the more the brain is able to adjust and compensate.
Few hospitals perform the operation, and Hopkins, one of the leaders in developing the procedure, has done only 68 hemispherectomies in the past 30 years. The patients have included children with Rasmussen's encephalitis and several other serious brain disorders. Four patients died from the surgery during the 1980s. But doctors said risks of the surgery have declined through their increased experience.
Last month, 22 of the children who underwent hemispherectomies at Johns Hopkins returned for a reunion organized by Lynn and Al Miller, whose daughter Jody, 7, had the surgery in 1993. All weekend long, the children played with each other while their parents attended workshops on dealing with the more painful social impacts of the disease and life after surgery. Some also visited Hopkins for checkups that sometimes included counseling and neurologic exams.
Many of these children were included in an study published in the August issue of the journal Pediatrics.
The study by Hopkins researchers found that 89 percent of the children with Rasmussen's syndrome had their seizures substantially decreased with the surgery. The procedure also does not appear to decrease a child's intellect, the study found, and can in fact help since intelligence can improve if the seizures and anticonvulsive medications are eliminated. However, all the children do face some difficulty using the leg and have a paralyzed hand on the side opposite of the removed hemisphere. Peripheral vision to one side is also hampered.
Kaci and Amber are near the age limit for those benefiting from the surgery, doctors said. The girls' age and the location of the disease in the dominant left hemisphere meant that surgery might severely harm their ability to use language.
"The older you are," pediatric neurologist Freeman said of the two girls, "the more difficult it is. Kaci and Amber came at the same time last September. Kaci had her surgery last November and is recovering nicely. Amber couldn't bring herself around to having surgery yet. You do surgery when people are ready. Initially, Amber wasn't ready and now her mother isn't ready. We have told her that we cannot guarantee that she will ever speak again. All of our children have spoken again with varying amounts of fluency. But the older you are, the more likely you are to have problems. It's a very tough decision."
Most important in Kaci's post-surgery results was the elimination of the seizures -- sometimes hundreds of them daily that began as a twitch on the right side of the face and by the fourth or fifth one, threw her right side into temporary paralysis. Now, she swims every day, an hour in the lake by their house in Tulsa, Okla. Her dad is showing her how to drive a golf cart in preparation for learning to drive a car.
In the fall, she will begin the ninth grade at Jenks High School, with some adaptations for her -- multiple choice instead of essay tests, read-aloud material instead of written. Her mother, Regina Caves, has great reservations about her returning to school. Kaci's speech is slowed and she has lost a significant amount of language ability. Her right arm is partially paralyzed and on her right leg is a brace.
"I'm afraid that kids will remember the old Kaci, and she hasn't seen some of them for a year and a half," her mother explained. "But she looks at me with those big brown eyes and rolls them and says, Trust me,' so I have to trust her."
The same way she trusted surgery. "We could either let the disease take over the brain or go ahead and do the operation and start rehab immediately and start her new life," Caves said, sitting next to her daughter. "That's exactly what this surgery's started."
She looked at her daughter and said determinedly, "A whole new life as a whole new Kaci. The new and improved model."
"Oh yes," Kaci said, flashing a braces-filled grin at her mother. Meanwhile, Amber continues to grow worse in Lincoln, Neb. Her right hand shows signs of deterioration, with her thumb uncontrollably pressing next to her index finger. She has trouble saying things easily, the way she used to. Her seizures continue, though not with the same frequency or intensity of Kaci's before her surgery. Still, they occur daily, throwing her entire right side into paralysis. Sometimes she falls down and gets scraped and bruised when there is no warning of dizziness to prepare her to sit down.
"As I saw Amber and her seizures, I do remember that I don't want to do that again," Regina Caves said at home in Tulsa. "It's hard right now, but I also realize that from this point, Kaci's going to go up."
Sneaking away from their mothers during the reunion at the Holiday Inn, Amber and Kaci sat close together on a couch and held hands, Amber's right with Kaci's still-functioning left. Around their necks, each wore a yellow smiley-face "Best Friends" necklace that Amber bought to give to Kaci the day before coming to Baltimore.
Kaci showed Amber how she could tie her shoes and dress herself with just her left hand. And how to talk to God. Gently lifting the paralyzed right hand with her left, Kaci clasped them tight in prayer.
Kaci searched for speech uneasily. Like a stroke victim, she now has difficulty finding the correct word for a situation.
"I can't say it right this instant. I can't think of it," Kaci told Amber slowly when she couldn't express what she wanted. "I'm sorry."
Amber didn't care. "It's not your fault, Kaci," she said, looking into her eyes. "It's not your fault. I understand perfectly fine."
The labor Amber sees in Kaci's language doesn't scare her. She knows that she may be like Kaci if her mother lets her have the surgery. Or she may be worse.
"Because I know in my heart," Amber whispers, "that she can think of the right word sooner or later."
Since the girls met at Hopkins, Kaci's mother, has been writing to Amber's mother, Sheri Ramirez, about what life after surgery has been like since November. She described the rock-bottom point they hit immediately after surgery, when Kaci stopped smiling for two months. She told her how Kaci could only express her feelings in simple statements, like, "I'm okay." How she couldn't orally tell time. How she changed from a straight-A student to a child who couldn't read.
"Doctor Freeman said, I'm going to have to make her a lot worse to make her a lot better,' " said Kaci's mother, who was a special education teacher before Kaci got sick in May 1993. "I didn't understand that quote until after the surgery, and I realized how limited the language was. I didn't understand it until I went through it. I don't think any parent could."
But then Kaci started the climb up. And her mother described that in letters too. Kaci was regaining her reading skills, moving up to second-grade books. She started smiling again. It was better than before the surgery. No longer did she fall asleep at 7 p.m., so drugged from her 40 pills of seizure medication a day. She went down to three pills. Kaci got out of the wheelchair that she despised, and was able to walk, although with a limp in her right leg.
Kaci's experience helps Amber not be afraid. "She's just doing great," Amber repeats over and over again at the reunion.
The neurosurgeon who performed Kaci's surgery said that Amber faces a tough reality.
"She's the kind of case where you'd really have to make it very, very clear that this is a major risk," said Benjamin S. Carson, director of pediatric neurosurgery at Johns Hopkins Medical Center. "Obviously you'd wish that you'd done it three or four years ago. But you can wish all you want, you can't go back in time. And the counsel I would give in a situation like that is, Look at your life. Look at the amount of seizures. The fact that they're intractable and can't be handled. Do you want to live this way or do you want to risk potentially not being able to speak?' "
Carson paused. "I'm not sure that's a medical decision." Amber's mother does not dispute the facts. "I think since Hopkins hasn't changed their recommendation for us," Ramirez said, "it's made it a little bit more permanent that we really need to do :a hemispherectomy: now."
Still, she pauses. She can't decide. She is afraid of Amber never speaking again. "I just haven't figured out whether the time is right for us or not."
Discussions with Amber inevitably lead to tension. "We know we're dealing with a time-bomb thing of age," Ramirez said, sitting close to her daughter. "But we haven't set a date. She's had a good summer without seizures."
Amber glared down at her white tennis shoes. "I don't care, Mom," she said, gesturing out with her right hand. "I just want the seizures to get done with. To go away."
As the reunion came to a close, all of the kids went to Old Mill Swim Club in Baltimore for a pool party and picnic. Amber swam close to her mother at the edge of the pool in case she had a seizure while Kaci swam around vigorously.
By the end of the night, Kaci couldn't wait to get back home to Tulsa. To go swimming and drive the golf cart and even to do her six hours of physical and occupational therapy a week. She's excited about starting high school.
For Amber, the night ended later. She didn't want to leave so quickly. Exhausted from another seizure by the pool, Freeman held her in his arms. Hugging him, she whispered, "I'm tired of seizures. I want to be left alone." Then she started to cry, afraid her mother won't let her have the surgery. Afraid her mother won't let her take the chance.
© Copyright 1998 The Washington Post Company