When the brain betrays
Mysterious seizures prompt a desperate search for answers
By CZERNE REID
czreid@thestate.com
Lorelei McKay | Living with
Epilepsy- Photo Gallery | Lorelei's story
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1 | When the brain betrays
Lorelei McKay still can’t remember everything that happened July 3, 2004. Her husband, Rob, has to fill in the details:
He woke up at 7:30 that Saturday morning to hear his wife making a strange dolphin-like sound — like the whistle of escaping air. Her eyes were wide and unfocused and she was shaking violently. She bit her tongue, her arms and legs flailed and something ran from her mouth.
His heart racing, Rob McKay called 911 to their home in Lexington. At 26, his wife was in great health. Was she now having a stroke? A heart attack? Was she going to die?
Lorelei remembers only seeing dark uniforms hovering over her bed, carrying her, shoeless, onto a gurney and into a cold, squeaky ambulance, the double doors opening to swallow her.
She woke up later that day at Lexington Medical Center to see her mother and mother-in-law in her room. Doctors said a “seizure condition” had made her lose consciousness.
It happened again three weeks later.
Six breath-stopping, eye-rolling, body-jerking, arm-flailing, seizures, one after the other.
So began a confusing time in Lorelei’s life that left her unable to keep a job, drive a car or even hold a conversation. She and Rob had to put their future on hold. From doctor to doctor they went, searching for answers, finally clutching at a thread of hope in the hands of a neurologist and a brain surgeon at the Medical University of South Carolina in Charleston.
“Do I want a normal life?” Lorelei said. “Yes! It’s torture sitting at home ... being completely dependent on somebody else.”
Lorelei, who had juggled two jobs, worked as a church secretary and co-owned an accounting business, suddenly lost control of her own body. Sometimes she wet herself. Sometimes her jaws locked. Sometimes the air was forced out of her lungs. Once she fell, and her teenage niece Stephanie ran away crying.
After four or five trips to the emergency room, Lorelei went to a Columbia-area neurologist. He never seemed to understand what was happening to her. A second doctor thought her problem was psychiatric. Still, he prescribed anti-epilepsy drugs — she took eight or nine in all.
The side effects were horrible. Lorelei’s thick mane of red hair grew coarse and fell out in clumps. Her 5-foot, 5-inch frame gained more than 25 pounds. Her mouth and tongue went numb. Her speech slurred. She saw double. Between medication-induced stupor and seizures, Lorelei’s family members got only rare glimpses of the young woman’s former personality.
“That’s the sad part because she was always going in two directions at once,” said her father, Rex Bumgarner. “Lorelei was such a lively, vivacious girl all through school.”
She loved computer science and biology but skipped school so much that her report cards were riddled with F’s. She barely graduated from high school but made up her missing half-credit Lorelei-style, in the nick of time, with an etiquette course at USC.
“Somehow it always just comes through at the last minute,” she said.
At 19, with just a high school diploma, she landed a corporate accounting job, and later ran an accounting firm and sold real estate.
But her condition made it increasingly difficult to talk with clients and forced her to stop working.
“That’s the hardest part,” she said. “I felt so independent, and now I feel so dependent.”
Rob started putting in extra hours at his job with a vending-machine company. He worked ticket booths at ball games and concerts. He did odd jobs — sanding and painting.
“It’s a whole lot different when you’re used to having two incomes, then you have one income, and not by choice,” Rob said.
Lorelei said: “You can tell by the way he works and the way he does things that he thinks of me first. I’m very secure in our relationship.”
Lorelei was at home alone for long periods with just the cats, Sunny and Silky. She had wanted to be at home full time, but not like this. She wanted to be semi-retired by her mid-30s, running a “Martha Stewart-type” business selling antiques, “shabby chic” furniture, pottery and folk art.
But she had become so useless, she told herself, so dependent. She couldn’t even do the simplest thing anymore — she burned herself and her food while trying to cook dinner, and forgot to turn off the stove afterward.
The stress got to Rob, too.
“I just want to be able to communicate and get back to normal,” he said.
Other family members also had a hard time coming to grips with what was happening to Lorelei. Her mother, Annelise Bumgarner, called her condition the “the stuff” in her head.
“We thought it was just a fluke. We didn’t even take it seriously,” said Rex Bumgarner. “It just gradually sank in.”
OUT OF THE BLUE
Lorelei has epilepsy.
Dr. Jonathan Halford, a neurologist at MUSC, diagnosed her condition in late 2004.
Also called seizure disorder, Lorelei’s malady was more of a symptom than a disease. The seizures came from part of her brain that was scarred. In that spot, the neurons — nerve cells that transmit electrical signals — started behaving abnormally. Instead of doing their separate duties, they kept pulsing the same signal over and over, like an orchestra with all the instruments bleating out the same note.
Lorelei’s seizures, called partial seizures, started in one area of her brain, then fanned out, like a brush fire spreading to consume an entire forest. The other main seizure type — called generalized — starts all over the brain at once.
As many as 5 percent of Americans have a seizure at some point. Lorelei is in that 1 percent where seizures recur.
Epilepsy most often strikes during childhood or old age. It is relatively uncommon for people to have first-time seizures in the middle years, as Lorelei had at 26.
Why had this happened out of the blue? Did she have defective genes? As children, Lorelei’s eldest brother, Jason, and a distant Austrian cousin had something that might have been seizures.
As far as Lorelei knew, no illness affected her central nervous system; she hadn’t suffered brain damage before or at birth — and no head trauma, infection or stroke, or any of the usual causes for seizures.
When none of those factors exist, doctors are unsure what causes the onset of epilepsy during early adulthood.
Lorelei researched her condition to come up with answers. She watched brain surgery on the Discovery Channel, trolled the Internet and pored over her medical records. Soon her speech was peppered with words like “complex-partial” and “post-ictal.”
“I’ve got a whole new interest now in the medical field,” she said.
She wondered whether stress had caused her seizures. Just before the first one, she and Rob had lost their home and savings because of a failed business venture.
WHAT WENT WRONG?
To learn more about Lorelei’s seizures, Dr. Halford arranged for her to be monitored with an electroencephalogram, or EEG, while she was having seizures, in a procedure called scalp monitoring. The results would help doctors pinpoint what part of her brain was malfunctioning.
Lorelei spent Thanksgiving 2004 in the hospital with electrodes attached to her scalp. She had 19 seizures in five days.
Doctors thought the seizures might be coming from her temporal lobes — the brain regions to the left and right. But they also could be coming from the area in the front of her brain, the frontal lobe. The EEG monitoring had not been conclusive.
Halford sent Lorelei home on one medication.
By April 2005, after months of tweaking dosages and adding a second drug, Lorelei’s big seizures lessened from five a month to about three. The dozen-a-day smaller seizures — during which she would stare into space or have difficulty speaking — slowed to six or fewer a day.
“There’s no way to predict what patients are going to respond to what medicine,” Halford said. “You’re just going to have to try them.”
The latest mix helped Lorelei venture out of her mental fog.
“I started figuring out that if I’m going to recover I need to start doing something with my brain,” she said.
She tried working on her computer but couldn’t remember how. She started balancing the checkbook. She read dense works of nonfiction — science, history and politics. She was fascinated with Colin Powell, former U.S. secretary of state, after reading his autobiography.
She was doing better.
But still having seizures.
It turns out Lorelei falls among that 30 percent of epilepsy patients whose seizures are not controlled by medication.
“After they have tried three medicines and the medicines have not controlled their seizures, the chances of a fourth, fifth, sixth or seventh medicine is a waste of time,” Halford said. “Looking at the studies, really the best chance for (patients) to be treated or cured of their disease is to be considered for surgery.”
FACING THE TEST
If doctors discover where in the brain the seizures arise, they can remove the offending part. A procedure called a lobectomy has been used for treatment of epilepsy since 1953.
Not to be confused with lobotomy, lobectomy removes diseased tissue and involves removing part or all of a given lobe of the brain. Lobotomy, on the other hand, is a surgery done to affect behavior, and involves disconnecting the frontal lobe from the rest of the brain, but leaving the lobe intact.
A temporal lobectomy gives patients a 70 percent to 80 percent chance of becoming seizure-free. In the frontal lobe, the operation gives a seizure-free rate of less than 60 percent.
As many as 25 epilepsy surgeries are performed at MUSC each year. It should be more, Halford said, but — as with Lorelei — many patients aren’t referred by their doctors. Others shudder at the thought of having their heads cut open.
Lorelei was scared, too.
But she was more scared of not having the surgery.
She felt a little guilty for wanting it so badly. She should be grateful for the improvements she’d had so far, she told herself. After all, lots of people out there have cancer and other more life-threatening conditions.
At MUSC, Lorelei had several months of pre-surgical testing including an alphabet soup of scans — EEG, MRI, PET, SPEC.
In October 2005, a final test could help identify the location of the seizures. Her skull would have be cut open, and electrodes placed directly on her brain.
Putting pea pod-shaped grids of electrodes embedded in silicone on Lorelei’s brain would help pinpoint where her seizures started. For the procedure to work, she needed to have seizures while the electrodes were in place.
For the past year, Lorelei had wished away her seizures.
For the first time, she found herself praying for them to come.
AND SO IT BEGINS
Lorelei met her neurosurgeon, Istvan Takacs, 10 days before the surgery to put electrodes on her brain.
“I’m nervous, Mama,” she whimpered to Annelise Bumgarner, who had chauffeured her on the two-hour drive for her 6 a.m. appointment.
A bearded Takacs and his white coat swept into the room. Lorelei had researched his profile on the MUSC Web site and felt reassured by his “kind-looking” eyes. Now those eyes rested on her.
Did she know why she was there?
What kinds of seizures had she been having?
Did she know what the procedure involved?
“So it’s a mystery till we put the grids in, huh?” he said. “It’s basically a discovery mission. Is it the right brain or the left? Can we take it out without causing you more trouble than you already have?
“Do you know how we’re going to do this? Let me draw you a picture.”
Takacs outlined a brain, showing where cuts would be made on her head and four holes drilled into her skull — one high on the forehead and one on the temple, on each side of her head. The long, narrow electrodes would be fed through the holes onto the temporal and frontal lobes of her brain. The wires from the grids would poke an electrical ponytail through her head bandages.
“You’ll look somewhat bionic,” he said.
Takacs explained to Lorelei the risks of the procedure. She could get an infection. Her brain’s surface could be damaged, leading to worse seizures — but the chance of getting through uneventfully was 97 percent.
Once the electrodes were in place, there was no telling how long it would take for the EEG, which monitors the brain’s electric al activity, to yield the needed information.
“It may take three days, it may take 30. Who knows?” Takacs said.
On Oct. 14, 2005, Lorelei was back at the hospital to have electrodes put on her brain. If the monitoring went well, she could look forward to her lobectomy.
She was ready.
She was happy.
But she was sad — for the first time since childhood, she was missing the rides at the State Fair.
Soon she was being prepared for surgery. In the flurry of activity, she got an IV and her anesthetic. The last thing she remembered was mouthing “I love you” to Rob, then surrendering to the darkness of sleep.
In the operating room, Lorelei’s head was held still with a vise. A swath of the locks that had earned her the nickname “Red” was shaved away.
Her skin was cut and peeled from the temporalis muscle underneath.
The muscle was peeled from the bone of the skull. Then the four holes were drilled.
The thin dura, a membrane that covers the brain, was opened. The grids were put in place, then the dura, bone, muscle and skin were put back together, and her head swathed in a white bandage.
Once back in her hospital room, her head wires were plugged into an EEG machine.
Now it was just a matter of time before the seizures came.
This was just the start. She wanted the monitoring to go by so she could have the next surgery.
The one that would change her life.
Reach Reid at (803) 771-8378.
Hoping for the best, waiting for the worst
By CZERNE M. REID
czreid@thestate.com
Lorelei McKay awoke with a nervous feeling. The pain came when she coughed.
She could feel where the surgeon had drilled four holes in her head just hours earlier. Neurosurgeon Istvan Takacs had put pea pod-shaped electrodes on the surface of her naked brain.
Gray electrical cords snaked from the white bandages on her head, down the side of the bed, across the floor and up to the video-EEG machine that would capture the activities of her brain and body when she had a seizure. Rows and rows of waves rippled across the machine’s computer screen, each representing electrical activity in a different part of Lorelei’s brain.
Someone had to be in the room with her at all times to call a nurse and hit a button to mark the EEG chart when the seizures came.
Lorelei was ready for them, and she wanted them to come strong and plentiful. Funny, because she had been trying to get rid of those same seizures for the past year and a half. But having seizures now was her best chance to eliminate them forever.
Epilepsy had struck her suddenly in 2004 over the July 4 weekend, changing her from an outgoing entrepreneur to a dependent shut-in.
Her situation seemed hopeless, until she was referred to Dr. Jonathan Halford, a neurologist at the Medical University of South Carolina.
There was a surgery — called lobectomy — that could eliminate her seizures, he told her. These holes in her head were a last step to pinpoint the part of her brain that was damaged. Then surgeons could remove it.
But first, she had to have seizures. She settled in and waited for them to start.
Three days came and went.
No seizures.
She had stopped taking her anti-epilepsy medications, but it seemed that wasn’t enough. Her brain needed a little coaxing.
Sleep deprivation had led to seizures in the past, so her doctor asked her to try to stay awake for extended periods. She hardly slept for several days until Dr. Halford warned her that lack of sleep could make her psychotic.
Anyway, it wasn’t working. They had to try something else.
Technician Maryhelen Britt brought in a pedal and a strobe light attached to a rolling stand. Lorelei pumped the pedal for a few minutes, then Britt flashed the strobe lights in her face for three minutes. Lorelei’s eyes burned as she tried to stare at the lights. Britt told her she could close her eyes; it’s the brain, not the eyes, that the lights were meant to stimulate.
Next came hyperventilation exercises. For three minutes, Lorelei breathed rapidly with her mouth open.
“How are you feeling, Lorelei?” Britt asked at the end of the routine.
“Tingly,” came the answer. “My skin is crawling on my neck.”
Still, no seizures.
Only pain.
Lorelei had a splitting headache, and her jaw ached where the surgeon had cut through the temporalis muscle.
After falling asleep one evening, Lorelei awoke with a start, her heart pounding and her mind confused.
A seizure!
Her husband, Rob, pressed “the button” and called a nurse.
The nurse found no seizure activity on the EEG. False alarm.
WAITING ...
While Lorelei waited for real seizures, a doctor had to re-stitch her scalp. Cerebrospinal fluid had seeped from her head, staining her bandage and pillow yellow. When she sat up so the doctor could better see where to stitch, some of the wetness from her scalp spilled onto her shirt.
“I started laughing, but I wanted to cry,” she said.
But crying would just make her head hurt more, and all she really wanted to do was have a seizure.
The pain in her jaw eased, so Lorelei could pick at some stewed beef, rice, vegetables, banana pudding and apple juice from her hospital lunch tray. She thought about how much her time in Charleston was seeming less and less like a hospital stay.
“For me it’s like a vacation — people serving me hand and foot,” she joked. “Other than this head problem, it’s an improvement over being at home all day. It’s great.”
She passed the time by chatting up attendants and nurses —some even came to visit because they remembered her from her hospital visit the previous year.
As time dragged on, however, her life of leisure began to frustrate her. On the fifth day of her stay, Lorelei swapped her hospital gown for a navy blue-and-white calico shirt, tan sweat pants, and black-and-gray Joe Boxer socks. Putting on more clothes wasn’t something Lorelei always had done willingly — as a child, she would shed her clothes and streak through the house and yard to the amusement of her family and neighbors.
Under the hospital lighting, she dabbed on face powder and pink lipstick from a cosmetics bag she kept nearby. She brushed the stringy red strands of unwashed hair that peeked out from the bottom of her bandage, and curled them with a hot iron.
“It just feels like such a pathetic attempt at looking human,” she said. “This might seem silly, but you feel better when you do something.”
She fussed around, tidying the room so much that her surgeon jokingly suggested she might look into choosing new wallpaper. She tried to do crossword puzzles but couldn’t get past the first clue. She didn’t get far with the books she’d brought, either — a religion-history book, old issues of Country Living magazine from her mother and a little New Testament and Psalms. Finally, she decided to while the hours away by counting the blips running across the computer screen.
At one point, the EEG trace disappeared from the screen. The computer had run out of memory. A nurse got it going again, after much tinkering and the aid of a technician on the other end of the phone line.
“You’re the one who’s supposed to be having the seizure, not the computer,” said Rob, who was on that shift of Lorelei’s seizure watch.
Rob did word search puzzles; read “Spurs and Feathers,” a magazine about sports at his alma mater, USC; and watched ESPN’s SportsCenter on a loop.
“I’m actually just brain dead,” he said. The waiting was driving him crazy, too.
“Have a seizure,” Rob said to Lorelei.
“It’s bad to be wishing seizures on her,” he said, “but that’s why we’re here.”
The sooner she had a seizure, the sooner they could stop racking up hospital bills, and Rob could stop losing money by staying away from his commission-paid job.
Luckily, the couple had good insurance coverage.
Lorelei’s mother, Rob’s parents and other family members had taken turns driving from Columbia to relieve Rob.
“It’s a big fiasco,” Lorelei said. “Everybody’s having to go through all that just to sit with me.”
Why wasn’t she having seizures? She tried to diagnose the problem. Was she eating less than she usually did at home? Was she too relaxed? Her thoughts, questions and doubts overwhelmed her. She crumpled in tears.
Even though she hadn’t had seizures yet, the doctors were learning something from her EEG. The abnormalities on the chart — sharp spikes that lasted for just fractions of a second throughout the day, and the same types that occurred repeatedly during a seizure — were showing up in the frontal lobe of her brain.
If she had frontal-lobe epilepsy, she still could have a lobectomy — but not at MUSC, which offers only temporal lobectomies. She would have to go to Duke Medical Center in North Carolina. Or she could have a pacemaker-type implant — a vagus nerve stimulator — that could sense and disrupt an oncoming seizure.
Lorelei’s 28th birthday rolled around 11 days after she entered the hospital. Her parents gave her a sewing machine.
What she really wanted was seizures.
Instead, a week after her birthday — almost three weeks after she had entered the hospital — doctors told her they would remove the electrodes from her brain.
Her heart sank.
She would not be cured.
What would people think about all the time she had spent in the hospital?
There would be questions.
And comments.
And jokes.
And whispers.
People had asked why she was so intent on having surgery. Why not wait the seizures out? Some thought she was wasting money by traveling to Charleston. Some thought she was faking the whole epilepsy thing. It all got to her, and sometimes she wondered whether she really had seizures.
MYSTERY
Why had they stopped as unceremoniously as they’d started?
She didn’t know.
The doctors didn’t know.
“It made me second-guess whether she had epilepsy in the first case,” neurologist Halford said. “I really do believe she had epilepsy... . I just don’t know what to say about it.”
Other doctors weren’t sure what to say, either.
“The functioning of the brain remains one of the greatest mysteries in science,” said Adam Mamelak, a neurosurgeon at Cedars-Sinai Medical Center in Los Angeles who specializes in epilepsy surgery. “The fact that the brain works at all. The fact that we aren’t all having a hundred seizures a day, and on top of that the capacity of being creative; the fact that we have conscious and unconscious behavior.”
Considering that the brain is a massive integrated computer made up hundreds of billions of cells each connected to thousands of others, “it’s not terribly surprising that we don’t understand these basic things,” said David Eagleman, a theoretical neuroscientist and assistant professor at the University of Texas Medical Center at Houston.“A single cell in the brain is about as complicated as a city is.”
Until the basic mysteries of the normal brain are unraveled, it’s difficult to even begin to try to understand disorders such as epilepsy, said Eagleman, author of “Ten Unsolved Questions in Neuroscience,” to be published later this year by MIT Press.
Current research into understanding epilepsy includes trying to figure out what genes are involved in genetic epilepsies, and by what mechanisms. Researchers are trying to develop ways to better pinpoint where in the brain seizures arise; others are trying to develop drugs that disrupt pathways involved in epilepsy. Still others test those drugs clinically.
LORELEI’S LIFE NOW
Lorelei left the hospital Nov. 2, 2005, with as many questions as she’d taken with her. And disappointment had forced hope out of her heart.
The bright side was that she was taking a very small dose of medicine, and still her seizures had not come back.
Maybe she had been cured without the surgery.
A miracle?
Surely this couldn’t last.
She was stuck at home with her uncertainty. She didn’t know when the next big seizure would hit her.
A month after leaving the hospital, she stopped going outdoors with the cats and started sleeping longer than usual.
She felt like a huge burden to Rob.
She wondered how many painkillers she would have to take to fall asleep and not wake up.
Her stress was Rob’s stress.
“You’re trying to console your wife every day ... not just because something happened,” he said. “How do I deal with this situation, plus depression?”
Lorelei went back to see Dr. Halford three days before Christmas and got a prescription for the antidepressant Cymbalta and the sleep aid Ambien. The doctor also increased the prescribed dosage of her anti-seizure medication by half a tablet. She declined his offer of a visit with a psychiatrist.
The antidepressant soon helped her to feel better.
But within the next few months, she started waking up at night with her heart racing, teeth clenched, body jerking, difficulty breathing, dizziness, hyperventilation and headache.
The episodes were similar to the ones she had before the first big one hit her in mid-2004. In the daytime, she would stare into space, feel tingling or shaking in her face, or start stuttering uncontrollably. But at least she wasn’t losing consciousness during the day.
Dr. Halford increased her medicine dosage again, and that reduced the symptoms.
But they didn’t go away.
While waiting to see what would become of her, Lorelei carried on with her life.
“I’ve been a lot more active, more productive, working on a lot of little projects,” she said.
She sewed a bathroom curtain for her father’s birthday, and a pink-and-green quilt and Care Bear pillows for her nieces. She started going to auctions and estate sales as she had before the seizures.
But Lorelei, a former bookkeeper, was having trouble cataloguing her inventory using a basic computer spreadsheet.
Rob would tell her, for example, how many items came in a package, then ask her to subtract the number of missing items to find out how many they had in hand. She could hear him, but she just couldn’t do the math. Even if he repeated it.
“I just want to cry, because it doesn’t make any more sense to you than the first time, even if they repeat it or say it louder,” she said. “I’m hearing numbers, but they’re not making sense.”
Lorelei froze in the supermarket while trying to write checks. She got stuck in conversation with store clerks. At her yard sale, Lorelei asked a woman to figure out her own change, and the woman stung her with, “You must be a product of the public school system.”
Incidents like those left her embarrassed and frustrated.
And tired.
At seven months after leaving the hospital, the little seizures weren’t going away.
And they were eerily similar to the ones she’d had just before the first big one struck.
Reach Reid at (803) 771-8378.
http://www.thestate.com/mld/thestate/14691679.htm
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