July 12, 2002 -- Fifteen-year-old Christina Santhouse is a smart and athletic teen, an *NSYNC fan who frequently chats on her cell phone and boasts a 94.7 GPA. What makes Christina unique is that she does it all on half a brain.
She told her story in the magazine Rosie and shared it with Good Morning America.
The nightmare began seven years ago, with strange symptoms that came out of nowhere. Christina, 8 at the time, was on what should have been a wonderful occasion: a family beach vacation on the New Jersey shore.
During the trip, Christina's foot began shaking uncontrollably, so her family rushed her to the hospital. Puzzled doctors made a hasty referral to St. Christopher's Hospital for Children in Philadelphia. After three days of torturous tests, they figured out what the problem was — and it wasn't good news.
Doctors diagnosed Christina with Rasmussen's encephalitis, a rare autoimmune disease, usually found in children, in which the body attacks the brain, killing off cells. There are fewer than 1,000 cases in the United States.
Cure Worse than Disease
Treating the seizures sounded worse than the disease. Doctors said she would have to get a hemispherectomy, a procedure in which the diseased half of the brain is surgically removed. In short, Christina would be left with half a brain.
Christina's divorced mom couldn't believe what she was hearing. To her, it sounded like the lobotomies of the 1930s. She worried that the surgical procedure would rob her daughter of her personality, if not her memory. Plus, she knew for sure that the removal of the right side of her brain would leave Christina with physical limits in her left arm and left leg, and the loss of left peripheral vision in both eyes.
"I was devastated," Lynne Santhouse-Catarro said. "I'd never heard of anything so barbaric."
Doctors said that Christina could go through some trial and error with different treatments — chemotherapy or steroids — but that those would just stop the virus for a while.
Dr. John Freeman, a neurologist at Johns Hopkins Children's Center in Baltimore, supervised Christina's case. At the time, he told the Santhouses that the hemispherectomy would be the only thing that would work, though he knew how startling the procedure sounded.
"For reasons that are very unclear, the syndrome only affects one side of the brain — never both," he said. "But how would you feel if I said I had to take out half your brain?"
150 Seizures a Day
When he first saw Christina, she was having seizures, but the family did not want her to have the operation, so Freeman told the family to come back when they were ready.
After she started the third grade that fall, Christina's seizures were getting worse, and she was sometimes having up to 150 of them a day, enduring spasms that shook her body from head to toe, and sometimes forced her to throw up. Her family knew that the almost certain future was having Christina in a wheelchair, and mentally retarded.
Finally, Christina gave her consent to the radical operation.
"I don't want to live like this … I'm ready now," she wrote to her doctor.
Doctors always make sure that patients know the risks.
"You never push this on somebody, because there's no going back," Freeman said.
Now, seven years later, the operation is the preferred way to treat Rasmussen's syndrome, although researchers are still looking for a new way to treat the autoimmune disease.
Prayers and Questions
In the beginning, Christina said she wanted to think positively about the operation, and she and her family prayed. But she did cry, and think "oh my gosh, I'm never going to be able to do certain things," Christina said.
The decision was not so easy on her mom.
"Well, you want your child to live a happy, healthy life — that's the ultimate goal of any mother," she said. "And here, you're thrown this curveball."
After the operation, Christina's left hand would become only a "helper hand," she'd have a limp on her left leg, and no peripheral vision on that side. So prior to the operation, her mother — who is a teacher — tried to prepare her daughter for the loss of some of the functioning on her left side.
Santhouse-Catarro wrapped the left part of her daughter's body so that she could not use it much, and had her practice putting on socks, and cutting sandwiches with one hand. They would have races to see who could get dressed fastest with one hand. Still, the abstract was nothing like the reality.
The surgery lasted 14 hours, an excruciating wait for her family, huddled outside. Surgeons opened Christina's scalp and skull and removed the right side of her brain. Christina woke up to a horrible headache and a swollen face, but nine weeks later, she was back in school. She was limping a bit, and scared of fitting in, just like every kid.
Before and After
Before the operation, in the third grade, she stayed in class, and would answer questions, even as she was having seizures every three minutes. She sat in a beanbag chair for comfort. Now she is going into her sophomore year of high school, is on the varsity bowling league, and took four academic honors at the end of last year.
It is possible to live a "normal" life after this operation, Freeman said.
"You just have to compensate for some of the physical things you lose," he said. For Christina, sometimes her friends don't even know what happened unless she tells them.
For more information on Rasmussen's encephalitis, go to theThe Epilepsy Foundation's Web site.