Lauren Ashley said she can still remember the moment doctors discovered her "phantom brain tumor," even though she only 5 years old at the time.
She was at the hospital recovering from her second spinal tap that year after repeatedly telling her mother her "eyes hurt." Ashley, now 15, said she still gets headaches daily.
"They are really sharp and burning, literally it feels like someone has a knife through your head. I'm constantly in pain," the Gahanna, Ohio, teen said.
An ophthalmologist took one look and told her family she probably had a brain tumor.
But later imaging tests showed no tumors growing in her brain.
Then, the day of the spinal tap, doctors came back with a confirmation. Ashley had pseudotumor cerebri -- an unexplained buildup of fluid in the brain that pushes on the optic nerve, often causing headaches, vision loss or even blindness. Some people with the condition may report visual problems, nausea and tinnitus, a ringing in the ears.
Most people who develop pseudotumor cerebri are obese, although the link is not well understood. Doctors say a growing number of children are developing the condition, which usually appears in middle-aged women.
"Individuals would come in with many of the same symptoms of headache or vision loss that a person with a tumor would have. That's where the name [pseudotumor] comes from," said Dr. Steve Roach, chief of neurology and vice chair of pediatrics at Nationwide Children's Hospital in Columbus, Ohio.
Roach, a professor at Ohio State University, is part of a team of doctors who opened up the first center dedicated to treating children with pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH).
"Probably two thirds of people with pseudotumor cerebri are obese," said Roach.
He said that in some cases, simply losing weight will stop the symptoms but not in others. "There's another third of people who aren't obese," he said.
Ashley falls into the category of children who are not obese.
But regardless of whether the person with the condition is obese, Roach says doctors are unsure why IIH develops in the first place.
Cause Behind 'Phantom Tumor' Remains a Mystery
For an unknown reason, the cerebral spinal fluid that surrounds the brain and cushions the spinal canal begins to build up.
"It turns out the spinal fluid is made inside the head, we make 500 to 600 ccs [cubic centimeters] of this every day," said Dr. Bernd Remler of the Medical College of Wisconsin in Milwaukee, and fellow of the American Academy of Neurology.
Remler said very recently scientists found out that areas in the skull, called dural sinuses, serve as a drainage system for the cerebral spinal fluid both expelling the fluid and some blood into the jugular vein. But if the fluid builds up enough, then a key sinus can collapse and in turn cause more fluid buildup and a vicious cycle.
The condition is rare enough that Roach said doctors do not have a good number for how many people have it. He said he hopes the new center at Nationwide Children's Hospital will offer an alternative to hectic appointments with various specialists in different locations. Instead, families could go to one place to get care.
He said he also hopes collaboration at the center will lead to some answers about the condition.
"Some of these things I'm having to say 'I don't know' to, hopefully I will know," Roach said.
For now, doctors say the most important thing is to stop the damage to people's vision before it becomes permanent. In IIH, the extra cerebral fluid in the brain can push on the optic nerve, slowly diminishing a person's peripheral vision.
Doctors can tell something is wrong with a visit to the ophthalmologist and often can see the swollen optical nerve, but many people don't know to come in until it's too late.
"That can be such a gradual thing that the individual doesn't appreciate it until it's really bad," Roach said. "Often times you don't get that vision back -- sometimes they don't know it until they've run into a door."
Specialists might perform an operation called optic nerve fenestration, which leaves a small window in the sheath of the optic nerve to release pressure and can sometimes slow vision loss.
Some People Can Recover From Phantom Tumor
"It's often possible to control this with weight loss and medication. We mostly see this in females that are obese in the adult age group," Remler said. "Because childhood obesity is on the rise, we see it more and more in adolescents."
"I saw a 16-year-old girl who was not treated in time and lost her eyesight," said Remler.
Ashley has luckily stemmed any major vision loss from her IIH. But she has had 30 operations -- including holes drilled into her skull, a shunt into her spine and a shunt into her brain to relieve the pressure.
Her headaches and pain are so severe that she only goes to school for two hours a day before coming home to rest and work with a tutor for the rest of the day. She sometimes has to go to the emergency room for pain medication strong enough to stop it, but on normal days she tries yoga and some relaxation time with her pet chihuahua Zoe.
"It's hard for her for to keep up with friends and she can't be involved in too many sports or activities because she can't keep up with it," said Diane Ashley, Lauren's mother.
Diane Ashley has joined together with other families who travel to Columbus from across the United States to get care. She has since developed the activism group for her daughter's condition, IH Gray Matters.
Diane Ashley said she was ecstatic to hear about a single center where children with IIH could get all their care.
"I've not been able to work full time since she was diagnosed," said Diane Ashley, who works part time as a nurse. "There isn't a week that goes by that we don't have a doctor's appointment."
Her daughter thought it might be a way to buy more time in her life.
"I thought it would be a great idea," she said. "I go to maybe two or three doctors appointments every week and if you could get it in at all appointments it would be wonderful, so you don't live at the hospital."