Jake Wiederhorn bounds onto the porch of his family’s Potomac home wearing a Captain America T-shirt and soccer shorts. He’s 9 years old, bright and thoughtful, hair parted on the left and cut short on the sides, and he shows no hint of the nightmare he’s been fighting to leave behind for three years.
“The nausea and the headache’s better,” Jake tells his parents. Around his neck he wears a gold medal that his brother, Max, 12, and his sister, Kayla, 7, made for him out of paper. It’s a reward for what he’s been through this week in early August. He spent the past two days playing Xbox at a doctor’s office while an IV dripped antibodies into his arm. The IV can make him feel sick at first, he says, but it’s meant to help him overcome a terrifying disorder that upended his young life. On a late summer day in 2012, Jake transformed from a fun-loving 6-year-old into a screaming, cursing child so paralyzed with fear that he refused to leave his mother’s side. It came without warning. One week, he was being treated for a double ear infection; the next, he was tearing apart his bedroom in a rage.
“It’s hard on the family,” his mother, Debbie, says. “It’s hard on the siblings. It definitely impacts everybody. But I feel like it’s made us stronger as a family.”
Jake looks at her, wide-eyed, as if it’s the first time he’s heard of anything good coming out of this experience. “Really?”
She smiles. “Absolutely,” she says. The Wiederhorns say they can pinpoint the moment the old Jake vanished. He had recently started first grade at Potomac Elementary and was heading out the door to catch the bus to school. Usually, the morning ritual was like a party—he and Max ran down the street to meet neighborhood friends at the bus stop. Kayla, too young for school, came along just to be with them. But that morning, Jake froze. When his friends boarded, he wouldn’t budge. The bus driver, a friendly man called “Wonderful Juan” by all the kids, waited as long as he could. But as Jake stood on the sidewalk with his mom and sister, the driver had to leave without him.
“This was a child who was happy, outgoing, funny, independent, responsible, caring, sensitive and so lovable,” Debbie says. “He just simply disappeared.”
Over the next few days, Jake’s behavior became even stranger. He threw tantrums, which were out of character for him, and spit swear words at his brother and sister, something he’d never done before. He wouldn’t sleep in his bed. He stopped eating. He fought so fiercely against going to school that his parents had to carry him into the building. His mother spent all day in the classroom beside him because he refused to go to school without her. Soon he wouldn’t go at all. For Jake’s family, the incident at the bus stop was the start of a journey into the heart of a medical mystery shrouded in controversy.
It’s nearly impossible for parents to keep their kids from getting strep throat. Scientists estimate that streptococcal pharyngitis is responsible for about 37 percent of the sore throat cases that send school-age children to the doctor. Most cases clear up quickly with a 10-day dose of antibiotics. Sometimes, though, strep goes undiagnosed. Children might carry the strep bacteria but show few or no symptoms—they may not even have a sore throat. Jake wasn’t tested for strep, but his parents believe it was probably the source of his ear infections.
In rare cases, some doctors believe, strep that isn’t fully treated can lead to PANDAS, a deceptively cute acronym for “pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections.” In children who have the debilitating psychological disorder, the strep bacteria are believed to trigger an immune response that goes haywire. Instead of attacking the bacteria, the body’s immune system attacks the brain. That causes inflammation in the basal ganglia, a region deep within the brain that’s associated with movement, learning and emotions.
Usually, PANDAS can be prevented by promptly treating the strep, but in cases where the antibiotics don’t completely kill the infection—particularly when children stop taking their medication too soon—PANDAS can still take hold. Some kids develop it after their first bout of strep. Others might get it after they’ve had multiple strep infections, each one apparently making the child more susceptible to PANDAS.
The disorder comes with telltale signs, including obsessions, compulsions, anxiety, tics and personality changes. Children can’t concentrate. Their math and handwriting skills decline.
They may stop eating, have trouble sleeping, wet their beds and act like they’ve aged backward. One Bethesda fifth-grader became so consumed by obsessive thoughts that she curled up in a ball on a doctor’s waiting room floor, trying to squeeze herself into the radiator. A Sandy Spring sixth-grader scribbled page after page of confessions to her mother about everything she thought she had ever done wrong.
“The children recognize how crazy the symptoms are, so they try to hide them until they can’t hold it in anymore,” says Dr. Susan Swedo, a senior investigator at the National Institute of Mental Health in Bethesda who was part of the team that first identified PANDAS in 1998.
While PANDAS can mimic other disorders—obsessive-compulsive disorder (OCD), Tourette syndrome, even schizophrenia—its sudden appearance is what makes it stand out. Like the Wiederhorns, many parents can remember exactly when their child’s behavior changed. Though firm PANDAS numbers are still unknown, Swedo believes that one out of between 250 and 500 children develop the illness. The disorder affects more boys than girls and appears to almost solely affect Caucasians, experts say, although researchers caution that it may be underreported or undetected in other ethnic groups. Many children who suffer from PANDAS tend to be smart, popular, active and well-behaved, but researchers aren’t sure if that’s due to reporting (they may hear less about the children who get PANDAS but don’t exhibit those traits), or if the disorder is actually tied to the genes responsible for qualities such as intelligence and leadership ability.
Dr. Beth Latimer sees about eight new PANDAS patients a week.
If PANDAS is caught early, doctors say, a heavy dose of antibiotics can knock it out, though a child may still have flare-ups in the future. More serious cases might require intensive therapies that can cost tens of thousands of dollars. Sometimes that’s a super-dose of antibodies slowly dripped into the veins—a $10,000 process called intravenous immunoglobulin, or IVIG. Sometimes it’s plasmapheresis, a $40,000 procedure in which the patient’s plasma is removed and replaced with healthy plasma. If treated, PANDAS should pass after adolescence, researchers say. Adults rarely get strep, and they rarely get PANDAS.
When the Wiederhorns learned about PANDAS from friends, the symptoms seemed to match their son’s perfectly. But when they told their family physician that they suspected PANDAS was causing Jake’s strange behavior, the doctor didn’t agree with them. A national registry lists only about 100 doctors who treat PANDAS. “We are on the forefront of the discovery of a new disease,” says Jake’s father, Alex Wiederhorn, 45, who works for Macquarie Equipment Finance, which provides lease financing to companies using expensive medical and technology equipment. “It takes time, unfortunately, until the medical community as a whole will accept it.”
Jake’s doctor suggested a psychiatrist, who referred the family to Dr. Beth Latimer, a pediatric neurologist. To Latimer, Jake’s condition wasn’t odd at all—the boy was showing the classic symptoms of a disorder she’s devoted her career to treating. Latimer, who lives in Bethesda, says she sees about eight new PANDAS patients a week at her Georgetown office.
They come from across the country and around the world. Some wait three months for an appointment. “I think the question is: Why isn’t everyone else treating it?” she says. “It’s not as if I’m a renegade.”
After a round of antibiotics, Jake’s initial symptoms went away and life returned to normal. But two years later, when he had a recurrence, he went in for IVIG treatments and the family’s insurance company balked. The Wiederhorns were left paying $15,000 out of pocket, a cost that included IVIG treatments and a tonsillectomy, another procedure for combating PANDAS. Latimer says battles with insurers are common. “They deny everything,” she says. “I don’t think they have a reason most of the time.”
Fortunately, Jake’s father says, the Wiederhorns can afford it. “We’re doing all we can for Jake,” Alex says. “The biggest concern for us is: What do the families do that don’t have the means?” Last summer, when Jake felt symptoms coming on again, his parents took him in for another round of IVIG treatments. They’re hoping a new insurance company will be easier to work with, but say they realize there’s no guarantee.
“Once a disease becomes controversial, then mainstream physicians shy away from treating it,” Swedo says. “Unfortunately, the children are the collateral damage.”