By Shawna CohenUpdated Oct 09, 2015
"Mommy, look! I can’t stop!” Those were the frightening words Stefanie Weiss woke to early one morning in 2008. She opened her eyes to see her son Cole,* age seven, at her bedside, exhibiting a severe, uncontrollable facial tic that she and her husband had never seen before. Later that day, Cole started standing in front of the refrigerator checking expiration dates on all the food, a pattern he repeated daily for weeks afterward. He also asked questions about food at the grocery store, at restaurants and at other people’s houses. A few weeks prior, he had contracted a stomach bug, and he told his parents that tossing any soon-to-be-expired food would prevent him from getting sick again. Weiss and her husband tried to convince Cole that contaminated food hadn’t caused his vomiting or his facial tic, but he couldn’t stop obsessing about food safety. It was like he was stuck on a loop.
“We were panicked,” recalls Weiss, then a stay-at-home mom of three kids. Prior to this incident, her son had been happy, healthy and highly social. He had some slight anxiety, she says—when he started nursery school, for example, he had a hard time separating from his parents—but was now generally a well-adjusted boy with lots of friends. He got good grades at school, and in the summer, he’d go off to day camp with a smile on his face. But when he woke up that morning, he was like a completely different child. Weiss and her husband weren’t the only ones wondering what was wrong with Cole. “I remember being at a birthday party soon after the tic started, and other moms were asking, ‘Is everything OK? Why is he doing that?’” says Weiss. “It was definitely strange behaviour.” They called a child psychologist. He suggested that they take Cole for a strep test.
“I was flabbergasted,” says Weiss. “We didn’t understand the correlation.” The psychologist told them about something called “PANDAS,” an acronym that stands for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. She immediately began to research this rare condition online. The disorder occurs when the antibodies that form to fight a common strep-throat infection instead begin to attack portions of the brain. Children with PANDAS experience acute onset of obsessive-compulsive behaviour, and may also develop tic disorders including Tourette’s syndrome.
Cole hadn’t had strep recently, just the stomach bug. The last time he’d had strep was a few years prior, before kindergarten. But as they ran down the checklist of all the possible signs and symptoms of PANDAS, they realized that their son had every single one. Cole’s throat culture for a strep infection, however, came back negative. The psychologist insisted they return to the paediatrician for a specific type of blood test, called an anti-streptococcal antibody titre, which determines whether there is evidence of a previous strep infection, not just a current one. Cole’s paediatrician was skeptical. He’d heard of PANDAS before, but was adamant that he didn’t want to do the titre. “I’m sure you’re a nervous kind of mom,” Weiss recalls him saying. “Three out of 10 boys get tics. Don’t worry about it—it’s just a phase.”
This is when Weiss says her maternal instincts kicked in. “I thought, ‘Something is off.’ The paediatrician was looking at me like, ‘You don’t know what you’re talking about, he’s fine.’ Maybe another mom who didn’t have the confidence would have gone home and felt sorry for causing a fuss, but I was put off by it. I needed to find another doctor who believed in me, and in my child.” She called an ear, nose and throat specialist (also known as an ENT), who agreed to test Cole’s blood for strep. His strep titres came back at 1350—well above the normal range of 0 to 400.
Two weeks later, on the advice of the ENT, Cole had his tonsils removed, to reduce the risk of recurring strep infections. Three months after the tonsillectomy, the strep levels in his blood had dropped by half. His doctors then put him on a strong dose of antibiotics and, within days, the tic had diminished. Over time, he stopped obsessing over food labels and contamination, and he returned to his normal self. “It was dramatic,” says his mother. “His behaviours dissipated, and everything started to go away.”
Today Cole is a vibrant, outgoing 12-year-old who plays basketball, writes rap songs and uploads web videos. He’s on a low daily dose of what’s called prophylactic antibiotics to prevent another strep infection, and hasn’t had an episode in five years. The biggest downside to being on antibiotics long-term is that your body can grow immune to them (and they wouldn’t work if you needed to treat a life-threatening infection), which is why some doctors don’t advise this course of treatment. (Instead, PANDAS patients are prescribed cognitive behavioural therapy and medications such as anti-obsessional SSRIs.) But Weiss’s concerns about Cole’s daily antibiotic dosage—and the overprescribing of antibiotics in general—are overshadowed by her memories of what her son’s episodes were like.
“I see the antibiotics as a bit of an insurance policy,” says Weiss, who also has a 10-year-old daughter and a seven-year-old son. “I have kids in my house all the time. There were times during strep season when another mom would mention, ‘Oh, I forgot to tell you, my daughter had strep last week.’ It was too nerve-racking.” As Cole reaches adulthood, his doctors hope he will become immune to strep and no longer need the prophylactic antibiotics, says Weiss.
In the years since Cole’s illness began, Weiss has launched a career as a mental-health consultant specializing in OCD, anxiety, ADHD, PANDAS and other related disorders. While she began building her network within New York state, where she lives, she now works with fellow PANDAS parents from all over the US and Canada. She describes her role as “handholding”—she tries to arm her clients with the right research papers, doctor referrals, lists of questions to ask and what types of tests to request. “Nobody was able to steer me in any direction, which was overwhelming. Strep is a common illness, and I wonder how many children are going undiagnosed,” she says.
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PANDAS is difficult to diagnose because there is no definitive or standardized test for the disorder. “It’s tricky,” says Paul Arnold, a staff psychiatrist and head of the Anxiety Disorders Program at Toronto’s Hospital for Sick Children. As he explains, many PANDAS symptoms—excessive eye blinking, or an unhealthy obsession with germs—look like typical childhood OCD or tic disorders. It’s often difficult to tell if the behaviours are the result of a recent strep infection or simply a coincidence. Arnold also points out that PANDAS is relatively new in the medical field, which means that many doctors are simply unfamiliar with it. He first learned of the disorder in 1998 when he read a study called “P.A.N.D.A.S.: Clinical Description of the First 50 Cases,” published by the National Institute of Mental Health (NIMH) in the US. The study’s lead researcher, paediatrician Susan Swedo, is credited with giving the disorder its name, as it was the first piece of literature published using the acronym as shorthand.
Swedo is not without her skeptics. Many people in the medical community still insist that her PANDAS theory is scientifically unproven and that it’s nothing more than a hypothesis. One doctor in particular, Roger Kurlan, director of the Movement Disorders Program and the Atlantic Neuroscience Institute in Summit, New Jersey, has been vocal in expressing his views. He believes that there’s no evidence proving that strep exacerbates tics. “His original criticism of PANDAS was that we made it up,” says Swedo. Kurlan argues that he personally has never seen a case, and therefore can’t confirm that it exists.
In 2010, Kurlan presented a webinar titled “Can Strep Infection Cause Tourette Syndrome: Is PANDAS Real?” on the New Jersey Center for Tourette Syndrome and Associated Disorders website. In the comments, he wrote, “Not many good scientists are convinced that the evidence really indicates that PANDAS ‘has been discovered.’ It is clearly a hypothesis that has some evidence in its favour, but more evidence against it right now. People should know that in the 1950s and 1960s a very similar controversy occurred when some doctors proposed that autism was due to strep infections. Eventually, good scientific studies proved this to be wrong. Since strep is so common, it is easy to find it in association with anything. What is important is not that it is present, but whether or not it actually causes the problems of interest.”
Doctors who say they have seen cases of PANDAS first-hand report that a child with the disorder will change overnight, as Cole did. “We are not referring to the child with a little eye blinking or handwashing,” says Swedo, now chief of paediatrics and the developmental neuroscience branch at the NIMH. “This is one who’s irritable and emotional, and who refuses to leave the house—a child who peppers his parents with five questions a minute during every waking hour.” Swedo says she recently saw a 12-year-old who, over the course of two to three days, went from being a normal kid on his school’s swim team to exhibiting terrible contamination fears. He wouldn’t get into the swimming pool, then refused to walk to the kitchen table unless his mother first scrubbed down the floor. He stopped eating because he feared all food was contaminated. Finally, he could no longer leave his bed, which he considered the only clean place in the house. Swedo treated him with intravenous immunoglobulin (or IVIG) therapy, a treatment sometimes used for other autoimmune disorders. This medication consists of filtered antibodies from a mix of blood donors. The new antibodies neutralize the harmful antibodies. Less than a month after the IVIG therapy, the boy reported he was feeling 95 percent better.
Ottawa mom Kelly O’Donnell knows all too well what it’s like to have your child go from a typical healthy kid to completely non-functional in a mere 24 hours. On a Thursday in September 2012, O’Donnell, a nutritional consultant, noticed her seven-year-old daughter, Sophie,* was more emotional than usual. Everything her older sister said made her burst into tears. Later in the day, Sophie’s teacher even called home to report that the girl was acting completely out of character. She was very anxious about perfecting her writing, and her school papers were full of holes made from erasing her work repeatedly. (O’Donnell later learned that a change in handwriting is a common symptom of PANDAS.) Sophie also kept repeating, “I’m sorry” over and over again, for no apparent reason. When O’Donnell said “Good morning” to her daughter on Friday, Sophie repeated “Good morning” 25 times in response, while patting her chest, hip and knee. This behaviour—both the parroting and the gestures—occurred throughout the day. O’Donnell thought her daughter was having a nervous breakdown.
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“In the beginning, it’s ridiculously scary. Twelve hours ago she was perfectly fine. She was running around and playing with other kids. She could hold conversations. Did a switch flick in her head, and we’ll never see that side of her again? You think, ‘Is this my new normal?’” she says. The O’Donnells took Sophie to the Children’s Hospital of Eastern Ontario, in Ottawa, where a doctor suggested they check her for strep. “My husband and I are looking at each other like, ‘Why strep? What for?’ She had no sore throat, no fever, no classic signs of strep whatsoever,” recalls O’Donnell. By noontime on Saturday, they discovered Sophie had indeed tested positive for strep. (About 12 percent of children are asymptomatic strep carriers.) The doctors put her on antibiotics and within 24 hours, O’Donnell says her daughter was almost completely back to health.
Sophie still remembers the episode well, and can differentiate between how she behaved when she was sick and how she behaves when she is well. She occasionally worries that her PANDAS will return. “She remembers all the things her brain would make her do over and over. And now, if she repeats herself for any reason, she asks us whether it’s PANDAS coming back.”
Like Weiss, O’Donnell spends much of her time empowering other families whose children are exhibiting PANDAS-like symptoms and don’t know where else to turn. “The disorder often gets passed off as something else, like ADHD or even autism,” O’Donnell says. She has found that many doctors have simply never heard of it. During one appointment, her own family doctor opened her laptop and looked it up in a medical dictionary in order to familiarize herself with the condition. She has also created a website for Canadians dealing with the disorder (pandascanada.wix.com/pandascanada), and the organization’s Facebook page acts as a support network for members.
Building awareness about PANDAS is not meant to scare parents into thinking that a typical case of strep is going to transform their child. The diagnosis is much more helpful to parents whose children are already experiencing strange episodes of anxiety and obsessive behaviour, and who are looking to pinpoint a cause, as well as find treatment to help their child return to health. Swedo is careful to explain that all cases of strep infection need to be taken seriously—regardless of whether you’re a PANDAS believer or not—to prevent rheumatic fever, an inflammatory disease that can develop when strep throat is left untreated.
There are no clear numbers identifying what percentage of children will develop PANDAS. Arnold, the Sick Kids psychiatrist, says there’s too much debate in the field over how to define PANDAS (personally, he looks for recurring patterns). Both doctors point out that boys are more likely than girls to develop tic disorders, one of the hallmark features of PANDAS.
We do know that millions of children will contract strep each year. According to the NIMH, almost all school-aged children get strep throat at some point in their lives, and the average grade-school student will have two or three strep-throat infections annually.
Daniel Flanders, a Toronto paediatrician, reports that if a child older than five has a sore throat, there is a 37 percent chance that it’s strep. Overall, he says, nearly four in 10 sore throats are strep (but many go undiagnosed). Children with symptoms such as high fever and vomiting are usually prescribed a round of antibiotics; kids with milder symptoms may wait it out. (Without antibiotics, strep is contagious for two to three weeks, instead of just 24 hours.)
In the meantime, parents like Weiss and O’Donnell are working fervently to make it easier for families to seek proper treatment for PANDAS. They have collected countless stories of parents begging doctors to do strep tests and being made to feel like they’re crazy. Despite these anecdotes, it proved difficult to find a doctor who would go on record arguing that PANDAS is not a real disorder. Many will simply say that it’s extremely uncommon, and that they have never seen a case. Flanders sums it up this way: “Though very possibly a true disease, PANDAS is probably over diagnosed. Behavioural disturbances in children are common and strep infections in children are also common, so the likelihood of both happening at the same time, without necessarily being related, is relatively high. Just because a child has OCD symptoms and a recent history of strep throat doesn’t mean that the OCD was caused by the strep throat. Correlation and causation are two different things.”
Most people will recognize that a bout of strep throat does not mean a child will become obsessive-compulsive, just as OCD does not always equal PANDAS—it’s far more complicated than that. But when it’s your kid who’s suffering, and you don’t know why, it’s a very scary situation. You’d do anything for some answers. Many parents would explore every avenue, including consulting Dr. Google, and asking for a strep test. As strange as it seems, finding a link between your child’s OCD and a recent infection can actually come as a relief.
*some names have been changed
A version of this story appeared in our December 2013 issue with the headline "By the throat," pp. 124-127.