Two years ago, Craig and Erica Mills packed up the family minivan and headed north from Toronto to a campground in Tobermory, Ont. Annual camping trips were loved by their three young sons—two days of sleeping in tents, cooking hot dogs over the fire and unmitigated mud play. But this time, something was different with their oldest son, seven-year-old Knox, who kept complaining about feeling dirty. “This is normally a super outdoorsy, roll-around-in-the-mud kind of kid, and out of nowhere he’s freaking out about whether his hands are clean enough,” Erica recalls. On the drive home, Knox became increasingly agitated—the car was sticky; his brothers were gross. He kept asking his parents to pull over so he could wash his hands. In a bid to distract him, they asked if he liked a song on the radio and he was unable to answer that simple question. Instead it was, “I don’t know, I might, maybe, I might, I don’t know.”
By the following morning, Knox was in full meltdown mode, crying constantly, wiping down surfaces with a cloth or his T-shirt, washing his hands several times an hour and covering himself—his arms, his legs, even his head—in hand sanitizer. The behaviour was so intense and out of character, his grandmother, a doctor, wondered if he had experienced a traumatic event. “We asked him, ‘Knox—did someone hurt you? Did someone touch you?’” Erica says. “We had no idea what was going on and were absolutely terrified. Two days earlier, Knox had been our happy, healthy little boy, and now he was a completely different kid. It was like he was in a trance.”
Like most parents—and a lot of medical professionals—the Mills family had never heard of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, or PANDAS. The disorder was identified in the late ’90s when Susan Swedo, now the chief of pediatric and developmental neuroscience at the National Institute of Mental Health (NIMH) in the United States, posited a connection between an abrupt onset of obsessive-compulsive disorder (OCD) and A streptococcus, a.k.a. strep throat. Twenty years later, the condition is still struggling to gain mainstream status in Canada. Meanwhile, families dealing with the life-altering symptoms are faced with stigma as they take on a frustrating fight for resources and recognition.
Getting a PANDAS diagnosis
“It was agony,” says Erica, of the two weeks between the start of Knox’s symptoms and when he began taking antibiotics to treat them. Knox repeatedly told his parents he wanted to make the obsessive thoughts stop by ending his life, once even standing in the middle of the street, another time opening the car door as the vehicle was in motion. “And then afterwards he would tell us, ‘That’s not me—don’t believe me when I’m in one of my rages,’” says his mom, who took her son to Toronto’s Hospital for Sick Children (Sick Kids) a few days after that 2016 camping trip. He had washed his hands so many times they were bleeding. An emergency room doctor diagnosed Knox with OCD and prescribed cognitive behavioural therapy (CBT) sessions. At the CBT appointments, he talked about his anxiety and learned deep breathing as a coping technique. Still, his condition wasn’t improving and his parents weren’t convinced their son’s experience lined up with his diagnosis. “It was just so fast, which is not something associated with typical OCD,” says Erica. Stressed and unable to sleep, she spent her nights online, researching rapid-onset OCD and eventually landing on a website that described Knox’s experience almost exactly: the physical tics, the rage, the decline of motor skills, the urinary dysfunction, the almost instant and acute onset of obsessive tendencies. “I thought, PANDAS. This is it.”
According to the PANDAS Network (an American advocacy group), one in 200 children is affected by the condition, but it cautions that estimate is conservative because of the lack of awareness of the disorder in the public and the medical system. While the underlying causes of PANDAS are unknown, the theory is that A streptococcus triggers an atypical autoimmune response. Normally, when a child gets strep (as almost all kids do, at some point), the immune system produces antibodies to fight the bacteria in the throat; when the infection is gone, the body stops producing the antibodies. But with PANDAS, the immune system just keeps going, and the antibodies begin to attack the basal ganglia, a part of the brain involved in motor and cognitive functions, as well as mood and behaviour. The result is a range of symptoms, many associated with rapid-onset, acute OCD (like motor or vocal tics, eating restrictions and fear of germs), extreme emotional swings (for example, from low depression to hysterical laughter) and ADHD (hyperactivity or extreme fidgeting), as well as the deterioration of basic motor skills (handwriting or tooth brushing), increased urinary frequency or overnight bedwetting, and sleep complications.
In mild cases, the condition can come and go undetected, resolving itself before the symptoms become notable. The more severe cases are life-altering, lasting months or even years. PANDAS symptoms wax and wane—some kids go weeks or months between “flares,” the term for active symptom periods. “Rages” is the word used to describe explosive outbursts in which a child may convulse uncontrollably, scream, bang their head against a wall or threaten violence. Intrusive thoughts, paranoia and suicidal ideations are also common.
Wendy Edwards, a paediatrician based in Chatham, Ont., became one of the country’s leading PANDAS authorities by accident. She first heard about the condition in 2012 at a conference on autism (immune dysfunction is prevalent among people who have autism, possibly placing them at a greater risk). A few weeks later, she was consulting on a young patient with extreme OCD symptoms and, remembering what she’d learned at the conference, suggested testing for strep, which was positive. The patient went on antibiotics, and the symptoms disappeared. “In the majority of cases, the kids who are treated right away do very well,” she says. Unfortunately, this is rarely the case. Instead, Edwards sees kids and families who have spent months, if not years, being bumped from one specialist to the next, hearing misdiagnoses such as anorexia, schizophrenia and degenerative brain disorders. This holding pattern is troubling since cases that go untreated become increasingly complicated to detect. If there wasn’t a test for strep done around the time of the onset of behaviour, doctors need to rely on symptoms to make the diagnosis. (There is a blood test that can indicate a previous strep infection, but Edwards says it can’t be counted on to confirm the child has PANDAS.)
Some skeptics point to the lack of a conclusive test as proof that the theory behind PANDAS doesn’t hold up. Others question the perceived strep–OCD connection, countering that since about two percent of American kids get OCD, and almost all kids get strep, the correlation may be mere coincidence. But PANDAS experts say this fails to account for the ways in which the disorder is distinct from OCD, such as the rapid, acute onset of tics and repetitive behaviour, loss of motor skills like printing, overnight bedwetting, extreme emotional swings and intrusive thoughts. Plus, let’s not forget that new conditions require new understanding. “If we only treated the conditions we learned about in med school, we’d be back in the Dark Ages,” says Edwards.
The only real point of agreement is about the need for further research. To this end, at least, the medical establishment seems to be coming around. In January 2018, the Canadian Medical Association Journal published an article called “Growing Consensus on Link Between Strep and Obsessive-Compulsive Disorder,” which referenced “a growing body of evidence [that] suggests that these bewildering changes may be triggered by a streptococcal (strep) infection.” In December of the same year, doctors from Sick Kids published a paper in the Canadian Paediatric Society’s journal Paediatrics & Child Health called, “PANDAS/PANS in Childhood: Controversies and evidence,” determining that “while definitive proof of the autoimmune hypothesis of PANDAS is lacking, given the heightened attention to this entity and apparent rise in use of this diagnostic category, addressing questions around diagnosis, treatment and etiology is imperative.”
Edwards says the swing in momentum—which includes plans for an Ontario provincial advisory council as well as medical conferences devoted to PANDAS and PANS (a related condition that is not associated with strep) in locations from Saskatoon to Sweden—is encouraging. “A year ago, there was this idea that people who believed PANDAS exists were crazy,” she says. “It’s a little bit better now, and parents are given more credit when discussing their child’s situation.”
Living with PANDAS
In 2015, Kerry Henrikson founded PANDAS/PANS Ontario, a public advocacy organization and online support group that now serves more than 700 members. She says PANDAS parents often feel traumatized twice over: “There is the trauma of having a child who is sick and then there is the other layer of uncertainty, doubt and a lot of stigma.” She has seen PANDAS moms and dads accused of Munchausen’s syndrome by proxy (in which a parent invents their child’s sickness to draw attention to themselves) and even investigated for child abuse.
Like most parents of kids with PANDAS, Henrikson first heard about the condition via Dr. Google. All three of her children have PANDAS (Edwards says she does see families that have multiple children afflicted so, while it is not the norm, it is not uncommon). Her two eldest have responded well to the treatment protocol endorsed by the NIMH for PANDAS—a combination of long-term antibiotics to handle infection, in addition to behavioural and pharmacological treatments, like CBT and anti-anxiety medications for emotional swings and OCD symptoms. Some doctors, however, are hesitant to prescribe antibiotics based on their potential to damage gut health and their role in the development of resistant antibodies. (The Canadian Paediatric Association currently does not have a position on PANDAS and does not endorse any guidelines. In her practice, Edwards follows the NIMH treatment guidelines.)
Henrikson’s youngest daughter, Marin, still suffers from frequent flares, having responded less positively to the medications than her older siblings. She may improve with age, because many kids who receive treatment see their PANDAS symptoms abate as they enter their teens. The theory is that the immune system matures out of whatever was causing it to produce antibodies. For those who aren’t treated, the effects of PANDAS later in life are unknown.
The Mills family has worked with several doctors, including Edwards, in an effort to help Knox. Initially treated with antibiotics for six months, he is now on an extremely low dose of anti-anxiety and anti-psychotic medication, which has been largely effective. Last summer the family went camping again, and all three sons were their usual mud-loving selves. It’s been over a year since Knox’s last significant flare. There are smaller, more manageable obsessive behaviours, which may be brought on by further exposure to strep. “In our house, if someone has even the tiniest tickle in their throat, we swab,” says Erica. “I have my son back. Dropping a few swabs off at the lab is no big deal.”