Thienemann: Parents often describe children as turning into somebody completely different overnight. A child who was functioning just fine might suddenly feel like they had been “possessed” by something. They may be tearful and unable to separate from their parents. They may suddenly develop vocal or motor tics. The child may suddenly, after never having had appreciable obsessive-compulsive symptoms, engage in long periods of checking behaviors or cleaning behaviors, over and over. It suddenly takes hours to get out of the house or get the child to bed.
In addition to OCD symptoms, the other cardinal symptom is food refusal. Kids will suddenly be afraid to eat for fear they will choke, or fear that the food is poisoned, contaminated or spoiled. They may say food smells awful or express fear of vomiting. Again, this appears out of nowhere.
It’s like being hit by a train. Most often, the child will miss some school and at least one parent has to take a leave from work. It’s extremely anxiety-provoking because a normally functioning child suddenly can’t go to school, sleep, eat or be apart from parents, and is involved in rituals and tics. Another thing that’s very disruptive is trying to find someone who will provide medical and psychiatric care for your child. How do you find someone if providers say “I don’t believe in it” or “I don’t know how to treat this”?
Q. Why were the PANS/PANDAs treatment guidelines needed?
Thienemann: Right now, there isn’t enough solid, empirical evidence to say definitively “this is what you do” for these patients. For some aspects of treatment we have evidence, but overall we have to bridge the gap between our current clinical experience and knowledge, and the clinical studies. We are continuing to collect evidence, but many children will be identified with PANS/PANDAS before that work is complete. Physicians and other professionals need to know what to do for them now.
Also, despite the fact that we published diagnostic guidelines for PANS/PANDAS in 2015, I think some physicians still feel it’s not legit. We want physicians to take this disease seriously and, even more importantly, we really want them to treat it.
Q. What are some of the most important elements of the guidelines?
Thienemann: The treatment is at least tri-part. The main thrust of treatment is that if there is a triggering infection, we should treat the infection, and also treat close contacts who may be exposing the child to infection. We also have to treat inflammation, which is what we think causes the brain symptoms.
The psychiatric portion of the guidelines recognizes that the child and family are really suffering and the child needs psychiatric treatment to help with symptoms. We have to manage the child’s distress so they can get medical treatment, get blood drawn, get MRIs, take medications, so the family can sleep, and the child can sleep. We also need to help the family understand what might be going on, what they can anticipate from treatment, how to interact with each other and their child around the illness, and how to interact with the child’s school.
Unfortunately, a lot of the tools in psychiatry are medications — for OCD, depression, ADHD, tics — that don’t work the same way on an acutely inflamed nervous system as they would on garden-variety, idiopathic childhood mental illnesses. When medications are used, usually for residual symptoms, the guidelines suggest beginning with one-fourth of the typical starting dose of medication and increasing the dose very slowly. There can be complex interactions between medications, and between the medications and other changing variables related to where a child is in the illness and concomitant treatments. It makes treatment complicated.
Q. What are some of the gaps in how our health care system is set up to treat these children?
Thienemann: Unfortunately, we lack a good place to hospitalize these kids.
If we put them in a pediatric hospital when they’re unpredictably agitated or rageful, it doesn’t work. Pediatric hospitals are not set up for kids who may run out of the hospital or hit someone. Little sounds bother PANS/PANDAS patients, so the constant beeping of monitors doesn’t help, and lights bother them, so they don’t sleep in a hospital setting. It can be very difficult on hospital staff.
Psychiatric hospitals are also hard for these patients to tolerate. They have a brain inflammation problem, not a behavioral problem, so typical behavioral interventions aren’t helpful. And in psychiatric settings, parents don’t get to stay with their children. For many other pediatric psychiatric diagnoses, the parents may be a part of the problem, and some of the process of diagnosis is seeing how the child acts away from parents. But most children with PANS/PANDAS have terrible separation anxiety as part of their illness, and it’s very hard on them to be away from their parents. Psychiatric hospitals also are usually not comfortable working up an infection or giving intravenous treatment, which is required for some of the immune therapies used for this disease, so the children’s medical needs can’t always be met in a psychiatric setting.
Often, there is no place other than home to treat children with PANS/PANDAS, which can be really hard on the family. Ideally, for the future, I see a day when we have med-psych units for these patients in the same way that we now have excellent med-psych units for eating disorders. We would need hospital staff who specialize in addressing the psychiatric problems and family stress that go along with PANS/PANDAS, as well as people who can diagnose and treat the more traditional medical problems.
Q. What other changes do we need next for PANS/PANDAs patients?
Thienemann: Early identification. When someone comes to their doctor with sudden behavioral change, emotional change, suddenly has tics or suddenly stops eating, this disease should automatically be on the differential diagnosis. If we identify it early, it’s less complicated to treat and the patients have better outcomes.
