Overcoming Sydenham Chorea and PANDAS—Three Sisters’ Healing and Autoimmune Challenges
Hear Gwen’s Story (3 sisters—age 11, and twins, age 8—at onset)
We first learned about the bizarre things strep can do to children when our oldest child lost control of her body at the age of eleven. It started with a couple of tics: flicking her hands, rolling her tongue, shrugging. We thought they might be some sort of pre-adolescent mannerisms. But over the next two weeks, she started moving constantly, twisting and flapping in a crazy-looking dance. She couldn’t drink from a cup without splashing the contents in her face. When I read to her at bedtime, her arm flew out and hit me in the mouth. Our family practitioner referred us to a neurologist, whose scheduler offered an appointment in a week. Worrying about Huntington’s or a brain tumor, I sobbed on the phone, and the scheduler got her in the next day. The neurologist took one look at our daughter’s attempt to walk down the hall to his exam room, turned to me, and said, “It’s not a brain tumor. It’s rheumatic chorea,” and admitted her to the hospital.
Later, we found out she had elevated strep titers, indicating a recent infection. She had not been sick, as far as we knew: no sore throat, no fever, no missed school. She had rarely been sick in her childhood. This was our first experience with the concept of “subclinical” infections, and our first lesson in post-infectious encephalopathy. What parent knows that a child can have strep without a sore throat or fever? What parent knows that strep can cause the nervous system to break down? Over 3 days of IV steroids, our daughter recovered control over her body. She went home on a prednisone taper, and instructions to take daily penicillin until the age of 21. When we balked at the extended penicillin prescription, the infectious disease doc-tor explained that if we didn’t prevent another strep infection our daughter could have a recurrence, with potential heart valve damage. We embraced the plan and believed the problem was solved. (This is how one expects medical treatment to work when one has had no experience with disability or chronic illness.)
During our experience with rheumatic chorea (AKA Sydenham’s chorea), we suffered through a series of emotional stages that we would later experience in a protracted way with PANDAS: terror that our child was being forever transformed; grasping at theories about what was happening to her; seeking help in many directions at once; relief at a diagnosis; relief with treatment and remission; resistance to the idea of long-term antibiotics; hope for a complete cure; fear of relapse. What we did not experience was physician denial of the diagnosis, refusal to investigate or treat, or confusion about what treatment was needed.
Less than a year after their older sister’s ordeal with post-infectious neurological problems, our 8-year-old identical twins succumbed within 5 months of one another to overnight onset anxiety and OCD behaviors. Each girl had a documented strep infection preceding symptom onset, but in their cases, none of the many doctors who investigated their symptoms focused on that. Our exuberant, adventurous girls woke up one morning, afraid to leave my side, afraid to eat, with constant stomach aches. For months, getting out of the house was a struggle. If they did manage to get through a day at school or camp, they might fall apart once home again: hours-long episodes of panicked screaming and pacing, furious oppositional behavior, repetitive anxious questions, compulsive rituals, refusal to be left alone.
Sometimes, unaccountably, the symptoms would disappear, like flipping a switch, and we would rediscover our normal children, suddenly, cheerfully going to school without a backward glance, coming home happy, eating normally. When that happened, we would begin to relax and hope that ordinary life had returned. Then, a few weeks or months later, we would lose them again to constant stomach aches, hours-long rages, distrubed sleep and intrusive thoughts instructing her to die, or to shred something or to unbuckle her sister’s seatbelt. Both girls had always loved to snuggle, but during what we came to call “flares,” their bodies became taut, and they couldn’t stand to be touched. They had always been enthusiastic eaters but now refused to eat, lost weight, and did not gain weight for 2 years. They became hyper-vigilant and perseverative, asking incessantly how many minutes or hours each activity would take, tracking our every plan and movement. Because these symptoms accompanied the constant stomach aches, the doctors who take care of bodies referred us back to psychiatric treatment, and they did not come together over the case. The girls had big circles under their eyes, their pupils were widely dilated, and we took comfort in the thought that something physical was happening.
Although I read about PANDAS within months of symptom onset, providers either didn’t know about it or denied it. A month after our third daughter fell ill, I reached out to the same neurologist who had treated our first daughter, whom we revered. He told me PANDAS isn’t real and declined to see the twins at all. After 18 months of full GI workups and psychotherapy, symptoms got so bad I convinced our sympathetic PCP to prescribe antibiotics and prednisone, based on my reading. (By then, reading about PANDAS had become my second job.) As had happened with our first daughter in the hospital, we saw all the twins’ symptoms cease abruptly on a short burst of steroids. But as soon as the steroids were finished, the symptoms roared back, seemingly worse than before. I had read that a positive response to a steroid burst indicates that a good response to IVIG is likely. But we couldn’t find an in-network specialist willing to prescribe IVIG.
Not only did IVIG seem impossible to obtain, we were also apprehensive about the invasiveness of the treatment. So, we followed advice from NIH and a local rheumatologist to manage our daughters’ cases with antibiotics, steroids and psychiatric care. Each treatment option brought a tangle of concerns. We read that SSRI’s can make symptoms paradoxically worse in PANDAS kids, so we feared trying them. We observed that cognitive behavioral therapy was of no use during symptom flares. One doctor suggested that a vaccine challenge might help confirm immune functioning, with disastrous results. It would be a total of four years before we finally tried IVIG.
Meanwhile, our girls struggled with relapsing/remitting symptoms: OCD, separation anxiety, food restriction, rages, abdominal pain and nausea, disabling emetophobia, sleep disturbance. Despite multiple rounds of high-dose antibiotics, years of antibiotic prophylaxis, several courses of prednisone, years of CBT/EXRP therapy (including a partial hospitalization at the age of 10 in the pediatric psychiatric unit at Major Illinois Hospital), full GI workups, elimination diets and various supportive therapies such as homeopathy and acupuncture, they continued to suffer, eventually receiving homebound services from the school system.
Our family wasted precious years seeking care in an atmosphere of mainstream medical skepticism and ignorance. As our twins were shunted from one reluctant silo practitioner to another, I longed for the kind of coordinated care our older daughter continually received at the local flagship children’s hospital. In the years following her SC, she developed a series of autoinflammatory problems. A coordinated care team (rheumatology, dermatology, neurology, ophthalmology, pulmonology) convened over each new symptom and treated her successfully despite scant evidence for some of the treatments. Meanwhile, her sisters, who by the third year of their illness, were so debilitated by psychiatric and physical symptoms that they missed six months of school, could not obtain any sort of coordinated care. After years of being turned away from various departments at the children’s hospital, we resorted to seeking IVIG treatment from an independent PANS doctor and putting tens of thousands of dollars on a credit card. This resulted in the first sustained remission of symptoms. The decision to seek care “off the grid” was agonizing for us, but we felt we had no choice as our children grew sicker. I now wish we had made that decision years earlier: not only did it greatly relieve our children’s suffering, but it ultimately enabled us to bring them back to the mainstream for treatment. Once we had a documented remission and had convinced insurance to authorize a second IVIG infusion, an in-network neurologist agreed to take our case and has continued to prescribe this life-restoring treatment.
Monthly IVIG has been the elixir that allows our girls to function. In their eighth year since onset, they are doing so much better. They attend a selective enrollment high school, have plenty of friends and many happy days. But they continue to cope with symptom flares of intrusive thoughts, severe fa-tigue and autonomic dysfunction. We continue to do a variety of things for their wellness, but there is no doubt that IVIG is key. We tell our story to offer hope to families and to encourage medical providers to recognize and treat this illness early. Had someone recognized and treated what was happening to our girls when they were 8, they might have achieved a lasting remission sooner and avoided years of suffering.
In the middle of our family’s PANDAS/PANS journey, I regularly combed the parent forums, trying to glean the collective wisdom of other P/P parents. I read many anguished posts and occasionally posted my own. Many of the posts and answers that buoyed me were from the veteran parents, whose wisdom came from the heights and depths of long experience, whose children were in remission, or had recovered and gone on to live normal lives.
As I clung to their advice and stories, a question stuck at the back of my mind: why were these parents of recovered children lingering on these forums? Why in heaven’s name wouldn’t they want to turn their backs on this difficult illness and enjoy ordinary concerns? Now that I am one of those veteran parents, I know why so many of us remain on the parent forums. Healing is possible, and when it happens, you rejoice. But the experience of parenting through PANDAS is so humbling, leaves such a mark, that you never forget. Recovery is possible, and so is relapse. PANDAS/PANS is always the wolf at the door.