In 2016, my daughter, Gracie, was a 4th grader on top of the world. She was a straight A student, a student council member and a talented cheerleader and softball player. She loved spending time with her friends, doing arts and crafts and being a big sister.
Then, seemingly overnight my girl was gone.
Gracie had recurrent strep infections that fall and a bout of pneumonia. Nobody likes to see their little one sick – but when school starts, you expect it. So, I never panicked. I don’t know, even knowing what I know now, that there’s anything I could have done differently. But I kick myself, because those ordinary infections, which I viewed as minor inconveniences, were powerful enough to turn our worlds upside down.
I know the day I realized she was gone, but when I look back the warning signs were there before, or rather, I guess there were symptoms that I overlooked. Her handwriting had become less legible over the summer and school work, which had always come easy to her had become a challenge. I thought she wasn’t giving it her all and I pushed harder. She started rocking back and forth, constantly. I harped on her to sit still. She couldn’t remember the simplest things. I pleaded with her to pay attention. Her attitude changed. There was defiance. I thought, “These tween years must make up for the lull that followed the terrible two’s.” Not to make light of a situation that wasn’t, but I didn’t know was I didn’t know.
These were small details, but they were small details that could have clued me in to a bigger picture.
Gracie’s body, as I’d come to find out, was attacking her brain. Antibodies that should have fought those ordinary strep and respiratory infections had turned against her, causing inflammation on her brain. And that inflammation led to everything from unprecedented tics, memory loss and anxiety symptoms, to catatonia, seizures and even psychosis.
A diagnosis did not come easy. In two years’ time, Gracie saw more than 20 specialists, across 7 hospital systems and in 3 different states. Most doctors agreed something was wrong, many speculated as to “what” but few were willing to put it on paper and even fewer were willing to treat.
Terrifyingly, if not for a mother’s intuition, one dedicated neurologist, and a whole lot of luck the reality is my girl would have been labeled as mentally ill and left for dead.
Like I said, I missed the “warning signs.” It could have been because my tolerance for odd behavior was high, or it could have been that her coping skills were extraordinary. Or maybe it was both. Whatever the case, I didn’t know something was wrong until I looked at my daughter one morning and she didn’t look back. Even then I didn’t panic. Until that night when she had her first seizure. And came back out of it convinced everyone was trying to eat her. That was the first time I couldn’t’ help her, and sat helpless, waiting for an ambulance to carry her away.
The medics who responded, and the ER staff all said she appeared “postictal.” Which would have meant she had a seizure. They stuck a bunch of wires on her head to monitor brain activity but reported that it, the EEG, showed nothing. It would be the first of many curious findings. They used an MRI machine to look at her brain. The part of it that controls visual stimuli was getting excessive blood flow – explaining the hallucinations, but still not answering “why.”
She had strep, again. So they gave her antibiotics. They gave her steroids, too. Maybe for the pneumonia, truthfully, I don’t remember. What I do know is curiously, she improved. Her discharging doctor suggested we follow up with the movement disorder clinic, to be evaluated for something called “PANDAS.” We did. But they told us after much research on the subject they, as a clinic, had concluded it “wasn’t a real diagnosis,” and sent us on our way.
A month or so later, things had gone downhill again. Rapidly. Gracie was so claustrophobic she would try and jump from moving vehicles, she was paranoid, her body was twitching, and her heels never touched the ground when she walked. And nobody knew why. And then another seizure happened, and again we went to the ER.
She was combative once the seizure stopped. And convinced a woodpecker was coming after her. They treated those symptoms with a drug called Ativan. Which should have calmed her, but didn’t. She, in fact, became more agitated. They tried again. She became angrier. And convinced that her dad and I were there to hurt her. Which was the hardest thing to hear. We had become part of her hallucination. The first resident who saw her said, despite her young age, it looked like schizophrenia. The attending doctor, thankfully, said “No, the onset was too abrupt.” Again… we had no answers. And she was admitted.
Another resident who was evaluating her again proposed PANDAS. It fit with what we were seeing, and some of the bizarre behavioral changes we’d noted, too. Like a texture aversion to all but dry fit materials and an insistence on matching socks, which had never been characteristic of her before.
PANDAS, or “Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infection” he explained results from “antibodies that should attack infections, like strep, attacking the brain.” It’s characterized by “sudden behavioral changes, hand writing deterioration, separation anxiety, OCD and tics.” It made sense but was “so rare.” The attending doctor instead proposed a diagnosis of Lyme. And a PICC line was inserted into her body and Gracie was treated with three weeks of IV steroids and antibiotics. There were some gains, but things still weren’t right. And the more I read, the more PANDAS made sense.
One of the experts for treating PANDAS was in Cleveland, just a few hours away. So, I called for an appointment. Gracie spent her 10th birthday in his office. And as I rattled off symptoms he nodded furiously and said, “I can help her.” Then he said, “But insurance will fight you every step of the way.” PANDAS, he explained, was an extremely controversial diagnosis. He said there was an insurance billing code for getting sucked into the engine of a plane, but not for your body attacking its own brain. We were disheartened but not deterred. We were ready to fight for our girl. We set a follow up appointment for after she was weaned off all the medications that had been trialed.
Things never go as planned. And the school called me one day not long after to say Gracie’s pupils were dilated and she was not responsive. I’ll never understand why they called me instead of an ambulance, but I took her home that day, and she never was able to return to school again. She was admitted to the hospital that night. And the attending from before, a miracle worker, collaborated with the doctor in Cleveland and without using the word “PANDAS” got IVIG, steroids, and antibiotics approved. And it was a surreal feeling to watch antibodies donated by others enter my child’s body, doing for her what I as her mother couldn’t, but I watched light come back into her eyes. The gains were huge. She was alert. She was independent. She was focused. And on cloud 9, we took our girl home.
But it didn’t last. Soon letters started to fall out of Gracie’s speech. And curiously, the same letters she couldn’t pronounce no longer appeared in her writing. Her appearance became disheveled. Her temper unpredictable. Gracie, who’d always loved being a big sister suddenly became mean to her brothers. Her empathy for others, a trait that previously characterized her, was gone. Her body ached. Her words were often garbled, and her memory was gone. She was still plagued by exhaustion, sleeping up to 18 hours per day. And at night, though she was sound asleep, I’d find her sitting straight up in bed, laughing.
Gracie was again in the hospital for a seizure, something that had become too normal to us. And looking at another EEG that showed nothing the attending doctor concluded “there must be nothing wrong.” The symptoms he explained were her body’s response to trauma. I fell speechless. What had I allowed to happen to my girl? But the psychiatrist who evaluated her wasn’t convinced. Those who were brave enough to look further are why she’s still here fighting.
Gracie had never been an artist. But with this mysterious illness had come the ability to draw anything she looked at, exact. And one day, she was drawing the characters from Beauty and the Beast. And for the clock, Cogsworth, both his hands were on one side. The psychiatrist asked her to number it. And the numbers too, were all written on one side. Proof he said, “There was nothing psychiatric about this.”
Through the nights she wasn’t sleeping, I pored over medical journals. I searched Google. I begged friends who were in school to access and share published articles from medical journals with me. I e-mailed every doctor who’d ever published an article on PANDAS. And hunted down parents of children who had beat this demon. And one day, holding piles of journal articles, I posed the question: “Could this diagnosis we’ve danced around have turned to encephalitis?” And her neurologist, who’d never been anything but wonderful said, “Let’s ask the experts.” And we traveled to the Children’s Hospital of Philadelphia.
Initially, they told me she wasn’t severe enough to have it. She wasn’t lifeless, yet. I countered – she’d had exactly half the treatment protocol for Autoimmune Encephalitis (AE), because of PANDAS. And she’d been going to three kinds of therapy, daily. And, probably most telling – treatment helped. Empirically they said “yes, this is AE.” And they recommended a treatment protocol of IVIG and a second line action, either plasmapheresis or chemo to be carried out back home.
We were trying to clear the red tape for treatment as Gracie deteriorated. She was sleeping upwards of 20 hours per day and hallucinating when she was awake. Then one night she went into a seizure that lasted nearly 3 hours. Again, she was admitted. After, she couldn’t swallow. A feeding tube was placed. I begged for someone to help my girl. And they ran IVIG. And again, it was magic. Light came back into her eyes. A few doctors “weren’t convinced.” They again told me “this is in her head.” Psychiatry still said “no, this is neurologic.” We sought another expert opinion, this time from Boston Children’s. They too agreed it looked like AE. And gave their recommendations to treat.
Our local hospital agreed to IVIG every 3 weeks, but because it’s donor antibodies, the results weren’t predictable. And due to side effects, it was discontinued. From that day forward, I watched my daughter deteriorate. Strange things happened. Her hair fell out. Alopecia, dermatology said. Characteristic of an autoimmune illness. She couldn’t talk. “Selective mutism,” they countered. She was choosing this. The hospital-maintained Gracie was crazy. The seizures worsened. And she was in and out of the hospital. Never with any answers.
By July, she was back admitted, and this time we were not leaving without answers. The seizures were so severe, and so long, she was choking on her own drool as it pooled in her mouth. Three times I thought I’d watch my daughter take her last breath. And I never wanted to feel that kind of hurt again. Neurology denied her to their service. Gracie’s doctors believed there was a problem, but the department didn’t. She was admitted to general pediatrics. And the doctor who came on board proposed that maybe she had PANDAS, and it became AE. But she questioned, did it matter what we called it, if it was treated? And the answer was a resounding “no.” And she ordered plasmapheresis.
Plasmapheresis meant an inter-jugular tube being placed. This was a line in her neck that would allow her plasma to be taken out and replaced with someone else’s. We talked risks. And I begged them to do it. She deserved the chance for recovery. Her doctors agreed. We were set to move forward when neurology, the department who said neither PANDAS nor seronegative AE existed, moved to shut it down. They said “since it was a neurologic condition,” they should be consulted. There was a lot of back and forth behind the scenes, but the treatment moved forward.
During this stay, a segment aired on the nationally syndicated program, 20/20, focusing on PANDAS, and the fight for care. And I learned there were more parents, fighting the same fight, while their kids suffered. And thankfully I found some of those parents, and through the information we exchanged, a ray of hope.
At first, we saw big gains with plasmapheresis. Then, arguably in error, they treated a headache with morphine and again, her words were gone. After nearly a month in the hospital, Gracie came home. She wasn’t talking, but some symptoms had resolved. There was more that could be done. I knew that from her doctors, and the journeys of other children. Plasmapheresis was never intended to work in isolation, but our hospital was done.
Again, I feverishly researched the next steps. I talked to doctors in Arizona, Texas, and Florida. And I found treatment protocols published in reputable medical journals. Gracie needed chemo, to target the problem cells and IVIG to replace hers. I took these ideas to the Cleveland Clinic and a collaboration begun. But before that could go anywhere, Gracie again slipped away. After “seizing” for hours, she appeared catatonic.
I was terrified, but couldn’t take her to the local hospital, because they didn’t believe she was ill. She was choking on her own spit and needed help, desperately. I made the decision to drive her nearly two hours from where I was for care. And by the grace of god, she made it. And when I carried her into the ER, they rushed her into their trauma space, administered rescue meds for seizures and like that, she was alert. I was infuriated nobody had done it sooner but relieved something worked.
The weeks to follow were a blur. I was tired and losing the fight. We had filed a complaint with our hospital, requesting they remove the doctor who refused to accept Gracie’s diagnosis from her care. And after six weeks in limbo over that, it was denied. I didn’t know her next steps, but I knew our community hospital could not, or rather would not offer the care she needed. And I swore we’d never set foot in their facility again.
But again, as I should have learned, plans mean nothing. And I found her lifeless one morning. And was terrified she wouldn’t make it to Columbus. So, I carried her in, saying nothing more than “I think she’s having a seizure,” until she was stabilized. No parent should ever have to worry their child’s diagnosis is so controversial a hospital will leave their child for dead, but with PANDAS and AE, you do. It took several rounds of rescue meds, but Gracie came back around. And then the question arose. She needed to be admitted, but to what service? Neurology wouldn’t accept her, though her neurologist was willing to oversee treatment. And the hospital was hell bent on doing nothing. So, what was next? The answer was again an admission to general pediatrics, and a transfer.
Gracie reached Nationwide Children’s two years after the onset of her symptoms. And as close to the end as I’d seen her. And with an acceptance of her diagnosis in two days, they began treatment and had her on the road to recovery.
To date, under their care, she’s had two rounds of chemotherapy and one of IVIG. She’ll return for another next week. The seizures that once plagued her daily are under control. She’s talking and swallowing on her own. Though she has a G tube for nutrition she is no longer reliant on it. Her therapy team sees HUGE gains and there is shared hope for a positive outcome.
When I look at my girl, and the road she’s traveled, I am overwhelmingly thankful for the doctors who fought so hard for her but am horrified to think how differently things could have attended. A childhood illness, one experienced by nearly everybody, turned her world upside down. And one physician’s refusal to recognize it nearly took her from me. Strep won’t turn to PANDAS for every child. But if it’s not recognized it will become deadly for those whom it does. And that’s a hurt no parent should ever come to know.