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Finding a herd
A zebra named Henry
"Mommy, the baby isn't moving."
Words no parenting class can prepare you for. As I rushed to my son’s aid, I would find him face down, unresponsive behind the couch. His skin cold to the touch, his eyes glazed over, drool dripping from his mouth. Alive, but my little boy was not there, he was not the same as he was just hours before.
You can learn CPR, the Heimlich maneuver, heck, you can even graduate medical school, but NOTHING can prepare you to find your own child face down, unresponsive and in a catatonic state after what was an apparent seizure.
Henry is in a bed at Texas Children's Hospital, scared, as a team of doctors hover around him. Most of the time when he looked at me, his mom, I could tell he had no idea who I was. My heart was breaking. I thought I was losing my little boy. “Ma'am, we are not completely sure what's going on with your son, but we think he has neuroblastoma, cancer.” I knew something was wrong, but in some extremely bizarre way, hearing that my child may have cancer wasn't as shattering as you would have thought. It's modern day I reassured myself, doctors cure cancer all the time. In the herd, we were still just a horse. We're going to get through this I whispered to him. He was whisked away for more tests. Results came pouring in. It wasn’t cancer. Toxicology, normal. Genetics, normal. Henry was struggling to walk, talk, swallow and continuously having seizures. Yet, with all the test they ran, nothing. At this point we knew he had cerebellar ataxia, but unfortunately ataxia is just symptomatic of something greater and that something greater was still undiagnosed. Do you know what it's like to go to the car wash and as you leave it begins to rain? Times that emotion by a million. That's what it's like going home with a child from the hospital without a diagnosis.
Although we didn't have a diagnosis, we did have a receptive, and willing neurologist, Dr. Garrett Burris,
who could tell this was an inflammatory process and began IVIG treatments almost immediately. I did not know then, but it most definitely saved Henry’s life.
Fast forward again.
My son is still having sporadic episodes in between IVIG treatments where he is struggling to walk, talk and swallow. Having extreme psychotic episodes, hallucinations and seizures. There were days he didn't know who I was and was certain I was trying to hurt him. Little did I know at that time, his body was at war with his brain. Neurology has referred us to rheumatology. In walks a tall doctor with a lot of meticulous questions. So meticulous, that I felt like I repeated my son's life story, over and over again, and yet he was intrigued by every detail. No scans were done that day, no blood panels were run, yet still for the first time we were given a name. This doctor, who I didn't know at the time, was going to change our lives. Dr. Eyal Muscal looked me point blank in the face and said I believe your son has autoimmune encephalitis (AE). En-cepha-who-dis? Encephalitis. An encephalopathy in the brain. AE is a serious medical condition in which the immune system disrupts the brain, impairing function. With rapid diagnosis and appropriate treatment, many patients recover most or all functions. However, not all patients experience full recovery; with approximately 6 percent mortality and other patients who never regain significant brain and/or bodily functions.
Just like that, my child was just diagnosed with a rare disease. And Dr. Muscal was right. Blood tests later confirmed dangerously high antibodies; Henry has AE. We were no longer a horse in the herd. Henry is considered a zebra, which is American medical slang for arriving at an exotic medical diagnosis when a more commonplace explanation is likely.
Dr. Muscal quickly formulated a treatment plan which involved frequent infusions of different medications to try and reset the autoimmune dysfunction.
Henry has endured two surgeries, had a pheresis catheter placed in his chest connected to his heart, a port, six rounds of plasmapheresis where they remove 80 percent of his blood each time and filtered it, close to 50 rounds of IVIG, MRIs, lumbar punctures, EEGs and multiple doses of chemotherapies. There's no cure for AE, just bandages. We regain him and his skills, and lose him all over again. He is better than the beginning, but still struggles with symptoms from this disease. Our Texas Children's team is striving for remission, and one day, with their help, he will achieve it.
Our lives were forever changed, and we were all alone. There’s local support for cancer, NICU and diabetes. There was nothing for us. We were a lonely zebra without a herd. Or so we thought.
Little did I know that in the same hospital, under the care of the same doctor, a family with a little zebra just days younger than mine, were just as lonely.
A Zebra named Hadley …
You always hear phrases like, “Your life can change in the blink of an eye,” or “You’re never guaranteed tomorrow,” but those phrases always refer to someone else. Not my family, not my kids, not my sweet, perfectly healthy, spunky 3-year-old little girl.
We woke up to what we thought was a normal Monday and found ourselves by the day's end standing in the Texas Children's emergency room with our daughter, Hadley, in a coma, near death. It was like a wicked nightmare. We stood there, my husband I, holding hands in the corner of the trauma room watching our lifeless daughter on the table. We stood in shock watching the doctors buzz around the room in perfect precision. We can’t lose her, I told my husband.
How did this happen? How did we end up here?
I laid down next to my perfectly healthy daughter on Sunday evening and didn't wake up to the same girl the next day. She woke up complaining of a headache and soon started vomiting. Her heart was racing but we still assumed it was a stomach virus. By early afternoon, she began hallucinating and reaching for things that weren’t there.
She’s just dehydrated right? Let’s get her some juice and watch her while we wait to hear from the pediatrician. Soon, she was unable to focus her eyes and had trouble seeing. We knew something was really, really wrong.
We loaded her up in the car and she hallucinated the whole way to the hospital. By the time we got her to the emergency room she no longer recognized who we were. I could see by the look in the nurse's eyes this wasn’t a run-of-the-mill stomach virus.
“Ma’am, her CT scan is normal but her white cell count is 35,000. We think your daughter is septic and in acute renal failure, we have to get her to Texas Children’s Hospital.”
My world stopped spinning. That moment is etched in our minds. The fear. The panic. The shock. It was all so much.
As the hours passed, she continued to deteriorate. Nothing prepares a mother for riding in an ambulance for what seems like an eternity, watching your daughter fade away. We were losing our girl by the minute to some unknown beast and we felt helpless. She arrived at Texas Children’s near death.
Once we arrived, we were whisked away to a trauma room and then immediately placed in the pediatric intensive care unit (PICU). With further testing we were told she was not septic and not in renal failure. We had no answers; we were fighting an unknown beast and it was winning.
She remained in a coma and within two days her brain began to swell. She was no longer able to breathe on her own and we had to say what we feared were our final goodbyes while doctors placed her on a ventilator. After a week-and-a-half she came out of the coma, still unaware of who we were. She was unable to speak, eat or use her left side. Wicked, violent hallucinations set in. She was awake for nearly 72 hours hallucinating and scared. She still had no recognition of who we, her parents, were. We couldn’t comfort her. We became part of her nightmare. She was trapped and we couldn't save her. Our hearts were in a million pieces.
We soon learned at some point during the night, before she got sick, her immune system launched an attack on her brain. After weeks of testing, we were given a name for this beast, AE. We were told this is a lifelong condition and there is no cure. We immediately began treatment with a high dose of steroids.
Hadley spent a month in the hospital and we brought home a very different little girl than the one I fell asleep next to the month before. She had to learn to walk, talk, eat, speak and function again.
We felt so alone. We had never even heard of this disorder and were so scared. Many people were watching Hadley’s story unfold as it happened on a page we had created, Holding Hadley. There, we ran across one other family being treated at the same hospital for the same disorder.
Henry and Hadley were both zebras. Born only a week apart, diagnosed only a month apart, we were no longer alone. We became fast friends on this journey we never wanted to be on. There is no cure for AE. Hadley was in remission for several months before we started seeing her stumble when walking, her heart racing, she began to have slurred speech and hallucinations returned during the night. The beast was back.
We were hospitalized again for a week and have since endured 10 rounds of IVIG. We continue strenuous treatments and Henry and Hadley are able to maintain the same infusion schedule. They have become best friends and have bonded by being rare.
Our lives were flipped upside down, just like that. We went to bed one night and woke up the next morning in a new world. She has since returned to us nearly 100 percent. We fight daily to keep our little girl with us and to keep her immune system in check. We are not guaranteed remission, so we treasure every moment.
We have been battling AE for over a year now. We have seen tremendous progress. God has been so good! We are not the same people we were a year-and-a-half ago. You cannot walk a journey like this one and remain the same.
Strengthen the herd …
Dr. Muscal has been a shining light in a dark tunnel of the unknown with this newer, rare disease. He has prayed with us, cried with us, rejoiced at positive gains with us, advocated for us, made himself available, positive and present. Our hearts are forever grateful for the continuing wrap around care provided by him, he’s not just a doctor to us, he’s family.
As moms, dealing with AE has been hard, really hard. At times, it’s manageable and life almost seems “normal,” and other times it’s completely overwhelming. As zebra moms, we have worked to create a purpose out of our pain because no one should journey this road alone. Together, we created a support group, Texas Children's Autoimmune Encephalitis Encephalopathy families. It's been a way for families to connect, share symptoms, insurance battles, tips and support. Families from out of state, even out of the country, have sought out care at Texas Children’s.
We are painfully aware that we are not promised tomorrow with our children, and as a result, the miracle of the mundane is not lost on us. We have found joy and strength in unexpected places. Being a zebra hasn’t been so bad because we have found our herd. The old saying is true, there is strength in numbers.
For more information about AE, visit aealliance.org
For more information about the support group for AE parents, email beAEaware@gmail.com
This blog was authored by Mandy Seiders and Devon Fults