September 20, 2018 | Kayla DeMay
I am the mother of three beautiful children, Dakota my eldest and only son is 6, Lena my middle child is 4, and Jenna my youngest is 6 months. My middle child, Lena had some very rocky weeks with her behaviors and emotions. I thought she was going through middle child syndrome. Her pediatrician told me when I was pregnant that one or both of my older kids could go through a regression in reaction to the new baby.
August 1st Lena just seemed to lose interest in the baby altogether when all along she just loved her to pieces. I noticed it but it was very subtle, the changes in her moods. August 10th, Lena comes to me bawling her eyes out, saying to me “Mommy it’s going to FLOOD! We’re all going to get flooded away and die in the WATER!” A little overreaction I thought, but I just calmed her down and assured her that we live on a hill, we will not get flooded out of our house, and that everyone will live, it’s just a little rain. We sit in the big picture window in the Livingroom and watch storms together all the time and she loves it.
August 14th, the entire Southern Tier of our state, NY, Flooded. Our house is on a hill in the northern most county, so we were fine, and I wasn’t wrong but still… I kept the tv off and the flooding footage on my phone out of her sight.
August 16th, Lena and Dakota’s friends Chloe, Alexandria, and Kingsley came over to play. Lena loves playing with her friends. It was loud, exciting and fun. At 1pm, in the middle of all that commotion, Lena fed her fish, dropped the fish food all over the floor, walked away from everybody, fell face first on her brother’s bed and took a nap. I dismissed it as just playing too hard.
August 17th, Lena started to Really act weird. Usually a very independent girl but that day she would stare at her dresser but not get dressed. Stare at her cereal but not eat it. Sit on the toilet but not pee and then pee her pants instead. I thought wow she really is going through a regression. So, I dressed her, I fed her, I helped her to and from the bathroom. I gave her more love, more attention, tried to assure her that she’ll always be my little girl. I WATCHED her, and I told myself if it she’s not any better tomorrow, I’m making an appointment to get her seen.
August 18th, 11am. Lena stared into the TV with tears in her eyes for a full ten minutes. I asked her what was wrong and in a strange monotone voice she said, “I’M SICK”. Then, after weeks of being very somber and anxious, she JUMPED off the chair, ran around the house laughing hysterically, also in weird monotone, and saying very outlandish repetitive things. She complained that the tv was stealing her toys. She was carrying on an argument with her brother, but he was not there. She had super over the top happy moments followed by instant anguish and whines. I called the pediatrician and said, “My kid is NOT acting like my kid, I think she’s hallucinating, what do I do?” and they told me “Take her to the Pediatric ED at Strong Memorial Hospital.” I packed up Jenna and Lena, picked up my Mom and headed out. We got Lena a happy meal, she was hungry she said, and she babbled in that voice and laughed in that laugh the entire way. She smacked her lips and jerked her feet while she talked. Her facial expressions did not match the things she was saying and doing. Forty minutes later we’re at the gas station around the block from the hospital. I checked on her she was eating and talking nonsense but calling me Mom and calling her Grandmother, Grandma, she was responding. Five minutes later we’re at the ER, and I go to get her out of the car and she’s slumped over Jenna’s car seat, drooling, eyes wide open and rolling around, blue lips, and red blotchy rash from the nose down. I picked up her limp little body and RAN into the ER screaming “MY BABY’S BLUE!”. She had had the first seizure. She came out of it on her own but continued to hallucinate and ramble. She was admitted.
Twelve hours after admission, she started to scream and throw herself out of bed and tried to run away. She ripped all her leads off and started biting anything near her face. I told her to go to sleep and she said, “I CAN’T”. And she didn’t. She eventually required a caged bed.
A caged bed was required 12 hours after admission to hospital
48 hours after admission, she was still rambling like this and hadn’t slept. Still having violent episodes and screaming episodes but no seizure activity. The first 7 days were a blur of tests, violence and confusion. CT scan, MRI, Spinal Tap, Blood toxicology, Urine toxicology, x-rays, Full Metabolic Panel, EKG, constant EEG monitoring, and an ultrasound of her ovaries, not necessarily in that order. All normal except 40 White Blood Cells in her perfectly clear spinal fluid, and two very small white markers on her MRI in a fluid filled area that’s supposed to be dark. Putting her through a spinal tap tore me apart but it was absolutely necessary because spinal fluid is 100% accurate whereas blood is not. I saw vials of my baby’s spinal fluid in a bag. I will wear her bite marks on my chest for the rest of my life.
Lena Hodge, age 4, August 19th, 2018 – One day after admission to hospital
The 7th day after admission, she started receiving steroid treatments. She was not diagnosed yet, but her neurologist had seen this recently in another patient and felt it was in her best interest to start treatment preemptively. That was 4 days. During that 4 days she went in and out of the ICU for going into continuous seizures and dropping her oxygen levels. She was given Keppra at first and then Phenobarbitol and then back onto Keppra because phenobarbitol made her sleep too hard. She was unresponsive and catatonic while she was awake, and she developed a movement disorder and facial dyskinesis. She stopped speaking altogether.
This picture was taken August 22nd, 3pm. She had a violent thrashing episode in my arms and I held her tighter and she bit my chest. It was a major reality check for me to find out that I can’t even handle holding my daughter. I didn’t hold her again for over a week because I didn’t want either of us to get hurt and it broke my heart.
The 11th day after admission, she started receiving IVIG, also for 4 days. During these 4 days, her seizures ceased, and her episodes began to calm down. The movement disorder and the facial dyskinesis continued, but she was keeping her oxygen up, so she got moved back out of the ICU to the regular peds unit.
The 14th day after admission, we received a positive result for the blood test for Anti-NMDA Receptor Autoimmune Encephalitis. We were lucky to have found out that way because as the disease progresses the antibodies leave the blood and enter the spinal fluid giving a false negative result. We were on the right track with treatment and she was starting to respond. She would react to voices, light changes and sound with her eyes but didn’t have motor control.
Over the course of the third week, she burst back to life. The movement disorder and facial dyskinesis went away. She started to show awareness of her body again, and especially the NG tube. She started to show emotions, like crying when it was time for me to go, smiling at me, snuggling with me. She sat right up on her own and struggled at first to balance but it came back so quickly. Her agitation eased back a lot to where she only got really upset in the evening before meds and bed.
Over the course of the last week, she has been walking, trying to run, balancing, using fine motor skills to manipulate toys. She has been playing catch with a ball and coloring. She likes to put toys inside other toys and she loves playing with water. She looks out the window at the helicopter pad and watches them land and take off. She can even aim and shoot stuffed animals with a nurf gun. We brought her brother to visit and she cheered right up and gave him a long hug and they played like old times. She laughed out loud and she even said her First Word since she went mute. Her brother asked her a question and she said “Yea!” out of the clear blue.
Today, September 18th, 2018, marks a full calendar month since Lena’s admission. She’s going to receive her first infusion of Rituximab this morning. She will receive another dose in 2 weeks. All the parents I’ve connected with whose younger children went through this highly recommend it and regard it as the major turning point that made their kids get better. Although she has shown amazing improvement, she hasn’t been able to eat or speak. I’ve been told that’s usually one of the last things to come back. Her team of neurologists agree that it’s necessary and better to receive Rituxan sooner than later for the best possible recovery. I’m putting my faith in her team and in the living experiences of others and we’re taking the dive. I don’t know what tomorrow holds but I’m grateful for this day.
In one retrospective study, anti-NMDAR encephalitis accounted for 1% of all admissions of young adults to an intensive care unit. anti-NMDAR encephalitis is most frequently recognized on clinical grounds. Anti-NMDAR encephalitis affects predominantly children and young adults (median age, 21 years), with a predominance of cases in females (4:1) that becomes less evident after the age of 45 years. Up to 58% of affected young female patients have an ovarian teratoma (extragonadal teratomas are a rare cause); in men and children, the association with tumors is less frequent. Young children typically present with insomnia, seizures, abnormal movements, or a change in behavior such as irritability, temper tantrums, agitation, and reduction of verbal output. Teenagers and adults more often present with psychiatric symptoms, including agitation, hallucinations, delusions, and catatonia, which may lead to hospital admission for psychosis. The disease progresses in a period of days or weeks to include reduction of speech, memory deficit, orofacial and limb dyskinesias, seizures, decreased level of consciousness, and autonomic instability manifested as excess salivation, hyperthermia, fluctuations of blood pressure, tachycardia, or central hypoventilation. Bradycardia and cardiac pauses are infrequent but require a temporary pacemaker in some patients. One month after disease onset, regardless of the symptoms at presentation, most children and adults have a syndrome that combines several of the above-mentioned symptoms. (1)
1. Antibody-Mediated Encephalitis Josep Dalmau, M.D., Ph.D., and Francesc Graus, M.D., Ph.D
International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.
Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles.