Our health journey with Sophia started in October 2017.  Sophia was 7, Katy had just turned 2 and we were living in our house in Oswego.  We let Sophia fall asleep on the couch that night, as we often did, and about 45 minutes later Sophia had her first seizure.  As any parent would be, Eric and I were terrified. The seizure stopped before the paramedics arrived but Sophia was hard to wake.  We later learned this is normal. We called the paramedics and she was brought to Rush Copley hospital in Aurora. They did some blood work and a CT scan and everything came back clear and normal. We followed up with her pediatrician about a week later and he explained to us that in children, seizures are not totally abnormal.  She could never have a seizure again, or if she does have more, she may grow out of them. He explained that the seizures were not harmful to her brain and are often caused by lack of or poor sleep or during an illness, especially one with fever. That night Sophia had her second seizure. Between then and January 6, 2018 Sophia had approximately 14 seizures.  All while sleeping. She never remembered them when she woke up. She saw a neurologist who did an MRI and an EEG. Both were normal. Due to the number of seizures Sophia had had, however, the neurologist did put her on anti-seizure medication and she has not had a seizure since.

The story continues with her behavior.  Once her seizures began, Sophia started to have some very extreme, uncontrollable fits or tantrums.  They were violent, forcing Eric and I to hold her down so she would not hurt herself or others. She would scream bloody murder, say hateful things, and curse.  It took everything Eric and I had to not lose our own tempers with her, but honestly, there were times when we did. We started her in therapy in January of 2018.  We also discussed these behaviors with her neurologist. He said it is not uncommon to have behavioral changes when a child has seizures. We did anything we could think of and anything that was suggested to us to help Sophiaś behavior including positive reinforcement, negative reinforcement, ignoring the behaviors, calm down strategies, behavior charts, etc.  

Once Sophia started her seizure medication we saw a reprieve in her behavior a few weeks later.  Towards the end of the school year and into summer, things ramped up again. Eric decided to take videos of a few of her fits and we showed them to her therapist who recommended we see a psychiatrist.  We made an appointment as soon as possible. At first, the psychiatrist increased her seizure medication explaining that this same medication is used for psychiatric help with children. A few days later Eric and I had to make one of the hardest decisions of our lives.  We sent Sophia to the behavioral program at Mercy hospital in Aurora where she did a number of days as an outpatient as well as inpatient. We felt we were failing our daughter.

Shortly after the hospital stay, third grade started for Sophia.  With the start of school came more differences in our daughter. Her schooling journey up until this point was wonderful.  Her teacher all loved her, she was a hard worker, and had many friends. The reports coming home from her teacher were that she was not finishing her work, she was distracted easily, she was having trouble with friends, etc.  As most of you know, she has also been doing martial arts (Taekwondo and Mixed Martial Arts) since she was about 4; after asking her coach, he said he noticed that while she was still understanding the material, she was slowing down.  We also saw her swim times getting slower instead of faster. At times it would seem that it would take her longer to get a thought out.

We brought our concerns to one of the pediatricans in the practice we have been with since Sophia was born.  We were less than happy with his evaluation. He felt that Sophia was acting out because Eric and I were net setting enough boundaries and limits, we were not punishing her or following through with punishments.  He said that Sophia was in total control and was acting out on purpose. He said she was fully capable of doing her work in school but was choosing not to. As he was saying this, Sophia was obviously getting upset in the office.  It turned into a full blown fit with the doctor stating, as he walked out the door, if I were you, I would take away her iPod forever. Needless to say we were disheartened.

In the time since her stay in the hospital, Sophia was also put on two more medications.  One to help with her explosions, and an anti-depressant/anti-anxiety. At this point, we were still not given any diagnosis and were told that it is hard to diagnosis such young kids with mental/emotional disorders.  We had an evaluation done for ADHD and were told that she did not have that.

She continued to have issues at school, and while they were less often, she still had fits at home.  The team at her school, along with Eric and I, decided to have Sophia evaluated for an IEP to see if they would be able to start any supports for her at school.  In the mean time, Eric and I decided we wanted to go back to the pediatrician, but to see a different doctor, one who we were told when we first chose this group is the one to see if there is a mystery illness.  This time we came prepared. We wrote down all the things we were seeing and the observations we were getting from others. We also decided that we wanted to speak to the doctor without Sophia in the room as that can be triggering for her.  He decided that the next step was to have Sophia do neuro-psych testing. This was decided the day after Christmas.

The Friday following New Years, Sophia was favoring her left side (her non-dominant side) and would also hold her right arm at strange angles.  There wasn't any pain, but she wasn't using it. She would grab her chicken nugget with her left hand and dip it in her catsup and her right arm would just dangle.  She could use it if reminded to. She was weak on her right side, confused, difficulty talking, sleepiness, walking with an abnormal gait, and was just not herself.

We took her to the pediatrician and were sent to Edward ER for a CT scan and blood work.  Those came back normal and we were sent home. We were working to scheduling an MRI and an EEG for her.  We could not get the MRI department to schedule an MRI. She was getting worse on Saturday and Sunday, so we called the pediatrician and went back to Edward ER on Sunday.  They were able to get her into the MRI there. The ER doctor pulled Valerie and I out of the room to tell us that they found something on Sophia's MRI. We got transferred to Lurie's Children's Hospital in Chicago, by ambulance that night.  

Once we got to Lurie's, they did a second MRI, this time with contrast.  (Sophia was a rock star by the way, did not need to be sedated) After some work with the neurology team, Sophia was diagnosed with Acute disseminated encephalomyelitis (ADEM) it is a widespread attack of inflammation in the brain that damages myelin – the protective covering of nerve fibers.  She was treated with several days of intravenous corticosteroids. She should be able to make a full recovery in a shorter period of time. She is going to be on a steroid for about a month.

The neurology team told us that in addition to the ADEM, they found multiple lesions on the frontal portion of her brain.  Sophia had a previous MRI in February 2018, and they appeared there, but faintly. They were much more pronounced in the most recent MRI scans.   They didn’t immediately know what these lesions were.

As we were getting ready to come home, the neurologists came in and told us that they have determined what the lesions were.  They diagnosed her with NMDA encephalitis. It is an autoimmune disease in her brain. This diagnosis explains what has been going on with Sophia since her seizures began.  The most distinctive features of NMDA include prominent psychiatric symptoms, seizures, confusion, processing speed difficulties, word recall, and memory loss. This disease is relatively new - it was discovered in 2005.  This also can explain some of the learning issues that she's been exhibiting this school year.

There is a treatment for NMDA and it is expected to be about a two-year timeline.   What we are hoping will occur is that Sophia will be able to continue treatment using IVIG.  From what we understand, IVIG is a blood product obtained from plasma donation. She's responded well to the first two treatments (no side effects)- the next step of treatment would be some Chemo drugs.  We are hoping to avoid that. Sophia will need to go to Lurie's about once a month to receive the 6 hour IVIG treatments and then follow ups with Neurology and Rheumatology. (Rheumatology is involved because the disease and treatments affect Sophias immune system)  We are hoping to get those on the same day - so she'll miss one day of school a month, instead of a day and a half.

Yesterday, we had a follow up with the pediatrician that saw Sophia when she was first exhibiting the right side weakness and she felt that there is already an improvement.  Today, Sophia was evaluated by a physical therapist. It was decided that she will receive PT twice a week. She will also return to school tomorrow (Yay!). She will return going half days, even through next week.  

Sophia came home on a number of different medications including steriods, and some medicines to help her sleep.  She will continue to be on her seizure and psych meds as we continue treatment for NMDA. The steriods are affecting her sleep.  She will wake up at some point in the middle of the night for a few hours. Sometimes will go back to sleep for a bit, sometimes not.  She has been napping during the day since she came home on Monday night. We do see her tire throughout the day, and as she fatigues, her symptoms do come back/get worse.  

Her school is being very supportive.  They are happy that she is returning and are more than willing to set up things that will help her in school.  The team at Lurie’s was exceptional. Every nurse and doctor we came across was attentive, kind and ensured that we understood what was going on every step of the way.  They were happy to answer any question we had, and made sure to make me feel better when I got the acronym wrong for the first diagnosis and scared all my medical friends that she had something worse.

We are blessed to have people around us to help.  My Mom canceled her trip back to Florida to help us and while she will need to return home next week to put her life in order, she is going to come back until we get a routine going and Sophia is back in school full time.  Eric’s Dad drove to and from Lurie’s countless times bringing items from home and many, many burritos. Joe came and shoveled our driveway, Tim carried all my Christmas decorations to the basement, and Jenn packed up bags for us so I didn’t have to ask my father in law to go in my underwear drawer.  We are trying to keep our humor about us, and if you ask Sophia how she feels, her answer is always, “fine.” The worst part for her was everytime she had to get poked with a needle. It’s her own fault though because she pulled our her IV…...twice.

Next up is an Occupational Therapy evaluation next week and a follow up MRI in February as well as follow ups with the Neurologist and Rheumatologist.  Swimming is out for the foreseeable future, but the PT said she could slowly reintroduce herself into her martial arts. Thank you for caring about our family and sending all your good thoughts and prayers.  In my heart, my Dad had something to do with getting all her symptoms figured out once he got himself settled with the big guy upstairs.