I am bursting with blog posts and it’s been a long while since I have posted to Hemi Heroes. As a mom, I tend to put my needs, wants, desires, and dreams last—way behind everyone else in the family, even the dogs!
Mothers are natural care takers and would rather make sure their family had everything they needed before they even take a potty break! So in the spirit of my dedication to everyone else, I just neglected my own goals and priorities, yet again. Today, I’ve reminded myself that I can’t get lost in the rabbit hole of being a special needs parent and sometimes we need to pause, take a deep breath, and hit reload for a fresh outlook.
Towards the end of last year, the Universe sent me signs that were hard to ignore. I was done feeling sorry for myself, found inspiration, was motivated to accomplish my goals, and achieve my dreams. I felt a rush of hope and contentment and I had felt this before, but something else always pops up to knock me off this fabulous feeling. Lucas, my youngest child, not only has Hemiplegia, but Epilepsy as well. He handles everything so gracefully, but life is always an uphill battle for him, fighting on two fronts of disabling conditions. So what knocked me off my high this time? Well, it was a new addition to his epilepsy. First, I’ll fill you in with Lucas’ epilepsy back story to put things into perspective.
When Lucas was just over a year old, we flew to San Francisco to meet with Dr. Heather Fullerton, she is the Director of the UCSF Pediatric Stroke and Cerebrovascular Disease Center at Benioff Children’s Hospital and a nationally recognized expert in pediatric stroke. At the time, we were still seeking hope and answers and part of that was Lucas’ risk of seizures. She had said that based on his MRI and where the stroke damage occurred, he had a 30% chance of having seizures, but that if he hadn’t had any in the first 12 months of his life, then his risk went down significantly. Well at the time, that was fabulous news and my husband and I were able to breathe a collective sigh of relief. Lucas had been seizure free thus far and 14 months old.
At age 3, he had his first seizure. We weren’t sure it really was a seizure; he woke up in the morning and stared off, turned pale, and started gagging and coughing up foamy saliva. He also seemed lethargic, but it was so quick and it really didn’t seem like a seizure. We took him to the emergency room and they said he looked fine and to follow up with his neurologist. Lucas’ neurologist told us to keep an eye out for more abnormal activity. The next “seizure-like” episode happened exactly a year later, the same symptoms and once again questioning if it was really a seizure. This happened once more exactly another year later. By the beginning of 2013, Lucas had caught the flu and he began having complex partial seizures regularly, about 2-3 per week. An EEG that April confirmed his epilepsy diagnosis and he began taking a drug called Trileptal (oxcarbazepine) because it was the most effective in treating complex-partial seizures and had the least side effects in children compared to other anti-epileptic drugs.
At first Lucas had no side effects, but he still had seizures. He began to gain weight, which was a positive outcome because he was severely underweight like many kids with cerebral palsy. We also began to notice that his speech had slowed down and he began slurring his words. He was sleeping in a lot and the seizures were still occurring. He also started having different kinds of seizures and these occurrences never happened prior to medicating with Trileptal.
In December of 2013, I made the decision to slowly wean him off Trileptal, I felt it did more harm than good. I had just lowered his dosage a tiny bit and looking back it most likely wasn’t the trigger, but Lucas woke up one morning having a large tonic-clonic (aka Grand Mal) seizure. To say it was scary would be an understatement! My hubby was traveling and I had to experience it all alone. Lucas was shaking all over, he was groaning and making these gurgling sounds of pain, his heart was racing and his breathing was slowing down, he turned pale and then began to turn blue. I knew he would stop breathing and with trembling hands I called 911. It felt like an eternity, but the paramedics arrived just as the seizure had stopped. I feel sick to my stomach even thinking about the details of that morning and thankfully Lucas has no memory of any of his seizures, but it was one of the scariest moments of my life. Of course, I was scared and continued to medicate him with Trileptal, blinded by the fear of losing my boy. We had to keep increasing the dosage and the seizures stopped, but the side effects began to get worse. He became very hyperactive, restless at night, he couldn’t remember simple things like if he had been to a place the day before, and he couldn’t retain anything he was learning. His quality of life was on a downward slope. I then heard the term “ESES” on a Facebook group for parents with hemiplegic children and it sounded a lot like Lucas could have this rare epileptic syndrome.
ESES is an acronym for Electrical status epilepticus during slow-wave sleep (pronounced “ee-sus”) and it is a rare epilepsy syndrome. In children with epilepsy, less than 1% have ESES. It usually appears in mid-childhood between the ages of 6-9 years old. It is also called “continuous spike-wave of slow sleep syndrome” and the cause of this syndrome is not known, nor is there a direct treatment for it with a 100% cure rate.
According to an excerpt from http://www.epilepsy.org/uk, “the first sign of any problem is usually that the rate of a child’s learning appears to slow significantly. Many of the children begin to have problems with either the understanding of speech and language (this is called receptive dysphasia) or expressing their own thoughts (expressive dysphasia) in the form of spoken language.
Epilepsy develops and different kinds of seizures can happen either during the day or during sleep. Many children will have absence (blank) seizures, some myoclonic (jerk) seizures and others will have partial motor seizure (seizures involving jerks of one side of the body only) particularly during the night. Some children will not have any seizures during the night.
However, despite the fact that sleep patterns are often severely disturbed due to the seizures, children with ESESS usually wake the next morning feeling quite refreshed.”
This syndrome is frightening because patients with ESES are most likely to die in their sleep from SUDEP (Sudden Unexpected Death in Epilepsy) and ESES causes severe regression cognitively. The irony is that this syndrome usually clears up in children by the teen years, but the damage would be vast and irreversible to a child’s cognition and intelligence.
So in June 2015, we checked into Hotel Rady (our awesome children’s hospital in San Diego) for a 5-day/24 hour in-patient EEG. After the first night, his epileptologist said that his EEG showed it wasn’t quite ESES yet, but was heading in that direction. His doctor added another seizure medicine called Onfi (Clobazam), which is a “benzo” aka Benzodiazepine, in the same class of drugs as Valium.
Onfi has showed promise in a good percentage of research participants that had been treated with it for ESES, so it was worth a shot. Lucas seemed better almost immediately! He had never slept so soundly, he was seizure free, and he seemed to be learning better. I felt we had finally got an answer to the gnawing feeling that something else was wrong with our son. He seemed to build tolerance to the drug because he began being restless again in his sleep, so we gradually raised the dosage. It always helped at first, but by October he started having staring seizures, a few complex-partial seizures, and the worst part was that he was a walking zombie. Quality of life was zero; he was slurring his words, clumsy, tripping over his own feet, and sleeping a lot. I knew something was severely wrong and we got in immediately to see our neurologist and Lucas had another 24 hour EEG right before Christmas. Lucas’ neurologist came in the next morning and said that he had full blown ESES. She told me that she thought I was just being a worrisome mother and she fully believed she would come in and tell us that it was nothing, but she was so sorry to say that it was indeed ESES. At least, my maternal instinct and special needs mom super powers were sadly on par again.
So what was the new course of action? Well, he had failed two medications and ESES has not been well researched because it’s so rare, but there were a few courses of action to take treatment wise. Just like seizure meds, every patient reacts so differently to treatment and it either works or it doesn’t. Since Onfi was the first course of action and failed, the next step would be high-dose steroids. Prednisone was what she prescribed and she warned me of the significant side effects he could have, but most were uncommon in children. With Christmas just three days away and feeling so overwhelmed, I needed to do my own research. We began to wean him off Trileptal immediately and it is a drug that is known to cause or exacerbate ESES in some patients. I had to wonder if that was the cause of all of this. The Trileptal wean was awful for him, he stopped eating, lost weight, was pale, was completely not himself. His last dose of this drug that caused him so much harm was on New Year’s Eve.
I really wanted to avoid steroids and repeat an EEG in a few weeks just with the Trileptal out of his sytem, but I knew that Lucas’ neurologist would think I was being difficult and our medical insurance company probably wouldn’t approve multiple EEG hospital admissions. I spent hours reading medical journals and university research studies on ESES and our best hope was steroid therapy. If the steroids fail, he would be facing the possibility of brain surgery, specifically a Hemispherectomy.
I was so hesitant because Lucas is immune deficient and steroids suppress immunity so much that a common cold could land him in the hospital or worse. January is in the thick of cold and flu season, so we pulled Lucas out of his class days. (Lucas attends a charter school that is a hybrid-homeschool program, so he only attends class 2 days per week.) His school was understanding and we started Prednisone at 60mg/day on January 4th.
This dose is super high for an adult, let alone a child. I anxiously awaited the side effects, but the worst side effects at this point were trouble sleeping and rapid weight gain. He was skinny to begin with and to-date he has gained just over 10 pounds in 8 weeks and his clothes are getting snug! We added Melatonin to help him fall asleep and that has been a success as well. We tapered down to 40 mg/day after two weeks, and then 30 mg/day after another two weeks.
On February 16th, we were back at Rady for another EEG and this result was so very important, either the Prednisone was working (it was only a 50/50 chance) or it was not and we would have to pursue surgery. I prayed, begged, and pleaded with God, the Universe, and all of Lucas’ angels to let this drug work. The news was good and the Prednisone was working! The ESES was gone, but there is still abnormal activity only on the left side (the damaged side), but none on the healthy right side. This was great news because it was a good indication that he could be a surgical candidate in case we needed to pursue that route in the future.
We tapered down the steroids again to 20/mg per day and last week we met with his neurologist to discuss the full EEG results. She showed us the December EEG vs. the February EEG and they were like night and day. One was ugly and jagged with spikes all over it and his last EEG was calm and almost flat line in comparison. I was cautiously and optimistically happy! Why was I not jumping up and down with excitement, you ask? Once he tapers off the medication completely, there is a significant chance of relapse and it’s not an option to have a child on steroids for an extended period of time.
I am happy to be able to pause and thank God, everyone that prayed for Lucas, and of course his brilliant and caring doctor for this small victory. The big bad wolf of ESES has been scared away for now! Lucas is doing well too-he’s seizure free, alert, better memory retention, and seems more like himself before epilepsy entered our lives. I’m thankful to have more answers and an action plan for his treatment and for now we continue enjoying each beautiful day bestowed upon us.