April 26, 2022 "Sedated 7 Layer MRI"

This day began very early. Rayden could not eat or drink anything due to being sedated for the 7 layer MRI. After shunt series x-rays we went to the surgical floor to check in. I am not sure who was more excited to see the other, Rayden or Mr. Lamont. He was the person that escorted Rayden from his room to surgery/wound vac changes during our 65 day hospital stay in 2021. They developed a special bond. Rayden even colored him a picture and signed his name on it while waiting to be sedated. If you know Rayden, you know that's a special gift because he doesn't enjoy coloring. Mr. Lamont was very excited to receive it as well. It really calmed Rayden's nerves and made him feel at home when so many nurses and child life specialists came to see how their 'little rock star' was doing. A new anesthesiologist soon arrived to talk to us about sedation. He said he was excited to meet Rayden because he couldn't even get down the hall without people talking about how amazing this warrior is. This made Rayden feel special as well. He proceeded to talk to him like an adult. He knew Rayden hates the mask, and fought off 5 nurses one time in the OR. He also knew how extremely difficult it was to start an IV or draw blood from him. He explained that there were only two ways to sedate him, mask or IV, and gave Rayden the choice. He talked about the bad smell and taste of the gas and promised him that if he took really deep breathes then it wouldn't last long. He even said Mom could hold the mask so he wouldn't be so scared. We could see Rayden's wheels turning as he was weighing his options and making a decision. He agreed to try the mask without fighting. After arriving in the sedation room near the MRI, the anesthesiologist did exactly as he promised. He talked so calmly to Rayden reminding him to take super long breaths through his mouth and gave me the mask to hold over his nose and mouth. Rayden drifted off soon while holding my hand. I gave him one last kiss before Michael and I walked to the waiting area. 

Two days later Dr. Lewis emailed me some information. Since Rayden has only had rapid sequence MRIs since birth it is hard to interpret how much has changed. They can't really explain his recent seizures. The MRI revealed Rayden has so much going on his little head. 

1-Massive hydrocephalus treated with VP shunt in the right lateral ventricle. The ventricles appear markedly dysmorphic with an enlarged CSF space between the parietal and occipital lobes possibly reflecting cystic encepahlomalacia (brain damage). Epilepsy is common in people with hydrocephalus.  

2- Missing septum pellucidum, which may cause learning difficulties, behavioral changes, seizures and changes in vision. 

3- Missing Corpus callosum, a bundle of nerves connecting the left and right sides of the brain. Symptoms range from minor intelligence issues to developmental delays and seizures. 

4- Stable Chiari II malformation (need for shunt). Seizures are reported in patients with and without symptoms. 

5- Dolichocephalic (long and narrow) head shape

6- Polymicrogyria (too many small folds) throughout the cerebrum bilaterally (both sides) which tends to cause more severe neurological problems. Symptoms can include recurrent seizures (epilepsy), delayed development, crossed eyes, problems with speech and swallowing, and muscle weakness or paralysis.  

7- The hippocampus on both sides of his brain are small and abnormal appearing. The hippocampus plays a vital role in regulating learning, memory encoding, memory consolidation, and spatial navigation. This part of the brain can frequently cause seizures as well. 

8-Bilateral parietal lobe periventricular white matter abnormality and cystic change, which can damage important pathways within the brain and can cause problems with memory, balance, and walking.  

She wants to do a longer, 24 hour EEG to hopefully capture any abnormal discharges and see where they may be coming from. She will then review it and the recent MRI with one of their pediatric epileptologists to see if they can pinpoint the origin of the seizures. It may take up to a week for the study to be read and then we will meet again. After seeing the MRI report, I honestly don't know how they are ever going to pinpoint the origin. There are at least 6 out of 8 abnormalities in his brain that can cause seizures! 

 

April 23, 2022 "3rd seizure in 3 weeks"

April 23, 2022

It was just a typical Saturday. I was cleaning the house, while Michael, Alex, and Rayden were outside doing things in the yard. They decided to go get some lunch at Mi Casitas, but I stayed home to finish cleaning. Rayden fell asleep on the way back home, so Michael brought him inside to finish his little nap on the couch. I sat in the recliner close to him eating the lunch they brought back for me. Rayden really wanted to help Daddy put the new thermostat on the grill so Alex came in to see if he was still asleep. He noticed Rayden shaking on the couch. I jumped up and saw that his eyes were open, fixed, and fully dilated. He was laying on his side already and his entire body was violently shaking. I stated out loud that it was 2:12pm. Alex ran to the back door to yell for Michael, and grabbed the rescue medication. It felt like hours before it finally turned 2:15 so I could give him the Diazepam. Here again, it did not work immediately. Alex called 911 while I prayed. Rayden stopped breathing, his lips turned blue, around his nose seemed to be turning white, and he was drooling really bad. I began praying out loud and yelling "He's not breathing!" I felt like dying because I didn't know what to do. I couldn't perform CPR because he was still seizing so I just blew in his face praying it would help him breathe. The seizure finally subsided at 2:17. His eyes were glassy and his speech was slurred, but at least it stopped. The Paramedics arrived at 2:35. They wanted to take him to Betsy Johnson Hospital because his eyes were still glassy and he had an elevated heart rate of 120. We decided to keep him home seeing as we went to UNC last week and they just watched him a couple of hours and sent us home. The paramedics sat with us for a while answering questions and reviewing seizure safety. I was so upset that Rayden had stopped breathing and I didn't know what to do. The paramedic said he understood why I blew into Rayden's face because he couldn't imagine how very difficult it must be as a Mom to watch your baby not breathing, but unfortunately, you cannot perform CPR on an active seizing patient. That is the most helpless feeling in the world. 

I messaged his neurologist for advice but don't expect to hear from her until Monday. He goes back to UNC Tuesday for a seven layer MRI. In the meantime, we are all taking turns keeping our eyes on Rayden at all times. 

Dr. Lewis replied before 9am Monday morning. She felt it was strange that he is having increased seizures all of a sudden and is glad the MRI is scheduled for tomorrow. She increased his oxcarbazepine from 6.0 to 7.5 ml twice a day. If the MRI shows that his shunt is ok and he has another seizure then we will push the dose to the maximum and talk about starting another medication. 

April 21, 2022 "Duke and UNC appointments"

We had two appointments today: Duke Neuro-ophthalmology and UNC Pediatric Neurology. 

Dr. Mays El-Dairi, Duke Neuro-ophthalmology, noted some thickening in Rayden's optic nerves. Of course, this was cause for concern, especially after his recent seizures. She started to schedule an MRI, but I told her Rayden was actually seeing his neurologist this afternoon. She was relieved, stating she was sending the neurologist a message about possible shunt failure and she still wanted to see in back in two weeks. Although she noted Rayden's difficulty with depth perception and peripheral vision, she stated no corrective lenses at this time. She reviewed his Formal Visual Assessment results and was thrilled to hear that Rayden is receiving vision therapy at school for his cortical visual impairment. 

Our appointment with Dr. Carolyn Zook Lewis, UNC Pediatric Neurologist, was after lunch. She spent lots of time with us reviewing his history, recent seizure activity, and safety measures. She agreed that a seizure detecting service dog would be a great asset but they are hard to get, and very expensive. 

She introduced the Embrace 2 seizure watch as a better alternative. She said Rayden would be an excellent candidate for it, especially due to his nocturnal seizures. It is a watch type band that detects seizure activity and sends a blue tooth signal to a cell phone that must be with him at all times. The phone will then send an alert message and phone call to all caregivers assigned in the app. Seeing as how Rayden is now sleeping in our bed because we are afraid for him to be alone, we agreed this would be a life changer for us. Dr. Lewis quickly got the ball rolling, writing the prescription and filing the needed paperwork/application for the watch. 

She is hoping this new dosage will do the trick because we are almost at the maximum dosage of his Oxcarbazipine. This means he will need a new medication if he has more seizures. We definitely don't want to change medications because you never know how it will affect you.

She 'translated' the EEG results from last week stating simply 'Rayden has developmental delays'. They couldn't really see the exact cause of his seizures. After putting the email from Dr. El-Dairi together with his recent seizures, she also decided to order a 7 layer MRI, which is much more in-depth than he has had in the past. 

Follow up in 6 months. 

April 15, 2022 "2 seizures and an EEG"

 April 6, 2022

Rayden had been fighting allergy symptoms for about two weeks. He was taking Zyrtec at night before bed for the past couple of days. Around 6:30am I was in the kitchen getting ready for work. Rayden began moaning. When I entered his room I noticed he had labored breathing and shivering all over. He didn't reply to my questions. He began gagging and had the strangest breathing pattern like gasping for air. I gave him a sip of water and he threw it up, still shaking/shivering and not responding. He was limp and couldn't hold himself up in the bed to throw up so I had to help him. He wasn't talking and his response to our questions were mumbled. He kept looking toward the wall (left). When I did get a look into his eyes it was like he wasn't in there. He reached to hold my hand. At 6:50 I told Alex to call 911, as I called Paige Dunn (family friend and Paramedic). Thank God she was working and not on another call. Michael went to get Rayden's rescue medication from the kitchen counter but we did not use it because the ambulance had arrived (7:00). I was so relieved to see Paige. She has no idea how much her presence just set me at ease. They did an EKG, blood pressure, sugar, and pulse ox. During all of that, his shaking/shivering slowed down and his eyes started to look around the room at everyone. He finally replied to their questions with only one word and was able to hold himself up in bed for a second without falling down. They said they could transport him to our local hospital but it looked like the seizure had subsided. We decided to stay home and allow him to sleep it off. Alex crawled in the bed beside him while he slept. When he woke up at 11am he didn't remember a single thing. I contacted his neurologist, Carolyn Zook Lewis, at UNC. She recommended increasing his daily seizure medicine (Oxcarbazepine) from 3.8ml to 5.0ml twice a day. 

April 12, 2022

Rayden went to bed around 9:00pm. I normally lay down with him until he falls asleep and then go to my bed. I truly believe God allowed me to fall asleep WITH him so I could be there when he had another seizure. Around 9:35pm, I was awakened by Rayden violently shaking. I called his name with no response. I touched him, only to find that his entire body was stiff and shaking. I yelled for Michael and Alex who were still watching TV in the living room. When they turned on the light I was able to see Rayden's eyes, the scariest sight! They were wide open, looking straight ahead without blinking. They were fixed and fully dilated. I could tell Rayden was not in there! His left arm was bent and going back and forth almost hitting himself in the face. I tried to stop him, but his limbs were so stiff I couldn't move them. Michael ran to get his rescue medicine and Alex called 911. After 3 minutes I administered the diazepam but it didn't stop it right away like we thought it would. He continued seizing so I just laid my body across him and started praying. Michael grabbed my phone and called Paige. She wasn't working so she and her mom came right over, beating the ambulance by about 5 minutes. Rayden's seizure subsided about 2 minutes after I gave him the diazepam, just before Paige and Dawn arrived. As soon as I saw them I began to cry. Even though Paige did not have one thing that could help Rayden, just seeing her seemed to relieve my fears. Dawn took me into the other room, prayed with me, and restored my confidence while Paige and Alex sat and talked with Rayden. The Paramedics arrived and checked Rayden out. Even though he looked alright, we were encouraged to take him to UNC Emergency Room for observation. Alex kept Rayden awake and talking the entire drive. We were just all afraid for him to fall asleep. 

The ER physicians monitored him for about two hours and decided to discharge him. They recommended increasing his Oxcarbazepine from 5.0ml to 6.0ml twice a day. They also ordered an EEG for next week. Since Zyrtec is the only thing new we could think of, we asked if it could be the cause of the new seizures. They really didn't think so but recommended we stop and discuss it further with his neurologist next week. We got home about 4:00am. I went straight to the kitchen and threw the Zyrtec in the garbage. Of course, we all stayed home that day and tried to get some sleep. We were too scared to let Rayden sleep in his bedroom alone, so we put him in our bed just in case. 

April 15, 2022

Rayden had an EEG around 10:30 this morning. He was surprisingly calm and brave. He talked to the technician the entire time she was attaching the 21 electrodes to his scalp. He kept asking her if she was "putting on 5,000" and she would jokingly reply "no, only 2,000." During the exam she asked him a few questions, of which he struggled to think of the answers. She also attached a flashing light to see if it would trigger a seizure. Ultimately, she wanted him to fall asleep, even asking me to lay down beside him. He may have felt drowsy but he never went to sleep. 

The EEG revealed activity in the right frontal leads had increased beta and sharp theta activity. The flashing lights stimulated a driving response at multiple flash frequencies but no epileptic activity was identified. The clinical interpretation stated the increased faster frequencies in the right frontal region is likely a breach rhythm indicating an underlying skull defect. It also showed generalized slowing reflecting a diffuse non-specific cortical dysfunction on the basis of a toxic-metabolic systemic or primary neuronal etiology. 

I know that all sounds foreign. We meet with the neurologist to discuss the results next week.