July 27, 2023 "Spina Bifida Clinic"

Asleep during renal ultrasound

Spina Bifida Clinic

    BMI- 18.91 (89th percentile)

    Weight- 73 lb. (86th percentile)

    Height- 4'4" (68th percentile)

We saw Dr. Theodore Yip for his mobility check up because Dr. Alexander was out sick. He completed the school forms regarding Rayden's emergency epilepsy medication, bathroom needs, and physical limitations. His biggest concern was Rayden's BMI of 89th percentile. Children whose weight and height exceeds the 95th percentile are overweight, and those who fall between the 85th-95th percentiles are of concern. Dr. Yip would like to see Rayden below the 50th percentile so he is making a referral for a video visit with a Nutritionist. Children with SB usually grow at the same rate as other children but they are not as active due to mobility limitations. At least half of children with SB are overweight by 6 years old. This can cause greater health problems. Mainly, it makes it more difficult to move around and maintain balance on weak legs and feet. It can also put more pressure on the skin causing it to breakdown. Looks like we have some work to do. 

Two toned kidney. Dark/light

Dr. Jada Dillner, Pediatric Urology PA, discussed the results of the renal ultrasound from today. His kidneys look normal but are showing some different color patterns so she wants to get updated blood work. Wendy said she would request the special team ahead of time so we don't have to fight them about his tiny veins. His bladder wall is thickening and there is minimal trabeculation, which are both normal for neurogenic bladder. Since he hasn't had a urodynamic study in 3 years, she would like to schedule one soon. We have been considering the MACE surgery in the future to help with flushing the bowels, so they need to decide which surgeon may need to use the appendix the most, or if they need to combine surgeries. We are switching to adult sized catheters for the Peristeen as well. 

Dr. Narotam, Pediatric Orthopaedics, discussed Dr. Vergun's plan for surgery when he is closer to 10 years old to straighten his legs and get him out of the twister cables. Although he found no curvature of the spine, he is going to continue to monitor him closely because boys tend to start showing signs of scoliosis between the ages of 10 and 12. His surgical scar is well-healed with no erythema or swelling. There is some slight prominence in the midline lumbar vertebral area where there is a slight drop off without significant asymmetry. The musculoskeletal exam shows hip abduction is about 30 degrees on both sides. The internal rotation differs though. Right is about 90 degrees versus 60 degrees on the left. His knee flexion contractures are less than 5 degrees on the right and completely absent on the left. He rests on the heals on both feet when standing and walking without his AFOs. Even though we have always done it, Dr. Narotam reiterated that we hold his hands when he is walking without his AFOs, like after a shower or getting out of bed. This can only be done for short distances. He noticed he is walking on the inside of his right foot and recommended his new AFOs go completely around the ankle for more support. He would like to do a video visit after we get his new braces just to see how they fit. 

Dr. Hildebrand, Neurosurgeon, discussed Rayden's MRI from today. His new, nonprogrammable delta 1.0 valve is working properly. The MRI demonstrates stable, well decompressed ventricles. She briefly discussed the process for epilepsy surgery should this be required in the future. 

Ice cream reward

July 13-19, 2023 "Myrtle Beach"

Rayden thoroughly enjoyed our time at Myrtle Beach. He loved digging in the sand, and looking for shells. We actually found 3 shark teeth. The resort had 2 lazy rivers and 4 swimming pools. It also had an amazing restaurant where we enjoyed breakfast most mornings. We went to Dave and Busters to play arcade games, and The Track Family Fun Park to ride go-karts and blaster boats. We went to three different evening activities, The Charles Bach Wonders Magic Show, Riga Tony's Murder Mystery Dinner Show, and the Alabama Theater. Rayden's favorite was the magic show. He literally sat on the edge of his seat with his mouth wide open the majority of the show. I was even called to the stage for some tricks. The magician made my watch disappear. He was amazing. Michael and Papa took Rayden fishing one evening. He was able to catch two little sharks. He was thrilled. I'm so thankful we were able to spend this family time together. 

 

July 13, 2023 "Epileptologist"

Our appointment with Dr. Qian-Zhou Yang, Pediatric Epileptologist at UNC, was extremely informative. It took over an hour because she was very thorough, which we appreciated. She reviewed SOME of his complex medical history and even took the time to watch the short video clips of his seizures. In her little exam she was quick to notice that his right eye was his dominant one even though he is left handed. She confirmed his diagnosis of poor peripheral vision and his left eye turning inward. As she viewed his MRI's and EEG's on the computer, she pinpointed and explained everything alarming. She pointed out the large, white area of hydrocephalus, which we have seen numerous times before. Then she pointed out the gray spots both inside and outside the white area. This is polymicrogyria, which I had never seen. They are scattered all over his brain but more being on the left. The white area should not have ANY gray spots in it. She said they believe the gray areas are what seizes. Since there are so many gray areas in his brain it will be very difficult to pinpoint exactly where his seizures originate. To complicate things even further, one gray dot could be misfiring and soon the others will decide to copy it, like the old saying "one bad apple can ruin the entire bushel." 

The language center is usually on the left side of the brain. Since he is able to talk just before he has a seizure, this makes her suspect they begin in the right hemisphere. His eyes go the left during some of his seizures which is a second reason for suspecting onset in the right hemisphere. She said all seizures are like sparks that can turn into a fire. So if he has little sparks they are small seizures from one area of the brain. A full body convulsion seizure would be the fire, meaning it has spread into larger areas of the brain.  

She concluded that his seizures are likely focal onset with secondary generalization. However, with diffuse polymicrogyria and multifocal sharps there are many potential culprits. His current medication, Lacosamide, is a good choice for focal seizures (one area of the brain). Since he has multifocal (many areas of the brain) she believes he needs a low dose of a broader spectrum medication like Zonisamide as well. If he has any breakthrough seizures while taking these two medications, she recommends admitting him into the hospital (Phase 1) for about a week. During this stay he would be attached to the EEG, taken off his medication, and monitored by continuous video in hopes to capture and record seizures. She discussed the many other phases needed before they would consider epilepsy surgery. 2-PET CT with sedation, 3-MRI brain with and without contrast, 4-Resting state functional MRI brain, 5-Neuropsychiatry testing before and after surgery, 6-Neurosurgery appointment, 7-Stereo EEG hospital stay (implant probes in brain) to record more seizures, 8-Recommendation for epilepsy surgery. There are three types of epilepsy surgery; laser therapy, neuropace implant device, and resection. It sounds like a long, tedious process but they want to be as precise and accurate as possible in finding the small area of brain where the seizures are coming from. Seizure networks are like tumors. Over many years, they settle into the brain and become harder to control. They also spread to other irritated parts of the brain. Usually, the earlier a child has epilepsy surgery, the more likely they will have a seizure free outcome. Younger children also recover more quickly from brain surgeries than adults. Please help me pray that together, these two medications will keep him seizure free so he doesn't have to go through all of that!!!