Kiki

The following article was written and shared by Kiki's mother, Katie Thompson.

At age 10 months old, Kierra Grace AubriAnne Thompson had her first tonic-clonic (grand mal) seizure in my arms as I dropped her off at the sitters. After hours at the hospital and numerous tests, she was diagnosed with Tuberous Sclerosis Complex- a rare multisystem genetic disorder that causes tumors to grow on organs all over the body. She had been developing normally but I was told to watch for signs that she would regress. Over the next 18 months I watched her speech decline to zero words; her gross motor and fine motor skills stall; and her social and emotional skills also slow. Over the next 11 years Kiki, as she would prefer to be called, would gain a few skills but still hover in the 2-year-old age range in most areas. Now at age 14, Kiki has many diagnoses that fall under the umbrella of TSC. Autism, Developmental Delay, Expressive and Receptive Language Delay, Sensory Processing Disorder, Pica, Stereotypic Movement Disorder, Lennox-Gastaut Syndrome - a rare and severe form of epilepsy with multiple seizure types including a recent Gelastic seizure diagnosis (also rare); and intractable epilepsy among many others not listed.

Kiki is a giggly child. She loves to listen to others, as long as it isn't about school work. She has started to engage in cooperative and pretend play and is understanding that it can be fun. Kiki loves to look at books especially if they have faces, birds, or the color blue on them. She prefers to be outside - but only if it's sunny. She likes to sit on the deck, listen to the birds talk to each other, and watch the leaves blow in the trees and twirl one between her fingers. She hates shoes and will often be found barefoot with her toes buried in sand or dirt. Kiki loves birds and Disneyland rides and specifically Donald Duck. She also loves watching football, jumping on her trampoline, running on the beach, and rocking out to music. I just love how an autism mind can group like things together. Donald Duck is the face of the Oregon Ducks, wears blue (on the cartoon), and is a bird.
She is fearless! She loves to climb things and be tall, and also ride on things that are fast. She loved Papa's 4-wheeler this summer. She also can be very persistent; she can spend hours doing the same thing. She has the memory of an elephant. We don't see my family out of state very often but she will remember them. As we are getting her seizures more under control, she is learning new things and retaining them. Her brother Cameron worked with her this past year about closing doors she opened and putting his hat on his head. I also taught her that socks belong on her hands as sock puppets. She recently had a tonsillectomy (to help reduce her risk of apnea and SUDEP) and to help comfort her she put on a sock puppet before she fell asleep.

One challenge is Kiki is up for many varied hours of the night. I used to think it was autism not letting her stay asleep at night. Until recently, when I changed Neurologists from OHSU to one in Eugene, did I get a clarifying answer. Kiki was diagnosed with Gelastic seizures. They are also known as "the laughing seizures". They can go on for hours. Out of over 40 different kinds of seizures, they are the most rare. They are also the least studied and understood. I had asked when she was 2 years old to her old neurologist if she had Gelastic seizures and he dismissed it without seeing my videos. Over the years, as I have learned about being an advocate, if I truly believe something isn't right with my child, I need to trust my gut. I presented a video to the new doctor without suggesting my thoughts and he was able to confirm what I had known for years.

For many years, Kiki's seizures were so bad she was basically limp for hours a day in her wheelchair. Having at one time up to 80 seizures an hour, Kiki couldn't even hold her head up by herself. We were dripping Pediasure into her mouth via a straw and talking about putting in a g-tube for feeding. She lost a lot of weight and muscle tone. She didn't even have the strength to smile or laugh. We tried to continue her day as normal as possible. She went to school, where there were a few times she seized and stopped breathing and I met the ambulance at school. There were times I even had to do mouth to mouth to revive her at home. Being CPR trained, I never thought I would use that training on my own child; 4 different times. It's a haunting I will never get out of my mind. I am grateful for being able to save her, but I hope I never have to do it again. I encourage everyone to become CPR trained.
Finding the balance of medications has been tricky. We have tried many different combinations over the years. Kiki also has a Vagal Nerve Stimulator. It’s a pacemaker-like machine in her chest with a wire that goes to the vagal nerve in her neck to the base of her brain to send electrical pulses to stimulate the brain, almost like a heart pacemaker. We have also tried CBD tincture at one point. Intractable epilepsy means seizures fail to come under control with treatment. Finding the best combinations of all possible treatments to give her the best relief is the only option. It varies for each person.

Kiki's plan right now when she graduates school is to live at home with her mom and step dad. Her brother will most likely be off to college. Kiki is content to be at home where she can be on her trampoline and pick the leaves off the tree, go for car rides to the beach, and go camping with the family. This plan has been in place since finding out she may become developmentally delayed so many years ago.
 
I want her to be happy with her life. It won't always be easy and I want to push her to try new things, but we are in this together and she will always have me by her side.