"Michael never, ever quits. He's the hardest working person I've ever met in my life." - Russ, Michael's dad
Donna Donovan [Michael's Teacher]: Here we go. Let's come up here with Donna. Let's do some work. I hear music. Up here.
Dana [Michael's Sister]: My brother's name is Michael, and he is eight years old. He has a neurological disease called tuberous sclerosis.
Donna: Mike looks. It stopped. We need more music. I have a switch. Would you like more music? Mike pressed the switch.
Russ [Michael's Dad]: Michael is not your average eight-year old.
Donna: Let's see what's next.
Russ: Developmentally he's probably six months old maybe to a year.
Dr. Elizabeth Thiele [Neurologist]: Michael is probably one of our more affected kids with TSC. It's in those kids that we often see the more severe cognitive impairment.
Dr. Thiele: Look here. Good job. Good job. Very nice eye contact.
Jane [Michael's Mom]: Oh yeah, he's doing great with the eye contact.
Dr. Thiele: What a handsome boy.
Dr. Thiele: Michael is nonverbal and he does have many autistic features. That being said, Michael is one of the happier kids I think I know. And he has a wonderful relationship with his parents and his sister.
Jane: Tuberous sclerosis to me is just an affliction that Michael has.
Dr. Thiele: Hi handsome. What do you know?
Jane: It's a genetic disorder that can affect every major organ. And it, from the heart, lungs, eyes, kidneys, skin. And Michael's happens to be in his brain.
Donna: Look who's here.
Jane: Hi Goo. How was your day? Was it good?
Donna: Michael had a great day today.
Jane: Michael's had seizures since the age of five months. That started with infantile spasms, and he's gone through tonic clonic seizures, drop seizures, he's had staring seizures, laughing seizures.
Dr. Thiele: We think, probably 80 to 90 percent of individuals with TSC have seizures or epilepsy at some point during their life. Usually, it has onset in early childhood, so about 67 to 70 percent of folks with TS have onset of seizures in the first year of life. So at least in childhood, epilepsy is probably the most significant medical problem, especially since it's not just the seizures.
Jane: You've got to do the work. Push, push, push.
Dr. Thiele: We know if we can't control the seizures, if the seizures don't respond to medications; if children need to be on multiple medications, then in addition to having seizures that child also is going to have a very significant time, trouble learning and developing, interacting with his or her world.
Jane: Which foot goes in first? No, not that one.
Dana: A seizure is like when your body basically, like shuts down. And then, like restarts. Like, when he was just learning to walk, and he had like a big seizure, he would forget everything he learned. And he would have to start from scratch.
Jane: What are you doing Goo?
Jane: We've been trying to control Michael's seizures since he was five months old, when he was first diagnosed.
Russ: Trying to find the right drugs, the amount of the drugs and the combination of the drugs is probably the most frustrating part.
Jane: You ready?
Jane: He went on a huge array of seizure medications starting uh with Gabitril. We even actually traveled to uh Canada to get Vigabatrin. These all worked for Michael for a, a few months. But then he would fall off and start having seizures again. And they'd come back just as hard, or even worse than when we started. For five years, yeah for five or six years, we've tried everything.
Dr. Thiele: Since the medications hadn't worked, we talked about other options. We had a lot of difficulty telling which side of Michael's brain the seizure activity was coming from. So surgery was not a great option. After talking about it we decided to try the vagus nerve stimulator.
Russ: The VNS. The vagus nerve stimulator. It is a battery that is implanted in Michael's chest. And, it is programmed to go off at a particular interval. And just send an electrical impulse to the nerve, which stimulates the brain, which helps control seizures.
Jane: Do you have a quiz tomorrow? In what?
Dana: Social studies and English.
Jane: All right. You've got to study, study. Where are you going Michael Charles? What are you doing buddy?
Dr. Thiele: In spite of the medications and the vagus nerve stimulator, unfortunately Michael continued to have some seizure activity, and we decided that the seizures he was continuing to have were still impacting his quality of life, and his health, and his ability to learn. So we also put him on another of our alternative treatments, the ketogenic diet.
Russ: Here we go to our menu.
Jane: We ran out of options so we said okay, let's do the diet.
Russ: This is the keto corner. Twenty-five grams of iceberg lettuce and fourteen grams of group B, so we have fresh broccoli. We'll nuke that up. And sixty-six grams of fat B. That would be mayonnaise.
Jane: it's a high fat, low calorie, very minimal protein, very minimal carbs and no sugar diet.
Russ: Nothing worse than getting half way through a meal and forgetting to zero out the scale.
Dr. Thiele: The ketogenic diet is one of our older treatments for epilepsy. It's a high fat diet that was designed and developed in the 1920s based on the observation that when people fasted, the seizures reduced in frequency. So the ketogenic diet was designed to trick our bodies into thinking we're starving. Because when you starve, you start breaking down your fat stores, to produce the energy our bodies need.
Russ: And desert ... his favorite meal of the day, peanut butter whip - butter, peanut butter and heavy cream.
Dana: The food Michael eats. It's not very appetizing. He basically eats this mayonnaise mush.
Russ: Is it good. Is daddy the best cook.
Jane: We did the diet starting two years ago last November and almost a year went by and we were almost going to give up. Then suddenly we just realized, he hasn't had a seizure in three days. And then it went a week. And then it went, knock on wood, and like don't even mention it. And it's, now it's been a year since he's had a, major seizure. So we're lucky in that it finally worked. We were, the labor has paid off.
Jane: Michael Charles, you coming in with us?
Russ: Hey Goo!
Russ: Life has gotten a lot easier since the seizures have come under control. The biggest change is all of our lifestyles, because he can roam around the house; he can go out on the deck; he can go out on the porch. No helmet on.
Jane: Try not to have a civil conversation with those two.
Russ: Hey Goo!
Jane: Michael Charles you gonna play your switch for us.
Russ: Come over here. We'll get your switch lined up.
Dana: Since Michael has stopped having seizures, he's laughing more; he's more like, it seems like he's more happy.
Donna [voice recorded on switch]: I had lots of fun in school today. We had PT, we had gym, we had story, we had a great time.
Russ and Jane: Yay!
Dr. Thiele: For Michael being seizure free, has meant the world. He was having such frequent seizure activity during the day, that it was very difficult to him, for him to participate in his therapies; for him to be out without someone standing around him all of the time. For him, seizure freedom has meant personal freedom, and also probably an increased awareness and appreciation of his world. What we will probably do is keep him on his current treatments. That's the medications, the vagus nerve stimulator and the ketogenic diet for about one year. Then we would start tapering one of his treatments. With the overall goal, over a period of time, of having him off all treatments for his epilepsy.
Russ: Michael never, ever quits. He's the hardest working person I've ever met in my life.
Jane: We have learned that you can't ask for giant steps. It's the baby steps we take every day.
Jane: You gonna give me a hug?
Russ: He's got his issues, his challenges, but he's a wonderful child.
Jane: I love you.
Russ and Jane: I love you.