The Key That Unlocks All the Doors
Griffin, Georgia (Senate District 16)
Even though it’s already late in June, the Cloud family haven’t quite firmly established “the summer routine” for this year because they just took two trips and had Vacation Bible School. They’re looking forward to things slowing down next week. That’s when they’ll start going to the pool in the neighborhood. As Alaina Cloud, 14, walks into the room, she looks around and, as soon as she sees she has visitors, starts to feign a cough. “I'm not feeling well,” she whines. “Oh, no, you feel just fine,” Shannon laughs. She lets us know that this is a frequent trick that Alaina tries to pull to get out of things.
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Alaina has Dravet (pronounced druh-VAY) syndrome, a rare form of epilepsy that begins in infancy. "Previously known as Severe Myoclonic Epilepsy of Infancy (SMEI), it affects 1 [in] 15,700 individuals, 80% of whom have a mutation in their SCN1A gene” (https://www.dravetfoundation.org/).
Alaina was not diagnosed until she was 4.5-years-old. These days, with her symptoms, she would likely have been diagnosed much younger. If they have one or two really long seizures as an infant, most children get immediate genetic testing. Alaina's first seizure was when she was 4-months-old. Her parents heard something through the monitor as she slept in her crib and went in to find her arm was jerking. They called their neighbor across the street, a nurse, who said, “You need to take her to the ER [emergency room].” They called 911. Usually a fever-related seizure only lasts five minutes. Alaina’s longest seizure lasted nearly an hour and half, which is an indication of a bigger problem.
Shannon says that Alaina was developmentally on track until she was 18-months-old. She was walking when she was about a year old. At Thanksgiving when Alaina was about 18 months, Shannon made a list of all the words Alaina could say. It was 50 words. “I was so pleased,” she says because she was having so many seizures. Then, about the time Alaina turned 2, she counted again and realized that the list of words hadn’t grown at all. Alaina wasn't progressing.
The longest seizure was when Alaina was 19-months-old. They were outside at a playground. They started out by trying to administer the rescue treatment they had on hand but wound up going to the ER. Alaina had to be intubated. She was in the pediatric intensive care unit (PICU) for several days, as they had to bring down the sedating medications and wake her up. “That was the scariest one,” says Shannon.
The neurologist recommended speech therapy. Because they were first-time parents, they didn’t really know what they should look out for. They went to the school district for speech therapy. The district came back and said that Alaina qualified for special education pre-K all day long. When her speech wasn’t getting better at around 4, they decided to take her off a common seizure medication and went onto another one. The new medication happened to be very bad for Dravet syndrome, but at that time they didn’t know she had it. She started having much more frequent seizures—five to seven per week, much more than she’d been having.
Doctors decided to send her to the hospital to evaluate Alaina for surgery. She was there for five days to observe the seizures. She was on lowered medications to see if a seizure would come on. She didn’t have one for several days, despite eventually going completely off the meds that were making things worse. She finally had one on day five. The fact that she went for so long without a seizure while off her meds clued in the doctors that genetic testing was necessary.
Once they got the diagnosis, when Shannon looked over the list of symptoms of Dravet syndrome, she said to herself, “I could have told you all this years ago.” Even Alaina’s slowdown in development was typical to the disorder. Shannon was angry with their neurologist for not figuring it out sooner. Dravet syndrome was not commonly diagnosed or on any of the foundation websites she’d read. As far as Dravet syndrome patients go, Alaina is on the higher-functioning end because she can walk and doesn’t have seizures every day. Many of the patients are nonverbal, cannot walk and have seizures every day.
According to Shannon, as soon as a child tests positive for Dravet, the parents get tested as well. Neither of them had it, which is the case in 80-90% of Dravet parents because it’s a genetic mutation. When they had their second child, they didn’t yet know about Alaina’s diagnosis.
Many children with Dravet share other characteristics, including a fascination with puzzles, a hunger for carbohydrates and a very unusual way of sitting, which involves folding the legs up tightly. Shannon says they push Alaina to stay physically active and to take hikes. One of the characteristics is that patients’ ankles start to turn in. They’re starting to see signs of that in Alaina.
Now on a new seizure medication, Alaina is very sleepy, not at all hungry and reluctant to talk. “You want to eat something?” Shannon asks, almost pleading, pointing at the pile of chips on the counter. Alaina calls putting a bunch of different kinds of potato chips together “a salad.” What kind of chips does she like? All of them. Shannon tries to get Alaina to drink some whole milk to increase her calorie intake for the day because the chips are all she’s eaten.
“One time I had a seizure in the waves,” Alaina pipes in. Shannon encourages her to share more stories. Where else have you had seizures? “In mom’s room this morning.” It had been 35 days without a seizure, which was her longest streak without one in three and a half years. Shannon says, “Of course, she had one this morning! But those 35 days were great!”
Alaina has grand mal seizures, which can be very short but have very rarely lasted up to an hour and a half. They mostly take place when she’s sleeping, which is good and bad. If they happen when she’s awake, then she could potentially fall. She’s taken some pretty hard falls, even hitting her head. In that way, it’s better that her seizures happen most when she’s sleeping. On the other hand, seizures when she’s sleeping have their own concerns because they don’t catch them all. There’s the potential that she could flip onto her stomach, head into a pillow, and be unable to breathe. These days, most of her seizures happen at the beginning and end of her sleeping, something that is likely tied to how sleep cycles work. Her parents keep a video baby monitor on her, so they can keep an eye out for seizure activity.
They’ve considered a dog that can alert them to the onset of a seizure, but they cost $20,000. There’s no guarantee that it’ll be a good match. The dog may not be able to detect their partner’s specific brain chemicals. It’s a big gamble. It would also be hard at Alaina’s age to get the dog approved with the school system.
Alaina starts talking about some math problems and Free Fridays at school, when she gets to play on the computer, do cool math and watch PBS KIDS and Starfall. Alaina is in a small group special education class most of the day, but they go to gym, art, and band as integrated electives. “We played the drums!” Alaina says. Shannon says that it was nice they had that opportunity and it was different, but Alaina really prefers the gym over everything else. She likes to shoot baskets, over and over. “A hundred baskets!” Alaina says, indicating how many she shoots in one period.
She also likes to play virtual basketball on their Wii. Recently, Shannon figured out that Alaina was changing everybody’s avatar pictures to have the same color shirt as what they were wearing each day. Color is very important to Alaina. She plays with diving rings in the pool. After doing this for many years, the family finally figured out that Alaina was bringing up her diving rings in the order of the colors of the rainbow (ROYGBIV) every single time.
Alaina struggles sometimes more with behavior than seizures. That’s because the medications make it hard for her to focus, but perhaps it’s just a part of the condition. An attention deficit hyperactivity disorder (ADHD) medication helps with focus and staying on task at school, as well as trying not to annoy her friends at school or her siblings. “She may not know a lot, but she knows how to press buttons,” Shannon laughs.
Alaina shows a picture of her younger self holding a sign that says, “When I grow up, I want to be a dentist.” Shannon says that she doesn’t really think Alaina has a concept of what it means to have a job or what she’d want to do, but she remembers this day they got her to hold this sign at school all the way back in fifth grade. Shannon says that, developmentally, Alaina really lives in the here and now (what I’m doing, where I’m going, what I’m wearing). She believes it’s about the mental equivalent of 4-years-old.
Alaina says, “I’m done.” She wants to go play with her iPad.
Shannon previously worked in IT consulting but has decided to take some time off so she can concentrate on helping to guide her family and its daily routines. Her husband works in finance at Cox Communications. They were hugely involved with the medical cannabis bill which passed in 2019.
The family actually knows a lot of legislators already because of their advocacy around the medical cannabis bill. The legislators would know their family through the lens of Alaina as a child with seizures, not because she has an intellectual/developmental disability (I/DD), but Shannon says, “It all goes hand-in-hand. Every conversation I have with a legislator is about medical cannabis. We will continue to be huge advocates because we’ve seen how much it’s helped so many people.”
Alaina is not currently on cannabis as of a week ago because they’re in the process of adjusting her regular medications. Shannon is certain she will be back on it again in the future. Compared to others who have seen a drastic reduction in seizure activity, cannabis only lessened Alaina’s seizures a little, but it did help with her cognition. “Because she’s having a harder time talking and stuff... I mean, I was just thinking about that this morning... Maybe we need to add back a little because it just seemed like before she was a little clearer when she was on it, but we’re on different meds now, so it’s hard to know.” She’s usually on mostly a high concentration of CBD (cannabidiol), but they have tried small amounts of THC (tetrahydrocannabinol). At this point, as Alaina has lost about five pounds on the new medication and was already quite thin, Shannon might consider adding in some THC just to see if she can stimulate Alaina’s appetite.
What did Shannon learn in the process of doing all that advocacy? “We’d never done anything like that before, so it was all new to us,” she says. They got together with other families with epilepsy and talked about the fact that Georgia would probably be the last state to pass a medical cannabis bill, but they decided, “Hey, if no one is working on this, it’ll never happen.”
“Advocacy at the Capitol is challenging for sure,” Shannon says. She talks about how everyone has high-paid lobbyists. It wound up helping them because they had lobbyists on their side too. “As bad as it is, if that’s what it takes, I’m glad that our bill passed this year.” She says, ultimately, they figured out it was good for business too. “We’ve learned that legislators are just people,” she says. “Some of them are down to earth and super nice people, and some of them won’t give you the time of day.”
Shannon opens the freezer to show the heavy, high carbohydrate meals that would normally be what Alaina goes for when she’s hungry. The fact that Alaina waves it all off—the kinds of food most kids would love to get unending permission to eat—speaks volumes about how not hungry she is.
Alaina will attend the same school this fall. They just had a great year. So much of success in special education always comes down to the teachers, paraprofessionals, and therapists. Shannon is nervous about next year because Alaina’s classroom is going back down to one paraprofessional from two, and she’s concerned about the level of support the students will get. For instance, with only one paraprofessional, they’ll be limited in how many different electives the students can go to. Additionally, Alaina must have an adult with her at all times because of her risk of seizures.
Their private insurance is pretty solid. However, one medication she’s taking costs $400, another is $800 a month. A few years ago, they decided to get Alaina on the Katie Beckett waiver. They paid someone once to help them fill out the forms, but Shannon never filed the application. They’ve never gotten back to trying again. “I know we need to do it, especially now that she’s 14. She’s getting closer to adulthood and will eventually need to be on the waiver. We see all these people in our network posting about how their kid’s been on Medicaid for years, and then all of a sudden, they lost their coverage, their kid’s been denied.” The process to get a waiver is so daunting to families that are already overwhelmed by duplicative paperwork, appointments and life in general, they often don’t have the resources to try. “The to-do list is so long that you just don’t get to it,” says Shannon.
* * * * * *
Five months later, it’s a blustery Friday afternoon in November. Alaina is home from school early, getting to miss her final elective for the day.
As Shannon opens the door, the two newest Cloud family members come bounding out. Rainy & Thunder (Cloud), Golden Doodle puppies, were a present from Shannon’s husband six weeks ago. They have quickly become dearly loved, if sometimes smelly, messy additions to the household.
Clearly more animated and talkative, Alaina is now down to four seizure medications. They are in the process of letting go of Topamax, one of the worst medications for Alaina's appetite. They’re very glad to be tapering off just in time for Thanksgiving, with hope that Alaina will be able to enjoy food in a way she hasn’t in a long time. It’s been 18 days since her last seizure. Most of them now only last 30 seconds.
Sadly, one of their young friends, in the network of people with Dravet they’ve come to know across the country, passed away just a month ago. The near-15-year-old from Alabama came to visit with Alaina, along with another young girl from Virginia, just a few weeks before he passed. Shannon looks out the window as she describes how a long seizure led to a cascading downhill slide where his organs went into failure. “You never know for sure that something’s not gonna happen with Dravet,” says Shannon. It’s brought into stark terms what they’ve known along, which is that all time is precious when it comes to living with this precarious disorder.
One of the most fun things right now is that Alaina’s special education classroom has created a social enterprise, Griffin Grind, where they deliver coffee to teachers. Alaina’s job is to push the coffee cart as they deliver orders. “Welcome to the Griffin Grind!” she says, as they enter a classroom. “Be careful. It’s hot!” she announces with each delivery. Not only does the coffee shop help students in her class increase their socialization, but they also are learning to count money. The money they make will be used for field trips in the future.
At first, Shannon was concerned about the fact that Alaina’s class moved into a new classroom. It’s further away from the central office and the nurse. However, the new place is spacious, and has lots of technology and couches. It’s also right next to the Home Economics classroom, so they’re easily able to go over to work on their cooking, laundry and life skills.
With the same fervor of her coffee announcement, Alaina announces that she’s done talking now. She’s ready to curl up on the couch with her iPad. It is a rainy afternoon, after all.
Writer: Shannon Turner, Photographer: Kelly Blackmon