Our patients’ stories: Taking back the night in epilepsy

Join us for a Twitter chat on epilepsy at noon on Monday, August 13, courtesy of the Boston Globe. Tweeters from inside and outside Boston Children’s Hospital will take questions and discuss the latest on epilepsy treatment, diagnosis and how families are living with the condition. You can sign up for a reminder and tweet your questions in advance or during the chat to @BeWellBoston (hashtag #EpilepsyChat).

Ian at Boston Children's

When Ian was diagnosed with epilepsy at age 3, his doctors said he’d outgrow it before he turned 5. But just the opposite happened: four months later, he abruptly started having much more frequent seizures, up to 40 a day.

With medications, the seizures stopped. But still, something wasn’t right. Ian seemed to be losing cognitive skills and language. He seemed to be in a fog.

“His outward seizures were controlled, but he wasn’t learning well,” says his mother, Michelle. “His development just stopped. He couldn’t learn anything new. We tried—I’m a teacher—and he was in a great special education program that gave him lots of support. But if he learned something one day, he couldn’t retain it.”

The family saw doctors at two different hospitals near their home in the upstate New York area. All of them said that learning disabilities tend to go with the territory in epilepsy. They suggested that Ian work with his therapist, his special ed teacher and his occupational and physical therapists to make the most of what he had.

But Ian’s parents weren’t willing to take Ian’s lost milestones as a given. They decided to take Ian to Boston, having heard that Boston Children’s Hospital had a good epilepsy program.

On an April morning in 2009, when Ian was 5, he met Tobias Loddenkemper, MD, an epileptologist at Boston Children’s who works with children who have tough epilepsy cases. It was a busy day in clinic, and Ian’s case took some sorting out. “I was looking at a lot of records and test results from other hospitals,” says Loddenkemper. “Ian’s seizures had resolved, but he continued to lose developmental milestones.”

Developmental problems could have many different causes. Was it a genetic or metabolic disorder? Loddenkemper ordered some tests, but felt there must be more he could do. After clinic that evening, he went over Ian’s case notes.

“He called us at 8:30 that night and said, ‘I couldn’t stop thinking about Ian. I think he might have something called ESES [electrical status epilepticus during sleep], and I’d like to admit him for monitoring,’” recalls Michelle.

ESES shows up on electroencephalograms (EEGs) as pronounced spikes, and Loddenkemper believed that the condition might be causing Ian to have frequent epileptic-like brain activity at night that was disrupting his ability to form new memories.

A few months later, Ian was admitted to the Long-Term Epilepsy Monitoring Unit for sleep EEG studies. Loddenkemper diagnosed ESES the very first night: When Ian slept, his brain was having as many as 10 or 11 abnormal spikes every 10 seconds—disrupting his sleep and literally wiping out what he’d learned that day.

“It’s like when you’re backing up your computer before you close down for the night,” Loddenkemper explains. “If you don’t save your data, it’s gone the next morning. This backup mechanism occurs during sleep, and when sleep is disrupted, you don’t learn as well.”

Loddenkemper and his colleagues had been finding that nighttime EEG spikes, by themselves, can lead to lost milestones, even in children without known epilepsy. This past May, Loddenkemper, along with research fellow Iván Sánchez Fernández, MD, and colleagues published another paper showing that the spikes could be suppressed with high doses of Valium given at bedtime.

In Ian’s case, the medication did reduce the EEG spikes but had some side effects. After another round of monitoring, Loddenkemper reduced the Valium dose and added prednisone this past January. This nighttime treatment, combined with Ian’s usual epilepsy medications during the day, got rid of the spikes almost completely.

But more than that—it led to an explosion of learning.

“When I see how Ian is now compared to a year ago, it’s stunning,” says Michelle. “Ian is 8 years old, and it’s almost like I’m meeting him for the first time. He’s using new words, he’s animated, he’s aware of what’s around him, he’s self-confident. He’s trying new things—jumping off the diving board, riding a bike, writing—things he never wanted to try before. They gave me my son back.”

Loddenkemper is thrilled, too. “In medicine, there are lots of red herrings, lots of other things to think of when assessing a child,” he says. “Once you find the needle in the haystack, it’s really rewarding.”

Read more stories of children with epilepsy in the Boston Globe, and learn more about Loddenkemper’s individualized approach to epilepsy in the Wall Street Journal and in this post on our science and innovation blog, Vector.

 

  • Sally

    This is such an uplifting story! I too, have 2 small children, who around 5 months of age started having seizures. We live in the Albany, NY area, and after no luck with treatment for our oldest child, we decided to go to Children’s Hospital in Boston too! Our experience has been wonderful and our daughter has been weaned off of her meds and has been seizure free for 6 months now! The doctors in Boston are fabulous and really caring, they always made me feel like my concerns were being heard and I knew my kids were getting the best treatment.
    Congrats to Ian and his parents for all his great improvements!

  • Uncle Peeto

    Way to go Ian! 
                        Uncle Peeto

  • Caraid Rose

    Way to go Ian! I’m so proud of you. I enjoy being around you and playing with you and all of our cousins! I can’t wait to see you again. Best of wishes! 

    Love always,
    Caraid xoxox

  • Jmbenson

    YAY!!!!!!!

  • P Sergio Ferreyra

    hola soy epileptico con epilepsia refractaria.  los medicamento que tomo son 4comprimidos de carbamacepina de 400mg,lamotrigina de 200 dos compri un divalproato de sodio de 250mg y 2 compri de pregabalina de150 mg.mi problema fue descubierto a 5 años de edad es producido por una leción cerebral que no es operable por miedo a que deje una lecion más profunda es un lecion de nacimiento los avances en epilepa me interesan mucho sobretodo para que un chico no pase lo mismo que pase en mi infansia . mi messenger es pedrosergioferreyra@hotmail.com