Quick thinking by parents, pediatric neurology staff at UPHS, cure James’ infantile spasms

James was treated for infantile spasms at UPHS.

MARQUETTE — James Larson is your typical 5-year old boy. He loves building things with LEGOs or K’NEX; looks forward to seeing his friends at school; and he has an artistic mind, showcased by his love for reading and a passion for drawing that’s fueled by his vast imagination. His parents, Michael and Bri, both describe him as rambunctious with the standard child goofiness that can be expected. James’ best friend is his 3-year old brother, Walter, who both act “like partners in crime” as Michael describes them.

It’s a normal life for James and his family, but the reality is it’s never taken for granted. Because if it wasn’t for the quick thinking of Michael and Bri, along with the pediatric neurology team at UP Health System — Marquette, James’ bout with infantile spasms could have erased this kind of childhood for him.

Spasms set in

Bri noticed it first at around 1 p.m., then Michael later that evening. At seven-months-old, James had been a healthy baby up until this moment. When Michael went to put James to bed, something didn’t seem right. His son was staring back at him with a blank face and began lurching forward.

“We didn’t know; we thought it was gas or something,” Michael said. “I brought him to Bri and said there’s something strange going on here, and she said he did it earlier in the day as well.”

Despite the typical scares of going through parenthood for the first time, Bri and Michael never considered themselves the hypochondriac type of parents, the ones who watch and worry over their kid with every little thing. But now they faced the unknown, something completely out of their control and knowledge; so they did what any parent this decade would do: searched the internet for answers.

Michael began googling key terms such as “6-7 month-olds, lurching forward repeatedly” and varied his searches. What he found was other people’s kids doing the same thing, swaying forward repeatedly in a way that is sometimes overlooked as hiccups instead of infantile spasms.

James, right, with Walter.

“Googling conditions isn’t necessarily the way to calm your mind,” Michael said. “You can go to worst-case scenario pretty quick.”

They saw the dire message on some of the sites; how babies with infantile spasms can have neurological impairment, and how more than half of the children with infantile spasms will develop other types of seizures later in life. But amid all the troubling information they found, one article would prove to be instrumental in James’ recovery. It suggested parents record a video of the child during the child’s spasm so the footage could serve as another resource for doctors to review since the spasms may be intermittent.

The third spasm breakout happened the next day, so Bri and Michael captured it on video with a phone before bringing James to the emergency room at UPHS.

“At the time it was happening, I was in action mode,” Bri said. “Thinking about what happens next, packing the bag for the hospital … At that time, I felt like I had to deal with it by doing those things. But it’s scary not being in control. That’s a tough lesson for parenting is that you’re never actually in control; that’s a tough way to learn it. You don’t ever know what’s going to happen, and you’re not going to really know the outcome.”

When James got to the ER, his spasms had stopped and he checked out as a normal, healthy baby. Michael decided to show him the video of James lurching forward during one of his episodes, and the doctor agreed it was a strange behavior.

Just five months prior to James’ ER visit, Dr. Amy Martyanov arrived at UPHS — Marquette. She is the only pediatric neurologist in the UP, and the only pediatric subspecialist in the area, meaning she’s able to better care for her pediatric patients and their families when faced with complex neurological conditions. Dr. Martyanov has had medical training in pediatrics, followed by neurology, pediatric neurology and epilepsy. Only 700-1,000 people in the country have the same training background as her, and after viewing the video, the ER doctor decided to send the Larson family to her.

Immediately, Dr. Martyanov recognized James’ bout with infantile spasms. She admitted him to a 24-hour electroencephalogram to analyze his brain wave activity where the hypsarrhythmia — disorganized, chaotic pattern of brain waves in children with infantile spasms — was confirmed.

Brie, Michael, James and Walter visiting Bond Falls this summer.

“It’s a very rare seizure syndrome,” Dr. Martyanov said. “It’s a very malignant type of epilepsy where if it’s untreated, these children often become seriously developmentally delayed, permanently delayed, and they can lose their vision and develop all kinds of neurological impairments if that’s not treated promptly.”

In the past, an adrenocorticotropic hormone (ACTH) was used to treat infantile spasms, but the downside to the injectable steroid was that it takes about a week for the family to receive it after it’s been ordered. And in a study done in April 2018 from Sri Lanka, high dose oral prednisone was superior to ACTH for treating infantile spasms.

Still, when James arrived at UPHS, prednisone was not universally accepted for the treatment of infantile spasms. But during Dr. Martyanov’s time at the Mayo Clinic, it was the treatment her mentors used because it was much easier to obtain and less expensive than ACTH. Therefore, that is what she decided to treat James with.

James’ fourth spasm episode occurred while hooked up to the EEG, and the fifth happened the next morning. He then started taking the prednisone, a medication he’d have for four months before taking Topiramate — an anti-seizure medicine — for two years.

Five seizures over two days. And that was it.

“Within 24 hours, we were able to treat him and the spasms stopped,” Dr. Martyanov said. “He just did wonderfully and is normal. That was really like this lifesaving treatment, so it’s very exciting being able to help somebody.”

James at 2 1/2-years-old during his last EEG.

“We can’t say enough good things about Dr. Martyanov and the entire hospital system. We ended up getting connected with the patient advocate and some of the children special health services available at the state level. From the emergency room to Dr. Martyanov to working with patient services for children special health needs across the state, they all worked and came together to work for us.” – Michael

Just as Michael found initial information during James’ early seizures, the Larsons found another online resource to guide them post-hospital visit: an internet forum dedicated to families who had kids deal with infantile spasms. With such a rare disease, it’s not as if the Larsons can walk out of their front door and ask another family about their child’s experience with infantile spasms. The forum acted as an outlet for the family to connect to others and allowed many of their concerns to be put to ease.

Dr. Amy Martyanov

“Parents in the group stay on it years and years after their experience, and some these people dig into the research 24 hours a day,” Bri said. “Obviously you can’t call a neurologist 40 times a day with every question, but the perspective of families who have been there was very educational and informative. It was comforting to have their perspective.”

One of the biggest revelations for the Larsons while browsing the forum was just how lucky they were to have a child show symptoms of a seizure disorder, be diagnosed and treated before being seizure-free — all in a span of three days. Michael and Bri also read stories of families driving anywhere from 2-8 hours, hopping from hospital to hospital across the country, to find a doctor who can treat or understands infantile spasms.

“Later came to find that many children do not receive correct or proper protocol for this treatment because a lot of the pediatric neurologists aren’t up to date on the most current research,” Bri said. “We were just incredibly fortunate that the pediatric neurology staff at UPHS was top-notch. Dr. Martyanov’s treatment and outcome, I think, was in the top 1 percent with other families we came across on the internet.”

Living a normal life

James continued to visit Dr. Martyanov for two years, which if you’re fortunate enough to stay seizure-free, you’re discharged from neurology, and as Michael said, “It’s like, see you never, hopefully.”

So far, so good.

Sure, there were some obstacles to still overcome. James did have noticeable delays in his gross motor, forcing him to go through physical and speech therapy for months to help him get caught up with his peers. And for Michael and Bri, now the worrying began. Every hiccup, every weird face James made would by micro-analyzed by them as they wondered if another seizure had struck their son.

One of James’ drawings of a monster.

“We were hyper-alert, watching every little movement to see if he’s going to have a seizure again because we didn’t want to miss it if he did,” Bri said. “You got to keep your eye on him like a hawk. Little babies are weird anyway, so you wonder anytime if it’s a seizure or a baby just doing baby stuff.”

Despite the setbacks, James is poised to start kindergarten this fall as a normal 5-year-old with a diverse imagination. He enjoys acting out the storylines of his favorite characters in cartoon shows or books, but it’s when he creates art with his hands that his creative mind is fully on display.

Colorful monsters, machines and robots, all with the power of magic — good guys and bad — going on daring missions are James’ favorite things to draw. In one drawing, there’s a two-legged green monster with pink stripes that resembles a large cat with sharp teeth. Another is an oval-shaped figure with stick arms and legs, two big circular eyes with the pupils looking up and two triangle horns on top of its head; all of this has a charming cuteness to it.

James and Walter working on a drawing together.

“It’s pretty amazing to see what goes on in his head,” Bri said.

It’s that kind of inquisitive mind that managed to bring up one of the major events in his life. With infantile spasms potentially leading to other seizure issues down the road, Michael and Bri always planned to address with James what he went through sometime in the future. But in June, when the topic of hospitals was brought up for the first time, Bri delivered what would be the first revelation to her son about what he went through during his own stay at a hospital.

“I told him that his brain is a very important part of his body because it controls everything,” she said. “When something is going wrong with your brain, it can affect your whole body, so it’s important. I told him his brain was having hiccups and we had to see a special doctor to make the hiccups stop right away.

“I said that when you have hiccups in your chest, it’s doesn’t really matter that much; it’s silly or annoying and doesn’t hurt you much. But when the hiccups are in your brain, that can cause a lot of problems so we had to get that fixed right away.”

James appeared genuinely curious with this explanation — not awestruck or anything, though. And that was it. He went on to talk about something else, allowing his childlike wonder to move on to the next topic, just like any other child his age.

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