Park Side kindergartners learning from ‘special student’
MARSHALL — If you look up “rare” in the dictionary, there are definitions such as unusual or uncommon.
If you ask the kindergarteners in Terri Zent’s class at Park Side Elementary, they’d most likely say that rare means someone special, like their classmate Everett Geurts.
Everett was born with an extremely rare disease — Beta-propeller Protein-associated Neurodegeneration (BPAN), a subtype of Neurodegeneration with Brain Iron Accumulation (NBIA) — though he wasn’t diagnosed until he was 2 ½ years old.
“All NBIA syndromes are extremely rare, but BPAN is even more rare because it doesn’t present with iron on the brain at birth,” Everett’s mom, Samantha Geurts said. “It’s also the only one so far that is not inherited by the parents in some way. It’s the only one that is spontaneous. We say it’s ultra-rare because there are less than 500 people with it in the whole world. And out of that 500, there are only about 20 males.”
In recognition of Rare Disease Day last week, the kindergarteners learned that Everett is one of millions of children living with a rare disease. While his diagnosis is complicated, the kindergarteners are just fine with simplicity.
“The kids love Everett,” Zent said. “They are so kind and gentle with him. And they want him to be included in everything. It’s super-neat to see. We’re so fortunate to be able to have him in class. There’s so much we can learn from him. He is so tolerant. We can also learn that it’s OK to be different.”
To help bring awareness to Rare Disease Day, Everett brought tattoos for the whole class.
“The tattoos are specific to his rare disease,” Zent said. “BPAN is a genetic condition that affects 1 in 10 million people, so it really is ultra-rare. And currently, there is no treatment and no cure for BPAN.”
Zent added some of the symptoms include: developmental delays, debilitating seizure disorders, limited expressive language and abnormal sleep patterns.
“We were grateful and honored be able to help bring awareness to this important day,” Zent said. “We thank Everett’s family for bringing it to our attention. We took a little bit of time to talk to the kids and share that it was Rare Disease Day and that Everett is one of those kids who has a rare disease. They thought it was really cool.”
Despite being non-verbal, the 7-year-old Marshall boy is able to engage with his classmates.
“The kids love to sit with him and hold his hand,” Zent said. “They’ll oftentimes stand next to him during story time. And he’s very tuned in to what we’re doing.”
Advancements in Everette’s education
Everett gers one on one support throughout the entire school day, according to Zent.
“A special education teacher comes into the classroom and works with Everett, primarily on his Tobii Dynavox device,” Zent said. “She helps him be able to develop his language. His vision is what makes it speak. It’s aligned to his eyesight — it will verbalize the picture he’s looking at. That’s how he interacts with people. The kids like to see him using that.”
Tobii Dynavox is a global leader in assistive technology for communication. Using eye-tracking technology, a device is said to know exactly where a person’s eyes are focused. By using the eyes as a “pointer” on a screen, communication is possible.
“He has no expressive language, so he’s considered non-verbal,” Geurts said. “But he is very expressive in his own way. We use ‘yes’ and ‘no’ wristbands and he also communicates using the Tobii eye gaze machine. He uses his eyes to pinpoint what he wants to say. He’s always had cognitive ability, but he’s just being able to express it more now.”
Everett piloted the device over the summer. Geurts said he got his actual device in November.
“As human beings, we rely a lot on our expressive language,” she said. “We’re just finding out that Everett has expressive language — and he’s a little bit of a stinker. He has nursing care, so he knows all of them very well. One of his nurses was taking him to clean up after lunch and said ‘If we don’t hurry up, we’re going to miss gym class.’ Using his machine, he said ‘You’re welcome’ because he knows she doesn’t care for gym class.”
Another time, Geurts was told Everett didn’t want to use his eye gaze machine one day in school.
“The speech therapist said she wished she and Everett really could have had more time together, but that she had to go,” Geurts said. “Everett said ‘Good-bye’ and then said the speech therapist’s name. I guess he just didn’t want to talk to her that day.”
Special education programs in the United States were made mandatory in 1975. The law was later modified to strengthen protections to students with disabilities and renamed to the Individuals with Disabilities Education ACT (IDEA), which entitles every students to a free and appropriate public education in the least restrictive environment. Inclusion, many say, benefits both children with disabilities and those without disabilities.
“I’ve gone into the class to read or help out,” Geurts said. “I remember the first time I walked in with Everett and Mrs. Zent said, “Good Morning,” and every single kid turned around and said, “Good Morning, Everett. That was so heartwarming. He loves his friends and loves being in that setting. It’s such a nurturing environment.”
While Everett has more needs than most, time isn’t taken away from other students in the classroom because extra staffing is provided for special education students — something school districts are mandated to do.
“The whole community at Park Side has done a great job of making sure Everett’s needs are being met so he can continue to stay mainstreamed and safe,” Geurts said. “It doesn’t affect the other children other than in a positive aspect, and it’s definitely benefiting Everett. I feel like this year has been really amazing for Everett.”
Since a typical cold can prove fatal for someone with Everett’s condition, a nurse is with him at school and also at home a lot of the time.
“We know we need to keep Everett healthy, but it’s a whole new level, so nurses come in and help. They’re there to watch for instability and to protect him from instability,” Geurts said
Geurts said the nurses know if another student has been sniffling all day and can be proactive by having them keep their distance from Everett.
“The nurses are part of our family,” she said. “They’re amazing. They help us create a more ‘typical life.’ They help us maintain Everett at home and make sure he’s safe there. They love him as much as we do, I think.
Everett uses a wheelchair, but his caregivers also help so that he uses other types of special equipment throughout the school day. “Everett can’t stand independently, so he uses a stander,” Zent said. “That allows him to stretch his muscles and be in an upright position, so it can put pressure on his legs. He uses different equipment throughout the day, just to be part of the group.”
Understanding Everett’s condition
Geurts said she and her husband, Jason, knew there was something going on with Everett as a newborn in 2010, but it wasn’t until he started missing milestones that they started pursuing answers.
“BPAN wasn’t founded as a syndrome until 2012, so Everett is older than the syndrome,” Samantha Geurts said. “We got Everett’s BPAN diagnosis at Sanford in Sioux Falls (South Dakota) in 2013. It was so new that you felt like the only one in the whole world that had it. There was no support group or anything, so we were just taking it one day at a time.”
Geurts said that BPAN is caused by a mutation in a specific gene.
“It’s always located in the WDR45 area in each individual who has BPAN, but their genetic coding can be different,” she said. “In Everett’s case, it was duplication, but for some, it may be a deletion or a mutation. It’s like a hiccup in your genetic coding and how that happens is how severe your syndrome will be.”
Everett’s seizures started when he was about a year old. While he has a feeding tube, he is able to eat some foods orally as well. Geurts said a ketogenic diet helps control the seizures.
“When we switched over our care to Gillette in St. Paul, we met with a neurologist there,” Geurts said. “We told her our feelings on the seizure meds and she said she was part of the ketogenic group. So he’s been on the ketogenic diet for almost two years and it’s controlled his seizures 90 percent. Anytime he gets a cold or has to take medicine for anything else, he has a break-through seizure. They take a lot out of him, so we try to prevent them.”
Geurts said that while the diet helps with seizures and also helped him break through “that cognitive fog,” increased monitoring is necessary. She added there is an increased risk of kidney stones and urinary tract infections and that his liver enzymes, cholesterols and sugars have to be closely watched.
Everett has some hearing loss, optic atrophy and Parkinson’s.
“He’s on palliative care, so everything we do for him treatment-wise is not about the longevity,” Geurts said. “It’s about the comfort and what life we have with him. There isn’t a cure for BPAN, so we focus on the day-to-day, making sure that Everett has everything he needs, is safe and is outside the hospital.”
The Geurts family, which also includes 3-year-old Lincoln and 19-month-old Landon, not only relies on the knowledge they’ve gained and the support they receive, but also on their faith. “We don’t ask for something to change in our lives, just for the strength and ability to walk through this life,” Samantha Geurts said.
Up until about two years, Geurts said there weren’t many online support groups.
“I was on Facebook and part of the ketogenic group and a mother had posted on there about her child having iron accumulation on the brain,” she said. “I knew that was part of BPAN. So from 2013-16, we knew nobody. Then all of a sudden, now we’re a community of about 200 that has about 50 kids and adults who have BPAN.”
Geurts said the oldest known individual living with BPAN is in his 30s and has less severe symptoms, though he was recently hospitalized with pneumonia and nearly died.
“Of all the people with BPAN in the Facebook group, there’s only like five males,” Geurts said. “It is extremely fatal for the male (most die before they are born). So Everett really is a survivor.”
An Extraordinary Everett Facebook page is set-up as a tribute to her son and to advocate for much-needed funding for research.
“We do our best to get the word out there,” Geurts said. “With BPAN being such a young syndrome and so rare, there is very little funding for research. Until we start researching it, we can’t cure it. We can treat the syndromes, but there are no cures to help our kids right now.”
Last summer, Geurts said the family had the opportunity to attend a conference, where they met the research team that discovered BPAN.
“Everett and his other BPAN cohorts are writing the book on BPAN,” she said. “I met a lot of them at the conference. It’s a bond that is unbreakable. We get a lot of support from friends and family, but it’s the sympathetic type. When you go and meet someone who has another child with BPAN, there a different level of empathy.”