For Lydia Butler, the Heart Institute Neurodevelopmental and Education Clinic at Cincinnati Children’s feels like a second home. “Going there just makes me happy,” says the bubbly 11-year-old. “And I got to be in a video!”
Diagnosed with congenital heart disease when she was only five days old, at age 7 she appeared in the hospital’s “Tell Me a Story” video to talk about being a “heart kid.” Lydia was born with the single-ventricle lesion called tricuspid atresia and needed an artificial tube inserted to allow blood to flow from her heart to her lungs and “pick up” oxygen.
“It was like ‘replumbing,’” her mom, Lisa, says. The condition required three open-heart surgeries by the time Lydia was 4 years old. But the surgeries were only one small step in Lydia’s heart journey. As Lydia began preschool, Lisa began to realize that her daughter may have cognitive problems.
The most common birth defect in the United States, congenital heart disease or CHD, occurs in nearly 1 percent of — or about 40,000 — births per year. In addition, some CHDs bring risk factors for neurodevelopmental problems that include underlying syndromes or genetic and developmental disorders, according to a 2012 American Heart Association Scientific Statement (see sidebar, “If Your Hospital Doesn’t Have Neurodevelopmental Services”).
This means children with some forms of CHD may face developmental delays and challenges in school, and with living independently. According to the Cleveland Clinic’s Neurocardiac Clinic, approximately 30% to 50% of children who have undergone cardiopulmonary bypass for complex CHD will require remedial school services, and 15% will need full-time special education. However, with early screening for neurodevelopmental issues, children with CHD can thrive in school.
In general, milder forms of CHD result in fewer issues (or no issues at all) compared to complex forms, according to Caitlin Rollins, M.D., Instructor of Neurology at Boston Children’s Hospital and co-author of a 2014 paper on the subject in the journal Circulation.
“There’s enormous variability in how children do because of so many different influences, and what’s true for one isn’t true for all,” says Dr. Rollins. “For example, some forms of heart disease can alter blood flow during the fetal period that affects in-utero brain development. Some children incur specific injuries that can have neurological consequences. I recommend parents be prepared to act preemptively and continue to think positively about a good outcome.”
Infants may show developmental delays such as low muscle tone, and then face challenges with feeding, language and social skills. Later, school attendance may unmask problems such as difficulty with calculation or concentration. In high school, as students balance multiple courses, they may have trouble with higher-level organizational issues.
Dr. Rollins adds that parents are often first to notice anything unusual, and that early identification is so important. A primary care doctor can refer a child to a developmental specialist — such as someone who works in early intervention services — or to a psychologist or other specialist if or when parents notice any concerns. “For certain forms of heart disease that carry higher risk, children should be evaluated even before concerns arise,” Dr. Rollins says.
For CHD children younger than 3 years, Dr. Rollins urges families to seek out free, federally mandated early intervention services that include periodic evaluations — “even if things appear to be going well.”
“We call ourselves ‘heart moms’ and we’re all a little ‘nuts,’” says Lisa Butler. “We handle ourselves differently than other moms do.”
Lisa recalls how she handled Lydia’s neurodevelopmental issues, beginning with kindergarten, where her daughter “lagged.” When Lisa blamed Lydia’s heart defect, school officials didn’t think the problems merited a special education curriculum. Then in first and second grade, when Lydia had trouble remembering things, teachers said she was “lazy.”
Her mom knew it was time to take action, and so she turned to the neurodevelopmental clinic, or NDEC, at Cincinnati Children’s. Here, heart kids like Lydia go through a five-step process, starting with an initial screening, followed by a comprehensive evaluation and then a case conference. With detailed documentation and recommendations, an NDEC team member shares results with the child’s school, either in person or on the phone, to make sure the child has every opportunity to excel.
Long-term monitoring continues as the child grows and develops, with age-appropriate support, follow-up appointments every six to 12 months, reevaluation and assistance with recommended treatments.
The program sounded ideal for Lydia. Once at the clinic, Lydia saw a comprehensive interdisciplinary team of experts all at once without needing individual visits. Specialists followed a “whole child” approach with her treatment; Lydia had access to pediatric cardiologists, neurologists, nutritionists and occupational therapists, as well as social workers, child life specialists, a school liaison and educational specialists.
“Lydia initially received a diagnosis of attention deficit hyperactivity disorder or ADHD — common among children with CHD — and was referred to occupational therapy to work on fine motor and functioning issues,” Lisa says. Lydia was also diagnosed with developmental coordination disorder.
A clinical special educator at the hospital who helped Lydia, Gretchen Carroll, M.A., works with schools to determine eligibility and implement necessary intervention services for children. These can involve speech therapy, occupational therapy, physical therapy and educational support under an Individualized Educational Program or IEP.
“We’re not just dealing with kids in a bubble, but trying to affect change for them in school,” Carroll says. “Many schools don’t know a child’s cardiac issues can impact school performance. We explain how early medical issues affect learning, attention, emotional anxiety, language, motor skills and more.”
The educator can help support a child’s case for a plan, and her suggestions also help school personnel better understand the child’s specific needs. Her own detailed outline differs from a school’s IEP, however.
“No outside agency can dictate to a school just because we think a child is qualified for an IEP,” Carroll says. “We want to be a partner, but schools go through their own eligibility process and sometimes this is a ‘dance.’”
“We got our 504 plan and later, the IEP,” says Lisa. “When you bring Children’s Hospital into the mix, schools do seem to listen better.” The goal of any educational plan is to provide appropriate services while the student needs them — and eventually to get to the point where he or she no longer requires them.
As a school intervention specialist at Children’s Hospital of Wisconsin’s Herma Heart Center, Kyle Herma sees heart kids with neurodevelopmental needs. And yes, those surnames are the same.
When Kyle’s sister was born with hypoplastic left heart syndrome, the cardiology department was later named in her family’s honor to recognize their extensive contributions to eradicating heart disease. As a former classroom teacher, Kyle was hired to head the pilot neurodevelopmental program that began in February 2015.
She presented a poster — a “mini” scientific paper — at the International Society for Heart Lung Transplantation Scientific Sessions and Pediatric Academic Society’s annual meeting, both in late April. A full manuscript is in the works on the complete pilot study.
In the paper, Kyle’s team noted that children with complex health needs are at greater risk of poor student engagement, disruptive behavior, low academic achievement and exposure to bullying. They also cited the rarity of structured programs for school re-entry and intervention.
At Children’s Hospital of Wisconsin, patients receive individualized assessments and tailored interventions to be shared with their schools, Kyle says. Kyle meets with and counsels school staff on best practices to foster children’s progress, coaching teachers on appropriate modifications and accommodations and helping parents understand their rights and responsibilities when partnering with schools.
This means determining the problem first, then putting supports in place to address it, she says. These might include creating a personal and individualized checklist or schedule so the child can cross off tasks accomplished in anticipation of what’s coming next. Maybe a child could benefit from a peer notetaker, sitting closer to the blackboard or teacher, or just carrying a water bottle throughout the day.
Kyle’s previous teaching job at a high-risk school taught her about sending kids “off into the real world,” she says. “They may not know how to interact with society in a meaningful, productive way. Keeping them in a protective little bubble doesn’t help, so I want parents and teachers to encourage safe risk-taking.”
Lisa Butler does just that for Lydia, who has more big plans thanks to the neurodevelopmental support she received and continues to benefit from in Cincinnati. Lydia is always eager to go ice skating and play volleyball with her friends, while working on getting good grades at school.
“I want to do more fun things,” Lydia says. For those who love her and have followed her progress, there’s no doubt she’ll do much more.
“Having a heart kid is never easy,” says Lisa. “But having a team behind you that you know genuinely cares about your child — that is a priceless experience.”
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