Last week, The Children’s Home of Pittsburgh Pediatric VIEW hosted the Innovations in CVI Conference, focusing on navigating teams and transitions. Founded in 1999 by Dr. Christine Roman-Lantzy as an extension to NICU follow-up programs at West Penn Hospital, the Pediatric VIEW Program now serves children from birth to age 22 at the Children’s Home and Lemieux Family Center. The philosophy at Pediatric VIEW is to put “families at the center of everything,” said Roman-Lantzy. This is their second annual conference, designed to educate professionals and families with a vested interest in children with CVI.
“The recent CVI conference highlighted the dynamic nature of learning for children with CVI, emphasizing that educational systems must evolve alongside students' academic and visual growth,” said board member and CViConnect Director Stephanie Steffer.
Attendees witnessed many exciting takeaways. Here are just a few of them.
Hooper kicked off the conference with an energetic presentation about CVI literacy and ways to ensure students have accessible materials, a key component of a free appropriate public education.
Accessible literacy materials
Jonathan Hooper, NYC Public Schools teacher and private consultant, emphasized the rights of students with CVI to have accessible learning materials. “This is a call to action for providing a service delivery model that meets the student’s needs” and to “challenge the field’s perception about accessible materials for CVI,” he said.
Although systems are in place to provide services and prepare braille materials for blind students, Hooper said the accessibility of materials “is often an overlooked need for CVI students.” He added, “The current scales for identifying service delivery are inherently biased against students with CVI.” He encouraged teams to clearly document, in the IEP, the necessity for staff training, consult time, advance access to classroom lessons to prepare materials, time to modify materials, and appropriate environments to work with a student.
Power mobility for students with CVI and complex communication needs
A team from The Watson Institute (PA), including a speech-language pathologist (SLP), teacher of students with visual impairment (TVI), and physical therapist (PT), demonstrated how they work together to prepare a student with CVI and complex communication needs to use a power wheelchair operated via eye gaze technology. Because visual fixations propel the chair, the TVI first evaluates the student’s vision. The PT ensures proper positioning and supports, monitors the chair’s mechanics, and works closely with vendors and manufacturers. The SLP sets up the communication system and ensures optimal eye placement and alignment for the student. Conference-goers were able to take the chair for a spin.
The team placed directional cues on a black background and covered the camera to eliminate outside visual distractions so that the end user could successfully fixate in order to propel the wheelchair.
An AAC decision-making framework
Lynn Elko and the cohort of Penn State researchers and SLPs, highlighted a framework for designing a responsive AAC system for people with CVI, which emerged from her daughter Emma’s experience. The details are in the 2023 article An Evidence-Based Approach to Augmentative and Alternative Communication Design for Individuals With Cortical Visual Impairment (which received 12,000 downloads in the year following publication). Developed in collaboration with Penn State and CViConnect, the framework emphasizes individual characteristics of CVI, communication partner responsibilities, and system design. This is “an opportunity for people who were silenced for too long,” said Elko.
"This is an opportunity for people who were silenced for too long."
The disconnect between our education system and society
In his keynote address, Chris Russell, special education teacher and TVI in the NYC Department of Education’s Hospital Schools, made a case for why we need more personalized and thoughtful ways to mark milestones other than those transition points mandated by the education system. He urged us to take a step back to see the big picture. What does it mean to lead a successful life and how does education prepare students for that? He advocated for person-centered planning, more research on self-determination and CVI, and allowing kids with CVI to experience the dignity of risk. Russell said a good education includes people who are “interested in your child, eager to collaborate with outside experts, and are not afraid to admit when something isn’t working,” “involves the family,” and features a “curricular model that fits your child.”
Russell's keynote presentation addressed what he calls "the unceremonious in our education system."
Pediatric VIEW Data: A twenty-year perspective
Over the past two decades running the Pediatric VIEW Program (PVP), Christine Roman-Lantzy, PhD, has collected educational and medical data on nearly 1,000 children with CVI from the US and other countries, ages 9 months to 21 years old. The data include DOB, appointment dates, age of diagnosis, ocular conditions, gestational age, seizures, NICU/PICU length of stay, birth weight, associated medical conditions, medications, services, CVI Range scores over time, and zip codes.
While the sample is not random (all of the families self-selected to visit PVP), it represents many communities and ethnicities, ages, and levels of ability and challenge. “These are the kids on your caseload,” Roman-Lantzy told the professionals in the audience. “Children with CVI don’t fit in a box.”
A statistician helped Roman-Lantzy explore the data set. Some highlights:
1 CVI is not a condition primarily associated with prematurity.
2 CVI is associated with many medical conditions and progress in functional vision is not correlated with any particular cause.
3 Most individuals seen at PVP were diagnosed with CVI after age 3.
4 The greatest number of individuals with CVI were in hospital intensive care for a prolonged stay (>35 days).
5 Nearly 70% of individuals with CVI were diagnosed with cerebral palsy.
6 Strabismus and optic nerve disorders are common in individuals with CVI and the presence of ocular conditions is not correlated with progress in functional vision.
This histogram shows the services that children seen at PVP receive. “Why are fewer than half receiving vision support?" asked Roman. "And what about O&M?”
Future analysis may explore how CVI Range scores change over time and compare those scores to other details like medical conditions, service access, and gestational age.
Optic nerve hypoplasia and optic atrophy in CVI
Dr. Mark Borchert, neuro-ophthalmologist from Children’s Hospital Los Angeles, zoomed in to discuss optic nerve disorders and CVI. In summary:
2 ONH and CVI can co-exist, but usually due to abnormal cortical development and/or seizures associated with ONH.
3 Optic atrophy and CVI can co-exist when the brain injury causing CVI also damages the optic nerve.
Borchert showed images of optic nerve abnormalities, which can confound the diagnosis and management of CVI.
Who gets invited to the transition party?
Parent advocate and special education consultant Mara LaViola provided many recommendations for families approaching the transition from student to adulthood. They included:
1 Focus on SPIN: Strengths, preferences, interests, needs.
2 Ask for person-centered planning conducted by an outside consultant.
3 Remember that an IEP can include extracurriculars.
4 Don’t limit your child to disability-specific options! Use Supplementary Aids and Services, if needed, for your child to participate (34 CFR 300.2).
5 You can invite any agencies responsible for any part of the IEP to the transition meeting.
6 Explore pre-employment transition services (Pre-ETS) for job exploration and counseling, self-advocacy instruction, workplace readiness, post-secondary exploration, and work-based learning.
7 Usually at age 18, rights transfer from the parents to the student. What will you do when you are no longer invited to meetings, the school does not need your consent for reevaluation or change of placement, and your right to request mediation or file due process disappears? In certain cases, parents might pursue supported decision-making or education power of attorney.
Building a team for a complex child moving toward the adult world
Lynn Elko shared lessons from recent experiences helping her daughter transition to the adult world. In what she calls an “act of love,” Elko spent countless hours compiling information from every US state, all of which differ in available resources and ease of navigation. Take a look at the links for more details.
Medicaid.gov with search feature to find waivers by state
List of state websites with information on applying for Waiver Program
NIH Perspective: Coordinating interdisciplinary research on CVI
Alicia Kerr, PhD, provided an update on the NEI and NIH commitment to CVI, which many of us follow closely. Two new developments:
1 The working definition of CVI is slated for publication in December 2024.
2 Next month, they will begin taking applications from organizations bidding to become a registry site that collects data for the CVI registry.
Additional presentations included case studies on preschool programming for AAC/CVI (Alyssa Sims, The Bridge School), getting to a CVI diagnosis (Dr. Preeti Patil, Children’s Hospital of UPMC), designing an elementary CVI intense support program (MaryAnne Roberto, TVI), and protecting the civil rights of students with disabilities through legal representation and advocacy (Stacy Gahagan, The Gahagan Paradis Law Firm).
The NIH working definition for CVI: 1) CVI encompasses a spectrum of visual impairments caused by an underlying brain abnormality; 2) the visual dysfunction in CVI is greater than expected by any comorbid ocular conditions; 3) the visual dysfunction in CVI may manifest as lower- and/or higher-order afferent visual deficits, leading to characteristic behaviors in affected individuals; 4) while CVI may be comorbid with other neurodevelopmental disorders, CVI is not primarily a disorder of language, learning, or social communication; 5) the underlying neurological insult of the developing brain may go unrecognized or undiagnosed until later in life.