For 10 years, PCVIS has engaged in multidisciplinary collaboration and advocacy for children and youth with CVI. It began with a curious clinician and self-described “CVI journeyman.” Over a 33-year career as an ophthalmologist, Dr. Richard “Skip” Legge built his CVI knowledge. Then he built bridges with other professionals dedicated to the cause.
In his plenary presentation at the 2024 Perkins CVI Conference, Legge explained how his quest for knowledge led him to develop the American Conference on Pediatric CVI and work with a group of CVI experts to launch the Pediatric Cortical Visual Impairment Society (PCVIS).
Legge admits he knew very little about CVI in 1991 when he finished a neuro-ophthalmology fellowship at Tufts New England Medical Center.
A Perkins CVI Conference recap, from conference sponsor PCVIS
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“I really didn't understand anything,” says Legge. “However, children were coming through my doors in droves, parents and TVIs with lots of questions.
“What does the child see? How does this child see? Does this child see at all? Is this child cortically blind? Is it worth spending any time on this child? Are the glasses worthwhile for this child? Is strabismus surgery worthwhile for this child? Is there anything that can be done to help this child see better? Is the seizure medicine that the child takes affecting their eyesight somehow? Lots and lots of questions. And we still wrestle with them.”
Legge responded to these questions by saying something to this effect: “Because we see a lesion on the MRI in this particular area of the brain, it's probably affecting their eyesight. Let's treat the things we can, of course, glasses, strabismus surgery, that kind of thing.”
A moment of clarity
He says his inability to answer CVI questions during appointments resulted in a very frustrated child, a very frustrated parent, a very frustrated teacher, and a very frustrated doctor.
In 2002, “I’d had enough and just started reading,” he says. Legge dug into articles by Dutton, Good, Huo, Jan, Hoyt, and others. Until 2009, he felt like he was “groping in the dark” as he attempted to wrap his mind around the condition. He explains that in this era, “we had this horrible understanding of CVI…I shudder to think but we did. We talked about how children with CVI had ‘Swiss cheese vision’ because they’d variably respond…but we were just trying to figure out a way to understand these kids.”
Then, a moment of clarity. Legge noticed that his patients often attended appointments with an entourage – parents, teachers, and various therapists. Children with CVI work with many professionals who operate in silos, each with their perspective of CVI.
Legge wondered what would happen if he could facilitate interdisciplinary communication among them. “I had this idea that if I just got the various specialists together in a room and we talked about the same topics, we could pool our knowledge and elevate our understanding,” says Legge.
After sketching out the idea on a cocktail napkin during a 2009 meetup with Dr. James Adams, who was the superintendent of the Nebraska School for the Education of Children with Visual Impairment, the American Conference on Pediatric CVI was born. Legge invited experts to speak, including Christine Roman, PhD; Mark Borchert, MD; Dominic Maino, OD; and Jacy VerMaas, PhD.
Some of the earliest interdisciplinary conversations about CVI happened at the American Conference on Pediatric CVI in Omaha, Nebraska.
Things fell into place:
1 Children’s Hospital in Omaha stepped up to sponsor the event.
2 Sara Olsen, a new hospital hire, coordinated the conference. It was the first project to land on her desk and she produced every subsequent conference.
3 The first conference hosted 75 attendees, mostly ophthalmologists and educators. Legge notes that parents were missing from the audience initially, but would become the largest contingent in years to follow and that “due to Lindsay Hillier's attendance from Canada, we could call it an international meeting!”
4 In 2012, the group’s principal accomplishment was a multidisciplinary working definition of CVI. “It’s very close to what we're doing now [developing a diagnostic definition with the Trans-NIH CVI workshop], which I think is really kind of cool.”
A timeline, created by Rachel Bennett and fellow PCVIS members in 2018, highlights important points in the history of CVI since 1987.
Participants agreed that increasing public awareness of CVI was imperative to generate resources for research and improved services. They even bought the domain brainblindness.org, which Legge calls a well-meaning mistake. “We proposed the term brain blindness which, of course, is totally horrible. But we believed in it so much that we bought the URL. We actually paid money for the URL. And of course, we did not renew it because we understood quickly that it was horrible.”
'Perhaps it's time for a society?'
Fast forward to the 2013 conference, when Dr. Christine Roman raised her hand. “Perhaps it’s time for a society?” she suggested.
“It became obvious that our CVI working group needed to become anchored in a more permanent structure,” Roman explains. “The priorities of CVI cannot be attached to a once-a-year meeting, but must be embedded into a society and as such, meaningfully serve the community of families impacted by CVI.”
This proposal generated buzz in the auditorium. To get started, they wrote bylaws based on those from the North American Neuro-Ophthalmology Society and received pro bono advice from John Elliott, CPA, on how to set up a 501(c)3. And in 2014, the group voted to establish PCVIS, a nonprofit with advocacy at the center of its mission.
As Legge puts it: “It’s advocacy for the health and well-being of children with CVI. It's advocacy for philanthropy for CVI. It's advocacy for policy for CVI. It's advocacy for the education of health care professionals. It's really all about advocacy.”
PCVIS members gather around a pop-up sign used to help spread awareness about CVI at conferences and events.
While CVI parents were initially missing from the audience of the American Conference on Pediatric CVI, they soon became the largest contingent. Many took on leadership roles once PCVIS was established.
Past-president Lindsay Hillier offers remarks.
PCVIS members used to gather at the conference in Omaha annually.
CVI collaborators Dr. Borchert and Dr. Roman.
CVI parents trade ideas for creating materials.
Unveiling a rebranded and updated PCVIS website in 2019.
Rebecca Davis speaks of the power of advocacy.
The original PCVIS exhibit.
The current PCVIS exhibit.
What's next for PCVIS?
Upon reflection, Legge may not have always been right about CVI. But he wholeheartedly pursued the one thing he knew for sure: Interdisciplinary collaboration is the driving force for change. When the National Eye Institute (part of the NIH) issued a call for input on its strategic plan, 163 of the 252 responses they received were about CVI. This remarkable showing from our CVI community captured attention and, as a result, CVI is officially on the radar. The NIH is building a CVI registry to capture data on kids with CVI and has convened an interdisciplinary group to develop a working definition of CVI.
“We have to stay the course with the NIH and the NEI,” Legge emphasizes. "We have to complete the work on the diagnostic criteria, the definitions, and the electronic case registry. And this work benefits the entire CVI community.
"This is not just about the scientists. This is about everybody. We can move everybody forward with this work. Children are going to be diagnosed more quickly. Parents are going to be more empowered. Teachers are going to receive clarification. Doctors are going to be able to diagnose. Researchers will have standards. And policymakers will have a foundation for understanding. Everybody wins."
Efforts by PCVIS to generate submissions for the National Eye Institute's strategic plan helped put CVI on the national health agenda.
An example of a past social media call to action.
PCVIS continues to operate with a multidisciplinary board of directors and engaged membership base – all advocates for children and youth with CVI. And we still hold an annual membership meeting in tandem with a CVI conference. This year, you’ll find us on Oct. 2 in Pittsburgh, preceding the Innovations in CVI Conference hosted by Children’s Home & Lemieux Family Center. Please make plans to attend!