Cortical Visual Impairment (CVI) & Cerebral Palsy: Underdiagnosed & Undertreated



This is part one of a two-part post I will be writing about CVI:

Cortical visual impairment (CVI) is a neurological condition that is the leading cause of visual impairment of children in the US and the First World and is commonly seen in people with cerebral palsy (1. Good, Jan, Burden, Skoczenski, & Candy, 2001, p. 56.). It is not an eye condition. Resulting from damaged or malformed visual pathways and/or visual processing centers of the brain, CVI presents very differently than other types of visual impairment. Whereas a typical visual impairment can be diagnosed with an eye exam and vision testing, CVI often presents with a normal eye exam that does not explain the individual’s significant lack of visual function. Because of this discrepancy, CVI has been difficult to diagnose and treat. As I have learned more about CVI, I have begun to grasp how important it is for parents of children with CP or TBI (traumatic brain injury) to be aware of it’s signs and symptoms and how this recognition can make a dramatic difference in a child’s learning and future.

At the 2013 AACPDM annual professional’s meeting, Jen, a fellow parent and friend, who also has a child with CP and CVI, encouraged me to attend a presentation by Dr. Christine Roman, a leading expert on CVI. Hidden in this small conference room in a hotel in Wisconsin, I uncovered critical information that I felt I needed to spread to the CP community.

The lecture was inspiring and heartbreaking. Tears rolled down our cheeks as Jen and I watched videos of several children, previously diagnosed as blind who were given a corrected diagnosis of CVI, and learned to improve their vision. For years they had been deprived of opportunities to interact with and learn about their world because they were misdiagnosed and no one knew how to work with them. The key was to provide the right setting for each individual child to process visual stimuli. Then it became clear that not only were these children not blind, but that a new world could be opened up to them.

I wanted to shout from the rooftops and tell parents, educators, and doctors, “Hey, you, over here!!” “This researcher is onto something and it’s not just another diagnosis!” “She has found a way to improve the vision of people who have CVI.”

I left the lecture and couldn’t stop thinking about whether CVI could explain Maya’s mysterious visual processing problems. When she was three, Maya’s OT kept telling me, “Something isn’t right with her vision but I don’t know what it is.” Her eye exams didn’t offer insight into these problems. Maybe now I had an explanation. Over 40% of the brain is devoted to visual function! Knowing this information, if a child like Maya has damage to her brain, there is a chance that her visual processing centers may have been damaged as well.

During her lecture Dr. Roman discussed how children with CVI are also often misdiagnosed as having other conditions. Some are diagnosed with autism because their behaviors are similar to children on the spectrum (it is also possible for children on the spectrum to have CVI). Children with CVI also may be mistaken for being less intelligent or less capable of learning because of the challenges they have processing visual stimuli.

Dr. Roman said, “Some of these children who have brain injuries that are considered ‘so severe’ have been approached by others with low expectations for their learning capabilities. But it’s interesting to see what happens when these children have visual access to their world. Some of these children may not have demonstrated a desire to move because they are not able to see the world in a meaningful way.” After evaluating thousands of children, Dr. Roman reports that a child’s ability to improve functional vision on the CVI range does not appear to be influenced by the type of brain injury.

The major causes of CVI:

Although CVI can be a stand-alone condition some people with CVI have additional disabilities including:

  • Epilepsy
  • Cerebral palsy **One former President of the AACPDM told me he believes more than half of the CP community has CVI and most of them don’t know it!
  • Moderate cognitive impairment or lower
  • Sensorineural hearing loss

(Adapted from Khetpal & Donahue, 2007; Matsuba & Jan, 2006)

Why haven’t I heard about CVI and how pervasive it is in the CP community?

Dr. Roman said that CVI didn’t enter the medical literature until about 20 years ago. Many pediatricians and other doctors are still unaware of the condition and it is likely significantly underdiagnosed. Despite the Academy of Pediatrics articulating guidelines for vision screening and monitoring, there is no protocol for CVI evaluation and monitoring. Without formal recognition and consensus on diagnosis and intervention, time spent on treatment would not be covered by insurance.

At this time Dr. Roman says it’s mostly parents and educators who approach her team because they want more answers about their child’s vision that they haven’t been able to get. Dr. Roman hopes that CVI begins to gain more widespread and formal attention from the medical community in order to support assessment and early intervention.

The redeeming news for me was that regardless of professional disagreements, Dr. Roman has developed what appears to be a reliable system of evaluation and monitoring for CVI which she readily shares (it’s in her book as well) and which can be incorporated into educational and home settings.

What is Dr. Roman’s interest in CVI and what has she done in the field?

Dr. Roman began her career working as a teacher of children with visual impairment for 17 years. She was frustrated that many of these children were overlooked and she felt dissatisfied with her ability to help them. She went back to school to learn whatever she could about “this mysterious form of visual dysfunction,” (which she later learned was CVI) and how she could better support these children.

She is currently the Co-Director of Pediatric View in Pittsburgh, Pennsylvania and serves as Project Leader of the CVI Project at the American Printing House for the Blind in Louisville, KY.  Dr. Roman also is a Project Consultant/Trainer to two 5-year multi-state CVI-mentor training projects working to teach professionals to evaluate for CVI and to employ the CVI educational principles she has developed.

Dr. Roman’s contributions to CVI are built upon the foundation developed by pediatric neurologist, Dr. James Jan. She furthered his ideas by establishing the CVI Range, a 10-point scale assessment for determining whether someone has CVI and evaluating their level of function, as well as principles for guiding improvement. Although medicine and special education have not identified a body of best practices for assessment and intervention for children and adults with CVI, Dr. Roman is deeply passionate about continuing to share her knowledge of CVI and what techniques she has discovered to best support identifying, understanding, and improving the vision and education of people with CVI.

Dr. Roman’s 3 components for confirming the presence CVI: 

1. Eye exam doesn’t match up with how child uses vision

2. Big neurological event-common to all children with CVI

3. Presence of 10 characteristic behaviors (assess and evaluate)

There are other evaluative techniques used but Dr. Roman has found that these components to offer the most reliable and comprehensive assessment.

The 10 behaviors evaluated by using the CVI Range Assessment Protocol:

  1. Attraction to color (this is often pointed to as a sign of autism but kids with autism don’t NEED color to see but kids with CVI do)
  2. Light gazing and/or non purposeful gaze–people with CVI often need more light to see or will gaze non-purposefully because they can’t make sense of what they see
  3. Require movement to see
  4. Difficulty with visual complexity and sorting visual information–things are confusing to look at especially when there are lots of visual patterns, or competing sensory information
  5. Visual latency–visual responses are slow or delayed
  6. Visual field preference–children with CVI see better looking at objects in certain directions (such as left or right periphery), often difficulty recognizing information in lower field
  7. Difficulty with distance viewing–this is related to the preference for visual simplicity. Objects far away may become lost among other objects or scenery.
  8. Visual reflex differences–often don’t blink when touching their nose, not blinking when threat coming at them
  9. Difficulty with visual novelty–child looks at familiar thing instead of what is new in the visual field. Because the whole world often looks new to children with CVI they tend to focus on what they can recognize rather than what is novel.
  10. Lack of visual-motor match–look and touch occur as separate functions, e.g., child looks, turns head away from item, then reaches for it

The CVI Range assessment is NOT a replacement for an eye exam, but helps to describe how the child sees. It compares visual functions across a diverse population of children 6-21 years old and provides a common language for describing levels of function. The Range is a change model and is supposed to be used to develop appropriate interventions leading to improvement. Dr. Roman’s approach is to develop more vision in the brainso the individual continues to make progress in the range and in turn his/her functional vision.The educator/professional uses the range to monitor changes in visual processing, and adapt the individual’s environment to correspond with those visual processing improvements.

Dr. Roman has a goal of getting all children to 7 on the range and, although most people with CVI naturally improve over time, Dr. Roman says progress is dependent on what can be done to foster improvement, as well as to offer support as early as possible.

Five Big Ideas for Working with Children with CVI:

(Adapted from Roman-Lantzy, 2007)

  1. Understand and be sensitive to the child’s visual world.
  2. Develop familiar routines and activities.
  3.  Offer visual stimuli at (but not above) the child’s level.
  4. Approach the child gently, with respect and humor.
  5. There is no universal CVI program- Each child is unique.
Stay tuned for Part 2 on CVI and Cerebral Palsy where I will detail more personal insights and ideas about CVI and visual processing.
Sources:

1. Good, W. V., Jan, J. E., Burden, S. K., Skoczenski, A., & Candy, R. (2001). Recent advances in cortical visual impairment. Developmental Medicine and Child Neurology, 43: 56-60.

CVI Internet Resources:

Locate a CVI expert or an individual who has been trained in administering the CVI range

Pediatric Cortical Visual Impairment Society

American Printing House for the Blind

American Foundation for the Blind

Dr. Roman discussing CVI on video 

Cortical Visual Impairment 101– blog post from the website “A Day in our Shoes” an IEP resource website for families with special needs children

Dr. Karen Pape discuss CVI in one of her blog posts, “Myths and Truths about Cortical Blindness: Baby Brains DO Recover”

Video-Strategies for Improving Literacy Skills in Students with CVI-Perkins Tutorial

Books on CVI:

Dr. Roman’s Book–Cortical Visual Impairment: An Approach to Assessment and Intervention (The educator/professinal CVI Range assessment is in the book)

Support Resources:

Thinking Outside the Light-Box-Vision Therapy Support Group is a CVI fb support group for parents

Little Bear Sees a website offering wonderful information for parents about children with visual impairment. They also have about CVI available through their website.

More on Dr. Roman’s credentials:

Dr. Roman was previously the Director and Assistant Professor in the Program in Visual Impairment at Marshall University Graduate College, South Charleston, West Virginia; Research Assistant Professor at the University of Pittsburgh Special Education-Vision Studies Program; and Infant Developmentalist in the Neonatal Intensive Care Unit of Western Pennsylvania Hospital and the Children’s Home of Pittsburgh. She also directed Project CRIB at the Western Pennsylvania School for Blind Children. She has contributed journal articles and book chapters on orientation and mobility, children with multiple disabilities, and visual assessment for infants and has presented lectures and workshops on cortical visual impairment all over the United States and around the world.

 

 

Q&A with Dr. Marshalyn Yeargin-Allsopp Medical Epidemiologist & Chief of the Developmental Disabilities Branch National Center on Birth Defects & Developmental Disabilities CDC

Dr. Marshalyn Yeargin-Allsopp
Medical Epidemiologist and Chief of the Developmental Disabilities Branch
National Center on Birth Defects and Developmental Disabilities
Centers for Disease Control and Prevention (CDC) USA

1. CP Daily Living: How can CDC help the CP community?

Dr. Marshalynn  Yeargin-Allsopp: CDC is committed to continuing to provide essential data on cerebral palsy (CP). Communities can use CDC’s CP tracking data on the number and characteristics of children with CP, including information on CP subtype, walking ability, and co-occurring conditions. This information may be helpful in in planning for services, guiding policy, and promoting full participation in community and family life.

We also co-host events to raise awareness of CP and engage local communities that collaborate with CDC on CP tracking. These events are free of charge and open to the public. This year, we will be partnering with University of Wisconsin-Madison to host an event on March 1, 2014. For more information about this event, please contact Julia Richardson at jrichardson1@cdc.gov.

2. CP Daily Living: Where do you get funding for your CP work?

Dr. Marshalynn  Yeargin-Allsopp: The Children’s Health Act of 2000 authorized the creation of the Autism and Developmental Disabilities Monitoring (ADDM) Network. The ADDM Network is a group of programs funded by CDC to estimate the number of children with autism spectrum disorder and other developmental disabilities. By using the infrastructure already in place for tracking autism spectrum disorder, and without additional funding, CDC has been able to track the number and characteristics of children with CP.

3. CP Daily Living: What type of studies do you conduct? What other types of studies or programming could you do to further our knowledge of CP?

Dr. Marshalynn Yeargin-Allsopp: CDC’s work in CP focuses primarily on tracking. For example, we know that about 1 in 323 children has CP based on tracking in multiple U.S. communities. Tracking has helped us learn more about causes and the risks for CP in hopes of finding ways to prevent CP. For example, we reported that approximately 10 -15% of CP is due to events that occurred after the newborn period, such as head injuries, meningitis, or stroke from sickle cell disease. Some of these events are preventable with public health interventions such as child passenger safety measures and vaccinations (shots).

CDC is well-positioned to build upon the existing ADDM Network to expand the scope of its current activities and answer more questions about CP. A comprehensive strategic plan for CDC’s CP activities might include:

  • Tracking younger children with CP in order to better understand when and what interventions are being used.
  • Learning more about the risks of CP and ways to prevent it by conducting research among children with CP, such as special studies of children with CP identified by the ADDM Network.
  • Studying issues across the lifespan, such as employment, by following up on adults with CP who were identified in childhood by the ADDM Network.

4. CP Daily Living: Why it is important to track how many people have CP? Are there any other agencies tracking this information?

Dr. Marshalynn Yeargin-Allsopp: It is important to know how many children have CP so that appropriate plans can be made to support children with CP and their families. Understanding the number and characteristics of children with CP is key to promoting awareness of the conditions, helping communities coordinate service delivery, and identifying important clues for further research.

CDC’s ADDM Network provides the most complete picture of the number and characteristics of children with CP in the United States. The ADDM Network is unique in that its methods are population-based, which means that we look at information on thousands of children from diverse communities across the country. In 2008, the ADDM Network sites that tracked CP represented approximately 4% of 8-year-olds in the United States. Because of the size of our population of children with CP, we are able to look at more than just the number of children with CP. We are also able to examine characteristics of children with CP and compare groups (such as boys and girls or White children and Black children).

5. CP Daily Living: I have heard people state that none of the issues which cause CP can be prevented, and the ones that can are already being studied by other fields (such as stroke and prematurity). What do you think about these statements?

Dr. Marshalynn Yeargin-Allsopp: In most cases, we don’t know why children develop CP. Some preventable risks for CP have been identified. For example, babies with severe jaundice can develop kernicterus, which is a condition that occurs when severe jaundice goes untreated for too long is a known cause of CP.  However, severe jaundice can be treated with special lights (phototherapy) and perhaps blood exchanges to stop the development of kernicterus. Similarly, routine vaccination of babies prevents many cases of meningitis, another known cause of brain damage that can, in turn, cause CP. Making sure that children are properly buckled in car seats or booster seats can help prevent head injury during a car accident; head injury is another cause of CP.  CDC’s ongoing CP monitoring efforts provide the opportunity to examine trends over time in the number and characteristics of children with CP for whom the cause of their CP occurred after the first month of life and may have been prevented.  This enables us to identify areas for additional prevention efforts as well as to evaluate whether current prevention efforts may  be contributing to decreases the number of children with CP overall.

6. CP Daily Living: Are there ways for the CP community to collaborate and extend or join studies in order to determine what links may exist between what other fields are studying and their condition to the development of CP such as prematurity?

Dr. Marshalynn Yeargin-Allsopp: There are many opportunities for collaboration between those studying CP and those studying other conditions. For example, in CDC’s most recent report, we found that many of the children with CP had co-occurring epilepsy and autism spectrum disorder. Of particular note, we found that about 7% of children identified with CP also had autism spectrum disorder. This means that autism spectrum disorder order is more common among children with CP than among their peers without CP. Information about the co-occurrence of CP and other conditions, such as autism spectrum disorder, can help direct research into shared risks and causes.

7. CP Daily Living: How does the CDC partner with private agencies and researchers to accomplish their goals and mission?

Dr. Marshalynn Yeargin-Allsopp: Partnerships are essential to CDC’s work in CP. At the local level, we partner with local organizations in each of the four U.S. communities where CDC tracks CP. Without the ongoing support of these organizations, which range from public schools to pediatric specialty clinics, we would not be able to collect this important information on the number and characteristics of children with CP. At the state and national level, we also partner with organizations that provide services to and advocate on behalf of children, families, and adults living with CP. These partners help us connect with families and help us remember that tracking is about more than just the numbers—it’s about individual children and families that need support. Our national partners also help move our data to action and use it to support their own efforts.

8. CP Daily Living: One of our followers commented that she didn’t understand why CDC would focus at all on the CP community since it isn’t a disease. Can you comment on this?

Dr. Marshalynn Yeargin-Allsopp: The mission of the CDC’s National Center on Birth Defects and Developmental Disabilities is to promote the health of babies, children, and adults and enhance the potential for full, productive living. Whether one considers CP a “condition” or “disease”, our ultimate goal to help parents, doctors, educators, and other key stakeholders understand more about CP so that improvements can be made to help children and families affected by CP.

 Additional resources:
Learn more about CDC’s most recent findings on CP

Read the 2013 ADDM Network Community Report on CP

Listen to a podcast in which Dr. Marshalyn Yeargin-Allsopp describes the causes, preventions, types, and sign and symptoms of CP

Download a free checklist to help track your child’s developmental milestones