Your stories – Cortical Visual Impairment

summer 2008

summer 2008

My name is Tobi and I am the mother of a beautiful, energetic and vision-impaired daughter, whom I will call Z.  This is the story of how we came to learn about Z’s vision impairment.  Unlike most of the visual issues you read about on this site, Z’s primary vision problems lie not with her eyes, or even with her optic nerves, but with her brain.  Her condition is called Cortical Visual Impairment (CVI), and according to at least one source it is the most common cause of permanent visual impairment in children.

Oh, and one more thing: children with CVI are frequently misdiagnosed as having autism spectrum disorders or mental retardation.  This is not to say that autism and/or retardation are never found in children with CVI.   It just means that often the symptoms and characteristics of children with CVI are confused by clinicians and therapists, and the children receive the wrong (or at least incomplete) diagnosis.
My husband and I adopted Z when she was 2 ½ months old.  She was rather small, but not premature.  We had no genetic or pre-natal information about her, other than that she came from an extremely poor country and probably did not get good prenatal care or nutrition.  Aside from a bad stomach bug when we first adopted her, she had no health problems.  During our first few months with Z, I often wondered if “something was wrong” – I couldn’t really put my finger on it, but she just seemed out of it most of the time.  She didn’t connect with us the way our son did when he was born.  She rarely smiled (except when you pretended to toss her in the air).  She rarely made eye contact.  She didn’t reach for objects or show much interest in the world (except in her big brother, who was about 3 at the time).  She didn’t roll over, and didn’t try.  She hardly engaged visually with the world, although when we’d carry her from room to room, or into a new place, she’d cock her head and listen intently.  For those first few months, we assumed that the trauma of adoption, even at her young age, explained these behaviors.

I remember on a few occasions wondering out loud to my husband, “I wonder if she can even see!”  But then she’d take visual notice of something – a spoon full of baby food heading toward her mouth, her brother jumping around and making funny faces at her – and I’d dismiss the idea that she had a vision issue.  To be honest, I was kind of hoping that she was blind, because that seemed a better alternative than cognitive impairment or severe attachment disorder or autism.  But the pediatric ophthalmologist said that although she had some visual acuity issues and strabismus, and she may need glasses, her eyesight was actually not bad.

2008-summer-203

Z in summer 2008

At about 13 months, she got glasses (so cute!) and a patch.  The glasses didn’t seem to help much, but soon she started walking (never really crawled), and a very lively personality started to emerge.  She got wonderful therapy and special ed services through the local school system.  All the while, I’m still thinking that something is really odd with her vision, things just don’t register with her, but the ophthalmologist, neurologist and pediatrician all just sort of shrugged when I would mention that and said it may simply be a cognitive thing (as in:  get used to it, your daughter is retarded).  And by the way, she never took to her glasses, which to me indicated that they weren’t really helping much.  In the meantime, Z was falling further and further behind her peers in just about every developmental area.  Although her personality seemed bright and curious, she never seemed to learn anything.  She couldn’t even feed herself.

When Z was about 2 ½ her special ed team said they wanted to bring in a vision specialist from the school system to evaluate her.  It took that woman (that WONDERFUL woman) about 20 minutes of observing Z before she said:  “I think your daughter has Cortical Visual Impairment.”  She proceeded to tell us what this is, which of our daughter’s behaviors she had seen that made her think this, and speculated (correctly) about other behaviors that she hadn’t witnessed but suspected appeared from time to time.

What a thrilling moment.  Someone finally said to us:  This is what is wrong with your daughter, it is a recognized condition, and even though it might not be curable, we can help her adapt and we can help her learn.  Hallelujah!

A couple months later, on our annual family road trip to the East Coast we stopped in Pittsburgh to see a CVI specialist named Christine Roman-Lantzy, PhD.  Dr. R-L had developed the diagnostic tool that is used to identify CVI and has written extensively on the topic.  During a 2-hour visit, during which Dr. R-L and a pediatrician observed and interacted with Z (and enlisted big brother’s assistance), she confirmed Z’s CVI diagnosis and talked to us more about CVI.  We had already read a lot about CVI by that point, but it was such a validating experience to have a conversation with someone who really understood, even more than my husband and I do, how our daughter experiences the world.  (I’m sure there are other good specialists out there, but we were extremely pleased with our visit with Dr. Roman-Lantzy – a very warm, caring and child-oriented professional.)

Summer 2008

Summer 2008

The diagnosis hasn’t solved our daughter’s vision impairment, but it has helped us (and her teachers) work with her.  We have learned that some characteristics of CVI may resolve over time, others will likely not, and so the lovely Z will probably go through life as a vision-impaired (and possibly legally blind) person.  We’ve been advised she should keep wearing her glasses (which she still protests), since she does also have an acuity issue – the glasses don’t help the CVI, but they at least improve somewhat the layer of blurriness that surrounds what Z does see.  We also don’t know if Z has a cognitive impairment, or if her delays are mostly attributable to her visually-limited and visually-confusing experiences to date.  Time will tell on that score.

Now, whenever people ask me about Z’s glasses, or comment on her constant motion, I tell them about her CVI.  I want people to at least have on their radar (unlike our own supposedly savvy medical specialists) the possibility of CVI for a child who “just doesn’t seem right.”  Here is what I’ve learned about CVI (in lay terms):

Children with CVI have a neurological disorder that can be caused by many things, including traumatic head injury, poorly developed brain, infection or lack of oxygen to the brain.  It used to be called cortical blindness.  It shows itself through ten (give or take) unique visual/behavioral characteristics:

  1. VISUAL LATENCY – that is, the person does not immediately respond to visual input – they can look right at something, but it doesn’t “register” right away.  For example, Z will gaze at a picture in a book for several seconds before she can recognize what it is.
  2. LIMITED VISUAL FIELD – the person’s field of vision is limited, and apparently in CVI certain locations of field loss are more common than others.  In Z’s case, her field limitations appear to be lower and left, so she prefers to look right, trips over things that are on the floor and bumps into things on her left.
  3. COLOR PREFERENCE – the person tends to show a preference for one color, and a preference for color over black and white.  We haven’t seen this so much in Z, except that she never showed any interest in those bold black and white baby books/graphics.  (I feel so bad – all that time I kept thrusting those books at her, and it probably just annoyed her!)
  4. LIGHT GAZING – the person tends to gaze into lights or shiny objects.  In Z’s case, it’s mirrors.  She could sit all day in front of a mirror; she likes to eat, read and play looking into a mirror.  Or anything with a reflective surface, like a dark oven window!
  5. PREFERENCE FOR MOVEMENT – this is such an interesting one, and really helped explain some of Z’s behaviors.  Unlike most children, kids with CVI are more visually attuned to objects that move rather than those that are stationary.  So, they might not see a ball lying in the grass, but might see a ball rolling across the grass.  This may be why Z seemed to “see” her big brother (zipping around like a typical pre-schooler) better than she saw her parents (who tend toward the couch potato variety).  This might also explain why Z is constantly on the move.  She just does not sit still.  Ever.  Her vision specialist thinks that if she’s in motion the objects around her might also appear to be in motion, and therefore more visible to her.
  6. VISUAL COMPLEXITY ISSUES – a person with CVI has trouble picking individual items out of a crowded visual field.  For example, if a spoon is on a highly-patterned placemat, Z will probably not see it.  If the spoon is on a solid-colored placemat with nothing else on it, she will see it.  This makes looking at books, faces, plates full of food, anything with more than one part, very difficult.  When I heard about this, so much made sense to me:  Z was always able to gesture toward my face when I asked her where my eyes (or nose, mouth, etc) were, but now I understood why she never could point to them.
  7. VISUAL-MOTOR DISCONNECTION – this is a hard one to explain, but it basically means that a child with CVI will often spot an object, then look away from it, then reach for it.  They tend not to rely on their vision to guide their actions or to coordinate their vision and actions.  The ultimate poor eye-hand coordination, but with a weird twist: Z has an uncanny ability to throw a ball accurately without looking at the person she’s supposed to be throwing at!
  8. INDIFFERENCE TO VISUAL NOVELTY – most kids are attracted to the thing that’s new or different.  Kids with CVI respond better to visual cues that are familiar.  They need to spend a lot of time with a new picture or other item before it really registers with them.  In Z’s case, she is curious about new people, but not new detail around her.
  9. UNUSUAL (POOR) VISUAL REFLEXES – doesn’t blink or look away from visually threatening movement or objects.  This is apparently something that has resolved pretty well for Z, because she does seem to have generally appropriate visual reflexes.
  10. NEAR-SPACE ATTENTIVENESS – a child with CVI is more likely to prefer to look at things very close to them, even if they are far-sighted.  When the TV is on in our house, Z puts her face right up to it – about 3 inches away.

Some other things we learned:  most kids with CVI have normal or near-normal visual acuity.  Also (but this is somewhat controversial), their visual preferences and behaviors may change from day to day, which could lead teachers and parents to believe that a child who is attentive and engaged on one day but not the next is simply “lazy.”  From my own observations, it seems to me that there are a lot of variables that play into Z’s visual engagement with the world – whether there is a lot of noise or other sensory input in her environment, whether she is tired or not feeling well, whether she feels successful or frustrated, etc.

Finally, people used to think that the brain doesn’t repair itself, especially with respect to vision.  But new research indicates that many parts of the brain support a person’s vision, and if you give them the opportunity – if you train the young brain to process visual input through other pathways – then you can positively change the outlook (so to speak) for the child with CVI.  Z is now 3, and we continue to work with her with the hopes of improving – or at least helping her adapt to – her unique view of her world.

So, that’s our (LONG) story!  I hope you learned something, and I welcome any comments or shared CVI experiences you may have had.

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15 responses to “Your stories – Cortical Visual Impairment

  1. Thanks, Tobi, for writing this. Though we have heard bits and pieces of Z’s medical odyssey, this has helped me understand it so much better. Z is a gorgeous little girl and so full of fun. Looking forward to getting the kids together soon.

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  2. Thanks so much for sharing your story. Our daughter is much younger than Z, but has many of the same visual traits and challenges-we have a preliminary CVI diagnosis. We’re hoping to visit Dr. Roman someday. You’ve summarized a lot of overwhelming information in a very readable way. Your positive attitude is inspiring too!

    Wishing all the best for you and Z and the rest of your family. Have a peaceful and healthy holiday season!

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  3. thanks for sharing your story on Z’s CVI. My daughter also has a CVI disorder and i have been working at it and other medical issues as well. it helps to hear others with the same fight that are battleing it out.God bless Z and your family

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  5. Thank you for writing such a detailed entry! I enjoyed reading about your daughter. We just found out that our grand daughter has some sort of visual impairment and two of her therapists have mentioned CVI but we don’t have that diagnosed at this time. We are restructuring our home and how we approach teaching her and she is starting to respond. I can relate to everything you said, and now I’m even more suspicious about Simone having CVI.

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  6. This was a wonderful account of living with a CVI child. My son is only four months old, but they have already given us a preliminary diagnosis of CVI. We have an appointment with a neuro-ophthalmologist and a pediatric ophthalmologist in the upcoming months. I have read some of Dr. Roman-Lantzy work and was interested to know that you were able to get an appointment with her. I would love to speak with you via email about your experiences.

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  7. My son is five. Everything that we heard said that cases like my son’s were unheard of. (Being cognitively fine, just can’t see sometimes and can see others) All the research I try to do disagrees (with other research) so it is interesting to read your research.

    We are considering moving to an area that has a school for the blind so he can learn brail because we are thinking his eyes will tire easily as he tries to read the tiny print in secondary school. His local school just informed us he would only get a teacher here for two hours a day :(. All this makes me so sad, but it is good to know that my son is not the only child with cvi who is cognitively fine.

    We didn’t find out till they did an MRI (or CT scan.. I can never remember the difference) when he was 1.5.

    Anyhow thank you.

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    • Chessica –
      I’m glad you had a chance to read this. Sounds like your son is doing great – I hope you find the right school placement for him.
      I want to clarify something about my daughter (who is now 5 1/2, so close to your son’s age). We don’t really know if she is cognitively impaired or not. There is disagreement among her teachers, other caregivers, doctors, even us, her parents. She is clearly developmentally delayed, but it isn’t clear whether that’s simply due to her visual impairment or if there is also cognitive impairment going on. I don’t know if we’ll every really be able to tease that apart, and I’m not even sure it matters.
      But either way, there is no question in anyone’s mind that her CVI has impacted her ability to learn/develop, and it really wasn’t until we got the diagnosis and started making CVI adaptations/interventions in her learning environment that she really started to learn. No one believed me when my daughter was an infant and I said I didn’t think she could see (because clearly she sometimes could!). So I really applaud you for taking it seriously that he sometimes can see and sometimes can’t.
      I went to a workshop led by Christine Roman last week, and she talked about some schools where she has been impressed with how they work with CVI children. If you haven’t been in touch with her already, I would strongly recommend it. She is a great resource and is very generous with her time and knowledge. We’ve visited her in person twice, and plan to go again this summer, but I know she does phone consults with parents and educators, too.
      Here’s her contact info:
      http://www.cviresources.com/index.html
      All the best to you and your family,
      Tobi

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  8. Thank you for writing this story. I am an Early Childhood teacher and is presently studying “exceptional children” and had to do a research on “Vision Impairment”. Your story has help me to better understand what is cortical visual impairment (CVI) and the behaviour of the child.

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  9. Hi….I read the story because my grand son is a victim of cvi ,he is 24 monthes old…..how is your daughter now ?…..do you find growing up improves the condition..? Do you know any grown up person with cvi to explain for us what does he see exactly and how his sight changed since childhood till adulthood……thanks

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    • Hi Nariman. Thanks for your comment. I’ll take your easier question first: No, I don’t know any grownups with CVI (or at least no one I know has told me they have CVI). But I’ve learned about what the visual experience is for people with CVI from a few sources. First, Dr Christine Roman-Lantzy in Pittsburgh has lots of great information, in her book and in her articles (and a few visits with her in person) about what CVI-ers see and don’t see – or more precisely, how they see and process visual information differently. Second, when my daughter was first diagnosed someone loaned me a DVD that I think was put together by the Perkins Institute in Boston that “showed” how people see with different visual impairments, and CVI was one of them. Third, I spend a lot of time observing my daughter and that gives me clues about her visual experience. I wasn’t really able to do this until she was 4 or so, but now I learn a lot by watching her. For example, when I saw that she would frequently bump into objects (door frames, chairs, etc) I learned more about how bad her peripheral vision is, and which side is worse. Similarly, I read a lot to her, especially when she was younger, and I noticed that she was more engaged with certain books than with others, and from that I figured out what her color preferences were.
      So although I’ll probably never know exactly what and how she sees, I’ve tried to piece all this information together to give me a better idea.
      As for how she’s doing – well, in some ways, she’s doing great! And in other ways not so great. The hardest part is that she SO desperately wants to be able to read, and to keep up in other ways with other 9 year olds, but she simply cannot – we haven’t found the “key” to her literacy yet. Some of this has to do with some cognitive impairments, and some with her visual impairment. And of course this is very frustrating for her, which can present in negative behaviors. But on the whole, she is very healthy, very happy, extremely funny, and participates in life joyfully and as much as she can. Which is a lot to be thankful for!
      Best of luck finding good care and support for your grandson and family. I highly recommend a visit with Dr Roman-Lantzy, if that’s possible, or with someone that she’s trained. If nothing else, it’s great to get affirmation and additional information about what your little one is experiencing, and ways you can help.

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  11. Hi Tobi, Thank you very much for valuable details. we are from south asian county called Sri Lanka. my 1 and 2 months old son has undergone for brain surgery at the age of 3 for hydrocepalus. implant a shunt and diagnosis as dandy walker variant syndrome then he got a fit attack and did the shunt revision, at that juncture our doctor refer to the pediatric neurologist and she decide my son has a CVI , we got the appointment in visual rehabilitation clinic at the lady ridgeway baby hospital on 07/05/2015. we hope to update our knowledge before go to that clinic. thank you very much. if you have more information pls send me. my emai- chandima.ranasinghe@nationstrust.com
    Postal Address – chandima Ranasinghe, 628/2B/1, Kottawa, Pannipitiya, Sri Lanka.

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