Recently, Paige added a new diagnosis to her resume…
*Drum roll please*
CORTICAL VISUAL IMPAIRMENT!!!
Cortical Visual Impairment or CVI as I will call it, from now on, basically means disturbed or reduced vision due to brain abnormalities. According to the large packet of literature I acquired at our appointment, CVI is the fastest growing visual impairment diagnosis today. Due to the fact that Paige’s brain is almost completely smooth, this diagnosis was kind of a no brainer. (I apologize for the reeeeally bad joke! Not my best work but we like to have fun around here!)
So, what does this diagnosis mean? Well, a few things! We know that Paige can see. She’s responsive, smiles socially, knows her name, and as we can tell, is aware of her surroundings. Sure, she can see, but we just don’t know how much she can see. Her vision therapist (who I will talk more about later) explained it to me like looking at a Magic Eye Puzzle. When you first look at one you see a bunch of jumbled colors and it appears to be blurry. After fixating on the page for 10, 20, 30, or however many seconds, an image appears. It makes sense because when you dangle an object in Paige’s range of peripheral vision, it usually takes her a few seconds to turn her head to it. What we have to do is to train her brain how to see.
One of the first red flags on Paige’s road to her Lissencephaly diagnosis was her vision. At her two month appointment, her pediatrician couldn’t get Paige to fixate on the light pen and she sent us to see a pediatric opthemologist. Her eye exam came back normal but was still lacking appropriate vision for her age. This sent us to get an MRI done and you know what came of that. Early Intervention services were immediately called and one of her therapies was Vision.
Let me tell you, Paige’s Vision Therapist is a Godsend! I don’t use names in this blog but if I did I would shout his from the rooftops!! Upon the initial visit, he was the first person to give us hope. From the very beginning he explained what CVI was and how Paige demonstrates all of characteristics. He told us that doctors don’t know everything and Paige will be the one to tell us what she’s capable of. Mostly, he talked to us like a friend and I appreciated that. We see him once a week for an hour at a time and I find this therapy to be the most intriguing. Since he has been working with her for over 6 months as if she already had a CVI diagnosis, the outcome of our CVI appointment was a pretty good one and I couldn’t be happier about it!
Her appointment was actually an evaluation with CVI Resources at West Penn Hospital in Pittsburgh. It was conducted by two doctors that are a husband and wife team. One of the doctors, the one that did most of the evaluation, is a trailblazer in CVI research and actually wrote the criteria for the test and the other was a highly respected pediatrician! Again, if I could name names (you could probably find them on a Google search), these two would also be shouted from a rooftop! They were incredibly personable, extraordinarily educated, and they had the most jovial and comforting personalities. The examination took about an hour and was completely non-evasive. Actually, it was the most entertaining appointment to date!
Visual Field Preference
Difficulties with Visual Complexity
Light Gazing & Purposeful Gaze
Difficulty with Distance Viewing
Atypical Visual Reflexes
Difficulty with Visual Novelty
Absence of Visually Guided Reach
Paige demonstrates all 10 of these visual and behavioral characteristics associated with CVI. HOWEVER, there is good news here!! There is a range of vision from 0-10 indicating how much she can see. 0 would be no vision and 10 would be perfect (it’s super rare to have perfect vision). There is a lot of criteria that goes into this range but as a frame of reference, most children with Lissencephaly initially range between 0-3 on their first visit and Paige was almost a 5! That might not seem like much of a jump but it’s actually hugely significant! It means that through her Vision Therapy, we have been successfully retraining her brain in our goal to appropriate vision. We will go back in about 6 months to get her reevaluated with hopes of more progress. I’ll keep you posted!
Let me say, Andrew and I are extremely thankful for all of the services, resources, and opportunities we have to Paige in Pittsburgh and are so humbled by the amount of support we’ve received from all over. Please know that your prayers, well wishes, and thoughts are all appreciated and we take them very much to heart. Our Paige is one loved little girl!