Story
Uncovering CVI at six months
My daughter was born with a hematoma on the left side of her brain, resulting in CVI. This is how we got help and embraced the journey.
Sweet Savannah Mae is my beautiful nine-year-old daughter. She is a rare gem, a true diamond in the rough with the most amazing sparkle. She has many diagnoses, including Cerebral Visual Impairment/Cortical Visual Impairment (CVI), a rare genetic mutation known as NAA10, epilepsy, cerebral palsy, severe developmental delays and more. Oh, how I love everything about her. My journey in life is led by my little girl. She is paving the way and my biggest inspiration. She is my tiny little teacher, and I am truly blessed with her life lessons.
On the day Savannah was born, my heart filled with the fiercest kind of love while my mind grew concerned. I first laid eyes on her while I was lying in a hospital bed about ten feet away as she was held up by one of the nurses before she was whisked away, requiring continuous positive airway pressure (CPAP). Savannah was born with a large hematoma on the left side of her brain, resulting in hydrocephalus and an extended stay in the NICU.
Massachusetts General Hospital (MGH) is where she spent her first six weeks of life as I took every opportunity to bond with my beautiful little girl, rarely leaving her side. Savannah was rarely free of tubes or wires. She had multiple MRIs, ultrasounds, spinal taps, and at four weeks old, her father and I handed our delicate warrior to a neurosurgeon who placed a VP shunt to treat her hydrocephalus. Nearly seven hours later, Savannah was returned to us with a heart-shaped bandage over her abdomen and hooked up to all kinds of machines. I was afraid to pick her up, as I might hurt my fragile little warrior, asking a nurse if she could bring her to me. It was a very scary day, one that will never be erased from my memories.
Following her release from the NICU, we had countless appointments with specialists providing care to our daughter, and we immediately started Early Intervention services. Initially, her team consisted of a social worker, who also served as a developmental specialist and a physical therapist. It was such a busy time trying to juggle my new life as a “medical mama,” but this was quickly becoming my “normal.” Motherhood was so new to me.
Near the age of six months, her Early Intervention social worker started having concerns about her vision. As she put it, “It’s like the lights are on, and nobody is home.”
I can honestly say, I didn’t have real vision concerns at the time. I related her downward gaze, love for lights, lack of eye contact and lack of visual curiosity to her hydrocephalus. After all, she loved looking up at the mirror in her swing. During feeding, she didn’t require any prompting to open as the spoon moved toward her mouth. We referred to the lights in our home as her friends so clearly she could see the lights. However, we agreed to follow up with a local pediatric ophthalmologist who reported that her eye health looked great but was very concerned, so he referred us to a specialist at Boston Children’s Hospital.
How our CVI journey started
On February 1, 2012, when Savannah was eight months old, our journey with CVI began. Savannah had another normal eye exam. She was a bit farsighted and had an astigmatism, but her ocular health could not be explained by her visual behaviors. The specialist in Boston said, “She has cerebral/cortical visual impairment. We will register her with the Massachusetts Commission for the Blind and Perkins School for the Blind.”
I tried listening, but all I heard was “blind,” not truly knowing a single thing about visual impairments and truly not understanding how this could be. She had already been through so much in her short life.
“I tried listening, but all I heard was ‘blind’…” – Lacey Smith
The visit concluded with a prescription for glasses. Savannah was prescribed glasses to treat her farsightedness and astigmatism, not for her CVI. She was getting glasses!! At eight months!! I couldn’t decide whether to dance in excitement with the thrill of a new accessory or cry because she was a baby, and I didn’t know a single baby that wore glasses. I do have to admit she looked adorable in her first frames that were entirely too large for her angelic chubby face. It also amazed me that the number one question people asked me was, “Are her glasses real?” I always wish I would have responded to at least one person by answering, “No, they are just a fun accessory, aren’t they cute?”
I can clearly remember the ride home. I called my mom sobbing to the point that she couldn’t understand anything I was saying. Savannah’s dad Mark was clearly emotional as he hit the side of the neighbor’s house, pulling into our narrow drive. The tears continued for a week. And then, suddenly, I decided to embrace our journey.
Seeking help and embracing the journey
The same month that Savannah was diagnosed with CVI, we met her first vision teacher, Mary Sparks. Mary worked as an infant-toddler teacher through Perkins School for the Blind. She came into our lives as we were navigating this new diagnosis offering support along with strategies of how to help our daughter learn to use her vision. She gave suggestions on environmental strategies as well as adaptive strategies. She was always reminding me to allow Savannah time to visually locate the shiny pompom or other objects that Mary presented. Mary brought a little desk and chair made just for Savannah, which we faced against a black tri-wall poster board and presented her with solid objects paired with light. And again, Mary reminded me, not to talk while Savannah was trying to visually locate an object. She set us up with many tools to be successful in our home, and I worked with Savannah daily.
Not everyone gets to Phase III
I ordered the book Little Bear Sees and immediately started reading it. I really enjoyed the book, but one thing that still gets me is something I read: “Of children with CVI who had highly motivated parents, 97% went from Phase I to Phase III in an average of 3.7 years.”
I believe Savannah’s Early Intervention providers would call me motivated. I also believe that I’ve always been motivated to give her the best opportunity to reach her potential. I do, however, realize that she has a unique profile, and her brain is very unique. I also can tell you, her vision is challenging, and while she has made great improvements due to neuroplasticity, she is not in Phase III. However, she continues, even at nine years old, to visually improve and integrate meaningful aspects of her world. She has a unique brain, and she checks many red flags for CVI including hydrocephalus, a hypoxic event, periventricular leukomalacia, epilepsy, along with rare genetic mutation.
Savannah received Early Intervention services in our home until she turned three. During that time, Mary continued to encourage me to join the infant-toddler playgroup at Perkins School for the Blind. I came up with every reason why this wouldn’t “fit in our schedule,” but finally, after my Savannah turned two, I decided I would like the opportunity to meet other families with medically complex visually impaired children, and that was one of the best decisions I have ever made. I gained so much support from families similar to mine. When Savannah turned three, she transitioned from the Early Intervention setting into a school setting on an individualized education program (IEP). I had told Mary for years that I was NOT going to send her to school, but when it became time for my little girl to spread her wings, I felt sending her to school was the best decision.
Navigating schools
Savannah started school with an out of district placement. I advocated for this knowing she was very complex, and she required unique individual instruction, always concerned that her visual needs would impact her learning. However, I wanted her to have typical peers in her class. She did well the first year, but during her second year of school, she became more frustrated with auditory sensory stimuli and engaged in self-injury (banging her head and biting her hand). As her mother, I knew we needed to look into another learning environment.
Savannah is now in a private school setting in a small controlled environment, which is exactly what she needs. The hardest part about this is that she doesn’t have the opportunity for developing peer interaction (never my first choice), but it allows her the opportunity to learn.
How Savannah’s CVI changed my career path
When Savannah was four, I started the University of Massachusetts vision studies program to become a teacher for students with visual impairments (TVI). I am currently in the CVI certificate program, and I love learning more and more about CVI. I absolutely love my job. I work with infants and toddlers with visual impairments and help support families as they begin to navigate their journey raising a child with a visual impairment. It is a rewarding career, and I love working with the special needs community.
Savannah continues to make “inchstones”
Savannah presents with a lot of complex needs. In addition to being visually impaired, she is nonverbal, non-ambulatory, she has seizures, and well, she’s just really complex. This makes learning challenging but not impossible. She continues to make “inchstones.” She is moving forward in the right direction. As her mom, I always push for vision first. Visual presentation is so important for her learning. If you give Savannah a choice between two real objects, she is likely to reach before looking, or locating the objects in her peripheral fields. While it is great she is noticing, she is not receiving enough detailed information about the objects presented. However, if you present her choices at an arm’s length away and provide the slightest movement, Savannah will look before reaching, sometimes looking away and sometimes using visual movement. She requires a multisensory approach and repetition to truly understand her world.
Savannah recently trialed an eye gaze system, and she has been showing success with identifying 2D photographs of highly familiar real objects. It’s been wonderful to see. Savannah is motivated by food, so she has had the most success with visually identifying her fork, cup and several familiar snack choices. This visual improvement is due to repetitive exposures in a controlled setting and due to the neuroplasticity of her brain. Savannah also continues to work with switches, visual and tangible symbols, and real objects to support her multisensory learning style.
Savannah works very hard. She makes me extremely proud, and she has taught me the most important lessons in life. She has taught me to accept, to include, to be hopeful, not to sweat the small stuff, but mostly to love because love truly wins. She is an amazing little girl with the best personality. She is very sweet, yet she is feisty enough to be a true warrior.
Embrace the uniqueness of your own CVI journey
So if you are new to this journey, hang in there. There will be bumps along the way, but the rewards will outweigh any challenges. Embrace the uniqueness of your journey because every child is so unique, and they have so much to teach us about life. Always see the ability.
“One day, you will tell your story of how you’ve overcome what you’re going through now, and it will become a part of someone else’s survival guide.” -Unknown
Lacey Smith is the mother of Savannah, now nine years old, living in Massachusetts. She is an itinerant TVI in the Infant-Toddler program at Perkins School for the Blind. Lacey will graduate from the CVI Certificate Program at UMass Boston in the Fall of 2020. In her spare time, Lacey likes to spend time with her daughter bike riding, visiting her family in Kansas and cooking tasty treats.
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