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How Bruce creates joy while living with CVI and Angelman syndrome

What goes into nurturing a child with complex needs, a rare disease, and CVI? Bruce's mom Danielle sheds light on creating everyday joys.

What does it mean to live life to the fullest? Is it all about taking risks and embarking on adventures, or embracing the beauty of your surroundings and finding joy in the everyday moments? For 8-year-old Bruce, it’s the latter. “He has a very bright view of the world,” according to his mother, Danielle. Bruce, who has CVI and Angelman syndrome, both creates and enjoys the pleasures in everyday life. To Danielle, it’s a reminder to cherish the simple things, embrace the complexities, and express gratitude for all life has to offer. Bruce has a wonderful sense of humor and a smile that can light up any room. He enjoys all the opportunities life brings him, particularly swimming, going for walks with his dog, and lunch dates. He adores his brother Grady and loves being with his family, who ensure that Bruce is living his life to reach his fullest potential.

As Bruce was nearing his first birthday, he started demonstrating signs of epilepsy, which was the beginning of medically uncovering a rare and complex diagnosis of Angelman syndrome. Angelman syndrome comes with a variety of challenges, including epilepsy, developmental delays, nearly universal lack of speech, ataxia, sleep challenges, and CVI. Having a rare disease and CVI can be very intense and complex to navigate. 

After diagnosis

When Danielle and her family first learned of Bruce’s diagnosis, they sprang into planning mode — how do we give Bruce the best chance at reaching his fullest potential in life? That meant exploring therapeutic options and medication options, finding the right team of medical care, and asking the tough questions, like, “How can we ensure he’s cared for for the duration of his life?”

These aren’t easy questions to ask or answer, especially when seeking understanding from others and reimagining what life will be like for Bruce and his family.

CVIer Bruce walks along a bridge using his white cane.

Bruce had been under the care of an ophthalmologist from early infancy largely due to his strabismus, although it was always obvious to Danielle that Bruce had difficulty tracking toys and difficulty with depth perception. He received a CVI diagnosis shortly after his Angelman syndrome diagnosis, as CVI often accompanies Angelman syndrome. “Bruce learned to walk around the age of 4 and a half years old. Danielle shares, “We weren’t sure if this was in the realm of possibility given his diagnosis of Angelman syndrome, so it was such a beautiful, surprising moment, filled with so much gratitude.” 

Bruce still struggles with “navigating solid steps on a playground structure, almost as if he does not see the individual steps due to his difficulty with depth,” something that is commonly seen in individuals with CVI. Similarly, Bruce tends to neglect his lower field of vision and will often trip over an object in his path. Although he independently navigates in his home, Danielle says, “We are constantly giving verbal cues and moving things to the side out of his walking path to support his navigation.” Bruce also benefits from an open space with reduced clutter, lights over the stairs to the basement, and brightly colored yellow tape markings in “harder to navigate” areas. 

Other barriers to access for Bruce include difficulty with contrast sensitivity, visual clutter, visual field loss, and the visual guiding of his lower limbs. “Bruce can, for example, find a lightly-colored Cheerio on a dark surface, but If I were to put a watermelon piece on a red plate, he would not necessarily be able to see the watermelon on the plate,” says Danielle. When viewing videos on an iPad, Bruce will position his face directly over the iPad to accommodate his best view of the screen. “Bruce needs very individualized instruction and interesting items to engage him, knowing his peers with CVI may experience something very different.” 

Successful supports and strategies

Bruce has found success with meaningful and motivating instruction. He uses a white cane to support his navigation and benefits from a highly adapted and controlled environment. His learning materials are presented with high contrast and low clutter. Bruce learns best with hands-on learning materials that are motivating and engaging, such as shiny, textured, and crinkly materials. Science is his favorite subject, and he enjoys “cause and effect” learning, such as experiments that involve pouring brightly colored water into different test tube vials to see what happens. 

Danielle attributes his success to the holistic approach and support from his caretakers and support from his educators at Perkins School for the Blind. Bruce has also been incredibly lucky to have his grandmother, Mémé, provide him — not only endless love and care – but the benefit of her learnings from a 30+ year professional as a case manager and then Registered Behavior Technician (RBT) experienced with adults with various intellectual and developmental disabilities. She was the first to suspect Angelman syndrome, the first to identify seizure activity, and is his strongest advocate always pushing for more opportunity for Bruce in life. “Family and educator commitment, adaptability, and a learning mentality are absolutely life-changing for Bruce,” shares Danielle. 

Nurturing a child with complex needs is not easy or straightforward. “You need to be agile as a family, and this demands constant and ultimate flexibility. It requires vigilant, relentless observations on how to enter his world, insights into what he needs, and a better understanding of what he is thinking and feeling,” shares Danielle.  

Danielle and Bruce hug, smile and look at the camera.

Danielle muses that for others looking in, “our family life is really hard to understand. As parents, we are managing a lot that is not typical and is often very intense, extreme, complicated, or even medically sensitive. Others may think they have an understanding, but I’m not sure truly understanding is possible without living it.” For example, there can often be a lot of energy is used for what even seems like simple day-to-day tasks to maintain the operation of a family’s daily life when raising a child with complex needs. She attributes her most successful relationships to those who acknowledge or seek to understand the complexities that her family embraces, but also honor when they can’t understand and just respect that disconnect. “Agility and flexibility are needed for Bruce, and relationships need to be flexible, too. It can feel incredibly solitary at times,” she says.

Life became a lot simpler when I decided just to let some people misunderstand me.

-Alicia Lockhard

Finding joy

CVIer Bruce and his little brother Grady share a hug.

A quote attributed to Alicia Lockhard helps Danielle navigate all of this and often grounds her in the more difficult engagements: “Life became a lot simpler when I decided just to let some people misunderstand me.”

Bruce requires 24-hour hypervigilant care. The meaning of self-care may have once looked like a weekend getaway but nowadays, it’s simpler. “I enjoy self-care in small doses,” says Danielle. It may look like “visiting with a friend for half an hour or sipping on a coffee with headphones for ten minutes. Equally, we find special places that our entire family can enjoy, such as visiting Wards Berry Farm or swimming, and then lean into those moments.”

Finding the beauty in raising a child with a rare disease and CVI is cherishing those small yet meaningful moments — “glimmers” as they are sometimes called. Danielle shares, “I’ll never forget the moment Bruce met his younger brother Grady. He was enamored and leaned in to give a gentle kiss on Grady’s cheek and then turned to me and gave me a big smile and huge hug,” showcasing how his joy enlightens and enriches the lives around him.

Bruce’s future is bright, and he and his family face the challenges that Angelman syndrome and CVI bring with optimism, courage, and faith.

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