Touching lives... one smile at a time

Even without the use of language, Alex VanRay can "say" more with a smile, hug and an exuberant yell than one could possibly imagine.

The son of Matt and Jane VanRay of rural Pingree can't help being the gifted dispenser of joy that he is.

"Whether it's at a ball game, at church or at the grocery store, Alex seems to seek people out and give them a wave, laugh or big hug," says Jane, his mom. "It seems like so many people know Alex and help look out for him."

Alex lives with a condition called Angelman Syndrome (AS), which is named for a British pediatrician, Harry Angelman, who first described the syndrome's symptoms in 1965.

AS occurs in one in every 12,000-20,000 births, according to studies published by the National Institutes of Health. It is caused by a faulty gene called UBE3A on the mother's side, which is responsible for brain development, speech, and learning.

While signs vary from person to person, those diagnosed with AS consistently display the following symptoms:

• Functionally severe developmental delays

• Speech impairment, with little to no use of words

• Lack of balance, or ataxia (gait abnormality), and

• An atypically happy personality with frequent laughter and/or smiling, easily excitable, and short attention span.

So, one could say the joy he brings to people comes naturally.

"Alex was always considered to be the happiest kid around," says Jane. "As a baby, he never cried. He was so content playing wherever he was, and he still has one of the most infectious smiles and laughs."

"A very tough pill to swallow"

Alex was born on August 14, 2005, the first of Matt and Jane's four children. He has two younger sisters, Kaydence (16) and Kenzie (10), and a younger brother, Garrett (12).

His mother described Alex's pregnancy and delivery as normal, but through his infancy and early development, he had subtle symptoms that didn't directly raise a red flag.

"He had difficulty latching on, microcephaly (small head), reflux, and torticollis (a condition in which the head becomes persistently turned to one side), along with difficulty eating solid food," she said.

By nine months, Jane says he was beginning to miss bigger gross motor milestones like not rolling over and sitting up.

Matt and Jane decided to put Alex in Early Intervention, and began therapy sessions through Anne Carlsen Center and their pediatrician, Myra Quanrud.

"After about six months of that, we did initial investigating for an underlying cause with minor abnormalities, but nothing that stood out," she said. "We were told that he would catch up."

At 22 months of age, Alex's doctor diagnosed him with AS based on the signs he was exhibiting. With genetic testing, a "rather significant" deletion on his 15th chromosome confirmed what the new parents had feared.

This came as a shock to Matt and Jane, who had just welcomed Kaydence to the family three months prior.

"In some ways, a diagnosis was a relief, as we learned that we were already doing everything we could do," Jane said. "But overall, it was heartbreaking, for learning he would have a lifelong disorder, that nothing could be done to help him, and to learn that your dreams for him would be rerouted."

While Alex was making some gains on skills, he wasn't closing the gap relative to other children his age, so the parents knew that something more was going on.

"During our appointment, the doctor said, 'He will never walk, he will never talk, there is no cure, and there is no treatment. Take him everywhere with you, and hope for the best."

Jane said that recommendations for Alex were to "get him involved in as many things that your family can manage" through therapies and activities, and he would need help and support for the rest of his life.

"Honestly, it was a very tough pill to swallow," Jane said. "Like all other difficulties in life, time and our faith in God has helped our family continue to be stronger and keep moving forward."

Daily battles with AS

The VanRays say that some of the biggest challenges Alex faces as an AS patient is sleeplessness, along with seizure control, but the hardest part would be the unknowns inherent with his behaviors.

"Imagine seeing your child's face light up, but you don't know the reason. Imagine having your child come home from school and never being able to tell you what they liked or didn't like. That's our reality, every second of every day," says Jane. "We try to do our best by reading his emotions and reactions, and that is all we can do."

In recent years, Matt and Jane have enlisted the help of a specialist from Family Voices to help them through Alex's guardianship process.

"Our respite care providers have probably been the most helpful," Jane says. "They're like the family we never knew we needed.

Since the age of three, a husband-and-wife team has worked with Alex to provide therapy sessions while Jane was focused on family tasks, but she says it grew into a relationship and a bond that they never imagined.

"They've been there in the darkest times, and celebrated in the tiniest moments," she says. "As a parent of a child with complex communication or medical needs, having a person who can step in and keep him safe to give you a break, truly is priceless."

Matt and Jane say that even though Alex is nonverbal, doesn't mean he's non-thinking.

"The Angelman Syndrome disrupts the messaging process in Alex's body," Jane explained. "Signals get lost in transit, so to say. In turn, he has a difficult time controlling his muscles, so motor skills like walking up steps are very hard."

Filtering out the noisy world around him is also a challenge, and a possible avenue for communication, sign language, is too challenging for him, she says.

"We're truly guessing as to what Alex is really thinking or feeling, but I wholeheartedly believe that he understands everything we say."

Alex is very sensitive to touch and displays aversion to anything on his head or neck, and does not wear glasses, hats, or neckwear.

So, it was a surprise when he tolerated Bluetooth-capable headphones for his iPad that he uses to focus and not be overwhelmed in large crowds or loud situations, such as his siblings' basketball games.

"He has a communication program on a different iPad that he is learning to navigate. At times, he can use this to make some simple choices," Jane says.

His play tablet is strictly for pleasure, and often times, his entertainment choices mirror what's going on around him.

"If he's at a basketball game, he'll watch basketball on YouTube. If it's softball, it's softball," she said.

While at school, a delivery system was developed for Alex where he delivers items from Happy Shack to CPS staff three times a week.

"He goes, picks them up on a cart, and delivers them to the appropriate person. They use picture cues to help him navigate the school," said Jane.

He also has delivered flowers from Petals and Stems, and other various items around campus.

Alex's support system

The VanRays have forged a bond with other parents of Angelman kids through online support groups.

In 2009, Matt and Jane attended their first ASF (Angelman Syndrome Foundation) biannual conference, and they also try to make events held by the Foundation for Angelman Syndrome Therapeutics (FAST).

"It was the best thing we ever did for helping us navigate Alex's life, and we share a connection that only someone who walks the same path can understand," she said.

The Carrington School District has done a good job of tailoring Alex's education to meet his needs, the family says. As in life, it's been a process of trial and error, with some aspects done well and some needing room for improvement.

"Overall, they've always upheld Alex's best interest," says Jane, who added that they've been receptive to some "zany" ideas that they've brought back from conferences.

"Not all Angelman kids have been so lucky, and have had an uphill battle the whole way," she added.

But most of all, the biggest supporters in Alex's corner are his family.

"Before we got married, Matt and I wanted to be close to our families," said Jane. "They're literally only a few minutes away, and a good number of his family and mine are close as well."

Having a sibling with AS has given Kaydence, Garrett and Kenzie what the parents call a "front row seat" to building kindness and compassion.

"The world can be quite cruel and embarrassing to someone who is different, and I've watched them shape themselves into becoming caring and well natured individuals," said Jane.

The future

This spring, Alex will walk the stage as a member of the Class of 2024 at Carrington High School, but he will remain in the school district until the age of 21.

"He's developed a special relationship with his classmates," Jane said. "We're still navigating as to what is the best plan for Alex, as one day he'll be moving to a different place of residence."

"We know he'll need a lot of support along the way, and when the time is right, he will let us know."

For more information on Angelman Syndrome and research on treatment and finding a cure, visit angelman.org.