Power Play: Bryce and the Bears

by James Leonard, University of Maine Campus Newspaper

April, 2000

When you meet Bryce Hubbard, the first thing you notice are his eyes. Two hypnotically-sparkling sapphires that dance with mischief. Looking closer, you discover the subtle starburst pattern on each iris. This anomaly enhances the effect of capture, denying you the ability to look away. Connect with these eyes and you belong to this precocious 3-year-old and his curious gaze, a gaze that doesn't look at you, but seems to look into you.

What you don't realize is that contained within those eyes are one of the calling cards of a disorder known at Williams Syndrome. It's hard to think of this child as ill. He's friendly, active, attentive and unreservedly happy as he cavorts with his younger brother, Chase, on the Alfond Arena ice. But behind Bryce's inexhaustable enthusiasm is a battle, one that he will wage for most of his life.

Bryce is a good soldier and has accepted the challenge in earnest. It's only natural, he comes to the world of military stock. Both of his parents, Garret and Ann Hubbard, are former Marines. His father remains a Marine in the Independent Ready Reserve.

Bryce, as Ann explained recently, came into the world battling. "He was due in October of 1996, but we had to take him early because he had stopped developing. He spent 9 days in the Neo-Natal Intensive Care Unit because he was diagnosed with failure to thrive." Bryce was fed through a feeding tube for 4 days until he was able to nurse. Unable to gain weight, he was allowed to go home with the thought that he may improve in that environment. When the Hubbards took their son home, he weighed only 4 pounds. After 3 weeks at home Bryce was taken to Eastern Maine Medical Center in Bangor after a cyanotic (turning blue due to lack of oxygen) episode. At EMMC a pediatrician diagnosed Bryce with reflux disorder and detected two heart murmurs as well. With the reflux, Bryce's underdevelopment posed a unique problem. "Most kids with reflux tend to throw it [sputum] out," Ann noted. "Because Bryce's esophagus hadn't matured enough, the sputum was sitting in his windpipe and cutting off his air supply." He was given medication and upon returning home was forced to sleep in a sling at a 45-degree angle until he was 8 months old. Eventually the reflux disappeared and Bryce began eating well. Still, he wasn't gaining much weight. On his first birthday, he weighed 13 pounds.

Aside from his lagging physical development, Ann says she and Garret didn't suspect anything serious. "His growth was not typical, but he was growing and with all the blood tests there was nothing to indicate that there was anything wrong."

She explains the discovery of Williams was the result of a progression through the health care system. "The public health nurse was coming by every week and she noticed some developmental delays, so we had him tested. The tests indicated that there was developmental delay and they recommended physical therapy. When we got those referrals, the doctor told us that she saw some abnormal facial features and wanted us to see a geneticist."

During that exam, Bryce fooled the geneticist. Ann recounts that "the geneticist told me when he first saw Bryce, he immediately thought Williams Syndrome. But after examining him and looking at family pictures, he didn't believe there was anything wrong. Still, he said he wanted to do some tests to rule it out." The geneticist performed a blood test using a technique known as the fluorescent in situ hybridization (FISH) test. The result was positive for Williams Syndrome.

"We were devastated," admitted Garret. "It was probably more devastating because when the geneticist didn't think Bryce had Williams he told us a few horror stories about it." The Hubbards were referred to the Williams Syndrome Clinic in Boston where Bryce was evaluated and they received counseling on Williams Syndrome.

Williams Syndrome is a rare genetic condition that occurs in one of every 20,000 births. First recognized as a distinct entity in 1961, it affects males and females equally. Most individuals with Williams are missing genetic material on chromosome #7, including the gene that makes the protein elastin (which provides strength and elasticity to vessel walls). Some medical and developmental problems are probably caused by deletions of additional genetic material near the elastin gene on the chromosome. The extent of these deletions varies among those with the disorder. In most families the child with Williams is the only one to have this condition in his or her entire extended family.

Common features of those with the disorder are characteristic facial appearance including a small upturned nose, long upper lip length, wide mouth, full lips, small chin, and puffiness around the eyes. Blue and green-eyed children with Williams can also exhibit the starburst pattern on their iris. These facial features become more apparent with age.

Individuals with Williams can also experience heart and blood vessel problems, elevated blood calcium levels, low birth weight, low weight gain, colic during infancy, dental and kidney abnormalities, hernias, sensitive hearing, musculoskelatal problems, overly friendly personalities, developmental delay, learning disabilities and attention deficit disorder. Since many of these problems develop over time, it is important for individuals with Williams to receive ongoing medical monitoring.

The disorder affects individuals differently and the outlook for those with Williams ranges from heavily supervised care to independent living situations. Since Williams Syndrome is underdiagnosed or diagnosed later in life, early detection is vital to overcoming the medical, developmental and learning disabilities associated with the disorder.

True to their nature, Garret and Ann began assembling information and evaluating their options. "Ann really took charge," says Garret. "She dedicated herself to finding out as much as she could about Williams Syndrome." They were encouraged by the response from their health care providers. "Our dentist already knew about Williams," said Ann. "Once Bryce's doctor found out he had Williams, he surfed the net, did some research and found out more. I have been so pleased with the quality of care that Bryce has received."

Armed with a new awareness of why Bryce was underdeveloped, the Hubbards were faced with a choice. "If he didn't start gaining weight, they were going to put a feeding tube in," Ann recounted. "That meant invasive surgery, opening up his tube in the middle of the night and pouring high calorie formula into the tube. We decided to find another way to help Bryce gain the weight. That's when we went to the women's hockey team." It was a move that would make a significant impact in a number of people's lives.

University of Maine women's hockey coach Rick Filighera describes Ann's approach. "Late last summer, Ann came into my office and we sat for an hour while she explained Williams Syndrome to me. When she asked if Bryce could skate with the girls, it was a no-brainer. He met the girls and they fell in love with him. Since then, he's a fixture here at the rink and a part of our team."

Twice a week throughout the hockey season, around noon, the women of UMaine hockey skate with Bryce. The effect has been profound on everyone.

"He's given me new insight on life," sophomore goalie Mandy Cronin says. "He loves life so much and that makes me think about what's really important." Brie Layman, a sophomore on Maine's defense corps is one of Bryce's favorite people. Seeing Brie recently he shouted her name and rushed, smiling, to her side. "He's such a cute kid," gushes Layman, "I'm really attached to him. At first he was a lttle shy, but he picks things up quickly and that's not a problem now." Filighera adds that "this has really got nothing to do with hockey. It's about life. These girls are learning things they can't get in a classroom. What makes it really great is the people involved."

The arrangement has paid big dividends all around. It's really unified our team," noted Cronin. "I think everyone has skated with Bryce and we just look to him for inspiration." Layman echoes the sentiment. "He's really helped bring our team closer. We share a common bond with Bryce. He's a big part of our lives and our team."

Bryce has also seen the benefits of his new exercise regimen. "Before he started skating, he couldn't climb up the stairs and he was a little shy around people," noted Ann. "Now he makes it up and down the stairs on his own with no problem and he's really come out of his shell." She smiles as Bryce carries on a conversation with a group of players out on the rink. Garret agrees that the skating has helped. "It's always hard for us to tell what is normal developement and what isn't. But skating with the girls has definately helped Bryce. He's much more active and outgoing."

As Ann watched Brie, Mandy and Nicole Munro skate and fawn with Bryce, she reflected on what the arrangement has meant to her. "These girls give me so much hope. They don't have to do this. Their athletes and college students, I know they're really busy. But they're always here and always so happy so be with Bryce."