From The Holland Bloorview Kids Rehabilitation Hospital Foundation
“Jacob now has a voice thanks to Dr. Tom Chau and the amazing team of researchers who work with him.” – Marcy, Jacob’s mom
If you want Jacob to bust into giggles, tell him a joke that involves, um… flatulence. Or just sit on a whoopee cushion. That should do the trick. You may want to. His giggle is infectious.
Goofy antics take a back seat when he’s in Holland Bloorview’s pool, however. With the freedom to move and appropriate support for his neck, Jacob now swims more than 100 meters.
“He is happiest in the water when he can move independently,” says his mom Marcy.
Jacob, who is nine years old, has Pelizaeus-Merzbacher Disease (PMD), a rare disorder caused by faulty myelin. Myelin is a fatty covering that insulates nerve fibres in the brain. As a result, Jacob’s vocal cords are paralyzed. Although he can make sounds, he is unable to protect his airway from food or drink. PMD also inhibits his movements. As a result he is unable to walk or clap his hands. But he finds ways to adapt so he can enjoy favourite pastimes like strumming and plucking the guitar.
Jacob is a very bright boy. “The worst thing is when people underestimate him,” says Marcy.
Both she and her husband, Andrew, have worked tirelessly to find a drug that would improve neurological function in those who have PMD. At this point there are no known treatments for this rare disorder.
While Marcy and Andrew are actively pursuing a treatment for Jacob, they also want him to be able to effectively communicate his needs and wants. For most of Jacob’s young life, his means of communication was limited to nodding his head or opening his mouth for ‘yes’ and making a sound for ‘no’. Often he would scream in frustration during his mom’s stream of questions to ascertain what he wanted. When his younger twin sisters, Sierra and Jamie, were learning to speak, Marcy made an important discovery.
“I noticed how their statements would jump around,” she says. “That got me thinking. If Jacob’s mind worked the same way theirs did, it was no wonder he got so frustrated with me. The yes/no questions were too limiting – he could only tell me what he wanted if I asked the right question.”
Marcy first learned of Dr. Tom Chau’s work on adaptive communications technology when she read an article about him in the Globe and Mail. The best news was that Dr. Chau, a renowned biomedical engineer, was located at Bloorview Research Institute in Holland Bloorview, the very place where Jacob enjoys swimming and the Snoezelen room, and accesses services like orthopedics.
After receiving Marcy’s request to work with Jacob to find communications solutions catered to his needs, Dr. Chau was quick to accept.
Dr. Chau began with a throat switch that enabled Jacob to choose items on a computer that best expressed what he wanted to say. Jacob, who triggered the switch by making a sound, grasped the concept of the switch immediately. The throat switch, however, was not always reliable.
When Dr. Chau adapted an Ipod and placed the switch, which is connected to the Ipod, just to the left of his head, suddenly Jacob could easily specify his needs and wants with the touch of his cheek. The first thing he asked for was a big hug.
Without waiting for mom and dad to ask the right question, Jacob can now tell others that he wants his favourite cat Spot, to talk to someone, go for a walk, go swimming, play the guitar, and accompany his mom on coffee trips. He has 30 programmed statements from which to choose.
Jacob uses his adaptive Ipod all the time in class at Sunny View Public School. It is helping him advance academically. Jacob was a key player in introducing this adaptive technology to Sunny View, a school for children with special needs, which is now home to Dr. Chau’s Satellite Infinity Communications Access Lab. The purpose of the lab is to find customizable communications solutions for kids who also cannot speak and have limited mobility.
Having observed Dr. Chau and his team work with Jacob, Marcy knows that Jacob’s classmates are in very good hands.
“It’s not just a bunch of scientists designing machines,” says Marcy. “They have to know the kids and more than that they have to be comfortable with them. Dr. Chau and his team will talk to Jacob before they talk to me which is the way it should be. They are very respectful of the kids.”
When asked what opportunities she envisions for Jacob, Marcy isn’t too sure – the possibilities are unlimited.
“They have such wonderfully creative things tailored to individual needs that I am constantly wondering what amazing things they will think of next,” she says. “It’s way beyond what I’d thought they’d be able to do.”