LITTLE Jace Knowles has a lot of people in his corner.
The mischievous three-year-old is the youngest child of Mick and Tara, and is their only son.
Besides the pressures of having three older sisters, Jace also has an exceptionally rare degenerative medical condition.
Mucopolysaccharidosis is a metabolic disorder caused by the absence or malfunctioning of lysosomal enzymes.
These essential enzymes are used to break down glycosaminoglycans – which help to build bone, cartilage, tendons, corneas, skin and connective tissue.
There are seven strains of the disorder, and Jace has MPS 2, known better as Hunter syndrome.
It’s a condition that occurs in only one in 150,000 male children.
“Basically his organs are shutting down,” Tara said.
“He has an enlarged abdomen due to an over-sized liver and spleen.
“His bones and joints are deteriorating, and there are thousands of holes all through his brain.”
Jace was diagnosed with the condition on February 5 this year.
It’s thought that he has had the condition since birth.
His condition is further complicated because of the neurological aspect.
“Enzyme replacement is normally offered for kids with Hunter,” Tara explained.
“But, if it’s in the brain, the government won’t fund it.”
In addition, he has difficulty with movement and has to be very careful due to the fragility of his joints.
“He is still walking and talking at the moment, but that will become difficult over time,” Tara says.
“Even know he is a little old man in a kid’s body.
“He’s very irritable because of all the pain he’s in.”
Jace has just started his third year at Columba Cottage Learning Centre.
He attends three days a week, with Columba providing a scholarship for him to attend one of those days.
The Cottage has been heavily involved with Jace and the Knowles family.
Earlier this year, two staff members, Lucy Sales and Linda Will, volunteered their services to assist the family.
They travelled earlier this month to the 13th National Mucopolysaccharidoses and Related Diseases Conference at Point Wolstoncroft .
The two carers were able to provide assistance and supervision for Jace and his older sister Lacie, who is a quadriplegic, throughout the conference.
At the time, Jace had just been diagnosed, so the opportunity for Tara to interact with specialists and the parents of other Hunter-afflicted children was essential.
The Cottage has continued pushing to help Jace.
Director Michelle O’Brien noted the involvement of multiple staff members in providing ongoing assistance.
“We’ve got four different people on the staff who’ve formed a committee to determine the best way to raise funds and help Jace,” she said.
“We’re looking at whether he needs occupational therapy, or how the fundraising can be best spent in the future.”
Oneof the fundraisers will be a trivia night and silent auction at The Westport Club on Saturday, May 25.
Prizes and auction items include VIP passes to theme parks on the Gold Coast, jewellery and snowboard equipment.
The Port Macquarie Roller Derby League is raising funds through an entirely different venture.
Tomorrow night, the group will host a dinner, with the Knowles as special guests.
They’ve been busy selling raffle tickets to raise funds.
The league is only a year old, and is already heavily committed to serving the community.
“We aim to do a charity event each time we have a round of recruitment,” the group’s secretary Jo ‘Psycho Bunnie’ Sampson, said.
“When we heard about Jace we just knew we had to get involved.
“This is actually our very first fundraiser and we’re going to try to make it a big event.
“We’re all women and we’ve all got children, so we’re going to do whatever we can to help.”
The lady at the centre of things is overwhelmed by the community’s show of support.
“It’s been so wonderful the way people have got involved,” Tara said.
The steadfast mother is unable to break her focus however, and is looking to the future.
“At the moment we’re trying to get a bone marrow transplant for Jace,” she explained.
“It involves chemotherapy and it’s potentially a huge step.
“At the end of the day it’s worth a shot.
“It’s all we can do.”
Little Jace Knowles with his mother Tara.
This story Administrator ready to work first appeared on Nanjing Night Net.