PORT AUX BASQUES, N.L. - Last August, at only five years old, Ava Short-Gaudet lost her battle with Batten disease. The nervous system disorder, which typically manifests in young children, is always fatal.
Her mother, Brittany Gaudet, hopes people will remember Ava for the loving, friendly child she was. Along with family and friends, the young mother is working to ensure a lasting tribute that would befit her late daughter.
Ava’s family has donated an annual friendship award in her memory and placed a friendship bench in the playground at St. James Elementary where she attended kindergarten. The six-foot bench was Brittany’s idea and was built by her cousin Neil Osmond to be sturdy and safe for children in a play environment.
“We initiated it though because we wanted to get the bench - something in memory but the kids could enjoy it,” said Brittany. “Having a plaque and stuff is great, but the kids don’t get to enjoy it every day.”
A friendly, outgoing child, Ava loved school and going out to the green swing on the playground.
“That was her swing. That’s a disability swing. They (the school) brought it in for her, so we placed (the bench) almost just behind that.”
Brittany wanted to make the bench even more personal and invited all of Ava’s classmates to put their two handprints on the bench in whatever colour they chose. The children happily obliged.
“We chose a friendship bench because in kindergarten they give out these little certificates and Ava got the Best Friend Award last year,” explained Brittany.
While the bench can be enjoyed by all of the school’s children, the plaque can only be awarded to a Grade 6 student.
“The plaque we did for 18 years and it’s the friendship award. It has to be given to a Grade 6 student because they don’t do those little award ceremonies for any other grade anymore, so it’s going to be a Grade 6 student who has shown friendship throughout their years at St. James.”
The family will attend the award ceremonies to present the plaque on Ava’s behalf. Brittany doesn’t believe either of two younger daughters, three-year-old Cali and 16-month-old Jersey, will qualify for their sister’s plaque.
“They’ll probably never get it because she’s too crazy,” laughed Brittany about Cali, who is a bit more rambunctious than Ava. “But I wanted them to even have the opportunity or have the opportunity to be in the school when it’s presented.”
'Selfless, caring, affectionate'
At first Brittany is reluctant to talk about Ava.
“I don’t know how to describe her. She was absolutely phenomenal as a child. She was very selfless, caring, affectionate,” began Brittany.
Although Ava was largely non-verbal except for only a few words, it didn’t prevent her from demonstrating affection to everyone. Brittany relates how a student caregiver and a teacher both mentioned being showered with hundreds of Ava’s kisses each day.
“She would kiss your hands a hundred times a day, just smiling,” remembered Brittany. “I swear that child was just unbelievable. And all she went through? She would cry if she was in pain and that was it. She would never act out. Nothing. She was so good.”
Ava’s two best friends were Maddie Walters and Emma Smith. The trio would attend each other’s birthdays and would look out for Ava, helping her eat at lunch and pushing her up and down the school hallway until Brittany arrived to take her home after school.
“That’s what friendship is.”
Ava continues to be remembered by a wealth of friends and family, some of whom believed they would have more time with her. Brittany knew her child and understood that wasn’t likely to be the case.
“I could tell. I said we don’t have that time,” Brittany recalled telling her own mother. “We don’t have it. I could feel it. We just don’t.”
Brittany’s suspicions proved correct.
On May 12, Ava posed for some photos. At that point she could barely lift her legs. By June 22, at the graduation ceremony she could no longer sit up. In July, doctors switched her pain medications to try to control her growing discomfort. Ava succumbed to Batten disease on August 30, 2017.
Brittany said Ava’s neurologist told her he has seen 18 cases of Batten and Ava's was the first time he saw a patient deteriorate so quickly.
Brittany said she has come to terms with losing her eldest daughter.
“I was more prepared than anybody I think, and as much as I say prepared, I was heartbroken. But I was so proud of her because she punched a rough year.”
It cost Brittany precious time with Ava just to send her to school, but she’s truly glad she made that sacrifice. Some of Ava’s happiest times were at school.
“I was like… I want every bit (of time) I can get but she deserved that. Those kids deserved to experience what they did.”
Not as rare in N.L.
Part of Ava’s legacy will likely be a book. Brittany says she is writing a children’s book to pass out to schools around the province.
“It is such a rare disease but not in this province. The statistics are way higher in this province,” said Brittany, who blames what she calls the island effect.
The National Institute of Neurological Disorders and Stroke have included Newfoundland among their geographical regions that seem to have a higher than usual occurrence, along with Finland, Sweden and other parts of northern Europe.
Brittany says she and husband Spencer are both carriers of Batten disease, something neither knew before Ava was diagnosed.
“I’ve communicated with so many people in the Batten support group from around the world and so many are from Newfoundland, and New Zealand and the UK – all different small communities.”
Both Cali and Jersey have a 25 per cent chance of being diagnosed with Batten disease, and a 50 per cent chance of being a carrier.
“Can’t change it whatever it is,” said Brittany.
It helps that she has seen no warning signs in Cali, and Jersey is still too young. Brittany is unwilling to let either of her two younger daughters undergo the painful muscle biopsy required to check for Batten disease, and notes that initially Ava’s biopsy came back negative even though she had tested positive on the blood test.
“We took it one day at a time.”
'She just didn't give up'
The Children’s Wish Foundation sent the family to Florida. That was in September and by November Ava couldn’t eat or drink and needed surgery.
“I look back and I’m crazy, but if we didn’t take Ava then?”
Being away from their support system just on a short trip made Brittany discard the notion of moving Ava closer to doctors in St. John’s. The doctors, nurses, teachers and other caregivers in Port aux Basques were all part of Ava’s family and made her feel safe and loved, and Brittany trusted them implicitly.
“Yes, they have medical teams there, but this hospital knows my daughter. This hospital is family,” said Brittany, who can’t praise Ava’s medical team enough.
Knowing she was doing the right thing for her daughter afforded Brittany some small measure of comfort toward the end of Ava’s journey.
“It was just, you know what? Ava’s finally at peace and that was the only thing that helped me get through it, besides (Cali and Jersey) keeping me busy,” said Brittany. “I never got to enjoy being her Mom at the end of it because she was so sick, and we never got (respite) help from the government at all.”
After she penned an open letter to the government about Ava’s care, the office of MP Gudie Hutchings stepped in. Only a couple of weeks ago, the Gaudet family finally received restitution for all of the out-of-pocket expenses incurred for Ava’s medical treatment.
"Ava’s finally at peace and that was the only thing that helped me get through it, besides (Cali and Jersey) keeping me busy." Brittany Gaudet
“I was evil,” admitted Brittany about the letter. “I never got them to pay for so much as a glove. We paid for everything. Her meds. Ava was on a lot of meds.”
Sometimes because of conflicting side effects, Ava couldn’t always take the cheaper generic brand. Brittany says people didn’t always understand that it’s not like switching from Tylenol to a generic acetaminophen.
For now, Brittany’s focus remains on Cali and Jersey. Cali is old enough to really miss Ava and for months after her sister’s death wouldn’t sleep in the bedroom they shared.
“She’s three-and-a-half," Brittany said. "She’s had to deal with things she shouldn’t have to deal with. What are you supposed to do? It’s the situation she was given.”
Brittany eventually sought counselling after Ava’s death.
“I feel like I lost a job because I did everything. My day started at seven o’clock in the morning and my day ended at probably 10:45 and that was Ava’s last dose of meds, and then repeat. For the first couple of months I felt like I was letting everybody else grieve.”
Brittany says she would like people to remember what a fighter Ava truly was and hopes that when life gets rough folks will remember her brave little girl who fought to the bitter end.
After Ava lost her ability to walk she kept on trying to do it anyway. Brittany has the video to prove it.
“She just didn’t give up. I feel like everyone should just take inspiration from that. She just didn’t give up no matter what.”