Hospital visits are a part of life for Eliza. Photo: supplied
This season, butterflies have been everywhere. As they flutter through the breeze, it is easy to forget their fragility and the way life can quickly change for them. Everyday life is a struggle with delicate wings. Much like a butterfly, those who live with the rare genetic disease, Epidermolysis Bullosa (EB), know just what it is like to be fragile.
Often called ‘Butterfly Children’, those who have the condition live with skin that is as fragile as a butterfly’s wings. The slightest touch can cause it to tear or blister, with the worst sufferers forced to live their lives in bandages or risk further injury.
Eliza Baird is 15 and, like most girls her age, goes to high school and has a number of hobbies, including cooking and scrapbooking. Eliza was born with severe recessive dystrophic EB. Her mother, Simone Baird, is family support coordinator for the Dystrophic Epidermolysis Bullosa Research Association (DEBRA) in Australia, working with families around Australia.
Eliza Baird with mum Simone. Photo: supplied
She supports families through the trials and discusses how DEBRA can assist with financial pressures, such as bandaging costs using the National EB Dressing Scheme. DEBRA predicts there are around 1000 people with EB in Australia, but some may not even be aware of the disease because their symptoms are so mild.
“There is no cure, there is no treatment – there’s just management,” Simone says.
“The hardest thing is to live it because it’s relentless. It never gives up, it doesn’t stop. It’s 24/7, even through the night. I love to be able to help and support other families because I live it and I know how damn hard it is,” Simone says.
Sunshine Coast ironman athlete and DEBRA Ambassador Matt Bevilacqua. Photo: Georgina Auton
Simone describes the moment Eliza was born and the feeling of confusion at not knowing what was wrong with her newborn.
“She [Eliza] was born with raw areas of skin on her right knee, shins and involving her whole right foot. It was bright red, like it had no skin on it whatsoever.
“The back of her hands were affected as well and the skin on the top of her tongue.
Eliza Baird, born with skin as delicate as a butterfly’s wings. Photo: supplied
“My paediatrician came down and had a look at her and said ‘If I didn’t know any better I’d have said you’d tipped boiling hot water over the top of her because that looks like third degree burns’,” Simone says.
Eliza was transferred to another hospital soon after her birth and tests revealed that she had severe recessive dystrophic EB; a form of the disease which gets worse, rather than better, with age.
“She’s in a wheelchair permanently. She can’t walk, she’s peg fed, and has a gastrostomy.
“Every 12 weeks she has a throat dilation … it’s an operation where they put a tube down her throat to stretch the skin open in her oesophagus so she can swallow,” Simone says.
Eliza also suffers from corneal abrasions, where the surface of her eyes can tear. The healing process requires three days in a darkened room keeping her eyes closed.
“Four years ago the secondary complications became involved and she went into renal failure and she was placed on – like dialysis – but it’s called plasmapheresis; four days a week all day.
“She has a big valve which went into her chest wall to do all of this and it basically took over the function of her kidneys.
“She was given six months to live so we did the whole Make-A-Wish thing and went to Disneyland,” Simone says.
Eliza’s renal failure is now classed as being ‘in remission’ however everyday life is a constant struggle and Simone says that no matter where they go, EB is always present.
Simone’s description of Eliza’s daily cocktail of drugs required to manage her pain and their strict daily routine is a poignant reminder of the strength which both Eliza and her family possess.
Every morning, her family changes some of her bandages before school. Three nights a week, Eliza has a bleach bath for two hours with one kilogram of salt to soak and cleanse her open wounds. After the bath, she is once again wrapped in bandages.
Eliza is often praised for having a positive outlook on life, despite living with constant pain. She seems unphased by it, saying; “I think that’s what you have to do; otherwise you just kind of…fall in a heap.”
She says that she often speaks to other EB sufferers in Australia on the phone.
“We just mainly talk about school and sports and things. We don’t really often talk about EB,” Eliza says. But she says it is good to have someone else to talk to if she needs to. She says that at school, everyone is really accepting and that she is just the same as everyone else.
DEBRA Ambassador Matt Bevilacqua, 23, also has EB. However, looking at Matt, you wouldn’t be able to tell. He doesn’t have to live his life wrapped in bandages or in a wheelchair and falls at the mild end of the EB spectrum.
Matt lives on the Sunshine Coast and is a successful Ironman who competes in the Nutrigrain Ironman series. However, Matt’s family has more experience with EB than most.
“EB is a genetic thing and it has been on my mum’s side of the family for a few generations now, so my Nan’s got it, my Mum’s got it and it just got passed down to me.
“Mum straightaway knew that I’d got unlucky … it’s a bit of a luck thing I think, a roll of the dice,” he says, adding that his siblings don’t have it.
Matt found it tough at school and during sport when he had to wear closed-in shoes. He says that he would often have to bandage his feet and strap them to prevent further friction.
“I was a really active kid and Tasmania’s good because it’s a really cold climate but as soon as I started running around playing football, soccer, cross country, athletics, that kind of thing my main problem was the shoes on my feet, and friction created really bad blisters,” Matt says, adding that running on sand in Ironman competitions caused far less aggravation to his skin.
Matt admits that though EB affected his life and that it was during sports when he was younger that EB became really apparent, it “wasn’t as bad as it can be”.
“I dealt with it, and it’s amazing – you’ll see videos of kids and how well they learn to deal with pain and so I just got used to it,” he says.
Matt says that the ambassador role within DEBRA is fairly new, however he will be competing in a 52-kilometre paddleboard race in Hawaii later this year and wants to try and raise money and awareness for EB through that.
For her future, Eliza says she dreams of what many young people take for granted: “Just growing up like everyone else, going to work and just dreams people have constantly like living out of home…that sort of stuff.”
EB is such a rare genetic disease that many Australians have never heard of it.
Simone describes her best memory – a moment when their entire family was having fun and EB wasn’t at the forefront of her mind.
“The biggest memory for me would be Disneyland, and seeing the night firework display and Tinkerbell in the sky and Eliza sitting on my knee looking up.
“I remember just thinking ‘this is what it’s all about.
“I was just thankful that we had that moment and whatever happens now [at that point Eliza had six months to live] at least we’ve done this.”
Originally pubished by Brisbane Times here