In Karen and Eleanor’s words …
Who/what inspires us: My two daughters (Karen), actress Mariska Hargitay for being an advocate for victims of sexual assault and domestic violence (Eleanor).
Best advice: I am responsible for my health, my happiness and my power (Karen), Don’t make excuses for your own failure (Eleanor).
Half-way through her pregnancy Karen Winnett knew there was something wrong with her unborn child. The growing life inside her barely moved. Unlike the wriggly baby she’d given birth to three years earlier, this child lay mostly still inside her. But the doctors told her not to worry. The heartbeat sounded strong. All babies are different, they advised.
When the tiny girl Eleanor was four months old, Karen was convinced something was wrong. Eleanor wasn’t meeting her physical developmental milestones. She couldn’t sit up, she couldn’t roll, her cry was weak, she could barely suck at the breast. But again doctors said not to worry. You’re just comparing her to your other daughter, they said – a daughter who was crawling at 4.5 months old.
By the time Eleanor was eight months old Karen was convinced. While Eleanor was mentally bright, she remained near motionless. Something was most definitely wrong. Karen remembers sitting in the neurologist’s room at Princess Margaret Hospital when the diagnosis finally came. Her baby had spinal muscular atrophy (SMA), type 1 – the worst type. Don’t expect her to live past two to four years old, he advised Karen. Take her home and love her while you’ve still got the chance.
Take her home and love her while you’ve still got the chance.
A death sentence
SMA is a genetic condition that affects motor neurons – the nerves that control muscle movement. Babies with type 1 SMA often die from choking – their weak muscles mean they are unable to chew and swallow properly. They are also at risk of death by pneumonia-induced respiratory failure, as they lack the strength to cough mucus from their weak lungs. Almost cruelly, their intellectual capacity is not affected – if anything patients with SMA are unusually bright.
One medical professional advised Karen to stop breastfeeding Eleanor to prevent her from becoming too attached to this baby who would only die. He told her to separate Eleanor from her older sister Kirsten, so Kirsten too didn’t come to love the baby and suffer the grief of her sister’s early death. Karen remembers a feeling of disbelief at being told that she should not love her child. “Before this child was dead, she was buried,” Karen recalls.
Fast forward 22 years and Eleanor is studying science at university, gaining distinctions, and planning to attend a paleontological dig. How did this young woman and her family defy the odds stacked against them?
“Before this child was dead, she was buried.”
Empowered by research
Karen returned from the hospital after the diagnosis feeling crushed, but defiant. She and her mother had both studied nutritional therapy and Karen had studied medical sociology while at university. Karen launched herself into her own research program, finding out everything and anything about the condition that gripped her baby. She came to learn about the condition’s effect on metabolism – if Eleanor didn’t eat for more than eight hours her muscles would start wasting away, never to recover. So Karen arranged to have a gastrostomy button inserted directly into Eleanor’s stomach. This would enable a specialised formula to drip feed a cocktail of nutrients into Eleanor while she slept.
“You hear that you should give up but you go and do your research, you become empowered, you get the tools and try whatever you can try to produce a different outcome,” Karen says.
“You … do your research, you become empowered.”
Karen took to sleeping with Eleanor every night – a practice she continues today. She turns her if she needs turning. She listens for her breathing, always on the alert for the next breath, awakening immediately at each cough.
But when Eleanor turned two, the flu struck. The doctor believed Eleanor had choked on her own mucus and would die from “silent aspiration”. The doctor advised Karen not to intervene. Eleanor would die anyway, he said. But Karen refused to give up.
It turned out Eleanor had contracted a nasty virus that would have proved dangerous to any child. She was actually faring quite well considering. “I just thought great, thank you for telling me I should kill my child,” Karen says.
“I just thought great, thank you for telling me I should kill my child.”
Around this time Karen discovered a breathing system in the United States, called a biPAP, which triggers ventilated air into a patient if they are unable to breath for themselves. A family in the US who’d lost their own child to SMA donated it to Eleanor and she had it flown across the globe to help her child breathe through the night. Not long afterwards Eleanor received her first wheelchair. At this stage she could draw, write and lift a cup. She was blossoming into a sweet, confident and highly intelligent young child.
Then another virus tore through the family. Karen’s elder daughter Kirsten was hospitalised with asthma, her stepson was hospitalised with asthma as well and, while doing a physio session to prevent mucus build up in Eleanor’s lungs, a piece of mucus dislodged and caused her to choke. Doctors scrambled to conduct an immediate tracheotomy – planning to slice into the toddler’s throat to create an air passage. But somehow Eleanor managed to cough strongly enough to remove the mucus and breathe on her own.
“I had all three of them in hospital – one of them nearly dying – and I just locked into battle mode,” Karen recalls. “It was a battle and I was going to win.” Eventually all three children recovered and the family soldiered on.
She was blossoming into a sweet, confident and highly intelligent young child.
In the meantime, Karen’s marriage to the girls’ father broke down. As a single mum Karen relied heavily on her own mother to manage. While nervous about Eleanor contracting colds and flus, Karen was determined the SMA would not affect her quality of life. She and her mother took the children to museums, to shows, to the zoo, to the park. “I was not going to stay at home with this child and watch her fade away,” Karen says.
Then, when Eleanor was three, another virus struck. This time she was in intensive care. This time Karen feared the worst. But, remarkably, Eleanor again defied predictions and survived.
“I was not going to stay at home with this child and watch her fade away.”
By now the staff at Princess Margaret Hospital in Perth had taught Karen the art of ‘assisted coughing’. Any time Eleanor contracted a cold, Karen would sit with her through the night, and every 30 seconds she’d encourage her to cough up the mucus and spit it out. She’d do this for two weeks at a time, every time Eleanor was sick with the flu. She had carers come during the day so she could catch up on sleep. But she was like a zombie. “I actually cannot tell you how we got through those times,” Karen says.
A move and a loss
Against all predictions, Eleanor reached preschool age and Karen decided to home school to reduce her chances of catching a potentially lethal flu. But Eleanor’s growth became problematic – her muscles were not growing along with the rest of her body. She became so weak she could barely move. It came to the point she could no longer feed herself. No matter. Karen would simply do it for her.
It was around this time that Karen and her mother decided to move to a rural block, north of Mount Barker in Western Australia’s rural south – somewhere they could be surrounded by nature instead of neighbours. They bought 6.5 hectares of land and had two houses erected.
But after moving Karen’s mum was diagnosed with cancer. Four months later she was dead. “I just thought ‘oh my god, what am I going to do?’” Karen says. “I was on this block, with one water tank and two little girls and home schooling.”
Getting on with it
Karen did what she’d always done. Got on with it. A friend of Karen’s stepson moved to the block next door. As a computer scientist and technician, he was able to adapt Eleanor’s bi-PAP machine to fit her wheelchair so she could breathe better during the day, as well as night. This freed Eleanor to move her wheelchair further than the closest power point. He also adapted a computer to enable Eleanor to use it with her limited mobility.
Despite her crippled body, Eleanor continued to excel at school, progressing through primary school and onto high school. But by now her body was twisted with two, 90 degree scolioses in an ‘S’ shape. And, when she was 14 Eleanor contracted kidney stones. Together Eleanor and Karen, along with Eleanor’s sister Kirsten and foster sister Jamie-Lee, attended 70 appointments in six months. Shoulders squared, chins high, they soldiered on.
Shoulders squared, chins high, they soldiered on.
Excelling as student
Despite her ailing health and repeated appointments, Eleanor managed to win the biology prize at high school when she was 16. She was doing so well at school she wanted to apply to university. But sitting her exams with the other kids was impossible. They asked the education department if it would cater to her, by allowing carers to be with her during the exam and extending the time permitted to sit the exams (Eleanor couldn’t sit for as long as her peers).
The education department got back to them – no, she wouldn’t be able to sit the exams, the bureaucrats said. No amount of pleading would make them budge. No matter. They’d go directly to the university. They approached The University of Western Australia’s Albany campus whose staff said Eleanor could do a bridging degree to enter. Eleanor completed the degree with high distinctions, and is now studying a Bachelor of Science with dreams of specialising in marine biology. Her mum helps her into their specially adapted van and drives her the 120-kilometre round trip to Albany to study with her peers on campus.
Eleanor completed the degree with high distinctions.
Since she was a kid Eleanor has possessed a fascination with palaeontology. So, when she heard about a dig with the Australian Opal Centre and Australian Geographic she was hooked. No matter that the dig would occur on the other side of the country in remote Lightning Ridge in New South Wales. Attending the dig became her dream. With the help of her sister Kirsten, Eleanor has launched a crowdfunding campaign to help her get there. She can’t fly – her lungs would never cope with the pressure – so she needs to kit her van with life support equipment and make space for carers to help her embark on the journey. “If she was a great fan of basket weaving that would be great but no, she’s into palaeontology,” Karen smiles.
As well as studying Eleanor is writing a novel in her spare time, sits on disability advisory committees, and loves taking her dog for walks around the property in her wheelchair, which she controls with the movement of a single finger.
“If she was a great fan of basket weaving that would be great but no, she’s into palaeontology.”
Determined to enjoy life
Eleanor contracted another virus recently which reminded the family of her mortality, and yet the thing that worries Karen the most is Eleanor’s love of adventure. “The thing that causes me the most stress is not the likelihood of her dying but the way she wants to live,” Karen says. “Eleanor is amazing in terms of the way she has risen to it – she’s not as comfortable as she could be but she’s not letting that stop her and she’s not letting that reduce her enjoyment of life. She could have chosen to just stay in bed and cry to herself but she’s getting out there, studying and tackling university with all these physical problems. She won the biology prize, she won the Dr Louisa Alessandri Memorial Fund Scholarship – they are things that any mother would be proud of but doing them when the most you can do is move a little finger is pretty good.”
“She could have chosen to just stay in bed and cry to herself but she’s getting out there …”
While Eleanor’s body has crippled, she has not lost her determination to enjoy life.
Karen has never once left Eleanor for more than a few hours in Eleanor’s 22 years – she believes doing so would be fatal to her daughter. And while Kirsten and two other young girls who live with them, Kerry and Jamie, are helping, Karen does worry about the future. Eleanor credits these young women with having helped her make it this far, for their help with Eleanor and the farm, while also studying at university and working.
Karen now suffers rheumatoid arthritis. But she says breaking down is just not an option. “What advantage would there be in pulling the covers over my head and saying I just can’t?” she says. “I choose not to. When those thoughts come crowding in I just don’t react to them.”
Karen is pinning her hopes on a new treatment options being trialled in the United States. “We’re really hoping that this treatment works and she’ll be able to move herself more, and feed herself and not need the ventilator,” she says. “If that happens she’ll be ok. I just wish it would hurry up.”
“What advantage would there be in pulling the covers over my head and saying I just can’t?”
Get involved …
You can contribute to Eleanor’s efforts to visit Lightning Ridge for a paleontological dig by contributing to her crowdfunding campaign. Find out more here: