There are some days when single mum Holley Radford feels like it’s just her and her precious son Xzavier against the rest of the world. Holley was a teenager when Xzavier was born and ever since, his many health problems have kept the pair largely confined to their flat in West Auckland.
“For the first year in particular, I hardly left the house unless it was a medical appointment,” says Holley, 21.
“Xzavier had a feeding tube and an oxygen bottle, and I became self-conscious because people would ask, ‘What’s wrong with your baby?’ I always had the same answer, ‘Nothing – he’s just a little bit special.’”
Special is an apt description for Xzavier, an engaging little boy with startling blue eyes, impossibly long eyelashes and an almost-permanent smile etched on his face.
“Xzavier smiled when he was just a few weeks old and from then on, it’s pretty much what he does all day, every day,” tells Holley, cradling her son and kissing the top of his head.
Xzavier, who is now two and a half, has a rare medical condition known as Prader- Willi syndrome (PWS), which results in a number of mental, physical and behavioural problems.
At birth, his condition was characterised by weak muscle tone, feeding difficulties, poor growth and delayed development. But more mysteriously, he will go on to develop an insatiable urge to eat that’s so strong, he could gorge himself to death.
“It’s like a switch goes off in the brain,” explains Holley. “That’s it – from then on, Prader-Willi kids are fixated on food.”
Born underweight and too weak to feed, Xzavier was fitted with a nasal gastric tube on day one.
Chronic overeating can lead to obesity, type 2 diabetes and, more dangerously, choking. “You need to lock the fridge and pantry, and watch them all the time,” she tells.
“Kids with PWS will do anything to get food, including going through the rubbish bin.”
Three weeks from Xzavier’s due date, his movements slowed and a subsequent scan at Waitakere Hospital showed he was small and his heart was irregular.
Holley was induced in the wee hours of March 13, 2015 – and tiny Xzavier arrived less than an hour later. “He was tiny and I knew he wasn’t breathing,” recalls Holley.
“The thing I remember most is how many doctors and nurses were in the room.”
Xzavier was given oxygen but was fitted with a nasal gastric tube because his poor muscle tone meant he was too weak to feed.
“In the first few weeks, it felt like he would sleep all day, only opening his eyes for a minute, then nodding off again,” says Holley.
Medical staff also suspected the baby was having seizures, so he and Holley spent his first four weeks in hospital while he underwent a host of tests.
“I knew something was wrong, but I tried to block it out,” admits Holley. “He was my first baby and it was hard to think about it.”
When Xzavier was four weeks old, he was diagnosed with PWS, caused in most cases by a genetic error in the 15th chromosome.
“I was given some reading material, but I couldn’t bring myself to look at it for a long time,” confesses Holley. “I was in denial.”
Amplifying her grief was a perception that other people blamed her for Xzavier’s health problems. “I know now that’s not the case – it’s not my fault,” says Holley.
Prader-Willi means the sweet tot, two, is tube-fed and still fits into baby clothes.
The hardest thing for parents of kids with PWS to manage is their child’s obsession with food. Xzavier is still mostly tube-fed, though he does pick at solids.
But sometime between the ages of two and five, he will develop a constant feeling of starvation. Some children will not just seek food, but also draw it, dream about it and even forage in the garden for it.
Xzavier is small for his size – weighing about 9kg and still fitting clothes for nine-month-olds – but Holley says every time there is a spike in his weight, she wonders if it’s his appetite kicking in.
She says she’s lucky that Xzavier was diagnosed early because it means he’s been on a feeding plan and under the care of a nutritionist since he was a month old.
A social worker, physiotherapist, speech therapist and two specialists at Auckland’s Starship children’s hospital also support the boy and his mum. Each week is a carousel of medical appointments and therapy, and Holley has recently reached out to a PWS support group.
Although he’s behind in meeting his milestones, Xzavier is doing well and happily bum-shuffles across the carpet to reach his toys, babbling his baby talk.
Holley knows she’s got a long road ahead and she’s doing it largely on her own. Adults with PWS struggle to live independently because their appetite can get the better of them, but Holley says she’s there for Xzavier.
“Some children grow up, get jobs and move away, and some kids need to be cared for all their lives,” says the devoted mum. “If that’s what I have to do for Xzavier, that’s fine by me. I love him and I would do anything for him.”
