Seven-year-old Keira Hubball is at her happiest snuggled up next to her mother and showering her with kisses. “I love you – you’re mine,” the gorgeous little girl tells her mum Vickie Hamilton in her distinctive sing-song voice.
Smiling at her only child, Vickie says, “Keira is in her own world. She just lives to love.”
Moments like these are precious and Keira’s parents, Vickie, 30, and James, 32, know they need to savour them while they can.
Keira is one of only three children in New Zealand with methylmalonic acidemia (MMA), an incurable disorder in which the body is unable to properly process certain proteins and fats.
Although chatty and full of life when Woman’s Day visits her at home in Christchurch, only a few months ago, Keira was critically ill following a catastrophic brain bleed.
After a fortnight on a ventilator in the paediatric intensive care unit of Auckland’s Starship children’s hospital, she came home under palliative care. As she clung to life, her grandparents returned from their holiday in the UK and her family gathered at her bedside.
“We’ve always known we could lose Keira at any time, but this was our biggest scare yet,” admits Vickie.
Incredibly, however, Keira has defied the odds. Although the gorgeous young girl’s future will always be uncertain, for now at least, she’s back to her affectionate and giggly self.
“We’ve been told by the doctors that there is often an up before a down, but all we have seen from Keira is up, up, up,” says Vickie. “She’s a miracle.”
Keira was a newborn when a heel-prick blood test detected MMA. By then, she had all the telltale signs – poor muscle tone, vomiting and failure to feed. “When they told us our baby had MMA, we said, ‘What the hell is that?'” recalls her mum.
Vickie and James now understand their adorable girl inherited the disorder because, unbeknown to them, they both carried the defective gene.
Although the condition can range from mild to life-threatening, in Keira’s case, the impact has been profound. She’s the size of a four-year-old and her developmental age is about the same.
She did eventually meet her milestones as a baby and a toddler, but she no longer has the strength to walk and has resorted to crawling. And although she picked at some solid food as a tot, she now relies entirely on liquid feeds via her stomach.
“Over the years, we have had to watch as Keira becomes more and more fragile,” tells Vickie, who cares for her girl full-time at home.
Most dangerous, though, is the effect on Keira’s liver, kidneys and pancreas. Keira is on a strictly controlled diet that restricts protein and the four offending amino acids, but dangerous levels of acid can build up in her blood.
“The easiest way to explain MMA is to say that it’s like Keira has antifreeze flowing through her body and it’s attacking all her major organs,” tells Vickie.
In October last year, Keira was admitted to Starship with pancreatitis, or inflammation of the pancreas. But not long after she was transferred from intensive care to the high- dependency unit, Vickie and James realised something wasn’t right.
“Her eyes went funny – they were all over the place,” remembers Vickie. “And I noticed she was losing strength down one side of her body.”
The young girl was rushed into surgery so doctors could drain a life-threatening excess of fluid from her brain. But after the procedure, Keira’s parents faced a tough decision. Specialists said the fluid was continuing to build at a dangerous rate. Keira needed more surgery, but there was a risk it could kill her or leave her severely disabled.
“We thought Keira had been through enough, so we decided to bring her back to Christchurch Hospital,” says Vickie. “It’s her home – it’s where she feels comfortable.”
Usually only one parent can accompany a sick child on the Life Flight plane, but Keira’s grip on life was so precarious that both Vickie and James were allowed to fly with their daughter.
Vickie recalls, “We were told there was no coming back for Keira and she came home under palliative care – but after three days, she told her father, ‘I love you, Daddy.'”
Although Keira suffered some minor brain damage during her ordeal, Vickie and James have their loving wee daughter back home.
Keira’s not mobile on her own, but she’s a joyful, fun-loving girl, who adores music, car rides, and visiting her cousins and grandparents. Vickie hopes she will regain enough strength in future to return to Merrin Primary School in Avonhead, where she has been a popular student.
For Vickie and James, the aim now is to keep making precious memories with Keira.
“Doctors have said we are on a slippery downhill slope, but for now she’s at a standstill, so we need to enjoy every second,” says Vickie. “Keira has done the hard yards and it’s up to us to keep fighting.”
