The brave parents of a tiny girl todayface an agonising moment in their daughter's life when doctors remove her breathing tube.
Seven-month-old Chrissa Squire has suffered irreparable brain damage because of an extremely rare reaction to an anaesthetic. Now her parents want to warn the public about the danger.
Chrissa, from Christchurch, was given an anaesthetic on September 29 so doctors could insert a breathing tube through her mouth because she had vomited into her lungs.
Her temperature shot up to 40 or 41C, her blood pressure rocketed, and an aircraft from Auckland's Starship Children's Hospital flew down to pick her up that night.
She has lost her sight and suffered severe brain damage, but her parents Chris Squire and Stephanie Allan have not given up.
"She is still in there," said Allan. "She will respond to our voices. Her eyes show, 'I know her, I know that voice'."
Her breathing has improved, but the family has been told there is only a 50/50 chance that she will survive when doctors remove her breathing tube today.
The outlook is bleak either way.
"If she does get through this, and they are monitoring her, she should be okay to live for another few months," Allan said.
"We can go home with this machine and that machine, and they will monitor that.
"We can provide her with the most love she can get. We can give her a better farewell than she can get in hospital."
The couple contacted the New Zealand Herald because they want to make other parents aware of the potential risks of an anaesthetic.
Doctors believe that Chrissa has a rare genetic condition called malignant hyperthermia, but there had been no previous indication of it because neither parent is aware of the condition in their families.
"I don't want to see any other child go through this," Allan said.
"If there is a test that can be done when a child is born, or before they go into surgery, there are many options for other anaesthetics that can be used."
Squire said the crisis had happened so suddenly that both parents were still in shock. Both have barely slept for the past week and were taking sleeping pills to try to get some rest last night.
"We don't have any complaints," he said. "It's nothing against the hospital or anything, it's just: be careful."
Much of Chrissa's short life so far has been spent in hospitals. She was born on February 16 with Pierre Robin Syndrome, which gave her an unusually small chin and consequent obstruction of the airway caused by backwards displacement of the tongue.
About three weeks after she was born, doctors also discovered a large hole in her heart.
She was allowed to go home on May 6, but her parents had to feed her via a tube through her nose, and were told that they might have to keep doing that for two or three years until her chin grew enough for her to eat normally.
"She thrived at home," Allan said. "She had outings to malls, was a known and loved wee girl at her big sisters' school."
But on September 16 she had to go back to hospital with bronchiolitis and, later, pneumonia. She was given an oxygen mask to increase the air flow through a nasal tube, and the crisis was caused by vomiting inside the mask.
Her paediatrician Dr Nicola Austin said Chrissa's reaction against the anaesthetic was "totally unpredictable".
"There was no family history known," she said.
A world expert on malignant hyperthermia, Massey University biochemist Professor Kathryn Stowell, said hyperthermic reactions occurred in between one in 10,000 and one in 250,000 anaesthetic procedures worldwide.
She said the genetic mutation was inherited, with a 50/50 chance that someone with the mutation would pass it on to each child.
Sixty families are known to have the mutation in New Zealand and a national testing centre has been set up at Palmerston North Hospital because one large Maori family in the lower North Island has it, raising the incidence to one in every 200 surgeries at that hospital.
But Stowell said every family she had tested had a slightly different mutation, so it was impossible to test the general population for it.
She said an antidote reduced the death rate from more than 80 per cent of people who suffered the reaction to the anaesthetic 30 years ago to less than 5 per cent today.
"I think the baby [Chrissa] was given it, but maybe there were other complications," she said.
The Palmerston North team has been informed about Chrissa's case but Stowell said they had been unable to confirm that she definitely has malignant hyperthermia because she was given a blood transfusion, which makes it impossible to test her own blood immediately.
What: A reaction against common anaesthetics, including very high temperature and heart rate.
Incidence: Ranges from one in 10,000 to one in 250,000 anaesthetic procedures; however recent evidence points to the genetic mutations that are associated with it occurring in one in every 400 people.
Treatment: The reaction can be controlled by very rapid injection of an antidote called dantrolene. Experts recommend that any hospitals administering general anaesthetics should keep dantrolene supplies.