Little Amber Mason nearly choked to death from a well-hidden birth defect.

Amber Mason was 10 months old before she sucked in her first deep breath of air. Until then, the Nelson youngster was being choked by her own body.

Now a bubbly 16-month-old, Amber was born with a life-endangering birth defect. Her left pulmonary artery was wrapped around her windpipe, literally strangling her.

A lifesaving operation by a cardio team at Starship Children's Health in Auckland was needed to clear Amber's airway and allow her to breathe cleanly.

But, in spite of spending almost half her life in hospitals in Nelson, Wellington and Christchurch, doctors had failed to identify the cause of her breathing difficulty until it was almost too late.

‘From the moment she was born, she was just a ticking time bomb waiting to go off,' says Philippa Mason, Amber's mum.

For months, Philippa had listened to the frightening ‘rattle' as the girl struggled to breathe. ‘It used to frighten people when they came round to visit,' she says.

Amber's breathing noises were so bad Philippa and her builder husband Paul jokingly called her ‘our little freight train'.

But other problems beset the toddler, distracting doctors from diagnosing why Amber was making such strange noises.

A 10-week premature baby, Amber spent her first three months in hospital with stomach problems and apnoea. Like many premature babies, Amber would stop breathing up to 10 times a day and needed to be ‘tickled' back into action.

When the stomach problem and the apnoea resolved themselves and Amber was finally cleared to go home, she still had the ‘freight train' rattle. Doctors thought it was a floppy larynx that would correct itself as she got older and stronger.

‘As she was our first child, we simply accepted what the doctors said,' Paul says. ‘She never cried very much at all.'

Then, seven months after going home, in the middle of ‘kick time' on the lounge floor, Amber started to choke herself.

The pulmonary artery, perhaps disturbed by the playtime exertion, tightened its grip on her windpipe.

‘Her eyes were staring straight ahead and she was going blue,' Philippa remembers.

At Nelson Hospital, Amber settled down and for two days there were no problems. However, a CT scan finally identified there was a kink in her airway and it was decided Amber should be sent to Starship, where there was more expertise.

However, hours later, as Philippa bathed Amber, the artery tightened its grip and she was going blue again.

Hospital staff came running to help, including a paediatric anaesthetist who happened to be driving past the hospital when his pager went off.

The anaesthetist managed, on his third attempt, to force a very narrow tube down Amber's airway, allowing her to breathe.

‘We don't think this was luck. We think God had a hand in it,' Philippa says.

The next 12 hours were a blur to Philippa. Amber was put in a coma, phone calls were made to Starship and their air retrieval plane arrived in the early morning hours.

But, at Starship, there was an agonising wait ahead. Paediatric cardio surgeon Kirsten Finucane was on leave and not due back for five days. It was decided to keep Amber in a coma until her return.

While they waited, Philippa and Paul stayed at the Ronald McDonald House, which is temporary home where families can stay for as long as they want while their children are undergoing treatment at Starship. There, they met other parents from around the country, who also had seriously ill children being treated at the children's hospital.

‘It is a humbling place. All these parents with sick children, children some of them know are going to die. There is an incredible feeling of camaraderie. You see people crying in a corner and you know what has happened,' Philippa explains. ‘You just pray you are not going to be next.'

As Philippa and Paul waited - ‘lying in our room, hugging each other and crying,' Paul says - they watched the heartbreak as two babies who were sharing the intensive care unit with Amber died.

Dr Finucane returned and met the parents, but the news was shocking.

‘She was not sure if she would be able to help Amber, and if it was as bad as the Nelson scan showed they would just let her go naturally,' Philippa says.

After a nerve-wracking hour, a new scan showed surgery was possible. The operation, which took almost eight hours, also involved patching a hole found in Amber's heart and cutting out part of the windpipe on which hard rings had formed.

Amber was kept in a coma for another four days before slowly being brought out and having the tubes removed.

Then, at 10 months old, Amber took the first big deep breath of her life.

‘It is like we brought a different baby home,' Philippa says six months later. ‘There is just so much more life to her. She laughs now. The first time we heard her laugh, that was amazing.'

She is still a small baby - ‘More like a seven or eight-month-old,' Philippa says. But she is crawling now and starting to talk. Her first words were ‘Mum Mum', followed very closely, according to Paul, by ‘Dad Dad'.

She is, Philippa says, a very special baby with a lot of ‘inner character'.

STARSHIP FACTS
Each year, Starship Children Health's national air retrieval service rushes medical experts to more than 200 life-threatening emergencies around New Zealand, from illnesses and accidents to long-term medical conditions.

Starship Foundation's Annual Appeal is now raising funds to purchase a new transport system comprised of a ventilator and incubator to transfer the most critically ill newborn babies to Starship's intensive care units for lifesaving care.

Donations to the appeal can be made by phoning 0900 STARSHIP (0900 782 774) to donate $20, texting STARSHIP to 469 to donate $3, or by visiting Starship online at www.starship.org.nz/appeal.