There were few comforts for Angela Schleif as she watched her second child die from the rare genetic disorder Alpers Syndrome, 17 years after her first.
But the fact Kody Schleif could take his last breath at home where he felt most safe, compared to his sister’s death as a baby in hospital, meant the world to his family.
Abby Schleif died during surgery when she was only 20 months old, while Angela was pregnant with Kody.
“It was bright lights, lots of people ... We were sort of trying to grieve, but there were people standing around and it was a completely different experience. We also had to then leave her down at the mortuary and go and, like, clear out her room,” Angela Schleif said.
“With Kody, we did not leave him. We stayed and cuddled and, you know, touched him and just were really present with him.”
It is a death that most terminally ill children in New Zealand are not fortunate enough to have, with the vast majority of paediatric deaths happening in hospital, compared to a minority of adults.
A 20-year study of national mortality data showed there were, on average, 270 children who died each year from conditions that would normally require palliative care, and 75% of them died in hospital.
The figures were higher for Māori with 78% and Pasifika at 88%, according to the study by the Child Youth Epidemiology Service at Otago University.
In contrast, Health NZ figures showed about 30% of adults were dying in hospital.
Paediatric palliative care specialist, Dr Amanda Evans, is on a mission to change those statistics.
She is one of only two such specialists in the country. Her Wellington-based team treat children wherever they are - be it their homes, marae, clinics, schools, or hospitals - from Hawke’s Bay down to the lower North Island.
Many children need more life-sustaining equipment over longer periods of time than adults. They have a higher rate of rare, serious illnesses that are specific to children, and care must factor in that they continue developing physiologically and cognitively as they die.
These children require different medication types and dosages than adults, and the care must include understanding of their needs for play, education and their difficulties comprehending their own illness and treatments. Many doctors are not confident in assisting families to care for children in their own homes.
Knowing that dying children and their families aren’t getting the support they need to lighten the burden of their last days, and allowing it to happen in a familiar setting outside hospital, is what keeps Evans up at night.
“What do these families and children do who don’t have us? That gives me a lot of moral distress,” she said.
Alpers Syndrome is a rare and ultimately fatal genetic disorder, usually beginning in early childhood. Death normally occurs about four years after symptoms begin, but Kody was able to live for about 11 years after being diagnosed at 5.
He spent a great deal of time in hospital over the years as his health progressively declined.
“He absolutely hated the hospital, never wanted to go near it,” Schleif said.
“Being able to do it at home and have all the support to be able to do it well at home meant everything to us.”
Evans’ team at Rei Kōtuku provided 24/7 support, meaning if anything happened to Kody or he was in pain, the palliative care specialists could give help, advice, or visit in times of need.
They help families access equipment and teach parents how to care for their children in their final days.
At the end of Kody’s last year, aged 17, he was sleeping in a hospital bed in his living room, with his little brother next to him, and his dog, Biscuit, resting on his legs.
“I just stayed in bed with him the entire day,” Schleif said. “Then about 4 o’clock in the afternoon, he just slowly stopped breathing ... he was just quietly dying. It just happened without any struggle or any distress. The dog was right there, just snuggled up.
“We were there, he wasn’t alone. He was actually in the place he wanted to be with the people that he loved, safe. For him, hospital didn’t feel safe.”
Schleif stayed cuddled up to Kody for some time after he died.
“I had this really small period of time where he still felt like Kody ... I’m just not going to go anywhere while he still feels like him.
“I wouldn’t trade those last few hours for anything.”
The family stayed with Kody as the sun set that night, and put a candle in the window as a symbol that Kody was gone, but that his light was still with them.
It is not a job for the faint of heart: getting to know and caring for children who are destined to die.
Evans has sat through her fair share of funerals.
“I have a little sob. That’s kind of a good time to think about the child and ... think ‘why am I doing this job?’ And once you’ve done a little crying, then you pick yourself up, and yeah, I’m ready for the next one.”
She and her team must recognise that it is not their emotional burden to carry. Nevertheless, it is not unusual to have a cry together on the Monday when they’re back at work, she said.
“I get more emotional when I know that people don’t have our support.
“The parents, this is the most painful thing they have to go through. If I can do anything to try to make it a little bit easier then we know that we’ve done a good job.”
A large number of their patients have neurological or metabolic conditions, and some can live for years after being referred to Rei Kōtuku.
Most of the children they look after are babies, with about 40% aged under 1.
Since the group started their work in June 2023, they have had 121 children referred for palliative care, 58 of whom have died. On average, the team have a child as a patient for 170 days.
It is a source of sadness for Evans that more children don’t have the chance at a better death. There is a “huge gap” for this type of care in New Zealand, she said.
Rei Kōtuku estimated it would cost $8 million annually to set up a nationwide service, and would save about $22 million in unnecessary hospitalisations.
The organisation, funded by an anonymous donor for a three-year pilot, was named a finalist in the Mitre 10 New Zealand Community of the Year Award.
Evans said it was the first time paediatric palliative care in Aotearoa has been recognised at such a level.
“Hundreds of other families need and deserve this kind of support, but cannot access it because, unlike other OECD countries, New Zealand does not have a publicly funded, nationwide paediatric palliative care service.
“We’re hopeful that this time next year, a community-funded organisation will no longer have to try and fill this gap.
“If I was unfortunate enough to have a child that needed this service, this is the service that I would want.”
The team get to know the child, their siblings, pets, and school. They visit them in hospital, and help care for them in the home. They help families organise trips, and, where possible, help kids achieve goals or experiences they have wanted.
“Every child that we see and meet is unique. Not one child is the same,” Evans said.
“I always kind of say to these kids and their families, ‘what we really want is for you to live your life to the fullest and to have really good days. How do we make these days the best days possible?”
Health NZ’s acting director of living well, Astuti Balram, acknowledged there were areas where the public health system can do better with improving access to palliative care.
Balram said the National Palliative Care Work Programme, started in 2023, had considered more than 2100 submissions from whānau, clinicians and service providers for more nationally consistent models of palliative care.
He indicated that Health NZ was now in the process of finalising new paediatric and adult models of care and their next step was to develop an implementation plan including a timeframe for roll out.
“We are committed to improving access to palliative care so that every person, regardless of where they live, can receive the care they need,” Balram said.
Evans recalls a child she “absolutely adored” who was attending American singer SZA’s concert in New Zealand.
The 15-year-old was a huge fan, and Evans, who had contacts in the music industry, decided to see if she could organise a backstage pass for her patient.
The result was more than she could have hoped.
“She actually really took him under her wing. She just made him feel like the most important person in the world.”
SZA took the boy on a marae visit with her while she was in New Zealand, and had planned to fly him to Disneyland before he unfortunately became too sick.
“When he was actually dying, she video-called him and then she paid for his funeral.
“The way that it made him feel was so much better than any medication that I could provide him.”
The team’s work spreads so much further, though. Not only do they care for the patient, but they provide support and advocacy to the family, including counselling for siblings and parents, logistical support with funeral arrangements, and more. This help continues for as long as the family need it.
A month or so ago, it was the third anniversary of a 16-year-old boy’s death. Evans sent the boy’s father a text.
“He rang me and he was crying down the phone and he said thank you so much for remembering my boy,” she said.
Some of the support includes helping patients come to terms with their deaths, whether that was through empowering parents to have those conversations, or by being a sounding board for the child themselves.
“It’s been pretty amazing having death and dying conversations with kids.”
One teen girl used to text Evans regularly, telling her how scared she was of being in pain before she died.
“We were able to say ‘this is what we’re going to do. We will know even when you’re unconscious [if] you’re in pain and we will be able to treat you.’ That was a big relief for her.”
Another boy would tell Evans about how he was going to go meet his mother, who had died a few years earlier.
The team are on call for parents to ring for help - particularly in a child’s last days.
“Sometimes they call me in the middle of the night if something’s happening. I can talk to them over the phone about what to do. [We] equip them with everything they need to make sure their child dies comfortably,” Evans said.
“When you’re there, you see how beautiful and amazing it can be. It’s so incredible what parents can cope with and what they do.”
She also remembers a 5-month-old baby brought home to die who had been on a ventilator with a heart condition.
“It’s not often we do at-home extubations. It’s a really beautiful experience to be involved with,” she said.
The baby, who had a large Pasifika family, came home in an ambulance. The parents and three siblings were there as the tube was removed. She ended up living for another three days after the tube was taken out.
“On the last night before she died, [the] community nurse rang me and she’s like ‘there’s 200 people in the house’.”
Extended family and loved ones were passing the baby around, singing together.
“To me that was like the epitome of what we can do. This baby died in her mother’s arms but with all of the people around.”
Evans said experiences like that were among: “the most beautiful and hardest moments we have”.
Melissa Nightingale is a Wellington-based reporter who covers crime, justice and news in the capital. She joined the Herald in 2016 and has worked as a journalist for 12 years.