Piata Tauwhare, 30, from Hokitika, was found dead at a tanning salon in Swansea on May 28.photo/provided
Every year, otherwise healthy New Zealanders die without warning. Health journalist Emma Russell writes that researchers hope the new funding will prevent more deaths.
Ifan Jones remembers kissing his 30-year-old wife goodbye and saying “I love you” as he does every morning before she goes to work.
The New Zealander was found dead at a sunbathing salon in Swansea, Wales, on the afternoon of Saturday 28 May.
Jones told the Herald on Sunday that Piata Tauwhare is healthy, likes to exercise, eats well, doesn’t smoke and rarely drinks alcohol.
While the coroner is investigating her cause of death, police have informed Jones that the cause of death is suspected to be Sudden Arrhythmic Death Syndrome (SADS), also known as Sudden Adult Death Syndrome.
SADS is an umbrella term used to describe death due to genetic instability of the heart after the heart stops beating, usually in healthy people under the age of 40.
This condition is different from a heart attack, which is a blockage in the arteries that carry blood and oxygen to the heart due to cholesterol-containing fatty deposits that build up over time.
Tauwhare, who was born in Hokitika, has no known heart disease and no family history of heart disease, Jones said.
Knowing nothing about SADS means Jones faces more questions than answers, he said, and his world is broken.
“It’s ruined my life. I go to football training and go home and she’s not there, my life is ruined,” he told the Herald on Sunday.
“She was everything to me, so down to earth and never spoke ill of anyone.”
The New Zealand Coroner’s Office has recorded 16 deaths from SADS in the past five years, but the total number of New Zealanders dying from the genetic disorder is likely much higher because not all deaths are referred to the coroner.
In 2008, cardiologist and electrophysiologist Martin Stiles and a team from Auckland University’s Waikato Clinical School developed a registry with seed funding from Cure Kids.
It is designed to help identify and protect young people who may be at risk of SADS.
Anyone who dies or survives an unexplained cardiac arrest can be referred by the hospital to Stiles and his team, usually a cardiologist. The Justice Department can also file death cases without cause.
Further testing was then performed to confirm SADS. The researchers then worked to track family members who might be at risk and whether they would be willing to have genetic blood tests.
In most cases, each first-degree relative is at a 50 percent risk, Stiles said.
“What we’re most interested in is that it affects young adults, so it’s usually between the ages of 1 and 40, after which non-genetic diseases start to dominate,” Stiles said.
Stiles said they had 5,092 registered members — most of whom were at-risk family members, and some died from SADS.
“Families feel sad when they see their loved ones pass away and, oddly enough, sometimes they feel guilty about the fact that they passed the genetic disease on to their children. However, they no longer feel that way because of the genetic disease. Guilt. The genetic disease is more important than the gene they passed on for blue eyes.”
He said his team, which includes social workers, psychologists and genetic counselors, is dedicated to helping families through the process of investigating the cause of their child’s death.
There are a number of preventive measures that can help reduce risk, including avoiding certain activities and reducing alcohol intake, Stiles said.
Fever can be a high-risk period, so people at risk need to “aggressively” treat fever with paracetamol and fluids.
People at risk for SADS may need to have a defibrillator implanted to shock their heart in the event of a cardiac arrest.
Stiles said while Auckland, Waikato and Wellington each had people looking for homes in their wider areas, none were available in the South Island.
“It’s kind of weird the way things are happening, but that’s how the money goes down.”
The team has applied to Pūtahi Manawa of Healthy Hearts in Aotearoa, New Zealand (HHANZ), for further research funding to fill this inequality gap.
Stiles said they will hear in the next few weeks on whether this is a success.
There is also a racial disadvantage, as far fewer people of Polynesian ancestry are known to have genetic disorders than those of European ancestry. That means it’s harder to test Māori and Pacific Islanders because their genetic database — made up of known mutations — is not as large as their Pakha counterparts.
Stiles said further funding would help address the issue.
“We’ll be looking at Māori families affected by a genetic disorder but without a genetic diagnosis, and we’ll be conducting detailed genetic studies aimed at ‘upgrading’ any genetic variant to a ‘causative mutation’.”
SADS’ South Island registry will help families like Greg Watchman, 57, who died after returning from an hour-long bike ride.
On a blustery November afternoon, Blenheim’s father used a ladder to retrieve an umbrella that had been blown to the roof.
When he came back, he was short of breath. He asked his 17-year-old stepdaughter to bring him a paper bag to breathe.
She complied, but by the time she returned, he had collapsed to the ground.
“Her boyfriend did CPR right away and I was home in six or seven minutes. The ambulance and fire brigade had arrived by then,” Greg’s wife, Andrea, said.
CPR was performed for 40 minutes, but he could not wake up. He passed away on November 15, 2018.
That day still haunts Andrea.
“It was totally unexpected, no one saw it coming. I still don’t understand what happened,” his widow said.
Meanwhile, warehouse worker Jones said the loss of his wife had been tough for him, friends and family.
“I’m a mess, I’m just frustrated and I don’t know what to do with myself.”
He said Tauwhare’s family and friends showed him great love and support when he visited New Zealand a few months ago.
Jones said her family was understandably devastated.
The couple met on an evening in Bristol, England, two years ago, while the Kiwis were travelling.
“She’s amazing, I’ve never [met] Anyone liked that before,” Jones said.
The couple married on September 1 last year in a small ceremony in Swansea, witnessed only by his parents.
Jones described his wife – who works for a mental health service called VitaMinds – as a very generous person and very proud of her New Zealand heritage.
Other Kiwis lost to SADS
Blenheim mum-of-two Anita Dell was 38 when her husband woke up gasping for breath. She went into cardiac arrest and couldn’t wake up. She had no previous heart disease or underlying health conditions.
Richmond mum-of-three Leanne Gardyne, 46, was 46 when her husband woke up in August 2019 to find her breathing hard. Her hands clenched, trembling, her pupils dilated. He moved her to the floor to help her breathe and called emergency services, but she couldn’t wake up. She worked as a seafood processing worker.
Charles Gray, 66, from Blenheim, was reported to be in “good spirits” on the day of his death. He was cooking the eggs when they started to burn and the house was filled with smoke. Soon after, he was found dead on the lawn.
Nikki Goodfellow, a 50-year-old cashier from Mapua, gasped loudly in her sleep. Her husband tried to wake her, but was unsuccessful. Firefighters were the first to arrive at the scene but were unable to revive her.
Gerald Scott, 70, from Nelson, was spotted by a passing motorist on a public footpath in Nelson. Emergency services performed CPR, but he was unable to resuscitate.
Phillip Patira, 52, from Christchurch, was playing golf when he was found breathing hard, clutching his chest, collapsing and becoming unresponsive. He suffers from non-insulin dependent diabetes, hypertension, gout and increased body mass index. The cause of death was SADS, the coroner found.