A choice no parent wants to make
Brisbane author Emma Gilkison and partner Roy Costilla were living in New Zealand, in 2014, when they were told that there was something wrong with their unborn son.
He had ectopia cordis, a condition where his heart was growing on the outside of his body. The congenital defect is so rare it only occurs in eight babies in every million.
He also had a second heart problem, hypoplastic left heart syndrome (HLHS) - the left side of his heart was severely underdeveloped.
Emma and Roy had to make a decision - end the pregnancy, or give birth knowing their baby - named Jesús - would die.
"It was going to be heartbreak whatever we did," she said.
"To be honest it was the hardest decision of our lives. There were two options, neither of which we wanted."
So she began to search for answers. Could her baby be saved? Were there other examples where a child has survived? Why was this happening? What could they do?
Emma has since turned the family's experiences into her new book, The Heart of Jesús Valentino.
"I hope that if there are any families that find themselves in circumstances similar to ours - that they have to face the grief of a child dying - hopefully this book can offer them something," she said. "It's also a way to honour our son. His life was so brief, but I like to think that something good will come from his life now."
Little Jesús lived for just 15 days.
Last year, the couple moved to Brisbane with their son Amaru, who is now two. Roy is a research fellow the Institute for Molecular Bioscience at the University of Queensland. Emma finished writing her book here - often at The Avid Reader, West End - and the manuscript was published by New Zealand's Awa Press in October.
"Life is good now," Emma says.
"We are happy to be here in Brisbane and Amaru keeps us very busy and is a source of great love."
She goes silent for a moment.
"When people ask how many kids I have always say I have two," she says, quietly. "The first one - JV as we refer to him - still feels very much like part of our family. I don't even like to say that I have lost a son, he still feels close to me and has always been such a big part of my life and is always in my heart."
The family is now passionately campaigning for the Victor Chang Cardiac Institute's Christmas appeal, which hopes to raise more than $120,000 this year for research into congenital heart defects.
"They are an amazing research institute that is right at the forefront of research into congenital heart detects like ectopia cordis and HLHS. I know that the institute is specifically targeting HLHS, and they are making amazing discoveries in that area. It was a no-brainer for us to be part of supporting them in their work," she says.
"We don't have any regrets (with our choice), but we'd like to think there's a future where families might not have to go through what we did."
She said it was also important to remember that Christmas can be a sensitive time for families who have lost a child.
"It's nice if our babies and children are still remembered in Christmas cards, or maybe in a special decoration on the tree," she says.
"I know lots of people feel awkward not knowing whether to mention a child who has died, but in my experience families appreciate the acknowledgment."
Below is an extract from The Heart of Jesús Valentino, about the moment Emma and Roy were told about their baby's rare condition:
■ The Heart of Jesús Valentino, Emma Gilkison, Awa Press,$35
■ Emma Gilkison will speak at Avid Reader Bookshop, 193 Boundary St, West End, Brisbane on Wednesday, December 19, 6.30-8pm.
■ The family is the face of this year's Victor Chang Cardiac Research Institute Christmas fundraising appeal, with the aim of raising more than $120,000. Every four hours another baby is born in Australia with a heart defect. While Jesús condition was too severe for him to be saved, the institute's work saves lives. Donate here.
Receiving the diagnosis: baby's heart outside
By Emma Gilkison
'What's that above our baby's chest?' I asked.
The sonographer was looking at the same thing as me on the scan screen. In the swirl of black and white it looked as if there was a little star flickering on top of our baby's sternum. 'I've never seen anything like that before,' she said. 'All I can tell you is that this is very unusual.'
Roy and I locked eyes and he squeezed my hand. This was our first baby and it had taken us a year to conceive. I'm a New Zealander and Roy hails from Peru. I'd already begun wondering what shade of caramel our child's skin would be; that he or she would have big brown eyes seemed a given.
In the first few weeks of my pregnancy I'd had some bleeding and had been on tenterhooks, fearing I was going to miscarry. But I hadn't. Now we'd finally made it to the 12-week mark, I thought I could safely say all was well with my pregnancy.
'They can sometimes do operations in utero … you're going to be quite the talk around here.' The sonographer was now talking in half-sentences. She finished the scan and told us a doctor would be in touch. At the reception, a staff member gave us a blue folder containing a souvenir scan image. In it, our baby was lying on its back and clearly visible above its chest was a small, round orb.
'What do you think it is mi amor?' (I asked).
'I don't know but we need to wait for the diagnosis before jumping to conclusions,' he said.
I agreed but after I'd dropped him at university and returned home my mind kept returning to the scan and what it meant. Could that star above our baby's chest possibly have been its heart?
I Googled my suspicions: 12-week scan baby with heart outside of chest. Immediately there was a potential answer: ectopia cordis. An extremely rare condition, affecting eight in one million babies, where the heart quite literally grows outside of the body.
I trawled through websites and saw images I couldn't help but recoil from, including a dead baby with an exposed heart upon its chest. My imagination went into overdrive and I saw Roy and I launching a campaign to raise money for an expensive, lifesaving operation. I saw a huddle of doctors in white coats animatedly discussing our baby's heart at a meeting. I saw a funeral at St Paul's cathedral with a tiny coffin and Roy and I in the front row, leaning against each other in our grief, too emotional to speak to anyone.
Later that afternoon my midwife called with the diagnosis made by the doctor who had looked at the scan images. She confirmed it was our baby's heart we had seen outside the chest. The condition was ectopia cordis. I had been right. Somehow I wasn't surprised. Our baby was one of the eight in a million.
One of my first reactions was to think that my body had done something wrong, causing my baby's heart problem. 'I'm so sorry little one, I'm so sorry,' I cried, my arms hugging my stomach.
In truth, the cause of ectopia cordis is unknown. The name comes from the Greek for 'away/out of place' and the Latin for 'heart'. After birth the prognosis for ectopia cordis is very poor. The outside world is too much for an uncovered heart. Most babies die shortly after birth due to infection, cardiac failure or lack of oxygen. Texas Children's Hospital estimates that 90 per cent of ectopia cordis babies are either stillborn or die within their first three days of life. In many cases, an ectopia cordis heart has other defects too.
Due to the rarity of the condition and the short lifespan of babies after birth, few treatment options have been developed.
Yet there are success stories. Surgery has been carried out to create a nest in the chest within which the baby's heart can sit and skin grafts to seal it over.
Soon after getting the diagnosis, I began researching the option of surgery. I found a clip on YouTube of a toddler called Audrina, who was born in January 2013 with ectopia cordis and was saved by a team of doctors in the States.
She wore a pink plastic corset to shield her heart for several months after surgery. She was reported to be doing well on release from hospital. I tracked down Audrina's mother on Facebook and sent her a message.
She replied: 'Hello Emma, I'm so sorry to hear about ur baby boy. It's hard to hear the Drs state that there is nothing to do besides terminate ur pregnancy. Audrina is doing quite well and if I woulda terminated we wouldn't never know what a blessing she would be. Every situation is different and I'm willing to answer any questions I can for u. I hope u have a blessed day.'
We were now under the care of a maternal foetal medicine team at Wellington hospital. They were unsure if surgery would be possible for our baby, but agreed to refer us to a paediatric cardiologist at Auckland hospital. By the time of this appointment I was almost five months pregnant and had begun to feel my baby's first kicks. Small thuds, that went knock-knock-knock against the drumskin of my stomach. I already felt deeply bonded with this little being inside me. We now knew he was a boy and had even begun thinking of names. I liked 'Valentino' because I'd found out I was pregnant on Valentine's Day. Roy suggested Jesús, pronounced Hey-Zeus, a common name in South America.
But the paediatric cardiologist in Auckland didn't have good news. Along with the ectopia cordis, there were other abnormalities in our son's heart's structure. One of the ventricles was too small, the aortic valve was too narrow. It was a list of problems too long for any surgeon to attempt to fix. We were told he might not make it to full term and if he did he would die at birth or shortly after. The paradox of modern medicine is that knowing doesn't always mean the outcome will be better. Sometimes you can just see death coming from a long way off.
My partner Roy and I now had to choose between two heartbreaking options: to end the pregnancy or to continue in the knowledge our son would die. It was the hardest decision we'd ever had to make.
I wondered if I would feel less grief if I terminated. Or should we let our baby's life unfold naturally? But if I continued the pregnancy, wouldn't I be miserable, feeling that I was waiting for my baby to die? What choice held the most possibility for love? Does a baby have a soul in the womb? Do souls endure and do they have any power to determine when and how they are born? Was there a reason our baby had the heart he did? What would our son choose if he could: to be born, even if only to live for a few breaths, or never to be born at all?
There was no easy way to answer these questions. Whatever we did was going to mean a leap into a dark unknown. To make matters more difficult, Roy and I began leaning in opposite directions. Although Roy was raised Catholic and I am Buddhist, religion wasn't a factor in our decision making.
Roy hated the idea of our baby being in pain at birth or afterwards and thought we would only be inflicting suffering on him by continuing the pregnancy. I could see his point, but I didn't know if I could go through with a termination. At the stage of pregnancy I was at, this would involve taking drugs to induce labour. A massive dose of hormones would abruptly undo all of my body's instincts to safeguard the baby inside. There would be contractions, pain, and the tiny baby I would deliver might still show signs of life.
I began to think I might want to continue the pregnancy, even though I knew my baby was destined to die. Before finding myself in this situation, if I'd heard of a woman making this choice, I would have assumed she was a religious martyr or that she was running away from the inevitable. As often happens in life, when you actually find yourself in an extreme situation, your perspective changes, you see things differently.
What it came down to for me was this: if a loved one was given six months to live, would I choose to farewell them as quickly as possible, or would I choose to cherish every moment I had with them? The situation wasn't that different with our baby, albeit we would be loving him within the womb and perhaps only for the briefest time after he was born.
After weeks of soul-searching, crying and arguing, Roy and I finally agreed that we would continue the pregnancy. We would meet our son who wore his heart on his chest.
An extract from The Heart of Jesús Valentino, Emma Gilkison, Awa Press,$35