New Brunswick·Feature

A complicated heart: Facing my baby's mortality before his life began

My son's only chance at life would be surgery on his walnut-size heart just 48 hours after he was born.

Avalanche of unknowns was unleashed by the news my son's heart needed to be taken apart and put together again

Ellis at home. About one in every 80 to 100 children is born with congenital heart disease, making it the number one birth defect. (Maria Jose Burgos/CBC)

My heart sank when I heard the doctor's words: "I can't tell if there's shadow covering what I'm trying to find in the baby's heart, or if I can't see it because it isn't there."

My husband and I were at the hospital in Saint John for an ultrasound. It was a formality. Our baby hadn't co-operated during the standard 20-week anatomy screen a couple of weeks earlier, and it was impossible to get decent images of his heart.

For this ultrasound, called a Level 2, the doctor took his time gathering images, so we could leave reassured and get back to the business of announcing the gender (another boy) and choosing a middle name for our second child.

We already knew we would call him Ellis.

And that day, we learned there was something critically wrong with Ellis's heart.

I went about setting up Ellis's nursery with worst-case scenarios playing out in my mind. (Maria Jose Burgos/CBC)

Double outlet right ventricle. Transposition of the great arteries. A ventricular septal defect. Interrupted aortic arch.

These complex congenital heart defects were going to derail the normal pathways for oxygen to get to his body, which he vitally needed to survive.

Open heart surgery was his only chance, and it had to happen within days of his birth.

The operation needed to be done at a hospital with highly specialized care, away from our family in New Brunswick. That ended up being SickKids hospital in Toronto, believed by doctors to be the best fit for our son's  complicated heart.

Doctors thought SickKids in Toronto would be the best hospital for Ellis's heart surgery. (Shutterstock/JHVEPhoto)

As the ninth month of my pregnancy began, we hastily made plans for the move two provinces away, my looming due date carrying a heavy sense of dread.

As long as he was growing inside my womb, where my placenta supplied the oxygen, our baby was safe. But he would quickly run into trouble once he was out in the world, taking his first independent breaths.

So many unknowns

Facing your child's mortality before he is even born exposes a parent to unimaginable stress and sorrow.

My husband, Chuck, described it as a computer program constantly running in the background, slowing down all the other software.

We moved to the Toronto region in my ninth month of pregnancy, where my husband, Chuck Teed, and I played the waiting game for Ellis's birth and heart surgery. (Submitted by Sarah Trainor)

We picked Ellis's middle name, Orion — a constellation that brings brightness to the dark night sky.

I washed and sorted his newborn clothes, my mind flooding with worst-case scenarios, and who I could to turn to in case they had to be discreetly put away.

For me, the hardest part was the build-up, and the avalanche of unknowns.

Numbers on a page told us his chances of surviving surgery — anywhere from 85 to 93 per cent.

A long checklist resembling a grocery order described the types of complications he could run into during the massive repair. We wouldn't know what form the surgery would take until we signed the consent form the day after he was born.

At one of my later ultrasounds in Toronto, a thoughtful technician added a personalized greeting for my older son on one of my scans. (Submitted by Sarah Trainor)

We were fortunate that advances in maternal screening and echocardiograms afforded us the gift of early detection, giving us time to mentally prepare and a skilful team time to assemble and plot a full course of treatment.

"Over the decades, there's been a huge improvement in our ability to diagnose these abnormalities, so children have a better chance of survival," said Dr. Kenny Wong, a pediatric cardiologist at the IWK Health Centre in Halifax.

Uncaught defects

Still, malformations in the heart often get missed in the ultrasound.

Another safeguard to catch critical defects is a non-invasive newborn test known as a pulse oximetry screen.

Sarah Trainor tells the story of her son Ellis and his complicated heart

5 years ago
Duration 3:56
CBC's Sarah Trainor faced an avalanche of unknowns when she learned her son had congenital heart disease. His survival depended on an open-heart surgery performed only 48 hours after he was born.

The test, which measures oxygen levels in the blood, isn't part of the universal newborn screens in New Brunswick. Nor is it being considered, said Health Department spokesperson Paul Bradley.

Isabelle Allain-Labelle's son, Theo, also faced open heart surgery at SickKids soon after he was born, but his heart defects weren't detected ahead of time.

Theo was born with multiple severe, complex heart defects and in a critical state when he needed his surgery. 

"We thought we were going to the hospital for two days and coming home with a healthy baby," Allain-Labelle said. "And that was not the case."

"It was very traumatic, as you're really not prepared."

Theo was taken by emergency airlift from Moncton to Halifax and then to Toronto.

Isabelle Allain-Labelle's son, Theo, was rushed to Toronto after multiple severe and complex heart defects were discovered. (Submitted by Isabelle Allain-Labelle)

My husband and I, on the other hand, arrived in Toronto on a commercial flight four weeks before our son was born.

My preparation involved coming to terms with my pregnancy suddenly veering off course.

Our oldest son, Alden, was born at term following a complication-free pregnancy. My labour progressed without need for interventions, not even an epidural.

We soaked in an hour of skin-to-skin time before Alden was measured and weighed, then promptly put into my arms, then my husband's, then mine again. We were home the next day.

Only a brief touch 

Nothing was the same this time. Now a high-risk pregnancy, I had ultrasounds every three weeks, then weekly.

Right after delivery at Mount Sinai Hospital, Ellis's heart had to be stabilized through intravenous medication.

The most skin contact I was afforded was about five minutes to stroke his hand and forehead before he was moved across the street through an underground tunnel to the cardiac critical care unit of SickKids.

Ellis gets one last cuddle and stroke of his smooth chest at 48 hours old, before he's handed over to a nurse to prep for open heart surgery. (Submitted by Sarah Trainor)

Five hours later, he was finally in my arms, though we had to manoeuvre around a pile of cords and sensors connecting his little body to monitors.

Once again I was discharged from hospital after 24 hours, only this time I was leaving my baby behind.

There was no picture-perfect introduction with his older brother — Alden remained with the rest of my family back in New Brunswick.

We stayed in frequent contact over video chat and did our best to explain at a two-year-old's level of understanding that doctors at a special hospital were making his brother's sick heart feel better.

9 hours in operating room

In the end, Ellis qualified for the type of surgery we desperately wanted, an arterial switch operation, like solving Rubik's Cube. If successful, it would move the malformed parts to their proper positions, giving him as close to a normal, functioning heart as we could hope.

His birth weight was almost nine pounds, meaning his surgeon would be working with a heart the size of an avocado pit — slightly bigger than the walnut-sized hearts these specialists normally see.

The night before his surgery, his "roommate" in the opposite bed — a creative girl with her own sewing and beadwork stand next to her cot — surprised us with a beaded necklace that spelled Ellis's name. She made it because she thought his IV pole looked empty.

Ellis's bravery beads represent the various tests and procedures he had to endure during his three weeks spent at SickKids. (Maria Jose Burgos/CBC)

I wore it the next day for good luck.

Ellis was 48 hours old when he went into surgery. It lasted nine hours, and five of those hours he spent on a heart bypass machine.

His aorta and pulmonary artery that incorrectly lay side by side were taken apart, criss-crossed and reattached to their right places in the lower chambers of his heart.

His coronary arteries that supply blood to the heart, only about one to two millimetres in diameter, also had to be delicately moved around and put back into place. The hole in his heart was closed, and the undeveloped part of his aortic arch was replaced with a patch material.

Done in isolation, these repairs posed minimal risk, but there was great potential for complications because of the number of corrections Ellis needed.

After surgery, Ellis spent a week covered head to toe in tubes and sensors, and so swollen the surgeons couldn't close his sternum for seven days. (Submitted by Sarah Trainor)

We were warned of the risks of stroke, brain damage, and the possible need for life support in case his heart needed to rest.

Knowing a baby's heart

Amazingly, aside from needing multiple blood transfusions, Ellis sailed through his surgery with a complete repair.

"If you were to tell me yesterday we'd have these results, I would have said, 'Good,'" our masterful surgeon told us in the waiting room, with the faintest hint of a smile.

I felt a weight lift from my shoulders for the first time in months.

Ellis's successful repair was largely due to innovations over the years that make it possible for surgeons to know the details of a baby's heart before they step into the operating room.

Doctors now use 3D printed models of hearts to get a better look at defects, and they rehearse complex surgical procedures on soft models.

After Ellis was extubated, a CPAP machine helped to slowly wean him off oxygen support. (Submitted by Sarah Trainor)

The heart surgery survival rate at SickKids is 98.5 per cent.

"The challenges for a family are always about the unknowns of what's going to happen next to their child," said Dr. Wong.

"And thankfully with research and keeping track of our results of our successes and challenges, we're in a position where we're able to tell a family what's likely to happen."

Doesn't end with surgery

Ellis had cleared a major hurdle, but his recovery was far from simple. His body was under so much stress from the invasive surgery, he spent a week covered head to toe in tubes and sensors, and so swollen the surgeons couldn't close his sternum for seven days.

Three days into his recovery, his heart rate spiked and blood pressure dropped, leading to a barrage of tests to rule out sepsis or a problem with the repair. Thankfully, his body simply needed more time on the medications that were supporting his heart.

For several more days we were in a holding pattern, then some steady progress. Chest closure. Extubation. A slow wean off medications and oxygen.

Nine days after the surgery, I was able to hold him. He was allowed some of my milk. A few days later we were moved out of the critical ward. Almost three full weeks after his surgery, we were discharged.

Life has become very typical for our family since Ellis was discharged from hospital. (Amy Stewart Photography)

Healing following open heart surgery isn't always this linear.

Some children with heart disease spend months in hospital, sidelined by strokes, brain injuries, cardiac arrest, blood clots, organ damage, feeding struggles and infection. Some are waiting for a new heart. Too many never go home.

Heart parents find each other

Thrown into the strange, scary world of critically sick children, heart parents become fierce advocates for awareness.

Allain-Labelle, Theo's mom, became a board member of the Braveheart Support Society, a group in the Maritimes for families of children with congenital heart disease, often shortened to CHD.

She juggles those duties with a part-time health career and caring for Theo, who is two surgeries into a three-staged reconstruction of his heart. Theo has a single-ventricle heart, with one pumping chamber rather than two.

There is no cure for congenital heart disease — only repair. (Maria Jose Burgos/CBC)

"For me, it's so important to connect with other parents who have gone through similar situations and support them as much as I can, because I know how scary it is to give birth to a child with complex medical conditions," she said.

Another of her goals is to raise awareness in the community.

About one in every 80 to 100 children is born with congenital heart disease, making it the number one birth defect. The defects range from simple conditions that require no surgery, to life-threatening defects such as those my son experienced.

Congenital heart defects kill babies and children at twice the rate of all childhood cancer, according to the American Heart Association.

There is no cure, only repair, and surgery can give rise to secondary issues: repairs that fail to grow with the heart, a reliance on blood thinner injections and other medication, tube feeding, an increased risk of developmental delays.

More than 250,000 children and adult Canadians live with CHD today, outnumbering those with more recognized conditions such as multiple sclerosis and Parkinson's disease.

We picked Ellis's middle name, Orion — a constellation that brings brightness to the dark night sky. (Maria Jose Burgos/CBC)

Krista Vriend, 43, has tetralogy of Fallot, a combination of four related heart defects that causes low oxygen in the blood.

By age 12, she had two open heart surgeries and other interventions that resolved certain problems but never completely corrected her heart defects.

"I've had to go back and have a stent put in," said Vriend, president of the Canadian Congenital Heart Alliance. "I had a pacemaker put in for a little while as a precaution. And I know eventually I'll have to have another surgery."

As they transition into adulthood, people with the congenital heart disease have to keep on top of it, she said.

Vriend remembers feeling isolated as a teenager living with CHD, wrongly thinking no one had what she had.

"The likelihood of you knowing someone in some way or another connected to CHD is probably high, but awareness of the disease is very low," she said. "I think on a scale of one to 10, you're probably looking at a three."

On learning our son would be born with life-threatening heart defects, my husband, Chuck, described it as a computer program constantly running in the background, slowing down all the other software. (Maria Jose Burgos/CBC)

Sixty years ago, only about 20 per cent of children survived to adulthood. Today, more than 90 per cent do, although Vriend said the care of these adults hasn't received much attention. Only a quarter of them see cardiologists with training in their complex defects.

"Before, it was if you can get them to adulthood, that's great, and everything else is a bonus," Vriend said.

"Now, over the last couple decades, more people with CHD have been living well into adulthood, and that's when problems begin to surface again."

Scars tell a story

These days, Ellis is the picture of health.

At one and a half years old, he runs, climbs, steals his brother's cars, and regularly demands to have a new book read, or another round of Wheels on the Bus sung to him.

He charms everyone he meets with his ever-growing vocabulary, and somehow finds new ways to get into trouble every single day.

If you didn't know his story, or see his scars, you would never know Ellis was ever sick. We live the typical life of parents to a preschooler and toddler.

These days, unless you saw Ellis's scars, you would never know he was ever sick. (Submitted by Sarah Trainor)

"You come to realize how resilient our children are," said Allain-Labelle, who is inspired every day by Theo, now almost three, and his big smile.

"They can go on and have a very happy life, despite everything they've gone through."

Ellis isn't defined by his heart defects, but his mended heart is the reason he is alive today.

His scars tell the story of a resilient newborn who won the fight for his life before he knew what life was all about.

After Ellis's diagnosis, when I thought about our future, I felt as though I was looking at a water-damaged photo of our lives, the lines and colours blurring together. Are there two children, or one? Do we look happy?

That picture is clearer today, and yes, we are happy.

We still can't know what the future holds, but the difference is we're now armed with an arsenal of interventions improving with time, more knowledge, and much more hope.

February is Heart Month, and Feb. 7-14 is recognized as Congenital Heart Disease Awareness Week.