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A Special Heart

Written by Jen Janzen

On April 6, Evanna Irvine was admitted into the Stollery for emergency surgery. The 23-month-old had developed an infection in her sternotomy (a sternum-separating procedure that allows surgeons to access a patients heart), and the Stollery team needed to put the toddler under so they could clean out the infection.

For many parents, having a child transported by air ambulance to a children’s hospital for emergency surgery doesn’t warrant a celebration, but for Kierra Irvine, Evanna’s mother, coming to the Stollery and having out in the pediatric cardiac intensive care unit (PCCIU) was almost like arriving at a family reunion.

“The Stollery PCICU was kind of my second home,” Kierra says, noting that in 2015 she and Evanna had lived in the unit for eight months. “Those intensivists, those respiratory therapists (RTs) and those nurses are my family. As weird as that sounds, I was kind of home.”

When Kierra’s husband arrived from their home in Calgary to join his family a few days later, he joked to Kierra that she was having “way to much fun” at the Stollery. “I was just totally in my element,” she says.

The April 7 surgery also happened to be little Evanna’s ninth ICU transfer, so the whole family, including Nick, Kierra’s husband and Evanna’s father, were pros at ICU life.

Evanna is a 26-month-old toddler with big blue eyes and fuzzy strawberry blonde hair. Born on April 29, 2014, Kierra says her daughter has always been a calm, happy baby. That mellow disposition has served Evanna well: born with a congenital heart defect known as tetralogy of Fallot with pulmonary atresia, ASD/VSD and MAPCAs, she spent a lot of time in the hospital.

Evanna’s condition is a tongue twister of a diagnosis caused by a rare chromosome defect. If the condition’s name is complex, the definitions are even more so. There’s pulmonary atresia, which means the pulmonary artery didn’t develop properly (in Evanna’s case, the pulmonary artery didn’t develop at all). A tube, known as a conduit, is placed in her heart, but will have to be replaced as she grows and, as an adult, will have to be replaced if the conduits start to get blocked.

ASD and VSD stand for atrial and ventricular septal defect, and refer to the hole between her top and bottom heart chambers. This causes oxygenation and deoxygenated blood to mix together, which means less oxygen gets to Evanna’s tissues.

If Evanna only had these three conditions, she would be eligible for a heart transplant should she ever need one. But she also has MAPCAs, or major aortopulmonary collateral arteries. It’s an extreamly rare condition in which, in the absence of pulmonary artery, the heart finds a way to the lungs via little pathways. “Collaterals” is a word you’ll hear Kierra use frequently, and these narrow, unpredictable and sometimes incomplete pathways are what she’s referring to. Those collaterals are the reason Evanna will never be a candidate for a heart transplant: because her heart and lungs are so thoroughly connected, her only chose is to wait for a heart and lung transplant, something she’s still too young for, and depending on the circumstances may not be an option.

Kierra explains it all on her website, Irvine Family Home, which is a blog she started before Evanna was born. It chronicles her life, her marriage to Nick, and Evanna’s “heart journey”. Initially started as a creative outlet, it’s evolved to fill a higher purpose: not only is it a way for Kierra to meet other parents of children with congenital heart defects but it will also be an important medical resource for Evanna as she gets older.

“She’s going to know what procedures she’s had and what those entailed. Evanna is going to know details surrounding every admission,” Kierra explains, adding that it’s been helpful for her and Nick to refer back to the Irvine Family Home website to remember important details about previous procedures or appointments.

And there have been a lot of procedures. Thought Evanna got to spend her first few months at home – with continuous monitoring of her oxygen levels, which were thankfully quite high – Kierra and Nick knew she’d have to have open-heart surgery once she’d grown a bit more. Evanna’s first open-heart surgery took place in October 2014, when she was just five months old. Clicking through Kierra’s blog, there was dozens of images of a tiny infant in a hospital bed, surround by complicated machines.

In her brief life, little Evanna has had nine surgeries, three of them being open heart. Due to a series of complications, extubation struggles and a string of infections – she spent more than 10.5 months consecutively in the ICU, transferring back and forth from the Alberta Children’s Hospital in Calgary to the pediatric cardiac specialists at the Stollery in Edmonton. Two of those transfers to Edmonton were via STARS air ambulance – the first for a clot in her heart’s mechanical valve and the second one for a sepsis infection, which happened last year on Mother’s Day.

Kierra vividly remembers that Mother’s Day emergency transfer. One of Evanna’s Stollery doctors, Dr. Adatia, who knew how long the little one had spent in the ICU, apologized for needing to bring Evanna back. Kierra quickly replied that there was no need to feel bad. “Evanna was taken by STARS – we followed behind by car – and we almost breathed a little sigh of relief the moment we entered city limits. We literally felt our bodies relax the moment we knew she landed in Edmonton.”

Last November, on Evanna’s 400th day in hospital, Kierra and Nick finally got to take their daughter home. Since they’ve been home, Kierra says they’re trying to pick up where they left off. Kierra returned to work earlier this year, resuming her post as an access and disclosure specialist for Alberta Health Services. Since Evanna has a tracheostomy and is on four litres of oxygen a day, she requires around-the-clock care (homecare nurses take over the nighttime shift).

Now over two years old and no longer confined to a hospital bed, Evanna is making up for lost time. “Because of our extensive hospital admission, developmentally she’s closer to a 10-month-old,” Kierra said. “She excels at sitting up and will hopefully start crawling by the end of the summer.” To help Evanna’s legs and feet get stronger, she spends sometime in a standing fame every day. “She should develop normally and she should be able to walk and run one day, but we’re very behind. She’s like a 10-month-old, even neurologically, because she was basically asleep and confined to a hospital bed for over a year.”

Although the Irvines hope that Evanna will be decannulated one day (meaning she no longer requires the tracheostomy), which would mean she wouldn’t need to visit the respiratory clinic anymore, she’ll need to see a cardiologist the rest of her life.

On the eve of Evanna’s first open-heart surgery, Kierra wrote a letter to her daughter and posted it to the family blog. In it, Kierra spoke of her hopes for the surgery, as well as her fears. “It’s been a count-down to his moment ever since we first found out about your special heart, and while at times it seemed to be such a long ways away, in hindsight, it came too quickly,” she wrote.

If Kierra and Nick had known the trials they and their daughter were going to face after that first open-heart surgery, there’s no doubt that they would have been terrified. But, after living through 400 days of dramatic ups and downs, they’ve learned how to take it one day at a time.

“She has defied some pretty big odds already,” Kierra said. And that, more than anything, is what explains Kierra and Nick’s calmness earlier this year as they returned to the Stollery’s PCICU for Evanna’s minor infection-clearing surgery.

Hope for children like Evanna is why Kierra is such a strong supporter of the Stollery. “Heart defects are actually the most common birth defect,” she says, nothing that one in every hundred babies is born with one. "More awareness brings more dollars and more dollars means more research. Even 20 years ago, the original open-heart surgery Evanna had back in October 2014 (called unifocalization) wouldn’t have existed.

That’s why she joined the Stollery’s annual radiothon in January 2015 while Evanna was in the ICU. Kierra couldn’t participate in this year’s radiothon, as Evanna was having another surgery, but she says she’s up for anything. “Anything I can do for the Stollery, they know they have me at their fingertips.”

Published Summer 2016, Stollery Children’s Foundation