St. Marys Journal Argus
Katie Simons of St. Marys now counts herself as one of “The Heart Moms” — a group of parents who, on a regular basis, communicate with each other about the challenges of raising a child with a congenital heart defect.
But information and a sense of community weren’t always so close at hand for Katie and her husband Matt, parents to two year-old twins Luke and Will — and now six month-old younger brother Nicholas. “Those first months were really tough because I didn’t know a soul who was going through this,” she told the Journal Argus in an interview last week.
Diagnosed in the 22nd week of Katie’s pregnancy with hypoplastic left heart syndrome — the severe under-development of one of the heart’s two “pumps” (ventricles) — Will went through one open-heart surgery at 10 days old and another six months later, and has continued to suffer minor complications arising from the fact that the oxygen saturation in his blood is only about 75 per cent that of a person with a fully-functioning heart. He’ll require one more open-heart surgery, possibly later this year, and the life expectancy of his one functioning ventricle is not as long as a normal person’s heart. It’s expected that, by the time he’s 30, he will require a heart transplant.
Congenital Heart Defect Week
With Feb. 10-14 being marked as Congenital Heart Defect Week — within the Canadian Heart and Stroke Foundation’s February-long Heart and Stroke Month celebration — the Simons are hopeful that the awareness will gradually increase that, out of 10 people who decide to have a child, one of those children will be born with some form of congenital heart defect.
Will’s condition is of a particularly rare, but also particularly life-threatening variety. He’s one of about 100 people now living in Canada who were born with hypoplastic left heart syndrome — known in non-scientific terms as “half a heart syndrome.” It’s estimated that the condition occurs once in every approximately 32,000 births.
“There’s a lot of awareness of so-called ‘lifestyle-acquired’ heart disease, but not so much for these little guys,” Katie offers. Congenital Heart Defect week allows the medical community and patients’ families to encourage people to support research into both cures and further improvements, to celebrate the technological advancements that have made possible the reconstructive surgery performed on Will (35 years ago, the only option at birth would have been a highly-risky infant heart transplant), and to let people know they’re not alone if they find out their baby is going to be affected.
“I’d love to be that person to contact if they’re in that situation,” Katie said, referring to the effect it had on her and Matt when they eventually were able to make connections with other parents going through the same things as them.
Surgery at Sick Kids
After an initial diagnosis during pregnancy, the real struggles for the Simons began when Katie was admitted in London for the birth of the twins by C-section. They knew in advance that Will would almost certainly have to be transported to Toronto’s Sick Kids Hospital for the surgery, so they had made arrangements with extended family in Toronto. Initially, however, doctors in London monitored his oxygen saturation levels and described him as “stable.”
On Day 8, the levels plummeted. The call to Sick Kids was made.
“We moved down to Toronto...On Day 9, they called from Sick Kids and said, ‘Tomorrow morning. 6 a.m. Be here. Open heart surgery’.”
“It was just a whirlwind. We were new parents, with twins. Everything was new.”
It’s called a “partial Norwood” surgery. They inserted a 3-mm shunt from Will’s aorta to his pulmonary artery. Matt describes it as a procedure to allow the blood to travel from the one working ventricle in two parallel streams to the lungs and to the body. It’s not how the system works in people with two working ventricles, but it’s what’s needed to keep alive someone like Will.
“If you can imagine, these surgeons were working on a walnut,” Katie says. “Because that’s the size of his heart at that time.”
“Those first 72 hours were pretty rough.” Will’s blood was clotting very easily. Before long, it stopped moving through the shunt and he went into cardiac arrest — the first of two times it would happen in those first few days. She vividly mimics the public address system in the hospital from that time, with a call of “Code Blue: Surgeon needed in the CU.” Then she recounts how the surgeon ran past them on his way to the operating room, and came by later with Will’s blood splattered on his surgical gown.
Will was placed on blood thinners to combat the clotting, and on a form of life support, but the tiny baby soon lost a huge proportion of his total blood volume. So he was taken off the blood thinners, and “they basically had to take him off life support to save him.”
“We tell people we hardly saw Will for two weeks. There were tubes coming out of him all over the place,” Katie continues.
Mom and Dad credit the staff at Sick Kids for saving their son’s life. That, and the fact that baby Will — who, as has been remarked on numerous occasions ever since, was certainly appropriately named — refused to give up.
“Basically, for the next three months, it was just Will trying to stabilize himself.” For 2 ½ weeks, he had an open sternum. He was swollen from the surgery and doctors didn’t want to put pressure on his insides by sewing him up.
“I’ve been asked several times, ‘are you a nurse?’” That’s because they both know so much of the terminology about Will’s condition. But it’s because Sick Kids is so adamant that the parents are empowered with knowledge about what’s happening to their child.
McDonald House waiting list
The twins were born on Oct. 11. Will was discharged from Sick Kids on Christmas Eve. They put themselves on a waiting list as soon as they knew they would be going to Toronto for the Ronald McDonald House, but only actually received a spot the week before Will was discharged. “We were lucky to have such a great support system in Toronto, but we still spent enough money as it was with eating out, and gas, and basically living out of our van for several weeks.” They met a woman from Nova Scotia who’s on her own with her child in Toronto and had been there for a year. Another person had been living for five years at Ronald McDonald House, which includes a school in the Toronto facility.
Staff at Sick Kids was very honest and forthcoming about Will’s condition. There was no attempt to sugar-coat the message, and the people at Ronald McDonald House were the same. They all had a particular language they would use, with particular words to refer to their children — how they had “coped” with the surgery; whether they were “stable.”
“One day, when I asked how Will was, the doctor smiled and said, ‘he’s doing well.’ And I still get chills when I think about that. Because that really meant something to me,” Katie recalls. “He got moved to another room that day. And I remember going on Facebook and posting how the doctor said Will was doing well today.”
“Pretty scary” trip home
The Christmas Eve trip back to St. Marys was “pretty scary” because Will had not really interacted with his parents yet. He had only interacted with medical staff. Matt and Katie were instructed to hold him firmly, not to gently stroke him, because that wasn’t what he was accustomed to. They were instructed to avoid loud noises because he would be scared. It took them six hours to drive home, with a stop along the way at a friend’s house and a number of other stops to deal with Will’s crying. And then they were greeted at their home, on a surprise basis, by four loud, enthusiastic, gently-stroking grandparents.
And so began the long adjustment to being at home with three-month-old twins — one the recent recipient of open-heart surgery.
Will still required a needle every day, which his parents had to administer. He was only six pounds at the time so, after a few days, finding a new injection site became a challenge. Luckily, a nurse also checked in every day for the first little while. There were once-a-week visits to either a pediatrician or cardiologist.
“We were lucky because Will really ate well.” They were told that his target weight for the next surgery was 10 pounds, but it was unlikely he would reach that weight before he required that surgery at wfour months of age. Instead, with regular check-ups with the cardiologist, it was determined that Will was able to wait until six months of age before his second surgery. And, because he was eating so well, by that time he was almost 14 pounds. When they went back to Sick Kids in April, the surgeons were thrilled.
This time, they were admitted on April 4 and discharged April 21. “It was harder, in a way, because now you know him. Before, yes, he was our offspring. But this time, he has a personality.”
The second surgery was called a “GLENN,” and it’s designed to get the blood flowing to the head.
“We joke that, this time, he only had a collapsed lung,” Katie smiles. This happened after a breathing tube was removed following surgery, and was something the doctors were prepared for. “We almost lost him last time — several times. So this was like a walk in the park.”
Because the second surgery targets the head, the patient typically suffers from headaches and swelling in the head as a result. So Will was on strong painkillers for several days after he returned home.
The third surgery, which may take place in the fall of this year, will bring the blood flow to the limbs in sync with the other workings of the single functioning ventricle.
“He’ll never have a full heart...but they’re trying to stimulate the heart as best as they can,” explains Mom.
Consider him lucky
They consider the complications since the second surgery as minor. A few days after returning to St. Marys, Will was re-admitted in London because he was having trouble with food getting into his respiratory tract. There were some kidney issues, and he has what his parents describe as “very low eyesight” that is being corrected through glasses.
“We’re very lucky. And William is very lucky in the fact that he’s eating,” Matt says. “Most kids with his condition are tube-fed.”
“I think, being a twin, at the end of the day, has really helped him,” Katie adds. “Luke doesn’t know that Will has a heart condition. He just wants him to be a brother.” And, rather than coddling Will, they’ve encouraged him to do whatever Luke is doing. From Day 1, despite long days spent in an ICU where Luke wasn’t allowed to be, they haven’t wanted Will’s condition to take away from the attention they provide for Luke.
“People say, ‘you’re so strong’. But I remember there was a sign on the door at the Ronald McDonald House, and it said, ‘You don’t know how strong you are until you’ve got no choice but to be strong’.”
They heard from doctors that Will “probably won’t eat food. He probably won’t grow. He probably won’t survive the surgery. And it’s almost like, step after step after step, he has just checked them off...He has been given these obstacles and he just keeps overcoming them.”
Katie reads information from the Canadian Congenital Heart Alliance about the outcomes for adults born with heart defects, and they’re troubling — the number of people with depression, etc. So they want to focus on the positive and encourage Will to do the best he can.
“He’s got a lot to look forward to,” she says, as he dumps another bucket of toys out onto the living room floor, wearing his red-and-white Canada sweatshirt, as images of Canadian Olympians — many of whom have also overcome long odds — play out on the television screen above his head.