how i care for congenital heart disease: 'we thought we were going to lose her'
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now 18, rachael, right, is off all meds, in first-year university, and doing well, says her mother lisa wright, left.
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lisa wright’s first pregnancy in 2005 was normal, healthy, and uneventful. when her baby was born via c-section, she was a beautiful, good-sized, pink-cheeked girl whom she named rachael. after checking over the infant and being satisfied that all was well, the obstetrician washed up and left. then, things took a turn. one of the nurses noticed a murmur in rachael’s tiny heart.
“but she said they hear this all the time, not to worry, they just have to double check it,” recalls wright.
there was cause for worry, however, when rachael’s oxygen levels dropped and she turned blue. the beautiful new baby had pulmonary stenosis, a narrowing of the pulmonary valve that sends blood flow to the lungs.
the london, ont. hospital was not equipped to handle such a serious situation, so rachael was loaded onto a helicopter and flown to sick kids hospital in toronto, her dad racing behind in his car. surgeons quickly performed a balloon valvuloplasty to open up the valves and improve blood flow through the heart valve.
but rachael’s heart still wasn’t pumping enough blood, and she went into cardiogenic shock. the newborn would spend three weeks in intensive care and, all told, six weeks at the hospital, where doctors ultimately diagnosed her with congenital heart disease (chd).
“those three weeks were probably the scariest time,” says wright. “we thought we were going to lose her.”
what is congenital heart disease?
chd is a heart condition you’re born with, “congenital” meaning present at birth, according to the heart & stroke foundation of canada. it happens when the chambers, walls or valves of the heart, or the blood vessels near the heart, don’t develop normally before birth. there are roughly 40 types of chd, ranging from minor and causing no issues to major, requiring a range of treatments, including open-heart surgery and heart transplant.
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according to the canadian congenital heart alliance (ccha), about one in 100 canadian babies is born with chd. about 260,000 canadians live with chd, two-thirds of those adults. sixty years ago, only about 20 per cent of kids survived to adulthood; today more than 90 per cent do.
there is often no known cause, but some chds are brought on due to:
- a mother’s illness, such as diabetes, rubella, or fever during pregnancy
- smoking, drinking or drug use while pregnant
- environmental factors such as air pollution, pesticides, and heat waves
- certain genetic defects and chromosomal abnormalities.
wright says there are no heart issues in her immediate family, but an aunt did have a heart defect.
chd can be diagnosed during pregnancy via ultrasound or after birth when symptoms appear, such as a rapid heartbeat or breathing, a blue tinge to the skin, and swelling and fatigue. treatment depends on the severity and can include lifestyle changes, medication, or surgery.
living with congenital heart disease
rachael’s type isn’t one of the named chds, says wright. instead, she has a mix of a hypoplastic right heart — a very small, tight right heart that didn’t form properly on the right side — and a hypoplastic tricuspid valve, in which the right-sided structures are also underdeveloped.
doctors performed a series of catheterizations, hoping to hold off on open-heart surgery until at least six months when rachael had grown, gained weight and become strong enough. luckily, they were able to wait 20 months. she then underwent bi-directional bilateral glenn shunt surgery to take the pressure off her right ventricle, which wasn’t pumping enough oxygenated blood. she also had a large atrial septal defect (a hole in the wall that divides the heart’s upper chambers) repaired at the same time.
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wright says the first five years were the hardest. rachael was limited in what activities she could do because of her reduced energy and endurance. then, in grade 1, she became allergic to the cold and spent all her school recesses inside.
at 10, doctors fitted her with a holter monitor, a type of portable electrocardiogram, to record her heart’s electrical activity for 24 hours. that revealed ventricular tachycardia, a type of abnormal heart rhythm that occurs when the lower chamber of the heart beats too fast, limiting the amount of oxygenated blood in the body.
back at sick kids, doctors implanted a loop recorder in rachael’s chest, designed to pick up electrical signals from her heart. she lived with that for three years, during which there were a couple of visits to the hospital when the recorder didn’t like what it was hearing.
wright says there was also a period when rachael had to carry a portable defibrillator, donated by ontario charity the mikey network, in her backpack wherever she went. “it was hard to say to another family who was having a birthday party, ‘rachael is coming, but i have to keep a defibrillator at your house.’ how many parents of eight-year-olds want to be the ones responsible for that? but at the same time, it allowed her to go and for us to know she was safe.”
at stages throughout her young life, rachael was on a dozen different medications, which her parents diligently kept track of via calendars and alerts.
“it’s definitely not an easy journey for parents of children with heart defects. it takes time to get used to that world.”
keeping calm and carrying on
wright says remaining on high alert for her daughter over so many years while keeping relatively calm was a challenge. “i probably was more [stressed] than my husband, i’ll admit that. if something came up, like a cold allergy, it became bigger because she has a heart condition. every time she got a cold, she would be sicker than the other kids. and you’re always concerned it will turn into pneumonia.”
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she says it’s doubly difficult when it’s your first child, where you’re already grappling with being a new parent. “and when you’re told your child has a heart defect, everything becomes questionable. is she supposed to have the hiccups? is that okay?
“i remember being at sick kids and asking one of the nurses, ‘what’s her life going to be like? what will she be able to do?’ and she said, ‘they find their own place. they just seem to know themselves.’ and it’s true. rachael never really shied away from anything.”
wright says the constant vigilance, appointments, and procedures also affected her other two children, no matter how hard she and her husband tried to keep the balance so it didn’t appear rachael was favoured over her siblings. “but other family members would give her the attention. if she was sick, there was always another stuffed animal showing up at the door. there probably has been a bit of jealousy here and there, but it’s always been worked out. they just learned that rachael has had some difficult times in her life.”
to connect with other parents of children with heart defects, she started a london chapter of the ccha called hearts of london/middlesex, where she and her husband offer and receive support and knowledge from others going through similar situations.
now 18, rachael is off all meds, in first-year university, and doing well, says wright. she’s making the all-important transition from pediatric cardiology care to adult care and taking on the responsibility of making her own appointments and seeing doctors by herself, which hasn’t been without its own set of challenges.
“there was no plan. it just sort of happened,” says wright of the process. “when we had her last appointment with pediatric cardiology in london, it was quite an emotional experience. she’s been going there since she was a newborn, and [medical staff] become like family. and then it’s, ‘okay, this is our last appointment with you. next time, you’ll be seeing dr. so and so.’”
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“it was eye-opening to see my daughter so vulnerable. it would have been nice to have been able to meet the new doctor for just five minutes. [practitioners] see this all the time; they must be able to anticipate things like that. maybe they could have a social worker available to support the family during the transition, to make sure she’s going to make the appointments and not feel scared to go to these appointments.”
like her mother, rachael is a member of the child and youth advisory council at children’s hospital, london health sciences centre, where she offers input on how to improve some of the systems. she’s also a child ambassador at the charitable organization children’s health foundation, sharing her own experiences to help others.
advice for other parents in caring for congenital heart disease
wright says, having been through 18 years of critical care for a daughter with a heart defect, she wants other parents to know how important it is to ask questions. the better informed you are, the better you are at spotting potential problems so you can act on them quickly.
“you know your child the best, what their norm is. we had to trust the doctors, and we had to start trusting ourselves that we knew rachael’s norm. we had to equip ourselves to be comfortable with that.”
she says she remembers when rachael was about six months old. wright was sitting in the waiting room of the cardiology unit during yet another appointment, looking at other healthy kids and wondering, is rachael ever going to be 10?
“it’s a scary thought when they’re so little, and all you think is that they have this terrible thing wrong with them. but you have to trust our amazing medical people. as much as there are things that could be better, there’s also amazing people who do care, who do get to know these amazing little people and help them.
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“where you are in this moment is not going to be where you will be forever. it gets easier, and it gets better.”
to learn more about chd, check out the information and resources offered by the heart & stroke foundation, the ccha, the cardiology unit at sick kids, and the western canadian children’s heart network.
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