what it feels like: one day at a time with congenital heart disease
nathalie lacoste-hofmann considers herself lucky that she didn’t have to wait long for a donor — lucky to get a donor at all — but strange to have another human’s heart beating inside her.
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"try to surround yourself with people who are supportive," nathalie lacoste-hofmann says of her journey living with congenital heart disease.
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when nathalie lacoste-hofmann was born in the summer of ‘69, she says her parents had “the best 20 minutes of their lives,” but the minutes that followed turned into their worst. baby nathalie’s pink little face faded to blue as she struggled to breathe.
she was quickly stabilized and nestled into an incubator, where she spent the next several weeks. her diagnosis: congenital heart disease.
“my parents were told they could keep me and have lots of debt — this was just before universal health care — or they could put me in palliative care,” says lacoste-hofmann. “bless their hearts, they kept me.”
there was definitely a high cost. it was more emotional than financial, as health care kicked in about a year after she was born, covering most of her medical bills. emotionally, it was a roller-coaster. once she was strong enough to go home,lacoste-hofmann’s parents kept emergency services on speed dial because their newborn would frequently turn blue and end up back in an oxygen tent.
later, when she was older and allowed to go to school, her mother had to stay home and near the phone — just in case.
what is congenital heart disease?
the word “congenital” means present at birth. the disease occurs when the chambers, walls or valves of the heart, or the blood vessels near the heart, don’t develop normally before birth, according to the heart & stroke foundation of canada.
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about one in 100 canadian babies is born with congenital heart disease (chd), according to the canadian congenital heart alliance (ccha). it is the most common congenital disability in the world. the ccha notes that there are roughly 40 types of chd, ranging from simple to moderately complex to highly complex.
there are currently about 260,000 canadians living with chd, two-thirds of them adults. sixty years ago, fewer than 20 percent of children with chd survived to adulthood; today, it’s more than 90 percent, according to the heart & stroke foundation.
congenital heart disease treatment: the road to a heart transplant
the cause of most chds is often unknown but can include the fetus being exposed to maternal illnesses such as:
- fever
- diabetes
- rubella
- smoking, alcohol or drugs during pregnancy
- exposure to air pollution or pesticides
- inherited genetic defects and chromosomal abnormalities
lacoste-hofmann, a retired teacher living in whitby, ont., says chd does not run in her family. her mother was a smoker, but she quit when she learned she was pregnant. she says her only sibling, a sister, was born with a cleft lip, another congenital condition. “i think there were genetic accidents at work, but we don’t know why; they’re not necessarily connected.”
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when she was nine months old, lacoste-hofmann’s doctors inserted a blalock-thomas-taussig shunt, a temporary measure to increase blood flow to her lungs and decrease her risk of turning blue. (it’s actually called “the blue baby operation.”)
at around four, she began taking commonly used drugs prescribed to chd patients, such as digoxin, amiodarone, coumadin, beta-blockers, inotropes, and, when she got infections, antibiotics.
at 10, lacoste-hofmann’s health took a downturn. luckily, her father’s employer had recently transferred him from montreal to markham, near toronto and that city’s sick kids hospital.
there, she underwent the fontan procedure, open-heart surgery, which allows oxygenated-poor blood from the lower body to bypass the heart and go straight to the pulmonary artery and onto the lungs.
“that made me pretty close to normal,” she says. “if we hadn’t moved, i probably wouldn’t be here.”
during her mid-twenties, she underwent a series of cardioversions — quick electric shocks to reset her heart rhythm. she also had a cardiac ablation to block irregular electrical signals. that helped only temporarily—a few years later, she had another, which was more advanced. with her heart ticking along relatively normally, she was able to have a child. “but i knew that i wasn’t going to have a second one biologically because it was a risky pregnancy.” two years later, she and her husband adopted a daughter.
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in 2013, with two young children to care for, lacoste-hofmann became very ill with multiple arrhythmias. admitted to toronto general hospital (tgh), she underwent a second fontan procedure, which included the insertion of a pacemaker. “that kept me going for another ten years so i could raise my kids,” she says
two years ago, at the age of 52, lacoste-hofmann got the big one: a heart transplant. “we were in lockdown because of covid. i was teaching online for nine hours a day, which pretty much sent me into heart failure. there was not much more to be done. they’d done all the palliative-type surgeries, so they recommended a heart transplant.”
she says it was a hard decision whether to go through with it. “i thought, ‘i’ve had a good life. my kids are almost fully cooked.’ but my husband, in particular, felt that he wasn’t ready for me to go, that i had lots of living still to do.”
she considers herself lucky that she didn’t have to wait long for a donor — lucky to get a donor at all — but strange to have another human’s heart beating inside her. “it was weird initially, but it’s also [about] being really grateful. somebody had to die for me to be better. that was a little rough. i didn’t want you to die, but thank you.”
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throughout, she had times when she was struggling mentally and emotionally. “when i was in the hospital in 2013, i asked to see a psychologist. and [again] in the year after the transplant. i don’t know if it was my age or how big the event was, but that seemed to have messed me up more than anything else in my life. so, i had a bit more trouble and was seeing a psychologist for about a year.”
living with congenital heart disease: ‘i try to focus on the positive’
lacoste-hofmann says she had a relatively normal childhood despite her condition, although she couldn’t go on long walks or do rigorous exercise. “there was a lot of imaginary play rather than riding bikes and such,” she says. her parents tried not to restrict her activities too much but were ever vigilant.
she begged to go into a swimming pool at two, against the doctor’s orders. getting too cold while having unoxygenated blood can shrink capillaries, further restricting oxygen into the blood, which can be dangerous.
“but i wanted to be in there so much that they took me in slowly, checked me, made sure i wasn’t turning blue.”
when she became an adult, that vigilance over her health fell to herself. she has always kept fit by walking, hiking, biking, and dancing. she has a healthy diet, never smoked or did drugs, and drinks alcohol in small amounts and only on special occasions. although she says her stress-reduction strategies could use some work, she does make sure to get plenty of sleep.
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after her heart transplant, she had to take early retirement, primarily because of her condition but also because her old job was no longer available. “i was a teacher-librarian for ten years and absolutely loved that position. if i could have gone back to that, i probably would have gone back to work.”
instead, the only option open to her was teaching language, science and math to classrooms of 60 germ-filled five- and six-year-olds — not ideal for someone immunosuppressed. so, coming out of major life-saving surgery, unemployed, having her mom just die and her kids away at school, lacoste-hofmann wasn’t sure what came next. “it was everything all at once, and i was trying to figure out how to reorient my life to the way it was before all this.”
she says she used the tools she learned from the psychologist. “she helped me find things that would make me happy, how to refocus where i wanted to go. i have a job now where i help elderly people, i volunteer with organizations that i like, and i get to learn some technology. i love it.”
while she says she was ultimately lucky to find things that gave her life meaning again, lacoste-hofmann says other heart patients she befriended throughout her journey didn’t always have such a smooth pathway.
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“some of the people i met were followed by cardiologists in their localities and only referred to tgh when things went bad. i suspect that if they had been followed at tgh their outcomes might have been better. i was extremely blessed to be where i was, but i am not the majority. i think a lot of people do not have the same care i have had. a lot of people are treated by normal cardiologists who do not have specialty [in chd].
according to the ccha, there are only 15 dedicated centres across the country that treat adults with congenital heart disease. for those who live away from major centres like toronto, montreal, vancouver and edmonton, accessing chd-specific care is more of a challenge.
as for advice for others on their own journey, lacoste-hofmann says, “it’s really important to be informed and ask questions. the ccha website is great for that but ask your doctor questions. if you don’t ask questions, you can’t advocate for yourself.
“also, try to surround yourself with people who are supportive. the ups and downs will always come. i get grumbly days, but i try to focus on the positive and understand that today might not be great, but tomorrow might be fine. i’m not religious, but i’ve always believed that life is good, that people are generally good, and if one door closes, another one will open. if you tough it out, things always turn around.”
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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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