Living Fully: Quality of Life with a Single Ventricle Heart

"Do you get scared when you have to go to the hospital?" I casually asked our six-year-old, Emanuela, following a Pediatric Quality of Life (PedsQL) questionnaire. My interest was piqued after reading about it in a study* assessing the quality of life (QoL) among 12-year-olds with serious Congenital Heart Defects (CHD). I wanted to understand how our daughter, with her single ventricle heart, perceives her own life.

The Burden of Clinical Interventions

"Never," she replied without hesitation. "Because I know everyone there," she added, offering a simple, childlike perspective on our hospital visits. She's familiar with her doctors. Nurses are friendly, kind, and gentle, and she likes them. She considers herself brave, even in the face of a "little poke-poke" as we refer to a blood draw; at least until she sees the needle. But that's looking at a one-off visit. What about the cumulative impact of countless hospital stays, surgeries, and being heavily medicated?

For many, quality of life is about nurturing relationships with friends and family, pursuing happiness, finding joy in small things, staying active, and striking a balance between work and life that favors meaningful life. Most of them, however, do not have a serious medical condition. Life with, for example, a single ventricle heart, can be fairly different.

Children with serious CHDs experience lower QoL than unaffected classmates. This appears related to the burden of clinical intervention rather than underlying cardiac diagnosis.
(conclusions of the study: "Patient-reported quality of life outcomes for children with serious congenital heart defects.")

Emanuela approached the questionnaire with a sense of playfulness, but we continued:
Do you catch colds more often than other kids? Sometimes.
Do you get scared while waiting to see the doctor? Sometimes.
Do you ever refuse to take your heart medicine? Sometimes.
Is it hard to ask the doctors and nurses questions? Sometimes.

As we progressed, her responses became more thought-through. It took her a moment longer to answer whether she has trouble expressing her feelings to doctors and nurses or explaining her heart condition to others. "Never," she finally said.

Promoting Good Coping Strategies

Lately, she's been taking books about a heart like hers, scars, and about being different, to kindergarten. Her teachers receive them warmly, reading the stories aloud to the class, and she returns home with pride. It melts my heart, unlike the experience from last summer.

"Why was she stitched so much?" asked an older girl, making a grimace and pointing to the scar peeking out from the top of Emanuela's swimsuit. Unfazed, Emanuela pulled down her swimsuit to reveal a tapestry of scars, tracing their path from the soft flesh between her collarbones to a few centimeters below her belly button. Triggered by the novelty of the situation, I jumped into the conversation: "That's the scar superheroes wear!" My instinctive need to (over)protect her in this way took me by surprise. While, at the same time, I was in awe of her unshakable composure. She wears over 16 scars on her upper body, but the inquisitive girl spotted another one on Emanuela's thigh. She pointed, eyes widening, and exclaimed: "She has a hole there!" then laughed straight at my girl's face. Suddenly, curiosity tasted mean. But Ema... she took it so innocently, as if everyone's skin would be like hers - scarred. With head held high, she immediately showed the finger-pointing girl another hole-shaped memory for life that she has next to her belly button and explained: "Yes, and here's where I had a small button through which I received milk as a baby!"

I sometimes wonder - how to keep her that way? Positive about her image. Humorous. Child-like. Kind and friendly. And above all, proud of her scars - a testament to the battles she won.

Increasing Emotional Well-being

Tyler Šajdák is an adult with Hypoplastic Left Heart Syndrome and a Fontan circulation. He was featured in a podcast Fontan With a Future, where he talked about his school years: "The biggest issue growing up was not my heart, it was more the small-town politics of sports." Issues like whether or not he will make the team, dealing with kids behaving mean, and being bullied for being unable to keep up... That takes a toll on a young person.

Yet, as adults... I recently met Tyler and a few others with hearts similar to Emanuela's. The lives they live... I never imagined it possible - sporty, educated, employed, active in their communities, some experienced pregnancy and gave birth or otherwise built their own families... Granted, their realities differ from those of their healthy peers, but witnessing their determination to maximize their mental and physical potential, refusing to let their diagnoses define them more than necessary, fills me with awe.

It is conceivable that some children with CHDs who participate fully in school and sports rate their psychosocial QoL high despite scoring their physical functioning lower, because they understand implicitly that they are ‘successfully’ negotiating the physical limitations of their condition.
(conclusions of the study: "Patient-reported quality of life outcomes for children with serious congenital heart defects.")

As Emanuela only begins to discover and ask questions about her heart, her scars, and what sets her apart from her peers, I take her answers to our little PedsQL survey exercise with a pinch of salt. After all, she's still just a child. Yet, a child's perspective should never be dismissed.

A child-centred approach is fundamental to communication between children, families, health and education professionals about individual care, as well as to promoting good coping strategies and social inclusion to enhance the lives of children with CHDs for whom long-term survival in adulthood is now a realistic expectation.
(conclusions of the study: "Patient-reported quality of life outcomes for children with serious congenital heart defects.")

Understanding the way Emanuela sees herself guides me in identifying areas to work on or seeking help when needed.

She occasionally struggles with writing letters or words, she continued answering, but never with numbers or math worksheets, nor with listening to the teacher (despite her teacher's contrary opinion 😄). It's reassuring that she never experiences difficulty breathing during sports activity or chest pain because, as the study suggests, despite children like my daughter reporting lower QoL, engaging in sports and staying active can significantly boost emotional well-being. Regardless of them being aware of sometimes needing more rest than their healthy friends.

*Knowles, R. L., Day, T. G., Wade, A., Bull, C., Wren, C., & Dezateux, C. (2014). Patient-reported quality of life outcomes for children with serious congenital heart defects. Archives of Disease in Childhood, 99(5), 413-419.