“People say I look well but I have ‘half-a-heart’: a hidden disability”

Scroll through 19-year old Amy Poynton’s Instagram grid and you’ll find a post flagged with a trigger warning. Behind that warning is a disquieting photograph of newborn Amy in an incubator, her tiny body visible beneath a complex web of tubes, bandages and breathing appartatus. Underneath the post is this poignant line: “I can’t begin to imagine how this would have felt for my parents to see their first born daughter like this”.

At four days old, Amy had open heart surgery in Boston’s Children’s Hospital and would go on to have two further major heart surgeries before her third birthday. Amy has ‘half-a-heart’ – hypoplastic left heart syndrome (HLHS), a complex, rare congenital heart condition in which the left-sided heart structures – which include the aorta, the aortic valve, left ventricle and mitral valve – are underdeveloped and can not support the circulation of oxygenated blood around the body. Without surgery intervention, HLHS is almost always fatal.

Surgical Procedure

Amy’s story begins in 2004. Her parents had moved seperately to Boston in the US for where they met at work and fell in love. When the couple found themselves expecting Amy, their first child, they were thrilled. Then a routine 20 week scan revealed HLHS, a rare congenital heart condition that affects about one in 5000 children. In Amy’s case, as in most, the cause of her HLHS in unknown.

Congenital heart disease (CHD) is the most common birth defect worldwide, encompassing myriad heart conditions ranging from mild to severe and affects approximately one in every 100 babies born in Ireland each year.

“At the time”, Amy says of her parents, “their plan was to move back to Ireland, but my mum spoke to a cardiologist in Our Lady’s Hospital for Sick Children in Crumlin. He said, “Stay where you are. The surgery and the survival rate is so much better in America”. In 2004, the survival rate for the first surgery Amy needed, the Norwood procedure, was only 5% in Ireland compared to 75-80% in the US. Amy’s parents stayed in the US.

HLHS generally requires three surgeries; the Norwood procedure normally within two weeks of birth; the bi-directional Glen shunt procedure at four to six months, and the Fontan procedure between eighteen months and three years. Its a harrowing schedule for any parent to contemplate. The multiple surgeries – which do not cure HLHS – increase blood flow to the body and bypass the heart’s poorly functioning left side. The right ventricle becomes the main pumping chamber to the body. From the outset, Amy’s journey was a tough one.

“My mum and I suffered very badly from ‘white coat syndrome’ – the fear of hospitals and doctors when I was around two to three. I got some medical notes from them recently. One said that I was so afraid, I wouldn’t let the doctor touch me. Mum said I’d start crying because I would think they were going to poke me with a needle.

Toddler Amy also had post-traumatic stress disorder, as well as night terrors and separation anxiety. She began seeing a psychologist to help her work through the trauma and she continues to work with a therapist today.

Amy began school in the US in 2009, having been supported since her surgeries with early intervention in the form of speech therapy, physio, a feeding specialist and a home nurse, on and off, for three years and in 2011, the family – Amy has two younger siblings – moved back to Ireland. Amy then seven, was thrilled. “I was really excited because my grandparents lived here. The only reason we stayed in the US was because of me. If the doctors don’t need to see you for a year, you get the ‘golden ticket’. The doctors told my parents, “It’s okay. You can move”. So they moved.

In Ireland, Amy came under the care of Dr Paul Oslizlok, Consultant Cardiologist in Our Lady’s Hospital for Sick Children in Crumlin, for whom she is full of praise. Amy’s school and teachers were wonderfully supportive, but the realisation that her energy levels did not match that of her peers gradually became apparent to her, as did the fact that her life was not the same as other children’s.

“I found it very difficult. As a child, you don’t understand what is happening to you. I had a lot of pain around my chest as a result of the surgeries. I didn’t understand why everyone else got to do cool things and I couldn’t. Why I had to go to the hospital all the time and why I constantly seeing doctors.

Secondary school brought its own challenges. “My school and my teachers were absolutely brilliant. But my mental health suffered. You have to deal with all that a normal teenager would go through but then there’s other stuff, the cardiac side, on top. When other teens were worrying about the Leaving Certificate, I was wondering, ‘am I going to have to have my next surgery and when are they going to schedule that?’

The surgery was cardiac catheterisation, an invasive diagnostic procedure that provides important information about the heart’s structure and functioning. She said, I love studying, so to be told ‘I don’t think you should sit your Leaving was very upsetting. They said it was putting too much stress and strain on me’. Despite not sitting her exams, Amy followed an alternative route to third-level education and is now studying psychology in UCD.

HLHS like all congenital heart conditions is an invisible disability. Amy, as far as she is aware is the oldest person living with the condition in Ireland. Thanks to medical advances the life expectancy for most HLHS patients stretches well into adulthood. Amy is determined to keep the conversation going.

Credit: Gemma Fullam, Irish Independent article, Feb 2024.