Since I was 11 days old, my every heartbeat has been dependant on a pacemaker. I was born with complete heart block, an abnormal heart rhythm that means the electrical impulses that control my heartbeat are blocked and do not reach the bottom chambers (ventricles) of my heart.

The surgery I received in 1978 at Glasgow’s Yorkhill Hospital was a world first. Since then, while I’ve lived a very full life, it’s one that’s been reliant on pioneering cardiac treatment. Life as a ‘heart child’ involved countless invasive, painful and often frightening cardiac interventions. By the age of seven I had been fitted with five pacemakers, each by thoracotomy – my ribs were split to gain access to my heart. Early pacemakers were set at a fixed rate, making active play, gym and sports day impossible. They were unreliable and often needing reprogramming. I remember lying still and feeling dizzy as a team of men in white coats armed with a magnet, leads, cold gel, stethoscopes, and electrodes made my heart beat faster, then slower as they learned how to interrogate the pacemaker. I was a medical curiosity, although my team always did their very best to support us. I needed open heart surgery at 13 years old to repair a hole in my heart and around this time I was fitted with a more physiologically responsive, variable rate pacemaker.   The challenges don’t end, last summer I was fitted with my 11th pacemaker involving complex surgery to extract and replace pacing leads.

“By the age of seven I had been fitted with five pacemakers, each by thoracotomy – my ribs were split to gain access to my heart.”

Complex congenital heart disease (CHD) cannot be cured, but due to medical advances a growing number of people are living with a lifelong heart condition. Babies born in the 1940s, had a 20% chance of surviving into adulthood. Today, the chances for a baby born with CHD living that long are 90%. Yet the success story of enhanced detection, medical and surgical advances yields new challenges which healthcare services have not developed to meet. Having overcome significant childhood adversity, many ‘medical orphans’, once celebrated as pioneers of medical advancements, are being let down in adulthood. But with adequate, reliable care many of us can live full lives.

Developing standards to improve services

Healthcare standards offer a means to significantly improve care by engendering trust, safety and patient empowerment. That’s why I’m delighted to be involved with Healthcare Improvement Scotland’s development group to create standards for CHD, and work with them to enable people with this condition to live as normal a life as possible.

Standards are statements relating to levels of performance that people should expect from health services. They are based on evidence relating to effective clinical practice, feasibility and service provision that is responsive to patients’ needs and views. They cover the key issues relating to the provision of safe, effective and person-centred care and treatment.

It’s been a long journey to get to this stage, supported by the cardiologists, nurses and surgeons involved in our specialist care, who witness daily what it means to live with CHD. Given my own experience, and my voluntary work as the Scottish Campaign Manager for The Somerville Foundation (a UK charity supporting adults with CHD), in 2012 I sought to raise awareness of CHD, submitting a petition to The Scottish Public Petitions Committee at the Scottish Parliament asking for healthcare standards and improved care provision, joining Cross Party Groups, asking MSPs to submit parliamentary questions and lobbying through social media.

“It’s vital that healthcare provision for CHD is underpinned by a psychologically-informed approach consistent with recent drives for trauma-informed care and to address adverse childhood experiences.”

The challenges for people with CHD

But what exactly are the issues that people with CHD can face that the standards will look to  address?

CHD is the most common congenital anomaly, affecting approximately 1% of babies. A quarter of these babies require medical intervention in the first year of life. Around 20,000 people with CHD currently live in Scotland. Approximately half of this estimated adult population are lost to specialist services, often having been discharged before the need for specialist lifelong care was recognised.

It’s vital that healthcare provision for CHD is underpinned by a psychologically-informed approach consistent with recent drives for trauma-informed care and to address adverse childhood experiences. While medical advances have saved many lives, they can involve invasive procedures which can be frightening, painful and overwhelming. Early separation due to medical care may also impact on attachment to parents. Living with CHD can reach far beyond any medical limitations to affect schooling, employment, finances, discrimination, missing out and issues having and raising a family, to name a few. Perhaps not surprisingly, CHD significantly increases vulnerability to neurodevelopmental issues, depression, anxiety and post-traumatic stress disorder (PTSD) while parental psychological wellbeing can also be affected. Yet these outcomes need not be inevitable. By adopting a psychologically-informed approach to care, these risks could be mitigated, improving wellbeing, physical health, recovery and ultimately reduce costs. 

Although I am grateful to the NHS for saving my life, I have experienced multiple life-threatening challenges in my care provision and a lack of understanding about the psychological impact of living with a serious lifelong health condition. At times, living with CHD can seem relentless. Having to fight for appropriate medical care, at your most vulnerable, can feel overwhelming. The challenges don’t end. Many of the challenges we face occur at a local level, for example, when we present to our GPs and emergency care. Standards for local care provision are vital to inform safe, equitable and consistent care. I am far from alone experiencing these challenges.

We should be proud of our NHS as testament of our collective humanity. But having experienced it at its best and worst since my first pacemaker all those years ago, I want to help to make it better. I owe that in return.

More information

Take part in the consultation on the scope of our congenital heart disease standards.

Liza is a Chartered Counselling Psychologist working in private practice and at Strathclyde University.  She is actively involved in research and advocacy to promote psychologically informed medicine and improved care for people living with a heart condition from birth. Liza represents patients on the Standards Development Group for CHD for Healthcare Improvement Scotland. You can follow her on Twitter @drlizamorton.