Reflections on Developing the CHD Action Plan
My name is Elle; I was born in 1983 with congenital heart disease and have had 5 open heart surgeries.
In 2018 I was fortunate enough to be asked to represent consumers on the National Steering Committee for the development of Australia’s first (CHD) National Action Plan. Naturally I jumped at this opportunity, and I have to say it’s been one of the most insightful and emotional experiences of my life.
Representing people with CHD and making sure our collective needs were included was something I took very seriously – this was our once in a generation shot and I wanted it to count. Using both my personal CHD experience and professional expertise I advocated hard on many issues to help shape this landmark piece of work.
Throughout the development of the National Action Plan I was truly flawed by how impressive the CHD community is. Their commitment and generosity for us is incredible. The importance of awareness about CHD was something that really hit home for me. Now I’m providing information about CHD to everyone and anyone. I even started my own social media to promote awareness.
From the bottom of my patchwork heart – thank you for this opportunity. I hope you are as proud of this achievement as I am, and that you can see issues that apply to your life in the National Action Plan and a pathway to make life better for everyone.
We are so lucky to have HeartKids! They did a fantastic job of pulling this whole thing off. I’ve had the chance to work with some impressive people in my career, but HeartKids (HK) and their Board take the cake. They are the only national charity dedicated to supporting kids and families affected by CHD, and have really lived up to their mission of Advocacy, Information, Research and Support for all of us.
The importance of consultation with the CHD community was front and centre in the development of the National Action Plan. I participated in workshops, teleconferences, and email exchanges along with parents of people with CHD, people with CHD, and the broader CHD community. The HK team worked tirelessly for the better part of a year to deliver this extremely important piece of work for us. Thank you HK team!
In Australia there are currently about 65,000-90,000 people with CHD, with around 1 in 100 babies born with CHD each year, and the adult population increasing annually by around 5%. That’s one heck of a community of people with CHD! The National Action Plan is for each and every one of them, their families, and the workforce and infrastructure that supports them.
Until the middle of 2018 I had met about 3 adults with CHD. From this process I now know hundreds. It’s been an inspiring and steep learning curve. It inspired me to join Facebook groups (like HeartKids) where I’m learning so much from others with CHD and their families. A simple question like ‘how much sleep do you normally need’ or ‘how do I prepare my child for school’ elicit up to a hundred responses and interesting discussions. In some ways it’s helped me validate things I’ve thought or felt my whole life, but in other ways it’s shown me the diversity of people with CHD and the struggles that some people live with.
CHD is a Chronic Condition
The Australian Government has officially classified CHD as a chronic condition, and the National Action Plan puts this at the forefront of all its plans. This fact was a complete revelation to me. You may think I’m a bit naive, but I’ve lived my whole life knowing I have complex CHD, but just thinking that things are sometimes just a little harder for me. I like more sleep, I have to be careful with how I exercise, overdoing can lead to serious illnesses, and my catch phrase is ‘I’m tired’.
I’ve put a lot of thought into what it means for me to have a chronic condition and I see how it can be a double edged sword. On the one hand, I’m not as harsh on myself when things are a bit hard – because I have a chronic condition you know. But on the other hand, I’m nervous that I may one day hide behind the label ‘chronic condition’ and not push myself to my full potential or use it as an excuse not to do something. Or worse yet, someone else will withhold opportunities because they see me as weak and unable to do everything - because I have a chronic condition you know. Luckily my personality is more inclined to push every boundary as hard as possible, and I tend to only work with people who appreciate my skills and push me when needed. It’s also more likely that my family and friends will remind me of my ‘chronic condition’ than anyone else.
A condition with no cure
While we have come a very long way in treating CHD, there is still no cure. Undergoing heart surgery is not a cure – people with CHD can face a lifetime of unique challenges including ongoing medical treatment and repeated heart surgeries. I’ve had 5 open heart surgeries and will need more surgery in the next few years to replace my pulmonary pig’s valve I had implanted in 2004. After that I’m not sure what else I will need, but something will need to be done to keep me ticking along. I understand that this is a very confronting prognosis, not only for people with CHD but their loved ones as well. HeartKids and the National Action Plan have a strong focus on research and other initiatives to improve the lives of people with CHD.
Setting the bar high
The Australian community of CHD medical specialists is pretty tight knit, they follow international guidelines, and have an informally high domestic standard of how things should be done for us. But… Australia does not have any formal national Standard of Care or Clinical Practice Guidelines. I found this rather odd as other countries have them (e.g. US and UK). I think that our geography and diverse population really dictate that we need our own domestic guidelines.
The National Action Plan calls for the development of a set of standards and service specifications that cover the entire patient pathway, from prevention, diagnosis, through to treatment, and on into care at home and end of life care, to make sure that every child, young person and adult with childhood heart disease, in every part of the country, will receive the same high standard of care. It also calls a subsequent road map and priorities for the development of national clinical practice guidelines.
The best of the best
To be a CHD cardiologist you not only have to be the best of the best, I learnt, but also very dedicated. These amazing humans do all the medical training of a cardiologist, but then have to do an additional 3 years and continuous training to be able to treat us. Problem is, there aren’t enough of them – especially for adults with CHD. There are about 34,000 adults with CHD, and only about 6 adult CHD specialists. Either I’m really bad at maths, or that’s not a sustainable ratio. There are also immediate workforce needs including CHD Clinical Nurse Coordinators, Sonographers (with portable echo machines) and Transition nurses. The National Action Plan outlines, wait for it… a Plan, to address these issues.
We’re going to have to research the heck out of this
There are so many unanswered questions around CHD that research and data could unlock for us. Research and surveillance are essential to strengthening evidence-based practice for the diagnosis and management of CHD; identifying ways of preventing or curing CHD; understanding the burden and impact of the disease; informing health policy and evaluating the effectiveness of efforts to reduce the physical, psychological and economic impacts of CHD including the uptake and impact of the National Action Plan. If our recommendations in the National Action Plan are taken up they will initiate and accelerate breakthroughs that guide support to change our lives.
Baby on board
Because it’s best that I don’t get pregnant due to my CHD I really haven’t kept up with neo-natal medical advances. In my ignorance I wasn’t aware that CHD is the most common congenital defect in newborns, and one of the leading causes of death and hospitalisation in infants. I also wasn’t aware that medical science had come so far as to be able to detect CHD prenatal. This is fantastic in helping families prepare for their special baby. It has also given parents, in specific circumstances, the choice of terminating the pregnancy. As someone with complex CHD I can see both sides of this choice and don’t envy parents who are in that position.
People with CHD have a greater risk of neurodevelopmental impairment and disability, including developmental delays and other learning difficulties. I got to meet one of the strongest, smartest, and most passionate heart mums whose child has CHD and a neurodevelopmental impairment. Meeting this special heart mum taught me so much about inconsistencies across services in Australia and it made me realise that more awareness, specialist care, and standardisation was required – which is what we advocated for in the National Action Plan.
It’s not particularly surprising that people living with a chronic condition for which there is no cure have higher mental health concerns. Just thinking about open heart surgery brings on mild anxiety, and it can be depressing on days when my body won’t do what I want it to. Almost 1 in 3 people living with CHD experience anxiety and depression. Additionally, almost 80% of parents of children with CHD have profound levels of psychological distress including anxiety, depression and post-traumatic stress disorder. The National Action Plan identified this as a key issue and proposes the development of world-first Mental Health and Neurodevelopmental Standards as part of the National Standards of Care among other initiatives.
Heart kids from the bush
The burden of CHD is disproportionately higher for heart kids from the bush (rural and regional Australia). Almost half of us live outside a metropolitan centre and need to travel more than 200km to see specialists and we pay more for the privilege to do so. HeartKids does a great job support kids from the bush, and the National Action Plan goes further in putting forward recommendations on how access and costs could be better addressed.
I grew up in Wagga Wagga, NSW and spent my childhood traveling between Wagga and Sydney for treatment. Luckily, my pediatric cardiologist did outreach day clinics a few times a year in Wagga which helped immensely. But, we still needed to travel the 8+ hours as a child for treatments (e.g. surgical interventions). My dad ran his own small business so when I had surgeries in Sydney he had to take time off work which meant less income, and increased out-of-pocket costs like accommodation, food, travel, and medical bills. It also disrupted my sibling’s education and life in general. Now I live in Canberra and still travel to Sydney to see my specialist adult CHD cardiologist as there isn’t one here. I am hopeful that other families don’t have to carry such a heavy burden in the future.
Aboriginal and Torres Strait Islander people experience higher rates of congenital and acquired heart disease and worse outcomes compared with non-Indigenous Australians. When I was sitting at the workshops to develop the National Action Plan I wasn’t too surprised with this fact, but I was pleasantly surprised at the dedication and generosity of the clinicians who supported this important population. These clinicians paid to hold clinics out of their own pocket, and they went above and beyond with their time to support people through the complex medical system. The National Action Plan has key focus on how we can better support Indigenous Australians across our country.
Lost CHD souls
The transition from pediatric to adult CHD care is fraught with issues resulting in more than 50% of people becoming ‘lost to care’. CHD is a lifelong condition that needs lifelong monitoring and care. The people who are lost through this transitional period have a higher risk of an adverse outcome. Furthermore, people with CHD need to see a specialist adult CHD cardiologist, rather than any old cardiologist, because they are more likely to have a better outcome – in plain English they will be less likely to die. HeartKids is onto this issue and are supporting Australia’s first National CHD Registry to ensure a continuum of care and assist with identifying those at a higher risk of complications later on in life. The National Action Plan supports this Registry and its expansion. We don’t want anyone else to be lost.
Adulting with CHD
If you were born with CHD in the 1980s or earlier, and are still around, you are part of the first cohort of people who survived. As we kept surviving into adulthood the question became ‘what’s next?’. I think that question still remains today. The National Action Plan tries to assist with the transition from pediatric care to adult care, and everything else that comes with being an adult with a chronic condition.
The transition from being a child with CHD to an adult with CHD for me was a bit jarring. The first time it really hit home was when I had to consent to a painful medical procedure I didn’t really want (but needed). Previously my parents signed for me so I didn’t exactly have a choice, but now it was my choice. There is also a whole lot of adult ‘stuff’ that I’ve had to learn to manage as an adult with CHD like insurance, work, study, travel, appointments, medications, surgeries, and romance. There was no guidebook, direction, or even hints at how to manage all of this. We just made it up as we went along (and still do). HeartKids is doing a great job to bridge this gap, but they need more resources. We need to prepare young adults for their coming reality in a way that doesn’t scare the bejebus out them, but leaves them empowered with the right knowledge.
Productive Australians with CHD
I haven’t come across a single person with CHD who doesn’t want to be a productive member of Australia. Not one. We don’t want to be a burden on Australia’s health care system, educational institutions, workforce, or broader economy. We want to work, travel, study, and make a contribution. Implementation of the National Action Plan will minimise the long-term adverse medical, psychological and economic effects that undermine the successes of early treatment and prevent individuals and families being their best and most productive selves throughout their life. This is why we need to have a good crack at implementing the National Action Plan.
Shout it from the rooftops
I really appreciate that you have read this and are aware of CHD, but unfortunately we are the minority. There is an urgent need to increase access to quality information, education and support to help ensure people with CHD and their families, regardless of where they live, have access to information, support and resources when and where they are needed. The National Action Plan advocates for a campaign including a 1800 service, support staff and awareness and education campaigns, that will provide critical social, psychological, information and economic support to people affected by CHD and their families.
To finish off, here is my quote surmising what the National Action Plan means:
To the CHD pioneers – the first generation of survivors, families and medical staff – the action plan is the embodiment of decades of love, loss and hope. It lights the path that many of us forged for future generations.
I’d love to hear what you think of Australias National Action Plan via HeartKidElle@gmail.com, facebook or Instagram
Also, let me know:
questions I might be able to help with (noting I’m not a medical professional),
what your experiences have been, and
any ideas for other blogs I could do.
*Disclaimer – I’m not an employee of Heart Kids Australia. They have just kindly agreed to post my blog for free.
About my heart condition: I was born with pulmonary atresia and intact septum with a leaky mitral valve. In English, this means that the connection from my heart to my lungs was “blocked” as one of the four normal heart valves never opened up – and one of the other heart valves was leaky. Also, one of the pumping chambers of the heart was much smaller than normal; the one under the missing valve. My surgeries have been to repair these problems.