The Roller Coaster of Congenital Heart Disease (or Pushing a Boulder Up a Hill)

I'm still alive. Barely. This has been a shitty year.

I'll get you caught up since we last spoke in February 2017. I ended up getting (what seemed on paper) an awesome job at a prestigious organisation. It wasn't in reality: neither awesome or prestigious; and I never got the chance to truly do the job I was hired for. A more accurate description is chaotic, a lost cause. I was there for approximately 16 months. Why did I stay there that long, after I had gauged the level of chaos within four weeks of having arrived? I don't give in that easily. I'm a dog with a bone. If you have a problem, I'll work my ass off to try and fix it because that's who I am. Maybe I shouldn't get that emotionally involved or have such high expectations of organisational structures or people? But that's not who I am, I don't put up with half-arsed and I care about the quality of work I produce and the impact I have on others. I want to contribute, make life better for others around me and leave my corner of the world that little bit of a better place than what it was before. 

It was weird, that place was so seductive, its people with such compelling stories and promises, with a decent job description, pay and benefits package and the promise of things getting better. I was lulled into a false sense of security that preyed on my loyalty, hard work ethic, commitment and natural inclination towards problem solving. It was like an abusive relationship: I kept on going back because I was promised things would be different next time, but they weren't. In fact they kept getting worse.

Something I haven't experienced before is the chronic stress element. It differs from standard stress, where you get pushed for a short period and then the valve is opened to release pressure and you get back to an even playing field again. Instead, chronic stress builds up over an extended period of time, constantly rising until something massive, like Chernobyl happens. There's warning signs, but you're so used to the stress and long hours (because that's your standard level of operation now) that you ignore or try to poorly manage the warning signs...and then BANG!

16 months of chronic stress and three heart incidents later, I accepted my reality: either that place was going to kill me, or I quit. I did the latter and I've been off work and recovering from my last incident for five and a half months already. I'm still angry though. I'm angry at myself for putting up with bullshit and not being kind to myself. I'm angry at the organisation and its people that didn't perform their duty of care. 

I sometimes forget that I have limits, that my heart and energy levels aren't as strong as someone who was born without heart issues. I forget because I can't sit idle waiting for my condition to claim me. I want to live my precious life and enjoy it just as much as anyone else, I want to challenge my mind and offer value to others without constantly thinking about illness or death.

But now I'm giving myself time to decompress and reflect. I'm learning my lessons and implementing strategies to better manage myself and my health. That's the beauty and often frustration of life: the power of retrospect and growth as an individual. Its a gradual, cumulative and an imperfect process.

I was incident free for over 3 years post having my defibrillator inserted. Then the job with the chronic stress happened and so did these incidents during extreme and prolonged periods of stress and exhaustion:

• Cardiac arrest, one appropriate and successful defibrillator shock to revert ventricular tachycardia  - six months into the job
  
• Atrial fibrillation, hospitalisation with transoesophageal echocardiogram (TOE) and cardioversion - nine months into the job
• Cardiac arrest, six consecutive and appropriate defibrillator shocks, with sixth successful in reverting ventricular tachycardia; cardiothoracic surgery for defibrillator replacement, medication change, rehab - 16 months into the job

There can never be definitive reasoning as to why these incidents happen, but doctors have a good idea. Stress and depression don't help, studies link these clearly to arrhythmias, particularly atrial fibrillation. Having congenital heart disease doesn't help - fucked heart from the get go, which puts patients into a higher risk category of having more complications or issues throughout their life. And finally scar tissue from open heart surgery can also cause arrhythmias. Or maybe it was a virus that weakened and affected my heart? Take a pick, I've had it all. And the severe stress and exhaustion made it so much worse.

People particularly in this modern day and age want a quick fix, a one pill solution, a reason they can grasp onto and fix. The best unsolicited advice I got from a family member recently was to fix my diet. I guess I shouldn't eat that donut or worry about all the other factors that come into play? Fuck it, I'm eating the donut.

One of the hardest things in this life is to live with uncertainty. People try to help, to simplify, to process.

There's limitations and doctors aren't gods. We're also all different constitutions that have our own way of ticking and working with genetic and environmental factors coming into play. The way modern health has advanced and information is disseminated makes us have a collective cultural mindset of being invincible and immortal. We're not and we don't have all the answers. Nature and life are both a beautiful and ugly miraculous mystery.  

I've been here before. I've been through this. I've got this. This wasn't my first cardiac arrest and it's probably not going to be my last.

I get moments of emotional weakness and complacency too. After my first out of hospital sudden cardiac arrest in 2014, I satiated my uncertainty by convincing myself that it was most likely a one off random event and the defibrillator was my insurance policy. I know better now. It's my survival mechanism. Until other heart complications; or other health issues or old age get me, just like any of us.

The last year and half has been at times a horrible nightmare; and at others a beautiful ethereal dream. 

In their book 'A Beginner's Guide to the End', Miller and Berger briefly talk about major chronic diseases and their patterns. They describe heart disease as having the pattern profile of a roller coaster. That's right, with the constant ups and downs, with good periods and bad ones. Where you're in hospital feeling rubbish and terrified for our life one day and the next you're recovered (for now), elated and discharged to go home to resume your normal life.

That's been my year. And here I am again, picking up the pieces and getting on with it. But it's alright, I've got this. I told you: I've been here before. 

It's familiar, but I wouldn't say that it gets easier. The darkness in my mind is real, with the insomnia and the sheer terror of going to sleep because I think I might die during the night. And the anxiety is taken up a notch or five now. Not only from the panic of dying, but I'm also emotionally paralysed from entering the work place again because I'm traumatised from my last job. 

There's a Greek myth that stuck with me recently: the story of the great Corinthian King Sisyphus. He was so cunning that he captured and escaped death. For this, he was eternally punished in Hades to push a boulder up a hill, which would then roll down upon reaching the hill's summit. Push, roll, repeat. For eternity. A fruitless and laborious task. A lesson in how we are unable to avoid the inevitable. Death and taxes come for us all.

So here I am, call me Sisyphus. Whether I'm riding a roller coaster or pushing a boulder up a hill, I'm stuck in this perpetual loop with no control, but I keep going. Welcome to life.

Making a Cardiac Comeback

How will you write your comeback story?

A question that has plagued me since I survived a sudden out of hospital cardiac arrest in March 2014. Mine, now anyway, is more an of existential question, of finding a way to make the shock and struggle mean something, a way to help other people, a way to have an impact on the world. But for a lot of people in the same boat, including people dealing with heart disease, it's more practical than that. It's about achieving a certain level and quality of life on a daily basis that someone without health issues most likely takes for granted.

Last Tuesday (11 August) I attended the ICD Support Group at Royal North Shore Hospital. I'd been to a couple before, but this one focused on the guest speaker Dr Samuel Sears from East Carolina University. Dr Sears is a Clinical Psychologist who specialises on the psychological care of people living with an ICD. You can access his bio here.

Apart from being a really warm, funny, approachable and knowledgeable guy, what became abundantly clear is his passion for the area of mental health for patients with ICDs. This is the area I struggled with the most post my cardiac arrest so I too am very passionate about it. It also seems to be an area lacking in Australia. As far as my knowledge extends, there doesn't seem to be someone like Dr Sears who specialises in a combination of cardiology and psychology. Specialised care has to be sought independently for either a psychologist or psychiatrist through a referral from your GP or Cardiologist. It was therefore refreshing to meet and listen to Dr Sears speak about this topic because he understands both sides of the coin: the mental and physical.

His focus is on providing his patients confidence in facing their fears and struggles in the face of threat. Some challenges that ICD patients have to tackle include:

1. Coping with their cardiac condition
2. Coping with an ICD
3. Resuming activity and quality of life

In the face of the above challenges, the aim of his talk was to: increase our understanding in what it means to be confident; as well as activate one strategy that each audience member believes will help them lead a more confident life.

Dr Sears presented findings from some recent medical studies.The gist of some of these was the discovery of distress, depression and anxiety experienced by patients with ICDs and their families; and the constant fear they live with which inhibits them from living their life. Furthermore this fear leads to a lack of engagement in activities. The most profound study presented was that of exercise and ICDs conducted over 2.2 years and on 98,000 patients. The discovery was that on average, these patients moved less than 2 hours per day, which means they spent at least 22 hours per day not moving. When the survival rate was looked at in terms of most active versus least active, the study concluded that the most active patients were most likely to be alive four years later. When talking about activity we're not talking anything complicated or extreme either - just some form of basic movement, as opposed to being sedentary. Conclusion: some, any movement is better than none.

In fact, data now suggests that doctors have been too safe with the prescription of types of activities that heart/ICD patients can partake in. What they do know is that muscles, joints, the human body and mind have been designed to function better as a whole with movement. Of course it is important to note that competitive athleticism remains very much a grey area and clinical judgement should be made by a cardiologist.

Dr Sears eloquently went on to discuss confidence in cardiac patients, how to achieve quality of life, and perceived safety and confidence in the context of threat. Some standout points:

• Quality of life (QOL) is personal and point in time dependant
• Your definition of QOL is under your control
• Confident living is the fuel for the achievement of QOL
• Arrhythmia is random, don't know for certain what causes it
• Doesn't matter what causes arrhythmia, but you have strategies to keep you safe (medicines, ICDs and monitoring)
• Can't control arrhythmia but don't allow it to control you!
• When it comes to confident thinking and living, work out what activities are your goal, and work towards these
• These activities should make you feel alive and well. Think about what activities you liked to do in the past, before getting an ICD or having an ICD shock?
• Be proactive, test the limits safely
• ICD gives safety to make a cardiac comeback, essentially helping you deal with a condition that is spontaneous, unpredictable and potentially life threatening
• QOL is an achievement, not an entitlement
• A shift from victim to survivor reduces stress
• Mental health is what we believe about the future; it's earned and deliberate
• Have an ICD shock plan
• Foster confident relationships by creating intimacy and finding ways to feel closer to the people who love you

People with heart disease are faced with their own mortality in a very real and immediate manner. Heart disease which some may think makes you weaker, actually makes you stronger. Regardless of the adversity in your life it's up to you to decide whether you face it as a victim or a survivor.

That's a lesson I learned early on in life and I guess what drives and motivates me to take action rather than sit back and be a passenger in my life.

So what was my one strategy I took out of this presentation that will help me lead a confident life? It was the reassurance that I'm doing everything I can possibly be doing to prolong my life: medication, ICD, regular monitoring by my cardiologist and GP, regular exercise, balanced eating, minimising stress, and most importantly taking time out to have fun and create and enjoy wonderful moments with the people I love.

How will I write my cardiac comeback? I think the above is a damn good start. Only time will tell what happens and evolves beyond that.
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Some interesting facts and statistics:

• The first human ICD implant occurred in 1980.
• There's 4-6% chance of and ICD shock in an average year.
• Activity avoidance in kids is 84.5% and 39-55% in adults.
• A weaker heart (e.g. 35% ejection fraction) has a tendency to act irregular.
• Risk of driving in ICD patients is the same as other people - it's equally as dangerous.

You can view a similar presentation that Dr Sears has done before, The Medical Trapeze: Living Confidently With a Safety Net.

Inaugural ACHD Education Day

Saturday 21 February 2015 marked the Inaugural ACHD Education Day. The event was run by HeartKids and held at the Mercure Hotel in Sydney.

Considering it was the first ever event of it's kind, the day included a good mix of content and professionals as speakers, including:

1. Professor David S Celermajer AO FAA (Cardiologist at RPAH; Head of Cardiology at University of Sydney; and Clinical Director of the Heart Research Institute)
2. Associate Professor Edwin Kirk (Clinical Geneticist)
3. Dr David Tanous (Cardiologist at Westmead Hospital)
4. Dr Rachael Cordina (Staff Specialist in Cardiology at RPAH)
5. Adjunct Associate Professor Amanda Gordon (Clinical and Health Psychologist)

Image courtesy Uni of Sydney

Professor David Celermajer was clearly the draw card for the day and (not surprisingly) it became quickly evident how knowledgeable and well respected he is in his field, as well as what a huge advocate he is of adult congenital heart disease. His expertise in the field of ACHD has not been an easy or short feat - it has taken him 16 years to fully qualify; illustrating his dedication and passion for his life's work.

He began by going through some startling statistics and facts, which I've summarised below.

During the 1970's and 1980's, it was mainly just children with congenital heart disease. There are now more adults with congenital heart disease than children. In the 1970's only simpler heart problems could be fixed whereas now, almost anything can be treated. This means a much higher survival rate and therefore adult population with CHD.

NSW has approximately 10,000 cases of adults with CHD.

• Only 2,500-3,000 of these cases are seeking or have sought care in an expert ACHD centre.
• There are 500-600 new cases of CHD which join the adults annually, increasing in number and complexity.

In Australia there are 55 cardiologists specialising in children with congenital heart disease vs. only seven cardiologists specialising in adults with congenital heart disease. I found this fact astounding, which further confirmed why I had such trouble finding a specialist cardiologist for my personal care. Not only are there not many ACHD specialists in Australia, in my view (and this may now be changing), there also seems to be little awareness by generalist cardiologists of the ACHD specialists that do exist.

The ACHD population is expected to grow 5% p.a. Translating to one in 150 people having some form of congenital hear disease, or 6-7 per 1,000 live births (including congenital problems which appear later in life).

Professor Celermajer also explained different heart conditions, which was really interesting. These were by no means definitive, but clearly outlined how every CHD case is complicated and so very unique.

Apart from caring for his patients, university and Heart Research Institute commitments, Professor Celermajer has also dedicated (it seems a vast amount of) time to establishing comprehensive adult congenital heart disease centres, which I've now listed on my blog. Furthermore he's been working intimately with the Cardiac Society of Australia and New Zealand to develop a paper outlining recommendations for the standards of care for adults with CHD. This is a massive piece of work and it's required due to the massive struggle that exists with transition of care from children to adults, which is something I can relate to. The main points outlined in this paper are:

1. ACHD is a relatively new and rapidly growing area of need.
2. The recommended (adequate) standards of care.
3.  Whole life planning (holistic care).
4. Paediatric patients transitioning to specialist care centres for adults when they come of age.

He had opened his speech by stating what a burden it is living with ACHD. With those words I felt a massive relief to know that someone else really understands, through his experience and exposure to ACHD, what I and everyone else there present in that room, go through on a daily basis. He came full circle and closed his talk by outlining some of the psychosocial aspects affecting patients with ACHD, which are highly important as they apply to our day to day living:

• exercise
• employment
• insurance
• intellectual and social development
• contraception and family planning.

As CHD patients there isn't much we can do about our physical limitations, but we do have to make daily decisions about other factors in our life and have to always take into consideration how these affect our CHD. All the technical and academic aspects aside, what really stood out for me was the empathy Professor Celermajer feels for patients with ACHD. It is comforting to know there are people out there that understand and that we have the established ACHD specialist centres where adults living with CHD can get tailored care and assistance for all areas in their life.

Associate Professor Edwin Kirk spoke about his area of specialty: genetics. He's been interested in genetics associated with congenital heart disease for over 15 years. Most of the information went over my head and there were a lot of charts used as visual aids, so difficult to transcribe. Basically what I took out from that presentation is that genetics is such a wide area of study and so complicated, that, to simplify, there's not much that they actually do know. CHD is due to multifactorial (meaning many factors) inheritance. They think it's approximately 70% genetics and 30% environmental. 1% of babies born have a chance of having some form of CHD.

What I understood from this information is that unless there is a clear pattern of heart disease in your family tree, then the reason you have it is most likely random, pure and simple luck.

Image courtesy zoominfo

Dr David Tanous specialises in CHD and pregnancy, having completed a fellowship in congenital heart disease and heart disease in pregnancy at the University of Toronto. He too had some interesting facts to share.

• The female body works extremely hard during pregnancy, there's a 50% increase in fluid volume, 30-50% increase in cardiac output and 30% increase in heart rate. It's between 28-30 weeks where the cardiac output peaks. These factors can have a massive impact on someone healthy, let alone a woman with heart disease.
• The risk of transmission of heart disease to offspring is 3-5%.
• Caesarian-section is not proven to be a better delivery method when it concerns the load on the heart. 
• There is added complexity when anesthetics are thrown in the mix. 
• Certain medications can impact a fetus adversely.

Therefore a good assessment before pregnancy is far more important, with preconception planning preferable. There needs to be a team approach to ensure that all the factors mentioned above (and more) are taken into account, including but not limited to:

1. Cardiologist
2. Obstetrician
3. Anesthetist
4. GP

Some useful resources Dr Tanous shared are listed below.

Mothersafe - NSW Government service assisting patients and their healthcare providers with concerns around exposures during pregnancy and breastfeeding

Heart Disease and Pregnancy - resource for patients and healthcare professionals outlining the risks and management strategies associated with pregnancy for women with heart disease.

Image courtesy HRI

Dr Rachael Cordina is the newest addition to the RPAH team and her passion lies in ACHD and exercise.

Patients with ACHD are automatically at an increased risk of being overweight and inactive. This can be due to many reasons such as being wrapped in cotton wool from early in life, or physical limitations or symptoms which make it difficult to exercise.

The good news is, habitual exercise has been shown to have a greater impact on fitness and exercise capacity than the heart muscle function itself. A tiny bit of exercise is better than no exercise at all, for example a slow walk for 10 minutes per day.

• The recommendation is that people with CHD should complete an exercise test to determine their peak heart rate, as there is no clear cut formula.
• Some sporting activities can be fatal to people with certain heart conditions. Always speak to your cardiologist to obtain approval for undertaking any exercise program or physical activity. 
• Even people with complex heart issues can exercise, but this needs to be done in a supervised environment under specialist care (exercise physiologist) with a tailored program.
• People with defibrillators and pacemakers should avoid contact sports and long distance swimming.
• Everybody with CHD can benefit from regular exercise.

Exercise and CHD is an area I too am very passionate about. Prior to my pregnancy and post my cardiac arrest, regular exercise helped me not only physically, but also mentally. I feel like it's one of the few positive things I can do to help my condition. In most cases it won't cure your condition, but it is a great preventative measure to help keep other problems or complications at bay. After my cardiac arrest I had a major realisation: just because I have CHD, does not mean I am exempt from other health issues. I don't see the point in exposing myself to greater risk of anxiety, cholesterol, diabetes, obesity, heart attack, or osteoporosis. So the only thing I can do is minimise these risks by exercising.

Image courtesy Armchair Psychology

Adjunct Associate Professor Amanda Gordon is a clinical and health psychologist in private practice, helping individuals with chronic illness and their families manage their lives and relationships. She began by emphasising the mind and body connection, that both are equally as important. Congenital heart disease although limiting, is not visible. So this makes it harder for others to empathise or understand. Surely, if you look fine, you must be fine?

The main areas covered for someone suffering from a chronic illness were:

• nurturing your relationships
• coping strategies
• self care
• building a support network
• managing loss and grief

Some helpful advice summarised below:

• Be informed about your disease, the more you know, the better you'll do.
• Accept limitations, but also the gifts.
• Positive psychology: focus on what's going well, rather than what is going wrong.

The three blessings is a technique Professor Gordon shared to help remain positive. It consists of keeping a gratitude journal, where you write three good things that happen every day. This can be a reference for your yourself when you are having a bad day or period, or something to share with your loved ones. Three x 365 days in a year =  1,095 great things to look back on every year.  

I'm a strong believer in our emotional well being having an impact on our health and manifesting physically. I think the three blessings can be a good technique particularly if negative thoughts tend to usually overpower the good ones, it helps refocus. Why spend our precious time being miserable...?

I am also very passionate about mental healthcare in general, but particularly when suffering from a chronic illness or having experienced a traumatic event. Mental well being is a minefield.  The information presented above is overly simplified and high level and perhaps, not as practical or tangible as some people may require it to be. Every person's condition and experiences are completely unique, so like physical exercise for an adult with CHD, I believe that tailored care is required. And seeing a psychologist, psychiatrist or counsellor one on one will be able to provide this specialised care, consisting of advice and techniques that should work for the individual.

Finally Jann Kingston, the CEO of Heartkids gave a short talk. She explained this day was organised because there was a demand for it because once heart kids grow up, there is nowhere to refer them to. Heartkids are working on a registry for CHD, this will help collate data to be able to develop further services and help people with CHD stay connected to these services.

 

The key points from the day for me were:

1. ACHD is a growing area increasing in size and complexity. Holistic care is required from cradle to the grave, encompassing all areas of life.
2. If you're suffering from CHD, ensure to be seen at least once by an established ACHD centre listed here.
3. Prevention is better than cure in context of pregnancy and ACHD. Pre-plan and enlist a team approach to managing your pregnancy.
4. Some exercise is better than no exercise.
5. Always speak to your cardiologist to obtain approval for undertaking any exercise program or physical activity. If possible, speak to an exercise physiologist for a tailored program.
6. Best to be well informed about your personal condition and state of health.
7. Nurture and look after yourself.

Some concerns, observations or comments that came up from the audience on the day were:

• There is no comprehensive guide of all heart conditions.
• There isn't a comprehensive list of cardiac programs or exercise physiologist gyms where adults with CHD can go to.
• Physicians need resources as much as patients do for example regional or non specialist doctors.
• Lack of quality information to help increase medical support and funding.
• No official organisation for adults with CHD.
• People are uninformed about the services available to them.
• Not sure of the best channels of communication.
• Development of some sort of mentoring program for children and adults with CHD.
• The clear need for a support group. The most frequent comment made by attendees was how good it was to connect with other adults suffering from CHD.

The intention is that this will become an annual event, the success and likelihood of which was pegged on this first off event in Sydney. Many of us suggested that we looked at moving it around Australia, so the location is not static and the event can be accessible to more adults with CHD.

All in all it was a wonderfully informative and touching day. Apart from learning many interesting facts and how to access some very useful resources, it was the connections with people that affected me the most. Some people shared some very personal and painful stories and for their (and the speakers') openness, experiences, love and kindness, I thank them.