Welcome to My World

What an exceptional time to be alive. The world interconnected in a waking nightmare, with the worst (most likely) yet to come.

Coronavirus, COVID-19, novel coronavirus, SARS-CoV-2, The Rona (in Australia anyway), whatever you want to call it, has brought the world to its knees. It’s invisible, it’s not biased, it's deadly and we still have no way to beat it. The best strategy is not to contract it in the first place. Wash hands, quarantine, wash hands, stay away from people (at least 1.5m), wash hands, don’t touch your face, wash hands, cough and sneeze into your elbow, wash hands - oh I said that already. I need some hand cream pronto!

The other side to all this is the mental impact and toll this is having on us. There’s still stupid people out there living in their own bubble thinking they can’t be affected nor do they have any social responsibility to others – let’s leave those fuckwits out of this. No doubt the rest of us living in reality and particularly being in the vulnerable part of the population, have clued into our own sense of mortality and existentialism, if we hadn’t before. (How can you not be when coverage and infection are so widespread; or simply just by sensing the eerie vibe out there?)

I was talking to a friend a few months ago who unfortunately had a very bad car accident. She thankfully survived with minimal bodily damage. The accident should have been fatal but thank whatever out there, dumb fucking luck, it wasn’t. I could tell immediately that she was just falling over the edge into the long dark spiral of PTSD (she was approximately 4 weeks post-accident, which is the common amount of time before PTSD kicks in). She was frantic, contemplative, shell-shocked, in disbelief, depressed yet elated. I just listened to the outpouring of information. She couldn’t comprehend that the night before the accident could have been the last time she ever tucked her daughter into bed. What can you say to that, to any of it? Most times the best thing to do is shut the fuck up and listen. Unsubstantiated meaningless niceties, wisdom or positivity don’t cut it. My husband piped up eventually to break the desperate silence. He told her if anyone can understand how she feels, that I can. I know down to the deep dark core of her soul exactly how she feels. I’ve been her. I should have died at least twice now (fuck I’m losing count) from a congenital heart defect and cardiac arrest, but I didn’t. I’ve been on this PTSD train before, toot toot all aboard. Perhaps I should have reveled in the fact that out of my friends, I wasn’t alone anymore. Instead I was sad that she must go through this. The only thing that gives me some solace is that I’m available to her if and when she decides she needs me.

People experiencing this pandemic in quarantine from their own houses doesn’t compare to having a near fatal car accident, or cardiac arrest. But what these experiences have in common is that they force people to pause, to take stock, to reflect on themselves, their lives and the world around them. I feel confident that most people out there whether you've been directly affected by COVID-19 or not – yes I’m talking to you – have now experienced this moment: where a sudden existential dark emptiness casts over your mind and seeps into the middle of your chest to take residence in your soul. It’s that dreaded moment of dawning, or rather setting, where you realise within the being of your cells that one day, you will die; and at the same time just how precious and finite life really is. It’s one of the truest and breathtakingly bittersweet moments in one’s existence. The first time I felt it I must have been no more than nine years old. I was sitting on the grey carpeted floor in my bedroom contemplating all that is life. Naturally I came to that unsettling conclusion. And just like most people, I shook it off and kept going about my business.

We all know this truth intellectually, but to understand it in the depths of your soul is something else. It’s the unique experience reserved for people having gone through a near death experience, having been in war, tending to the sick and dying, being the sick and dying. And now it’s for the masses, televised and transmitted to your lounge room 24/7.

All the things you’re feeling now, during this pandemic, that’s me, feeling those things and having those morbid thoughts all the time.

Welcome to my world.

It’s a waking nightmare we’re all living, it’s difficult to comprehend our new reality. You distract yourself and go to sleep and try to escape it and maybe you’re lucky and you do. With my PTSD, there’s no escape. I’m living trapped in this endless cycle of mental and emotional exhaustion where at times the only choice is to become numb. I stay awake to try to distract myself from thoughts of death, what if scenarios and that creeping existential dark emptiness that keeps seeping through me trying to drown me every chance it gets. I’m too scared to go to sleep in case I don’t wake up again. Eventually when I do sleep, my anxieties and fears pervade my sleep to form nightmares. Sometimes I wake suddenly in the middle of the night gasping for air to violently, as quickly as possible, fill my lungs to know that I’m alive; and then I’m too scared to go to sleep again because I’m so grateful to be alive and terrified of dying all at the same time. When I wake up for good, I’m thoroughly spent, zombified, groggy, fatigued. Having more sleep to fend off the fatigue makes it worse because the nightmares are so vivid and visceral, they exhaust me further and for longer.

It takes time, work and kindness to oneself to unravel this cycle, to fight the PTSD and come out the other end as a highly functioning human being; to seek out the light as you move away from the darkness.

Another friend’s mother suddenly passed away about a month ago overseas. She had visited Australia some months prior where I distinctly remember my last interaction with her. I had gone to pick up my daughter from their place and she asked if I wanted a coffee. I wasn’t particularly in the mood for a drink but accepted regardless because I thought to myself I’m not sure when, if ever I’m going to see her again. Probably a morbid thought for the average person, but this is now my modus operandi, it has been most of my life, but more pronounced since my first cardiac arrest. Most interactions I have, I make a choice to treat it as being potentially the last one. Because one thing my congenital heart defect and cardiac arrests have taught me, is who the fuck knows what’s in store for any of us? As shocking as the news of her passing was (she was super healthy and active, nor too old), I was grateful we had that coffee together where I got to find out about her life and love of bike riding through nature, that essentially, I shut the fuck up, listened and got to have a genuine connection with her over that handful of minutes. All it takes is a pause, to be authentic and show someone that they matter, that they’re just as human as you are. A smile, a laugh, an inquiry or some banter with the checkout person in the supermarket to make a difference and to potentially make a person’s day, or yours one day down the track. To live without regret that you’ve said and done everything you’ve wanted to, that you’ll be remembered as that kind person, not the asshole. For me it’s simple: it’s to tell my family and friends I love them when we chat, or when the mood strikes; to hug them just that little bit longer and to take in their scent (creepy I know, but you take what you can when your immediate family lives abroad and interstate); the weekly phone call;  it’s the good morning in passing; the kiss, hug and eye contact hello when my husband gets home from work; and it’s the kiss and hug goodnight as I tuck my daughter into bed. If you're wondering, I smell her also, the most out of all people, my husband a close second. They’re the only things I can control and that matter the most to me because when the end comes, we can only hold on to the memories we made and the last interactions we had with people.

The world is so strange right now. It’s scary but it’s also beautiful and hopeful. Breathe in the air, it means you’re alive. Hug your loved ones or yourself, because you love them. Talk less and listen more, because we’re always learning. Take joy in the micro things because as big as the world is, we should all just stay home in our little universe for now. And remember, you’re not alone, we’re all in this together and we’re taking it one day at a time. As for today, I’m glad I’m still here. This is our world now.

Rhythmic composition in yellow green minor (1919), Roy de Maistre

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.

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.