Since birth over 8,600 needle sticks, 700 blood transfusions and 2,200 blood packs to stay alive, and still counting!
SURVIVING BETA THALASSAEMIA MAJOR
If you ask people about their earliest memories, most will recall holidays and family parties. But for Arthur, many of his earliest memories include going with his parents to regular hospital visits and staying overnight for blood transfusions. These treatments have been a frequent part of his life since birth because he has beta-thalassemia major: a rare genetic disorder that reduces his body’s ability to produce enough healthy red blood cells.
When reaching adolescence, most teenagers want more freedom, independence and control in their lives. For Arthur, it was the opposite, as he discovered that his lifespan would only last up to adulthood. After becoming an adult, Arthur was waiting for his death. It was at the eleventh hour, at the age of twenty-one, when Arthur was introduced to a miracle treatment, but only after the damage of iron overload from all the blood transfusion was done to his body.
Grateful to be given a chance to survive for a few more years, Arthur decided to do something with his life; to get married, buy a house and also to have children, knowing he had no prospect of any future for himself.
At the age of sixty, Arthur and his wife Helen celebrated their thirty-five-year marriage anniversary.
Iron Boy is a heart-breaking, gutsy and honest story about Arthur’s fight back from the darkness, and of his will to survive and prosper against all odds. To date, he has had 8600 injections, 700 blood transfusions and 2200 blood packs. In September 2016, Arthur was awarded an Order of Australia Medal (OAM) on the Queens’ Honour list as a result of over two decades as the CEO for a disability organisation, supporting disadvantaged people.
Beta Thalassaemia Major
Beta Thalassaemia Major is passed from parent to child in genes. Genes carry information about human characteristics such as eye colour, hair colour and haemoglobin. Haemoglobin disorders vary in their symptoms, ranging from mild to life-threatening. Even within each condition, different people are affected to different degrees.
Haemoglobin disorders include beta thalassaemia major, sickle cell disease, alpha thalassaemia minor and haemoglobin E.
All haemoglobin disorders cause some level of anaemia, which can make you feel tired and drained, and puts extra strain on your heart as it tries to pump oxygen around your body. Thalassaemia is inherited. It is not contagious or transmitted by germs.
People born with beta thalassaemia major are unable to make healthy red blood cells like everyone else and need frequent blood transfusions to survive. In the past, people with beta thalassaemia major did not live past early adulthood. The treatment caused iron to build up in the heart and other organs from all the blood transfusions and that eventually results in heart or organ failure.
This and other related information can be found at the following thalassaemia society links:
- Thalassaemia International Federation https://thalassaemia.org.cy/
- Thalassaemia and Sickle Cell Australia https://www.tasca.org.au/
- Thalassaemia & Sickle Cell Society of NSW https://thalnsw.org.au/
Or contact your closest thalassaemia society near you.
Where was my wife? I found myself sitting alone in front of the obstetrician, who my wife, Helen, and I had met for the first time only minutes earlier.
But now Helen was gone. After another quick look around the doctor’s plush office, I excused myself and went out to search for her. Surely she wanted to hear what the doctor had to say?
After dismissing her unusual behaviour as a possible toilet dash, I was stunned to find her outside in the carpark next to our car. She was in hysterics, crying and shaking uncontrollably.
‘What’s up sweetheart, are you alright? Is the baby okay?’
‘I’m not going back in there, I’m not!’ Helen screamed through her tears.
‘No, of course not, why? What happened honey?’ I was confused and now very concerned.
‘You know why, you heard him! I’m not terminating our baby!’ Helen burst into tears again.
‘What?’ I said in disbelief. This was news to me. Apparently, I had been more distracted than I’d realised as I had missed the doctor’s words.
It was October 1986, I was twenty-five years old, she was twenty-three and this was our first pregnancy. The sense of achievement of being a husband was still new, and soon I was to be a dad. The very thought was overwhelming, wonderful, and scary. It sent me flying high with exhilaration, but then I would remember my medical condition and crash back to earth. The ensuing fear was crushing.
What if our child inherited my condition? Would I be responsible for denying both my wife and our child the chance of a normal life? Panic then manifested inside me, sending my thoughts spiralling out of control. I also now envisioned our baby being born with huge deformities that wasn’t even related to my condition.
These misgivings had started the day we’d found out Helen was pregnant. After that, on a daily basis, worst-case scenarios constantly bombarded my mind, exhausting me.
As I’d sat next to her in front of the obstetrician, I’d been assailed yet again by those tumultuous thoughts. Perhaps, if I had reined in my troubled introspection, I would have noticed her leave the room. But overwhelmed by my fears, I was oblivious.
‘Look, I can’t offer you anything else. Hello, your wife needs you!’
‘Sorry, did you say something?’
‘I SAID, your wife needs you!’ The doctor practically shouting while pointing pretentiously over my shoulder toward the door behind me.
It was twelve when I was first told I wouldn’t live past my twenty-fifth birthday. Born with a chronic blood disorder called beta thalassaemia major (Thal), the doctors said my body was unable to produce healthy red blood cells and therefore, I required regular blood transfusions to survive. Since then, it had only been the generosity of wonderful blood donors that had kept me, and others like me, alive. We are known as Thals.
Unfortunately, after so many blood transfusions over the years, all Thals suffer from iron overload and finally succumb to heart and other major organ failures. The doctors said this could happen from childhood through to late teens or, for the lucky ones, early twenties.
Back then, comic books were still the rage. Batman and Superman were always my favourites but every once in a while, I came across an Iron Man comic and was captivated by the hero’s suit of armour. The stories were okay, but I was fascinated with what Iron Man could do.
So when I was first told I was going to die from iron overload before my mid-twenties, I wasn’t concerned because I truly believed I was Iron Boy. At the time, I felt my older Thal friends didn’t know what they were talking about when they said I was going to die because I thought iron made me stronger. I was convinced that I was impenetrable, just like Iron Man. Iron Man comics saved my life at a time when reality would have destroyed my spirit at the most delicate age in my life.
Now I was twenty-five and soon to be a father. I couldn’t believe that I was there with Helen visiting an obstetrician, knowing I had reached my shelf life. How could I be so irresponsible, so thoughtless, so stupid?
Then I remembered I was in love with the most beautiful woman in the world.
I may not have been normal, but who is? I knew Helen loved me and that’s all I needed to be strong for her and the baby. Then and there, in the doctor’s office, I decided to take on whatever challenges this doctor’s visit brought, good or bad, because I knew I could achieve anything in life with her love. It was this epiphany that snapped me out of my introspection and made me notice Helen was missing.
But my nightmares were coming true. With my heart racing, and feeling light-headed, I helped Helen into our car while trying to calm her down with a gentle voice. At the same time, my heart was breaking.
‘What do you mean the doctor had recommended termination of our baby because of my condition?’
‘He said that this baby, being our first, was more likely to have inherited your condition and suggested we should terminate and then try again for a better chance at a healthy baby.’
‘Hey, sweetheart, stop crying, he’s just an old fart who is stuck in the past. Let’s get a second opinion, from another obstetrician. Don’t worry about what he said.’ But nothing I could say could pacify either of us.
He made this vile and monstrous diagnosis at our very first consultation without taking a blood test from either of us, without consulting with any specialists and without even examining Helen. A doctor’s profession is called ‘practising medicine’ but that advice was unprofessional and unfounded. This advice, from a senior doctor with his experience, a specialist in his field, is something no one would have expected to hear.
Already struggling with a heavy heart regarding my mortality, my worst nightmares were coming true. How was I going to remain strong for Helen?
New Australians, 1956
The Menzies government era was full of hope and prosperity for Australians. Back then, my mum and dad were both referred to as new Australians. Dad arrived in Australia after first starting off from Patras, Greece by ship just before his twenty-first birthday in 1956. His ship was bound for Sydney but ran aground in Melbourne, so he completed his journey by train. He was sponsored to immigrate to Australia by his first cousin, Bill Bourakis who was originally from Corinth, Greece.
My Uncle and Aunty Bourakis already had a beautiful home in Summer Hill, an inner suburb of Sydney. At the time, they were expecting their first child (my cousin John), and my Uncle Bill was eager to show Dad the ropes, to help him find a job and get established in Sydney. Uncle Bill barely knew a dozen English words, which was a great deal more than my dad, who was impressed with Bill’s language skills.
My dad, in desperate need of a job, found an advertisement in the newspaper for a machine operator position and told Uncle Bill he could operate any machine, given the chance. This put a smile on Uncle Bill’s face, and they set off for the address specified in the ad, an office on the sixth floor of a building in George Street, right in the middle of the city.
On arrival, they found they were the only males answering the advertisement once noticing only women waiting. Uncle Bill assured Dad that this was a good sign. Then he waved the newspaper advertisement under the nose of the receptionist to attract her attention.
‘Please job… she for job… she job!’ Uncle Bill explained confidently while pointing at Dad at the same time.
Impressed with Uncle Bill’s multilingual abilities, Dad stood back, quietly hoping.
The receptionist looked at them with disbelief. ‘You know this is for a typewriter machine operator? Is your friend a typewriter machine operator?’
‘Yes, oh, yes!’ Uncle Bill answered, nodding his head.
The receptionist escorted them to a side office where, to their bewilderment, they discovered a state-of-the-art typewriter sitting on a bare desk in the middle of an empty room. ‘Here you go. Tell your friend to show me his skills,’ shouted the receptionist rudely.
Uncle Bill looked at Dad and encouraged him to have a go. Without hesitation, he planted himself in the chair and proceeded to type his fingers off. The typewriter sounded like a music box with a ring of a bell every time he started a new line. Uncle Bill and the receptionist watched in wonder as Dad typed away. After completing almost half a page of typing, he sat back, looking confident, and rested his hands behind his head.
With a smile from ear to ear, the receptionist reached over and removed the paper from the typewriter. She stared at the page in bewilderment and then turned the sheet of paper upside down. ‘What is this?’ she yelled, her smile transforming into a scowl as she shoved the paper in front of Uncle Bill’s face.
Uncle Bill looked at it and confidently replied, ‘This is Greek!’
‘Greek? I want someone who can type Australian, not Greek.’ She threw them both out of the office, locking the door behind them.
Jobless and bemused, they exited the building. Once outside, Uncle Bill turned to Dad. ‘When did you learn how to type?’
‘I didn’t. I saw it in the movies, and I always wanted to try it!’ Dad replied wickedly.
For the next two years, until my mum arrived in 1958, life for my dad revolved around looking for jobs during the week and playing soccer every afternoon and on weekends. At the height of his playing career, Dad played for Sydney Olympic Football Club. He lived and breathed soccer, dedicating every available minute he had to the sport. Soccer kept him fit and sharpened his mind at a time when he was struggling to adjust to a new life, far away from his family, in a country at the other end of the world.
Yes, soccer wasn’t just a pastime for Dad, it was a way of life, seven days a week, for him and for his newfound mates, who were mostly also new Australians.
First Born, 1959
When Mum finally arrived in Australia from Lafka, Greece, also by ship, she was only twenty-one and felt vulnerable after a voyage of almost three months. Away from her home and family for the first time, she felt alone in this strange new country. She even lost contact with new friends she had made along the way almost immediately after they got off the ship in Sydney.
Mum’s name was Panawoolla, a name that didn’t go down too well in Australia in those early days. Co-workers at the biscuit factory in Camperdown where she got her first job on the conveyor belt would refer to her as ‘bloody Nora’ because they couldn’t remember her name. The name ‘Nora’ stuck.
When Mum and Dad got married, Mum legally changed her name to Nora. Within a year of her arrival in Australia, Mum and Dad met, fell in love, got married and gave birth to their first son. Nicholas was born on Mum’s twenty-second birthday, 1959. He was fortunate to miss out on inheriting Thal, but my parents were soon to discover the truth about our family’s so-called ‘curse’.
When Nick was born, Dad was working as a labourer and Mum as a cleaner anywhere she could find work, places like large government office buildings in the city and later, an assortment of bus and train depots around the suburbs.
To them, Australia was the lucky country, and people overall were kind and supportive. A large majority of immigrants like my parents settled in the larger cities of Australia, particularly Sydney and Melbourne, contributing significantly to economic and numerical growth. The result of this process has been the development of the nation’s multicultural society and my parents were very much a part of this.
In early 1960, they moved to Erskineville, which was home to a large number of immigrants from all over the world, especially from Greece. This made Mum and Dad feel almost at home and was a comfort to them in this trying period of their lives. They were able to comfort one another in their homesickness.
Still adjusting to life in a strange land on the other side of the world, these newlyweds with a newborn baby and a second child on the way were soon given some earth-shattering news.
‘BLOOD BROTHERS’ PODCAST
Peter Karamihalis – Born 1959
Arthur Bozikas OAM – Born 1961
Professor Joy Ho AM
MBBS(Syd) DPhil(Oxon) FRACP FRCPA FFSc(RCPA)
Clinical Professor, University of Sydney
Head of Department, Haematology and Director of Research
Institute of Haematology
Royal Prince Alfred Hospital
Peter Karamihalis and Arthur Bozikas are Beta Thalassemia Major patients at the Royal Prince Alfred Hospital in Sydney.
At the time of recording this broadcast, they were both in their late fifties. They met as kids getting monthly blood transfusions – they’ve been through a lot together, and have a pretty special bond to show for it.
Broadcast: Mon 9 Jan 2017
IMAGES FROM IRON BOY