Sotirios - living with thalassaemia

‘I was born in Melbourne, to Greek Australian parents. Part of my heritage included thalassaemia. Although it was not diagnosed at the time of my birth, my mother had heard of thalassaemia in Greece. Later, when I was appearing rather ill and anaemic as a four year old, it was diagnosed at the hospital.

‘An illness that made me feel very tired as a child, unable to walk long distances or to play with the energy of other children soon provided other ‘nightmares’ as part of the treatment. Around five years old, I began having regular blood transfusions at hospital. These varied from fortnightly to eight weekly. My haemoglobin level was around six before I began transfusions. After I was initiated into the transfusion regime, doctors aimed to maintain my haemoglobin level at or above nine prior to transfusion. Due to the lack of volume of blood in my body and the weak development of my veins, transfusions were particularly traumatic for me as a child. The painful search for a suitable vein to put the drip into and the stays in hospital became part of the experience of living with thalassaemia.

‘Around seven years old, I had my spleen removed as it was using too much of my valuable red cells (it was trying to do its job of removing the sick thalassaemic red cells and had become bloated with its desperate drive to destroy them). Without a spleen my immune system was further compromised and penicillin was prescribed. To this day, however, I have not suffered a major

infection other than the regular cold.‘Regular transfusions now provided another challenge. The extra iron left from all those blood transfusions could not be removed by my body and was beginning to store itself in my vital organs. An iron-chelating agent, Desferal (desferioxamine) was prescribed to be administered as a single intra-muscular injection daily. This became another painful daily reminder of thalassaemia. The Royal District Nurse would come to school on weekdays and I would be called to have the injection. Other children saw this and it added to their curiosity. I did not reveal to them what I had to live with.

‘During my early teen years the Desferal injection was replaced by a more effective administration regime. Using a device called a slow-infusion pump, I now administered the Desferal injection myself. The pump administered the injection over a ten-hour period (overnight). Of course, as a teenager, this meant a severely curtailed nightlife at home. I did not want to have this thing strapped to me while going out. I didn’t want people to see me as different or pity me, and I did not want to deal with their questions, no matter how well intentioned.

‘Diet was not a real issue for me apart from abstaining from high-iron foods like lentils, which I did not care too much for. As long as I kept up with regular blood transfusions and using my pump, I could lead a very normal life. But using the pump is painful physically and a hassle socially. If I was not vigilant in its use I was warned that other complications could arise such as heart and liver problems, diabetes and eventually death. Ironically, the transfusions would kill me.

‘It’s a life long-condition but one that is not impossible to deal with. Excellent support at hospital by people that I now have known for over 25 years means I have an extended family to support me. Plans for the future need to be realistic. In finding a life partner I have to be conscious of the possibility that thalassaemia could be passed on to my children and its severity depends on my partner’s carrier status.

‘Over 30 years ago, my parents were first confronted with thalassaemia in a language and environment that was very difficult. They suffered as much or more than I have. I was asked once whether I regret being born with this condition. I sincerely say that I would live through it all again because I have learned so much and it has made me a stronger-willed person.’