As chairman of the Cambodian Thalassaemia Association and father of two children with the inherited blood disorder, Ieng Auntouch works to raise awareness about the condition. Photographer and writer Charles Fox learns what can be done to help people with the genetic disorder, which can create an abnormal form of haemoglobin that reduces the ability of blood to carry oxygen around the body, causing severe weakness.
What is thalassaemia?
Thalassaemia is an inherited blood disorder caused by a genetic defect of the haemoglobin molecule, which does not allow the body to produce enough haemoglobin. There are three degrees of thalassaemia and the severe form, thalassaemia major, requires regular blood transfusions as the main therapy to survive. Depending on the patient, that can be every week.
Without regular blood transfusions the spleen becomes enlarged, as it has to work harder, and eventually has to be removed if the condition continues. If patients receive regular blood transfusions, iron will build up in their body, which needs to be chelated (removed). Currently there is no cure except for a bone marrow transplant.
Does the condition affect life expectancy?
Without blood transfusions the life expectancy of a patient with thalassaemia is approximately 10 years. With transfusions, but without other treatment such as iron chelation, it’s about 30 years. With regular blood transfusion and other therapy such as iron chelation, people with thalassaemia are expected to live as long as normal people.
Can you tell me a bit more about the work of the Cambodian Thalassaemia Association?
We were established in 2008 with the support of the Thalassaemia International Federation in Cyprus. The reason that I set up the society was that back in 2007, my first child was diagnosed with thalassaemia. My brother-in-law [a doctor] saw my son’s appearance and requested a complete blood count in Cambodia. It showed a low level of haemoglobin and my brother-in-law said he needed further tests. In Cambodia, to his knowledge, there was not a lab which could analyse the symptoms and he needed further tests to confirm what it was. He took us to Vietnam and they told us.
How did you find out more about the condition?
I went on the internet and searched for thalassaemia, and it came out with a lot of things. Basically it said that there is a high prevalence in Southeast Asia. I asked myself how come this kind of disorder is in such high prevalence in Cambodia and I don’t know about it? I did not believe it really. I thought there was something wrong with Wikipedia. I then asked myself how I could help those vulnerable people with no choice.
What are the association’s objectives?
The CTA vision is ‘a society free from thalassaemia’. Selected objectives are: to raise awareness about thalassaemia; to seek affordable, accessible treatment and adequate supply of safe blood for patients by lobbying and soliciting support from the Ministry of Health, drugs companies and international organisations; to work with the National Blood Transfusion Centre to ensure an adequate blood supply is available for transfusion dependent patients; and to raise funds to meet the objectives and activities of CTA.
Some patients need regular transfusions. Is there enough blood donated in Cambodia to do that?
No. Based on the National Blood Transfusion Centre, they need about 160,000 packs of blood a year and up to now they only have 60,000. People still fear pain. They are not sure when they give blood what will happen to their body. Probably, they think they will become weaker and pale.
How can people donate blood?
They can go to the blood bank. Anyone can come. You tell the people that you want to donate blood and they will take you through the process. The best time to come is Monday to Friday during working hours. It’s completely safe.
Are there any other ways of tackling the condition?
I want to have projects for [raising] awareness, training and population screening for free for couples intending to get married, young couples and those in need. Thalassaemia is preventable through screening and other measures, which proved to be cost effective in other countries but are not available in Cambodia.
What would screening involve?
Prevention involves the screening of married couples before pregnancy so they can then make an informed decision of the risk involved for their offspring if they are both carriers. Another method is general population screening, through which people can know whether they are carriers or not. With the generational increase over time, the blood requirements for treating thalassaemia patients will become unmanageable unless prevention strategies are introduced as soon as possible.
If you would like to donate blood, please visit www.facebook.com/DonateBloodCambodia to find out more about the National Blood Transfusion Centre.