Few aware of blood disorder

Photo by: Tracey Shelton
Yun Kimti, 4, waits for treatment for Thalassaemia at the National Paediatric Hospital in Phnom Penh last week.

The Phnom Penh Post
http://www.phnompenhpost.com/

Written by Tracey Shelton
Friday, 08 May 2009

To mark World Thalassaemia Day, a blood drive is to be launched to aid patients dependent on transfusions

AT the National Paediatric Hospital (NPH) Wednesday, doctors prepared 18-month-old Thalassaemia patient Meng Leng for her monthly blood transfusion.

As they held her down and tried - for the 13th time - to locate a vein in her tiny limbs, she screamed in anticipation of the pain that was sure to follow. Having been diagnosed two months earlier with severe Thalassaemia - an inherited condition that inhibits the body's ability to produce normal red blood cells - Meng Leng will need transfusions every month for the rest of her life.

A 2003 study completed at the Angkor Hospital for Children (AHC) in Siem Reap, the only completed study of Thalassaemia in Cambodia to date, revealed that 48 percent of subjects were carriers of the hereditary blood disorder.

Particularly prevalent among Mediterranean and Asian nationalities, Thalassaemia can cause severe anaemia, stunted physical growth, weakened bones, expanded bone marrow, inhibited sexual development and premature death, according to the Thalassaemia International Federation.

Children with parents who are both carriers face a 25 percent chance of developing severe Thalassaemia and a 50 percent chance of becoming carriers themselves.

Without treatment, the life expectancy for a patient with Thalassaemia ranges between two and 20 years, depending on the disease's severity, said medical laboratory scientist Robyn Devenish.

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I was shocked to find... that there was almost no awareness.
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Devenish began researching the disease in 2003 after noticing that a large percentage of anaemic patients assumed to be iron-deficient presented with Thalassaemia symptoms. The blood samples of 300 patients were sent to Thailand for testing.

"The results came back with 68 percent having at least one form of Thalassaemia," she said. "These were all anaemia patients, so the results were quite high. So I conducted a study across the board of 300 outpatients and found 48 percent were either carriers or had severe Thalassaemia."

Two studies currently under way are expected to yield similar results, suggesting that 140,000 Cambodians - most of them undiagnosed - suffer from Thalassaemia, while millions more could potentially pass the disorder on to their children.

No cure exists, but patients can live a full life with regular blood transfusions. In very severe cases, transfusions need to be given monthly, particular during childhood, so that the body can develop normally.

NPH hematologist Dr Chean Sophal, who treats around 100 Thalassaemia patients, said frequent transfusions can lead to complications.

"In Cambodia, there is no facility for filtering the red cells from the white, so whole blood must be given to a patient," he said. "It is these white cells that carry diseases such as hepatitis B and C and HIV."

Multiple transfusions also cause an iron overload in the body, which Chean Sophal said damages vital organs. The treatment, an iron-chelating agent, is currently only available in one location in Phnom Penh and costs patients with severe cases of the disease between US$12 and $15 per day.

"Nearly all [my patients] need this but cannot afford it," Chean Sophal said, adding that many are already experiencing organ complications.

Ieng Auntouch, chairman of the Cambodian Thalassaemia Association (CTA) and the father of two boys with severe Thalassaemia, said he can afford the treatment his children require but noted that many Cambodians cannot. He said the Ministry of Health does not allocate funds to promote awareness of the disease and training in how to treat it because it is not considered a priority disease.

"When my first son was diagnosed, I was shocked to find that in a country with such high prevalence there is almost no awareness," he said.

Lack of blood
A shortage of blood donors is one obstacle to treating patients.

Dr Hok Kim Cheng, deputy director of the National Blood Transfusion Centre, said the government provides blood free when available. He said only 25 percent of the centre's blood supply comes from voluntary donors, meaning relatives are often called on to donate.

"Currently, we cannot fill all requests," he said.

The blood must be fresh to be effective. To fill the need, Devenish said, a new campaign is to be launched today, on World Thalassaemia Day, to find donors willing to contribute blood once or twice a year for a particular patient. This way, blood could be donated and transfused within days when the child needs it, increasing its effectiveness, she said.

"If a donor was willing to spend just one hour twice a year donating blood," Devenish said, "they could allow one child who would otherwise be without hope to live a normal life and take a huge burden off the parents who are always under tremendous pressure to find a donor."

For information regarding the Thalassaemia blood donor program or CTA, contact Robyn Devenish at RLD@iinet.net.au