Thalassaemia has not as yet been recognised as a significant health problem by the government. But it should be considered seriously.

Unlike malnutrition, diarrhoea, acute respiratory infections, thalassaemia gets less emphasis, which may become a visibly major health problem in developing countries like ours.

The world population of carriers of beta-thalassaemia is estimated to be more than 100 million. Carriers of the abnormal haemoglobin, HbE is quite common in Bangladesh (53 million worldwide).

To date, limited data on the state of thalassaemia — both carrier and patient — are available in Bangladesh perspective. However, a study carried out by Dhaka Shishu Hospital Thalassaemia Center in 2004 indicates higher carrier rate than previously thought, as well as demonstrates considerable regional variation. According to a report by the World Health Organisation, 3 percent of the population of Bangladesh are carriers of beta-thalassaemia and 4 percent are carriers of HbE.

The Dhaka Shishu Hospital study looked at carrier state among the school children. The study revealed that 4.1 percent children are carrier of beta-thalassaemia and 6.3 percent children are carrier of HbE. In tribal school children the prevalence of the HbE trait was 41.1 percent, while that of beta-thalassaemia was 4.2 percent.

A burden for future generations
Using the Hardy-Weinberg equation on the data according to the latest census of Bangladesh, the expected births of beta-thalassaemia affected children are about 1,040 per year, with a further 6,443 HbE affected births. Thus more than 7,000 children are born with thalassaemia each year in Bangladesh.

Thalassaemia poses demanding challenge in terms of managing with limited resources. For example, thalassaemia patients need huge amount of donated blood. Data collected by Dhaka Shishu Hospital Thalassaemia Centre indicates that 60 percent of donated blood is used by thalassaemia patients although most patients can not afford adequate blood for transfusion. Furthermore, blood screening is increasingly expensive. As a result, it is not always properly carried out, contributing to further complications, including hepatitis B, hepatitis C and HIV — all of which contribute to tremendous psychological pressure on patients and their families.

Blood donation is not the ultimate solution of these ailing community. These patients also need drugs to remove iron from their body, which accumulate due to early breakdown of their own blood cells and excess load of iron excretion due to huge amount of transfused blood. This is again a very expensive procedure.

Looking ahead
Children are needlessly born with thalassaemia. We need to stop it now and only awareness can prevent it. To bring thalassaemia into focus, the significance of the disease burden and the sufferings of the patients must be recognised by the stakeholders and concerned bodies.

Prevention programmes should therefore focus on:

1. Promoting awareness of the disease

2. Population screening

3. Genetic counselling

4. Prenatal diagnosis

Promoting awareness
Raising awareness of thalassaemia involves seminars, workshops and close co-operation with the media. International Thalassaemia Day is, of course, a great opportunity to promote awareness of the appropriate management and prevention of thalassaemia.

Population screening
Identifying carriers of thalassaemia trait is an essential step in preventing further affected births. In many countries, screening and educational programmes are carried out in high schools, with considerable success.

Pre-marital screening for men and women is also essential. Married couples may also be screened, and should be sent for counselling if both of them test positive.

Counselling
Counselling plays an important role in preventing further affected births, although responses vary depending on the availability of prenatal diagnosis.

The experiences of counsellors at Dhaka Shishu Hospital Thalassaemia Centre suggest that many parents, particularly who have thalsaaemic child, do not wish to have another child until prenatal diagnosis is available.

Prenatal diagnosis
Prenatal diagnosis permits the diagnosis of a foetus with thalassaemia major. If the parents wish, they may decide to abort the foetus, usually in the 9th to 10th week of pregnancy.

Treating thalassemia in Bangladesh
The care of thalassemia patients in Bangladesh is very poor. Ninety percent of patients can not afford adequate treatment. The majority of the patients can not afford the iron chelating therapy.

Desferal, an injectable drug to remove iron is only available at irregular intervals and at fluctuating prices. Moreover, the sources of the drugs are unknown. Another oral iron chelating drug is available in Bangladesh Thalassemia Foundation and Bangladesh Thalassemia Society.

There is an urgent need to focus on the prevention, care and management of thalassemia patients. A thalassemia center should be established at all major hospitals of the country and essential drugs for the treatment of thalassemia should be subsidised and sold in designated outlets.

Widespread screening for the thalassemia trait combined with prenatal diagnosis must be introduced as a matter of urgency. Prevention of the births of further affected children in our country should now be a priority.

Professor Waqar Ahmed Khan is the President of Dhaka Shishu Hospital Thalassemia Center and Professor Bilquis Banu is the Professor of Pathology at Dhaka Shishu Hospital. [Professor Khan can be contacted through e-mail: waqarkind@gmail.com]