Thalassemia Prevention: In Need of New Policy Directions

President Nasheed rightly rejected the Thalassemia Control Bill recently. His reasons for rejecting the Bill may be wrong, but this gives an opportunity for policy makers to make a comprehensive legislation to achieve the goal of preventing thalassemia, rather than controlling or sustaining thalassemia.

The Thalassemia Prevention Program was initiated in 1992 and has made marked contributions to the prevention of thalassemia in the Maldives. However, the impact of the prevention program has not achieved its desired effect: the prevention of thalassemia. New policy goals and directions need to be set and implemented to achieve that goal.

Background

Since the implementation of the program the average number of new children affected has dropped from 47 to 28 per year. However the last decade has not seen any significant decline in the number of cases despite the widespread awareness campaigns, screenings and genetic counseling, which forms the basis of the existing prevention program.

Most new affected children are being born to those who know of the risk, and to those who already have an affected child, either in the hopes of having a normal child, or to have a child who will act as a donor for the affected child.

The compliance with the treatment is poor in view of the lack of the necessary counseling in treatment, both for the children and the parents. Health seeking behavior is largely dependent on survival (the need for transfusions is well respected) and not on long term management ( poor compliance with other aspects of care).

Furthermore, the financial difficulties the key implementing agencies, the National Thalassemia Center and the Society for Health Education, are facing pose further challenges in both the preventive and curative aspects.

Objectives

The objective of a new Prevention Program should be:

• To stop the affected births (within 10 years) and improve treatment for existing affected children

Proposed Policy Decisions

In order to achieve the above objective, the existing policy decisions need to be strengthened and broadened:

1. Health education and awareness: The existing programs need to be directed and focused to identified groups.

• The NTC and SHE have data on where the disease is most prevalent and awareness programs should be directed to these areas.
• Health education program need to be started directed at affected children and parents to educate them the treatments and complications of the disease. Patient and parental involvement in the ongoing care to need to be highlighted.

2. Mandatory premarital screening:

• Screening options to be made available in the atoll/province level for high risk partners
• Screening for one partner only initially.

3. Genetic counseling and general counseling:

• Counseling program to be strengthened, to include not just the information of the risk, but the implications of having and caring for a child with thalassemia.
• A system to be set in place to identify the number of couples counseled and the number of couples who decide to separate or get married in view of the risks involved.
• To widen the counseling component to affected children and their parents to address the psychosocial implications of the disease.
• To integrate the genetic counseling with prenatal diagnosis.

4. Prenatal Diagnosis:

Prenatal diagnosis is key in reducing the number of affected children being born, as is evident from the experience of other countries. And at present several Maldivian couples already seek PND abroad. Following figures provided by SHE are the number of couples who have sought PND abroad:

In 2006 23 couples, in 2007 46 couples, in 2008 47 couples, in 2009 55 couples, and in 2010 (till July) 23 couples

• PND services thus should be made available in the Maldives, to address the above demand. Three doctors have already been trained in the diagnostic method and SHE has the capability to get the tests done. Further support should be provided to SHE to establish the system.
• PND should be coupled with option for abortion and counseling (for those who do not wish to terminate the pregnancy)

5. Abortion:

• As is it already legalized in the Maldives, abortion should not pose much problems most other countries faced in implementing this approach towards preventing thalassemia.
• A policy on abortion for health professionals should be drawn up and implemented.

6. Establishment of a Hematology Center:

• The NTC should be expanded into a hematology center to address all patients with blood disorders. This will provide a means of equal and just distribution of resources and health care services who are afflicted with disease conditions similar to thalassemia.
• The required funds for the establishment of such a center could be generated from international donors. To my knowledge, a trip Saudi Arabia by the Minster of Health and family and the Thalassemia Society was promising in this regard.

7. PND to be covered by health insurance scheme:

• Couples are already spending huge sums going abroad for prenatal diagnosis and abortion.
• The view that the affected children are born as a result of conscious decisions parents make and thus should not be covered by the insurance scheme contradicts the justification for the current provision of treatment under Madhana, or the existence of NTC.
• All efforts should thus be put towards preventing this preventable disease.

8. The Thalassemia Control Bill:

It is unfortunate that legislators have ignored these facts in drafting and debating the the Thalassemia Control Bill despite being shared. Now that it has been rejected by the President, this provides an opportunity to amend it to enforce an approach to prevent, rather than sustain thalassemia in the Maldives.

Implementing Agencies

The cooperation and coordination amongst several key agencies are necessary if the above policy goals are to be reached effectively. Each agency should be provided by clear roles and responsibilities. Key agencies include:

a) The Ministry of Health and Family: to develop these policy guidelines and to provide diagnostic and treatment protocols to NTC and all others involved in the care of thalassemic children.

b) National Thalassemia Center: As the main center providing services, the NTC should be given stronger powers in terms of procuring and arranging treatment services, making them less dependent on other agencies for directions.

c) Society for Health Education: Proper acknowledgment need to be made of SHE’s continuing contributions. A system for smoother coordination between NTC and SHE is needed.

d) Male’ Health Services Corporation: As NTC is under the Corporation, it should provide all the assistance to NTC in achieving the goals. Its current failure to provide for the care is a reflection of their inability to manage and lead. It is time for a overhaul of the whole system.

e) Maldives Thalassemia Association: the role and responsibility of the Association need to be broadened. A journal could be published by the Association to involve thalassemia children in providing information to their peers, and as an outlet for their creative expressions.

f) National Social Protection Agency: The prenatal diagnosis program need to be under the insurance scheme.

Conclusion

In concluding I would share the success of Cyprus in preventing thalassemia. Thalassemia was a serious health problem in Cyprus. They tackled this problem by aiming to stop the affected births, not by controlling it. This decision was made in 1976. In 1979 high risk families started to be screened. In 1980 premarital screening was mandatory. In 1984 prenatal diagnosis was started. Between 1991 and 2001there were only 5 thalassemia children born ( one in every 2 to 3 years) unlike the previous 18-20 cases per year. Within the last 5 years no thalassemic babies have been born (data from 3 years ago). Majority of the patients are now over 25 years old, living and working as the normal population. 38% are married and have children.

There is no doubt and reason why we should not be able to achieve the same in the Maldives. Question is, will anyone decide to do this?