Safe Marriage for Prevention of Thalassaemia: Sri Lankan Experience, Appraisal of the Concepts and Challenges of Implementation

Mudiyanse Rasnayaka; Mudiyanselage .

Safe Marriage for Prevention of Thalassaemia: Sri Lankan Experience, Appraisal of the Concepts and Challenges of Implementation

Mudiyanse Rasnayaka, Mudiyanselage .

Abstract

Thalassemia prevailing mostly in the Mediterranean region has gradually distributed all over the world due to migration and cross-cultural marriages. It is caused by the deficiencies of production of alpha or beta globin chain required for the stability of haemoglobin molecule. Lack or inadequacy of globin chains leads to a chronic haemolytic anaemia with a wide spectrum of phenotype ranging from asymptomatic normal life to total transfusion dependency for survival. Bone marrow or stem cell transplant and genetic therapy are not accessible to majority. Therefore, management is entirely based on regular blood transfusion and chelation of iron that accumulate due to blood transfusions. Provision of required amount of blood has become a concern in some countries while the cost escalation due to accumulation of cases due to the palliative nature of management has strained health budget, making thalassemia prevention inevitable. Most popular and successful method of thalassemia prevention by antenatal diagnosis and abortion of affected babies is not practiced widely in many parts of the world due to legal sanctions on abortions, lack of facilities or due to self imposed restrictions based on socio-cultural or religious belief. The concept of safe marriage has been introduced in Sri Lanka. A marriage is considered safe when at least one of the partners of the couple is NOT a thalassemia carrier. Public education, voluntary screening of school leavers and providing them with a pink or a green card indicating carrier or non-carriers status has been initiated. Screening protocol and a visual aid depicting the popular concept of matching horoscope has been deployed.
The thalassemia prevention programme in Sri Lanka has two main challenges; inadequacy of screening coverage due to voluntary nature of screening and doubts about the merit of the MCV and MCH as a screening test due to the possibility of missing some E beta thalassemia carriers. The screening coverage could be addressed by incorporating thalassemia screening in to the routine practice and monitoring at risk marriages at the time of registration to offer them medical counselling. Impact of missing some of the E beta thalassemia is probably over emphasised. The rare chances of inadvertent birth of a baby with E-beta thalassemia can happen only once in several years and should not thwart screening and counselling to promote safe marriages in a country.

Keywords

thalassemia prevention, safe marriages, sensitivity of screening test, criteria for population screening

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