An islandwide hospital based epidemiological survey of haemoglobinopathies and an assessment of standards of care in 23 centres

Abstract

INTRODUCTION & OBJECTIVES: There is no database for haemoglobinopathy patients in Sri Lanka resulting in imprecise knowledge about the distribution and standards of care among patients managed in different centres in the island. The prevention programme commenced in 2007 is not centrally monitored. METHODS: We carried out centre-wise visitation and data gathering of patients with haemoglobinopathies in 2015. 23 centres were visited by researchers who gathered information from patient records. RESULTS: Data was obtained of 1768 patients. Three centres had over 200 patients each and another three centres had between 50-100 patients. There were 8 centres with less than 10 patients each. Beta thalassaemia major (BTM) accounted for 1207 (68.26%) patients. There were 363 patients (20.53%) with Haemoglobin E-β thalassaemia. Sickle cell-β thalassaemia accounted for 51 patients (2.88%). The mean age of BTM patients was 13 years (range 2-44). Ethnic distribution of the haemoglobinopathies (82.5% Sinhalese, 12% Muslims and 5.2% in Tamils) was discrepant to the national ethnic data. Islandwide mean number of new births of all thalassaemics recorded showed a reduction from 66/year between 2004 - 2009 to 48/year between 2010 to 2015.Clinical record keeping was not systematic in most units thus complication rates were hard to obtain. Death data were available only in two units. CONCLUSION: This study identified significant inconsistencies in haemoglobinopathy care between centres. Existence of small centres needs to be recognized by the Ministry of Health. A reducing trend of new births over the last decade was observed.