An Assessment of Frequent Haemolytic Crisis in G6PD Deficiency Subjects Linked with Chronic Disease States in Al Ahsa Community, Saudi Arabia

Abstract

The main treatment strategy for glucose-6-phosphate dehydrogenase (G6PD) deficiency is averting the use of oxidative precipitants like drugs or foods that trigger haemolysis. However, subjects could experience acute crisis in a comorbidity state of diabetes, hypertension or infection. In addition, the condition could in turn predispose carriers to these chronic diseases. Aim of the study was to evaluate the frequency of haemolytic crisis in carriers with attendant chronic diseases, and also assess awareness and knowledge of aggravated reactions from these conditions. 282 subjects consented and participated in the study. A cross-sectional semi-structured interview questionnaire conducted from May to August 2013 was adopted. Questions focused on demographics, awareness and when disease was diagnosed, type and frequency of crisis, family history, screening and presence of other diseases and awareness of trigger from these conditions. A total of 69.9% were G6PD deficiency gene carriers and mostly fell within the age group of 31 - 40 (43.6%). 75.6% of the carriers were not aware that other disease conditions could exacerbate crisis. Non-drug related crisis was 85.3% compared with 14.7 drug related reactions. Out of the 69.9% carriers, 17.3% are diabetics, 18.3% of them are hypertensive and 25.9% are with bleeding disorders. Also, 29.1% had frequent episodes with bacterial infection. Symptoms experienced that were not drug related included, 44.8% attacks of pallor, 25.3% jaundice, 28.5% with shortness of breath, while 21.7% had dark urine frequently. No routine screening for other diseased conditions was carried out for these subjects despite experiencing frequent crisis that were not drug related. Carriers with other diseased conditions experienced more non-drug related crisis and were not aware of it.