Abstract 110: Quality of life in Beta Thalassemia

Abstract

Numerous scientific evidence support that quality of life of the patients have significant prognostic impact in various heart's diseases. Studies on quality of life in general population have led to the validation of different questionnaires. Currently there are no specific questionnaires for assessing the quality of life in thalassemic patients. Questionnaire on health status SF-36 is a validated and simple tool that, through two synthetic indices, which refers to physical (Physical Component Summary, PCS) and psychic health's domain (Mental Component Summary, MCS), provides important information on quality of life. PCS reflects concepts related to physical morbidity, while the index MCS reflects psychological or mental morbidity. Questionnaire SF-36 requires an assessment of the patient's activities of daily living (personal hygiene wait, walk, climb stairs, carry weight, etc.), subjective health status, emotional state, on social life and relationships. The aim of our study was to assess whether, in this population, the derived indices have diverged significantly from the healthy reference population and, if so, whether there was a correlation with the severity of underlying heart disease. To assess quality of life in a population of patients (n. 46) with thalassemia major and/or intermediate followed at our center, we administered the Italian version of the questionnaire SF-36 (Apolone, 2005), relating derived indexes with the main clinical and instrumental parameters, especially functional NYHA class, ejection fraction and indices of left ventricular diastolic function, T2* values, echocardiographic signs of pulmonary hypertension and right ventricular dysfunction, such as PAPs values and RV FAC. The mean values of the indices PCS and MCS in the entire study population were, respectively, 47.50 and 51.24, overlapping those of healthy reference population (Survey on health status of Italians, ISTAT 1999-2000). Subgroup analysis showed that the presence of pulmonary hypertension and/or right ventricular dysfunction was associated with a significant deterioration in the perception of physical health (PCS 35.9 versus 48.3 in patients without pulmonary hypertension). The analysis of correlation between PCS and clinical-instrumental variables studied has also returned frankly negative values with the values of PAPs (r -0.86), confirming the negative impact of the presence of pulmonary hypertension on health's perception. No other significant association was identified. In conclusion, our study highlighted how detection of pulmonary hypertension in an unselected population of patients with beta-thalassemia has a detrimental impact on perceived quality of life.