Abstract

Introduction: Thalassemia patients have experienced a dramatic increase in lifespan secondary to improved and more complicated therapy. The impact of the disease and its treatment on quality of life is largely unknown. Determining the degree of health impairment as perceived by the patient is essential information needed to recommend suitable therapy. Factors that affect the quality of life of thalassemia major patients may differ from thalassemia intermedia patients. Identifying the specific causes for negative patient assessment of overall health status can result in successful intervention. The objective of this study is to determine the factors that affect quality of life in both transfused and non-transfused thalassemia patients.

Methods: The Dartmouth Care Cooperative Chart System (COOP) questionnaire is a standardized, validated, visual screening tool that screens the major domains affecting quality of life. Patients rate quality of life from excellent (1) to poor (5). Scores of 1 and 2 are normal. A Score of 3 are mild to moderate impairment, and 4 and 5 are severe abnormalities. 50 patients were randomly selected during a comprehensive visit to complete a COOP questionnaire. These results were augmented by a brief medical history and chart review.

Results: Forty-eight thalassemia patients, including 25 transfusion dependent (Tx) and 23 non-transfused (Non-Tx) patients (50% female) were randomly selected and completed the COOP questionnaire. The mean age of the total population was 19 years (0.9–37.9 yrs) and was similar in both groups. Half of both groups had scores of 3 or less indicating no severe impairment. However, 56% of patients had ratings of 3 indicating mild to moderate impairment of major domains. Four patients (2 Tx, 2 Non-Tx) had repeated 4–5 ratings in multiple domains indicating severe impairment of quality of life. The most commonly reported affected domains were feelings, such as anxiety, depression, and concern of overall health status or indications of recent deterioration in health.

Conclusion: At least 50% of transfusion and non-transfusion dependent thalassemia patients demonstrate some impairment in quality of life. Forty-four percent of the population had a severe impairment in at least one domain. In particular, mental health issues are widespread. In contrast to previous beliefs, non-transfusion dependent patients also suffer serious impairment in quality of life. This data suggests all patients should undergo quality of life assessments and intervention, which focuses on affected domains. Studies to determine if quality of life affects patients’ adherence to chelation therapy are needed.

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