ABSTRACT
BACKGROUND: Longtime outcome in case of thalassemia depends on the patients' adherence in home treatment to reduce hemosiderosis. This study describes the patients' perspective, their typical coping strategies, health related locus-of-control-beliefs and psychosocial influences on adherence. PATIENTS AND METHODS: A battery of questionnaires was employed to 43 patients with thalassemia major (3 to 26 years old) treated in Germany according to the german multicenter study respectively their parents: the Ulm Thalassemia Inventory, the KIDCOPE, the Multidimensional Health Locus of Control Scales and the Giessen Complaint List. Clinical symptoms of hemosiderosis were correlated with psychosocial variables. RESULTS: The patients feel more distressed from their treatment than from their illness itself. They react to disease-related distress with a variety of coping strategies. Some of the most frequent coping strategies are maladaptive, indicating feelings of helplessness. Internal locus-of-control-beliefs were low and fatalistic locus-of-control-beliefs were high compared with other clinical groups. The self-reported adherence to the iron chelation treatment is correlated with age, gender, age at the start-point of the treatment and emotional distress. Complaints, coping strategies and locus of control are independent from adherence as well as from hemosiderosis. CONCLUSION: Patients with thalassemia major need more information about their disease and about the benefits of iron chelation therapy. Additional psychosocial support should reduce emotional distress, strengthen coping competence and lead to a better integration of therapy in daily life.
