The adolescent thalassemic. The complicant rebel

Abstract

Chronic hereditary conditions are expected to have a strong influence on the psychosocial development of the adolescent. Thalassaemia major is the commonest of these disorders in Mediterranean countries. In the past it was rarely seen in adolescence because the majority of patients died in childhood. The availability of treatment has allowed survival but the optimum treatment is difficult, especially chelation which consists of daily subcutaneous infusions of 10 hours duration. The adolescent patient must achieve social independence while he/she has a dependency on family, doctors and nurses and a need to strictly adhere to a difficult regime. Non-adherence will result in complications and possible fatality. Family and caretakers may become overprotective. Short stature, bone deformities and poor sexual development influence self image and self confidence. The purpose of this paper is to examine how various published studies have viewed the adolescent with Thalasaemia and to draw conclusions about the necessity for supportive services for the patients and their families. Earlier studies gave emphasis to negative psychosocial trends such as dysphoric moods, dependency, low self esteem, fears, anxiety etc. These were attributed to family stresses and over-protectiveness as well as the condition. Other studies from centers in Italy mainly, have demonstrated normal psychosocial development with better social adjustment than normal peers. Recently published data suggest that Italian patients are more compliant to treatment compared to Greek and Cypriot patients. This may be due to better psychosocial support. The need for such support is emphasized as is the need for training of doctors and nurses dealing with Thalassaemia major patients.