PSYCHOSOCIAL ADAPTATION IN ADOLESCENTS AND YOUNG-ADULTS WITH MARFAN-SYNDROME - AN EXPLORATORY-STUDY

We conducted a pilot study to evaluate the psychological effects and c onsequences of Marfan syndrome in 17 patients between 16 and 35 years of age. Through a semi-structured interview, we investigated how the p atients coped daily with Marfan syndrome and evaluated the impact of t he disease on specific items such as schooling, occupational choices, self-image, and social behaviour. A second part of the study consisted of a battery of standardised psychological tests to evaluate the pati ents' anxiety and depression levels and coping styles. The following p sychological tests were used: State and Trait Anxiety Inventory (STAI) , Beck Depression Inventory (BDI), and the Utrecht Coping List (UCL). The interviews showed that the disease represented a significant burde n on the daily physical activities of the patients, as well as on thei r schooling and job opportunities. During childhood, most of them were insensitively teased by peers because of their typical phenotypic fea tures. This had its consequences on their social behaviour in that the y are all more introvert. In the female patients, the risk associated with child bearing represented a major concern. Most patients emphasis ed the need for accurate information about the illness immediately aft er knowing the diagnosis and all found psychological support helpful. Depression and anxiety levels were higher in the female than in the ma le group, without being significantly different from the normal popula tion. Nevertheless, most patients have come to terms with their diseas e and consider themselves happy most of the time.