Marfan syndrome in adolescents and young adults: psychosocial functioning and knowledge

Twenty-two subjects with Marfan syndrome (age range 11-24 years, mean 15.7 years) were studied to evaluate the impact of Marfan syndrome on their lives, level of psychosocial adaptation, concerns about their disorder, compliance with medical regimens, and knowledge of Marfan syndrome. Subjects were interviewed and then completed the Offer Self-Image Questionnaire and two questionnaires designed for the study. Parents also were interviewed. Subjects demonstrated normal psychosocial adaptation on the Offer Self-Image Questionnaire. Nevertheless, the subjects perceived that their lives would be significantly better without Marfan syndrome, especially in the areas of physical activities and self-image. They expressed concerns about their illness most often to their parents, less frequently to their doctors. Their compliance with medical regimens were suboptimal and similar to the published description of compliance among teenagers with other chronic illnesses. Their knowledge base was weakest in the areas pertaining to future childbearing. Thus, although these subjects showed normal psychosocial adaptation, doctors caring for them may foster better coping with Marfan syndrome by discussing self-image issues and Marfan syndrome-related concerns, and by encouraging compliance and imparting knowledge.