Attitudes antecedent to transition to self-management of a chronic genetic disorder

Marfan syndrome (MFS) is the exemplar of chronic genetic disorders that require multiorgan system management by health care providers (HCPs) and lifelong self-management by affected individuals. This article describes the ways to facilitate transition to self-management (TSM) by the adolescent with MFS. This thematic content analysis uses data collected for a larger grounded theory investigation of TSM of a chronic genetic disorder in adolescents and focuses on the system issues related to transition. A total sample of 107 included three groups of participants: parents (n = 39), adolescents (n = 37, ages 14-21 years) and young adults (n = 16, ages 22-34 years) with MFS, and HCPs (n = 15), including physicians, genetic counselors, and nurses. Data were derived from 180 transcripts of audiotaped interviews and a sociodemographic survey. Frames of mind that are antecedent to transition were belief in the diagnosis, wanting to understand and appreciate the cause and effect of MFS, and willing to share responsibility in problem solving. These frames of mind occurred primarily within the context of the family relationship. Parents taught children self-surveillance as 'listening to one's body'. The parent's fears and need to stay involved in the child's health care slowed the child's independent work on self-management responsibilities. A systematic, institutionalized transition program for adolescents might involve parents and the child soon after diagnosis and incrementally build acknowledgment, understanding, and rapport.