The transition to adulthood in adolescents and young adults with Marfan Syndrome : a scoping review Ng, Tiffany Anne
BACKGROUND: In healthcare, one type of transition is the planned movement of adolescents or young adults with chronic conditions from the pediatric health care system to the adult one. During this time, there is a high risk for these individuals to be lost to follow-up. Patients with Marfan Syndrome (MFS), a potentially life-threatening genetic condition, are one group that is subject to this risk. With improving life expectancy, an organized transition process is needed to prepare adolescents and young adults for the challenges of both adulthood and their condition. Nurses can be key players in facilitating this process. OBJECTIVES: 1) To map the range of evidence on the transition process for patients with MFS, as identified in both peer-reviewed and grey literature; 2) to examine the concepts and themes of transition discussed in the literature for patients with MFS; and 3) to identify gaps in the current literature and draw recommendations from another chronic condition, specifically congenital heart disease (CHD), to determine areas for further research. METHODS: Four databases and grey literature were searched for English publications published between 2003 and 2018, focusing on adolescents or young adult patients with MFS or CHD during transition. RESULTS: Thirty-one publications (24 research articles, 7 websites) met the inclusion criteria with the majority of the literature originating in North America (n = 20). Schumacher and Meleis’ 1994 transition framework guided the analysis of the literature. Twenty-seven publications (87%) provided a definition of transition and the level of planning was the most commonly discussed factor affecting transition. However, indicators of healthy transition were rarely measured. A thematic analysis revealed four components of the transition process: the need for knowledge and skills; the impact of parental involvement; preparing and planning for the future; and the transition readiness of the adolescent or young adult. CONCLUSION: The existing literature highlights that the transition process is important in the transition of adolescents and young adults with chronic conditions to adulthood; however, a gap is evident in our knowledge of this process for patients with MFS. Further research in this area is indicated to identify the needs of MFS patients and to test interventions to facilitate a smooth transition to adulthood.
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