Open Collections

UBC Graduate Research

The transition to adulthood in adolescents and young adults with Marfan Syndrome : a scoping review Ng, Tiffany Anne 2018-08

Your browser doesn't seem to have a PDF viewer, please download the PDF to view this item.

Notice for Google Chrome users:
If you are having trouble viewing or searching the PDF with Google Chrome, please download it here instead.

Item Metadata

Download

Media
42591-Tiffany_A_NURS595_Transition_to_adulthood_Marfan_Syndrome_2018.pdf [ 971.82kB ]
Metadata
JSON: 42591-1.0371940.json
JSON-LD: 42591-1.0371940-ld.json
RDF/XML (Pretty): 42591-1.0371940-rdf.xml
RDF/JSON: 42591-1.0371940-rdf.json
Turtle: 42591-1.0371940-turtle.txt
N-Triples: 42591-1.0371940-rdf-ntriples.txt
Original Record: 42591-1.0371940-source.json
Full Text
42591-1.0371940-fulltext.txt
Citation
42591-1.0371940.ris

Full Text

 1 THE TRANSITION TO ADULTHOOD IN ADOLESCENTS AND YOUNG ADULTS WITH MARFAN SYNDROME: A SCOPING REVIEW  by TIFFANY ANNE NG BSN, Langara College, 2010  A SCHOLARLY PRACTICE ADVANCED RESEARCH (SPAR) PROJECT SUBMITTED IN PARTIAL FULFILLMENT OF THE REQUIREMENTS FOR THE DEGREE OF  MASTER OF SCIENCE IN NURSING  in THE FACULTY OF GRADUATE AND POSTDOCTORAL STUDIES  THE UNIVERSITY OF BRITISH COLUMBIA (Vancouver)  August 2018 © Tiffany Anne Ng, 2018  2 Abstract 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  3 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.     4 Table of Contents Abstract .................................................................................................................................2  Table of Contents .................................................................................................................4 List of Tables ........................................................................................................................7 List of Figures .......................................................................................................................8 Acknowledgements ..............................................................................................................9 Chapter 1: Introduction ......................................................................................................10 Definition of Terms ...........................................................................................................10 What is Marfan Syndrome? ..............................................................................................11 History ..........................................................................................................................12 Statistics and Current State ...........................................................................................13 Effect of Marfan Syndrome on Youth ..........................................................................14 Defining Transition ...........................................................................................................15 Distinguishing between Transition and Transfer .........................................................16 Goals and Objectives of Transition ..............................................................................16 Key Players in the Transition Process ..........................................................................17 The Transition Framework ...........................................................................................18 Rationale of Interest ..........................................................................................................19 Significance and Focus of Analysis ..................................................................................20 Objectives ..........................................................................................................................21 Chapter 2: Methods .............................................................................................................22 Defining Scoping Review .................................................................................................22 Information Sources ..........................................................................................................22  5 Search Strategy ..................................................................................................................23 Inclusion and Exclusion Criteria .......................................................................................24 Screening and Selection Process .......................................................................................25 Analysis Process ................................................................................................................25 Chapter 3: Results................................................................................................................26 Search Results and Selection of Literature .......................................................................26 Marfan Syndrome and Transition .................................................................................26 Congenital Heart Disease and Transition .....................................................................26 Summary of Literature ......................................................................................................29 Timing of Publications .................................................................................................33 Research Methods Employed .......................................................................................34 Geographic Origin of Publications ...............................................................................35 Perspective/Target Audience ........................................................................................36 Schumacher & Meleis’ Transition Framework ............................................................37 Universal properties of transition – defining transition ...........................................37 Transition conditions ...............................................................................................38 Indicators of healthy transition ................................................................................39 Identified Themes .........................................................................................................40 Need for Knowledge and Skills ...............................................................................42 Parental Involvement ...............................................................................................43 Preparation and Planning for the Future ..................................................................44 Transition Readiness ................................................................................................44 Summary of Results ..........................................................................................................45  6 Chapter 4: Discussion/Conclusion ......................................................................................46 Key Findings .....................................................................................................................46 Evolution of the Literature over Time ..........................................................................46 Schumacher and Meleis’ 1994 Transition Framework ................................................49 Comparison of Themes .....................................................................................................50 Identified Gaps and Recommendations for Future Research ............................................51 Implications for Nursing ...................................................................................................53 Knowledge Translation .....................................................................................................55 Limitations ........................................................................................................................56 Conclusion .........................................................................................................................56 References .............................................................................................................................58 Appendix A: Search Strategy for Marfan Syndrome and Transition ............................71 Appendix B: Search Strategy for Congenital Heart Disease and Transition .................73 Appendix C: Research Literature Findings ......................................................................74 Appendix D: Grey Literature Findings .............................................................................86 Appendix E: Analysis Using Schumacher and Meleis’ Transition Framework ............88    7 List of Tables Table 1. Search Terms for Marfan Syndrome and Transition Search ...................................23 Table 2. Search Terms for Congenital Heart Disease and Transition Search ........................23 Table 3. Literature Included in Scoping Review ...................................................................29 Table 4. Themes Found in Transition Literature ...................................................................41    8 List of Figures Figure 1. PRISMA – Marfan Syndrome ................................................................................27 Figure 2. PRISMA – Congenital Heart Disease ....................................................................28 Figure 3. Publications on transition, by condition and year ..................................................34  Figure 4. Research studies, by research method ....................................................................34 Figure 5. Grey literature publications, by type ......................................................................35 Figure 6. Type of publications, by geographical location .....................................................35 Figure 7. Research publications, by perspective ....................................................................36 Figure 8. Grey literature publications, by target audience .....................................................36 Figure 9. Publications that defined transition ........................................................................37 Figure 10. Publications measuring/discussing transition conditions .....................................39 Figure 11 Publications measuring/discussing indicators .......................................................40 Figure 12. Thematic focus in the literature ............................................................................42    9 Acknowledgements First and foremost, I would like to dedicate this work to my mom who has been my greatest supporter through this journey. Thank you for always believing in me and motivating me to strive for greatness in everything I do.  I would like to express my deepest gratitude to my supervisor, Dr. Martha Mackay, for all of the guidance, support, and encouragement throughout this SPAR project. Thank you for helping me bring my ideas to life and develop this paper into what it is today.  I would also like to express my sincerest appreciation to Karen LeComte, my committee member for this SPAR project. Your expertise helped enhance this paper significantly. Thank you for all your contributions and for being a part of this journey with me. To Heztan James, I could not have made it through this journey without you. Thank you for your patience, understanding, and the drive to keep going.  To my entire family, thank you for always standing behind me and for supporting me through all my educational endeavors. To Uncle Steve, I could not have made it through all this writing without you. Thank you for always encouraging me to become a better writer. Finally, to my brother. I would not have chosen to embark on this SPAR project without inspiration from you. Our journey as a family has been and always will be unique and that continually motivates me to learn more about your condition and to share this knowledge with others. I know dad would be proud looking down on us. I also dedicate this work to you.    10 Chapter 1: Introduction In British Columbia (B.C.), the legal age of adulthood is 19 years old (Queen’s Printer for British Columbia, 2018). In healthcare, young adults graduate from a number of childhood services, such as school, insurance, and home care, at the age of 18 (Provincial Health Services Authority, 2018). They also transfer from the pediatric health care system to the adult one, regardless of whether they are prepared and/or ready (Provincial Health Services Authority, 2018). As a result of this shift, adolescents or young adults are at a high risk of being lost to follow-up and not becoming attached to the new providers or clinic. Those who are affected are the individuals with chronic conditions that begin in childhood and require continuous care into adulthood, due to the potential complications that may arise with the disease. One of these conditions is Marfan Syndrome, a genetic disorder that may result in life-threatening cardiovascular complications (Sivasankari, Mathew, Austin, & Devi, 2017). With the continuing advancements in technology, the life expectancy and the survival rates of these patients with chronic illnesses are improving; therefore, a coordinated, organized, and smooth transition process is needed for adolescents and young adults to prepare them for the potential challenges of both adulthood and their condition as they move from a pediatric to adult care setting (Kovacs & McCrindle, 2014).  Definition of Terms The terms ‘adolescent’ and ‘young adult’ have different meanings, but both are often included in the discussion of transitioning to adulthood. Adolescent describes an individual between the ages of 10 and 19 years old (World Health Organization, 2018) and is a term often used in pediatric care. A young adult is between the ages of 18 to 34 years old (Clark, 2014).  11 Considering that transition occurs over both age groups, the term ‘youth’ will be used throughout this paper to describe the entire spectrum of these patients. What is Marfan Syndrome? Marfan Syndrome (MFS) is an autosomal-dominant genetic disease caused by a mutation in the gene that codes fibrillin-1 (FBN-1), a gene that contributes to the elastic and non-elastic structures of connective tissue (Sivasankari et al., 2017; Fusar-Poli et al., 2008). Patients with MFS may exhibit a variety of signs and symptoms in multiple organ systems, because all body tissues contain connective tissue. These may include abnormalities in the skeletal system (tall and thin body structure, long limbs, and arachnodactyly or spider-like extremities); the eyes (lens dislocation); the skin (stretch marks); and the lungs (Sivasankari et al., 2017; Fusar-Poli et al., 2008). The most life-threatening manifestations of MFS are cardiovascular, such as dilatation of the aorta leading to dissection or rupture (Sivasankari et al., 2017; Fusar-Poli et al., 2008).   Diagnosing MFS can be challenging as some clinical manifestations may be age-related; therefore, patients who exhibit suggestive features of MFS require repeated clinical assessments, periodic follow-up, and genetic screening of family members (Sivasankari et al., 2017). To assist with the diagnosis of MFS, the Ghent criteria was proposed and adopted worldwide as the guide in diagnosing this condition (Faivre et al., 2012). The Ghent diagnostic criteria consider a patient’s family/genetic history, involvement of organ systems (skeletal, cardiovascular, and ocular) and whether the clinical manifestation is major or minor with major symptoms being specific for MFS (Sivasankari et al., 2017). A confirmed diagnosis of MFS requires regular monitoring and evaluation, including diagnostic tests and routine follow-up with health care providers (HCPs), for maintaining and monitoring the disease progression.  12 History Marfan syndrome was named after the French pediatrician Antoine Bernard Jean Marfan (Chaffins, 2007). In 1896, Marfan documented an assessment of a 5-year-old girl with an unusually long, disproportionate body, extremities that were spider-like or long and thin, kyphoscoliosis or an abnormal curvature of the spine side-to-side and back-to-front, an anterior chest deformity, and pulmonary tuberculosis. Forty years after, Marfan had reviewed over 150 similar cases and the disorder was also found to affect other organs, such as the eyes and the heart. By the early part of the 20th century, similar clinical manifestations were reported by other physicians, which led to Marfan’s name being linked to the condition. The term “syndrome” was then added to describe that the disorder entailed a group of physical signs and symptoms that occurred together and characterized the condition (Chaffins, 2007; Merriam-Webster, 2018).  Individuals with the clinical manifestations of MFS have been present in our society for a long time, even before Marfan’s initial assessment of the 5-year-old girl. A physician named E. Williams made these discoveries approximately 20 years before Marfan; however, due to a lack of documentation, his findings were unrecognized for some time by the medical community (Chaffins, 2007).  After these initial findings of the signs and symptoms, further developments related to the condition became known. In 1943, complications in the aorta were initially described, but it was not until 1955 that the full extent of the cardiovascular abnormalities were known (Chaffins, 2007). It became evident that patients with MFS had a genetic defect that affected both the eyes and the aorta, but the questions of what and how were still unanswered. The first possible answer came from Sakai and her team in 1986, who suggested that the defect was on the protein named fibrillin, but it was the researchers at John Hopkins Hospital that discovered that the actual cause  13 of MFS was a defect on FBN1, the gene that encodes the protein fibrillin (Chaffins, 2007; Pyeritz, 2000). After further testing with the FBN1 gene, in 1991, researchers were able to unquestionably determine that this defective gene was the main cause of MFS.  Statistics and Current State According to the Marfan Foundation (2014), MFS affects about 1 in 5000 people with both men and women of all ethnicities equally affected. People with MFS inherit the condition from an affected parent 75% of the time, which occurs because MFS is an autosomal-dominant genetic condition, which means that each child of an affected parent has a 50% chance of inheriting the condition (Marfan Foundation, 2014). However, a spontaneous genetic mutation on the FBN1 gene may occur, in which case an individual may be the first in their family to have it.  Because MFS affects only 1 in 5000 individuals (Marfan Foundation, 2014), people are unaware of its existence. There are also a limited number of studies that assesses the psychological, social, and behavioral aspects of living with MFS (Peter, Kong, Horne, Francomano, & Biesecker, 2001; Velvin, Bathen, Rand-Hendriksen, & Geirdal, 2015). MFS is a lifelong disease that is most often diagnosed in childhood or young adulthood, and less commonly in adults, following a major cardiac event (Marfan Foundation, 2014). Velvin et al. (2015) found that patients with MFS “experience a significant impact of their diagnosis on their lives regarding aspects of being an adolescent and transition to adulthood, family life, education and work, psychological adjusting and health-related quality of life (HRQoL)” (p. 114).     14 Effect of Marfan Syndrome on Youth  Having MFS during one’s youth may complicate the physical, emotional, and developmental changes that occur during this period. There are two areas that the literature identifies as highly affected by having MFS: physical activity and physical appearance (Schneider, Davis, Boxer, Fisher, & Friedman, 1990; Van Tongerloo & De Paepe, 1998). Affected youth are often limited in their physical activities and restricted from participating in certain sports, such as those that have physical contact (Schneider et al., 1990). The physical limitations may also interfere with the activities of daily living and cause stress on daily life, school attendance, work opportunities, and social interactions (Velvin et al, 2015). The physical manifestations of MFS (tall stature, long and skinny legs, and thick glasses) make the youth appear differently than their peers. Van Tongerloo and De Paepe (1998) found these differences resulted in teasing, verbal bullying, and even total exclusion, which as a result, negatively impacts the adolescent’s self-esteem and self-confidence. This may cause a disruption of body image and create social and interpersonal difficulties, which may lead to higher levels of anxiety and depression (Van Tongerloo & De Paepe, 1998).   Planning for the future is also affected by MFS. Youth with MFS need to consider appropriate educational and vocational choices that would not cause unnecessary stress for their body. They also need to understand that they are at increased risk for severe morbidity and early mortality and therefore, the decisions they make can potentially impact their lifespan (Schneider et al., 1990). Family planning also needs careful consideration: pregnancy in a patient with MFS increases the mother’s mortality risk and there is a 50% chance of transmitting MFS to the baby (Schneider et al., 1990).  15  Youth may be angry about their situation (Backhouse, 2005). They may feel burdened by having to attend treatments and check-ups, to take medications, and to possibly sacrifice some of their favourite activities, which may lead to failure to adhere with the recommendations (Backhouse, 2005). The emotional distress caused by the condition may disrupt the relationships with HCPs and parents, as anger may be directed towards them and may cause the patient to isolate themselves from their peers (Backhouse, 2005). In summary, MFS can cause both physical and psychological challenges; therefore, understanding these effects from the perspective of the youth is important to ensure their needs are addressed appropriately. Defining Transition  According to the Merriam-Webster dictionary (2018), transition is the passage, movement, development, or evolution from one state, form, or place to another. In the healthcare literature, and for the context of this paper, transition is defined as “the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centred to adult-oriented health-care systems” (Blum et al., 1993, p. 570).  It involves preparation and planning and a gradual change in attitudes and behaviours that is influenced by the youth, their parents, and the HCPs involved (Sawyer, Blair, & Bowes, 1997; Giarelli, Bernhardt, Mack, & Pyeritz, 2008). The recommended age to begin transition is 12 years old; however, the patient’s medical and developmental status should also be considered (Warnes et al., 2008; Sable et al., 2011). Therefore, the transition process should be individualized to each youth (Sable et al., 2011).  One of the key components of transitioning to adulthood is the transition to self-care. Self-care involves three components: maintenance (actions and behaviours that maintain stability of the condition); monitoring and surveillance (attention and recognition to changes in  16 symptoms); and management (response to symptoms and participation in treatments) (McCabe et al., 2015; Giarelli, Bernhardt, Mack, et al., 2008). The literature on transitioning to adulthood indicates that self-care is part of the shift in responsibility from the parent to the youth. The youth is expected to know, understand, and be able to describe their condition, their treatments, the necessities to promote their own health, and the signs and symptoms that require medical attention (Sanders, Kuo, Levey, & Cheng, 2009). Meeting these expectations is the first step towards becoming an independent and autonomous adult, which may facilitate the transition to the adult health care system.  Distinguishing between Transition and Transfer The literature on transition often includes the term ‘transfer’. Transfer is the actual point in time when the responsibility for a patient’s care is moved from one healthcare team to another, which, in the context of this paper, is the move from the pediatric to adult team (Sable et al., 2011; LeComte et al., 2016). The recommended age for transfer is between 18 and 21 years old; however, like the transition process, each individual youth’s transition readiness and level of maturity also needs to be considered (Sable et al., 2011; Uzark & Wray, 2018). The transfer of a youth does not mean that the transition process is completed, but rather, it continues until the patient has all the necessary skills and abilities to take full control and responsibility of their condition (Thomet, Schwerzmann, & Greutmann, 2015). Goals and Objectives of Transition  The goals of transition to adult care for youth are summarized in four main points (American Academy of Pediatrics, American Academy of Family Physicians, & American College of Physicians-American Society of Internal Medicine, 2002; Rosen et al., 2003; Sanders  17 et al., 2009). First is to ensure continuity of care and resources, preventing patients from being lost to follow-up. The collaboration and communication between pediatric and adult HCPs can help to facilitate this continuity. Second is to optimize health through prevention and promotion strategies and recognition of signs and symptoms that require medical attention (Scal, 2002). HCPs from both systems can provide education the youth needs to fill any knowledge gaps about their condition. Third is to ensure youths are able to maintain a quality of life in their daily living in spite of their chronic condition. Knowledge, skills, and understanding on how to self-manage their condition can help patients perform their activities of daily living and ease the stress of having this chronic condition. Fourth is to promote the independence of the youth and facilitate the attainment of their maximum potential. The shift of responsibility from the parent to the child is expected and involving the youth in discussions about future aspirations, educational/vocational goals, and family planning is the first step towards promoting autonomy. Key Players in the Transition Process  The transition process frequently involves the youth, the parents, and the HCPs. Each participant has different responsibilities and roles to play in achieving the four goals of the transition process. They also have different perspectives and needs, which may positively or negatively impact the process (Kovacs & McCrindle, 2014). Including all of these stakeholders in the transition planning will help to ensure an effective and satisfactory transition and transfer to adult care (Kovacs & McCrindle, 2014).   During the time of transition, youths experience many changes physically, cognitively, emotionally, and developmentally (Thomet et al., 2015). They are learning to navigate through the challenges of life and to plan for their future. Undergoing multiple transitions simultaneously can be stressful, but for some, it gives a sense of empowerment and maturity.   18 Parents of the youth are considered an integral part of the transition process. They have managed their child’s condition since birth and know their child’s abilities the best. During the transition, parents are expected to shift the responsibility of managing the chronic illness to the youth; however, because many years of their family life may have centered on the sick child, their attitudes often favor overprotection, leading to difficulty letting their youth take full control of their condition (Thomet et al., 2015). These attitudes may be detrimental to the transition process and may hinder the youth’s progress towards self-management and foster a dependency towards the parent, therefore delaying the actual transfer to adult care (Thomet et al., 2015; Uzark & Wray, 2018; Gray, Schaefer, Resmini-Rawlinson, & Wagoner, 2018). Involving parents in the transition and assessing parental needs is an important aspect to consider, as the process may be more difficult and anxiety-provoking for them, compared with their child.  The HCPs involved in the transition can support the process for both the youth and their parents. These HCPs include physicians, nurses, and other allied health members contributing to the care of youth, such as social workers and dieticians. The HCPs are considered the ‘experts’ during this transition and can help prepare the family for the transfer with education, resources, and emotional support.  The Transition Framework Schumacher and Meleis’ (1994) transitions theoretical framework was created based on a review of nursing literature that identified transition as a central concept in nursing. It views transition as a process that consists of elements individualized to the patient’s needs and the resources available with the aim of promoting successful and healthy changes for the patient (Schumacher & Meleis, 1994; Lugasi, Achille, & Stevenson, 2011). The framework shows the interrelationship between five categories: 1) type of transition (developmental, situational,  19 health/illness, or organizational); 2) universal properties (process direction, change in roles, relationships, identity, and behavior); 3) transition conditions (meanings, expectations, levels of knowledge/skill, environment, level of planning, and emotional and physical well-being); 4) indicators of healthy transition (subjective, well-being, mastery); and 5) nursing therapeutics (promotive, preventive, and interventive). This holistic perspective emphasizes the importance of understanding the various aspects involved in transition, including the subjective, behavioral, and interpersonal dimensions, in order to thoroughly address the challenges and health consequences associated with the process.   For this paper, three categories of this framework will be used to guide the methodological premise and type of inquiry in the literature: universal properties, transition conditions, and indicators of healthy transition. Applying this transition framework helped to identify the key transition concepts that have already been examined for patients with MFS and the areas that need to be addressed further. Rationale of Interest My interest in the transition for individuals with MFS stems from our family’s first-hand experience with this process. At the age of 6, my brother was diagnosed with MFS. Our pediatrician would regularly monitor our height and weight, but information about what this condition was and how it would manifest was not provided. My brother’s care was transferred to our family physician a few years later as our pediatrician retired. At the age of 12, my brother had grown significantly and was found to have scoliosis. He was referred to a physician at Children’s Hospital, whose recommendations included wearing a back brace to correct his posture and administering injections to stunt his growth. He was followed for a few years until his physical growth was completed. During this time, our mother had thought my brother was  20 simply growing faster than his peers. No one had informed her that this was a manifestation of MFS. To my family, MFS was a condition my brother had, but none of us knew the severity of the disease or what was to expect.  At the age of 19, my brother endured a major cardiac event, resulting in a lengthy open-heart surgery. He had a dissecting aneurysm and a severely leaking heart valve. It was after this event that we were informed these cardiovascular complications were caused by his MFS. From the time of diagnosis until this event, my brother had had no specific follow-up for his MFS, nor did we have any education regarding this condition. The lack of professional guidance, the gap in our knowledge about the condition, and a delayed re-connection to the health care system made the transition process difficult as we navigated the health system to manage his health condition while facing the challenges of transitioning to adulthood. Significance and Focus of Analysis The transition from childhood to adulthood is a crucial developmental stage during which youth experience immense life changes (Gorter, Stewart, & Woodbury-Smith, 2011). A chronic disease may pose an extra burden on the individual and may impact development and independence (Mora, Sparud-Lundin, Bratt, & Moons, 2017). Many transition studies have explored management of more common chronic diseases, including diabetes, cystic fibrosis, and congenital heart disease; however, there are limited studies exploring this process in rarer conditions, like MFS. A preliminary search reveals that much of the literature found on MFS focuses on the management of the clinical manifestations of the condition and the progression of the disease, but rarely on the emotional, psychological, and social needs of these individuals. Addressing these needs during the transition process is crucial to the life-long coping and self-management of the condition.   21 Objectives For this Scholarly Practice Advancement Research (SPAR) project, there are three 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 of further research.    22 Chapter 2: Methods Defining Scoping Review For this project, a scoping review was conducted to summarize the current extent and range of evidence available (Levac, Colquhoun, O’Brien, 2010). This type of review is useful for this project because it can incorporate different sources of emerging evidence, including both published and grey literature, examine the topic from a broader perspective by including different study designs, and identify gaps in the current evidence that may lead to further areas of research (Arksey & O’Malley, 2005; Joanna Briggs Institute, 2015). Rather than addressing specific research questions and assessing the quality of included studies, a scoping review provides a descriptive overview of all the research activity related to a topic (Pham et al., 2014). As the topic of transition to adulthood in patients with MFS is limited within the literature, a scoping review will be beneficial in systematically examining the current evidence in this area. Information Sources  A variety of information sources were examined to ensure a comprehensive search of the literature. Four academic databases that were comprised of literature from nursing, medicine, allied health, global health, social and behavioral sciences were searched for relevant scholarly literature: Cumulative Index to Nursing and Allied Health Literature (CINAHL), Medline (OVID), Web of Science, and CAB Direct (CAB Abstracts and Global Health). Additionally, search engines, including Google and Google Scholar, were explored to retrieve grey literature, including websites, newsletters, and fact sheets. Hand searching was also completed through the reference lists of relevant articles, by referring to the “similar articles” tool in the Medline database that identified related articles, by searching authors of direct hit articles, and by searching through the Congenital Heart Disease Journal.  23 Search Strategy  A comprehensive search strategy was developed to identify as much relevant literature as possible. The search terms/keywords used in the searches are listed in Table 1 and 2. The full details of each search strategy, including search/subject terms and MeSH headings used in each of the databases are identified in Appendix A and B. If a combination of search/subject terms used resulted in very few results, a more generalized search was conducted using only one of those terms. All searches were conducted during the months of March, April, and May 2018. Throughout the search process for this SPAR project, the University of British Columbia (UBC) librarian for Nursing was consulted to ensure a systematic, strategic, and thorough exploration of the literature was completed.  Table 1.  Search Terms/Keywords for Marfan Syndrome and Transition Search Marfan Syndrome OR Marfan’s Syndrome Adolescent OR Adolescence Transition Teenagers Self-Management  Young Adult Self-Care Transition to adult care Self-Regulation  Self-Monitor  Psychosocial Factors   Table 2. Search Terms/Keywords for Congenital Heart Disease and Transition Search Congenital Heart Disease Adolescent OR Adolescence Transition Teenagers Self-Management  Young Adult Self-Care  Self-Regulation  Self-Monitor      24 Inclusion and Exclusion Criteria  Because a preliminary search identified a limited amount of literature on the transition to adulthood of patients with MFS, the inclusion criteria for papers or other items was kept broad. Therefore, literature was considered for inclusion in this scoping review if the article (1) was published in English, (2) focused on adolescents or young adults, and (3) focused on the transition process and/or concepts related to it in patients with MFS from the perspective of the patient, the parents, and/or the healthcare provider. Literature was excluded if (1) its purpose did not focus on transition; (2) it did not specifically discuss the transition process or discussed a concept that spans the illness rather than only during the transition period; and (3) focused on a transition intervention. Grey literature, such as websites on MFS, was included if it met the inclusion criteria.  Due to the limited amount of literature found in the preliminary search, a high-level expanded search was also conducted to include academic literature on the transition process in adolescents or young adults with congenital heart disease (CHD), a chronic disease in pediatrics where lesions during embryonic development lead to abnormalities in heart wall, valves, or blood vessels formation (Niwa, 2015; Dray & Marelli, 2016). Articles that (1) focused on congenital heart disease (CHD) in adolescents or young adults, (2) were written in English, (3) were published in or after 2003, (4) were quantitative and/or qualitative research studies and/or systematic reviews (meta-analyses or meta-syntheses); and (5) focused on the transition process and/or concepts related to it were included in this scoping review. Articles were excluded if they were (1) intervention studies; and (2) opinion articles, non-research studies, and non-systematic reviews. A search for grey literature on CHD was not conducted; however, items on CHD found through the grey literature search for MFS were included in this review.  25 Screening and Selection Process The literature on MFS was selected by a two-step process and the literature on CHD was selected by a three-step process. In the first step, for both conditions, all the literature found was screened by their title and/or abstracts. Articles that discussed or mentioned the transition of youth with MFS and/or CHD to adult care in the title or abstract was saved and labelled as “to be examined further”. In the second step, for MFS, the inclusion/exclusion criterion and a full-text assessment was used to determine whether the article met the criteria. These steps were done simultaneously because the amount of literature for MFS was limited. For CHD, the screening using the inclusion/ exclusion criterion was the second step and the full-text assessment was the third.  Analysis Process After completing the second step of the screening process, the remaining articles on MFS and CHD that are included in this scoping review were examined more in-depth. The date, geographical region, type of study, study findings, and key themes of each piece of literature were summarized. Additionally, using Schumacher and Meleis’ (1994) transition framework, each item was analyzed according to the three categories of the framework: universal properties, transition conditions, and indicators of healthy transition. Each source of evidence was explored as to how it defined transition and whether it measured or assessed the transition conditions and the indicators of healthy transition. The extent of the discussion in these three categories was considered and gaps in literature were identified.    26 Chapter 3: Results Search Results and Selection of Literature Marfan Syndrome and Transition The search for literature on Marfan Syndrome (MFS) and transition in the four selected academic databases, two search engines, and through hand searching reference lists of direct hit articles and using the “Similar Articles” tool in Medline (OVID) yielded a total of 2,538 records. After removing duplicates (N=386) using the Ref Works program, the titles and abstracts of the remaining 2,152 records were reviewed for appropriateness and relevance to the topic. This process led to the removal of another 2,115 records. A total of 37 records, 12 academic publications and 25 grey-literature publications, were then assessed using the inclusion/exclusion criterion and read fully. An additional 31 records were excluded, resulting in six citations in the final sample. Three of the articles were academic literature, while the other three were grey literature, specifically websites. Figure 1 outlines this systematic process. Congenital Heart Disease and Transition  The expanded search on congenital heart disease (CHD) and transition in the four databases and through hand searching resulted in a total of 453 articles. Using the Ref Works program, 132 duplicates were removed. The remaining 321 articles were reviewed for relevance based on their title and abstract and another 206 were excluded. Using the inclusion/exclusion criteria, the remaining 118 articles were screened accordingly through their abstracts and a quick scan of the full text. Twenty-eight articles met the criteria and after being reviewed fully, seven more articles were excluded, resulting in 21 citations. Four grey literature records were found on CHD through the MFS search and were added, thus totalling 25 citations in the final sample. Figure 2 outlines this systematic process.  27 Figure 1. PRISMA - Marfan Syndrome                        Identified through academic database searching N = 2472  CINAHL n=266 Medline (OVID) n=1890 CAB Direct n=98 Web of Science n=218   Screening Included Eligibility Identification Identified through other sources N = 66  Google Scholar n = 6 Google n = 25 Hand-searching ref lists, “similar to function”, n = 35 Duplicate citations removed (N = 386) Screened N = 2152  Academic Literature n = 2127 Grey Literature n = 25  Excluded based on title and abstract review (academic literature) N = 2115   Inclusion/exclusion criteria applied, full-text assessed N = 37  Academic Literature n = 12 Grey Literature n = 25 Excluded, with reasons N = 31  • Study purpose not focused on transition n = 16 • Not primarily focused on MFS n = 5 • Not focused on adolescents/young adults n = 1 • Focused on the care/management of MFS n = 3 • Focused on an intervention or program n = 3 • Focused on living with MFS n = 3 Records included in scoping review N = 6  Academic Literature n = 3 Grey Literature n = 3   28 Figure 2. PRISMA – Congenital Heart Disease                        Identified through academic database searching N = 404  CINAHL n=114 Medline (OVID) n=97 CAB Direct n=20 Web of Science n=173   Screening Included Eligibility Identification Identified through hand-searching (reference lists, ‘similar to’ function) N = 52 Duplicate citations removed N = 132 Screened N = 324    Excluded based on title and abstract review for academic literature N = 206   Inclusion/exclusion criteria applied N = 118   Excluded based on inclusion/exclusion criteria:  N = 90  • Focused on a transition intervention or tool n = 20 • Not specifically focused on transition process n = 13 • Focused on adolescents living with the condition n = 5 • Focused on the care/management of CHD n = 6 • Non-research studies n = 42 • Not focused on CHD n = 4 Included in scoping review N = 25  Academic Literature n = 21 Grey Literature n = 4  Full-text assessment N = 28   Excluded based on full-text assessment: N = 7  • Systematic reviews not specifically focused on CHD n = 5 • Study purpose not transition related n = 2  Grey Literature (websites) on CHD found through the MFS search N = 4  29 Summary of Literature  A total of 31 publications (24 academic articles and seven websites) were identified for this scoping review. For the MFS literature, only three academic articles (#1, 2, 3 in Table 3) and three websites (#25, 26, 27 in Table 3) were found. The three articles found were all written by the same authors and based on the same study sample from a larger grounded theory study. Each one focused on an aspect of transitioning to self-management. For the literature on CHD, 21 academic articles (#4 to 24 in Table 3) and four websites (#28 to 31 in Table 3) were identified for this scoping review. Table 3, and appendices C, D, and E provide an overview of the articles included in this scoping review and the findings are discussed in greater detail in the following sections. Table 3. Literature Included in Scoping Review # Author Year Journal/Website Title of Paper 1 Giarelli, Bernhardt, Mack, & Pyeritz  2008 Qualitative Health Research Adolescents’ transition to self-management of a chronic genetic disorder  2 Giarelli, Bernhardt, & Pyeritz  2008 Clinical Genetics Attitudes antecedent to transition to self-management of a chronic genetic disorder  3 Giarelli, Bernhardt, & Pyeritz 2010 Health Education & Behavior Self-surveillance by adolescents and young adults transitioning to self-management of a chronic genetic disorder  4 Bratt, Burström, Hanseus, Rydberg, Berghammer 2018 Child: Care, Health and Development Do not forget the parents – Parents’ concerns during transition to adult care for adolescents with congenital heart disease  5 Ekim, Kolay, & Ocakci 2018 Journal for Specialists in Pediatric Nursing Readiness for transition from pediatric to adult care for adolescents with chronic heart disease  6 Burström, Bratt, Frenckner, Nisell, Hanséus, Rydberg, 2017 European Journal of Pediatrics Adolescents with congenital heart disease: Their opinions about the preparation for transfer to adult care  30 # Author Year Journal/Website Title of Paper & Öjmyr-Joelsson  7 Chen, Su, Chiang, Shu, & Moons 2017 European Journal of Cardiovascular Nursing Healthcare needs of adolescents with congenital heart disease transitioning into adulthood: A Delphi survey of patients, parents, and healthcare providers  8 Downing, Oster, & Farr 2017 Congenital Heart Disease Preparing adolescents with heart problems for transition to adult care, 2009-2010 national survey of children with special health care needs  9 Stewart, Chahal, Kovacs, Manlhiot, Jelen, Collins, & McCrindle  2017 Pediatric Cardiology Readiness for transition to adult health care for young adolescents with congenital heart disease 10 While, Heery, Sheehan, & Coyne 2017 Child: Care, Health and Development Health-related quality of life of young people with long-term illnesses before and after transfer from child to adult healthcare  11 Bohun, Woods, Winter, Mitchell, McLarry, Weiss, & Broberg 2016 Cardiology in the Young Challenges of intra-institutional transfer of care from pediatric to adult congenital cardiology: The need for retention as well as transition  12 Burström, Öjmyr-Joelsson, Bratt, Lundell, & Nisell 2016 The Journal of Cardiovascular Nursing Adolescents with congenital heart disease and the parents: Needs before transfer to adult care  13 Harbison, Grady Jr, Chi, & Fernandes 2016 Pediatric Cardiology Provision of transition education and referral patterns from pediatric cardiology to adult cardiac care  14 Asp, Bratt, & Bramhagen 2015 Journal of Pediatric Nursing Transfer to adult care – experiences of young adults with congenital heart disease  15 Heery, Sheehan, While, & Coyne 2015 Congenital Heart Disease Experiences and outcomes of transition from pediatric to adult health care services for young people with congenital heart disease: A systematic review  16 Lopez, Karlsten, Bonaduce De Nigris, King, 2015 Congenital Heart Disease Understanding age-based transition needs: Perspectives from adolescents and adults with congenital heart disease  31 # Author Year Journal/Website Title of Paper Salciccioli, Jiang, Marelli, Kovacs, Fordis, & Thompson  17 Uzark, Smith, Donohue, Yu, Afton, Norris, & Cotts  2015 The Journal of Pediatrics Assessment of transition readiness in adolescents and young adults with heart disease 18 Moola & Norman 2011 Child: Care, Health and Development ‘Down the rabbit hole’: enhancing the transition process for youth with cystic fibrosis and congenital heart disease by re-imagining the future and time  19 van Staa, Jedeloo, van Meeteran, & Latour 2011 Child: Care, Health and Development Crossing the transition chasm: Experiences and recommendations for improving transitional care of young adults, parents and providers  20 Fleck 2010 PhD Dissertation Correlates of Self-Care in Emerging Adults with Congenital Heart Disease  21 Clarizia, Chahal, Manlhiot, Kilburn, Redington, & McCrindle 2009 Canadian Journal of Cardiology Transition to adult health care for adolescents and young adults with congenital heart disease: Perspectives of the patient, parent and health care provider  22 Hilderson, Saidi, Van Deyk, Verstappen, Kovacs, Fernandes, Canobbio, Fleck, Meadows, Linstead, & Moons  2009 Pediatric Cardiology Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of American and Europe 23 Moons, Pinxten, Dedroog, Van Deyk, Gewillig, Hilderson, & Budts 2009 Journal of Adolescent Health Expectations and experiences of adolescents with congenital disease on being transferred from pediatric cardiology to an adult congenital heart disease program  24 Reid, Irvine, McCrindle, Sananes, Ritvo, 2004 Pediatrics Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with  32 # Author Year Journal/Website Title of Paper Siu, & Webb complex congenital heart defects  25 The New England Regional Genetics Network  2017 Genetics Education Materials for School Success (GEMSS) https://www.gemssforschools.org/conditions/marfan/default  https://www.gemssforschools.org/transition/transition-landing  26 Okamura & Williams  2015 Patient Safety Network (PS Net) for Agency for Healthcare Research and Quality https://psnet.ahrq.gov/webmm/case/348/transitions-in-adolescent-medicine  27 Marfan Foundation  2014 Marfan Foundation http://www.marfan.org/  28 Nationwide Children’s Hospital  2018 Nationwide Children’s Hospital https://www.nationwidechildrens.org/conditions/marfan-syndrome  https://www.nationwidechildrens.org/specialties/adolescent-and-adult-congenital-heart-disease-achd-program/services/congenital-heart-disease-transition-program  29 Seattle Children’s Hospital/ Research/ Foundation and the University of Washington Medical Center  2018 Seattle Children’s Hospital http://www.seattlechildrens.org/medical-conditions/chromosomal-genetic-conditions/marfan-syndrome/  http://www.seattlechildrens.org/clinics-programs/heart/treatments-and-services/adult-congenital-heart-disease/  30 UCLA Children’s Hospital  2018 UCLA Children’s Hospital http://heart.ucla.edu/clinical-services  https://www.uclahealth.org/Mattel/Adolescent-Transitional-Cardiac-Care-Program/  31 Provincial Health Services Authority 2018 B.C. Children’s Hospital http://www.bcchildrens.ca/our-research/research-focus/childrens-heart-centre  http://www.bcchildrens.ca/our-services/support-services/transition-to-adult-care      33 Timing of Publications  All of the academic and grey literature found on transition for both MFS and CHD were published between 2004 to 2018, despite imposing very few publication date limits on the literature. As depicted in Figure 3, the majority of literature (n = 21; 68%) was published between 2014 to 2018. There is an additional cluster of literature (n = 9; 29%) published between 2008 to 2011. All of the grey literature (n = 7) was dated from 2014 to 2018. Specific to the literature on MFS (n = 6; 19%), the research articles (n = 3) found were published between 2008 to 2010 and the grey literature (n = 3) between 2014 to 2017. For CHD (n = 25; 81%), the literature spanned from 2004 to 2018, with the majority published within the past four years. This literature search ended in May 2018; however, considering the increasing trend seen in Figure 3 in the past four years, I anticipate the number of publications will continue to increase.  The majority of the included publications focused on the transition process, and two particular studies also assessed the number of patients who successfully transitioned and transferred to adult care. The earliest study identified in this scoping review (Reid et al., 2004) focused on Canadian young adults with CHD, and found that only 47% of 360 patients successfully transferred to the adult care system and 27% had had no follow-up since they were 18 years old. Similarly, Bohun et al. (2016) found that in the United States, 34% successfully transferred, while 46% were lost to follow-up. The findings of these two studies highlighted a gap in the transition process.  34  Figure 3. Publications on transition, by condition and year.  Research Methods Employed  Out of the 31 total citations, 24 (77%) were original research studies that utilized various methodologies. The majority of these research studies were qualitative, utilizing thematic content analysis and grounded theory methodologies. Figure 4 displays the number of research studies found for each category.   Figure 4. Research studies, by research method. 012345672003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017 2018*Publicaions (N = 31)Publication YearMFS Research Articles MFS Grey Literature CHD Research Articles CHD Grey Literature024681012Qualitative Research Quantitative Research Mixed-Methods Research Systematic ReviewPublications (N = 24)Type of ResearchMFS Studies CHD Studies*Up to May 31, 2018  35 The type of grey publications retrieved is depicted in Figure 5.  Figure 5. Grey literature publications, by type.  Geographic Origin of Publications  The majority of the literature originated from North America (n = 20; 65%), specifically the United States and Canada. Figure 6 displays the number and type of literature for other geographic regions of publications.  Figure 6. Type of publications, by geographical location.  012345Foundation/ Network/ Association Website Hospital WebsitePublications (N = 7)Type of WebsiteMFS Literature CHD Literature0510152025North America Europe Asia More than 1 continentPublications (N = 31)Geographical Location (By Continent)MFS Research Articles MFS Grey Literature CHD Research Articles CHD Grey Literature 36 Perspective/Target Audience  As previously mentioned, the transition process involves youth, parents, and HCPs. A difference was found between the academic literature and the grey literature; the academic literature explored transition from the perspective of the youth, parent, or HCPs, whereas the grey literature was written for patients and families by HCPs. Figure 7 highlights the perspectives explored by the research literature and Figure 8 displays the target audiences of the grey literature.  Figure 7. Research publications, by perspective. Figure 8. Grey literature publications, by target audience. 01234Youth Parents Health CareProviders(HCPs)Youth &ParentsYouth & HCPs Parents &HCPsYouth &Parents &HCPsPublications (N = 7)Target AudienceMFS Grey Literature CHD Grey Literature024681012Youth Parents Health CareProviders(HCPs)Youth &ParentsYouth & HCPs Parents &HCPsYouth &Parents &HCPsPublications (N = 24)PerspectiveMFS Research Articles CHD Research Articles 37 Schumacher & Meleis’ Transition Framework  The retrieved literature was also examined and analyzed with respect to three categories of Schumacher and Meleis’ (1994) transition framework: universal properties of transition (each study’s definition of transition was examined); transition conditions (whether they were measured or discussed); and the indicators of healthy transition (whether each was measured or discussed). The findings are discussed in more depth in the following sections. Additionally, Appendix E provides an overview of these findings. Universal properties of transition – defining transition. Of the 31 citations, 27 records (87%) provided a definition of transition. Each of these 27 citations provided a unique definition of transition; however, nine (33%) of these records (Ekim et al., 2018; Stewart et al., 2017; While et al., 2017; Heery et al., 2015; Lopez et al., 2015; van Staa et al., 2011; Clarizia et al., 2009; Reid et al., 2004; Okamura & Williams, 2015) adopted the exact or similar definition of transition provided by Blum et al. (1993) who defines transition as “the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health-care systems” (p. 570). Figure 9 highlights the number of publications that defined transition.  Figure 9. Publications that defined transition.  051015202530Yes - Defined transition No - Did not define transitionPublications (N = 31)MFS Research Articles MFS Grey Literature CHD Research Articles CHD Grey Literature 38 Transition conditions. As previously mentioned, transition conditions are the factors that affect the transition process (Schumacher & Meleis, 1994). Figure 10 displays the number of both academic and grey publications that measured and/or discussed each of these conditions. The most commonly discussed condition was level of planning (n = 30; 97%); one article (While et al., 2017) did not measure this condition. Level of planning was measured or discussed in the literature as the activities or concepts that were identified as important to be done or addressed during the transition process. These included discussions about: transitioning to a new environment, family planning, insurance, and future education and vocational options; HCPs determining the most appropriate time to start the transition process and actually making the referral process; how pediatric and adult HCPs shared knowledge to provide a smooth transition; and whether the needs, such as access to resources or ability to self-care, were addressed before and during the transition. The second most commonly discussed transition condition was level of knowledge and skill (n = 23; 74%). These publications assessed whether youths had enough knowledge about their condition to know what to expect and knew how to self-manage their illness. These publications also assessed whether individuals knew about the adult health care system and how their care would be transferred. The systematic review by Heery et al. (2015) identified only one study that assessed the level of knowledge and skill of the adult HCPs in managing CHD. The environment, the third most frequently mentioned condition, (n = 19; 61%) measured levels of social support (e.g. family members, peers, partners, HCPs) available to the youth during the transition process. Figure 10 shows that a gap exists, as none of the publications assessed how the youth or parent thought the transition affected their life or the meaning they attributed to it.   39 Figure 10. Publications measuring/discussing transition conditions.  Indicators of healthy transition. In comparison to the number of publications that discussed the transition conditions, a limited number measured and/or discussed the indicators of healthy transition (n = 2; 6%) (Giarelli, Bernhardt, Mack, et al., 2008; New England Regional Genetics Network, 2017). Giarelli, Bernhardt, Mack, et al. (2008) defined successful transition as ‘becoming fit and fitting in’ and evaluated it qualitatively during their interviews with participants. “Becoming fit” was further identified as how the adolescent demonstrates illness-awareness, health-preservation, self-surveillance, and self-advocacy, whereas “fitting in” was indicated by whether the adolescent “fit with their chosen peer group and fit their limitations and strengths with expectations” of the condition (Giarelli, Bernhardt, Mack, et al. 2008, p.446). The New England Regional Genetics Network (2017) highlighted the steps towards a successful transition, which included considering the whole life of the person and addressing the needs of the individual, which may help him/her effectively cope with their condition and the transition. This site did not define indicators of success but did identify that transition involves moving towards independence in a way that makes sense for the individual, which means that the 05101520253035Meaning Expectations Level ofKnowledge/SkillEnvironment Level of Planning Emotional/PhysicalWell-beingPublications (Max. N = 31)Transition ConditionsMFS Research Articles MFS Grey Literature CHD Research Articles CHD Grey Literature 40 achievement of the skills and self-confidence to manage the transition is customized for each person (New England Regional Genetics Network, 2017). Thus, it can be seen that there is a gap in the literature, as these three indicators of successful and healthy transition identified by Schumacher and Meleis (1994) are rarely measured and/or discussed.  Figure 11. Publications measuring/discussing indicators of healthy transition.  Identified Themes  The findings from this scoping review can be further classified into four key components of the transition process from pediatric to adult health care systems: the need for knowledge and skills; the impact of parental involvement; preparing and planning for the future; and the transition readiness of the youth. Many of the publications discussed more than one theme. The following sections discuss the thematic findings in greater detail. Table 4 and Figure 12 summarize the identified themes.    0123Subjective Well-being Role Mastery Relationship Well-beingPublications (N = 31)Indicators of Healthy TransitionMFS Research Articles MFS Grey Literature CHD Research Articles CHD Grey Literature 41 Table 4.  Themes found in transition literature # Year Method/Source Need for Knowledge & Skill Parental Involvement Preparation and Planning for Future Transition Readiness 1 2008 Qualitative  X  X 2 2008 Qualitative X X X X 3 2010 Qualitative X    4 2018 Qualitative X X X X 5 2018 Quantitative    X 6 2017 Qualitative X X   7 2017 Quantitative X X  X 8 2017 Quantitative   X X 9 2017 Quantitative X X X X 10 2017 Quantitative  X X  11 2016 Quantitative X  X  12 2016 Qualitative X X X X 13 2016 Quantitative X  X  14 2015 Qualitative X X X X 15 2015 Systematic Review X X X X 16 2015 Qualitative X  X X 17 2015 Quantitative X X X  18 2011 Qualitative   X  19 2011 Qualitative X X X X 20 2010 Mixed-Methods X   X 21 2009 Mixed-Methods X X   22 2009 Quantitative X  X X 23 2009 Qualitative X X X  24 2004 Quantitative   X X 25 2018 Foundation/ Network/ Association Website X    26 2015 Foundation/ Network/ Association Website X    27 2014 Foundation/ Network/ Association Website X X   28 2018 Hospital Website X    29 2018 Hospital Website X    30 2018 Hospital Website X X   31 2018 Hospital Website X X X    42 Figure 12. Thematic focus in the literature.  Need for Knowledge and Skills  Twenty-five records (81%) highlighted that there is a need for knowledge and skills for youth during the transition process. The majority of the literature indicates that knowing about and understanding their condition and its trajectory can help to alleviate anxiety and uncertainty and promote a willingness to take responsibility for their own care and to seek treatment and follow-up when needed (Giarelli, Bernhardt, & Pyeritz, 2008; Chen et al., 2017; Stewart et al., 2017; Asp et al., 2015). Increasing knowledge was also found to promote higher self-efficacy and confidence, which as a result, improves self-management and their understanding of transitioning to adult care (Uzark et al., 2015; Harbison et al., 2016). The need for knowledge also relates to information about the new setting and about future plans, including educational/vocational choices and family planning (Bohun et al., 2016; Burström et al., 2016). Lopez et al.’s (2015) study further investigated the need for knowledge by exploring the preferred method of information delivery and found that desired information should be provided through different outlets, such as by HCPs or the internet. Bratt et al. (2018) also identified that the need for knowledge is also present for parents, who experience anxiety and insecurity 2517 1815051015202530Need for Knowledge & Skill Parental Involvement Preparation and Planningfor the FutureTransition ReadinessPublications (Max. N = 31)Themes Found in the Literature 43 without the appropriate information provided. In addition to many sources that discuss the importance of knowledge, one publication identified self-surveillance as an important skill during transition (Giarelli, Bernhardt, & Pyeritz, 2010). The individual must be able to self-monitor, collect information about his/her own problems, feelings, and needs, and decide the next course of action (Giarelli, Bernhardt, & Pyeritz, 2010).  Parental Involvement  Parental involvement was another common theme and was addressed by 17 (55%) of the publications. Many of the sources indicated that parental involvement is common during the transition process; however, there was a disagreement when it came to the impact of involving the parents during this time. Some publications indicated that parents can be beneficial to the transition process, as they helped improve their child’s self-management, ensured the transfer felt safe, and were considered by their child to be an expert source of information and skill (Giarelli, Bernhardt, Mack, et al., 2008; Giarelli, Bernhardt, & Pyeritz, 2008; Stewart et al., 2017; Burström et al., 2016; Asp et al., 2015). In contrast, three articles highlighted that parental involvement may be a barrier to transition, specifically to the youth’s transition readiness (While et al., 2017; Heery et al., 2015; Uzark et al., 2015). These studies indicated that parents may feel the need to control the care or become overprotective because they may not feel their child would be ready to take full responsibility. This overbearing nature may result in poorer psychological well-being, lower feelings of autonomy, feelings of dependency, and lower self-efficacy for the youth. The transition period is described as more difficult for the parents than for the youth, as it can be seen as a time of loss and a shift in roles (Burström et al., 2016; van Staa et al., 2011). Therefore, supporting the parents, facilitating the parent-youth interaction, and  44 encouraging open communication and sharing of knowledge was recommended as important for HCPs to consider during the transition process (Bratt et al., 2018; Chen et al., 2017).  Preparation and Planning for the Future  Eighteen citations (58%) identify that preparation and planning are crucial for a successful transition. The future was seen as uncertain terrain by adolescents (Moola & Norman, 2011); therefore, preparation is needed to prevent poor medication adherence, increased emergency care use, hospitalizations and death (Lopez et al., 2015). Information and education on future treatments, potential complications, the adult care organization and its team members, future follow-ups, high-risk behaviours, family planning, educational/vocational desires, and insurance coverage were considered as important in many of the studies (Bratt et al., 2018; Downing et al., 2017; Harbison et al., 2016). Investigators have also found that discussions with youth about transition and transfer to adulthood with HCPs and/or parents should begin at an early age and should include why and how the patient would be transferred (Stewart et al., 2017; Heery et al., 2015; Hilderson et al., 2009). With the potential to become overprotective, discussions with parents were recommended to include the impact of parental overprotection on their youth’s quality of life and how they can support their child’s need for greater autonomy (While et al., 2017). Having patients actively referred from pediatric to adult services and utilizing a gradual and systematic process to coordinate the care between them can help youth feel more informed, prepared, and secured, which can foster the shift to taking responsibility for self-care (Giarelli, Bernhardt, & Pyeritz, 2008; Bohun et al., 2016; Asp et al., 2015). Transition Readiness  The transition readiness of the youth is another theme in the transition literature and was found in 15 (48%) of the publications. An aspect of transition readiness discussed in many of the  45 publications was the timing of the transition process and the actual transfer. Older adolescents were found to be willing to have discussions about the transition and actually had discussions with their parents about it (Downing et al., 2017). They were also found to have higher transition readiness scores than those who did not have transition discussions, which may indicate they felt prepared and equipped to start the process (Stewart et al., 2017). Six studies identified that rather than solely considering chronological age, the developmental maturity of the individual, the mental readiness for discussions, and the acquisition of self-care abilities needs to also be considered (Bratt et al., 2018; Ekim et al., 2018; Asp et al., 2015; Heery et al., 2015; Lopez et al., 2015; van Staa et al., 2011;). A youth’s perception, beliefs, and focus were also found to impact their transition readiness. The individual may or may not believe in or want to understand their diagnosis or they may have a certain perception of when and where adult follow-up should be (Giarelli, Bernhardt, & Pyeritz, 2008; Reid et al., 2004). They may have other priorities, desires, and needs in their life that would be considered important to them during this time (Giarelli, Bernhardt, Mack, et al., 2008; Giarelli, Bernhardt, & Pyeritz, 2008; Chen et al., 2017; Burström et al., 2016). Understanding these wants from the perspective of the youth is important, as transition readiness can affect his/her initiative to take responsibility and perform self-care (Fleck, 2010). Summary of Results The 31 publications included in this scoping review consisted of 24 academic articles and seven websites. The trend of an increasing yearly number of publications suggests that more research will be produced in the coming years. Analysis of the publications using Schumacher and Meleis’ transition framework facilitated finding the key components that affect the transition process. These elements are crucial in ensuring a successful transition to adulthood.  46 Chapter 4: Discussion   In this scoping review, the range of evidence on the transition to adulthood in youth with MFS was mapped. A limited amount of both academic and grey literature was found on this specific topic, which identifies a gap in the existing literature. Expanding the review to include literature on the transition process in youth with CHD helped to determine where the gaps are in the MFS literature and what areas may need further research. It also identified key aspects of the transition process, which can easily be adapted to individuals with MFS, considering that both of these conditions require active life-long surveillance and care by a specialized multidisciplinary team.  Key Findings  Evolution of the Literature over Time  The academic and grey literature found on MFS was published between 2008 to 2010 and 2014 to 2017, which when compared to the current state of literature for CHD, is outdated. It is possible that the lack of current literature on this topic could be related to the integration of MFS and CHD, which many of the recent grey literature sources have begun to do. Websites owned by a children’s hospital, such as Nationwide Children’s Hospital (2018), Seattle Children’s Hospital (2018), UCLA Children’s Hospital (2018) and B.C. Children’s Hospital (Provincial Health Services Authority, 2018), highlight transition programs for individuals with CHD and lists MFS as one of the conditions they manage. The information provided on the transition process from these websites is therefore more generalized to include all patients with any chronic condition. The earliest published research study found on transitioning with MFS was from 2008. Considering that the exact cause of MFS has only been known since the early 1990s (Chaffins,  47 2007) and its rarity in society, it is not surprising how long it took for this first study to be published. Many of the publications found between 1991 and 2008 were about the clinical manifestations and the care and management of the condition (Backhouse, 2005; Ryan-Krause, 2002). Therefore, it is possible that many HCPs were just starting to learn about the condition and how to care for patients with it. Transition literature began to arise in the 1990s when advances in medical science, practices, and technologies were increasing the survival rates of children with chronic conditions (Blum et al., 1993). As knowledge on MFS grew, and with the improvement in technology, MFS was then considered as one of the complex, chronic conditions that requires life-long surveillance (Giarelli, Bernhardt, Mack, et al., 2008), and therefore, the transition process was seen as crucial to developing the skills needed to self-manage the condition.  An examination of both the MFS and CHD literature found that there was a difference in the focus and purpose of the research studies as the years evolved. The earliest study included in this review (Reid et al., 2004) investigated the number of Canadian young adults with CHD who successfully transitioned and transferred to adult care. Their research could have been considered as an assessment of the current transition situation and from their findings, it became evident that there was a gap in the transition process.  Starting in 2009, the purpose of the studies started to examine the transitioning experiences of these youth, which led to the identification of specific needs during this time. Researchers also started examining the perspectives of the parents and HCPs and how they impact the transition process.  Bohun et al.’s (2016) study, which also identified a gap in successfully transfer and follow-up, could have another assessment of the current situation with transitioning. Subsequent  48 studies continued to evaluate the needs and expectations of the evolving youth population to determine the underlying cause of this gap. As previously mentioned, the technological improvement over the years has significantly increased the life expectancy and survival rates of children and adolescents with CHD (Kovacs & McCrindle, 2014); therefore, considering these improved outcomes, the focus has shifted towards continuing this progression by aiming for a smooth transition between providers for a continuity of care for the patient. The increasing amount of publications within the last four years reflects this shift in focus.   All of the included literature on transitioning with MFS originated from the United States. As previously mentioned, MFS equally affects both men and women of all ethnicities (Marfan Foundation, 2014), so it is surprising that literature from other countries was not found. However, considering the delay between the confirmation of the cause of MFS to the first study on transitioning with MFS, and MFS’s rarity in North America, it is reasonable to assume this may be similar in other geographical regions. A Google Scholar search of MFS in Asia resulted in many articles that discussed the difference in clinical features between Western and Asian populations. Two studies found in this Google Scholar search by Yoo et al. (2010) and Franken et al. (2013) identified that early and proper diagnoses of MFS in Asian patients may be delayed due to subtle physical manifestations, which may result in reduced screening, poor recognition, and an inadequate understanding of MFS by both the general public and physicians in these countries. Although the transition process and MFS has received attention in North America, this may not be the case in other countries yet, as their current priorities may be focused on other aspects of the disease process.     49 Schumacher and Meleis’ 1994 Transition Framework  The use of the transition framework by Schumacher and Meleis (1994) helped to capture the extent to which the literature on MFS and CHD addressed some or all the components of this framework. Their framework identifies the universal properties of transition, which authors usually captured in their transition definitions. Blum et al.’s (1993) transition definition was commonly used in the literature; however, their definition only identified one property, that transition is a movement from one state to another. Other definitions in the literature may be preferable as they describe the change that occurs in a youth’s role, relationship, identity, or behavior. These definitions capture exactly how the transition impacts the youth.   Level of planning, level of knowledge and skill, and social support were the top three conditions that the studies attempted to measure. Although these three factors are considered important during the transition process, one condition that could also be considered significant (and which is included in Schumacher and Meleis’ (1994) framework) is the meaning of the transition to the youth. None of the publications measured what the transition meant to the individual or how it would affect their life. Understanding the meaning of this event for the young person could help determine where the transition to adult-centered care falls on their priority list.   Subjective well-being, role mastery, and relationship well-being were identified by Schumacher and Meleis (1994) as the indicators of healthy transition; however, many of the publications did not measure or identify these indicators. One study mentioned these three indicators, but did not expand further (Burström et al., 2016). Three articles indicated that successful transition and transfer was defined by attendance to follow-up appointments within a specific time frame after the transfer (Harbison et al., 2016; Heery et al., 2015; Reid et al., 2004).  50 One possible reason for the lack of these indicators in the literature is that they may be more challenging to measure, considering they are very subjective and unique to the individual.  Although this framework provided an approach to analyzing the literature, it was challenging to use, as many of the publications did not directly discuss the conditions or the indicators of transition. This resulted in my personal interpretations of the study’s findings.  Comparison of Themes   Four reoccurring themes emerged from the transition literature: (1) need for knowledge and skill; (2) parental involvement; (3) preparation and planning for the future; and (4) transition readiness. Although all four themes were found in the literature on MFS, two were emphasized more than the others: the need for knowledge and skill and transition readiness. The three articles on MFS and transition by Giarelli and her colleagues (Giarelli, Bernhardt, Mack, et al., 2008; Giarelli, Bernhardt, & Pyeritz, 2008; Giarelli, Bernhardt, & Pyeritz, 2010) revolved around the concept of transitioning to self-management, which, as previously mentioned, is a crucial skill to learn and develop during the transition process that facilitate the readiness of an individual. The need for knowledge and skill was emphasized by Giarelli et al. in both their 2008 and 2010 articles and by the three grey literature sources. This need was also emphasized in a similar way in the CHD literature.  All three websites included in this review also provided resources and information about MFS and about the transition process. Knowledge and skills help individuals to be informed and prepared for the next steps and reduce the fear of the unknown. An adequate level of knowledge and skill also promotes confidence and has been found to enhance adherence to medical recommendations due to a greater understanding of their condition’s trajectory. This is considered to be a step towards taking full responsibility for one’s condition.   51 Transition readiness was highlighted in both Giarelli, Bernhardt, Mack, et al. (2008) and Giarelli, Bernhardt, and Pyeritz (2008). Rather than considering only chronological age, readiness also entailed the willingness to understand their condition and consider it a priority in their life. The literature on MFS emphasized the idea of being mentally ready for the transition, but it did not consider the developmental maturity of the individual, which many of the CHD studies highlighted. One may conclude that developing mental readiness comes as one matures and a shift in priorities and perceptions occur.  These themes, the need for knowledge and skill and transition readiness, complement one another; therefore, it is not surprising that they were found together in the MFS literature. Knowledge helps to facilitate preparation, which helps patients develop self-care, self-advocacy, and decision-making skills during the transition process. (American Academy of Pediatrics et al., 2002). Development of skills and feelings of being prepared can enhance transition readiness.  Identified Gaps and Recommendations for Future Research Comparing the number of citations found for the transition to adulthood with Marfan Syndrome (MFS) to that of Congenital Heart Disease (CHD), it is evident that there is a gap in the literature on MFS. Additionally, these publications on MFS are older, which could be a barrier to understanding our current youth’s transition needs. It would be valuable to conduct a longitudinal research study that investigates the personal experiences of these individuals during the transition process, to provide a more current perspective on their needs and expectations and to help HCPs understand how they can support them more effectively.   Another gap in the literature is the lack of quantitative studies on transitioning with MFS. As previously mentioned, quantitative studies may help to establish relationships between two variables (Polit & Beck, 2017). Incorporating surveys, questionnaires, or scales into research  52 studies, such as the Transition-Q readiness scale, may help to provide more information on how certain aspects impact the transition process.   The current literature related to the transition process with MFS only focuses on the youth within North America and our present understanding is reflective of this. Differences in cultures, values, languages, and expectations of other geographical regions may create different views on transition or they may produce a unique transition experience for their young individuals. It would be valuable to understand how culture and ethnicity affect the transition process, to support the diverse population served by our health care system. An exploration of the transition experience and how it might be different between the genders, as well as the influence of socioeconomic status, would be useful in supporting individuals with MFS.   The literature on CHD highlights the importance of knowing and understanding one’s condition; becoming aware of the new environment, education and vocational planning, family planning, and insurance; and learning skills to be able to self-manage. However, one of the areas that is not identified in the CHD nor the MFS literature is addressing the concept of body image. Youth with CHD may have large sternotomy scars amongst other physical and cognitive issues; however, they may not be as obvious as in individuals with MFS who often have visible manifestations of their condition that may affect their self-esteem and self-confidence. This could lead to feelings of anxiety and anger and refusal to manage their condition. An area of further research could be evaluating how the physical manifestations of MFS affect the youth’s perception of themselves and how they prefer to be supported.    Considering that the internet is the most popular source of information for youth (Park & Kwon, 2018), I would expect that information websites on MFS would also provide resources on the transition process; however, there are evident gaps in these sources. Two websites on MFS  53 included in this review did provide a link to transition resources, but the information was generalized towards all chronic conditions. The Marfan Foundation (2014), recognized as a leading source of information on MFS, provides links to resources for the youth, their parents and HCPs; however, the actual document on transition for teens seems to require the provision of personal information before being able to access it. However, even though the document is actually fully accessible without signing up, by searching ‘transition’ in the search bar on the page, the challenge to gain access to this document is problematic. Clinic websites who identify their goals as coordinating follow-up care for the child as they transition to adulthood, such as Children’s Hospital Colorado (2018) and large associations, like Genetic Aortic Disorders Association (GADA) Canada (2015) and Rare Diseases Foundation (2013), do not provide any information on the transition process. Similarly, a website tailored towards kids, teens, parents, and educators provides information on MFS, but again, nothing on transition (KidsHealth, 2014). It becomes even more problematic when smaller, lesser-known sites on MFS refer to these larger associations for more information.  Implications for Nursing Current literature has identified nurses, in collaboration with other HCPs, as key players in ensuring a successful transition in various, well-studied chronic conditions. However, their role in the transition of patients with MFS has not been thoroughly explored. The limited awareness and knowledge about MFS and the transition needs of this particular group can also create barriers to effectively supporting youths and their families and therefore, nurses are in a prime position to begin bridging this gap.  Nurses need an understanding of the disease and the transition process to be confident in providing the necessary psychological and emotional supports for the individual and his/her  54 family. As previously mentioned, the transition to adult health care can be a frightening and uncertain time for both youths and their families (Joly, 2015). A contributor to this anxiety is the lack of information on both the condition and the transition process (Morsa, Gagnayre, Deccache, & Lombrail, 2017; Rutishauser, Sawyer, & Ambresin, 2014). Nurses must be knowledgeable and competent in both of these to be able to comfortably and confidently engage in the conversations that may arise, particularly with this age group. They must also be able to empathize with their patients and build trusting relationships with them. It is through this collaborative partnership that a successful and healthy transition can occur. In addition to educating young individuals about their condition, how to manage it, and about the importance of treatment adherence (Betz, 1998), nurses are well-positioned to develop, implement, and deliver transition programs or processes that address the needs of this particular population. They can assess the youths’ readiness to transition and develop an individualized plan that utilizes the strengths of the youths to target specific gaps and needs, such as knowledge gaps and the development of self-care skills (Betz, 1998). With a well-planned transition, nurses are positioned to encourage young individuals to begin taking responsibility in managing their condition, empower them to become more active in the decision-making process, and allow their parents to transition to a more supportive role. Nurses can also educate other HCPs on the transition process for youths with MFS. Using knowledge translation strategies, nurses can organize in-services or presentations on MFS and the transition needs of this population. They can become leaders in ensuring that patients and their families receive the highest standard of care for a successful transition from child to adult care.    55 Knowledge Translation Determining the appropriate audience and adapting the message to that audience are the key steps for disseminating this review’s findings (Canadian Institute of Health Research, 2016). Interested stakeholders may include: HCPs working with patients with MFS, like those at B.C. Children’s Hospital, St. Paul’s Hospital or other specialized clinics across Canada, the Genetic Aortic Disorders Association Canada, and the Marfan Foundation.   Disseminating this scoping review’s findings at the local level at B.C. Children’s and St. Paul’s Hospital will be my first focus. I will contact the Cardiology Nurse Clinician at BC Children’s and inform her of this review. If the HCP team at B.C. Children’s Hospital is interested in learning more about the literature related to the transition process to adulthood in MFS, a presentation to summarize the findings of this review can be developed and provided. A similar process for disseminating the findings of this review can be applied to the HCPs at St. Paul’s Hospital. These HCPs can use the evidence compiled to develop new transition programs or tailor existing ones to address the crucial components/concepts of the transition process, as knowledge on the transition process with MFS may be limited. This review may assist in bridging the knowledge gap and provide HCPs with the evidence required to ensure a successful transition. A manuscript could be prepared for publication in an international journal, such as the Congenital Heart Disease Journal.  For patients and their families, the findings of this scoping review may be translated into a presentation at MFS conferences or information sessions for patients with MFS, or packaged into an infographic to be displayed at congenital heart clinics. Highlighting the gaps that exist in the current literature may spark an interest in patients and families to participate in future studies  56 on this topic. They may have unanswered questions or new ideas that would be worth investigating, and would, therefore, expand our knowledge on the transition process even further.  Similarly, this review may give other stakeholders or researchers of the transition process an idea of what areas require further research. It can be used as a starting point for the conduct of a systematic review or larger research studies, such as a qualitative description of the experience of patients with MFS on during transition. Limitations  A limitation to this review is the exclusion of non-English literature. During the search process, articles written in French and German were excluded. Although some of the publications included in this scoping review were based in Europe and Asia, there may have been components of the transition process only mentioned in non-English articles; therefore, including other languages would have been beneficial, though not feasible.  Secondly, the use of only a single theoretical framework of transition to analyze the literature limits the findings of this scoping review. However, conducting a thematic analysis separate from the framework analysis should have uncovered findings that may not have been apparent by only using the transition framework. Use of the framework was challenging as many of the studies/publications did not directly discuss the categories of the framework, which potentially resulted in a biased interpretation of the researcher’s findings. Utilizing multiple transition frameworks may be beneficial to provide a wider, comprehensive view on the process and reduce the risk of personal bias.  Conclusion  This scoping review provides a map of the literature on the transition process from childhood to adulthood in patients with MFS. With the limited literature on the MFS, this  57 scoping review also maps the literature on CHD, which is acceptable, considering many recent sources amalgamate MFS with CHD. It is evident that there is a significant gap in the literature on the transition process in youth with MFS. Although the current information on transitioning with CHD does overlap for those with MFS, there are other aspects that patients with MFS may consider more important to be addressed during this time which are not as emphasized in CHD, such as body image. Therefore, there is a need to conduct further research in this specific area to ensure that youth with MFS successfully transition to adulthood. With advancing technology and rising numbers of children and adolescents with congenital conditions surviving into adulthood, it is important to shift our attention to making the transition process simple, smooth, and stress-free. Youth are the future of our world and helping youth with MFS maximize their potential and function, as any other person in society, can be beneficial for the generations to come.    58 References American Academy of Pediatrics, American Academy of Family Physicians, & American College of Physicians-American Society of Internal Medicine. (2002). A consensus statement on health care transitions for young adults with special health care needs. Pediatrics, 110(6), 1304-1306. Arksey, H. & O’Malley, L. (2005). Scoping studies: Towards a methodological framework. International Journal of Social Research Methodology, 8(1), 19-32. doi: 10.1080/1364557032000119616 Asp, A., Bratt, E., & Bramhagen, A. (2015). Transfer to adult Care—experiences of young adults with congenital heart disease. Journal of Pediatric Nursing, 30(5), e3-e10. doi: 10.1016/j.pedn.2015.05.025 Backhouse, R. (2005). Nursing considerations for people with Marfan syndrome. Nursing Times, 101(2), 32-35. Betz, C. L. (1998). Adolescent transitions: A nursing concern. Pediatric Nursing, 24(1), 23.  Blum, R. W., Garell, D., Hodgman, C. H., Jorissen, T. W., Okinow, N. A., Orr, D. P., & Slap, G. B. (1993). Transition from child-centered to adult health-care systems for adolescents with chronic conditions: A position paper of the society for adolescent medicine. Journal of Adolescent Health, 14(7), 570-576. doi:10.1016/1054-139X(93)90143-D Bohun, C. M., Woods., P., Winter, C., Mitchell, J., McLarry, J., Weiss, J. & Broberg, C. S. (2016). Challenges of intra-institutional transfer of care from pediatric to adult congenital  59 cardiology: The need for retention as well as transition. Cardiology in the Young, 26, 327-333. doi: 10.1017/S1047951115000220 Bratt, E. L., Burström, Å., Hanseus, K., Rydberg, A., & Berghammer, M. (2018). Do not forget the parents—Parents' concerns during transition to adult care for adolescents with congenital heart disease. Child: Care, Health and Development, 44(2), 278-284.  doi:10.1111/cch.12529 Burström, Å., Bratt, E., Frenckner, B., Nisell, M., Hanséus, K., Rydberg, A., & Öjmyr-Joelsson, M. (2017). Adolescents with congenital heart disease: Their opinions about the preparation for transfer to adult care. European Journal of Pediatrics, 176, 881-889. doi: 10.1007/s00431-017-2917-9 Burström, Å., Öjmyr-Joelsson, M., Bratt, E., Lundell, B., & Nisell, M. (2016). Adolescents with congenital heart disease and the parents: Needs before transfer to adult care. The Journal of Cardiovascular Nursing, 31(5), 399-404. doi: 10.1097/JCN. 0000000000000288 Canadian Institutes of Research. (2016). Knowledge translation. Retrieved January 30, 2018, from Government of Canada website: http://www.cihr-irsc.gc.ca/e/29418.html Chaffins, J. A. (2007). Marfan syndrome. Radiologic Technology, 78(3), 222. Chen, C., Su, W., Chiang, Y., Shu, Y., & Moons, P. (2017). Healthcare needs of adolescents with congenital heart disease transitioning into adulthood: A Delphi survey of patients, parents, and healthcare providers. European Journal of Cardiovascular Nursing, 16(2), 125-135. doi: 10.1177.1474515116643622  60 Children’s Hospital Colorado. (2018). Marfan syndrome clinic. Retrieved from https://www.childrenscolorado.org/doctors-and-departments/departments/heart/programs-and-clinics/marfan-syndrome-clinic/ Clarizia, N. A., Chahal, N., Manlhiot, C., Kilburn, J., Redington, A. N., & McCrindle, Brian W. (2009). Transition to adult health care for adolescents and young adults with congenital heart disease: Perspectives of the patient, parent and health care provider. Canadian Journal of Cardiology, 25(9), S317-S322. doi:10.1016/S0828-282X(09)70145-X Clark, W. (2014). Delayed transitions of young adults. Retrieved July 27, 2018, from Statistics Canada website: https://www150.statcan.gc.ca/n1/pub/11-008-x/2007004/10311-eng.htm Downing, K. F., Oster, M. E., & Farr, S. L. (2017). Preparing adolescents with heart problems for transition to adult care, 2009–2010 national survey of children with special health care needs. Congenital Heart Disease, 12(4), 497-506. doi:10.1111/chd.12476 Dray, E. M. & Marelli, A. J. (2016). Chapter 1: Changing epidemiology of congenital heart disease. In M. Schwerzmann, C. Thomet, & P. Moons (Eds.), Congenital heart disease and adolescence (pp. 3-14). Switzerland: Springer. Ekim, A., Kolay, M., & Ocakci, A. F. (2018). Readiness for transition from pediatric to adult care for adolescents with chronic heart disease. Journal for Specialists in Pediatric Nursing, 23(1), n/a. doi:10.1111/jspn.12199 Faivre, L., Collod‐Beroud, G., Adès, L., Arbustini, E., Child, A., Callewaert, B., . . . Jondeau, G. (2012). The new Ghent criteria for Marfan syndrome: What do they change? Clinical Genetics, 81(5), 433-442. doi:10.1111/j.1399-0004.2011.01703.x  61 Fleck, D. (2010). Correlates of self-care in emerging adults with congenital heart disease: A mixed-methods study (Doctoral dissertation). Retrieved from http://www.library.ubc.ca/ Franken, R., den Hartog, A. W., van de Riet, L., Timmermans, J., Scholte, A. J., van den Berg, Maarten P, . . . Mulder, B. J. M. (2013). Clinical features differ substantially between Caucasian and Asian populations of Marfan syndrome. Circulation Journal, 77(11), 2793-2798. doi:10.1253/circj.CJ-13-0584 Fusar-Poli, P., Klersy, C., Stramesi, F., Callegari, A., Arbustini, E., & Politi, P. (2008). Determinants of quality of life in Marfan syndrome. Psychosomatics, 49(3), 243-248.  Genetic Aortic Disorders Association Canada (GADA). (2015). Marfan syndrome. Retrieved March 11, 2018, from http://www.gadacanada.ca/marfan-syndrome/ Giarelli, E., Bernhardt, B. A., Mack, R., & Pyeritz, R. E. (2008). Adolescents’ transition to self-management of a chronic genetic disorder. Qualitative Health Research, 18(4), 441-457. doi: 10.1177.1049732308314853 Giarelli, E., Bernhardt, B. A., & Pyeritz, R. E. (2008). Attitudes antecedent to transition to self-management of a chronic genetic disorder. Clinical Genetics, 74(4), 325-337. doi:10.1111/j.1399-0004.2008.01052.x Giarelli, E., Bernhardt, B. A., & Pyeritz, R. E. (2010). Self-surveillance by adolescents and young adults transitioning to self-management of a chronic genetic disorder. Health Education & Behavior, 37(1), 133-150. doi:10.1177/1090198109331670  62 Gorter, J. W., Stewart, D., & Woodbury‐Smith, M. (2011). Youth in transition: Care, health and development. Child: Care, Health and Development, 37(6), 757-763. doi:10.1111/j.1365-2214.2011.01336.x Gray, W. N., Schaefer, M. R., Resmini-Rawlinson, A., & Wagoner, S. T. (2018). Barriers to transition from pediatric to adult care: A systematic review. Journal of Pediatric Psychology, 43(5), 488-502. doi:10.1093/jpepsy/jsk142 Harbison, A. L., Grady Jr, S., Chi, K., & Fernandes, S. M. (2016). Provision of transition education and referral patterns from pediatric cardiology to adult cardiac care. Pediatric Cardiology, 37(2), 232-238. doi:10.1007/s00246-015-1267-5 Heery, E., Sheehan, A. M., While, A. E., & Coyne, I. (2015). Experiences and outcomes of transition from pediatric to adult health care services for young people with congenital heart disease: A systematic review. Congenital Heart Disease, 10(5), 413-427. doi:10.1111/chd.12251 Hilderson, D., Saidi, A. S., Van Deyk, K., Verstappen, A., Kovacs, A. H., Fernandes, S. M., . . . Moons, P. (2009). Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of America and Europe. Pediatric Cardiology, 30(6), 786-793. doi:10.1007/s00246-009-9442-1 Joanna Briggs Institute. (2015). Methodology for JBI scoping reviews. Joanna Briggs Institute Reviewers’ Manual 2015. Retrieved January 30, 2018, from http://joannabriggs.org/assets/docs/sumari/Reviewers-Manual_Methodology-for-JBI- Scoping-Reviews_2015_v2.pdf  63 Joly, E. (2015). Transition to adulthood for young people with medical complexity: An integrative literature review. Journal of Pediatric Nursing – Nursing Care of Children & Families, 30(5), 91-103. doi: 10.1016/j.pedn.2015.05.016  KidsHealth. (2014). Marfan syndrome. Retrieved May 2, 2018, from https://kidshealth.org/en/kids/marfan.html Kovacs, A. H., & McCrindle, B. W. (2014). So hard to say goodbye: Transition from paediatric to adult cardiology care. Nature Reviews. Cardiology, 11(1), 51. doi:10.1038/nrcardio.2013.172 LeComte, K., Sinclair, B., Cockell, S., Iacoe, E., Gillespie, A., & Human, D. (2016). Ensuring a successful transition and transfer from pediatric to adult care in patients with congenital heart disease. BC Medical Journal, 58(7), 389-395.  Levac, D., Colquhoun, H., & O’Brien, K. K. (2010). Scoping studies: Advancing the methodology. Implementation Science, 5(1), 1-9. doi: 10.1186/1748-5908-5-69 Lopez, K. N., Karlsten, M., Bonaduce De Nigris, F., King, J., Salciccioli, K., Jiang, A., . . . Thompson, D. (2015). Understanding age-based transition needs: Perspectives from adolescents and adults with congenital heart disease: Transition needs in congenital heart disease. Congenital Heart Disease, 10(6), 561-571. doi:10.1111/chd.12283 Lugasi, T., Achille, M., & Stevenson, M. (2011). Patients’ perspective on factors that facilitate transition from child-centered to adult-centered health care: A theory integrated metasummary of quantitative and qualitative studies. Journal of Adolescent Health, 48(2011), 429-440. doi: 10.1016/j.jadohealth.2010.10.016  64 Marfan Foundation (2014). Teens and Marfan syndrome. Retrieved May 2, 2018, from http://www.marfan.org/resources/patients/teens Marfan Foundation (2014). What is Marfan syndrome?. Retrieved January 30, 2018, from https://www.marfan.org/about/marfan McCabe, N., Dunbar, S. B., Butler, J., Higgins, M., Book, W., & Reilly, C. (2015). Antecedents of self-care in adults with congenital heart defects. International Journal of Cardiology, 201, 610-615. doi:10.1016/j.ijcard.2015.08.125 Moola, F. J., & Norman, M. E. (2011). ‘Down the rabbit hole’: Enhancing the transition process for youth with cystic fibrosis and congenital heart disease by re‐imagining the future and time. Child: Care, Health and Development, 37(6), 841-851. doi:10.1111/j.1365-2214.2011.01317.x Moons, P., Pinxten, S., Dedroog, D., Van Deyk, K., Gewillig, M., Hilderson, D., & Budts, W. (2009). Expectations and experiences of adolescents with congenital heart disease on being transferred from pediatric cardiology to an adult congenital heart disease program. Journal of Adolescent Health, 44(4), 316-322. doi:10.1016/j.jadohealth.2008.11.007 Mora, M. A., Sparud-Lundin, C. Bratt, E., & Moons, P. (2017). Person-centered transition programme to empower adolescents with congenital heart disease in the transition to adulthood: A study protocol for a hybrid randomised controlled trial (STEPSTONES project). BMJ Open, 7(4), 1-11.doi:10.1136/bmjopen-2016-014593  Morsa, M., Gagnayre, R., Deccache, C., & Lombrail, P. (2017). Factors influencing the transition from pediatric to adult care: A scoping review of the literature to conceptualize a  65 relevant education program. Patient Education and Counselling, 100(2017), 1796-1806. doi: 10.1016/j.pec.2017.05.024 Nationwide Children’s Hospital. (2018). Congenital heart disease transition program. Retrieved May 2, 2018, from https://www.nationwidechildrens.org/specialties/adolescent-and-adult-congenital-heart-disease-achd-program/services/congenital-heart-disease-transition-program New England Regional Genetics Network (2017). Marfan syndrome at a glance. Retrieved May 2, 2018, from Genetics Education Materials for School Success website: https://www.gemssforschools.org/conditions/marfan/default Niwa, K. (2015). Adults with congenital heart disease transition. Current Opinion in Pediatrics, 27(5), 576. doi:10.1097/MOP.0000000000000270 Okamura, M. J. & Williams, R. G. (2015). Transitions in adolescent medicine. Retrieved May 2, 2018, from Patient Safety Network Agency for Healthcare Research and Quality website: https://psnet.ahrq.gov/webmm/case/348/transitions-in-adolescent-medicine Park, E., & Kwon, M. (2018). Health-related internet use by children and adolescents: Systematic review. Journal of Medical Internet Research, 20(4), e120. doi:10.2196/jmir.7731 Peter, K.F, Kong, F., Horne, R., Francomano, C.A., & Biesecker, B.B. (2001). Living with Marfan syndrome: I. Perceptions of the condition. Clinical Genetics, 60, 273-282.  66 Pham, M. T., Rajić, A., Greig, J. D., Sargeant, J. M., Papadopoulos, A., & McEwen, S. A. (2014). A scoping review of scoping reviews: Advancing the approach and enhancing the consistency. Research Synthesis Methods, 5(4), 371-385. doi:10.1002/jrsm.1123 Polit, D. F. & Beck, C. T. (2017). Nursing research: Generating and assessing evidence for nursing practice (10th ed.). Philadelphia, PA: Wolters Kluwer.  Provincial Health Services Authority. (2018). Transition to adult care. Retrieved May 2, 2018, from http://www.bcchildrens.ca/our-services/support-services/transition-to-adult-care Pyeritz, R. E. (2000). The Marfan syndrome. Annual Review of Medicine, 51(1), 481-510. doi:10.1146/annurev.med.51.1.481 Queen’s Printer for British Columbia. (2018). Age of majority act [RSBC 1996] chapter 7. Retrieved June 2, 2018, from the Government of British Columbia website: http://www.bclaws.ca/civix/document/id/lc/statreg/96007_01 Rare Diseases Foundation. (2013). Marfan syndrome. Retrieved June 2, 2018, from https://www.rarediseasefoundation.org/wiki/marfan_syndrome Reid, G. J., Irvine, M. J., McCrindle, B. W., Sananes, R., Ritvo, P. G., Siu, S. C., & Webb, G. D. (2004). Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with complex congenital heart defects. Pediatrics, 113(3), e197-e205. doi:10.1542/peds.113.3.e197 Rosen, D. S., Blum, R. W., Britto, M., Sawyer, S. M., Siegel, D. M., & Society for Adolescent Medicine. (2003). Transition to adult health care for adolescents and young adults with  67 chronic conditions: Position paper of the society for adolescent medicine. Journal of Adolescent Health, 33(4), 309-311. doi:10.1016/S1054-139X(03)00208-8 Rutishauser, C., Sawyer, S. M., & Ambresin, A. (2014). Transition of young people with chronic conditions: A cross-sectional study of patient perceptions before and after transfer from pediatric to adult health care. European Journal of Pediatrics, 173(8), 1067-1074. doi:10.1007/s00431-014-2291-9 Ryan-Krause, P. (2002). Identify and manage Marfan syndrome in children. The Nurse Practitioner, 27(10), 31-36.  Sable, C., Foster, E., Uzark, K., Bjornsen, K., Canobbio, M. M., Connolly, H. M., . . . on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, Council on Clinical Cardiology, and Council on Peripheral Vascular Disease. (2011). Best practices in managing transition to adulthood for adolescents with congenital heart disease: The transition process and medical and psychosocial issues: A scientific statement from the American heart association. Circulation, 123(13), 1454-1485. doi:10.1161/CIR.0b013e3182107c56 Sanders, R. A., Kuo, D. Z., Levey, E., Cheng, T. L. (2009). Transitioning adolescents to adult care and adulthood: Is it time yet?. Contemporary Pediatrics, 26(11), 46-52. Sawyer, S. M., Blair, S., & Bowes, G. (1997). Chronic illness in adolescents: Transfer or transition to adult services? Journal of Pediatrics and Child Health, 33(2), 88-90. doi: 10.1111/j.1440-1754.1997.tb01005.x  68 Scal, P. (2002). Transition for youth with chronic conditions: Primary care physicians' approaches. Pediatrics, 110(6), 1315-1321. Schneider, M. B., Davis, J. G., Boxer, R. A., Fisher, M., & Friedman, S. B. (1990). Marfan syndrome in adolescents and young adults: Psychosocial functioning and knowledge. Journal of Developmental and Behavioral Pediatrics: JDBP, 11(3), 122-127. doi:10.1097/00004703-199006000-00005 Schumacher, K. L. & Meleis, A. I. (1994). Transitions: A central concept in nursing. Image—the Journal of Nursing Scholarship, 26(2), 119-127. doi: 10.1111/j.1547-5069.1994.tb00929.x Seattle Children’s Hospital/Research/Foundation & the University of Washington Medical Center. (2018). Adult congenital heart disease. Retrieved May 2, 2018, from http://www.seattlechildrens.org/clinics-programs/heart/treatments-and-services/adult-congenital-heart-disease/ Sivasankari, T., Mathew, P., Austin, R. D., & Devi, S. (2017). Marfan syndrome. Journal of Pharmacy and Bioallied Sciences, 9, 73-77. doi: 10.4103/jpbs.JPBS_326_16 Stewart, K. T., Chahal, N., Kovacs, A. H., Manlhiot, C., Jelen, A., Collins, T., & McCrindle, B. W. (2017). Readiness for transition to adult health care for young adolescents with congenital heart disease. Pediatric Cardiology, doi:10.1007/s00246-017-1580-2 Syndrome. (2018). In Merriam-Webster.com. Retrieved June 2, 2018, from https://www.merriam-webster.com/dictionary/syndrome  69 Thomet, C., Schwerzmann, M., & Greutmann, M. (2015). Transition from adolescence to adulthood in congenital heart disease—Many roads lead to Rome. Progress in Pediatric Cardiology, 39(2), 119-124. doi:10.1016/j.ppedcard.2015.10.012 Transition. (2018). In Merriam-Webster.com. Retrieved June 2, 2018, from https://www.merriam-webster.com/dictionary/transition UCLA Children’s Hospital. (2018). Adolescent transitional cardiac care program. Retrieved May 2, 2018, from https://www.uclahealth.org/Mattel/Adolescent-Transitional-Cardiac-Care-Program/ Uzark, K., Smith, C., Donohue, J., Yu, S., Afton, K., Norris, M. & Cotts, T. (2015). Assessment of transition readiness in adolescents and young adults with heart disease. The Journal of Pediatrics, 167(6), 1233-1238. doi: 10.1016/j.jpeds.2015.07.043 Uzark, K., & Wray, J. (2018). Young people with congenital heart disease — transitioning to adult care. Progress in Pediatric Cardiology, 48, 68-74. doi:10.1016/j.ppedcard.2018.01.010 van Staa, A. L., Jedeloo, S., van Meeteren, J., & Latour, J. M. (2011). Crossing the transition chasm: Experiences and recommendations for improving transitional care of young adults, parents and providers. Child: Care, Health and Development, 37(6), 821-832. doi:10.1111/j.1365-2214.2011.01261.x Van Tongerloo, A., & De Paepe, A. (1998). Psychosocial adaptation in adolescents and young adults with Marfan syndrome: An exploratory study. Journal of Medical Genetics, 35(5), 405-409. doi:10.1136/jmg.35.5.405  70 Velvin, G., Bathen, T., Rand-Hendriksen, S., & Geirdal, A. Ø. (2015). Systematic review of the psychosocial aspects of living with Marfan syndrome. Clinical Genetics, 87, 109-116. doi: 10.1111/cge.12422 Warnes, C. A., Williams, R. G., Bashore, T. M., Child, J. S., Connolly, H. M., Dearani, J. A., . . . Webb, G. D. (2008). ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American college of Cardiology/American heart association task force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease): Developed in collaboration with the American society of echocardiography, heart rhythm society, international society for adult congenital heart disease, society for cardiovascular angiography and interventions, and society of thoracic surgeons. Circulation, 118(23), e714-e833. doi:10.1161/CIRCULATIONAHA.108.190690 While, A. E., Heery, E., Sheehan, A. M., & Coyne, I. (2017). Health-related quality of life of young people with long-term illnesses before and after transfer from child to adult healthcare: Transfer of young people from child to adult healthcare. Child: Care, Health and Development, 43(1), 144-151. doi:10.1111/cch.12410 World Health Organization. (2018). Adolescent health. Retrieved July 27, 2018, from http://www.who.int/topics/adolescent_health/en/ Yoo, E., Woo, H., Ki, C., Lee, H., Kim, D., Kang, I., . . . Kim, J. (2010). Clinical and genetic analysis of Korean patients with Marfan syndrome: Possible ethnic differences in clinical manifestation. Clinical Genetics, 77(2), 177-182. doi:10.1111/j.1399-0004.2009.01287.x    71 Appendix A Search Strategy for Marfan Syndrome and Transition Source Search Strategy Results Total CINAHL (Marfan Syndrome OR (MH "Marfan Syndrome") OR "marfan syndrome") AND (Transition OR (MH "Continuity of Patient Care+") OR "transition")  2 266           (Marfan Syndrome OR (MH "Marfan Syndrome") OR "marfan syndrome") AND (self-manag* or self-care or self-regulat* or self-monitor* or transition* OR self N1 manag* OR self N1 (care caring) OR self N1 regulat* OR self N1 monitor * OR transition* OR (MH "Self Care"))  10 (Marfan Syndrome OR (MH "Marfan Syndrome") OR "marfan syndrome") AND (psychosocial factors OR (MH "Attitude to Change") OR (MH "Attitude to Health+") OR (MH "Attitude to Illness+") OR (MH "Attitude to Life") OR (MH "Patient Attitudes") OR (MH "Uncertainty") OR (MH "Empowerment") OR (MH "Decision Making, Ethical") OR (MH "Family Functioning+") OR (MH "Family Relations+") OR (MH "Interpersonal Relations+") OR (MH "Life Change Events+") OR (MH "Life Experiences+") OR (MH "Psychosocial Aspects of Illness+"))  33 (Marfan Syndrome OR (MH "Marfan Syndrome") OR "marfan syndrome") AND (adolescents or teenagers or young adults OR (MH "Young Adult") OR (MH "Adolescence+") OR (MH "Adolescent, Hospitalized") OR "adolescents or teenagers or young adults")  221 Medline (OVID) (Marfan Syndrome OR marfan* syndrome OR Marfan Syndrome/) AND (self-manag* or self-care or self-regulat* or self-monitor* or transition* OR self adj1 manag* or self adj1 (care or caring) or self adj1 regulat* or self adj1 monitor* OR self care/ or self-management/)  46 1890 (Marfan Syndrome OR marfan* syndrome OR Marfan Syndrome/) AND (Transition OR exp "continuity of patient care"/ or transition to adult care/)  41 (Marfan Syndrome OR marfan* syndrome OR Marfan Syndrome/) AND (Adolescent* OR adolescence OR young adult* or teenager* OR Adolescent Behavior/ OR Adolescent health services/ OR Adolescent/ OR Adolescent Health OR Psychology, Adolescent/ OR Adolescent Development/ OR Adolescent Medicine/)  1803 CAB Direct (Marfan Syndrome OR Marfan’s Syndrome) AND (Transition*)  1 98 (Marfan Syndrome or Marfan’s Syndrome)  97 Web of Science (Marfan’s syndrome OR Marfan syndrome) AND (transition* OR "self-monitoring" OR "self manag*" OR "self care" OR self-care OR "self regulat*)  64 218 (Marfan's syndrome or Marfan syndrome) AND (youth OR adolescen* OR "young adult" OR teen*)  154  72 Google Scholar (Marfan Syndrome OR Marfan’s Syndrome) AND (transition* OR transition to adult care)  6* 6 Google (marfan syndrome AND transition) OR (intitle:"marfan syndrome" OR intitle:"marfan's syndrome" AND "transition") OR (“marfan syndrome” OR “marfan’s syndrome” AND “transition”) 25* 25 TOTAL 2,503 Note. Hand searched results are not included in this table.  *Only the relevant results were included in the count for Google Scholar and Google    73 Appendix B Search Strategy for Congenital Heart Disease and Transition Source Search Strategy Results CINAHL  Filters: -Age: Adolescents: 13-18 years -Publication Date: 2003-2018 -Language: English (Congenital Heart disease OR (MH "Heart Defects, Congenital+")) AND ((self-manag* or self-care or self-regulat* or self-monitor* or transition* OR self N1 manag* OR self N1 (care caring) OR self N1 regulat* OR self N1 monitor * OR transition*) OR (MH "Self Care")) AND ((adolescent or adolescence or teen or teenager or young adult) OR (MH "Adolescence+") OR (MH "Adolescent, Hospitalized") OR (MH "Adolescent Parents+") OR (MH "Adolescent Health Services") OR (MH "Young Adult"))  114 Medline (OVID)  Filters: -Language: English -Publication date: 2003-2018 -Humans only -Age: Adolescents 13-18 years  ((Congenital Heart Disease) OR (Heart Defects, Congenital/)) AND ((adolescent* or adolescence or young adult* or teenager*) OR (Adolescent health services/ OR Adolescent Health/)) AND (Transition OR exp transition to adult care/) 97 CAB Direct  Filters:  -Language: English -Humans only -Unable to filter date due to limited number of studies found – Date range: 1928-2018  (Congenital heart disease) AND (Transition*) 20 Web of Science  Filters: -Language: English -Publication Date: 2003-2018  (Congenital heart disease) AND (Transition*) AND (Adolescent* or adolescence or teen* or teenager* or young adult*) 173 TOTAL 404 Note. Hand searched results are not included in this table.   74 Appendix C Research Literature Findings # Author, Title Journal Year Purpose Findings  Themes 1 Giarelli, Bernhardt, Mack, & Pyeritz  Adolescents’ transition to self-management of a chronic genetic disorder  Qualitative Health Research 2008 To explain the socially complex process by which parents transfer, and children take on, the responsibility for managing a chronic genetic disorder The core concept for an adolescent transitioning to self-management with a chronic disorder depended on how the individual dealt with the need to be fit and fit in with one’s social group or “the need to integrate continually and actively the facts of one’s chronic disorder with the expectations for one’s life” (p. 446). Five psychosocial processes were identified to be related to this core concept: shifting perception (making visible that which is invisible); shifting orientation (changing focus and goal-directed activities from present orientated to future oriented); shifting ownership (taking self-responsibility and relieving another of the responsibility for maintaining and guarding their health); shifting reasoning (changing of problem solving and conflict resolution from competitive to cooperative); and shifting sphere (moving from simply being a member of a family that might or might not have other affected members, to a member of a larger network or community of people with shared interests).   -Recognizing the idea of becoming fit (recognizing limitations and how to be healthy) and fitting in (social groups).  -Transition readiness -> how the individual dealt with the need to be fit and fit in with one’s social group or “the need to integrate continually and actively the facts of one’s chronic disorder with the expectations for one’s life” (p. 446) 2 Giarelli, E., Bernhardt, B.A. & Pyeritz, R.E.   Attitudes antecedent to transition to self-management of a chronic genetic disorder  Clinical Genetics 2008 To examine systemic factors that influence transition to self-management (TSM) Prior to effective transition to self-management (TSM), three major attitudes were identified by children, parents, and HCPs as necessary: belief in the diagnosis, wanting to understand the cause and effect of MFS, and willing to share responsibility in problem solving. Factors that influenced the TSM were psychosocial, highly modifiable, and largely attainable through direct influence by the HCPs in the context of the patient-parent-HCP relationship. Health care system-centered factors that created obstacles were lack of formal transitioning services, lack of coordinated care between pediatric and adult services, and scarcity of comprehensive centers for adults with MFS. Six recommendations came from patients, parents, and HCPs: to specify acceptable and unacceptable activities, discuss when to act on symptoms, discuss future needs, encourage questioning, discuss career choices, and engage child in decision-making.    -Provision of knowledge  -Systematic approach to transfer of care -> preparation and planning  -Understand adolescent’s priorities to create an effective treatment partnership and not to over- or underestimate adolescent’s level of autonomy -> Transition Readiness 3 Giarelli, Bernhardt, & Pyeritz  Self-surveillance by adolescents and young adults transitioning to self-management of a chronic genetic disorder  2010 To describe the self-surveillance activities, skills that are the foundation of self-management, performed by adolescents and young adults with MFS.   To answer (a) What are the self-surveillance activities that are performed by Self-management of a chronic disorder involves an individual’s self-directed participation in lifelong self-surveillance and care in their health and healing. It relies on the acquisition of certain behaviors such as recognizing physical activity limitations, learning about the purpose of treatments, and being aware and sensitive to symptoms that signify the worsening of the disease. The idea of self-surveillance is defined as the “day-to-day attention to and interpretation of evidence gathered by the patient pertaining to his or her diagnosis, signs of disease, and responses to treatment” (p.133). There are two types of surveillance: -Self-surveillance also includes self-focus, self-awareness, self-consciousness, self-monitoring, self-attention, and self-regulation -> knowledge and skill  -Shifting perception – emerging self-awareness or the ability to perceive and understand that-which-is-not apparent with respect to the chronic illness.  -the adolescent monitored his/her own psychological and social well-being in relation to the effect of having MFS on  75 # Author, Title Journal Year Purpose Findings  Themes Health Education & Behavior individuals with MFS? and (b) How do people with MFS use the information from self-surveillance as they TSM? planned, which involves participation in medical screening, monitoring, and following treatment guidelines; and incidental (self). The self-surveillance activities that were identified by the participants were grouped into 4 thematic categories: tracking phenotype (monitoring appearance, physical fitness, medical problems, and making other observations); tracking medical care (monitoring and appraising the use, timing, changes to, and physical/health effects of medical treatments or procedures provided by HCPs); tracking behavior (monitoring one’s daily choices of behavior and interactions with others in context of one’s awareness of MFS); and tracking emotions (monitoring of the emotional impact of having MFS and how emotions are affect by thinking about one’s diagnosis from day-to-day). The tracking phenotype theme dominated self-surveillance behaviors. Gender and age influenced self-surveillance behaviors with females and older participants being more engaged in self-monitoring activities.   his/her own behavioral choices and emotions.  -Before self-advocacy and self-care occur, one may be able to collect accurate info about one’s own problems, feelings, and needs on which to base judgments or decisions, which is self-surveillance -> knowledge and skill  -Instructions for self-monitoring need to be tailored to age of the child and his/her cognitive level and ability -> transition readiness 4 Bratt, E. L., Burström, Å., Hanseus, K., Rydberg, A., Berghammer, M.  Do not forget the parents – Parents’ concerns during transition to adult care for adolescents with congenital heart disease  Child: Care, Health and Development 2018 To explore parents’ expectations and needs during their adolescent’s transition to adult care Two main themes emerged with five subcategories.  The first theme was “feeling secure – the importance of being prepared and informed” with the three subcategories, “being involved, prepared, and informed”; “bring assured that the adolescents get essential information”; and “preferred information channels and methods”.  Parents wished to be involved in the transition planning. They had to learn adaptation to a new role and hand over responsibility to the adolescent. They wanted to be prepared and informed beforehand of any plans. They heard very little about the ACHD organizations and wanted more information on future follow-ups including what was adult care, how to get there, the ACHD care organization, team members, and the frequency of future medical follow-up. Lack of information caused anxiety and insecurity. Parents also wanted their adolescent to have information about managing daily life with CHD, current and future treatment, potential complications, and about smoking, alcohol, contraceptives, sex, and pregnancy. They felt the nurse or pediatric cardiologist were the best people to provide this education. Parents emphasized that presentation of the information and preparation should be appropriate to their level of maturity. Receiving information in group meetings with other parents and adolescents would be beneficial.   The second theme was “the process of knowing when it is time to hand over” with two subcategories, “start of transition process and shift in roles” and “the process of handling over responsibility”.  Parents felt the appropriate age to start the process was 15-16, depending on maturity. Transferring should be decided based on maturity rather than chronological age. Parents expressed grief that they were not longer being a parent to a child, but rather to -Support the parents’ role during transition – awareness of shifting roles -> Parental involvement  -Preparation and information provision is important during the transition. -> knowledge and skill  -Well-informed and self-assured parents are better equipped to pass on their knowledge and experience in order to support and improve the adolescent’s self-management skills (Ford et al., 2011) -> preparation  -Knowledge helps to ease anxiety -> knowledge and skill  -Information provided to adolescents should address CHD-related issues linked to an adolescent’s daily life-situation.  -Have a written healthcare transition plan – structured transition planning with input from parents contributes to supporting autonomy and self-care management of the adolescent  -Transition readiness -> Transferring should be decided based on maturity rather than chronological age  76 # Author, Title Journal Year Purpose Findings  Themes an adult. Close to none of the parents had handed over all contact with the HCP.  5 Ekim, A., Kolay, M., & Ocakci, A. F.   Readiness for transition from pediatric to adult care for adolescents with chronic heart disease  Journal for Specialists in Pediatric Nursing     2018 To determine transition readiness of adolescents with chronic heart disease to adult care The study found a positive relationship between age and Transition-Q total mean score, meaning that self-management skills of adolescents improve with age. Communication skills of adolescents improved as they became older, but there was still a lack of confidence in communicating with HCPs.  -Consider age and developmental maturity for disease management skills -> Transition readiness  -Focus on skills when assessing transition readiness 6 Burström, Å., Bratt, E., Frenckner, B., Nisell, M., Hanséus, K., Rydberg, A., & Öjmyr-Joelsson, M.  Adolescents with congenital heart disease: Their opinions about the preparation for transfer to adult care  European Journal of Pediatrics  2017 To explore what adolescents with congenital heart disease view as important in the preparation for the transfer to adult care. One main category emerged from the data, becoming a manager of the condition, and four subcategories: Sufficient knowledge about the health, be a participant in the care, Parental support, and Communicating with others about the health. -Sufficient knowledge needs to be provided about their health status  -Parental support is important  7 Chen, C., Su, W., Chiang, Y., Shu, Y., & Moons, P.  Healthcare needs of adolescents with congenital heart disease transitioning into adulthood: A Delphi survey of patients, parents, and healthcare providers  European Journal of Cardiovascular Nursing 2017 To identify the healthcare needs of adolescents with CHD transitioning into young adults by collecting the perspectives of patients, parents, and healthcare providers. Using the Delphi survey, the researchers found that adolescents/young adults, their parents, and HCPs identified 25 healthcare needs during the transition to adulthood: 8 related to health, 2 for family, 5 individual, 2 interpersonal interaction, and 8 on policy dimensions that were classified as important.   Health dimension (1, 5, and 7 were deemed highly valued): 1) provide more info about illness and clarify doubts promptly; 2) simultaneously explain the condition to the patient and family caregiver; 3) encourage the patient to learn health self-management; 4) discuss the important and methods of weight control; 5) increase the patient’s knowledge about the course of the disease; 6) discuss the progression, prevention, and management of symptoms with the patient; 7) teach the patient how to recognize symptoms, and 8) discuss the significance and important of regular follow-up.  -Encouraging self-management of health condition and cultivating a positive attitude toward the illness were deemed most important.  -HCPs should focus on the healthcare needs of young people rather than focusing on a timely transfer and a structured transition process -> Transition readiness  -Not being lost to follow-up is essential to achieving successful transition   -Provision of knowledge is important  -Parental involvement – encouraging parent-child interaction  77 # Author, Title Journal Year Purpose Findings  Themes Family dimension: 1) make an effort to facilitate parent-child interaction, such as communicating worries; and 2) encourage parents to assist children in learning about self-care.  Individual dimension (1, 3, and 4 were highly valued): 1) cultivate a positive attitude toward the illness; 2) improve self-assessment and self-control abilities and engage in a physical activity that enhances health; 3) encourage timely communication with teachers and provide information about the disease, such as instructions about medications, exercise, and work; 4) encourage timely communication with employers and provide information about the disease; and 5) maintain a sense of personal privacy.  Interpersonal interaction dimension: 1) appreciate empathy from others; and 2) value the companionship of going to the doctor.  Policy dimension (1, 3, and 6 were highly valued): 1) establish feasible referral and follow-up system; 2) set up individualized medical records; 3) shorten health service gap between urban and rural areas; 4) establish a network resource; 5) provide a health passport; 6) notify patient and parents of the results of applying for catastrophic illness cards; 7) simplify process of obtaining that card; and 8) provide individualized health info.  Dissimilar concerns were also present among the health, family and policy dimensions between the three groups.  8 Downing, K. F., Oster, M. E., & Farr, S. L.   Preparing adolescents with heart problems for transition to adult care, 2009-2010 national survey of children with special health care needs  Congenital Heart Disease 2017 To (1) examine national prevalence of transition planning among Children with special health care needs (CSHCN) ages 12-17 years with heart problems compared to CSHCN without and (2) examine factors associated with transition planning for CSHCN with heart problems using the 2009-2010 National Survey for Children with Special Health Care Needs (NS-CSHCN) data The study found that a quarter of adolescents with heart problems had parents who discussed with providers their child’s eventual need to shift to adult care and health insurance as an adult. Less than half had parents who discussed with providers their child’s healthcare needs as an adult. However, 88% of adolescents had providers that encouraged them to take responsibility for their own care. Factors associated with transition planning included age, having a medical home, and the number of comorbidities. Older adolescents had parents who discussed their child’s shift to an adult HCP compared to younger adolescents. Adolescents with heart problems and more additional comorbidities were less likely to have parents who discussed shifting to an adult provider and adult health care needs compared to those with no comorbidities. Discussion of needs were more prevalent in those whose activities were greatly impacted by their condition.   -Transition preparation and planning should include education on condition, maintenance, follow-up recommendations, vocational planning and insurance information -> preparation and planning  -Age of adolescent when beginning discussions; older adolescents had parents who discussed their child’s shift to an adult HCP compared to younger adolescents -> transition readiness 9 Stewart, K. T., Chahal, N., Kovacs, A. H., Manlhiot, C., Jelen, A., Collins, T., & McCrindle, B. W.  2017 To evaluate transition readiness, medical condition knowledge, self-efficacy, and illness uncertainty in young adolescents (ages 12 to 15 years) with congenital The study found that 24% of patients recalled discussing transferring with a HCP and 41% of patients recalled discussing this with a parent. Older patients were more likely to have discussed transfer with their health care team, scored higher in transition readiness and knowledge scores. Significant correlations were found between sociodemographic, medical, -Older adolescents were more likely to discuss transfer with their health care team, had higher transition readiness scores  -Discussion of transition needs to occur to facilitate transition readiness   78 # Author, Title Journal Year Purpose Findings  Themes Readiness for transition to adult health care for young adolescents with congenital heart disease  Pediatric Cardiology heart disease (CHD).  To identify socio-demographic, medical, patient, and parental factors associated with transition readiness. patient, and parental factors with patients’ transition readiness scores. An increase in transition readiness score was correlated with age, patient medical condition knowledge, and patient’s self-efficacy, and not disease severity or uncertainty of medical condition. Self-management scores were higher when parents recalled speaking to their child about transfer, when parents recalled a HCP discussing transfer with them, when the patient recalled either their parent or HCP discussing transfer with them. Low transition readiness scores were found in young adolescent males and those currently taking cardiac medications.  -Readiness is associated with age, more knowledge, and patient’s self-efficacy.  -Lack of cardiac knowledge and self-management skills may result in loss to follow-up -> knowledge and skills  -Transition programs need to focus on patient and family with emphasis on education and preparation for self-managed care to guide successful transfer.  -Parental involvement can help children transition  10 While, A. E., Heery, E., Sheehan, A. M., & Coyne, I.  Health-related quality of life of young people with long-term illnesses before and after transfer from child to adult healthcare  Child: Care, Health and Development 2017 To examine health-related quality of life pre- and post-transfer from child to adult healthcare for young people with three long-term illnesses This study examined young people with cystic fibrosis, congenital heart defects, and/or diabetes using the HRQoL questionnaire, KIDSCREEN-27, and the ‘overprotection’ scale of the Parental Bonding Instrument (PBI). Scores from the HRQoL questionnaire between pre- and post-transfer groups indicate that physical well-being was significantly lower in the post-transfer group and social support and peers dimension had a lower mean in the post-transfer group.  Some of the results provided were specific to CF or DM.  Post-transfer group reported lower overall psychological well-being than their pre-transfer counterparts. Females reported lower psychological well-being than males. Maternal overprotection was also associated with poorer psychological well-being, lower overall scores on autonomy and parent relation dimension, lower overall scores on the social support and peers dimension.    -Parental overprotection may be detrimental to the transition process  -Parental preparation for transition should include discussion on the impact of parental overprotection on their child’s quality of life in order encourage and help parents to support their child’s need for greater autonomy  -Gender plays a role in the transition process 11  Bohun, C. M., Woods, P., Winter, C., Mitchell, J., McLarry, J., Weiss, J., & Broberg, C. S.   Challenges of intra-institutional transfer of care from pediatric to adult congenital cardiology: The need for retention as well as transition  Cardiology in the Young 2016 To define the number of patients who were transferred successfully and to identify factors associated with successful transfer to adult care providers versus failure to transfer in CHD patients who were eligible to transfer Based on the assessment of follow-up, each patient was categorized into 3 groups: Group A – patients who were successfully transferred to adult congenital heart disease service, defined by attending at least one adult clinic visit; Group B – patients who continued to be seen by pediatric care providers, but not adult provides; and Group C – patients not seen by any provider and considered “lost to follow-up”. Thirty-four percent transferred successfully to an adult congenital cardiologist (Group A), 21% continued to be seen by pediatric cardiologist (Group B) and 46% were lost to follow-up (Group C). Group A patients had more complex and moderate CHD, were more likely to have insurance, and had been referred to the adult congenital heart disease service. For group C, only 5.8% of these patients were able to be contacted and the researchers found that the patients had simple to complex CHD, were insured, employed, and had other medical problems in addition to CHD. Some had moved residence and became further away from tertiary-care centers.  -Need to education patients and facilitate their healthcare into the adult setting – education should include issue of insurance challenges, educational/vocational choices   -Patients who were actively referred were more likely to transfer successfully – no difference in successful transfer by age, gender, or race  -Severity of the condition determines likelihood of transfer – adults with simple CHD were least likely to transfer to adult CHD care providers   -Main barriers identified to referring patients eligible for transfer are attachment of a clinician to the patient/family and lack of qualified adult CHD care provider.  -Need formalized transition program    79 # Author, Title Journal Year Purpose Findings  Themes 12 Burström, Å., Öjmyr-Joelsson, M., Bratt, E., Lundell, B., & Nisell, M.  Adolescents with congenital heart disease and the parents: Needs before transfer to adult care  The Journal of Cardiovascular Nursing    2016 To identify and describe the needs of adolescents with congenital heart disease and their parents during the transition before transfer to adult cardiac healthcare. Three categories emerge from the analysis of the interview data from the adolescents: change of relationships, knowledge and information, and daily living. The authors depicted the meaning of these categories as: Safety and control, indicating needs of continuity, knowledge, and taking responsibility. For the parents, two categories emerged: change of relationships and daily living. One of the key findings was the importance for parents and the adolescents to end the relationship with the pediatric team before moving to the adult team. They wished to see the new HCPs before actually transferring. Adolescents did not see themselves as different compared to their peers and were used to the limitations.  -Provision of knowledge is important – about the new setting, about their condition, about future plans (i.e. pregnancy and contraception)  -Building relationships with the providers brought a sense of trust  -Parental involvement – sense of loss for the parent, adolescents expressed confidence in their parents’ expertise  -Adolescents want more control in their lives – taking more responsibility 13 Harbison, A. L. Grady Jr, S., Chi, K., & Fernandes, S. M.   Provision of transition education and referral patterns from pediatric cardiology to adult cardiac care  Pediatric Cardiology 2016 To describe the transition and transfer practices for a cohort of young adults with pediatric onset heart disease (POHD) and to determine factors associated with successful transfer to adult-oriented cardiac care Through retrospective chart reviews, the researchers found that for the provision of transition education, majority of the charts documented providing education on the underlying cardiac disease and a little over half documented education about follow-up and current medications. Education about exercise recommendations/limitations, dental prophylaxis/hygiene and symptoms requiring assessment were less documented and documentation on high-risk behaviors, risk of pregnancy or impact of disease on offspring and career/education was even less noted.  Majority of the subjects had documentation that they should transfer to adult cardiac care. Of those, 68% presented for follow-up in adult clinic within 2 years of their pediatric clinic appointment. The ones who successfully transition had cardiac surgery, were on cardiac medications, and their frequency of follow-up was less than a year.  -Provision of knowledge is important – children with more knowledge even simply about their diagnosis also demonstrated better understanding of their transition to adult care  -Transition education needs to be consistent  -Engaging patients and families to take charge of their health using available resources through the web  -coordinated process to guide patient from pediatric to adult care would be beneficial 14 Asp, A., Bratt, E., & Bramhagen, A.   Transfer to adult care – experiences of young adults with congenital heart disease  Journal of Pediatric Nursing 2015 To explore the experiences of young adults with congenital heart disease in Sweden after transfer from pediatric cardiac clinic to the adult congenital heart disease clinic. Five themes emerged from the data: feeling secure during the transfer process, experiencing trust in the care, expecting to be involved, assuming responsibility for one’s health is a process, and lack of knowledge leads to uncertainty.   Feeling secure during the transfer process relates to feelings of security through being informed in advanced and feeling prepared for the transfer. Details of how the transfer would be carried out, which doctor they would meet, where they would go for future check-ups, and who to contact should be provided.  Experiencing trust in the care was related to the familiarity of the environment and the team members. Continuity was a prerequisite for trust in one’s doctor. Confirming their worry and fear also increased trust in the care. Expecting to be involved emphasized importance of being involved and prepared before changes are implemented, i.e. shifting towards self-responsibility. -Provision of information about the transfer process  -Parental support helped ensure the transfer felt safe  -Involvement in care or shifting to being more self-responsible depends on the cognitive maturity of the individual  -Need to feel involved in their care  -Knowledge about their condition is important – helped to provide a certainty when seeking care on their own.  -Encourage young people to play an active role from an earlier age   -Collaboration between pediatric and adult care setting is needed   80 # Author, Title Journal Year Purpose Findings  Themes Assuming responsibility for one’s health is a process describes how the informants became aware of their change caused by the transfer in terms of taking responsibility for their condition.  Lack of knowledge leads to uncertainty describes how lacking knowledge about their medical history and its implications on everyday life can affect an individuals’ willingness to take responsibility and seek treatment for their CHD.    -Structured, gradual, and prepared transfer process helps ensure adolescents to shoulder the responsibility for self-care for their CHD in connection with transferring to adult clinic.   15 Heery, E., Sheehan, A. M., While, A. E., & Coyne, I.   Experiences and outcomes of transition from pediatric to adult health care services for young people with congenital heart disease: A systematic review  Congenital Heart Disease 2015 To examine the outcomes and experiences of transfer and transition from pediatric to adult care for young people with congenital heart disease through a systematic review. This systematic review found that only two outcomes have been examined in relation to transfer from child to adult health care settings in patients with CHD: loss to follow-up and lapse of care. Many studies identified that a specialized ACHD service was needed to ensure a successful transfer. Other indicators of successful transition included: referral by pediatric cardiologist, and medical history (more pediatric surgeries, more comorbidities). Parental involvement was both a risk and a protective factor for loss to follow-up.  Seven papers were found to explore the experiences of transfer and transition. Three dominant themes emerged: (1) transition awareness and readiness; (2) knowledge and expertise; and (3) divergent approaches to care. Parental concerns about transition was about the belief that their child would not be ready to take full-responsibility. Barriers to transfer included: emotional attachment of parents and patients to pediatric cardiologist; presence of emotional/cognitive delay, and attachment of the clinician to the patient/family; lack of adult providers with expertise in CHD -Importance of transition preparation – early discussions about need for lifelong care is important  -Family support was valued  -Parental involvement considered a barrier to transition readiness – parents had divergent expectations of care leading to conflicts over control of care, role confusion, and dissatisfaction with care  -Collaboration between pediatric and adult cardiologist  -Provision of knowledge -> more knowledge about their care equal more likely to feel confident in their ability to look after themselves and resulted in greater independence from parents in navigating health system; knowledge about adult services beforehand  -HCPs also need to be knowledgeable about CHD  -Young people valued greater involvement in decision making in adult services  -Structured transition process and education for patient on the implications of their cardiac condition, and the differences between pediatric and adult care  -Flexibility in timing of transfer – transfer should occur when patient is medically stable and has necessary skills to cope in an adult service -> transition readiness  16 Lopez, K. N., Karlsten, M., Bonaduce De Nigris, F., King, J., Salciccioli, K., Jiang, A., Marelli, A., Kovacs, A. H., Fordis, M., & Thompson, D.   Understanding age-based transition needs: Perspectives from adolescents and adults with congenital heart disease  2015 To explore the perceived transition needs of adolescents (pretransition) and transition experiences of adults (post transition) with moderate or severe CHD.  To define needs and delivery method for an adolescent transition curriculum. The study interviewed both adolescents before transition and adults after transition.  The study found that a majority of adolescent respondents (70%) reported having a good understanding of their condition including the type of heart defect and the treatment they had received. Almost half indicated that they had enough information for managing their condition; however, the participants were split when asked if they would have liked to receive more information. Fifty percent expressed concerns of managing their condition into adulthood and these concerns were related to: understanding life expectancy, the impact of CHD on work/career options, learning about health insurance, going to medical checkups and visits on their own, dealing with medical emergencies and explaining their condition to new doctors when -Inadequate transition may result in poor medication adherence, increased emergency care use, hospitalizations, and death  -Understanding the long-term impact of CHD (need for future surgeries), career options given their CHD, and the risk of early unemployment would be beneficial.  -Provision of knowledge  -Connecting with other individuals with CHD can be useful in identifying informational needs and having emotional support   -assessment of adolescent maturity and readiness for discussion and implementation of transfer  81 # Author, Title Journal Year Purpose Findings  Themes Congenital Heart Disease transitioning. Female adolescents expressed some concern about understanding the impact of CHD on pregnancy and childbirth. Preferred methods of receiving information included: directly from the conversation with their doctor, internet, or via text. Sixty percent indicate that talking to an adult with CHD to help understand adult life with CHD would be helpful or very helpful and 90% indicated they would be interested in interacting with other adolescents with CHD either in person or through social media.  For the adults after transition, the study found 45% wished they had more information because they lacked awareness about the severity and long-term implications of their condition, and/or not being ready to process some of the information because of their age. They wished for more information about their condition, treatment, severity, and causes, and long-term issues. Topic areas that would have been helpful to learn in adolescence included information about exercise, nutrition, and lifestyle. Discussing the impact of CHD on health insurance, future careers, and work planning would have been beneficial. Education on how to make appointments, manage medications, talk to HCPs, pregnancy and birth control,  and mental health and managing stress were also considered important. Sources of information considered valuable were talking directly to physicians and the internet.  Both groups identified that an online checklist on transition would be helpful to 1) support transition as parents become less involved in care; 2) manage emergency situation with doctors who are not familiar with the diagnosis; 3) increase awareness of importance of follow-up care for parents and children; 4) self-monitor; 5) set expectations for the future to be able to plan around them; and 6) learn about the importance of lifestyle choices.    -Information on the condition and transition should be available through different outlets – physicians, internet, text, videos, and presentations  -A checklist tool may facilitate to close the gap in CHD knowledge and improve the transition process. 17 Uzark, K., Smith, C., Donohue, J., Yu, S., Afton, K., Norris, M. & Cotts, T.  Assessment of transition readiness in adolescents and young adults with heart disease  The Journal of Pediatrics 2015 To evaluate the transition readiness, including perceived knowledge deficits, self-efficacy, and self-management behaviors, in 13 to 25-year-olds with congenital heart disease or heart transplant.  To examine the relationship between transition readiness assessment, information seeking behavior, and quality of life (QOL)  The study found that adolescents under the age of 18 had more knowledge deficits than those older and that self-efficacy and self-management behavior scores were higher as adolescents got older. Areas of knowledge deficits were related to health insurance, pregnancy, contraception, how to contact your doctor, and symptoms to call for. Knowledge deficits were correlated with self-efficacy and self-management; higher perceived self-efficacy scores were associated with higher self-management scores. No correlation was found between psychosocial functioning scores and self-management.  -Provision of knowledge is important – lack of knowledge about their heart disease was a barrier to taking a more active role in decisions about their treatment and care.  -Greater knowledge = great self-efficacy or confidence in their ability to care for themselves, among patients with CHD< promoting self-management behaviors crucial to successful transitioning   -Parental overprotection may foster feelings of dependency and low self-efficacy 18 Moola, F. J. & Norman, 2011 To determine how The study resulted in a few themes for both CF and CHD, but -Future planning – career, education, family planning   82 # Author, Title Journal Year Purpose Findings  Themes M. E.  ‘Down the rabbit hole’: enhancing the transition process for youth with cystic fibrosis and congenital heart disease by re-imagining the future and time  Child: Care, Health and Development  adolescents with CF and CHD negotiate and embody developmental time and the future in order to better understand the transition process and concerns associated enhance the capacity to manage complex transition concerns.  To explore how young people with CF and CHD – and their parents – understand their health in the future and the perspectives they bring towards the concept of time.   only one theme was relevant to CHD. The theme, the future as an uncertain terrain, included careers aspirations, educational goals, development of romantic relationships, travel, and the concerns about how their condition would affect their future.  19  van Staa, A. L., Jedeloo, S., van Meeteran, J., & Latour, J. M.   Crossing the transition chasm: Experiences and recommendations for improving transitional care of young adults, parents and providers  Child: Care, Health and Development 2011 To map experiences with the transfer to adult care of young adults with chronic conditions.  To identify recommendations for transitional care of young adults, their parents, and healthcare providers. The researchers studied the transfer practices in seven chronic conditions, one of those is CHD. The interview data indicated that timing of transition depending on the adolescents’ developmental readiness and not fixed aged, but only one chronic disease department tested knowledge and self-management skills.   Two themes emerged related to the process of ‘moving on to adult care’: leaving pediatric care is a logical step and transition is complicated by cultural gaps between pediatric and adult services. Parents have more difficulty leaving trusted pediatric environment than young adults and young adults are tired of being ‘patronized’ by pediatric staff and parents. Parents and young adults who had frequent contact with the pediatric HCP found it difficult to establish trust and familiarity with the new staff, but this was temporary. The environment of both settings was considered as different and parents and young adults identified they were not prepared for the difference. Parents found it difficult to step aside, shift responsibility and management to their child, and gain trust in their capacity. The researchers recommended that better patient and parent preparation and better organization and communication between pediatric and adult care is needed for better transition.   -different views on age of transfer – age boundaries should be flexible, depending on adolescent and his/her parents   -Preparation for patient and parent  -Collaboration between pediatric and adult are – having more staff exchange, holding consultation meetings about patients before and after transition and setting up joint clinics  -More information at an earlier stage  -More time to make choices  -Need more knowledge about their condition and should improve self-management skills  -Transition should be incorporated in a comprehensive, lifespan perspective on healthcare  -Shift in roles 20 Fleck, D. (PhD Dissertation)  Correlates of Self-Care in Emerging Adults with Congenital Heart Disease   2010 To identify corelates of self-care in CHD.   To describe how emerging adults with CHD perform self-care.  The study found 12 major findings: “1) self-care confidence is influential in self-care; 2) the individual’s developmental stage is influential in self-care, suggesting that self-care is a developmental task; 3) participants’ specific understanding of CHD and associated therapies is more helpful than general knowledge of CHD; 4) cognitive function and depression did not influence self-care; 5) similarities exist between self-care and transition; 6) self-care is expressed inconsistently, is an iterative process, and is enhanced with experience and skill development; 7) responsibility is shared for a period of time; 8) definitions of self-care differed between providers and participants 9) - Transition readiness -> the individual’s developmental stage is influential in self-care, suggesting that self-care is a developmental task; readiness enhances self-care  -Knowledge promotes understanding -> participants’ specific understanding of CHD and associated therapies is more helpful than general knowledge of CHD  83 # Author, Title Journal Year Purpose Findings  Themes participants used CHD to their advantage to avoid peer pressure; 10) number of repairs rather than complexity was influential in the performance of self-care; 11) readiness enhances self-care; and 12) families are influential in the performance of self-care”.    21 Clarizia, N. A., Chahal, N., Manlhiot, C., Kilburn, J., Redington, A. N., & McCrindle, B.W.   Transition to adult health care for adolescents and young adults with congenital heart disease: Perspectives of the patient, parent and health care provider  Canadian Journal of Cardiology 2009 To examine the attitudes and opinions regarding transitions and its needed preparation from the perspectives of patients, their parents, and health care providers in a pediatric cardiology outpatient clinic. Patients demonstrated a basic understanding of their heart condition (74%); however, there was a gap in knowledge, with only 36% showing a clear understanding of the implications and changes association with their future transition to adult care. A total of 43% of patients expressed a desire for more information about their heart condition.   Parents (86%) were aware that their child would transition to adult care. Almost half (41%) expressed concerns about the transition and only 50% felt their child was ready. Parents were found to be very involved in their child’s care, with 95% of parents accompanying their child to medical appointments, 68% staying with them the entire visit, and 45% administering their medications. Parents were unsure and unclear about their role during this transition preparation, but 81% felt that the institution was preparing their child for the transition.  HCPs (69%) felt that, currently, children were not sufficiently prepared for transition. They thought that children should start learning about transition between 13 and 16 years old (72%). Physicians were found to be more involved in teaching about diagnosis and symptoms than nurses.   An association between a child’s knowledge, self-advocacy in the health care setting, and his/her understanding of the implications of transition was observed. Children with more knowledge about their heart condition were more likely to respond to providers’ questions themselves and demonstrate an understanding of the implications of transition to adult care. Parents who indicated that their child was ready to take responsibility were more likely to let their child answer providers’ questions and discuss their child’s heart condition in medically relevant situations.  Physicians saw that a lack of a formal transition program was a problem, while nurses indicate that parental involvement can be a barrier.  -Knowledge -> children with more information were more likely to respond to providers’ questions  -Parental Involvement   22 Hilderson, D., Saidi, A. S., Van Deyk, K., Verstappen, A., Kovacs, A. H., Fernandes, S. M., Canobbio, M. M., Fleck, D., Meadows, A., Linstead, R., & Moons, 2009 To describe the current practice of transfer and transition of children with congenital heart disease in the U.S. and European countries.  Within the 51 centers (out of 69) that transfer all or some of their patients from pediatric to adult-focused care, the transfer is optional at 25 centers and mandatory at 26 centers. The mandatory transfers occur because of the pediatric cardiologist’s policy, hospital policy, and governmental regulations. Three transfer destinations are observed: 41 centers transfer to a formalized Adult Congenital Heart Disease (ACHD) program, 8 -Timely transfer to adult-focused care is important.   -Transfer depends on intellectual and emotional maturity of the patient and acquisition of necessary skills to function at an adult clinic  -Important for parents and patients to learn that facilities that  84 # Author, Title Journal Year Purpose Findings  Themes P.  Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of American and Europe  Pediatric Cardiology To explore the attitudes of pediatric cardiologists regarding the transfer and transition of patients with congenital heart disease.  centers transfer to cardiologists or physicians with special interest in ACHD, and 5 centers transfer to the general community. At the 35 pediatric centers that transfer patients, adult-focused care is provided at the same hospital as the pediatric program. At other centers, the median distance to the nearest adult center is 12 miles. At 30 centers, patients are transferred to adult care at a specific age or age range, which median was 18 years. Centers with flexible policies transferred between 15 to 18 years old.   In the 51 centers that transfer patients, factors that influenced transition were identified. Likeliness to transfer was influenced if patients have comorbidities seen in adults, when patients considering pregnancy, or when adolescents or parents request to leave pediatric cardiology. Initiators of transfer included communication between pediatric and adult cardiologist, programs that indicated adult-oriented providers knowledgeable of CHD, financial incentives, and age. The aspect of transfer and transition that were indicated as important was the location of provision of education and resources explaining why and how patients would be transferred and providing education about the heart and treatments and health behaviors. Only 15 of the 51 centers have a formal transition program where adolescents are prepared for transfer and for taking responsibility for their care. Pediatric cardiologists were identified as most responsible for program coordination, followed by the advanced practice nurse and administrative assistant. Centers that do not currently have a formal transition program are making plans to develop such programs for the future.   One-quarter of the participating centers do not transfer their adolescents to adult care providers which means these centers either continue to care for their patients or the patients are discharge without referral to other HCPs for continued follow-up; therefore, some patients are lost to follow-up. Countries with governmental policies on transfer to adult care or when insurance companies do not reimburse when adults are care for at a children’s hospital are at greater risk of becoming lost to follow-up.   specialized in providing competent care for adults with CHD are becoming more available  -Trusting relationship and communication between pediatric cardiologist and adult care provider is important to facilitate transfer  -Structured transition programs and/or clinics should aim to improve adolescents’ and their families’ knowledge of the medical condition, to enhance adherence to medical regimens, to prevent high-risk behaviors, to enhance self-management, to prevent loss of follow-up, to avid inappropriate health care utilization, to maintain medical insurance, to achieve educational and vocational goals, and to improve overall QOL and psychological status. 23 Moons, P., Pinxten, S., Dedroog, D., Van Deyk, K., Gewillig, M., Hilderson, D., & Budts, W.  Expectations and experiences of adolescents with congenital disease on being transferred from 2009 To investigate the expectations and experiences of adolescents on transferring from a pediatric cardiology program to an adult congenital heart disease program Six themes emerged from the interview data: leaving pediatric cardiology was viewed as normal; leaving behind familiar surroundings; a positive wait-and-see attitude toward the adult congenital heart disease program; adjusting to a new environment; a need for better information; and a shift in roles between the adolescent and his parents.  -Formal transition process to prepare adolescents and their parents is important – aware of differences between two centers, be introduced to team members before the transfer  -Direct communication to adolescent  -Transfer is more difficult for parents who have developed a trust-based relationship with the pediatric cardiology providers.   -Understanding transition from different perspectives is  85 # Author, Title Journal Year Purpose Findings  Themes pediatric cardiology to an adult congenital heart disease program  Journal of Adolescent Health important.  -Transitioning is a positive experience for adolescents because they are recognized as responsible young adults   -Preparation is responsibility of the pediatric care team  -Need for better information – need for knowledge  24 Reid, G. J., Irvine, M. J., McCrindle, B. W., Sananes, R., Ritvo, P. G., Siu, S. C., & Webb, G. D.  Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with complex congenital heart defects  Pediatrics   2004 To determine the percent of young adults with CHDs who successfully transferred from pediatric to adult care and examine correlates of successful transfer. The study found that 47% of the 360 patients included in the study had successfully transferred to an adult care based on the Canadian Adult Congenital heart (CACH) centered data. Twenty-seven percent of those who completed the questionnaires had no cardiac appointments since 18 years old. Successful transfer to adult care was found to be related to: living close to a CACH center, more cardiovascular surgeries, older age at last visit to the HSC, and having recommendation from pediatric cardiologist for CACH follow-up. However, 50% of patients had no details related to adult care written in their pediatric records. Additionally, frequency of adolescent cardiac appointments, patients’ beliefs about where and when adult follow-up should take place, health status, CHD-specific health beliefs, and activity and engagement in risky behaviors were also related to success of transfer. Age, educational attainment, primary diagnoses, gender, residence in parents’ home, and average family income did not impact the success of transfer.   -Referral from pediatric cardiologist helped to ensure successful transfer  -Patients’ beliefs about time for adult follow-up were not related to documented recommendations  -Discussions involving the patient throughout adolescence may help patients acquire beliefs and understanding of the need for ongoing care.   -Longer patients receive care as adolescents, the more likely pediatric cardiologists are to make recommendations for adult care and patients are to have appropriate beliefs about adult care, in turn leading to successful transfer    86 Appendix D Grey Literature Findings # Year Author and URL Highlights/Themes Gaps 25 2017 The New England Regional Genetics Network  Genetics Education Materials for School Success https://www.gemssforschools.org/conditions/marfan/default  https://www.gemssforschools.org/transition/transition-landing    -the MFS info page has a link to transition resources that generally discusses the transition process as it applies to all genetic conditions. -> knowledge -identifies specific questions that may be pertinent to the adolescent or parents -Provides personal stories of patients with genetic conditions -Provide a PDF file that identifies the features of the condition, strategies to manage the manifestations of the condition and the complications that may occur, discusses situations that may affect schooling, discusses possible emergencies, and provides resources for more information. -provides general transition resources, nothing specifically on MFS  -Although linked to the MFS page, there is no area that specifies the transition process for patients with MFS. The information is general for all genetic conditions.  26 2015 Okamura, M. J. and Williams, R. G.  PS Net (Patient Safety Network) for Agency for Healthcare Research and Quality  https://psnet.ahrq.gov/webmm/case/348/transitions-in-adolescent-medicine    -Presents a case study and then a following commentary about the issues identified with the transition process of the patient in the case -Recommendations of facilitating the transition process was directed towards HCPs and was generalized across all disease conditions  -Information is general to chronic conditions – not specific to MFS although the case study is based on MFS.  27 2014 The Marfan Foundation  http://www.marfan.org/    -Provides general information about the condition -> knowledge -Provides resources for the individuals the condition affects, including parents, HCPs, adolescents or young adults -Tailors the resources to the age group -Offers connections to teen council members to ask questions  -Highlights stories of teens with MFS and their talents  -The resource for teens that talks about the transition appears to only accessible after one signs up; however, by searching the term “transition” in the search bar on the webpage, the resource PDF for teens appears under the title “Layout 4” -There is no one webpage for the transition – not easily accessible.  -The information provided in that document is not specifically on transition to adulthood but how MFS can affect certain aspects of their life.  28 2018 Nationwide Children’s Hospital  https://www.nationwidechildrens.org/conditions/marfan-syndrome  https://www.nationwidechildrens.org/specialties/adolescent-and-adult-congenital-heart-disease-achd-program/services/congenital-heart-disease-transition-program    -Has a page that explains MFS -Describes a program that helps transition adolescents and young adults to adult congenital heart disease care -Emphasizes the importance of certain aspects in the goals of transitioning -Identify the current need that less than 10% of adult CHD patients are actually receiving the recommending cardiac care because: pediatric cardiologists are not allowed to care for patients over age of 18; patients do not continue with follow-up -No direct link from the MFS information page to the transition clinic page – searched ‘transition’ in the search bar on the webpage.  87 # Year Author and URL Highlights/Themes Gaps    care; lack of education about heart defect; belief that they are “fixed” or “cured”; no longer “want to deal with heart disease”; perceived lack of cardiac symptoms; access to transportation, financial resources; location; lack of awareness of how to find a new heart specialist   29 2018 Seattle Children’s Hospital/Research/Foundation and the University of Washington Medical Center  http://www.seattlechildrens.org/medical-conditions/chromosomal-genetic-conditions/marfan-syndrome/  http://www.seattlechildrens.org/clinics-programs/heart/treatments-and-services/adult-congenital-heart-disease/  Focus: -Congenital Heart Disease    -The MFS page contains a link to the adult congenital heart disease program. -Program that offers a range of services to diagnose, manage, and treat heart defects -Between ages 14 and 18, teens get care at Seattle Children’s and then between ages 18 to 21, young adults’ transition to care at UWMC. -Provides links to transition resources -Provides PDFs of resources for pre- and post-heart surgery and medications like warfarin and enoxaparin. -Section entitled “Transition for Adolescents and Young Adults” – explains how the program can help address the specific needs related to their lifestyle choices that might affect or be affected by their condition.  -Doesn’t provide in-depth details about the areas that are usually included in discussions about transition. 30 2017 - 2018 UCLA Children’s Hospital  http://heart.ucla.edu/clinical-services  https://www.uclahealth.org/Mattel/Adolescent-Transitional-Cardiac-Care-Program/    -The Adult Congenital Heart Disease center webpage that identifies they also care for MFS patients has a link to the ‘Adolescent/Young Adult Transitional Care program, for congenital heart disease’, -Program that assists adolescents in their transition to adult-based care -Provides information specifically directed to parents and information specifically directed to teens  -No mention of a geneticist or genetic counsellor -No mention of the importance of social support through friends 31 2018 B.C. Children’s Hospital Provincial Health Services Authority  http://www.bcchildrens.ca/our-research/research-focus/childrens-heart-centre  http://www.bcchildrens.ca/our-services/support-services/transition-to-adult-care      -MFS is mentioned as one of the conditions the Children’s heart center treats.  -Discusses ON TRAC, which is a province-wide transition initiative – 4 main areas of work: 1) clinical support; 2) youth and family engagement; 3) education and knowledge translation; 4) research and evaluation  -Provides resources specific to the parents, youth, and HCPs in the transition process -The resource for the youth includes a brochure that identifies a timeline with steps that should be followed towards transitioning to adult care -Each section of the youth toolkit addressed a certain aspect of the transition process: voice, action, connection, future planning, sexual health, and safety -Each section of the family toolkit addresses the same aspects as the youth toolkit except from the perspective of the parents  -The page that discusses MFS and the Children’s Heart Center doesn’t provide a link to the transition page – find the page on transition by searching for it through the search bar on the website or going to ‘Our Services’ and scrolling all the way down to ‘Support Services’.  88  Appendix E Analysis Using Schumacher and Meleis’ Transition Framework # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments 1 Qualitative  Grounded Theory Adolescents aged 14-21 years old  Young adults aged 22-34 years old  Parents  HCPs  “Transitioning to self-management is more than planning the transfer of services from pediatric to adult care, and involves gradual changes in knowledge, attitudes, and behavior influenced by parents, peers, and health care providers.” It is an evolving model of participation in life-long surveillance.   Meaning of transition  Subjective Well-being X  Expectations of the transition X Level of Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being X Emotional/Physical Well-being X 2 Qualitative  Thematic content analysis from data from larger grounded theory study        Adolescents aged 14-21 years old  Young adults aged 22-34 years old  Parents  HCPs Transition “encompasses a range of activities performed over a lifetime to deal with the physical, emotional, and social issues associated with chronic illness”. Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  3 Qualitative  Thematic content analysis from data collected from larger grounded theory study Adolescents aged 14-21 years old Transitioning to self-management requires the adolescent/young adult to “acquire an elaborate set of behaviors such as adhering to treatment guidelines, practicing health promotion, and seeking accurate information about their disorder”. It involves “gradual changes in attitudes and behaviors influenced by parents, peers, and healthcare providers”. Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill X Role Mastery   Environment   89 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 4 Qualitative   Parents of adolescents aged 14-18 years old with moderate to complex CHD Transition is… “The process by which adolescents with CHD are prepared to take charge of their lives and their health in adulthood. It involves collaboration with family members and patients and HCPs.  Meaning of transition  Subjective Well-being  -No mention of vocational issues or physical limitations or restrictions from parents – are they unaware of these issues? Expectations of the transition  Level of Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 5 Quantitative  Adolescents aged 12-18 years old Transition is… -“The purposeful, planned movement from one form, stage, or style to another” (Clarizia et al., 2009) -“The transfer of adolescents with chronic disease from child-centered to adult-oriented care systems” (Sable et al., 2011; Warnes et al., 2008) -“Is gradual, coordinated, and permanent” -Goal is to “optimize lifelong functions through high-quality permanent care  Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  6 * Qualitative Adolescents aged 14-18 years old  Parents  Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill X Role Mastery   90 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  7 * Quantitative Adolescents and young adults aged 15-23 years old  Parents  HCPs Transition “should entail a purposeful plan that aims to provide age-appropriate information regarding self-management and to satisfy the medical, psychosocial, educational, and employment needs of young patients”. It occurs between ages 15 to 24 years old and is a continual process.  Meaning  Subjective Well-being   Expectations X Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 8 Quantitative  Adolescents aged 12-17 years old  Parents Transition planning is “the process by which health care providers give anticipatory guidance for patient’s transfer to adult healthcare”. Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill  Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  9 Quantitative Adolescents aged 12-15 years old  Parents Transition is “the purposeful and planned movements of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems”. Begin the process at the age of 12 and educate between 13 to 16 years.  Meaning  Subjective Well-being   Expectations   91 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Levels Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  10 Quantitative Adolescents and young adults aged 14-25 years old Transition is “a purposeful, planned process that addresses the medical, psychosocial, educational, and vocational needs of adolescents and young adults as they move from child centered to adult-oriented healthcare systems”. Meaning of transition  Subjective Well-being  -Examined CHD, cystic fibrosis, and diabetes Expectations of the transition  Level of Knowledge/Skill  Role Mastery  Environment X Level of Planning  Relationship Well-being  Emotional/Physical Well-being X 11 Quantitative  Retrospective Young Adults > 18 years  Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill  Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  12 Qualitative Adolescents aged 16 to 18 years Transition is “a developmental process when adolescents prepare to leave the pediatric environment”. Meaning  Subjective Well-being  -didn’t measure  92 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments old Expectations  healthy transition Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  13 Quantitative  Retrospective Young Adults >18 years old Transition education includes “preparation for self-care and self-advocacy”. Meaning  Subjective Well-being  -Successful transfer was defined as subsequent attendance at appointments with adult cardiac providers within 2 years following the study time. Expectations  Levels Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  14 Qualitative Young adults aged 19-24 years old who have recently transitioned “Preparation for transfer involves letting the patient gradually assume responsibility for his/her own health and self-care”. Meaning  Subjective Well-being  -Care staff and parents focus on the meaning and consequences of the CHD whereas informants concentrate on their present well-being and feel no need to act.  Expectations X Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being   93 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments 15 Systematic Review Adolescents and young adults  Parents  HCPs Transition is “a purposeful, planned process that addresses the medical, psychosocial, educational, and vocational needs of adolescents and young adults  Meaning  Subjective Well-being  -Reported on outcomes and experiences  -2 outcomes examined: 1) loss of follow-up and 2) lapse of care Expectations X Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  16 Qualitative Adolescents and young adults aged 16-20 years old  Adults aged 21-40 years old with moderate to complex CHD Transition is “a purposeful and planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented healthcare systems”. Meaning of transition  Subjective Well-being   Expectations of the transition X Level of Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 17 Quantitative Adolescents and young adults aged 13-25 years old  Parents of children aged 13-18 years old Transition is “the process by which adolescents and young adults with chronic childhood illnesses are prepared to take charge of their lives and their health in adulthood”.  The goal is to “maximize lifelong functioning and potential through provision of high quality, developmentally appropriate health services that continues uninterrupted as the individual moves from adolescence to adulthood”. Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being   94 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Emotional/Physical Well-being X 18 Qualitative Adolescents and young adults aged 11-17 years old  Parents  Meaning  Subjective Well-being  -Examined CHD and CF Expectations  Levels Knowledge/Skill  Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  19 Qualitative Young adults aged 16 years old or older who have already or about to be transferred to adult care  Parents  HCPs Transition is “a purposeful, planned process” which occurs “prior to and after the transfer event (actual shift from pediatric to adult care)”.  It requires a “cultural shift in staff’s attitudes, effective transition programmes and teaching adolescents to be active partners in their own care”.  Meaning  Subjective Well-being  -Examined CHD and 6 other chronic conditions Expectations X Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  20 Mixed Methods Young adults aged 18-25 years old Transition is “a multifaceted, organized, prolonged process by which individual learns to assume responsibility for his/her health care (i.e. self-care) and life”.  Meaning  Subjective Well-being  -PhD dissertation Expectations  Levels Knowledge/Skill X Role Mastery  Environment X  95 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 21 Mixed Methods Adolescents aged 9-18 years old  Parents  HCPs Transition is “a purposeful and planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems”. The goal is “to ensure continuity of quality care”. Meaning  Subjective Well-being   Expectations  Levels Knowledge/Skill X Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 22 Quantitative HCPs Transition is “the process by which adolescents and young adults with chronic childhood illnesses are prepared to take charge of their lives and their health in adulthood”. It is “an educational process that ideally begins before children reach adolescence and continues until they are capable of taking full responsibility”. Meaning of transition  Subjective Well-being   Expectations of the transition X Level of Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 23 Qualitative Adolescents aged 15-17 years old Transition is “the process by which adolescents and young adults with chronic childhood illnesses are prepared to take charge of their lives and their health in adulthood”. It is “an educational process that ideally begins before children reach adolescence and continues until they are capable of taking full responsibility of care”. Meaning  Subjective Well-being   Expectations X Levels Knowledge/Skill X Role Mastery   96 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  24 Quantitative  Retrospective Young adults aged 19-21 years old Transition is “a purposeful, planned movement of adolescents and young adult with chronic physical and medical conditions from adult-centered to adult-oriented healthcare systems; it addresses both physical and psychosocial factors”. Meaning  Subjective Well-being  -Successful outcome to transfer to adult care is defined as the patient having attended at least 1 appointment of any time at a CACH center. Expectations  Levels Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 25 Foundation/ network/ association website General Public Transition is “a change or passing from one setting/ grade/ care system etc. to another”. It is “an ongoing, collaborative process that involves preparing for, facilitating and adjusting to a shift to more and more independence”. Meaning of transition  Subjective Well-being X  Expectations of the transition X Level of Knowledge/Skill X Role Mastery X Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  26  Foundation/ network/ association website HCPs Transition is a “purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems”.  Meaning  Subjective Well-being   Expectations X  97 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments Levels Knowledge/Skill X Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 27 Foundation/ network/ association website Parents  Children, Adolescents, and Young adults  HCPs None Provided Meaning  Subjective Well-being   Expectations X Levels Knowledge/Skill  Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  28 Hospital website Parents  Adolescents Transition is “the shift for adolescents from child-centered to adult-centered care that is gradual”. Meaning  Subjective Well-being  -Knowledge provided is about the condition, not the transition -Very superficial discussion about the goals and the importance certain aspects that affect transition.  Expectations  Levels Knowledge/Skill  Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being X 29 Hospital website Parents of adolescents and Transition is “a time with more activity and decisions to make on their own”. The goal is “to empower and prepare Meaning  Subjective Well-being  -Knowledge provided is  98 # Type of Literature Perspective or  Target Audience DEFINING TRANSITION Category 1: Universal Properties  (Process Direction, Change in Roles, Relationships, Identity, Behavior) FACTORS AFFECTING TRANSITION Category 2: Transition Conditions  (Meanings, Expectations, Level of Knowledge/Skill, Environment, Level of Planning, Emotional and Physical Well-being)  OUTCOMES Category 3: Indicators of Healthy Transition (Subjective Well-Being, Role Mastery, Well-being of Relationships) Comments young adults up to age 21 years old adolescents and young adults to take on a greater role in their care as they prepare to enter the adult healthcare system”. Expectations  about the condition, not the transition  Levels Knowledge/Skill  Role Mastery  Environment X Level of Planning X Relationship Well-being  Emotional/Physical Well-being  30 Hospital website Parents  Adolescents and Young adults Transition is “a purposeful movement of young adults and adolescents with chronic physical and medical conditions from child-centered to adult-oriented health care”. Meaning  Subjective Well-being  -Communication and collaboration among pediatric and adult cardiac specialists with their young adult patients and families.  Expectations X Levels Knowledge/Skill  Role Mastery  Environment  Level of Planning X Relationship Well-being  Emotional/Physical Well-being  31 Hospital website Parents  Adolescents  HCPs Transition is “a journey taken by youth together with their families.” The goal is “to support youth with special health care neds and their families or caregivers to gain confidence, skills, knowledge, to be ready to enter the adult care system”. It starts at the age of 12.  Meaning  Subjective Well-being   Expectations X Levels Knowledge/Skill  X Role Mastery  Environment  X Level of Planning  X Relationship Well-being  Emotional/Physical Well-being  X     

Cite

Citation Scheme:

        

Citations by CSL (citeproc-js)

Usage Statistics

Share

Embed

Customize your widget with the following options, then copy and paste the code below into the HTML of your page to embed this item in your website.
                        
                            <div id="ubcOpenCollectionsWidgetDisplay">
                            <script id="ubcOpenCollectionsWidget"
                            src="{[{embed.src}]}"
                            data-item="{[{embed.item}]}"
                            data-collection="{[{embed.collection}]}"
                            data-metadata="{[{embed.showMetadata}]}"
                            data-width="{[{embed.width}]}"
                            data-media="{[{embed.selectedMedia}]}"
                            async >
                            </script>
                            </div>
                        
                    
IIIF logo Our image viewer uses the IIIF 2.0 standard. To load this item in other compatible viewers, use this url:
https://iiif.library.ubc.ca/presentation/dsp.42591.1-0371940/manifest

Comment

Related Items