Background
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Due to a lack of coordination of the necessary processes, patients run the risk of receiving insufficient treatment during the transfer to adult care. This insuffient care can lead adolecents to discontinue therapy or to stop taking prescribed medications.
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This lack of coordination means that transfers into adult care are unplanned. There is no room for coordinated support in the transition process.
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During adolescence, young patients not only have to deal with a chronic disease, but also deal with compelling questions regarding their sexuality, leaving their childhood homes, and planning their professional lives.
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The nonsurgical treatment: this means that patients are asked to manually place successive dilators on the perineal dimple for 30 min to 2 h per day. Another option is sitting on a bicycle seat stool. The bicycle seat stool provides the perineal pressure. Use of dilators in the management of MRKHS is appropriate and successful for mature, highly motivated patients who wish to avoid surgery because the treatment endures several months [5] and is very painful and dilation of the uretra could occure, It is no option, if the vaginal dimple is to short.
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The surgical treatment should avoid big scars and transplanted tissue like skin, bowl or peritoneum. Therefore the laparoscopic-assisted creation of a neovagina is the treatment of choice. This method creates after a few months a neovagina by minimal invasive access with a normal epithelium after full epithelialization [10, 11].
Purpose and methods
Additional information about the inclusion process during the literature research |
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• Identification Phase To identify appropriate studies, a systematic literature search was carried out using the PubMed database (online portal of the National Library of Medicine) from June 2012 through March 2013. The literature search was updated in 2014/2015 to include newer publications. The search was carried out for clinical picture, target group, treatment management, and outcome using keywords and keyword combinations defined by the project team. The search was performed with a broad scope, also including findings from publications on transition management for other relevant diseases when deriving recommendations. This was necessary, since few studies on transition for rare diseases could be found in 2012 and 2013. Two search strategies were applied: - Search strategy 1 comprised a search for publications generally related to the transition phase. The following keywords were used: “young girl AND puberty AND disease course“ and “girl AND transition“ - Search strategy 2 comprised searching for publications dealing with the disease MRKHS in the context of transition. The following catchwords were used: “parents AND MRKH“, “female AND adolescent AND gential malformation AND MRKH”, “MRKH AND transition“, “rokitans* AND organisation“, “female AND adolscent AND gential malformation AND transition“, “female AND rokitans* AND transfer”, “female AND adolscent (Mesh Terms) AND rokitans* AND disease management AND quality of life” The search in PubMed using both search strategies resulted in a total of 2,149 hits. |
• Screening Phase After excluding duplicates (n=365), all publications found in the search were first evaluated according to title and abstract by two reviewers from the project team, independently from one another, with regard to suitability for the study question. This resulted in the exclusion of 1,615 hits. After a positive evaluation by both reviewers, the full-text was used for further analysis. |
• Full-text Analysis Phase Following the screening phase, 166 hits were available for the full-text analysis. The full texts were read by a third person who made the final decision on inclusion in this review, and extracted the necessary content from the publications. The reference lists of the full-text publications read were also viewed for possible other suitable publications. In the course of this process, an additional 154 hits were excluded (editorials, descriptive publications, and therapy studies). Publications were excluded that were not written in either German or in English. Studies were also excluded if, due to the target group, the subject, or the disease, there was no transferability to the situation of patients with MRKHS. |
• Integration Phase A total of 12 publications were included from the full-text analysis and an additional 7 publications from the reference lists of the included publications were integrated into the literature review (4, 7, 14-30). |
Results
Authors | Study design | Objective | Country | Numbers (Participants, Studies) | Instruments |
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Bean et al. (2009) | Narrative literature review | Examination of psychological effects and quality of life following the diagnosis and treatment of MRKHS | USA | 31 Studies (differing methodologies) | none |
Heller-Boersma et al. (2007) | Randomized Control Trial | Examination of the effectiveness of group therapy based on a cognitive behavior approach with regard to the psychological outcome before therapy, after therapy (7 weeks), and 3 months after therapy in women with MRKHS | UK | Intervention Group (n = 19) (response rate: baseline 100 %, after treatment 84 %, follow-up 84 %; age: 28.9, SD 10.0) Control Group (n = 20) (response rate: baseline 100 %, after treatment 80 %, Follow-up 80 %; Alter: 27.6, SD 7.1) | Symptom Checklist (SCL-90-R)/Rosenberg Self-Esteem Scale (RSE)/Impact of Events Scale (IES)/Short version of the Inventory of Interpersonal Problems (IIP-32) |
Heller-Boersma et al. (2009) | Cross-sectional study | Examination of the psychological effect of MRKHS when comparing women with MRKHS and healthy women | UK | 66 women (response rate: 20 %; age: 27.9, SD 1.0) 31 healthy women as a control group (response rate: 73.8 %; age: 27.8, SD 1.5) | Symptom Checklist (SCL-90-R)/Rosenberg Self-Esteem Scale (RSE)/Inventory of Interpersonal Problems (IIP-32)/Eating Disorder Inventory (EDI) |
Holt et al. (2003) | Qualitative study | Examination of personal experiences of women with MRKHS, to gain insight into the psychological, social, and emotional consequences of diagnosis and treatment | UK | 7 women (response rate: 17.5 %; age: 18–37) | Semi-structured interview guide |
Huber et al. (2009) | Narrative literature review | Overview on the state of research on psychological aspects of women with MRKHS | Germany | 43 studies (differing methodologies) | none |
Loeser et al. (2002) | Qualitative study | Examination of mother-daughter relationships throughout the course of peri- and postoperative creation of a neovagina | Germany | 8 women (response rate: no information; age: 14–21) 7 mothers (no additional information) | Structured interview guide |
Patterson et al. (2014) | Qualitative study | Examination of the importance of MRKHS to gain insight into social challenges during the transition into adulthood | Scottland | 5 women (response rate: no information; age: 18–22) | Semi-struktured interview guide |
Weijenborg et al. (2000) | Cross-sectional study with pre-post design | Evaluation of the effectiveness of group therapy with regard to psychological burden before, at the beginning, and after treatment of women with MRKHS | Netherlands | 17 women (for analysis: information from 15 women; age: 27.1; SD 10.0) | Symptom Checklist (SCL-90-R) |
Authors | Study Design | Main Topic | Country | Numbers (Participants, Studies) | Instruments | Main Results |
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Castrejón (2012) | Narrative literature review | Overview of information available in the literature on transition units with regard to rheumatic diseases | USA | 46 studies (differing methodologies) | none | Recommendations for interacting with adolescent patients – Respect, confidentiality, avoiding mistakes, continuity, autonomous visits without parents, use of the HEADSS assessment Components of a transition unit – Early age, transition coordinator, transition program, involvement of pediatricians and physicians for adults, parents, multidisciplinarity , qualified personnel, evaluation |
Crowley et al. (2011) | Systematic literature review | Review of the efficacy of transition programs in young patients between the ages of 11 and 25 with chronic diseases or disabilities and the identification of successful components | UK | 10 studies (Evaluation of interventions during transition, but with different methodology) | none | Patients – Disease-related education (4x successful intervention) – Improved education and skills traning (2x successful intervention) Healthcare Facility – Transition coordinator (2x successful intervention) – Liaison between pediatric and adult hospital (3x successful intervention) Service offers – Separate clinics for young adults (3x successful intervention) – Telephone support service (1x successful intervention) – Appointment reminders by telephone (2x successful intervention) |
Forbes et al. (2002) | Systematic literature review | Identification and evaluation of practices that assure adolecents with chronic diseases and disabilities of continuity during the transition | UK | Search A: 61 studies (Identification of Best-Practice.models) Search B: 39 studies (inclusion of five diseases) Search C: 26 studies (interview of key persons) | none | Structural component – Transition worker; transition teams; professional continuing education; information for specialists; use of existing services; inter- and intra-organization of networks and arrangements; organizational planing; theoretical framework; promotion of equality and accessibility. Process components – Preparation for the transfer; active management of the transition; case management; responsibility for the process; strengthening of therapeutic relationships; representation of interests; joint care management; flexibility with regard to the transfer; specific communication systems; regular assessment of provision of services. Result components – Disease-specific or general outcomes like satisfaction. Other outcome components are benchmarks of how service quality can be measured. Components of the practice regarding young people: – Specific services provided; developing competence in autonomy and self-determination; support for psychosocial development; inclusion of young people; peer Einbindung der junge Leute; involvement of peers; support for the changed relationship with parents/caregivers; making appropriate choices; availability of information; concentration on young people’s strengths for their future development Components in practice regarding parents and caregivers: – Support adjusting to the changed relationships to the young people; inclusion of parents in work scheduling; family-centered approach and provision of information. |
Price et al. (2011) | Qualitative Study | Evaluation of a transition model based on interviews with young adults with diabetes mellitus | UK | 11 young adults (age: 16–18 years) 2 young adults after 1 year | Semi-structure interview guide | Initial topics: – The transition process – Experience with, and organization of the transfer – Organization of services – Information and education – Health counselling – Integration. Primary topics – appropriate health care of young people – Recognizing individuality in health care |