Development and evaluation of a patient education programme for children, adolescents, and young adults with differences of sex development (DSD) and their parents: study protocol of Empower-DSD

Background: Differences in sexual development (DSD) are rare diseases, which affect the chromosomal, anatomical or gonadal sex differentiation. Although patient education is recommended as essential in a holistic care approach, standardised programmes are still lacking. The present protocol describes the aims, study design and methods of the Empower-DSD project, which developed an age-adapted multidisciplinary education programme to improve the diagnosis-specific knowledge, skills and empowerment of patients and their parents. Methods: The new patient education programme was developed for children, adolescents and young adults with congenital adrenal hyperplasia, Turner syndrome, Klinefelter syndrome or XX-/or XY-DSD and their parents. The quantitative and qualitative evaluation methods include standardised questionnaires, semi-structured interviews, and participatory observation. The main outcomes (assessed three and six months after the end of the programme) are health-related quality of life, disease burden, coping, and diagnosis-specific knowledge. The qualitative evaluation examines individual expectations and perceptions of the programme. The results of the quantitative and qualitative evaluation will be triangulated. Discussion: The study Empower-DSD was designed to reduce knowledge gaps regarding the feasibility, acceptance and effects of standardised patient education programmes for children and youth with DSD and their parents. A modular structured patient education programme with four generic and three diagnosis-specific modules based on the ModuS concept previously established for other chronic diseases was developed. The topics, learning objectives and recommended teaching methods are summarised in the structured curricula, one for each diagnosis and age group. At five study centres, 56 trainers were qualified for the implementation of the training programmes. A total of 336 subjects have been already enrolled in the study. The recruitment will go on until August 2022, the last follow-up survey is scheduled for February 2023. The results will help improve multidisciplinary and integrated care for children and youth with DSD and their families. Trial registration: German Clinical Trials Register, DRKS00023096. Registered 8 October 2020 - Retrospectively registered

Massenmedien

Friedrichs-Liesenkötter H. Massenmedien. In: Horn K-P, Kemnitz H, Marotzki W, Sandfuchs U, eds. Klinkhardt Lexikon Erziehungswissenschaft. KLE Band 2: Gruppenpuzzle-Pflegewissenschaft. Bad Heilbrunn: Klinkhardt UTB; 2011: 338-340

Medien

Friedrichs-Liesenkötter H. Medien. In: Horn K-P, Kemnitz H, Marotzki W, Sandfuchs U, eds. Klinkhardt Lexikon Erziehungswissenschaft. KLE Band 2: Gruppenpuzzle-Pflegewissenschaft. Bad Heilbrunn: Klinkhardt UTB; 2011: 347-349

Medienpädagogik

Friedrichs-Liesenkötter H, Sander U. Medienpädagogik. In: Horn K-P, Kemnitz H, Marotzki W, Sandfuchs U, eds. Klinkhardt Lexikon Erziehungswissenschaft. KLE Band 2: Gruppenpuzzle-Pflegewissenschaft. Bad Heilbrunn: Klinkhardt UTB; 2011: 363-365

Sleep Timing in Patients with Precocious and Delayed Pubertal Development

Previous studies have reported a shift in the timing of sleep during adolescence toward a later time. To date, it is unclear whether hormonal changes during puberty might contribute to this change in sleeping behavior. We systematically assessed pubertal development and sleep timing in a cross-sectional case-control study in girls with precocious (n = 42) and boys with delayed pubertal development (n = 19). We used the Munich ChronoType Questionnaire and the Children’s ChronoType Questionnaire to assess sleep timing in patients and age- and sex-matched controls (n = 309) and used the midpoint of sleep on free days, corrected for potential sleep debt accumulated during the school week, as a marker for sleep timing. Compared to the controls, girls with central precocious puberty showed a delay in sleep timing of 54 min, and girls with premature pubarche slept on average 30 min later. Male adolescents with delayed pubertal development showed an average sleep midpoint that was 40 min earlier compared to the control group. The results of this pilot study suggest an association between pubertal onset and shifts in sleep timing, which is a novel finding in human sleep behavior. Prospective studies in larger cohorts will be needed to examine the robustness and generalizability of the findings