Background and aims: Cystic Fibrosis (CF) is the most common genetic, life threatening disorder in the UK (Cystic Fibrosis Trust, 2010). Given the trajectory of the illness, adolescence may be a particularly challenging period, during which young people become more aware of differences from peers, and are faced with the task of balancing increasing illness demands with the drive to aspire to developmental goals. Nevertheless, little research specifically explores how young people with CF reconcile their illness experiences with the emerging sense of self. In an attempt to address this gap in the literature, this study sought to hear the narratives of young people with CF with reference to the local and broader contextual factors influencing their construction, with the aim to further understanding, inform clinical practice and improve support for young people with CF. Methodology: A qualitative approach was employed. A purposive sample of six participants diagnosed with CF and aged between 12 and 16 years was recruited. Participants were asked to take photographs of their experiences of life as a young person with CF which were used alongside a semi-structured topic guide in individual interviews to explore the young person’s narratives. The interviews were audio-recorded, transcribed, and analysed using a narrative approach to explore both what was said and how it was told.Analysis and Findings: The researcher’s global impressions of each person’s narratives, along with details of the local context of the interview were presented. Following this, similarities and differences across the narratives were considered with particular attention to how the main storylines were interwoven with participants’ emotional experiences, the identity work taking place through the narrative, and the broader narratives available to them. It emerged that (i) CF was perceived as part of participants’ normality which they had grown accustomed to over time, (ii) participants drew on cultural narratives to position themselves as normal teenagers, to maintain a positive sense of self, though also leading them to minimise difficulties and distress, and (iii) participants continued to position themselves within the norm as they talked of their futures, describing similar hopes to their peers, and again played down concerns about how CF might impact on their futures. These findings are discussed with reference to the clinical implications, strengths, and limitations of the methodology, and directions for future research.

Objective: The aim of this study was to compare equations used to calculate BMR to values of BMR obtained from measurements in order to determine the most suitable equation to be used on children and adolescents with CF. Design: The participants where children and adolescents with CF. Data was collected from the division of Clinical Nutrition at Uppsala Akademiska Sjukhus. Age, weight, height, BMI, BMR, FFM and FM was recorded from 27 measurements. BMR from the participants collected from the indirect respiratory calorimetry was compared to the results obtained from five equations used to obtain a calculated value of BMR. Results: Tverskayas equation was best correlated with the measured BMR for the whole group. The equation underestimated BMR for the participants with a measured BMR over 1400 kcal/24h and overestimated BMR for participants with a measured BMR under 1400 kcal/24h. Similar results could be seen in the subgroups boys, girls and adolescents. Maffeis equation can be applied on the subgroup children. However, larger studies are needed to guarantee the results. Conclusion: It is desirable that an equation for calculating BMR would be developed that is specifically developed for children and adolescents with a low to normal BMI that takes into consideration a heightened metabolism and is therefore suitable for children and adolescents with CF. / Syfte: Att utifrån uppmätt BMR jämföra olika ekvationer för att hitta den mest lämpliga ekvationen för beräknandet av ett förutsätt värde av basalmetabolism hos en grupp barn och ungdomar med cystisk fibros. Metod: Deltagarna var barn och ungdomar med CF. Data till arbetet hämtades från enheten för klinisk nutrition vid Uppsala Akademiska sjukhus. Ålder, vikt, längd, BMI, BMR, FFM och FM samlades in från totalt 27 mättillfällen. Deltagarnas BMR, som uppmätts genom indirekta respiratorisk kalorimetri, jämfördes med fem ekvationer som räknar ut BMR utifrån olika antropometriska mått. Resultat: Tverskayas ekvation överensstämde bäst med uppmätt BMR i gruppen som helhet. Ekvationen underskattade dock BMR hos deltagare med ett BMR-behov över 1400 kcal/dygn och överskattade BMR hos deltagare med ett BMR-behov lägre än 1400 kcal/dygn. Liknande resultat sågs i undergrupperna pojkar, flickor och ungdomar. Maffeis ekvation skulle möjligen kunna tillämpas på gruppen barn, dock behöver större studier utföras för att kunna dra säkra slutsatser. Slutsats: Det vore önskvärt att det utvecklas en ekvation för BMR, särskilt utvecklad för barn och ungdomar med lågt till normalt BMI, som tar hänsyn till förhöjt BMR och som därmed lämpar sig särskilt för barn och ungdomar med CF.

Mémoire en recherche-création. Master in research-creation / Vers l’âge de 7 ans, les patients atteints de fibrose kystique (FK) sont amenés à suivre une thérapie par pression expiratoire positive (PEP). Celle-ci consiste à réaliser une série d’exercices respiratoires répétitifs et méthodiques. Cependant, ces exercices peuvent être ennuyeux et démotivants pour des jeunes enfants et entrainent un taux d’adhésion quotidien de moins de 50%. Ce faible résultat affecte grandement la qualité et l’espérance de vie à long terme des patients, en plus d’avoir un impact social et familial considérable. Ces derniers utilisent les aspects ludiques et interactifs des jeux vidéo pour motiver et susciter l’engagement des sujets envers l’activité et son contenu. Ils ont déjà faits leurs preuves dans le domaine de la santé et de l’éducation en favorisant l’apprentissage ou la réhabilitation par exemple. Ce projet de recherche-création cherche à valider la possibilité d’utiliser des jeux spécialement conçus pour la thérapie PEP ainsi que l’intérêt des jeunes atteints de FK à les utiliser dans le cadre de leurs exercices thérapeutiques. L’objectif est de favoriser l’adhésion des jeunes envers cette thérapie et faciliter l’apprentissage et l’application des techniques associées. Cette approche ludique vise ultimement à améliorer la qualité de vie des jeunes atteints de FK dans la durée. Grâce à une collaboration entre des étudiants de l’Université de Montréal et des spécialistes en santé de la clinique de FK du CHU Ste-Justine, trois prototypes de jeux et un prototype d’interface électronique, utilisé pour contrôler les jeux et pour être compatibles avec l’appareil PEP le plus utilisé en Amérique du Nord (le PariPEP®), ont été conçus dans le cadre de ce projet. Les prototypes ont été testés sur place, à la clinique FK de l’hôpital pour enfants, dans le cadre d’une étude pilote effectuée auprès de 10 jeunes patients, toutes et tous âgés entre 8 et 10 ans. / At around the age of 7 years old, younger patients diagnosed with cystic fibrosis (CF) are usually prescribed a new therapy using positive expiratory pressure (PEP). For PEP therapy, patients are required to perform a series of repetitive and methodical breathing exercises to improve lung capacity among other health benefits. However, these exercises can be boring and demotivating for young children. As a result, daily adherence rate to PEP therapy is less than 50%. In turn, this affects the quality of life and long-term health of patients as well as directly impacting their families. In the education and health sectors, serious games are already used with children to help them with learning, therapy and even physical rehabilitation. Serious games leverage the fun and interactive aspects of video games to motivate and improve the commitment of the player to learn the targeted material or adhere to a specific health behaviour. This research-creation project seeks to validate the efficacy of using games specially designed to help patients adhere to PEP therapy exercises more regularly. The objective is to encourage them to get involved in their daily exercises and to facilitate the learning and application of the breathing techniques. This playful approach ultimately aims to improve the quality of life of CF children over time. Leveraging a multi and interdisciplinary collaboration between students from University of Montreal and specialists from the CF clinic at CHU Ste-Justine, three games prototypes and an electronic device prototype, used to control the games, were developed for the most popular PEP device in North America (PariPEP®). The games and electronic device prototypes were tested at the CF clinic, within the hospital for children, as part of a pilot study with 10 young patients, aged from 8 to 10 years old.