Introduktion: Cystisk fibros är en progressiv kronisk sjukdom. Att leva med cystisk fibros påverkar personens liv och personer med cystisk fibros har beskrivit att de upplever en försämrad livskvalitet med tidskrävande behandlingar, fysiska begränsningar och psykosociala påfrestningar vilka begränsar möjligheten till att leva livet fullt ut. Hur sjukdomen upplevs av yngre personer hade bidragit till att ge sjuksköterskor ökad förståelse för patienters behov i omvårdnaden. Syfte: Syftet med studien var att beskriva barns, ungdomars och unga vuxnas upplevelser av att leva med cystisk fibros.  Metod: En beskrivande litteraturöversikt baserad på tio kvalitativa artiklar utfördes. Artiklarna kvalitetsgranskades enligt SBU:s granskningsmall för kvalitativa studier (2022a). Studiernas resultat sammanställdes och analyserades utifrån en modell beskriven av Popenoe et al. (2021). Resultat: Analysen resulterade i tre kategorier: Begränsningar i det dagliga livet, Upplevelser av hälsa och Påverkad självbild. Cystisk fibros medförde upplevelser av kroppsliga begränsningar, tidskrävande behandling, sociala restriktioner och en oro inför framtiden. Att ses som mer än sjukdomen ansågs var en faktor till bättre upplevd hälsa. Självbilden påverkades då deltagare eftersträvade normalitet. Det fanns även en rädsla för att bli dömd, att göra andra besvikna och upplevelser av bristande autonomi. Slutsats: Cystisk fibros påverkar unga ur aspekter såsom självbild, socialt liv och fysiska begränsningar. Kunskap om sjukdomens påverkan ger sjuksköterskan bättre förutsättning för god personcentrerad omvårdnad och att hjälpa unga patienter hantera sin sjukdom och uppnå bästa möjliga livskvalitet. / Introduction: Cystic fibrosis is a progressive chronic disease. Living with cystic fibrosis affects the person's life and reduces quality of life with time-consuming treatments, physical limitations and psychosocial stresses which limit the opportunity to live life to the full. How the disease is experienced by younger people could contribute to giving nurses an increased understanding of patients' needs. Aim: The aim of this study was to describe the experiences of children, adolescents and young adults living with cystic fibrosis. Method: A descriptive literature review, based on 10 scientific qualitative articles. The articles were quality reviewed according to SBU's review template for qualitative studies (2022a). The results of the studies were compiled and analyzed based on a model described by Popenoe et al. (2021). Results: The people's experiences resulted in three categories: Limitations in daily life, Experience of health and Affected self-image. Cystic fibrosis brought limitations such as physical limitations, time-consuming treatment, social restrictions and a worry about the future. Being seen as more than the disease was considered a factor in perceived health. Self-image was affected as participants sought normalcy, there was also a fear of being judged, fear of disappointing others and an experience of a lack of autonomy. Conclusion: Cystic fibrosis affects young people from aspects such as self-image, social life and physical limitations. Knowledge of the impact of the disease gives the nurse a better basis for good person-centered care and to help young patients manage their disease and achieve the best possible quality of life.

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In cystic fibrosis (CF) therapy, the recent approval of CF-transmembrane conductance regulator (CFTR) channel modulators is considered to be the major breakthrough. However, the current first-line approach based mainly on pulmonary function to measure effects of the novel therapy, tested by forced expiratory volumes in one second (FEV1), provides restricted sensitivity to detect early structural damages. Accordingly, there is a need for new sensitive surrogate parameters. Most interestingly, these should quantify inflammation that precedes a decline of pulmonary function. We present a novel method assessing inflammatory markers in the upper airways’ epithelial lining fluid (ELF) obtained by nasal lavage (NL). In contrast to broncho-alveolar lavage, ELF sampling by NL is an attractive method due to its limited invasiveness which allows repeated analyses, even performed in a home-based setting. In a longitudinal cohort study (ClinicalTrials.gov, Identifier: NCT02311140), we assessed changes of inflammatory mediators in 259 serially obtained nasal lavages taken up to every second day before and during therapy with ivacaftor from ten CF patients carrying a G551D mutation. Patients were trained to sample NL-fluid at home, to immediately freeze and transfer chilled secretions to centers. Neutrophil Elastase, Interleukins IL-1b, IL-6 and IL-8 in NL were quantified. During 8-12 weeks of ivacaftor-treatment, median values of IL-1b and IL-6 significantly declined 2.29-fold (2.97!1.30 pg/mL), and 1.13-fold (6.48!5.72 pg/mL), respectively. In parallel, sweat tests and pulmonary function improved considerably. This is the first study assessing changes of airway inflammation on a day-to-day basis in CF patients receiving a newly administered CFTR-modulator therapy. It proves a decline of airway inflammation during ivacaftor-therapy.

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