Background: Studies have shown that sleep disorders occur in cystic fibrosis (CF) patients and may be present before daytime clinical manifestations.Objectives: To evaluate the presence of sleep disorders among children and adolescents with CF, attempting to identify associations with pulmonary function, nutritional status, days in hospital, and days taking antibiotics.Methods: Individuals with a diagnosis of CF aged between 6 and 18 years were included. Information on sociodemographic, clinical profile, history of hospitalizations, and use of antibiotics in the last year were collected. Spirometry, bioimpedance, and polysomnography were performed. The presence of nocturnal hypoxemia and obstructive sleep apnea syndrome (OSAS) were evaluated and participants divided according to their presence.Results: Thirty-one patients were included. The prevalence of OSAS was 32.3% and nocturnal hypoxemia was 29.0%. Average nocturnal peripheral oxyhemoglobin saturation (SpO 2 ) correlated (P < .001) with forced vital capacity (r = .55) and forced expiratory volume in the first second (r = .62). The higher the percentage of total sleep time (TST) with SpO 2 less than 90%, the lower the pulmonary function.Individuals with OSAS and nocturnal hypoxemia had lower spirometric values compared to patients without these disorders, but the nocturnal hypoxemia group also had lower Shwachman-Kulczycki score, longer hospitalization time and antibiotic use. TST with SpO 2 less than 90% was associated with length of hospitalization (r 2 = .53). Conclusion:Children and adolescents with CF have sleep disorders, including OSAS (32.3%) and nocturnal hypoxemia (29%). Individuals with nocturnal hypoxemia presented lower lung function, worse clinical score, and higher morbidity. TST with SpO 2 less than 90% was associated with length of hospitalization.

Objectives To investigate the adherence and the self‐reported barriers to general and respiratory exercises reported by individuals with cystic fibrosis (CF). Study Design An exploratory, experimental study. Methods Community‐dwelling individuals aged 16 years and over, diagnosed with CF, who were accompanied in referral centers were included. Information regarding adherence to exercises was obtained by a questionnaire and reported as a ratio between prescribed exercises and self‐reported adherence. The weekly frequency was used to verify adherence to exercise initiation, and the amount of session duration concluded was used to verify adherence to exercise duration. Values above 0.70 were considered as high adherence. Eight demographic and clinical factors were examined to explore their relationships with adherence, and the barriers to exercises were also collected by questionnaire. Results Thirty‐four participants met the inclusion criteria. Overall, adherence to exercise initiation was 0.40 (standard deviation [SD] = 0.3) for general exercises and 0.63 (SD = 0.4) for respiratory exercises. Adherence to exercise duration was 0.76 (SD = 0.4) for general exercises and 0.73 (SD = 0.4) for respiratory exercises. Forced vital capacity (r = 0.39; P = .02) was associated with adherence to the duration of general exercises, and body mass index (r = −0.33; P = .05) was associated with adherence to the duration of respiratory exercises. The main reported barriers were lack of interest, motivation and time, tiredness, noncommitment, and do not recognize the benefits of exercises. Conclusions Individuals with CF minded completing the sessions of prescribed exercises once they have initiated it, but most of the days they did not practice general or respiratory exercises.

This study explored the evidence of validity of internal structure of the 12-item Functional Assessment of Chronic Illness Therapy—Spiritual Wellbeing Scale (FACIT-Sp-12) in Brazilian adolescents with chronic health conditions. The study involved 301 Brazilian adolescents with cancer, type 1 diabetes mellitus, or cystic fibrosis. Exploratory Factor Analysis (EFA), Confirmatory Factor Analysis (CFA), and Item Response Theory (IRT) were used to test the internal structure. Reliability was determined with Cronbach’s Alpha and McDonald’s Omega. The EFA suggested a one-dimensional scale structure in contrast to the original 2-factor model or the 3-factor model which were not reproduced in the current CFA. All quality indicators for the EFA one-factor exceeded the required criteria (FDI = 0.97, EAP = 0.97, SR = 3.96 and EPTD = 0.96, latent GH = 0.90. and the observed GH = 0.85). The FACIT-Sp-12 for adolescents yielded strong evidence for a 1-factor model and with good reliability.

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ResumoObjetivo: Identificar os principais fatores relacionados à qualidade de vida de crianças e adolescentes com fibrose cística. Métodos: Estudo transversal, com indivíduos com fibrose cística, entre 7 e 18 anos, de um Centro de Referência Estadual. A qualidade de vida foi avaliada com o Cystic Fibrosis Questionnaire. Testes usados na análise estatística: Mann-Whitney e Correlação de Spearman. Nível de significância adotado: 5%. Resultados: Na percepção dos pacientes, os domínios da qualidade de vida com piores médias foram imagem corporal (64,3±29,6) e peso (47,6±42,4). Na percepção dos responsáveis, peso também apresentou a pior média (52,8±43,7). O impacto dos tratamentos na qualidade de vida foi maiora partir dos 14 anos (p=0,012), e o impacto da alimentação foi maior entre menores de 14 anos (p=0,04). Indivíduos brancos apresentaram melhores escores do que negros/pardos no domínio imagem corporal (p=0,049). Houve correlação positiva moderada entre pIMC/I e o domínio respiratório e correlação positiva forte entre pIMC/I e imagem corporal. Conclusões: A correlação entre pIMC/I e imagem corporal sugere que o baixo-peso afeta negativamente a imagem corporal, o que merece atenção, pois imagem corporal e peso foram os domínios com piores pontuações na percepção dos pacientes, sendo que o peso também teve a pior pontuação na percepção dos responsáveis. Além disso, o baixo-peso aumentou o impacto dos sintomas respiratórios na qualidade de vida. O impacto dos tratamentos aumentou com a idade, enquanto o da alimentação diminuiu com a idade. Também se observou impacto da raça/cor no domínio imagem corporal da qualidade de vida.DOI: 10.12957/demetra.2018.32295