Adherence to inhalation therapy and quality of life in children with cystic fibrosis: a cross-sectional study


  • Thaís Peruch Programa de Pós-Graduação Medicina: Ciências Pneumológicas, UFRGS; Serviço Pneum
  • Taiane dos Santos Feiten
  • Josani da Silva Flores
  • Paulo de Tarso Roth Dalcin
  • Bruna Ziegler


Cystic fibrosis, adherence, nebulization, quality of life


Introduction: Inhalation therapy is a crucial part of the cystic fibrosis (CF) treatment regimen. Drugs that assist in mucociliary clearance and inhaled antibiotics are used by most patients. Methods: This is a cross-sectional study where patients with CF and their caregivers answered questionnaires regarding their adherence to inhalation therapy and QoL. Demographic, spirometric, and bacteriological data, as well as S-K scores and hospitalization frequencies were also collected. Results: We included 66 patients in this study; participants had a mean age of 12.3 years and Z-scores of -1.4 for forced expiratory volume in 1 second and 48.6 for body mass index. Patients were divided into 2 groups according to their self-reported adherence to inhalation therapy: high adherence (n = 46) and moderate/low adherence (n = 20). When comparing both groups, there was no statistically significant differences in age, sex, family income, and S-K score (p > 0.05). The high-adherence group had had shorter hospitalization periods in the previous year (p = 0.016) and presented better scores in the following domains of the QoL questionnaire: emotion (p = 0.006), eating (p = 0.041), treatment burden (p = 0.001), health perception (p = 0.001), and social (p = 0.046). Conclusions: A low self-reported adherence to inhalation therapy recommendations was associated with longer hospitalizations in the previous year and with a decrease in QoL in pediatric patients with CF.


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Peruch T, Feiten T dos S, Flores J da S, Dalcin P de TR, Ziegler B. Adherence to inhalation therapy and quality of life in children with cystic fibrosis: a cross-sectional study. Clin Biomed Res [Internet]. 3º de fevereiro de 2021 [citado 27º de setembro de 2022];40(2). Disponível em:



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