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

Thaís Peruch, Taiane dos Santos Feiten, Josani da Silva Flores, Paulo de Tarso Roth Dalcin, Bruna Ziegler


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.


Cystic fibrosis; adherence; nebulization; quality of life

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