title: Respiratory therapy: a problem among children and adolescents with cystic fibrosis.
abstract: OBJECTIVE
  To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life.
  
  
  METHODS
  This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores.
  
  
  RESULTS
  We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048).
  
  
  CONCLUSIONS
  Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.
authors.affiliation: Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brasil.
authors.forename: Taiane Dos Santos
authors.initials: Tdos S
authors.lastname: Feiten
authors.affiliation: Faculdade de Medicina, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil.
authors.forename: Josani Silva
authors.initials: JS
authors.lastname: Flores
authors.affiliation: Faculdade de Fisioterapia, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil.
authors.forename: Bruna Luciano
authors.initials: BL
authors.lastname: Farias
authors.affiliation: Faculdade de Medicina, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil.
authors.forename: Paula Maria Eidt
authors.initials: PM
authors.lastname: Rovedder
authors.affiliation: Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brasil.
authors.forename: Eunice Gus
authors.initials: EG
authors.lastname: Camargo
authors.affiliation: Faculdade de Medicina, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil.
authors.forename: Paulo de Tarso Roth
authors.initials: Pde T
authors.lastname: Dalcin
authors.affiliation: Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brasil.
authors.forename: Bruna
authors.initials: B
authors.lastname: Ziegler
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000628:therapy*; D005260:Female; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D010349:Patient Compliance / Q000706:statistics & numerical data*; D011788:Quality of Life; D012138:Respiratory Therapy / Q000379:methods / Q000706:statistics & numerical data*; D057566:Self Report; D013147:Spirometry; D018709:Statistics, Nonparametric; D016896:Treatment Outcome