ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713
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ORIGINAL ARTICLE
Respiratory therapy: a problem among children and adolescents with cystic fibrosis.
Fisioterapia respiratória: um problema de crianças e adolescentes com fibrose cística
Taiane dos Santos Feiten1; Josani Silva Flores2; Bruna Luciano Farias3; Paula Maria Eidt Rovedder2; 3; Eunice Gus Camargo4; Paulo de Tarso Roth Dalcin2; 5; Bruna Ziegler1; 2
1. Serviço de Fisioterapia, Hospital de Clínicas de Porto Alegre, Porto Alegre (RS) Brasil.
2. Programa de Pós-Graduação em Ciências Pneumológicas, Faculdade de Medicina, Universidade Federal do Rio Grande do Sul, Porto Alegre (RS) Brasil.
3. Faculdade de Fisioterapia, Universidade Federal do Rio Grande do Sul,
Porto Alegre (RS) Brasil.
4. Serviço de Psiquiatria, Hospital de Clínicas de Porto Alegre, Porto Alegre (RS) Brasil.
5. Serviço de Pneumologia, Hospital de Clínicas de Porto Alegre, Porto Alegre (RS) Brasil.
Submitted: 26 March 2015.
Accepted: 4 August 2015.
Study carried out at the Hospital de Clínicas de Porto Alegre, Porto Alegre (RS) Brasil.
Correspondence to:
Bruna Ziegler. Travessa Miranda e Castro, 70/204, Santana, CEP 90040-280, Porto Alegre, RS, Brasil.
Tel.: 55 51 3335-1286. E-mail: brunaziegler@yahoo.com.br
Financial support: Bruna Luciano Farias is the recipient of a Young Investigator Grant from the Fundação de Amparo à Pesquisa do Rio Grande do Sul (FAPERGS, Foundation for the Support of Research in the state of Rio Grande do Sul).
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.
Resumo
Objetivo: Avaliar o grau de adesão autorrelatada às recomendações fisioterapêuticas em pacientes pediátricos (6-17 anos) com fibrose cística (FC) e determinar se os diferentes níveis de adesão se correlacionam com a função pulmonar, aspectos clínicos e qualidade de vida. Métodos: Estudo transversal no qual os pacientes e responsáveis responderam um questionário sobre a adesão à fisioterapia recomendada e um questionário da qualidade de vida em FC. Foram coletados dados demográficos, espirométricos e bacteriológicos, assim como a frequência de internações e resultados do escore clínico de Shwachman-Kulczycki (S-K). Resultados: Participaram 66 pacientes. As médias de idade, VEF1 (em % do previsto) e IMC foram, respectivamente, 12,2 ± 3,2 anos, 90 ± 24% e 18,3 ± 2,5 kg/m2. Os pacientes foram divididos em dois grupos: alta adesão (n = 39) e moderada/baixa adesão (n = 27). Não houve diferenças estatisticamente significativas para idade, sexo, renda familiar e escore clínico de S-K total na comparação dos dois grupos. Houve diferenças estatisticamente significativas entre os grupos alta adesão e moderada/baixa adesão, este último mostrando valores significativamente menores para o domínio "achados radiológicos" do escore clínico de S-K apresentou (p = 0,030), um maior número de hospitalizações (p = 0,004) e de dias de internação no último ano (p = 0,012), assim como menores escores para os seguintes domínios do questionário de qualidade de vida: emocional (p = 0,002), físico (p = 0,019), tratamento (p < 0,001), saúde (p = 0,036), social (p = 0,039) e respiratório (p = 0,048). Conclusões: A baixa adesão autorrelatada às recomendações fisioterapêuticas associou-se com piores achados radiológicos, maior número de hospitalizações e diminuição da qualidade de vida em pacientes pediátricos com FC.
Keywords: Cystic fibrosis; Patient compliance; Physical therapy modalities; Quality of life.
Palavras-chave: Fibrose cística; Cooperação do paciente; Modalidades de fisioterapia; Qualidade de vida.
INTRODUCTION
Cystic fibrosis (CF) is an autosomal recessive genetic disease characterized by chronic pulmonary infection, exocrine pancreatic insufficiency, and high concentrations of sweat electrolytes. Respiratory system changes constitute the primary cause of morbidity and mortality. (1-8) Data from the Cystic Fibrosis Foundation show that the life expectancy of individuals with CF was 37.8 years in 2012.(9)
The standard therapeutic approach to lung disease includes antibiotic therapy, airway clearance, physical exercise, mucolytics, bronchodilators, anti-inflammatory agents, nutritional support, and oxygen supplementation. (10,11) Outpatient treatment is provided by a multidisciplinary team every two or three months and is aimed at educating parents and patients regarding home treatment, monitoring disease progression, and improving treatment adherence, being adjusted as needed.(12)
Airway clearance techniques are considered essential components of the treatment of CF. In older children and adolescents, the use of techniques that prioritize their independence is encouraged.(13,14)
Flores et al.(15) studied 63 adult CF patients and showed that 40% had moderate to poor adherence to airway clearance therapies, a finding that was associated with the level of education and the severity of disease. The use of techniques preferred by patients was found to be associated with increased adherence, the level of agreement between physician-recommended therapy and self-reported adherence being highest for positive airway pressure.
In the pediatric population, poor treatment adherence is associated with patient dependence on their parents or caregivers to perform the recommended treatment techniques and a lack of understanding of the long-term implications of the disease.(16) The difficulty that parents have in establishing a treatment routine and the use of trial and error are barriers to treatment adherence, whereas anticipatory guidance provided by a multidisciplinary team can facilitate disease management.(17) Misconceptions, knowledge gaps, and errors regarding CF can have an impact on disease progression.(18) Few studies have examined adherence to therapy in patients with CF.(15,19,20)
A total of 2,669 patients are currently registered in the Brazilian CF Registry. Rio Grande do Sul ranks second among the Brazilian states with the highest number of CF patients, i.e., 356 individuals (13.3%). There are currently 1,918 pediatric patients (under 18 years of age) with CF in Brazil, pediatric patients accounting for 77.6% of all CF patients in the state of Rio Grande do Sul. The number of patients diagnosed with CF has increased annually since the advent of neonatal screening for CF by immunoreactive trypsin assay. Therefore, strategies to improve treatment adherence and prevent complications are increasingly necessary.(21)
The objective of the present study was to evaluate the level of self-reported adherence to physical therapy recommendations in pediatric CF patients and to ascertain whether the different levels of adherence correlate with lung function, clinical scores, and quality of life.
METHODS
Study design
This was a cross-sectional study of pediatric CF patients. The objective of the study was to evaluate the level of adherence to respiratory therapy.
Study population
Patients with CF were recruited by the Pediatric Pulmonology Team of the Hospital de Clínicas de Porto Alegre (HCPA), located in the city of Porto Alegre, Brazil. The study sample consisted of children and adolescents in the 6- to 17-year age bracket diagnosed with CF in accordance with a consensus statement.(2)
The study sample was consecutively selected from among all patients being followed and meeting the inclusion criteria. Data were collected in the period between May and October of 2014. The study was approved by the Research Ethics Committee of the HCPA (Protocol no. 14-0157), and the parents or legal guardians of all participants gave written informed consent.
We included clinically stable patients, clinical stability being defined as no hospitalizations or changes in the maintenance therapy regimen for at least 30 days. Patients with neurological changes resulting in difficulty in completing the questionnaires were excluded.
Measurements and instruments
After having accepted the invitation to participate in the study, patients and their parents or legal guardians gave written informed consent and headed for an area outside the ambulatory care environment.
Initially, patients (accompanied by their parents or legal guardians) completed a questionnaire regarding adherence to physical therapy recommendations, developed from a previous study(15) conducted at our referral center and consisting exclusively of objective questions, such as the following: "How many days a week and how many times a day do you perform physical therapy techniques for airway clearance?"; "How long does each session last?"; "What techniques do you perform?"; "What techniques do you like the most and what techniques do you like the least?"; "Who helps you?"; and "What are your reasons for not performing the recommended technique(s)?". Subsequently, patients completed a CF quality of life questionnaire previously validated for use in Brazil.(22) Both questionnaires were administered outside the ambulatory care environment by a professional who was not involved in the treatment of the patients. The patients themselves answered the questions, being helped by their parents or legal guardians when necessary.
Subsequently, a physical therapist who was a member of the treatment team answered the same questions, recording physical therapy recommendations for each patient.
A data collection form was used in order to gather information on the following: date; gender; age; ethnicity; family income; age at CF diagnosis; BMI; resting SpO2; sputum bacteriology; lung function; frequency of hospitalizations in the previous year (information collected from the electronic medical records of the participants, the day on which the questionnaires were administered being used in order to mark the end of the one-year period); and Shwachman-Kulczycki (S-K) clinical scores.(23)
The pulmonary function test results used in the present study were those of the spirometry tests requested in routine clinical care. Spirometry was performed at the Pulmonary Physiology Clinic of the HCPA Department of Pulmonology, with the patient in the sitting position. A v4.31a spirometer (Jaeger, Würzburg, Germany) was used, and all tests were performed in accordance with the technical acceptability criteria recommended in the Brazilian Thoracic Association guidelines for pulmonary function testing.(24) Values of FVC, FEV1, and FEV1/FVC were recorded. All parameters were also expressed as a percentage of the predicted values for age, height, and gender.(25)
Statistical analysis
Data were entered into a Microsoft Excel 2011 database, after which they were processed and analyzed with the Statistical Package for the Social Sciences, version 18.0 (SPSS Inc., Chicago, IL, USA).
On the basis of their answers to questions regarding adherence to physical therapy recommendations in the last two weeks, patients were divided into three groups: the high-adherence group (which included patients who always performed the airway clearance techniques recommended by the physical therapist and rarely failed to undergo the recommended number of sessions); the moderate-adherence group (which included patients who always performed the airway clearance techniques recommended by the physical therapist but often failed to undergo the recommended number of sessions); and the poor-adherence group (which included patients who never or almost never performed the airway clearance techniques recommended by the physical therapist and never or almost never underwent the recommended number of sessions). For analysis purposes, the high-adherence group was compared with the moderate/poor-adherence group.
Quantitative data were expressed as mean and standard deviation or as median (interquartile range). Qualitative data were expressed as frequency and proportion.
Continuous variables were compared by the independent sample t-test. Ordinal variables or continuous variables without normal distribution were compared by the Wilcoxon signed-rank test. Qualitative data were analyzed with the chi-square test, Yates' correction or Fisher's exact test being used when necessary. The kappa coefficient of agreement was used in order to assess the level of agreement between physician-recommended therapy and self-reported adherence. All statistical tests were two-tailed, and the level of significance was set at p < 0.05.
In order to calculate the sample size, proportions between the high-adherence and moderate/poor-adherence groups were taken into consideration. For an expected 40% proportion of patients with moderate/poor adherence(15)-a total amplitude of 0.25 and a 95% confidence interval being used-the minimum sample size was calculated to be 59.(26)
RESULTS
During the study period, the HCPA Pediatric Pulmonology Team followed a total of 109 patients with CF. Of those, 72 were between 6 and 17 years of age. Of those 72 patients, 66 were included in the study and evaluated. Two patients were excluded from the study: one was excluded because of pulmonary exacerbation requiring hospitalization, and the other was excluded because the parents did not allow the patient to participate in the study. Another 4 patients failed to return for follow-up during the study period.
On the basis of self-reported adherence, patients were divided into two groups for analysis: the high-adherence group (comprising 39 patients and accounting for 59% of the sample as a whole); and the moderate/poor-adherence group (comprising 27 patients and accounting for 41% of the sample as a whole).
Table 1 presents the general characteristics of the CF patients in the present study, by level of self-reported adherence to respiratory therapy. There were no statistically significant differences between the groups regarding age, gender, family income, or total S-K clinical scores. When the "radiological findings" domain of the S-K clinical score was analyzed separately, scores were found to be significantly lower in the moderate/poor-adherence group than in the high-adherence group (p = 0.030).
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