A percepção do impacto da Covid-19 na vida de crianças e adolescentes com fibrose cística / Perceptions of the impact of the Covid-19 pandemic on the lives of children and adolescents with cystic fibrosis / La percepción del impacto de la Covid-19 en la vida de niños y adolescentes con fibrosis quística

Resumo Este artigo descreve o impacto da Covid-19 na vida de crianças e adolescentes com fibrose cística (FC) na percepção de seus responsáveis. Participaram deste estudo exploratório, por meio de um questionário, 27 responsáveis por crianças e adolescentes com FC. Pela técnica de análise temática do conteúdo na perspectiva de Minayo, verificamos três categorias analíticas: os impactos da Covid-19 no cuidado das crianças com FC; o impacto do isolamento social na vida dos pacientes e suas famílias; e as incertezas e os medos da pandemia. Inferimos que a pandemia intensificou as dificuldades vividas pelos pacientes e trouxe rupturas na trajetória de cuidado à saúde, desgastes físicos e psicológicos. O teleatendimento evitou a descontinuação do cuidado. Informação confiável evita a disseminação da Covid-19 e os fatores estressantes que geram medo e insegurança. Ações visando o acesso à informação devem ser desenvolvidas.(AU)

Abstract This article describes the impact of the Covid-19 pandemic on the lives of children and adolescents with cystic fibrosis (CF) from the perspective of their parents and guardians. We conducted an exploratory study with 27 parents and guardians of children and adolescents with CF using a questionnaire. Drawing on Minayo, we performed a thematic analysis of the responses to the questionnaires, identifying three core categories: the impacts of the Covid-19 pandemic on the care of children with CF; the impact of social isolation on the lives of patients and their families; and uncertainties and fears generated by the pandemic. The findings suggest that the pandemic aggravated difficulties experienced by the patients, disrupted health care, and caused both physical and mental strain. Teleconsultations avoid the discontinuation of health care. Reliable information can prevent the spread of Covid-19 and mitigate the stressors that generate fear and insecurity. Actions to improve access to information should be developed.(AU)

Resumen Este artículo describe el impacto de la Covid-19 en la vida de niños y adolescentes con fibrosis quística (FQ), según la percepción de sus responsables. En este estudio exploratorio participaron, por medio de un cuestionario, 27 responsables por niños y adolescentes con FQ. Por medio de la técnica de análisis temático del contenido desde la perspectiva de Minayo, verificamos tres categorías analíticas: los impactos de la Covid-19 en el cuidado de los niños con FQ; el impacto del aislamiento social en la vida de los pacientes y sus familias y las incertidumbres y miedos de la pandemia. Inferimos que la pandemia intensificó las dificultades vividas por los pacientes y causó rupturas en la trayectoria del cuidado de la salud, desgastes físicos y psicológicos. La teleatención evitó la interrupción del cuidado. La información confiable evita la diseminación de la Covid-19 y factores de estrés que generan miedo e inseguridad. Es necesario desarrollar acciones cuyo objetivo sea el acceso a la información.(AU)

Telemedicine of patients with cystic fibrosis during the COVID-19 pandemic.

OBJECTIVE: To describe then experience of implementing routine teleconsultations in respiratory physiotherapy at a reference center for Cystic Fibrosis (CF) in Rio de Janeiro / Brazil, during the COVID-19 pandemic. METHODS: Cross-sectional, descriptive, study with children and adolescents with CF. The sample was divided between participants and those who did not participate in the teleconsultations. The teleconsultations were multidisciplinary and carried out by videoconference or telephone, depending on the patient's availability. The sequence of care provided by the team was organized together with the professionals, so that everyone could carry out individual and sequential teleconsultations. Physiotherapy appointments were divided into two segments: teleconsultation and telemonitoring. Demographic and clinical data were collected. RESULTS: Among the 184 patients assisted in the center, 153 (83.2%) participated in the teleservices and, of these, 33 (21.6%) required telemonitoring; 31 (16.8%) patients did not participate in the teleconsultations for not answering the calls. There was no statistical difference between the group that participated or not in teleservices, nor among those who participated in teleconsultations and telemonitoring. The mean age of the studied population was 7.0±0.4 years. Regarding the CFTR gene mutation, 64.7% had at least one F508del allele and 30.9% of the sample had no pathogens in the sputum test. CONCLUSIONS: Most participants with CF participated in teleconsultations, highlighting the importance of remote assistance activities during the COVID-19 pandemic period. This strategy was considered as positive, and it may become permanent in the care of patients with CF.

FACTORS ASSOCIATED TO QUALITY OF LIFE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS.

OBJECTIVE: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). METHODS: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. RESULTS: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. CONCLUSIONS: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

Respiratory physical therapy techniques recommended for patients with cystic fibrosis treated in specialized centers.

BACKGROUND: Respiratory therapy is a part of the treatment of patients with cystic fibrosis (CF). However, there is no information about the main factors influencing the recommendation of the use of different techniques or devices by physical therapists from different specialized centers. OBJECTIVE: To determine the respiratory therapy techniques recommended for the treatment of patients with CF seen in specialized treatment centers in Brazil. METHODS: This is a descriptive study including a sample of Brazilian CF specialized treatment centers. Data on demographics, spirometric variables, and recommended respiratory therapy treatment techniques were collected. RESULTS: Twelve specialized treatment centers with a total of 974 patients were included (13.5±11.3 years old and FEV1 (%) 75.7±27.9). The most recommended techniques were huffing (61.1% of patients), high frequency oral oscillation (HFOO) (52.0%), and positive expiratory pressure (PEP) (45.3%). Most often, recommendation was to perform treatment once (54.8% of patients) or twice (34%) a day. There was great variability in the recommendation among the different states. When data were separated by age, there was a predominance of performing conventional and manual techniques in infants and preschool children. There were no significant variations according to pulmonary function. Based on the literature, techniques based on volume, huffing, and PEP were most prevalently performed in international centers. CONCLUSION: The most recommended treatment techniques for patients with CF in Brazil are huffing, HFOO, and PEP, followed by volume-based techniques. There were geographical variations in the preferred treatment techniques, as well as based on patient age, but not based on the level of pulmonary function.

Factors associated to quality of life in children and adolescents with cystic fibrosis / Fatores associados à qualidade de vida em crianças e adolescentes com fibrose cística

ABSTRACT Objective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

RESUMO Objetivo: Verificar associação entre qualidade de vida (QV), capacidade funcional e estados clínico e nutricional em crianças e adolescentes com fibrose cística (FC). Métodos: Estudo transversal incluindo pacientes de oito a 18 anos de idade com FC. A QV, a capacidade funcional, o estado nutricional e o estado clínico foram avaliados por meio do Questionário de Fibrose Cística; do teste de caminhada dos 6 minutos (TC6M) e da força de preensão manual (FPM); dos percentis de estatura para a idade e do índice de massa corporal (IMC) para a idade; e da prova de função respiratória, respectivamente. Para a análise dos dados, utilizaram-se os testes de correlação de Pearson e de Spearman e a regressão logística. Resultados: Participaram do estudo 45 pacientes com 13,4±0,5 anos, sendo 60% do sexo feminino, 60% colonizados por Pseudomonas aeruginosa e 57,8% apresentando pelo menos uma mutação F508del. Ao avaliar a percepção da QV, o domínio peso alcançou os escores mais baixos e o digestório, os mais altos. Na prova de função pulmonar, o volume expiratório forçado do primeiro segundo médio foi 77,3±3,3%, e o TC6M e a FPM apresentaram valores na faixa de normalidade. Observou-se associação da QV com a capacidade funcional, o estado nutricional e o estado clínico dos pacientes com FC. Conclusões: Os participantes do estudo apresentaram boas condições clínicas e valores satisfatórios de capacidade funcional e QV. Os achados reforçam que a avaliação da QV pode ser importante para a prática clínica, no manejo do tratamento.

Fatores influenciadores da percepção da qualidade de vida em crianças e adolescentes vivendo com fibrose cística: um estudo longitudinal / Factors influencing the perception of quality of life in children and adolescents living with cystic fibrosis: a longitudinal study

A fibrose cística (FC) é uma doença genética, autossômica recessiva e multissistêmica caracterizada por doença pulmonar crônica, insuficiência pancreática e altas concentrações de cloreto no suor. O caráter crônico da doença assim como a necessidade de tratamento extenso pode levar ao comprometimento da qualidade de vida destes pacientes. O objetivo deste estudo foi verificar a associação entre as características clínicas, nutricionais e funcionais e os domínios da qualidade de vida de crianças e adolescentes com FC, transversal e ao longo de 2 anos. Assim, realizou-se um estudo longitudinal oriundo da coorte de pacientes com FC atendidos no ambulatório de fisioterapia respiratória do Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Observou-se medidas relacionadas às características demográficas (sexo e idade), clínicas (prova de função pulmonar e mutação genética), nutricionais (peso, estatura, índice de massa corporal para a idade e estatura para a idade), capacidade funcional (distância percorrida no teste de caminhada dos 6 minutos-TC6M e força de preensão manual-FPM, obtida na dinamometria) e de qualidade de vida pelo questionário de qualidade de vida em fibrose cística (QFC-R). Os testes t de student não pareado e Mann-Whitney foram utilizados para a comparação entre grupos, de acordo com a distribuição dos dados, paramétrico ou não paramétrico, respectivamente. Utilizou-se os testes de correlação de Pearson e de Spearman e um modelo de regressão linear para avaliar os fatores associados com as variações percentuais dos domínios do QFC-R comuns a todas as faixas etárias, considerando a variação percentual destes domínios nos períodos de 2017 a 2019. A amostra da primeira avaliação, em 2017, foi composta por 38 crianças e adolescentes, sendo 55,30% do sexo feminino, com idade média de 13,16±2,94 anos. A amostra da primeira avaliação, em 2017, foi composta por 38 crianças e adolescentes, sendo 55,30% do sexo feminino, com idade média de 13,16±2,94 anos. Foi observado uma frequência de 63,10% de colonização por Pseudomonas aeruginosa e 65,79% de mutação F508del. No período de seguimento (2017 a 2019) não foram observadas diferenças significativas nestas características, mesmo com perda de 12 participantes. Na primeira avaliação, a prova de função pulmonar, mostrou um volume expiratório forçado do primeiro segundo (VEF1) médio de 77,3±3,3%, a distância percorrida no TC6M e a FPM apresentaram valores na faixa de normalidade. Neste momento, houve associação da qualidade de vida com a capacidade funcional, o estado nutricional e o estado clínico dos pacientes. Analisando todo o período estudado, a capacidade funcional relacionou-se com os domínios físico, social, alimentação e tratamento apresentando uma relação diretamente proporcional. A presença de colonização bacteriana no escarro influenciou negativamente o domínio respiratório. Diante dos resultados apresentados, conclui-se que as crianças e adolescentes constituintes da amostra apresentaram boas condições clínicas e valores satisfatórios tanto relacionados às características funcionais, nutricionais e clínicas, quanto à qualidade de vida. Entretanto, analisando comparativamente os dois períodos, observou-se que as alterações nas características clínicas, nutricionais e funcionais se associaram às mudanças nos domínios da qualidade de vida de crianças e adolescentes com FC. Com o aumento da sobrevida destes pacientes, se torna importante o conhecimento dos fatores influenciadores da qualidade de vida para que estes indivíduos envelheçam sem prejuízo social e emocional.

Cystic fibrosis (CF) is a genetic, autosomal recessive and multisystemic disease characterized by chronic lung disease, pancreatic insufficiency and high concentrations of chloride in sweat. The chronic characteristic of disease as well as the need for extensive treatment can lead to impairment of quality of life of these patients. The aim of this study was to verify the association between clinical, nutritional and functional characteristics and the domains of quality of life of children and adolescents with CF, both transversely and over 2 years. Thus, a longitudinal study was carried out from the cohort of CF patients treated at the respiratory physiotherapy outpatient clinic of the National Institute of Health for Women, Children and Adolescents Fernandes Figueira. Measures related to demographic (gender and age), clinical (pulmonary function test and genetic mutation), nutritional (weight, height, body mass index for age and height for age), functional capacity (distance covered in the 6-minute walk test-6MWT and handgrip strength-FPM, obtained from dynamometry) and quality of life using the cystic fibrosis quality of life questionnaire (QFC-R). The unpaired Student's t and Mann-Whitney tests were used for the comparison between groups, according to the data distribution, parametric or nonparametric, respectively. Pearson and Spearman correlation tests and a linear regression model were used to assess the factors associated with the percentage variations of the QFC-R domains common to all age groups, considering the percentage variation of these domains in the periods of 2017 to 2019. The sample of the first evaluation, in 2017, was composed of 38 children and adolescents, 55.30% female, with an average age of 13.16 ± 2.94 years. A frequency of 63.10% of colonization by Pseudomonas aeruginosa and 65.79% of F508del mutation was observed. In the follow-up period (2017 to 2019) there were no significant differences in these characteristics, even with the loss of 12 participants. In the first evaluation, the pulmonary function test showed an average forced expiratory volume of the first second (FEV1) of 77.3 ± 3.3%, the 6MWT and the HGS showed values in the normal range. At this time, there was an association between quality of life and functional capacity, nutritional status and clinical status of patients.Analyzing the entire period studied, functional capacity was related to the physical, social, food and treatment domains, presenting a directly proportional relationship. The presence of bacterial colonization in sputum negatively influenced the respiratory domain. In view of the results presented, it is concluded that the children and adolescents in the sample had good clinical conditions and satisfactory values, both related to functional, nutritional and clinical characteristics, and to quality of life. However, by comparatively analyzing the two periods, it was observed that changes in clinical, nutritional and functional characteristics were associated with changes in the domains of quality of life of children and adolescents with CF. With the increased survival of these patients, it becomes important to know the factors that influence quality of life so that these individuals age without social and emotional damage.