RESUMO
INTRODUCTION: Cystic Fibrosis (CF) is the most frequent chronic hereditary disease in the white race. Although the impact on the quality of life of this disease is significant, there are no validated instruments in the Chilean population to measure it. OBJECTIVE: To carry out a cultural and linguistic adaptation and validate the content and reliability of the CFQ-R Cystic Fibrosis Questionnaire, Spanish version 2.0. PATIENTS AND METHOD: The process was carried out in two stages. The first stage consists of an ins trumental design to adapt it culturally and linguistically, evaluate content validity by consulting ex perts, and test the comprehension of the questionnaire in patients and parents through qualitative interviews and a focus group. In the second stage with an observational and cross-sectional design in a sample of 122 people with CF or their caregivers, the behavior of the questionnaire was analyzed using descriptive statistics and Cronbach's alpha for reliability. RESULTS: Stage 1: the instrument in its three versions is considered valid with Lynn's index > 0.8 and Validity Coefficient > 0.7. Stage 2: The adolescent/adult and parent/caregiver versions obtain Cronbach's a > 0.7 and an average > 3 in most dimensions. CONCLUSION: The questionnaire is adapted and validated in the Chilean population and requires minor modifications. This version is reliable, valid, and allows the assessment of the quality of life in people with CF. It is suggested to increase the sample for the analysis of construct validity with a larger number of patients.
Assuntos
Fibrose Cística , Qualidade de Vida , Adolescente , Adulto , Criança , Chile , Estudos Transversais , Fibrose Cística/diagnóstico , Humanos , Reprodutibilidade dos Testes , Inquéritos e QuestionáriosRESUMO
The nocturnal noninvasive ventilation (NNIV) at home is often used in chronic respiratory failure (CRF) and obstructive sleep apnea (OSA) and, less frequently, in central hypoventilation and cystic fibrosis. We present a 6 year period (1996-2002) retrospective review of 10 children treated with noninvasive respiratory support describing indications, type of NNIV and the follow-up. Patient's age ranged from 1 to 13 years old; 6 were females and 4 males. Six had a neuromuscular disease, three a cerebral palsy and one an Arnold Chiari Syndrome Type II. Nine children presented kyphoscoliosis. NNIV was indicated in the following conditions: compensated CRF (n = 3), decompensated CRF (n = 1), acute respiratory failure with underlying neuromuscular diseases and unclear diagnosis of CRF (n = 2); OSA with pharyngeal collapse (n = 3) and central hypoventilation (n = 1). Two patients had moderate to severe pulmonary restriction and subnormal maximal airway pressures. Six patients were discharged with CPAP and four with BiPAP after being hospitalized during 3 to 90 days. In average, the follow-up lasted 2 years. Three children had training of their respiratory muscles. One patient needed a tracheostomy, the other nine improved: five without re-admissions and four reduced them. Five patients went back to school. One patient with congenital myopathy and kyphoscoliosis, surgically treated, improved her pulmonary function and the 6 minutes walking test. Two patients used NNIV irregularly and other two stopped it after one year. No deaths were reported. In our experience NNIV at home lessens admissions and promotes a better social and school re-integration. BiPAP is useful in restrictive conditions meanwhile CPAP is helpful in upper airway obstruction with OSA.
La asistencia ventilatoria no invasiva domiciliaria nocturna (AVNIDN) se indica en insuficiencia respiratoria crónica (IRC), síndrome de apnea obstructiva del sueño (SAOS) y ocasionalmente por hipoventilación central y fibrosis quística. Se hace una revisión retrospectiva de 10 pacientes sometidos a esta técnica de soporte ventilatorio no invasivo durante un periodo de 6 años (1996-2002), describiendo las indicaciones, tipo de AVNIDN y el seguimiento. Sus edades fluctuaron entre 1 a 13 años; 6 mujeres y 4 hombres. Seis pacientes tenían enfermedad neuromuscular; 3 parálisis cerebral y uno Arnold Chiari tipo II. Nueve presentaban xifoescoliosis. Las indicaciones fueron: IRC estable 3 pacientes; posterior a IRC descompensada 1; luego de insuficiencia respiratoria aguda en enfermos neuromusculares sin diagnóstico claro de IRC, 2 pacientes. SAOS secundaria a faringomalacia en 3 y por hipoventilación central uno. Dos presentaban restricción pulmonar moderada a severa y presiones máximas de vía aérea subnormales. Seis pacientes recibieron CPAP y 4 Bipap. El tiempo de hospitalización previo al alta fue de 3 a 90 días y la duración promedio del seguimiento fue 2 años. Tres recibieron entrenamiento muscular respiratorio. No hubo mortalidad; un paciente fue traqueostomizado. Nueve mejoraron, 5 no tuvieron rehospitalizaciones y 4 las disminuyeron. Cinco pacientes asisten al colegio. Un paciente con miopatía congénita y artrodesis mejoró sus pruebas funcionales respiratorias y la distancia recorrida en 6 minutos. Dos usaron irregularmente la AVNIDN y dos suspendieron tratamiento luego de un año. En nuestra experiencia, la AVNID minimizó las hospitalizaciones y promovió la reinserción social y escolar. La modalidad Bipap fue útil en patologías restrictivas y el CPAP en obstrucción de vía aérea superior/SAOS.
