Realização:
ASSOBRAFIR - Associação Brasileira de Fisioterapia Cardiorrespiratória e Fisioterapia em Terapia Intensiva
GBEFC - Grupo Brasileiro de Estudos de Fibrose Cística

PREFÁCIO

Nos últimos anos, o diagnóstico e as condutas para o manejo da fi brose cística tiveram evolução significativa no mundo e no Brasil. Após a fundação do Grupo Brasileiro de Estudos de Fibrose Cística (GBEFC), no ano de 2003, muito se agregou na condução desta enfermidade.

Com a implantação do Registro Brasileiro de Fibrose Cística, da triagem neonatal, propiciando o diagnóstico precoce, a doação de cloridrômetros para diversos centros brasileiros e a criação de núcleos de trabalho pela nossa entidade, a sobrevida e qualidade de vida dos pacientes melhoraram signifi cativamente.

Em 2017, foram publicadas as Diretrizes Brasileiras para Fibrose Cística, que vieram nortear as condutas médicas em nosso país, com o objetivo de fornecer a todos 46 centros brasileiros de referência em fi brose cística, cadastrados no GBEFC, informações atualizadas e condizentes com a realidade brasileira.

A possibilidade de ter a genotipagem disponível, para a maioria dos pacientes registrados, também, trouxe a possiblidade de tratamento efetivo na gênese da enfermidade, e, em muitos casos, com possibilidade de ganhos expressivos no controle da doença.

Mas, pouco adiantam, as novas tecnologias e tratamentos medicamentosos efetivos, frequentemente dispendiosos, se não forem cuidadas as bases do tratamento, onde a nutrição e a fisioterapia são fundamentais no resultado final.

A fisioterapia respiratória sempre será um pilar robusto no controle da doença, e nada poderá substituí-la, sob pena de perdas progressivas da função pulmonar e descontrole da enfermidade.
Desta maneira, o núcleo multidisciplinar - fisioterapia, com o apoio do GBEFC e de eminentes fisioterapeutas brasileiros, desenvolveu esta excelente obra, ora presenteada a todos profissionais brasileiros, que se dedicam a cuidar de pacientes com fibrose cística.

Dr. Norberto Ludwig Neto
Presidente do Grupo Brasileiro de Estudos em Fibrose Cística (GBEFC)

Faça o download do documento completo clicando aqui

Schindel CS, Hommerding PX, Melo DA, Baptista RR, Marostica PJ, Donadio MV.

J Pediatr. 2015 Mar;166(3):710-6.e2. doi: 10.1016/j.jpeds.2014.12.001. Epub 2015 Jan 13.

Abstract

OBJECTIVE
To evaluate postural changes and the distribution of plantar pressures in patients with cystic fibrosis (CF). We also sought to evaluate the effects of an educational guideline for physical activity on body posture in children and adolescents with CF.

STUDY DESIGN
This was a 2-phase study of individuals between age 7 and 20 years. Phase I was a cross-sectional study in which healthy subjects were selected for postural evaluation and baropodometry, aiming to perform a later comparison with patients with CF. In phase II, we performed a randomized controlled clinical trial to assess the influence of the exercise guideline on the postural changes. Patients were assigned to 2 groups: control and intervention. The intervention consisted of a handbook with instructions for aerobic exercise and stretching. Main outcomes were postural abnormalities, plantar pressure distribution, and lung function.

RESULTS
In phase I, 34 patients with CF and 34 healthy matched individuals were included. No significant baseline differences were identified. Children with CF presented more postural deviations compared with healthy subjects (P < .05), as to alignment of the head, shoulder girdle, and pelvis, increased cervical lordosis, and lateral chest distance. In phase II (n = 34), there were no baseline differences between groups. The intervention caused (P < .05) a decrease in cervical lordosis, thoracic kyphosis, lumbar lordosis, lateral chest distance, and abdominal protrusion, as well as in the baropodometric mean pressure and contact area.

CONCLUSIONS
Children and adolescents with CF present postural changes when compared with healthy individuals. The educational guideline for exercise practice helped to improve posture, preventing the progression of some postural disorders.

Copyright © 2015 Elsevier Inc. All rights reserved.

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McIlwaine MP1, Alarie N, Davidson GF, Lands LC, Ratjen F, Milner R, Owen B, Agnew JL.

Thorax. 2013 Aug;68(8):746-51. doi: 10.1136/thoraxjnl-2012-202915. Epub 2013 Feb 13.
 

Abstract

BACKGROUND
Positive expiratory pressure (PEP) is the most commonly used method of airway clearance (AC) in Canada for patients withcystic fibrosis (CF) whereas, in some countries, high frequency chest wall oscillation (HFCWO) is the preferred form of AC. There have been no long-term studies comparing the efficacy of HFCWO and PEP in the CF population.

OBJECTIVES
To determine the long-term efficacy of HFCWO compared with PEP mask therapy in the treatment of CF as measured by the number of pulmonary exacerbations (PEs).

METHODS
A randomised controlled study was performed in 12 CF centres in Canada. After a 2-month washout period, subjects were randomised to perform either HFCWO or PEP mask therapy for 1 year.

RESULTS
107 subjects were enrolled in the study; 51 were randomised to PEP and 56 to HFCWO. There were 19 dropouts within the study period, of which 16 occurred prior to or at the time of randomisation. There were significant differences between the groups in the mean number of PEs (1.14 for PEP vs 2.0 for HFCWO) and time to first PE (220 days for PEP vs 115 days for HFCWO, p=0.02). There was no significant difference in lung function, health-related quality of life scores or patient satisfaction scores between the two groups. PEP masktherapy required a shorter treatment time.

CONCLUSIONS
The results of this study favour PEP and do not support the use of HFCWO as the primary form of AC in patients with CF.

Clinical trial registration number: NCT00817180.

Keywords: Cystic Fibrosis; Health Economist.

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