Developed by:
ASSOBRAFIR - Brazilian Association of Cardiorespiratory Physiotherapy and Physiotherapy in Intensive Care
GBEFC - Brazilian Cystic Fibrosis Study Group

FOREWORD

In recent years, the diagnostic methods and management approaches for cystic fibrosis have evolved significantly in Brazil and worldwide. After the Brazilian Cystic Fibrosis Study Group (GBEFC) was founded in 2003, much was added to the management of this disease.

Patient survival and quality of life significantly improved with the implementation of the Brazilian Cystic Fibrosis Registry and neonatal screening providing early diagnosis, the donation of chloridometers to several Brazilian centers, and the creation of work units by our group.

In 2017, the Brazilian guidelines for cystic fibrosis were published and now guide medical practices in our country. The aim was to provide all 46 Brazilian cystic fibrosis care centers registered with the GBEFC with updated information that is consistent with the Brazilian reality.

The possibility of making genotyping assay available for most registered patients also meant the possibility of effective treatment in the genesis of the disease and, in many cases, the possibility of significant advances in disease control.

However, new technologies and effective drug treatments, often expensive, are of little use if the bases of treatment are not given appropriate attention, as nutrition and physical therapy are crucial to the outcomes.

Respiratory physiotherapy will always be a robust pillar in cystic fibrosis management, and nothing will be able to replace it, under penalty of progressive loss of lung function and disease control.

Thus, the multidisciplinary unit - physical therapy, with the support of GBEFC and eminent Brazilian physical therapists, developed this excellent guide, now presented to all Brazilian health professionals who are dedicated to caring for patients with cystic fibrosis.
 

Dr. Norberto Ludwig Neto
President at the Brazilian Cystic Fibrosis Study Group (GBEFC)

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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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