Nutritional rehabilitation for children with cystic fibrosis: Single center study.

BACKGROUND: Cystic fibrosis (CF) is an inherited disorder that causes severe damage to multiple organs in the body. Nutritional management has a dramatic effect on growth and survival in CF patients. This study aimed to assess the nutritional status of Egyptian pediatric patients with CF before and after proper nutritional counseling and supplementation. METHODS: This is an interventional observational study conducted on fifty children with CF recruited from CF clinic, Children's Hospital, Cairo University. The patients were subjected to history taking, clinical examination, full nutritional assessment, including signs of vitamins deficiencies, Subjective global assessment (SGA), anthropometric measurements, laboratory assessment (complete blood count, kidney, liver function tests and Prealbumin as a nutritional marker). After the initial assessment all the patients were subjected to a proper nutritional plan plus vitamin and mineral supplementations then reassessed them after 3 months. RESULTS: SGA showed that 60% of the patients had severe malnutrition, 62%, 78%, and 48% of the patients were below -2 Z score for height, weight, and BMI respectively which decreased to 45%, 40%, and 32% after nutritional supplementation. 43% of the patients were anemic, 29% were hypoalbuminemic, and 80% had low serum Prealbumin levels. After 3 months of follow up, only 17% had anemia, 11% had hypoalbuminemia, and 41% had low serum Prealbumin. Compliance was detected in 76% of caregivers during the follow up. CONCLUSION: CF patients had considerable impairment in their nutritional status. Proper caloric intake and vitamin supplementation had a valuable impact on their growth and prognosis.

Consenso chileno para la atención integral de niños y adultos con fibrosis quística / Chilean consensus for the integral care of children and adults with cystic fibrosis

Cystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.

La Fibrosis Quística (FQ) es la enfermedad hereditaria de pronóstico reservado más frecuente en raza blanca. Desde el año 2003, Chile inicia un Programa Nacional de Fibrosis Quística, de carácter integral, dirigido por la Unidad de Salud Respiratoria del Ministerio de Salud. Hasta la fecha, los principales resultados del Programa registran una significativa mayor sobrevida (promedio 27 años) y una significativa reducción en la edad de diagnóstico de los pacientes ingresados desde 2006 en adelante. El acceso a la canasta GES (Garantías Explícitas en Salud), la implementación del tamizaje neonatal en algunas regiones del país, la organización y la constitución de equipos entrenados en FQ de diversas especialidades, ha contribuido a mejorar los resultados. Si bien las principales manifestaciones son del aparato respiratorio y digestivo, el carácter multisistémico de la FQ obliga a conocer los distintos aspectos involucrados en su manejo, a fin de optimizar los resultados del tratamiento y los recursos invertidos, tanto en el sector público como privado. Este documento es una revisión y actualización sobre los principales aspectos del diagnóstico, seguimiento y tratamiento de las manifestaciones respiratorias y no respiratorias de la FQ.

Effects of inspiratory muscle training on postural stability, pulmonary function and functional capacity in children with cystic fibrosis: A randomised controlled trial.

BACKGROUND: Previous research has found conflictive results regarding the benefits of inspiratory muscle training (IMT) for cystic fibrosis (CF) patients. Also, involvement of postural stability is a rising concern in chronic lung diseases but its role in CF patients is poorly understood. Our aim was to investigate the effects of IMT in CF patients as well as analysing the factors which may be related to postural stability. METHODS: Thirty-six children aged between 8 and 18 years with CF were randomly allocated to either "comprehensive chest PT" group (PT) or "IMT alongside comprehensive chest PT" group (PT+IMT). Both groups trained for 8 weeks. Dynamic and static postural stability tests on Biodex Balance system®, spirometry, respiratory muscle strength and 6-min walk distance (6MWD) was assessed at baseline and after 8 weeks of training. Determinants of postural stability was also analysed on baseline values. RESULTS: Maximum expiratory pressure (MEP) was found to be an independent predictor for overall limits of stability (LOS) score explaining %26 of variance (R = 0.514, p = 0.003). Overall LOS score, FVC, FEV1, peak expiratory flow, MEP and 6MWD significantly improved in both groups, with no significant differences between groups. Maximum inspiratory pressure (MIP) also improved in both groups but the magnitude of improvement in MIP was greater in PT+IMT group (38 cmH2O vs 13 cmH2O; p < 0.001). CONCLUSIONS: Combining IMT with chest PT failed to provide further improvements, except for MIP, suggesting that a comprehensive chest PT program may be individually effective in improving overall LOS score, spirometry, respiratory muscle strength and 6MWD. TRIAL REGISTRATION: www.ClinicalTrials.gov; registration number: NCT03375684.

Actualización en kinesiología respiratoria en pacientes con fibrosis quística / Update of chest physiotherapy in cystic fibrosis patients

Chest physiotherapy is an essential component of cystic fibrosis treatment. Its aim is to delay lung deterioration and preserve physical function, improving quality of life and long-term results. It is possible to proceed early, even when the child is asymptomatic.The therapist must be a competent professional, able to participate in a health care team and to make therapeutic decisions. Currently, the therapist is responsible for optimizing mucociliary clearance, but in, addition, their work has extended to education, aerosol therapy, physical activity recommendations, non-invasive ventilatory support, oxygen therapy.This article provides a general description of the chest therapist proceedings related to the integral management of cystic fibrosis

La kinesioterapia respiratoria es parte del tratamiento integral de la Fibrosis Quística. Con ella, apuntamos a retrasar el deterioro pulmonar y preservar la función física, mejorando la calidad de vida y los resultados a largo plazo. Es posible actuar de manera precoz, incluso cuando el niño es asintomático.Es de importancia que el kinesiólogo que trate a pacientes con Fibrosis Quística sea un profesional competente, con capacidad de formar parte de un equipo de salud y participar activamente en las decisiones terapéuticas requeridas, ya que en la actualidad, el kinesiólogo ha ampliado su labor, ya no solo circunscribiéndose a las técnicas manuales e instrumentales para optimizar el drenaje bronquial. Este articulo entrega una descripción general del actuar del kinesiólogo en el manejo integral del paciente con Fibrosis Quística

Kinesiología en pacientes con fibrosis quística en atención primaria de salud / Physioterapy for cystic fibrosis patients in primary health care

Physiotherapy is a mainstay treatment of cystic fibrosis (CF) patients. It has been adapted to the greater survival of patients and is partly responsible for this achievement. The scope of the physiotherapist in this condition should not focus only on “bronchial hygiene”, because as CF is a systemic disease. It must consider the individual as a whole, and therefore include: education and use of aerosol therapy, encouragement and advice in general physical activity and sports, posture and mobility, noninvasive ventilatory assistance, rehabilitation and transplantation. The physiotherapist must be highly trained, have deep knowledge of the disease and the ability to integrate a multidisciplinary team to determine the best therapeutic actions required by each patient. This article shows the physiotherapist’s main actions, as part of a multidisciplinary team to treat CF patients. However, no reference is made to post transplant physiotherapy, situation that requires a particular approach that is beyond the scope of this manuscript.

La kinesiología respiratoria se ha constituido un pilar fundamental del tratamiento y manejo de la enfermedad, la cual se ha adaptado a los cambios que ha implicado la mayor sobrevida de los pacientes con fibrosis quística (FQ) y en parte es responsable de este logro. El campo de acción del kinesiólogo en esta patología no sólo debe estar enfocando en la “higiene bronquial”, ya que como se trata de una patología sistémica debe considerar al individuo en su totalidad, por lo el enfoque debe incluir también: educación y uso de la aerosol terapia, incentivo y asesoramiento de en la actividad física general y deportes, postura y movilidad, asistencia ventilatoria no invasiva, rehabilitación y trasplante. El kinesiólogo que trate paciente con fibrosis quística, debe ser un profesional altamente capacitado, con conocimientos profundos de la enfermedad con la capacidad de integrar un equipo multidisciplinario que determine las mejores actuaciones terapéuticas requeridas por cada paciente en forma particular. En este artículo se han abordado las principales actuaciones del profesional Kinesiólogo integrante de un equipo multidisciplinario requerido para el tratamiento de los paciente con FQ, sin embargo no se ha abordado el manejo kinésico en paciente trasplantado que requiere un enfoque particular que escapa a la intención de esta publicación.

Meditative movement for respiratory function: a systematic review.

BACKGROUND: Meditative movement, such as tai chi, yoga, and qi gong, may benefit people with cystic fibrosis (CF), as a form of gentle exercise incorporating meditation, breathing, and relaxation. Respiratory function is the most common issue in CF. In this systematic review we synthesized the evidence on the effect of meditative movement on respiratory function in patients with CF. METHODS: We searched Chinese and English language databases with terms relating to tai chi/yoga/qi gong, and respiratory function/cough/dyspnea. Articles were screened and selected by 2 researchers. We included controlled studies published in English or Chinese after 1980, and extracted data using a specially designed spreadsheet. Two researchers independently evaluated study quality and reporting, using 3 standardized checklists. Meta-analysis was not possible due to heterogeneous methods. RESULTS: We found 1,649 papers, included 43 (30 in English, 13 in Chinese), 23 of which were randomized controlled trials, and 20 were non-randomized trials. No studies were concerned with CF. Eleven studies included patients with respiratory disorders, and 27 included healthy people. Very few studies were high quality. The main problems with the randomized controlled trials was the randomization and non-random and/or poorly reported sampling. The main problems with the non-randomized studies were poor reporting of samples and non-equivalent groups. Although no clinically important changes were found, meditative movement may improve FEV1 in healthy people, compared to no treatment/exercise (the intervention groups showed effect-size changes from 0.07 to 0.83), but meditative movement did not appear to affect FEV1/FVC in subjects with COPD. Key study limitations were: poor reporting of sampling or methods; inadequate sample size; non-randomized design; inadequate description of randomization; randomization by center; no blinding; lack of reporting of important aspects of meditative movement; and short-term follow-up. CONCLUSIONS: The available evidence does not support meditative movement for patients with CF, and there is very limited evidence for respiratory function in healthy populations. The available studies had heterogeneous populations and provided inadequate sampling information, so clinically relevant conclusions cannot be drawn. Well powered, randomized studies of meditative movement are needed.

Interval exercise training in cystic fibrosis -- effects on exercise capacity in severely affected adults.

The aims of the present study were to investigate the effects of IT on lung function power (P) and oxygen uptake (VO2) at peak performance (peak) and ventilatory anaerobic threshold (VAT) in CF patients who were unable to participate in a standard exercise program (SEP) and to compare these IT responses with corresponding effects in CF patients performing SEP. 20 patients (FEV1 25.5 ± 7.5%; pred; SpO2 < 90% at rest or P lower than 0.3 W/kg) who were unable to participate in SEP were allocated to IT (5 × 20 min weekly). 23 patients (FEV1 31.6 ± 4.2%; p < 0.05) did 5 × 45 min per week of SEP. Lung function remained unchanged in both groups. VO2peak and PVAT increased in both groups (p < 0.05). However, only after the SEP an increase in Ppeak (p < 0.05) and only after IT a higher VO2VAT (p < 0.05) were found. Compared to SEP, IT improved submaximal exercise capacity to a greater extent whereas responsiveness on peak performance was higher in SEP. This seems to indicate a specific potential of IT for positive peripheral muscular adaptations in spite of diminishing potential of pulmonary improvement. IT represents an alternative, effective and safe training regimen with patients with CF and severe lung disease, with a greater potential than SEP.

Exercise with incorporated expiratory manoeuvres was as effective as breathing techniques for airway clearance in children with cystic fibrosis: a randomised crossover trial.

QUESTION: : Can a session of exercise with incorporated expiratory manoeuvres substitute for a session of breathing techniques for airway clearance in children with cystic fibrosis? Are children with cystic fibrosis as co-operative and satisfied with the exercise regimen as with the breathing techniques?. DESIGN: Randomised, cross-over trial with concealed allocation and intention-to-treat analysis. PARTICIPANTS: 34 children with cystic fibrosis in a stable clinical state. INTERVENTIONS: Participants underwent two 20-min airway clearance interventions on two scheduled clinic days: one involving three bouts of various whole-body exercise modalities each followed by independent expiratory manoeuvres, and the other involving breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique. OUTCOME MEASURES: Wet weight of expectorated sputum, change in lung function, co-operation with treatment, perceived treatment quality, and satisfaction with treatment were all assessed after each intervention. RESULTS: The wet weight of sputum after exercise was 0.6g higher after the exercise intervention, which was not statistically or clinically significant (95% CI -0.2 to 1.4). However, lung function and participant satisfaction with the treatment were both significantly better after the exercise intervention. Co-operation with treatment and perceived treatment quality were equally high for each intervention. CONCLUSION: A session of various whole-body exercises interspersed with independent expiratory manoeuvres could be an acceptable substitute for a session of breathing control, thoracic expansions with manual expiratory compressions, and the forced expiratory technique in children with mild cystic fibrosis lung disease.

Inpatient paediatric rehabilitation in chronic respiratory disorders.

Inpatient pulmonary rehabilitation programs have evolved from tuberculosis sanatoriums to modern medical centres providing standardized comprehensive care in a multidiciplinatory environment. Goals of rehabilitation programs for children and adolescents include restoration of professional activity, improvement of health condition, compliance and disease management as well as restoration of quality of life. Eligibility for an intervention is assessed by defined social and medical criteria. Comprehensive pulmonary rehabilitation programs provide a wide range of health care recourses, including diagnostic procedures, specific medical care, educational interventions and a multiprofessional team. Paediatric rehabilitation programs for chronic respiratory diseases, such as asthma or cystic fibrosis, have been shown to reduce symptoms, increase aerobic fitness and physical strength, improve pulmonary function and inflammation and enhance compliance, self-management, quality of life and psychological symptoms. Regional climatic effects have demonstrated an additional positive effect on the rehabilitation outcome. In addition, first evidence suggests an overall reduction of health care costs.

Effects of chest physiotherapy and aerobic exercise training on physical fitness in young children with cystic fibrosis.

BACKGROUND: Cystic fibrosis is a multisystem disease where the main problems are existing in the respiratory system. Aerobic exercise programs are effective in increasing physical fitness and muscle endurance in addition to chest physiotherapy. OBJECTIVE: The aim of this study was to evaluate the effects of chest physiotherapy and aerobic exercise training on physical fitness in young children with cystic fibrosis. METHODS: Sixteen patients with cystic fibrosis, between the ages 5-13 years, were included in this study. All children were assessed at the beginning and at the end of 6 week of the training. Modified Bruce protocol was used for assessing the cardiovascular endurance. The sit-up test was used to evaluate the dynamic endurance of abdominal muscles, standing long jump was used to test power, sit and reach, trunk lateral flexion, trunk hyperextension, trunk rotation and forward bending tests were used to assess flexibility, 20 m shuttle run test and 10-step stair climbing tests were used to assess power and agility. All patients received chest physiotherapy and aerobic training, three days a week for six weeks. Active cycle of breathing technique and aerobic exercise training program on a treadmill were applied. RESULTS: By evaluating the results of the training, positive progressions in all parameters except 20 m shuttle run and 10 stairs climbing tests were observed (p < 0.05). Active cycle of breathing techniques were used together with exercise training in clinically stable cystic fibrosis patients increases thoracic mobility (p < 0.05) and the physical fitness parameters such as muscle endurance, strength and speed (p < 0.05). Comparison of the results in sit and reach and forward bending tests were not significant (p > 0.05). CONCLUSION: It is thought that in addition to medical approaches to the systems affected, the active cycle of breathing techniques along with aerobic training helps to enhance the aerobic performance, thoracic mobility and improves physical fitness in children with cystic fibrosis.

Musculoskeletal techniques for clinically stable adults with cystic fibrosis: a preliminary randomised controlled trial.

AIMS: To assess the sensitivity of selected outcome measures to any change resulting from treatment of adults with cystic fibrosis with physiotherapy musculoskeletal techniques, use the data for sample size calculations for future studies and assess the acceptability of the methods to potential participants. DESIGN: Preliminary, prospective, single-blind, randomised controlled trial. SETTING: Specialist cystic fibrosis centre. PARTICIPANTS: Adults recruited from a cystic fibrosis outpatient clinic. INTERVENTIONS: The control group received normal optimal physiotherapy care and the intervention group received weekly musculoskeletal treatment for 6 weeks in addition to normal optimal physiotherapy care. OUTCOME MEASURES: Recorded at baseline, 3, 6 and 12 weeks. The outcome measures were posture (thoracic index), chest wall excursion, forced expiratory volume in 1 second (FEV1), visual analogue scale for pain, modified shuttle test and Cystic Fibrosis Quality of Life Questionnaire--Section One (physical functioning). STATISTICAL ANALYSIS: Descriptive statistics [using medians and interquartile ranges (IQRs)] and linear regression mixed model. RESULTS: From a total of 20 subjects, 10 were randomised to each group. Fifty percent of subjects were male, with a median age of 27 years (IQR 25 to 34), median FEV(1) of 1.75 l (IQR 1.4 to 2.4) and median body mass index of 20.8 (IQR 20.0 to 23.5). Baseline differences between groups in thoracic index and modified shuttle test made any differences difficult to interpret, but the results for thoracic index and chest wall excursion at the third rib in the treatment group showed a trend towards improvement. The usefulness of FEV1, the visual analogue scale for pain and the Cystic Fibrosis Quality of Life Questionnaire as measures is unclear. CONCLUSION: Further musculoskeletal studies in people with cystic fibrosis should consider using thoracic index and a measure of lung function in addition to FEV1. The musculoskeletal techniques appear to be acceptable to people with cystic fibrosis, and do not seem to have associated adverse effects.

Differing perspectives of sputum and its expectoration: a qualitative study involving patients with cystic fibrosis and physiotherapists.

Sputum specimens are frequently requested from individuals with chronic suppurative conditions such as cystic fibrosis (CF). For a proportion of people, expectorating sputum can be difficult. Our goal was to explore the potential barriers and facilitators to expectorating sputum from the perspective of those with CF and physiotherapists involved in their care. Eighteen interviews were conducted with people who have CF and three focus groups with physiotherapists. Data were recorded, transcribed verbatim, and analysed using a framework approach. Variation emerged in perceptions between physiotherapists and people with CF in terms of sputum's impact on everyday life and the importance of its role in managing the condition. These differences were reflected in the following themes: (1) seepage of bodily boundaries, (2) discrediting nature of sputum, (3) embodied representation of chronic illness, and (4) non-production as a decisional balance. Differing views between participants could have arisen from the personal nature of sputum for people with CF, whilst physiotherapists may see sputum specimens as a necessary part of holistic management of this condition. Education could assist individuals in overcoming some of the barriers associated with expectorating, but physiotherapists may have to individualise the collection of sputum, recognising not everyone will be at ease with this procedure.

[Physical activity and exercise training for patients with cystic fibrosis]. / Activité physique et réentraînement à l'effort du patient atteint de mucoviscidose.

In France patients with cystic fibrosis benefit from a multidisciplinary follow-up in Cystic Fibrosis Centres. In this follow-up, despite the numerous therapeutic benefits of exercise in this disease, little emphasis is placed on the promotion of physical activity. The aim of this article is to improve this aspect of management, giving advice from a working group of experts, based on the medical literature and clinical experience. These proposals include quantification of physical activity, evaluation of exercise, training and rehabilitation programs and finally, modification of behaviour to include physical activity in the overall cystic fibrosis treatment strategy. It is intended to set up multicentre studies to evaluate the impact of these proposals.

[Sanatorium-and-spa treatment of children with mucoviscidosis and pancreatic lesions].

Over 5 years ago, the Yunost' sanatorium, Essentuki, and the Russian Centre of Mucoviscidosis, Moscow, began to jointly implement a rehabilitation program for children with mucoviscidosis. The data obtained made it possible for the first time to propose incorporation of drinking carbonate mineral water in the combined sanatorium-and-spa treatment of such patients. The new method allowed effectiveness of this therapy to be improved by 16-18%.

Effects of inspiratory muscle training in cystic fibrosis: a systematic review.

OBJECTIVE: We performed a systematic review to determine the effect of inspiratory muscle training (IMT) on inspiratory muscle strength and endurance, exercise capacity, dyspnoea and quality of life for adolescents and adults living with cystic fibrosis. DATA SOURCES: MEDLINE, EMBASE and CINAHL electronic databases were searched up to January 2008. REVIEW METHODS: We performed a systematic review using the methodology outlined in the Cochrane Collaboration protocol. Articles were included if: (1) participants were adolescents or adults with cystic fibrosis (> 13 years of age); (2) an IMT group was compared to a sham IMT, no intervention or other intervention group; (3) the study used a randomized controlled trial or cross-over design; and (4) it was published in English. Data were abstracted and methodological quality was assessed independently by two reviewers. RESULTS: The search strategy yielded 36 articles, of which two met the inclusion criteria. Both studies used a targeted or threshold device for IMT. Meta-analyses were limited to forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC), which showed no difference in effect between the IMT group and the sham and/or control group. Individual study results were inconclusive for improvement in inspiratory muscle strength. One study demonstrated improvement in inspiratory muscle endurance. CONCLUSION: The benefit of IMT in adolescents and adults with cystic fibrosis for outcomes of inspiratory muscle function is supported by weak evidence. Its impact on exercise capacity, dyspnoea and quality of life is not clear. Future research should investigate the characteristics of the subgroup of people with cystic fibrosis that might benefit most from IMT.

Influência do método Reequilíbrio Toracoabdominal sobre a força muscular respiratória de pacientes com fibrose cística / Influence of the technique of re-educating thoracic and abdominal muscles on respiratory muscle strength in patients with cystic fibrosis

OBJETIVO: Avaliar o efeito do método Reequilíbrio Toracoabdominal na força dos músculos respiratórios de pacientes com fibrose cística, acompanhados no Ambulatório de Fibrose Cística da Universidade Católica de Brasília. MÉTODOS: A amostra, constituída de 29 fibrocísticos, foi caracterizada com base em dados antropométricos, genéticos e de colonização bacteriana. Espirometria, manovacuometria e antropometria foram realizadas antes e depois do tratamento fisioterapêutico, no qual se utilizou o método Reequilíbrio Toracoabdominal, duas vezes por semana, durante quatro meses. RESULTADOS: Houve aumento da pressão inspiratória máxima e da pressão expiratória máxima após o tratamento fisioterapêutico em todos os pacientes, naqueles sem distúrbio ventilatório obstrutivo e naqueles com distúrbio ventilatório obstrutivo leve (p < 0,05). Foi encontrada correlação positiva entre a idade e a pressão expiratória máxima para a maioria dos grupos. A pressão inspiratória máxima só apresentou correlação positiva com a idade no grupo com distúrbio ventilatório obstrutivo leve (p = 0,012; r = 0,817). Para o sexo feminino e para o grupo sem distúrbio ventilatório obstrutivo houve correlação negativa entre a pressão expiratória máxima e a colonização por Pseudomonas aeruginosa (p = 0,036; r = -0,585). CONCLUSÃO: Para os fibrocísticos avaliados, o método Reequilíbrio Toracoabdominal aumentou a força dos músculos respiratórios, o que reafirma a importância do tratamento fisioterapêutico para estes pacientes.

[Cystic fibrosis: physiotherapy and the risk of cross infection]. / Mucoviscidose: le kinésithérapeute face au risque infectieux.

INTRODUCTION: Respiratory infections play a deleterious role in the progression of patients suffering from cystic fibrosis. Cross infections and epidemics have been documented in these patients, justifying a rigorous prevention regime. BACKGROUND: This prevention regime concerns all health workers and rests on various recommendations. Segregation of patients and hand hygiene are important factors. Respiratory equipment and, more specifically, nebulisers represent a potential source of bacterial contamination requiring special attention. In the same perspective the risks related to the health care environment, particularly the lung function laboratory, should not be ignored. The modalities of prevention sometimes have a significant psychological impact that may often be reduced by careful explanation. CONCLUSIONS: In view of the time spent in contact with these patients the physiotherapists are naturally involved in this prevention regime which is an integral part of their treatment.

Influence of the technique of re-educating thoracic and abdominal muscles on respiratory muscle strength in patients with cystic fibrosis.

OBJECTIVE: To determine the effect that re-education of the thoracic and abdominal muscles has on the respiratory muscle strength of patients with cystic fibrosis evaluated over time at the Cystic Fibrosis Outpatient Clinic of the Universidade Católica de Brasília (Catholic University of Brasília). METHODS: The sample consisted of 29 cystic fibrosis patients, characterized based on anthropometric, genetic and bacterial colonization data. The patients were submitted to physical therapy sessions, involving re-education of the respiratory muscles, twice a week for four months. Spirometry, pressure manometry and anthropometry were performed before and after each session. RESULTS: Comparing baselines values to those obtained after physical therapy, increases in maximum inspiratory pressure and maximum expiratory pressure were observed in all patients, those without any obstructive respiratory disease and those with mild obstructive respiratory disease (p < 0.05). A positive correlation between age and maximum expiratory pressure was observed for most of the patients. Maximum inspiratory pressure correlated positively with age only in the group with mild obstructive respiratory disease (p = 0.012; r = 0.817). In female patients and in the group of patients without obstructive respiratory disease, a negative correlation was observed between maximum expiratory pressure and colonization with Pseudomonas aeruginosa (p = 0.036; r = -0.585). CONCLUSION: Use of the thoracic and abdominal muscle re-education technique increased respiratory muscle strength in the cystic fibrosis patients studied, a finding that underscores the importance of including physical therapy in the treatment of these patients.