Chronic Respiratory Disease 2005; 2: 139149

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REVIEW SERIES: practical management of cystic brosis

Physiotherapy management of cystic brosis

ME Dodd1 and SA Prasad2
1

Adult Cystic Fibrosis Centre, South Manchester University Hospitals NHS Trust, Manchester, UK and 2 Cystic Fibrosis Unit, Great Ormond Street Hospital for Children, London, UK

For many years physiotherapy for cystic brosis (CF) was synonymous with a daily routine of postural drainage and percussion. Improved longevity and a desire for independence, together with a clearer understanding of the pathophysiology of the disease, has led to the development of many airway clearance techniques. The contribution of chest physiotherapy remained unchallenged until recent times when lack of robust evidence and the presentation of asymptomic infants and adults led some physiotherapists to question this traditional approach. As survival increases into the fourth and fth decade unique complications have evolved and physiotherapy management is challenged with nonrespiratory aspects of treatment. This review will consider the evidence for, and current practice of airway clearance, the value of exercise and the emerging problems of musculoskeletal complications and incontinence. Chronic Respiratory Disease 2005; 2: 139 149 Key words: airway clearance; cystic brosis; exercise; incontinence; physiotherapy; posture

Introduction
Cystic brosis (CF) is the most common inherited recessive disease of the Caucasian population in the UK with a carrier rate of 1:25 and incidence of 1:2500 live births. It is characterized by chronic pulmonary infection and inammation, pancreatic insufciency and an imbalance of sweat electrolytes. Major advances over the past 60 years in the management of CF have resulted in dramatic improvements in longevity and survival, in what was for many years considered to be a paediatric disease. The predicted median survival is now over 30,1 however respiratory dysfunction continues to be the main contributory factor to the morbidity and mortality associated with this disorder. Physiotherapy is an integral part of the management of CF. Airway clearance was, when the disease was rst described, the mainstay of respiratory management and continues to play an integral role. However, with changing disease patterns from infancy to adulthood, the role of physiotherapy in this disorder has altered signicantly and is no longer limited to airway clearance but also emphasizes the importance of
Correspondence: Mary E Dodd, Consultant Physiotherapist, Adult Cystic Fibrosis Centre, South Manchester University Hospitals NHS Trust, Manchester, M23 9LT, UK. E-mail: mary.dodd@smtr.nhs.uk
# 2005 Edward Arnold (Publishers) Ltd

physical exercise, postural care and addresses the unique complications which are emerging as longevity improves.

Airway clearance
Bronchial damage results from persistent infection and inammation within the airways.2 4 Chest physiotherapy is an integral part of the respiratory management of this disorder. Airway clearance techniques (ACT) are used to clear mucopurulent secretions from the airways and aim to delay the progression of lung disease. Traditionally chest physiotherapy has been undertaken as a twice-daily routine, usually consisting of a combination of postural drainage and percussion in gravity assisted positions, administered with the help of an assistant. Improved longevity and the desire of those with CF to lead active and independent lifestyles has, over the last two decades, led to the development of several different ACT which allow treatment to be administered independently and effectively (Table 1). Evidence for airway clearance techniques Daily ACT regimens are time consuming and burdensome. The well documented problems with adherence to chest physiotherapy in CF are therefore
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Table 1
Technique

Airway clearance techniques

Device(s) Principles Cycle of breathing exercises comprising: thoracic expansion exercises breathing control forced expiration technique Three phase breathing exercise regimen: Adjustment of tidal breath to low/mid/high lung volumes according to location of mucus External thoracic wall oscillation applied via a jacket or rigid shell Internal airway oscillation applied via mouthpiece during inspiration Variable, oscillatory PEP applied during expiration Continual positive expiratory pressure Applied via mask/ mouthpiece at levels of 10 20 cmH20 (50100 cmH2O for HPEP) Gravity-assisted positioning combined with chest clapping/shaking

Active cycle of breathing techniques (ACBT)

Autogenic drainage (AD)

High frequency chest compression (HFCC) Intrapulmonary percussive ventilation (IPV) Oscillating positive expiratory pressure Positive expiratory pressure (PEP)

Hyek oscillator Vest Intrapulmonary percussive ventilator Flutter Cornet Acapella PEP Bubble PEP High pressure PEP (HPEP)

effective as chest physiotherapy in terms of sputum clearance and short-term lung function.13,14 The benets of physiotherapy in the CF population who are asymptomatic in terms of overt chest symptoms are more controversial. While some evidence, albeit small, indicates benecial effects of chest physiotherapy in those with established disease, there is no evidence to support its use in those with little in the way of respiratory symptoms. Improvements in medical management and diagnosis have led to increasing numbers of patients who are only mildly affected by the disorder as well as a population of patients with atypical CF who have very mild or no symptoms of the disease. In both of these populations a routine twice-daily treatment regimen which is associated with a signicant burden of care may not be justied. Despite the changing characteristics of the patient cohort and the lack of evidence to support current practice, many clinicians are reluctant to alter current practice of a treatment that has become an integral part of the respiratory management. Choosing an airway clearance technique The choice of which of the various airway clearance modalities to use tends to be dependent on therapists personal experience and to some extent by geographical therapeutic trends rather than robust long term scientic data. Several comparative studies attempt to evaluate the short-term efcacy of ACT. The results of these are contradictory, some report specic advantages of one modality over another, whilst others fail to reproduce similar results. A few long-term comparative studies have compared various ACT.8,9,15,16 These studies mainly compare newer techniques or devices with conventional physiotherapy (postural drainage and percussion).8,9,16 Reissman et al. reported conventional therapy to be more effective than treatment using a forced expiration technique,8 whereas other studies reported advantages in the use of high frequency chest wall oscillation and positive expiratory pressure (PEP) over conventional physiotherapy9,16 and of PEP compared to Flutter.15 Confusion in the existing literature might be explained in terms of study design, protocol and methodology.17 Studies which include well dened techniques, may describe a very different treatment protocol. The majority of studies are short term (less than two weeks), and although they may provide certain useful information relating to treatment safety and the short-term physiological effects of treatment, it is the long-term effects of daily treatment on clinical well

Postural drainage and percussion

not surprising.5,6 Furthermore, the demand for evidence-based practice has required physiotherapists to scrutinize their practice carefully as scientically proven justication of therapy might serve not only to persuade patients and carers of the value of a routine which often does not offer immediate or obvious benet, but also reassures therapists themselves that their practice is worthwhile. It is generally agreed that daily chest physiotherapy regimens are benecial in the long term for those who have established respiratory disease. Associated data from the few long term comparative studies of various ACT in CF do indicate that regular chest physiotherapy may inuence outcome.7 9 There is however, no robust scientic evidence available to support this supposition,10 and no long-term studies have been undertaken which rigorously examine a treatment versus no treatment group in the CF population. A short-term study reported detrimental effects on lung function when treatment was withheld for a three-week period in CF patients with established pulmonary disease.11 Other short-term studies have reported no demonstrable benet,12 and some report coughing alone to be as
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being and quality of life which are most important. Very few studies are of sufcient duration to evaluate the impact of an intervention on measures of long term clinical status.8,9,15,16 More recently two systematic reviews of the existing data have compared the use of conventional chest physiotherapy and PEP with other airway clearance modalities.18,19 Neither demonstrated an advantage of any one treatment modality, although in the PEP review patients preference for PEP was reported. This is perhaps not surprising in such a complex, long-term disease where it is more reasonable to assume that individuals are likely to react differently and that specic treatments may suit certain individuals to a greater or lesser extent, depending on the stage of the disease process, clinical and social circumstances. Optimal treatment is therefore most likely to be provided by an individually tailored approach to treatment regimens, taking into account both clinical and social factors. Infants and small children Until relatively recently, diagnosis of CF has been synonymous with obvious respiratory symptoms and poor nutritional status. However, with advances in medical care and earlier diagnosis (particularly since the introduction of neonatal screening for CF), a population of infants with CF but without symptoms of respiratory secretions or infection has been exposed. Evidence from bronchoalveolar studies on infants with CF suggest that manifestations of lung disease such as inammation/infection,2 4 alteration of lung function parameters20 and structural abnormalities as shown on CT21 are present from a very early stage in the disease process before the child shows any signs of lung disease. It could therefore be hypothesized that if physiotherapy is not administered, insidious deterioration may be inevitable. Conversely, it could be argued that the effect of airway clearance regimens on these parameters of inammation, infant lung function and structural changes in the absence of excess secretions is completely unknown and may have little or no benecial effect and that anti-inammatory therapies, antibiotics and inhaled medications are likely to be more benecial. It is often advocated that the early introduction of a twice-daily routine of treatment facilitates acceptance of treatment which then becomes a habitual part of the daily routine. Later introduction of such a regimen may meet with resistance and nonadherence. This is however, not substantiated by evidence from several studies of adherence to treatment in chronic illnesses

where it is well recognized that daily, time consuming therapies which have little or no immediate and apparent benet are poorly adhered to.22 24 Therefore it is possible that this combination of factors in an asymptomatic population of infants is likely to magnify problems of adherence, and potentially damage adherence during respiratory exacerbations, when co-operation is most desired. Opinions amongst clinicians regarding the physiotherapy management of asymptomatic infants and children with CF are divided and there remains a need to address the shortfall of evidence in this area. However, it is generally agreed that all parents/carers should be carefully taught an appropriate regimen of chest physiotherapy soon after diagnosis. The modality chosen and the frequency of treatment should then be dependent on the individuals clinical status and the family circumstances. Gastro-oesophageal reux Considerable attention has recently been focused on the association of gastro-oesophageal reux (GOR) with the use of gravity-assisted positioning in CF. In a study of 20 infants diagnosed with CF by newborn screening, chest physiotherapy incorporating a head down tip as part of the postural drainage regimen, was associated with more frequent episodes of GOR when compared to postural drainage avoiding a head down tilt.25 Seventy per cent of the original cohort were followed up at ve years and those having modied PD were reported to be associated with fewer respiratory complications.26 Further analysis reported postural drainage to be associated with distressed behaviour and lower oxygen saturation.27 While these nding are signicant and may have clinical implications, this study involves relatively small subject numbers and other studies have reported different results, albeit in a somewhat different cohort of infants.28 Further research to conrm the effects of chest physiotherapy are needed, however it would seem prudent to be alert to the possibility of pathological GOR not only in infants but at all ages.29,30 If present, it should be treated and this may include modication of the physiotherapy regimen. The value of postural drainage in newly diagnosed infants, many of whom are asymptomatic, is perhaps a more pertinent issue and has been previously discussed.

Exercise
Unlike airway clearance, traditionally exercise was considered harmful and has earned its place in the
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routine management through research and evaluation in the clinical setting. The safety of exercise in CF was rst documented by two studies, which stated that patients with a forced expiratory volume in one second (FEV1) of more than 55% predicted could exercise to the same level as their peers and that those with more severe disease could also participate in physical activity programmes but required careful monitoring with exercise testing prior to exercise prescription.31,32 Benets of exercise Several short-term studies have since demonstrated the benets of exercise in terms of improved cardiorespiratory tness,33 increased exercise tolerance,34,35 decreased breathlessness,36 improved respiratory muscle strength and endurance,37,38 improved activities of daily living39 and the psychosocial aspects of improved quality of life40 and morale.35,36 A single bout of exercise partially blocks amiloridesensitive sodium conductance in respiratory epithelium and may lead to an increase in the water content of CF mucus.41 It has been suggested that exercise could replace daily airway clearance. However the levels of daily physical activity required for this would be high and perhaps unrealistic. Later studies have suggested that exercise should be complimentary and not exclusive to airway clearance.42,43 The multiple benets of physical activity and the difference in patient perception of exercise compared to other routine treatments often means that patients prefer exercise as a form of treatment.5 It is seen as a treatment over which patients have control and not driven by the anxieties of their disease.44 Clinicians opinions of the value of exercise in the longer term altered signicantly when a retrospective study analysing the results of maximal exercise testing reported that higher levels of tness as measured by peak oxygen uptake (VO2peak) decreased mortality.45 It has also been shown that tness can be maintained in the long term despite a deterioration in lung function,46 but whether improving tness by training can improve survival remains to some extent unanswered. Two randomized controlled trials (RCT) have examined this issue. A three-year RCT in children reported a reduction in the annual rate of decline in forced vital capacity (FVC) with regular training.47 Improvement in lung function and tness have also been reported in adults undertaking regular submaximal exercise over a 12-month period.48 The importance of aerobic exercise in the long-term management of cystic brosis for both children and adults has been highlighted by these trials.49
Chronic Respiratory Disease

Limitations to exercise It is well recognized that the ability to exercise is determined mainly by pulmonary function and nutritional status.50,51 The reported determinants of anaerobic performance have been conicting. A decrease in peak sprint work has been reported as both comparable and defective when compared to healthy controls.52,53 Such a decit suggests a problem with muscle quality and when reported was worst in patients with poor nutritional status.53 It has been suggested that in adults aerobic exercise is determined by lung function and anaerobic performance by nutritional status.54 In children recent studies report both FEV1 and fat free mass (FFM) to be important determinants of anaerobic performance55 and that changes in peak aerobic performance over a two-year period correlate with changes in FEV1 and FFM.56 Genotype appears to inuence exercise tolerance in children, with all classes of mutations showing a reduction in anaerobic performance but sparing of aerobic performance in those with milder mutations.57 No difference in lung function has been reported between the classes and the relationship between genotype and tness remain unexplained. Recent attention has focused on the quality of specic muscles and the association of lung function with muscle mass, although the evidence remains unclear.58 61 Peripheral muscle strength has been shown to be reduced in children even when fat free mass and pulmonary function are normal,58 reduced in some muscle groups and normal in others,61 and normal in children with mild to moderate disease.60 In adults strength appears to be reduced, but when normalized for mass is no different from healthy controls.59 This concurs with other studies suggesting that that the quality of muscle in CF is preserved and the disease per se does not alter muscle quality but any associated poor nutritional status may do so.52,60 Reduced maximal exercise performance and maximal muscle force at VO2 levels equivalent to controls has been reported in children with normal lung function and nutritional status.58 Moser et al. showed equivalent muscle quantity to controls as measured by crosssectional area (CSA) but a reduced VO2peak and VO2/ WR slope, indicating a muscle related abnormality in oxygen metabolism, as previously reported.62,63 Selvadurai et al. studied adolescent CF female athletes with normal lung function and nutritional status and reported higher habitual activity levels, equivalent peak aerobic capacity but a reduced anaerobic capacity compared to healthy matched controls.64 Measurements at a cellular level were equivalent at rest but decreased with physical activity, suggesting

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deciencies in tness and muscle metabolism despite normal lung function and nutritional status and, unrelated to lack of activity. Clearly both the quantity and quality of peripheral muscle function are important factors in determining exercise capacity as is deconditioning due to inactivity and ill health.

Barriers to exercise The evidence for including exercise in the long-term management seems clear. Increasing aerobic activity is likely to inuence survival. Abnormalities of muscle quality and quantity in children despite good lung function and nutrition is a cause for concern and attention should be paid to addressing this during childhood. The benets of physical activity should be strongly emphasized to the whole family from the time of diagnosis and continually reinforced. There are several perceived barriers to exercise and various factors inuence the success of any training programme (Table 2). Parental attitudes towards exercise are important. Children are more likely to be active if their parents have an ethos of a healthy lifestyle. Parents with CF children reported greater barriers to exercise and perceived it as having fewer benets,65 though this improved if there was a healthy sibling within the family. Longitudinal measurements of habitual levels of activity may be useful,66 68 and health professionals should be attentive to the well documented decline in activity levels in adolescents, which occurs to a greater extent in girls than boys.69 71 Although Nixon et al. reported no difference in the number of hours engaged in physical activity between those with CF and their healthy peers, the CF group undertook less vigorous activities.66 In view of the impact that regular physical training may have on survival, patients should be encouraged to
Table 2 Factors inuencing successful training programmes
Factors which promote Parental involvement and encouragement74,77 Parents with ethos of active lifestyle Perception of activity levels5 Enjoyment and satisfaction76 Competency and self-esteem75 Individualized prescription48 Health beliefs and perceptions Aid to sputum clearance42
44

participate in more vigorous activities. Childrens activity patterns are characterized by short bursts of high activity producing an anaerobic response. A 12week anaerobic training programme has shown increases in both aerobic and anaerobic performance together with improvements in quality of life, but at 12week follow-up the improvement in aerobic capacity had not been sustained.72 As anaerobic training enhances aerobic performance it should be included in training programmes. Transferring exercise programmes to the community setting and more importantly sustaining them, is a considerable challenge both in the normal and CF population. It is difcult to devise programmes which are easily adhered to for long periods of time. Adherence to exercise programmes which employ a variety of activities based on subject preference are more likely to succeed.73 Factors shown to enhance adherence include social support, perceptions of competency and self-esteem, enjoyment of activities and availability of a variety of activities.74 The additional treatment-related burden of CF needs to be acknowledged by the family and healthcare team. Prescription of inexible routine exercise programmes may improve competency and a general improvement in psychological status and physical appearance but are likely to be unacceptable to patients,75 whereas individualized programmes where the exercise is enjoyed and training leads to a feeling of satisfaction are much more likely to be sustained.47,76 Behaviour changing strategies have also been shown to be successful.77 Types of activity The types of activity and the principles of exercise programmes are similar to those used for the general population. Pauses in training or decreasing work loads during acute infective exacerbations will be necessary and certain sporting activities may carry a medical risk for some patients (Table 3).78 A short term RCT of in-hospital exercise training versus resistance training
Table 3 Sporting activities which carry a medical risk
Sporting activity Contact sports e.g., rugby, judo, karate, kick boxing Bungee jumping Scuba diving Exercise at altitude e.g., skiing, snowboarding Endurance sports e.g., marathon, half marathon, triathlon Medical risk Hepatosplenomegaly Low bone mineral density Portal hypertension Sinus disease and air trapping Hypoxia Acute right heart failure Dehydration and heat stroke

Factors which hinder Parental attitudes barriers to exercise and perceived lack of benet65 Decreased activity levels in adolescents69 Girls less active than boys69,70 Participation in less vigorous activities71 Unacceptability of rigid training programmes75

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in children indicated that aerobic training resulted in better peak aerobic capacity, activity levels and quality of life, while resistance training led to better weight gain, lung function and leg strength.79 Exercise programmes should therefore be individualized to patient preference and required outcome, include aerobic and anaerobic activities, stretching and exibility to prevent injury and variety and enjoyment to enhance long term adherence.80 Inspiratory muscle training has also received attention but with conicting results. Reported improvements in inspiratory muscle strength resulting in improvements in lung function and exercise performance81 have not been conrmed by other studies.82 Exercise testing The evaluation of exercise programmes has led to the development and use of a variety of outcome measures and validated methods of exercise testing. Laboratorybased maximal exercise testing including respiratory gas analysis remains the gold standard but may not be readily available in the clinical setting. A variety of eld tests are currently used to assess functional capacity in CF. The modied shuttle walk test (MSWT) is a graded test, usually maximal and shown to be a reliable and sensitive measure of exercise capacity in adults and children with CF. Good correlation has been reported between MSWT distance walked and VO2 peak.83 85 More recently the 10 m and 20 m shuttle tests have been validated in children with a wide range of disease severity.86 Nonincremental tests include the six-minute walk and the three-minute step test. The reliability and repeatability of the six-minute walk has been studied,87,88 although as an effort dependent test it may have some disadvantages. The three-minute step test is an externally paced, simple and portable test which is independent of effort and has been validated for use in children over six years of age, although its use may be limited in those with well preserved lung function and tness levels.89 Subjective assessment of perceived breathlessness, fatigue or exertion are all useful additions to exercise testing. Using subjective measures of breathlessness in children, although useful, may be difcult to perform. Objective scores such as the 15-count breathlessness score,90 count score or maximum phonation91 to evaluate ease of breathing may be useful to complement subjective measures. The use of psychosocial measures, such as The Chronic Respiratory Diseases Questionnaire (CRDQ) reect important outcomes of interventions.92 The Sickness Impact Prole and Quality of Well Being have also been used.40,93
Chronic Respiratory Disease

Evidence suggests that aerobic exercise inuences survival in the long term and exercise tolerance has been shown to improve following short periods of training.94 A recent systematic review concluded further work is needed to conrm the overall benet of exercise but that there is no evidence to discourage current practice.95 Exercise testing, to measure exercise tolerance and the limitations of exercise, provides the foundation of exercise prescription and advice, monitors changes in clinical status and emphasizes the importance of physical activity in the long-term management of the disease.96

Bone disease and musculoskeletal problems

Bone disease Musculoskeletal problems in CF arise predominately as a result of abnormalities in bone mineralization,97 99 and altered respiratory mechanics caused by pulmonary deterioration.100 102 As survival in CF improves the prevalence of these complications is increasing.103,104 Two-thirds of adults with CF have low bone mineral density (BMD).97 99 Although BMD appears to be normal in healthy well nourished children105 107 it decreases in the rst decade of life and accelerates during adolescence and early adulthood.108 These data support the evidence of above normal fracture rates from the age of six years,108 increasing to patient reported levels of 35 50% in adults.99,109 In a study of 55 patients assessed for transplantation, X-ray observations revealed 62 spinal compression and 14 rib fractures which had not been previously reported, suggesting that the true level of fracture rates may be underestimated.109 Rib fracture pain in particular, is difcult to treat and is often unresponsive to nonsteroidal anti-inammatory agents and opiates. Sputum clearance may be severely impaired and can result in an acute infective deterioration. Subcutaneous calcitonin has been found to provide quick resolution of pain, permitting airway clearance and mobility.110 The use of manual chest physiotherapy techniques, such as chest clapping and vibration should only be used with caution as they may predispose to rib fracture in those with osteoporosis although there is no clear evidence for this.49 Posture and pain As lung disease advances, an increase in the work of breathing,100 hyperination101 and coughing102 contribute to soft tissue contractures and muscle

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imbalance, causing abnormal posture and pain.101 A number of methods for assessing spinal deformity and posture have been developed. Habitual and corrected posture assessed using the validated Quantecw scanning technique found patients with CF to be more kyphotic in both postures compared to healthy controls.111,112 In habitual posture, worsening back asymmetry correlated with a decrease in vital capacity and an increased kyphotic angle correlated with decreased FEV1. In some patients the kyphotic deformity improved in corrected posture suggesting a reversible component. In contrast, Rose et al. reported a similarly low incidence of kyphosis (8.7%) to healthy controls (10%) but a higher rate of vertebral wedging.101 Postural assessment revealed decreased muscle strength and mobility in the chest, trunk and shoulders, and it was suggested that in the absence of structural kyphosis in CF, the changes in soft tissue and spinal geometry are more likely to be attributed to an increased work of breathing and the hunched exed posture assumed during excessive coughing. Discrepancies between the studies may be explained by the difference in age of the subjects and measurement techniques. The incidence of back pain associated with abnormal posture has been reported to be 80 94%101,102 compared to 14% in healthy controls.101 Pain was related to severity of lung disease (FEV1% predicted), and the onset associated with episodes of coughing.101 Those with more severe pain were more kyphotic, produced more sputum, were more breathless on exercise and considered their posture to be poor.102 A greater corrected-to-normal kyphosis ratio, indicated greater spinal exibility and suggested that the postural abnormalities may be reversible. This suggests that postural abnormalities and resultant back pain are likely to be related to the complications of advancing lung disease and may be responsive to appropriate back exercises and postural advice. Other methods used for assessing spinal deformity include the exible ruler technique, validated in adults with cystic brosis for measuring thoracic kyphosis and this may provide a simple and noninvasive tool for routine use in the clinical setting.113 Treatment for musculoskeletal problems To date, no prospective studies have evaluated the efcacy of spinal joint mobilizations, specic muscle strengthening exercises or postural advice on back pain, airway clearance, sputum expectoration or lung function. McVean et al. analysed the referrals to a newly established service to treat musculoskeletal

problems in CF adults. The majority of attendees presented with low back pain and had poor posture. Symptoms improved or resolved in 66% following joint mobilizations, exercise and postural advice. Bone mineral density was normal in over half of these patients. Despite small numbers and confounding issues of self-selection,114 the study demonstrates that musculoskeletal pain may be reversible and emphasizes the importance of prompt treatment to prevent any deterioration in lung disease which may occur as a result of compromised airway clearance and mobility. Any exercise regimen prescribed to correct asymptomatic abnormalities in posture is time consuming and further adds to the treatment-related burden. Poor posture is likely to be a cause for concern but patients may not seek advice even when symptomatic.115 Chronic pain is a recognized problem in CF and evidence suggests clinicians should be proactive in developing strategies to address this problem in the acute stage.116 In some, the abnormalities will be reversible and the associated pain responsive to treatment. Larger controlled trials are required to clarify the value of mobilization techniques and exercises in the long term. Attention should be paid to maintaining normal posture and BMD in children and exibility exercises should be integrated in to the general exercise programme from an early age. Weight-bearing activity is associated with BMD in children117 and although unproven in CF it would seem prudent to include high impact exercises into routine activities.

Urinary incontinence
Prevalence It is now internationally recognized that urinary incontinence (UI) is a problem for many women118 121 and girls122 126 with CF. Prevalence rates for women range from 30% to 69% and for girls 20% to 49%. The numbers are much smaller for the male population with reports of 2 5% for men118,127 and 10% for boys.124,125 Faecal incontinence generally is reported as a rare occurrence in adults,118,121 although a recent study reported 40% of women to experience bowel symptoms.122 Faecal incontinence in children is reported to be higher in males (12.5%) than females (4.7%),124 and occurred frequently in 20% of children in the study.125 The symptoms did not increase with chest exacerbations and it was postulated that the high prevalence was linked to enzyme usage. To date, no questionnaire has been validated specically for use in CF, a chronic respiratory disease with gastro-intestinal malabsorption in young
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people and the majority of whom are nulliparous. Prevalence rates in the CF studies have been determined from previously validated but modied questionnaires or those developed specically to address the problems encountered in CF. Only one study in women122 and one in children126 included a healthy control group and/or another respiratory disease group in the study population. Other reports have used reference values for comparison. Severity and impact of UI Most of the attention has been paid to UI in females, where it is most prevalent and interferes considerably with day to day activity. The earliest age of onset is reported to be between seven and 10 years of age,123,128 although there seems to be no association between age and prevalence120 or occurrence,118 but age is associated with severity of leakage.119 It is now well recognized that leakage occurs across all ages and should be addressed at both paediatric and adult level. For the majority of women the amount of urine leakage is described as a few drops118,120,121 but during a respiratory exacerbation the severity can increase dramatically with some experiencing leakage down legs.120 Most young girls report minimal leakage123,125 but for a few a change of clothes is necessary125 and large volume leakage or emptying whole bladder is also reported.126 These ndings are based on patients self-report and have not been validated with urodynamic investigations. It is well known that patients with CF under report symptoms and minimize the severity of their disease, and therefore it is likely that these results accurately represent or underestimate the severity of the problem. Leakage appears to impact mainly on the medical aspects of the disease, affecting the ability to perform airway clearance, exercise and spirometry 120,121 rather than physical or social limitations.120,121,129 Women with CF seem to be more tolerant of their symptoms than healthy controls or those with chronic lung disease122 and very few request help for the problem.119,120,126 As in the normal population embarrassment is a barrier to discussion120 but it is likely that the major reason is the perception that leakage is not a priority compared to the seriousness of their chest disease. Some patients are unaware that treatment is available for these problems,123 and only seek help when leakage signicantly interferes with their life.129 The low number who seek help is a cause for concern because once leakage occurs there is a chronic and progressive deterioration of the pelvic neuromuscular function.
Chronic Respiratory Disease

Mechanisms and treatment Coughing is reported as the major cause of leakage,118 120,123,128 with sneezing and laughing also being common precipitators.120,123,128 Exercise and spirometry cause leakage to a lesser degree.120,123,128 Leakage is usually reported as being more common in females with poor lung function (FEV1% predicted), indicating a link with disease severity, although one study reported no such association.128 Compared to other lung diseases, coughing is reported to be more frequent and intense in CF.130 Coughing is recognized as a causal factor of leakage but is a poor prognostic indicator for the success of treatment. 131 No association has been reported between leakage and the various airway clearance modalities,119 although the subjects in this study had all been taught the knack, a technique of contracting the pelvic oor and abdominal muscles, known to reduce leakage during a cough.132 Pelvic oor muscle exercises (PFME) have been reported to have no effect on moderate muscle strength but to signicantly improve endurance.129 However, patients found the exercises burdensome and repeated exacerbations hindered progress, an improvement in symptoms was reported and subjects felt that treatment was worthwhile. Despite the small numbers, this study highlights the signicant problems with investigation and treatment of this complication in CF, with 30% of women who had requested help being reluctant to undergo further assessment or even withdrawing. The precise mechanisms of leakage in cystic brosis are unknown and although it is likely that stress incontinence plays a major role, it is unlikely just to be caused by excessive coughing. Routine PFME as a preventative strategy may be considered inappropriate and would add further to the treatment burden. It would seem appropriate to teach patients with excessive cough the knack in order to control the problem should it occur. Further work is needed to clarify the mechanisms of urinary incontinence in CF and the most appropriate treatment strategy. In the meantime CF centres should be proactive to address this potential problem from an early age and where appropriate refer patients to a specialist womens health physiotherapist.49

Conclusion
Physiotherapy management of cystic brosis in the early days responded to the need of babies and infants with excessive secretions and respiratory complications. The changing cohort of infants with minimal respiratory complications, the diagnosis of milder or

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atypical forms of CF and the increasing longevity into adulthood has been accompanied by a progressively changing physiotherapy regimen. A variety of effective ACT that allow greater independence and are based on various physiological concepts allow physiotherapists to individualize treatment programmes. The efcacy of routine chest physiotherapy in milder and asymptomatic individuals continues to be debated and requires further research. Exercise is now an established part of the physiotherapy programme, with proven benets both in the short and long term. The emerging complications of incontinence, abnormal posture and musculoskeletal pain are internationally recognized and further research is required to determine both the mechanisms and interventions needed to alleviate these problems. Improved survival may be attributed to aggressive medical treatment but is not without cost, and often requires an onerous daily programme of self-care. Conscious of the ever increasing treatment burden and the need for evidence-based practice physiotherapists should continue to look for solutions to the many unanswered questions through rigorous research and evaluation, but not lose sight of the immediate goal which is to provide optimal individually tailored holistic care.

Declaration of interest
The authors of this paper have no interests to declare.

References
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