Indications for antibiotics

Oral antibiotics can be given as prophylaxis, for occasional infective exacerbations or continuously for repeated severe infections. Viral infections can produce a bacterial infective exacerbation, which will often require a course of prophylactic oral antibiotics. Patients with severe disease and persistent purulent sputum, who repeatedly relapse following a short course of antibiotics, can be maintained on long-term oral antibiotics. Those commonly used are the penicillins and more recently the macrolides.

Nebulized antibiotics may be used for patients with severe bronchiectasis whose disease is progressive and difficult to control (Currie 1997). Nebulized antibiotics can delay persistent infection with Ps. aeruginosa in patients with cystic fibrosis if instituted at the time of first colonization (Valerius et al 1991). Although there are no randomized clinical trials, this practice may be used in bronchiectasis as acquisition of Ps. aeruginosa is associated with greater morbidity (Evans et al 1996). Randomized controlled trials, using nebulized antibiotics for bronchiectatic patients chronically infected with Ps. aeruginosa, have shown a reduction in sputum density of Ps. aeruginosa (Barker et al 2000) and a lessening of disease severity (Oriols et al 1999). Tobramycin solution for inhalation (TOBI) has been used in a pilot study in patients with severe bronchiectasis (Scheinberg & Shore 2005). Although there was an improvement in respiratory symptoms, there was a significant intolerance of the medication, with 10 withdrawals from the study (n = 41). Intravenous antibiotics are used for severe disease, patients who fail to respond to oral antibiotics and those chronically infected with Ps. aeruginosa.

Other treatment measures

Influenza vaccination should be given annually to all patients with bronchiectasis unless there is a medical contraindication.

Topical medication may be indicated for chronic mucopurulent rhinosinusitis and the recommended technique for inhaled topical deposition of drugs is the head-down and forward position to encourage entry of the drops to the ethmoid and maxillary sinuses (Wilson et al 1987).

Where there is an immunoglobulin deficiency, replacement therapy should be given in an attempt to prevent further lung damage.

Surgical resection should be considered only if the bronchiectasis is localized, but there are no randomized controlled trials to compare surgical versus conservative treatment in the decision-making process (Corless & Warburton 2000). In very severe widespread bronchiectasis with respiratory failure, lung transplantation may be considered.

The inhalation of recombinant human deoxyribonuclease (rhDNase) does not appear to improve ciliary transportability, spirometry, dyspnoea or quality of life in patients with non-CF bronchiectasis (Wills et al 1996).

Inhaled steroids have been evaluated, with a trend to improving some respiratory parameters, but larger studies are needed (Kolbe & Wells 2000). A subset of bronchiectatic patients respond to bronchodilators and all patients should be tested for a response (Hassan et al 1999).

Excess bronchial secretions

It is important that the patient understands the pathology of the condition and the reasons for treatment. Clinically, effective physiotherapy should reduce the episodes of superimposed infection and may help to minimize further lung damage.

An airway clearance technique (ACT), for example the active cycle of breathing techniques (ACBT) or autogenic drainage (AD) (Chapter 5), should be introduced and self-treatment encouraged (Rosen 2006). Each patient should be assessed to determine the positions that may increase the efficiency of secretion clearance and a CT scan, if available, may facilitate this. The sitting position may be adequate for patients with minimal secretions. The horizontal position may be a more acceptable and comfortable alternative to the head-down tipped position and has been shown to be equally effective in patients who expectorate >20 g of sputum per day (Cecins et al 1999).

In patients who present with gastro-oesophageal reflux (GOR) there may be concern that the head-down tipped position will exacerbate the problem. The head-down tipped position is now rarely indicated. Chen et al (1998) reported no difference in the duration or frequency of symptoms in the various drainage positions. Other techniques that may be used to facilitate airway clearance have proven efficacy when used in gravity-assisted positions, e.g. Flutter and the active cycle of breathing techniques (Eaton et al 2007, Thompson et al 2002) and Acapella (Patterson et al 2004, 2005, 2007). These are discussed in Chapter 5. It has been suggested that a test of incremental respiratory endurance (TIRE) may be a useful method of airway clearance. In a single treatment session comparing the TIRE with ACBT, significantly more sputum was expectorated with ACBT (Patterson et al 2004). In contrast, Chatham et al (2004) demonstrated that TIRE was more effective than the ACBT. Further research is needed in this field.

Regular daily treatment is often necessary, but the frequency will vary among individuals. For many patients, treatment once a day is sufficient but the frequency should be increased during episodes of superimposed infection. Some patients find their chest is ‘dry’ at the beginning of the day. The timing of treatment should take into consideration both the time of day that the chest is most productive and the patient’s lifestyle. Adherence may be increased by agreeing a suitable home programme with the patient. Elderly or frail patients may require assistance from a relative or carer who should be carefully instructed by a physiotherapist.

It is important that physiotherapy techniques and positions for treatment are reassessed at regular intervals (Currie et al 1986). Most patients should be reassessed within 3 months of initial instruction and then at least annually. A diagnosis of bronchiectasis may be confirmed in the absence of a daily productive cough. For these patients it would seem advisable to teach an ACT to be used during acute exacerbations of pulmonary infection (British Thoracic Society Guidelines 2007). In some patients with bronchiectasis it may be the increase in ventilation to the bronchiectatic area (use of the dependent position) rather than any ‘drainage’ from the area in an uppermost position that will increase airway clearance. Individual assessment is imperative for effective management and the end-point of a treatment session must be recognized by self-assessment.

Hypertonic saline

Hypertonic saline has been shown to increase the ciliary transportability of bronchiectatic sputum, probably through its action of altering sputum rheology (Wills & Greenstone 2006). Kellet et al (2005) compared ACBT with three other treatment arms: ACBT preceded by nebulized terbutaline alone or in combination with either isotonic or 7% saline, in stable bronchiectatic patients producing less than 10 mg sputum per day. Hypertonic saline was shown to improve sputum weight, ease of expectoration, and lung function and reduce viscosity compared with the other treatment arms. Longer-term studies are required to determine the place of hypertonic saline on infection rates, quality of life and lung function.

Acute exacerbation of infection

Patients may be admitted to hospital with an acute exacerbation of pulmonary infection. The patient will probably be expectorating an increased amount of more purulent sputum, may be febrile, dehydrated and breathless. Haemoptysis is not uncommon and pleuritic chest pain may be present. The most severely affected may present with respiratory failure.

Mechanical adjuncts will be required in addition to an airway clearance technique to assist the clearance of excess bronchial secretions. A nebulized bronchodilator and/or humidification (Conway et al 1992) before treatment may help in the mobilization of tenacious secretions. Intermittent positive pressure breathing (IPPB) may help both in the clearance of secretions and in reducing the work of breathing. Patients who, many years ago, received the more radical treatment of resection of more than one lobe will probably have very poor respiratory reserve by the time they reach middle age. A superimposed infection in these patients may precipitate respiratory failure. Modified positioning, for example side lying or high side lying, combined with IPPB may be an effective form of treatment in minimizing the effort of clearing secretions. Non-invasive ventilation (Chapter 11) may be indicated in acute respiratory failure, although the outcome may be less successful in the presence of excess bronchial secretions.

Following resection of lung tissue, the anatomy of the bronchial tree may alter and the traditional positions for drainage of segments of the remaining lobes may be unsuitable or inappropriate. The physiotherapist should try various positions until the optimal ones are found.

The presence of blood streaking in the sputum is not a contraindication to physiotherapy and treatment should be continued. If there is frank haemoptysis, physiotherapy should be temporarily discontinued but resumed as soon as the sputum is only mildly blood stained to avoid retention of old blood and mucus. Before discharge from hospital, it is important that the patient is able to take the responsibility for their treatment and is confident with the positions and techniques required to continue regularly at home. If a bronchodilator has been prescribed, this should be taken before treatment and a few patients with bronchiectasis may also be prescribed nebulized antibiotic drugs, which should be inhaled after airway clearance. A breath-enhanced nebulizer or adaptive aerosol delivery (AAD) device is recommended for delivery of antibiotics (Chapter 5). If a patient is on the waiting list for lung transplantation, a preoperative rehabilitation programme should be established and postoperative treatment would be as outlined in Chapter 15.

Breathlessness

Some patients with bronchiectasis also demonstrate a degree of bronchospasm and may benefit from the inhalation of a bronchodilator before physiotherapy to clear secretions. Instruction in the use of an appropriate device for drug delivery is important.

A minority of patients with bronchiectasis complain of breathlessness. For these patients rest positions to relieve breathlessness and breathing control while walking and stair climbing should be included in the treatment programme (Chapter 5).

Reduced exercise tolerance

Exercise should be encouraged to improve general physical fitness. It will also assist the mobilization of bronchial secretions. Patients with severe bronchiectasis may benefit from a group pulmonary rehabilitation programme (Chapter 13). There is little research evaluating the place of exercise in bronchiectasis although there is a suggestion that inspiratory muscle training (IMT) may improve endurance exercise and quality of life (Bradley et al 2002). Newall et al (2005) evaluated an 8-week high-intensity pulmonary rehabilitation programme in combination with IMT or IMT sham or a control group. Both exercise groups showed significant improvements in exercise performance and inspiratory muscle strength. This was maintained at 3 months only in the IMT group. In the short term there would appear to be no advantage of including IMT in an exercise programme although it may prove beneficial in the longer term.

Chest wall pain of musculoskeletal origin

The management of this problem is discussed in Chapter 5.

Respiratory

The respiratory signs and symptoms of cystic fibrosis vary. The majority of older children and adults have a cough productive of sputum with varying degrees of purulence. The respiratory pathogens most commonly isolated in sputum are Pseudomonas aeruginosa (61%), Staphylococcus aureus (28.3%), Haemophilus influenzae (8.9%) and Burkholderia cepacia (3.2%) (FitzSimmons 1993). Infection with B. cepacia is often associated with accelerated pulmonary disease and a worse prognosis (Muhdi et al 1996).

Chest pain is common as the disease progresses and may be musculoskeletal or pleuritic. Breathlessness may be associated with infective exacerbations and increasing disease severity. Pneumothorax should be considered if there is an acute onset of breathlessness and pain. As breathlessness increases, appetite may fall and weight loss is common. Haemoptysis is common in adults but is usually mild, although episodes of frank haemoptysis may occur. Most patients develop finger clubbing which is associated with more severe disease.

Auscultation is often unrewarding when compared with the severity of radiological disease. Coarse inspira tory crackles are often heard. A pleural rub may be heard in association with infective exacerbations. The chest radiograph is often normal at birth but early changes include bronchial wall thickening, initially in the upper zones. As the disease progresses, hyperinflation may be noticeable with ill-defined nodular shadows, numerous ring and parallel line shadows indicating bronchial wall thickening and bronchiectasis (Chapter 2). High-resolution computed tomography (HRCT) imaging provides detailed evaluation of the lungs in CF (Chapter 2). Studies using HRCT in infants with CF have demonstrated early structural changes, even in those who have minimal symptoms (Long et al 2004).

Pulmonary function tests initially show signs of airways obstruction, but with advanced disease a restrictive pattern may be superimposed on the obstructive defect and a diffusion abnormality will also become apparent. Pulmonary function tests in infants with CF have shown early changes with diminished airway function soon after diagnosis, even in infants with no clinical history of clinical infection. These changes seem to persist during early childhood (Ranganathan et al 2001, 2004). More recently the use of multiple breath washout techniques to measure lung clearance index (LCI) (Chapter 3) have been shown to be effective and sensitive in terms of detecting early lung disease in cystic fibrosis (Aurora et al 2005a, Lum et al 2007). Pulmonary function measurements (FEV₁, PaO₂, PaCO₂) have been shown to be predictors of mortality (Kerem et al 1992). As the disease progresses ventilation/perfusion imbalance occurs, leading to hypoxaemia and pulmonary hypertension. Carbon dioxide retention occurs in patients with severe disease.

Asthma is as common in patients with CF as it is among the general population. Many patients with CF have a positive skin test to Aspergillus fumigatus. This is often seen in the sputum of patients and can be isolated in 40–60% of patients (Chen et al 2001). Colonization of the lower airway with Aspergillus fumigatus and the complication of allergic bronchopulmonary aspergillosis (ABPA) is well recognized in patients with cystic fibrosis (Skov et al 2005) and occurs in up to 10% of patients (Mastella et al 2000). ABPA is recognized by recurrent wheezing, deteriorating chest symptoms, fleeting fluffy shadows on the chest radiograph and elevated IgE levels which are specifically raised to Aspergillus.

Some patients develop nasal polyps. These may grow rapidly, are frequently recurrent and may require surgical removal. They may be related to chronic sinus infection.

Gastrointestinal

Distal intestinal obstruction syndrome (DIOS) is obstruction of the small bowel occurring in children and adults and is similar to that seen in neonates presenting with meconium ileus. This may be related to poor adherence with pancreatic supplements. It presents as small bowel obstruction with abdominal distension and discomfort, vomiting and reduced or absent bowel signs. Diagnosis is confirmed by the classic radiographic appearances of small bowel obstruction.

Cystic fibrosis-related diabetes (CFRD) has become a significant complication as a consequence of improved survival and is a result of progressive fibrosis damaging the endocrine cells that produce insulin (Bridges & Spowart 2006). The onset of diabetes, if not detected and treated promptly, can result in a decline in the patient’s clinical condition (Lanng et al 1992). The basic defect also affects the hepatobiliary system, which can result in a biliary cirrhosis. Patients with severe disease can develop portal hypertension. The main complication is bleeding from gastric or oesophageal varices. Liver transplantation may be required.

Other

Puberty may be delayed for both male and female patients. Most women with cystic fibrosis have normal or near-normal fertility. Improving survival has resulted in an increasing number of the female population having children. Outcome of pregnancy is improved if pulmonary function is greater than 60% predicted (Edenborough et al 1995). Pregnancy has been reported to have a slight adverse effect on the health of women with CF (Gillet et al 2002). Women with CF require a greater intensity of treatment during pregnancy (McMullen et al 2006).

Most males are infertile because of developmental defects of the vas deferens, which is either absent or blocked, but they can produce sperm. Improved technology, whereby sperm can be aspirated from either the testis or epididymal sac in conjunction with intracytoplasmic sperm injection (ICSI), has resulted in CF biological fathers (Phillipson et al 2000).

Approximately one-third of adult patients with CF develop rheumatic symptoms (Bourke et al 1987). The two most common forms are an episodic and recurrent arthralgia /arthritis and hypertrophic pulmonary osteoarthropathy. They are characterized by joint pain, tenderness, swelling and limitation of movement, usually symmetrical and affecting particularly the knees, ankles and wrists (Johnson & Knox 1994). More important has been the recent recognition of the high prevalence of low bone mineral density in children and adults (Bachrach et al 1994, Bhudmkanok et al 1996, Haworth et al 1999), which leads to a high incidence of fractures. Rib fractures can result in considerable pain, sputum retention and morbidity.

Home treatment

In many countries the emphasis on treatment is moving from hospital to home. The benefits for patients of treatment at home include less disruption to school, work and family life while avoiding the isolation from friends and family that hospitalization incurs. Increasing numbers of CF patients are receiving their intravenous antibiotics at home usually because quality of life is better, but clinical outcome is better for the patient treated in hospital and patients treated at home require close supervision (Nazer et al 2006, Thornton et al 2004).

For the newly diagnosed or newly referred patient, home visits by members of the specialist team (usually the clinical nurse specialist) provide an opportunity for advice, education and support for the patient and family, as necessary. Domiciliary physiotherapy services are sometimes available and can provide the opportunity for discussion and demonstration of physiotherapy techniques in the home, an opportunity for a more effective assessment of the necessity and appropriateness of equipment and the possibility of specialist physiotherapy during terminal care. There is also evidence of improved adherence with treatment and a reduction in the stress of coping with the disease (Rogers & Goodchild 1996).

Many patients awaiting heart-lung transplantation can be cared for at home, with a clinical nurse specialist visiting to provide assessment and to identify the need for changes in treatment to maintain optimal health status. It may be appropriate for a patient to receive either a course of intravenous antibiotics at home or to continue a course started in hospital.

Infants and small children

Historically a diagnosis of CF was confirmed in babies at some time during the first year of life, often following a symptomatic presentation of respiratory infection and failure to thrive. Traditionally ‘chest physiotherapy’ was instigated twice daily as soon as the diagnosis had been confirmed and treatment comprised the use of gravity-assisted positions and chest clapping. In the absence of specific radiological signs, most physiotherapists adopted a general drainage regimen (alternate side lying and prone in a head-down tipped position and supine flat) with the addition of the sitting position for the apical segments of the upper lobes in infants, as they spend much of their time lying down. Once the child began to sit and stand, the apical segments were omitted from treatment.

In the past few years, the approach to airway clearance in babies with CF has changed considerably. Many other airway clearance techniques have been developed (Chapter 5) and some of these, such as positive expiratory pressure (PEP) applied via a facemask, assisted autogenic drainage (AD) and physical activity are now used in the infant/paediatric population. Many centres throughout the world have modified the traditional approach by omitting the use of gravity-assisted positioning in the drainage regimen. This has been to a large extent due to growing clinical concerns regarding gastro-oesophageal reflux. Gravitational effects in the tipped position theoretically lead to a lowering of intra-abdominal pressure and an increase in intrathoracic pressure. This, together with the increase in diaphragmatic activity, may enhance the competence of the oesophageal sphincter (Sindel et al 1989). Despite this theoretical assumption, infants with CF are known to have a higher incidence of gastro-oesophageal reflux (GOR) and studies have suggested that this is exacerbated by use of the head-down tipped position during airway clearance (Button et al 1997). Other groups have not reproduced these findings, albeit in slightly differing cohorts (Phillips 1996, Taylor & Threlfall 1997). Longer-term follow-up data from the subjects included in the study by Button et al (1997) suggest that GOR may result in both short- and long-term sequelae in terms of respiratory status (Button et al 2003, 2004). These results have led many centres to discontinue using the head-down tipped position. This remains a slightly contentious issue, and there are centres that continue to use gravity-assisted positioning judiciously. When GOR is suspected, it should be investigated rigorously and if confirmed the airway clearance regimen may need to be modified and anti-reflux medication should be started.

Whichever airway clearance treatment is chosen, it is usually advised that it be undertaken before feeds and for approximately 10–15 minutes. If an infant or child has specific radiological signs, or in the presence of a lower respiratory tract infection, treatment may need to be intensified in terms of frequency and duration.

Early diagnosis of CF, particularly since the introduction of neonatal screening in many parts of the world, along with early and aggressive multidisciplinary care, has led to a novel cohort of infants who are apparently free of any respiratory symptoms and are nutritionally healthy. The appropriateness of implementing a routine daily airway clearance regimen in this cohort of infants has raised much debate. Unfortunately there are no studies that have evaluated whether the routine instigation of airway clearance regardless of clinical status is beneficial. The debate continues widely on the international stage and opinion remains divided (Prasad & Main 2006).

Arguments for routine treatment are threefold. First, many feel it is essential to establish a daily routine in order to ensure adherence to therapy in the long term. Secondly, the anatomical and physiological differences in the infant respiratory system (Chapter 10) may make them more vulnerable to chest complications. Finally, there is conclusive evidence that pathophysiological changes in the lungs occur very early, before the onset of clinical signs, with evidence of early inflammation and infection (Armstrong et al 1995), altered respiratory function (Ranganathan et al 2001) and structural changes radiologically (Martinez et al 2005).

However, while the evidence that pathophysiological changes occur early in the disease process is compelling, the early picture is usually not one associated with copious secretions. The value of a daily airway clearance regimen is therefore unclear (Bush & Gotz 2006). In addition there is no doubt that a significant burden of care is imposed on families by routine treatment regimens.

There is little robust evidence with respect to best physiotherapy practice in this group of infants (Button 1999, Constantini et al 2001). The few existing studies that attempt to evaluate the efficacy of airway clearance or to compare the various airway clearance modalities have been undertaken in older populations with established disease (Desmond et al 1983), as have the majority of studies comparing the efficacy of the various techniques (Elkins et al 2006b, Main et al 2005). It is unlikely to be appropriate to extrapolate findings from these studies to a healthy infant who shows no overt signs of respiratory involvement. In the United Kingdom the proposal of a national neonatal screening programme provided a unique opportunity to undertake a randomized controlled trial of the efficacy of routine daily chest physiotherapy in screened infants with CF. Unfortunately this trial did not come to fruition but in attempting to address this important issue, the Association of Chartered Physiotherapists in Cystic Fibrosis (United Kingdom) undertook a consensus exercise based on the Delphi process (Jones & Hunter 1995) in order to provide expert opinion and guidance for the future care of these infants (Prasad et al 2008). The results of the Delphi process have resulted in guidelines which state that physiotherapists are not required to initiate routine airway clearance if, following careful assessment, the child is well and felt not to have symptoms which would respond to respiratory physiotherapy. It is important to stress that all parents and carers should be taught an appropriate airway clearance regimen, and this is practised and revised to maintain competency. Airway clearance is instigated whenever there are respiratory symptoms and an assessment tool is being developed to assist parents with assessment (Fergusson, personal communication 2007). The Delphi process has also recognized that the more recently developed airway clearance modalities may be more appropriate for these infants, rather than traditional postural drainage or modified postural drainage and percussion, and that physical activity should be greatly emphasized from the outset. The suggestion is not that treatment should be withdrawn, but that a different emphasis be placed on the management to include physical activity and a more flexible and holistic approach supported by an easily accessible specialist physiotherapy service.

Even at a young age treatment should be fun. The young child can be bounced up and down on the parent’s knees, exercises such as ‘wheelbarrows’, jumping on a mini-trampoline (Fig. 18.2A) or the use of a gym ball (Fig 18.2B). Laughing often also stimulates coughing. From the age of 2 years the child can be encouraged to actively participate in breathing techniques in the form of play and other airway clearance modalities (Chapter 5) can be introduced as the child grows. PEP can be made more enjoyable if administrated in the form of bubble PEP (Chapter 5). From as early an age as possible children should be encouraged to play a more active role in their treatment. With increased cooperation, the child can be introduced to various airway clearance techniques and become independent with treatment (Box 18.1).

Figure 18.2 (A) Jumping on a mini-trampoline. (B) Exercise on a gym ball.

Box 18.1 Airway clearance techniques

Active cycle of breathing techniques (ACBT)

Autogenic drainage (AD)

Modified autogenic drainage (M AD)

Exercise

High-frequency chest wall oscillation (HFCWO)

Intrapulmonary percussive ventilation (IPV)

Oscillating positive expiratory pressure:

Acapella^®

Flutter^®

R-C Cornet^®

Positive expiratory pressure (PEP):

PEP

High PEP

Postural drainage and percussion

Infants and small children swallow their bronchial secretions, but as soon as possible expectoration should be encouraged. Nasopharyngeal suction should only be used in babies if it is essential to obtain a sputum specimen or if the infant is distressed by secretions. Learning to blow the nose is also important, to keep the upper airways clear.

Airway clearance

The removal of bronchial secretions remains the mainstay of physiotherapy management as bronchial infection and respiratory failure continue to be the major causes of morbidity and mortality.

Infected bronchial secretions are responsible for many complications in the airways and lung tissue (Fig. 18.3). Obstruction occurs initially in the small airways, with repeated infections and hypersecretion resulting in damage to the airway wall, central airway instability and hyperreactivity. Infected secretions in cystic fibrosis are dehydrated, hyperadhesive and hyperviscoelastic. Studies of airway clearance techniques have attempted to identify characteristics to address some of the problems of the airway and secretions. The presenting pathological problem should be considered when choosing an airway clearance technique (Lapin 2000).

Figure 18.3 The pathophysiological consequences of sputum.

The physiological principles of airway clearance are discussed in Chapter 5. In order to achieve effective treatment, secretions should be mobilized and removed without causing an increase in airway obstruction or fatigue. The evidence-based airway clearance techniques (see Box 18.1) all aim to enhance airflow, increase lung volume and may alter the rheological properties of mucus. In people with CF who have bron chial wall instability, expiratory airflow is likely to be reduced when intrathoracic pressures are high (Zach et al 1985). Huffing generates lower intrathoracic pressure than coughing (Langlands 1967) and it has been suggested that it is as effective as coughing for mucociliary clearance (Hasani et al 1994). By altering the intrathoracic pressure and the lung volume at which the expiratory manoeuvre is performed, the physiotherapist can tailor the point of compression to the area of obstruction without causing airway collapse. Autogenic drainage prevents airway collapse by maximizing airflow at different lung volumes and avoiding high-pressure peaks (Schöni 1989).

The changing clinical picture of people with CF, with improved clinical status and a generally more active lifestyle, together with an increased understanding of the pathophysiological concepts of airway clearance techniques, have obviated the emphasis on gravity-assisted positioning for airway clearance. Research has shown that ventilation to the dependent area of the lung may be more effective than gravity-assisted ‘drainage’ from the uppermost lung (Lannefors & Wollmer 1992). Elkins et al (2005a) have shown that huffing and coughing are compromised in the ‘head-down tilt’ and side-lying positions.

Currently there is a lack of robust evidence to support the long-term benefit of airway clearance. Trials are confounded by small numbers, inconsistency of techniques and no control population (Prasad & Main 1998). To date the majority of trials have been short term and undertaken mainly in adults. A meta-analysis of chest physiotherapy suggested that airway clearance produced significantly greater sputum expectoration than no treatment and the addition of exercise further improved lung function (Thomas et al 1995). However, a systematic review was not able to demonstrate a benefit for treatment compared with no treatment, although short-term studies indicate that there may be deterioration in lung function during periods without treatment in those with established disease (van der Schans et al 2000).

Systematic reviews have not identified any single airway clearance technique as being superior (Elkins et al 2006b, Main et al 2005) and longer-term RCTs support these findings (Accurso et al 2004, Pryor et al 2006a). However, patient preference is likely to be an important factor in terms of adherence. Constant review of techniques is essential to determine the most effective regimen to meet changing lifestyles and disease progression during the clinically stable and acute state.

Exercise offers an important contribution to sputum expectoration (Baldwin et al 1994, Sahl et al 1989), but in the majority of patients it should be complementary and not exclusive (Bilton et al 1992). Patients perceive exercise differently to other forms of treatment (Abbott et al 1996) and some prefer this method of airway clearance. It is important for the physiotherapist to be sensitive to the patient’s beliefs (Carr et al 1996), but encourage formal airway clearance during an acute exacerbation when the patient is unable to exercise at his normal level.

The frequency and duration of treatment will vary. When secretions are minimal, treatment once a day may be sufficient but additionally some form of exercise should be encouraged. Many patients will require treatment two or three times a day, but the programme should be realistic and allow for other normal activities. Some techniques may be more time consuming to perform, difficult to learn and may be position dependent. Others involve equipment that requires meticulous cleaning. The choice of technique should be individualized to suit the patient’s age, lifestyle, preference and disease severity.

Maintenance / increase in exercise tolerance

The value of exercise in the management of CF is now well established. Short-term studies of exercise training programmes in cystic fibrosis have been shown to have considerable therapeutic benefit and the majority of patients wish to include exercise in their routine self-care (Webb & Dodd 2000). Studies have shown improved exercise tolerance (Andreasson et al 1987, Edlund et al 1986, Freeman et al 1993), ventilatory muscle endurance (Keens et al 1977), cardiorespiratory fitness (Orenstein et al 1981), muscle bulk and body image (Strauss et al 1987), decreased breathlessness (O’Neill et al 1987) and improved quality of life (de Jong et al 1997). Two randomized controlled trials of home exercise programmes have demonstrated the long-term value of exercise (Moorcroft et al 2004, Schneiderman-Walker et al 2000). Early studies demonstrated that patients with mild to moderate disease (FEV₁ = 55% predicted) could exercise to the same level as their peers, but those with more severe disease (FEV₁ < 55% predicted) would require individualized recommendations and supervised exercise programmes (Cropp et al 1982). Everyone can exercise and no patient should be excluded because of disease severity (Webb & Dodd 2000). The benefits of exercise should be introduced and emphasized from a very early age.

Assessment of exercise capacity

The patient’s baseline exercise capacity should be assessed, when the patient is clinically stable, to determine their level of fitness and limitations. The results will give guidance for effective and safe exercise recommendations. The test will provide a baseline measure for further testing to monitor improvement or change in any values and evaluate an intervention. Assessment should consider the choice of protocol, the type of test and the measurements required. The choice of protocol (Table 18.2) depends on the information required, the facilities available and the patient’s clinical condition. It may be desirable to determine a functional level of exercise in preference to peak performance (Jones 1988).

Table 18.2 Types of exercise test

Space may be limited and a cycle ergometer may therefore be more appropriate than the modified shuttle test (Bradley et al 1999). The patient may be too breathless to perform a maximal test and some tests may be too difficult for children to perform. The step test may be a validated alternative to measure functional exercise capacity (Balfour-Lynn et al 1998). The measurements and equipment required are outlined in Table 18.3. The standard measures of work capacity (distance walked, wattage), pulse, oxygen saturation and a subjective measure of breathlessness and muscle fatigue are sufficient for routine assessment (Fig. 18.4). More sophisticated measures give additional information but are not necessary for routine use. Safety precautions during testing should include personnel trained in resuscitation; oxygen and appropriate drugs for resuscitation should be immediately available in the exercise area. From the results of the test, the physiotherapist can recommend a level of exercise to provide an appropriate training effect that is safe. It is also useful to establish the pattern of habitual activity. Some patients are unwilling to participate in formal exercise programmes, but are happy to increase everyday activity.

Table 18.3 Measurements and equipment required for assessing exercise capacity

VAS, visual analogue scale; _E, minute ventilation; V_T, tidal volume; TiTOT, ratio of time spent in inspiration to total respiratory time; RR, respiratory rate; CO₂, carbon dioxide; PaO₂, partial pressure of oxygen in arterial blood; PaCO₂, partial pressure of carbon dioxide in arterial blood

Figure 18.4 Assessing exercise capacity with cycle ergometry and pulse oximetry monitoring.

Exercise programmes

Exercise is limited by the symptoms of breathlessness or muscle fatigue. The aim of an exercise programme is to improve exercise performance, make a given level of exercise more comfortable and increase the activities of daily living. It is important to establish the goals of an exercise programme for the individual patient, which may be different for carer and patient (Table 18.4). Exercise programmes must be tailored to the individual, based on disease severity, level of fitness and patient preference.

Table 18.4 The differing aims of exercise programmes for the patient and carer

Types of exercise

An exercise progamme should combine endurance and strength-training exercises for upper and lower body. Attention has also been paid to the value of anaerobic exercise and strength training, especially in children whose natural activity patterns are characterized by short bursts of vigorous physical activity (Klijn et al 2004, Orenstein et al 2004).

Endurance exercise aims to improve the capacity to endure more exercise without discomfort, e.g. swimming, running, cycling, skipping, aerobic classes, step aerobics, trampolines (Edlund et al 1986, Orenstein et al 1981, Sahl et al 1989) (Fig. 18.5).

Strength training aims to increase muscle mass and strength, e.g. weights and sprint training (Strauss et al 1987) (Fig. 18.6).

Interval training may be useful for those patients unable to sustain long periods of exercise. Short bursts of exercise at higher rates will enhance a training response. It may be of benefit for those patients with prolonged periods of desaturation.

The evidence for inspiratory muscle training (IMT) has previously been conflicting (Asher et al 1982, Sawyer & Clayton 1993) and to date has shown no advantage over general upper body muscle training (Keens et al 1977). Two randomized controlled trials of IMT in adults and children have shown a positive outcome on exercise performance and quality of life (Albini et al 2004, Enright et al 2004)

There are no studies to date evaluating the benefits of a lifestyle change. Parents of children and adolescents with CF have been reported to be less positive about the benefits of exercise than the parents of healthy children (Boas et al 1999). It is essential to establish from the time of diagnosis the importance of the contribution that exercise makes to a healthy lifestyle and to encourage participation of the whole family. There is a decline in physical activity in late adolescence (Britto et al 2000). Careful consideration and encouragement should be given to this age group at this time. Contact by the physiotherapist with local gym and sports facilities may offer reduced fees for exercise sessions.

Figure 18.5 Exercising on the treadmill.

Figure 18.6 Exercising with dumbbells.

Intensity, duration and frequency

Endurance exercise.

An effective exercise programme should make reasonable demands on the patient’s physical capacity and be progressive. The intensity can be derived from the results of the exercise test. Various recommendations have been suggested:

50% of peak work capacity is below the anaerobic threshold for most patients and represents a functional level of activity (Marcotte et al 1986)

50–60% peak oxygen consumption (V. Opeak) is necessary to improve physical fitness (Astrand & Rodahl 1977) and the patient’s capability is related to the percentage of their individual V.O₂peak

70–85% of the measured maximum heart rate is sufficient to achieve a training effect (Orenstein et al 1981)

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70% of the maximum speed attained on a maximal field test (Pryor et al 2006b)

‘breathlessness without distress’ for those patients with severe disease who are limited by ventilatory mechanics (Godfrey & Mearns 1971).

Exercise should begin at the chosen intensity for a period of time sufficient to cause breathlessness or muscle fatigue without undue stress and progress to 20–30 minutes 3–4 days a week. Progression for the patient who is limited by breathlessness will be at a slower rate than the unfit patient who is limited by muscular fatigue.

Precautions

There are no absolute contraindications but exercise should cease temporarily for the following medical problems: abdominal obstruction, an acute bronchopulmonary exacerbation associated with fever, transient arthralgia and arthritis, pneumothorax, persistent haemoptysis and surgery including a Caesarean section. Exercise-induced bronchoconstriction is rare in CF, but can usually be controlled with pre-exercise bronchodilators. Patients undertaking exercise in hot climates should be well hydrated and advised to supplement their salt intake (Bar-Or et al 1992). Exercise for the diabetic patient should be encouraged (see section on CF related diabetes). Certain sporting activities carry a medical risk (Webb & Dodd 1999). Contact sports, bungee jumping and parachute jumping are not advised for those patients with diagnosed low bone mineral density, portal hypertension and significant enlargement of the spleen and liver. Scuba diving could be hazardous for patients with air trapping and sinus disease. In the hypoxic patient, exercise at altitude poses a potential risk (Speechly-Dick et al 1992). Careful advice should be given to patients contemplating skiing and any strenuous/high-intensity aerobic and anaerobic exercise at altitude.

Exercising the patient with advancing disease

There is no evidence that carefully tailored and supervised exercise is harmful in these patients and they should not be excluded from a training programme. For patients with severe disease and those awaiting transplantation, deconditioning occurs rapidly. Maintaining mobility is crucial and strength and endurance exercises should be encouraged (Webb et al 1996). A maximal exercise test will define the limits of breathlessness and muscle fatigue. Exercise programmes should then be planned with these in mind. Positions for breathing control (Chapter 5) should be introduced to alleviate exertion breathlessness and increase mobility.

Studies have reported the benefits of oxygen supplementation for patients with severe disease (Heijerman et al 1992, Marcus et al 1992). Oxygen may be required to ease the symptom of breathlessness and increase exercise performance. Many patients are reluctant to use oxygen for activity, especially outdoors. Considerable benefits are achieved using oxygen before and after exercise for recovery. Patients can use small oxygen cylinders discreetly to enable them to travel and socialize in the community. During exercise and periods of re-covery, inspiratory flow rates increase, so the oxygen prescription and delivery device should be adjusted to relieve exercise-induced breathlessness. Patients mouth breathe during exercise and periods of breathlessness and a fixed concentration mask at low concentration and high flows may provide greater relief than nasal cannulae (Dodd et al 1998). The oxygen flow should be titrated to a level of comfort which provides an arterial oxygen saturation >90% (Heijerman et al 1992). For patients who are dependent on non-invasive ventilation, exercise should also be performed using the equipment (Webb et al 1996). This can take the form of weight training in sitting and step-ups in standing or walking on a treadmill (Fig. 18.7). Careful assessment with attention to respiratory rate, perceived breathlessness and exertion can provide a comfortable level of exercise without undue breathlessness, with or without oxygen to maintain mobility and quality of life.

Figure 18.7 Exercising with non-invasive ventilation.

(Reproduced with permission from Webb et al 1996.)

Bronchodilators

Bronchodilators are prescribed to relieve airway obstruction. Careful evaluation by reversibility testing and subjective benefit will determine the response and the merits of the inhaled or nebulized route for the individual patient. Beta-adrenergic drugs increase cilial action, improve mucociliary clearance (Wood et al 1975) and may be of benefit before chest clearance (Kuhn & Nahata 1985). During an acute exacerbation, when secretions are excessive and obstruction is increased, the nebulized route may be more effective (Conway & Watson 1997). Inhalers are available in a range of doses. It is preferable and less time consuming for the patient to be prescribed a device that will deliver a higher dose of medication than to increase the number of puffs of a lower dose.

The value of long-acting b₂-agonists has been described (Bargon et al 1997) but the place of inhaled corticosteroids is questionable (Balfour-Lynn et al 1997, 2000, Bisgaard et al 1997). Withdrawing steroids in selective patients would however appear to be safe and not affect respiratory exacerbation rates (Balfour-Lynn et al 2006). CF patients often show a dip in PEFR and report symptoms of tightness in the early evening. One can speculate that this is because drugs are metabolized faster in patients with CF so that the benefits of long-acting drugs are shortened. It is important to select a device that is both acceptable to the individual patient and matches their inspiratory flow rate. Generally CF patients have high inspiratory flow rates. There are various teaching devices to assess patient technique and suitability for the required flow rate of the device, but careful consideration must be given to the risk of cross-infection in this patient group. Some commercially prescribed devices require specific inspiratory flow rates for effective deposition. The Accuhaler^®(GlaxoSmith-Kline) is a dry powder inhaler, which is less dependent on inspiratory flow (Hill & Slater 1998). It is less confusing for the patient if the same device is prescribed for all medications. It is sometimes possible to combine two types of drug in one preparation, e.g. Seretide^® (GlaxoSmithKline) combining a corticosteroid and long-acting bronchodilator. The physiotherapist should always be mindful of the treatment burden on CF patients.

Mucolytic agents

Inhalation of hypertonic saline by an ultrasonic nebulizer has been shown to improve lung function and perceived effectiveness of chest physiotherapy over a 2-week period (Eng et al 1996) and to improve mucociliary clearance after one inhalation (Robinson et al 1996). In both of these studies the positive effects were significantly increased when compared with 0.9% saline. Hypertonic saline may induce bronchoconstriction (Rodwell & Anderson 1996). A test dose should be given, with recordings of PEFR or FEV₁ before and 5 minutes after inhalation, to identify any increase in airflow obstruction. In a large controlled long-term study of twice daily 7% saline, respiratory exacerbations were reduced and the treatment was considered to be safe if preceded by a bronchodilator (Elkins et al 2006a).

The optimal drug effect of rhDNase is different for each individual and can vary from 30 minutes to several hours. It is recommended initially that inhalation should be performed before early evening to avoid coughing during sleeping hours and 30 minutes to 2 hours before chest clearance (Conway & Watson 1997). Some patients who have difficulty clearing sputum in the morning gain considerable benefit from inhalation of rhDNase at night, without coughing during sleeping hours. Treatment regimens should therefore be individualized and frequently assessed as the disease changes. When the optimum time has been determined, inhalation in relation to chest clearance can be recommended. The effect on lung function has been shown to be equally as effective with alternate day dosing as daily (Suri et al 2002). This may decrease the treatment burden and cost, but the effect on sputum viscosity should be carefully monitored. rhDNase should not be mixed with other drugs as it requires isotonic conditions and a neutral pH for maximal activity. If nebulized antibiotics or inhaled steroids are part of the treatment regimen, the pH of these solutions is acidic and may denature the protein (Ramsey & Dorkin 1994). At least 30 minutes should separate antibiotic and rhDNase inhalation (Conway & Watson 1997). A sidestream nebulizer (Respironics) has been recommended for inhalation (Shah et al 1997). The nebulizer chamber used for rhDNase should not be used for any other drug. rhDNase should be stored at 0–4°C and should be removed from the refrigerator and brought to room temperature (approximately 15 minutes) before inhalation to avoid bronchoconstriction. Other mucolytic agents, for example acetylcysteine (Parvolex^®), are also said to reduce mucus viscosity, but have been less frequently used since the introduction of rhDNase and hypertonic saline.

Antibiotics

Aerosolized antibiotics should be inhaled following airway clearance. Studies have shown that bronchoconstriction may be induced by some preparations of inhaled antibiotics. (Chua et al 1990, Cunningham et al 2001, Maddison et al 1994, Nikolaizik et al 1996). A test dose should be performed when the patient is clinically stable, to detect any increase in airflow obstruction. Spirometry should be performed before, immediately and up to 30 minutes following inhalation (Webb & Dodd 1997). Bronchoconstriction may be prevented by altering the tonicity of the solution to iso- or hypotonic (Dodd et al 1997). The long-term effects of repeated bronchoconstriction are unknown and it is now recommended that the inhalation of antibiotics should be preceded by an inhaled bronchodilator (Chua et al 1990, Webb & Dodd 1997).

The details of inhaling several drugs can be complex. It is crucial that the patient is given verbal and written instructions of how to use (including the cleaning and maintenance of any equipment), when to use and the order of use in relation to airway clearance and other drugs. This complexity may be simplified by the admixture of inhaled solutions /suspensions (Wolfgang et al 2006). While the physico-chemical compatibility is known, the aerodynamic characteristics of a mixture require further study.

Intelligent nebulizers

The development of a new generation of nebulizer delivery devices utilizing vibrating mesh technology e.g. eFlow^® rapid (PARI), I-neb^TM AAD^® System (Respironics UK), has revolutionized the efficiency and efficacy of drug delivery, deposition and patient acceptability of this burdensome treatment. Treatment times are shorter with audible feedback on completion of inhalation. The devices are small, silent and battery driven. Incorporated within this technology in some devices (e.g. I-neb^TM AAD^®) is an adaptive aerosol delivery (AAD) and ‘controlled inhalations’. These devices adapt to the individual’s breathing pattern and deliver a preset dose during a proportion of the inhalation phase only. A precise dose is always delivered irrespective of the breathing pattern and environmental contamination is minimized. A controlled inhalation by the device restricts the patient to a slow deep inhalation to maximize peripheral airway deposition (Mullinger et al 2004).

Humidification of oxygen

It is recommended that oxygen should be humidified in CF, due to the basic defect in the airway. It is suggested that water vapour humidity is preferable to nebulized humidity for maintenance of PaO₂ (Kuo et al 1991). Oxygen delivered by nasal cannulae can be humidified with a bubble through water vapour humidifier to a maximum flow of 6 litres. A heated humidifier (e.g. Aerodyne, Kendall, Basingstoke, Hampshire; Fisher & Paykel, Maidenhead, Berkshire) is necessary to humidify the large volumes of gas delivered by 24% and 28% concentration masks and a high-flow generator because the time interval for moisture transfer decreases as flow rate increases above 20 l/min.

The large volumes of air delivered by NIV may cause drying of secretions and require humidification by a heated water vapour source. This may increase the problem of breakdown of the skin on the bridge of the nose, and in those patients humidification may have to be reserved for an acute exacerbation. In the terminal stage of the disease, when secretions are very tenacious, it may be necessary to humidify the oxygen delivered to the nasal mask with a bubble-through humidifier.

Domiciliary oxygen

Transferring oxygen into the community can be a challenge (Dodd et al 1998). Oxygen concentrators are usually prescribed for night-time use. The driving pressure is much lower than that of a cylinder and although adequate for use with nasal cannulae and NIV, there may be difficulties with the use of Venturi masks. Cylinders and 24% or 28% Venturi masks are recommended for relief of breathlessness. Liquid oxygen is the ideal system for CF patients and allows mobility outside the home. Careful assessment is required for overnight, ambulatory and short-burst oxygen to determine the required flow rate, concentration and hours of use. In the United Kingdom, explicit details are given to the contractors for domiciliary oxygen to enable them to work with the patient to decide which technology would best suit their therapeutic need (Department of Health 2004).

Advanced cystic fibrosis

The late stages of the disease are characterized by repeated exacerbations, often with continuous intravenous antibiotics and increased hospitalization, respiratory failure and reduced mobility. It is essential to maximize the reversal of airway obstruction with airway clearance techniques, nebulized and intravenous bronchodilators and steroids. Sputum viscosity is reduced with rhDNase and intravenous fluids, and expectoration facilitated with hypertonic saline.

Shortness of breath may be reduced with the use of humidified oxygen, careful positioning and relaxation techniques. Type I respiratory failure requires meticulous evaluation at rest and during mobility for the requirements of oxygen. Hypercapnic respiratory failure may be reversed with NIV. Mobility is crucial for patients who are listed for transplantation and to maintain quality of life. Some patients have difficulty coming to terms with their loss of independence and input from a clinical psychologist experienced in the treatment of cystic fibrosis may prove beneficial.

Despite optimal medical and physiotherapeutic care, the terminal stage of the disease will ensue. It is inappropriate to withdraw support in the terminal stages even though physiotherapy may no longer be effective. Assistance can be given with positioning breathless patients in high side lying or sitting leaning forward, to make them as comfortable as possible. Occasionally IPPB can be used to assist the clearance of secretions from the upper airways, but care must be taken to use it only as a part of physiotherapy treatment and not as a form of pseudoventilation. Nasotracheal suction is not indicated as it would serve no useful purpose at this stage. Morphine or one of its derivatives will help to relieve anxiety and breathlessness.

Allergic bronchopulmonary aspergillosis

Allergic bronchopulmonary aspergillosis (ABPA) leads to airway narrowing, gas trapping and small airways disease. Tenacious mucus plugs, often brown in colour, and wheezing are common presenting symptoms of ABPA. Airway obstruction is increased and there is often a marked reduction in lung function (Kraemer et al 2006). Medical management includes oral steroids and antifungal agents (e.g. itraconazole or voriconazole). Mucus plugging can lead to lobar or segmental collapse. Bronchodilators may be helpful and airway clearance techniques should be adapted to minimize airway obstruction. IPPB may be indicated. Exercise tolerance may be reduced (Simmonds et al 1990) and programmes may need to be modified.

Arthropathy and joint pain

CF-related arthropathy can present as hypertrophic pulmonary osteoarthropathy (HPOA) or periodic arthritis (Koch & Lanng 2000). HPOA is related to pulmonary disease and presents most frequently in adults. Episodes of transient arthritis appear to be unrelated to pulmonary disease. HPOA is characterized by pain, swelling and warmth of the involved area and occasionally small joint effusions. Arthritis can be associated with fever and vasculitis. Active physiotherapy is not generally indicated. Joints should be rested and gentle mobilization encouraged, within the limits of the symptoms. The medical management for a pulmonary exacerbation may lead to a resolution of symptoms (Rush et al 1986) and CF arthritis usually resolves spontaneously (Turner et al 1997).

Cystic fibrosis-related diabetes

The specific changes in glucose metabolism in CF are well described (Bridges & Spowart 2006, Koch & Lanng 2000). The incidence increases with age and it is suggested that survival is reduced in this group. Hyperglycaemia causes polyuria, which leads to dehydration and a possible increase in sputum viscosity. Expectoration is difficult and lung function declines. The physiotherapist should be alert to the results of blood glucose monitoring (BM) in the analysis of chest symptoms and formulation of a treatment plan. It is essential that the medical management includes control of blood sugar and rehydration with intravenous fluids. When diabetes is difficult to control, attention should be directed to the application of airway clearance techniques which reduce viscosity and the consideration of rhDNase.

Insulin requirements will change with exercise. Exercise can sometimes improve overall blood glucose control by reducing insulin requirements. When diabetes is well controlled, exercise may induce hypoglycaemia either during exercise or up to 24–36 hours after exercise (Cystic Fibrosis Trust 2004c). The balance of insulin requirement and carbohydrate intake should be discussed with the CF dietitian and physician and individualized for both normal activity and exercise programmes. It is important to maintain exercise during admission to hospital because reduced activity will result in an increase in BM values. If the diabetes is labile during an acute exacerbation, a pre-exercise BM is advisable in addition to close liaison with the dietitian.

Distal intestinal obstruction syndrome

This complication describes intestinal obstruction occurring after the neonatal period (Davidson 2000). It is characterized by abdominal pain, distension, vomiting, palpable fecal masses and partial or complete intestinal obstruction. Urgent medical management includes intravenous rehydration, Gastrografin^® and possibly a balanced electrolyte solution. The symptoms of immobility, pain, decreased functional residual capacity and dehydration compromise the clearance of secretions. It is important for airway clearance to continue, but techniques should be modified until the problem resolves. Mobilization is encouraged and may help bowel movement. For patients with a persistent problem, a lower body exercise programme may be useful.

Gastro-oesophageal reflux

This is well recognized in CF with a higher incidence in infants. The cause and effect of physiotherapy positioning for this group is unclear and has been discussed earlier. Treatment is directed at medical management with the use of prokinetic agents and/or gastric suppressants, thickening of feeds and appropriate positioning. The adult is often unaware that gastro-oesophageal reflux (GOR) is a complication of CF and the symptoms may be under-reported. The physiotherapist may be alerted to the possibility of GOR if the patient reports symptoms during airway clearance (for example upper gastrointestinal discomfort). In adults with more severe disease GOR, both silent and symptomatic, can be a significant problem (Button et al 2005a). Oxygenation and reflux scores have been shown to deteriorate in the head-down tilt position. This may be of significance when undertaking airway clearance treatments, particularly in the acutely unwell individual with CF (Elkins et al 2005a).

Haemoptysis

Blood streaking of sputum frequently occurs, especially at the time of an acute pulmonary exacerbation. It is appropriate to continue with the normal airway clearance regimen and to reassure the patient. Frank haemoptysis can be considered moderate at <250 ml or severe at >250 ml (Hodson 1994) and it is important to establish the volume expectorated over 24 hours to determine the treatment plan (Table 18.5). It is important to continue with chest clearance to remove the blood and infected secretions (Hodson 1994). Management is aimed at clearing secretions without increasing the bleeding. Noting the activity and position at the time of haemoptysis will provide a clue to the cause and influence management. The weakened artery may rupture due to increasing heart rate or increasing the flow of blood when the area of lung supplied by the artery is dependent (the bronchial arteries lie posteriorly). If the patient can establish the location of the bleed, it is advisable to avoid chest clearance with the affected lobe dependent (Bilton et al 1990).

Table 18.5 The treatment of haemoptysis

When haemoptysis is severe, the location of the bleeding should be dependent to avoid asphyxiation (Jones & Davis 1990). When the bleeding has subsided, chest clearance can be resumed. Bronchoscopy will locate the bleeding for those patients with persistent haemoptysis and the management usually requires embolization (King et al 1989, Vidal et al 2006). Chest clearance can resume following the procedure in consultation with the radiologist. Implantable venous access devices, which require flushing with an anticoagulant to maintain patency, may increase the risk of haemoptysis during intravenous therapy. If this occurs, the use of normal saline for flushing may lead to cessation of bleeding.

Liver disease

Liver disease usually presents as biliary cirrhosis, but fatty infiltration is a recognized feature. Hepatosplenomegaly and portal hypertension ensue with the development of oesophageal varices and potentially life-threatening haematemesis (Westaby 2000). Liver transplantation is an increasing feature of adult CF care. An enlarged liver and spleen will compromise respiratory mechanics and cause breathlessness. Careful consideration should be given to positioning during airway clearance. Airway clearance should be discontinued during acute haemorrhage. Careful exercise advice is recommended (see exercise section).

Low bone mineral density

Low bone mineral density (BMD) is a recognized complication of CF in both children and adults (Bachrach et al 1994, Bhudmkanok et al 1996). The correlates of low BMD include poor absorption of vitamins, minerals and protein from the gut, oral steroids, inactivity, increased cytokines from infection and hypogonadism (Haworth et al 1999). The problem is one of reduced bone accretion in childhood and early and increased rate of bone resorption in adult life. Bone mineral density appears to be normal until the age of about 10 years and seems to decrease during the adolescent years (Cystic Fibrosis Trust 2007). Lack of physical activity is a known correlate, but there is no direct evidence that lack of physical activity leads to low BMD and it is currently unknown if weight-bearing activities can increase peak bone mass, preserve BMD or increase BMD in those with low BMD. However, it would seem prudent to encourage weight-bearing exercises based on studies in the healthy population (Cystic Fibrosis Trust 2007) There is an increased risk of rib fractures (Bachrach et al 1994) and there is some concern that manual techniques during airway clearance could cause a rib fracture. While there are no reports to date of manual techniques causing rib fractures in CF, there are other methods of airway clearance with proven efficacy which should be considered as alternatives (Cystic Fibrosis Trust 2002b). Coughing has been reported to cause a rib fracture in CF (Elkin et al 2001). A regular review of airway clearance techniques is essential in this vulnerable group of patients.

Rib and vertebral fractures are extremely painful and inhibit airway clearance and mobility. A chest X-ray should always be performed to exclude a pneumothorax. Mucus plugging and an infective exacerbation may quickly ensue. Effective analgesia is a priority before airway clearance and mobilization. Effective coughing should be facilitated by support to the chest wall and the use of antibiotics and rhDNase should reduce sputum volume and ease the mobilization of secretions.

Musculoskeletal dysfunction

Patients with CF develop alterations in chest wall mechanics and spinal deformities due to progressive lung disease, malnutrition and poor bone mineralization (Tattersall et al 2000). This leads to abnormal posture, pain and poor quality of life (Dodd & Prasad 2005). Postural screening and specific testing will direct the design of targeted interventions to improve musculoskeletal and neuromuscular impairments (Massery 2005). Musculoskeletal abnormalities are secondary to respiratory impairment. An understanding of the relationship between the respiratory and postural muscles of the trunk is critical to planning an effective strategy to address the musculoskeletal problems in CF. Current clinical evidence would suggest that an optimum time to screen and intervene, to minimize or prevent musculoskeletal problems, would be during the prepubescent years. Postural adjustments may correct kyphosis and lordosis, but the value of postural therapy needs further evaluation in CF. The flexible ruler technique has been validated in adults with CF to assess spinal deformity and may provide a simple non-invasive tool for routine use in the clinical setting (Boyle et al 2003). Other outcome measures to be considered would include optimal postural development, improved breathing mechanics, prevention of joint conditions, development of fewer pulmonary complications and functional and quality of life issues (Massery 2005). Manual therapy techniques may increase thoracic mobility and may improve lung function (Vibekk 1991). Musculoskeletal abnormalities are a consequence of improved survival and the physiotherapist should be proactive in addressing postural concerns and symptoms and treat appropriately. Flexibility and postural exercises should be introduced in childhood as part of an exercise regimen to prevent the problems that are reported with increasing age and disease severity.

Pneumothorax

In people with CF a pneumothorax usually occurs with advanced disease and chronic Pseudomonas infection (Penketh et al 1982). It is associated with increased mortality and may be an independent indicator of prognosis. A pneumothorax may occur due to rupture of a subpleural bleb through the visceral pleura or, rarely, as a result of misplacement of a central line. The physiotherapist may be alerted to the problem by a sudden increase in breathlessness and shoulder tip pain.

The aim of physiotherapy management is to clear secretions, aid re-expansion of the collapsed lung and prevent infection in the re-expanding lung (Table 18.6). It is usual for prophylactic intravenous antibiotics to be given at this time because airway clearance and mobility are compromised. When the pneumothorax is small (<20% of lung volume) treatment is conservative and airway clearance should continue with the aim of clearing secretions without increasing the size of the pneumothorax. Techniques that increase intrathoracic pressure and paroxysmal coughing should be avoided and positive pressure devices should be used with caution. Mobility is encouraged but exercise programmes should temporarily cease. An increase in symptoms may be an indication that the pneumothorax has increased in size. This should be immediately reported and an urgent chest X-ray requested.

Table 18.6 The treatment of a pneumothorax

VATS, video-assisted thoracoscopic surgery

Larger pneumothoraces will require an intercostal drain and, if persistent, video-assisted thoracoscopic surgery (VATS) is the preferred treatment. In the light of lung transplantation, pleurodesis should be avoided. While the drain is in situ, airway clearance techniques are reintroduced as indicated at assessment and with appropriate analgesia. Mobilization and gentle exercise are encouraged. When the intercostal tube is removed, care should be taken with airway clearance, in the short term, to prevent any reoccurrence of the resolving pneumothorax. Normal activities are advised, but strenuous exercise and lifting are not recommended. Patients should be cautioned about driving and air travel is contraindicated for 6 weeks (British Thoracic Society 2004). Following surgery, airway clearance should be restarted as soon as possible with adequate analgesia.

Pregnancy

Improved survival has resulted in more females reaching reproductive age. Pregnancy is well tolerated by patients with an FEV₁ >60% predicted, but associated with increased maternal and fetal complications in those with an FEV₁ <60% predicted (Edenborough et al 1995). As pregnancy can stress the pulmonary, nutritional and cardiovascular reserves of the patient with CF, knowledge of the normal changes (Elkus & Popovich 1992, Weinberger et al 1980) and their implications in CF (Kotloff et al 1992) is useful (Table 18.7). Airway clearance techniques should continue throughout pregnancy and be modified as pregnancy progresses with consideration of the degree of breathlessness and discomfort. Breathlessness commonly occurs in the first and second trimesters and may be normal. Careful monitoring of the vital capacity is essential to identify the onset of an acute exacerbation. Elevation of the diaphragm reduces functional residual capacity and causes early airway closure. Attention should be paid to clearance of the lung bases to prevent the possibility of trapped secretions.

Table 18.7 The normal changes of pregnancy and the implications for patients with cystic fibrosis

AP, anteroposterior; TV, tidal volume; PaO₂, partial pressure of oxygen in arterial blood; PaCO₂, partial pressure of carbon dioxide in arterial blood; ERV, expiratory reserve volume; RV, residual volume; FRC, functional residual capacity; VC, vital capacity; TLC, total lung capacity

Gentle exercise during pregnancy should be encouraged and established aerobic programmes may be continued. Non-weight-bearing exercises, e.g. swimming and cycling, have a lower energy cost than weight-bearing exercises (Kotloff et al 1992). Weight training should not be introduced during pregnancy, but an established programme should be modified and discussed with the medical team. The importance of pelvic floor exercises should be stressed at the beginning of pregnancy and reinforced at each clinic visit. Leakage of urine is a problem for many women (Orr et al 2001) and pregnancy will further stress the pelvic floor. Abdominal supports are available for patients who suffer lower back pain or sacroiliac strain, in combination with postural advice and gentle exercise. Gastro-oesophageal reflux may present for the first time. Medication should relieve symptoms and prevent potential aspiration.

Following the birth, airway clearance begins immediately and physiotherapy may be required while the patient is in the delivery room. It is important to liaise with the midwife and medical team to ensure that pain relief is adequate. Sputum production may increase as the diaphragm descends and ventilation to the lower lobes improves. Gravity-assisted positioning, if indicated, can resume as soon as the mother feels comfortable. Pelvic floor exercises should continue and foot and leg exercises are introduced while the mother is immobile. Mobility begins as soon as the mother feels comfortable. The return to physical activity depends on prenatal fitness, maternal health during pregnancy and progress during the postnatal period. It is usual to begin with gentle forms of exercise such as walking and to build up to low-impact aerobics. Returning to an established programme will depend on the discomfort of the women’s abdomen and perineum; every mother will require individualized advice. In the postnatal period pelvic floor exercises should continue for at least 12 weeks, but many mothers give up after the postnatal check up at 6 weeks.

Surgery

Routine surgical procedures, for example insertion of totally implantable venous access ports and gastrostomy, have become increasingly common. In addition the improved longevity of people with CF has led to an increased frequency of surgical interventions either directly or indirectly associated with the disease. Careful pre- and postoperative assessment of this patient group is essential to minimize postoperative pulmonary complications. A preoperative admission for intravenous antibiotics and intensive physiotherapy is often undertaken before elective surgery. Physiotherapists in some centres use these elective surgical procedures as an opportunity to perform perioperative airway clearance during intubation and anaesthesia. Effective airway clearance during anaesthesia may in theory compensate for any postoperative respiratory deterioration related to the anaesthetic and surgery or compromised airway clearance techniques because of postoperative discomfort. In addition, bronchial lavage and suction during anaesthesia can provide valuable sputum samples for microbiological culture.

Relatively little is known about the immediate or postoperative effects of physiotherapy treatments under anaesthesia. A small paediatric study reported a significant reduction in lung function parameters (compliance and peak inspiratory pressure) associated with physiotherapy during anaesthesia compared with a group receiving airway care as deemed appropriate by the attending anaesthetist (Tannenbaum et al 2007). Both groups showed a non-significant decline in FEV₁ the day after surgery, compared with preoperative values, although there were no longer-term effects on FEV₁. Perioperative chest physiotherapy should not be performed routinely in patients undergoing elective surgical procedures; however, it should be considered if the benefits of removing secretions are deemed to outweigh the short-term risk. In these circumstances it may be prudent for the anaesthetist to consider modifying ventilatory support to counteract any short-term negative effects of treatment (Tannenbaum et al 2007).

Transplantation

The physiotherapist should be involved both before and after heart-lung or lung transplantation as outlined in Chapter 15.

Urinary incontinence

Leakage of urine is recognized internationally as a problem for women and girls with CF (Cornaccia et al 2001, Orr et al 2001, Prasad et al 2006), with higher prevalence rates (30–68%) than the normal population (12.8% in women aged 16–20 years and 35% in women over 35 years of age) (Chiarelli et al 1999, Thomas et al 1980). The studies report no relationship between prevalence and age, with onset occurring as early as 10 years (Dodd & Langman 2005). Leakage in men is described in CF, with higher than normal prevalence rates of 16% (Gumery et al 2005) compared with 1.7% in the general population aged 15–64 years (Maral et al 2001). Coughing is the major cause of leakage for the majority of women with increased frequency and severity during acute pulmonary exacerbations.

Studies have addressed the assessment and treatment of the pelvic floor muscles (Button et al 2005b, 2005c, McVean et al 2003). Button and colleagues measured pelvic floor muscle activity using electromyographic (EMG) activity, and ultrasound imaging during coughing and huffing. The authors concluded that the problem of leakage was likely to be related to reduced endurance during prolonged coughing rather than the strength or the timing of the contraction. Following a 3-month intervention of pelvic floor muscle training (exercise, electrical stimulation, biofeedback and bladder training), EMG and ultrasound imaging measures all improved and patients reported significantly fewer incontinent episodes. The improvement was sustained over the following 3 months. Over the same time period, McVean et al (2003) reported an improvement in pelvic floor muscle endurance (Oxford grade), with no change in strength, in a self-selected group of patients.

Despite the physical and social implications of urinary incontinence (UI), women report the major impact of the problem to be their ability to perform airway clearance and spirometry. The physiotherapist should pay particular attention to teaching controlled and effective coughing during ACTs (Langman et al 2006). Cornaccia et al reported no association between airway clearance and leakage, but all subjects were taught to practise ‘the knack’ (Miller et al 1998) before performing a cough. Continence during a cough depends on the complex interaction of the muscles of the abdomino-pelvic capsule, which work to their greatest advantage with the lumbar spine in a neutral lordotic position (Sapsford et al 2001). Careful positioning advice, to optimize cough and continence, will avoid the flexed spinal posture encouraged by the intense prolonged coughing in women with CF (Figs 18.8 and 18.9).

Figure 18.8 The flexed posture associated with coughing showing bulging of the abdominal wall.

Figure 18.9 Corrected positioning during A ACT and B coughing. Support is given to the pelvic floor.

Studies have revealed that women and girls are rarely forthcoming about the problem, are tolerant of their symptoms and face assessment and treatment with apprehension and embarrassment. A sensitive and open approach with early recognition of symptoms needs to be developed to evaluate this distressing problem.

As a result of our emerging knowledge it would seem prudent to adopt both preventative and active strategies to the management of UI (Button et al 2005b, Dodd & Langman 2005). This requires the collaborative expertise of specialist respiratory, incontinence and musculoskeletal physiotherapists.

Sputum weight

In clinical practice sputum weight or volume is a useful outcome measure of airway clearance in an individual in a stable state. It provides a baseline measure of the normal sputum production for the individual. Any increase in sputum, either during treatment or out of treatment, is a possible indication of an acute exacerbation of pulmonary infection. Total daily sputum weight during an acute exacerbation is less informative, but, together with lung function, will determine if the problem is one of increased infection or sputum retention (Table 18.8). During an admission for an acute exacerbation, the goal is to maximize sputum expectoration during periods of airway clearance and minimize expectoration at other times. Measures of treatment and out-of-treatment sputum weight will provide this information and indicate, to the less adherent patient, the value of airway clearance techniques. Radiolabelled aerosols have been used to measure rate of mucus clearance in efficacy studies of airway clearance techniques (Elkins et al 2005b, Lannefors & Wollmer 1992), but remain a research tool rather than a practical clinical measurement.

Table 18.8 The value of spirometry and sputum weight during an acute exacerbation

Lung function

FEV₁ is considered a gold standard measure of lung function. However, as an outcome measure in airway clearance studies it is not ideal as it may not be sufficiently sensitive to detect changes caused by sputum clearance. In addition, there are a number of other confounding factors that may influence this outcome in patients with chronic respiratory disease such as CF (Gibson 2005, Prasad & Main 1998, van der Schans et al 1999). However, measurements of FEV₁ in an individual, as a clinical outcome tool, are useful, in terms of assessing the response to an intervention (for example initiation of rhDNase therapy), and longitudinal measurements are helpful in monitoring disease progression (rate of decline). In children particularly, measurement of FEV₁ depends on effort and cooperation and is not always a reliable or sensitive measure of early lung disease (Aurora et al 2004). It cannot therefore be considered a robust tool for paediatric physiotherapy intervention studies. Lung clearance index (LCI) calculated from multiple breath inert gas washout has recently been proposed as a more sensitive alternative measure of peripheral lung disease (Aurora et al 2005b). Measurement of lung function is now possible in infants and preschool children (Ranganathan 2006), although it is not widely available.

Other spirometric measurements are also used. The expiratory flow volume curve identifies the location of any flow limitation throughout expiration from total lung capacity (TLC) to residual volume (RV). This will vary from the early detection of small airway limitation to large airway collapse and gives the physiotherapist an invaluable insight into the problems of airway clearance. The manoeuvre is performed with an open glottis and therefore mimics a huff from TLC to RV. In the long term, measurements of small airway function (FEF₂₅ and FEF₇₅) are not comparable due to changes in TLC and RV and should therefore be interpreted with caution.

Arterial / capillary blood gases

Serial measurements of PaO₂ and PaCO₂ monitor decreases in oxygen tension and ensuing hypercapnia. PaCO₂ levels inform the physiotherapist of ventilatory drive and the work of breathing. Pulse oximetry gives a quick measure of oxygen saturation, but should not be used to prescribe oxygen as the corresponding PaCO₂ is unknown.

Subjective measures

Visual analogue scales (VAS) and Borg scores (Chapters 3 and 14) provide important subjective measures of breathlessness, chest tightness, muscle fatigue and pain. The 15-count breathlessness score and ease of breathing scores have been validated in children (Orenstein 2002, Prasad et al 2000).

Other useful measures include health status/quality of life questionnaires (Congleton et al 1997, Gee et al 2000, Orenstein et al 1989, Shepherd et al 1992) and activities of daily living. More recently emphasis has been placed on the importance of patient-reported outcomes that are disease-specific and one such measure has been validated in the CF population (Quittner et al 2000).