Anita Watson and Kim Pollard. Jan, 2001. Physiotherapy [online]. Seacroft University Hospitals, Leeds, UK. Available from http://www.cysticfibrosismedicine.com
Chest physiotherapy is an integral part of the management of cystic fibrosis. The aims are to reduce airway obstruction by improving the clearance of secretions, to reduce the severity of the infection by clearing infected material and to maintain optimal respiratory function and exercise tolerance (International Physiotherapy Group for Cystic Fibrosis booklet 'Physiotherapy in the Treatment of Cystic Fibrosis', 2nd Edition, 1995). All patients require an individual regimen to manage their airway clearance. This may be altered with disease progression or changing symptoms. All techniques can be modified if poorly tolerated or inconvenient.
Airway clearance techniques
The Active Cycle of Breathing Techniques: (Pryor et al, 1979; Webber et al, 1986; Webber, 1990). This consists of a combination of deep breathing exercises (DBE); thoracic expansion exercises (TEE); forced expiratory technique (FET) and breathing control. The Active Cycle of Breathing Techniques (ACBT) is not a rigid treatment method and is modified to suit all ages and individual needs.
Thoracic expansion: exercises with the emphasis on inspiration, expiration being quiet and relaxed.
The forced expiration technique or huff: is used to mobilise and clear secretions. One or two forced expirations are combined with a period of breathing control. A huff from high lung volume (when a breath has been taken in) will clear secretions from the upper airways and a huff from mid to low lung volume will clear secretions from the lower more peripheral airways.
Postural drainage: This may be used in conjunction with other techniques, e.g. ACBT, positive expiratory pressure (PEP) and percussion - it involves positioning to allow gravity to assist drainage of secretions based on bronchial tree anatomy (see seperate section on postural drainage for more details).
Percussion / Chest clapping: Can be performed with cupped hand(s) over the area being drained. It should be performed for approximately 15-20 seconds with pauses for 5 seconds or longer to minimise the risk of desaturation in patients with moderate or severe lung disease (Pryor et al, 1990). Mechanical percussors have not been shown to increase sputum clearance or lung function (Pryor et al, 1981). Percussion in short bursts can be used with ACBT until independent effective treatment can be performed by the individual. Self treatments consist of postural drainage, thoracic expansion exercises, breathing control and forced expiratory technique. We consider that self percussion has provided no additional benefit when breathing exercises are performed effectively. Self treatment is initially supervised and this continues until the patient, carer and physiotherapist consider the treatment is carried out effectively. At the time of respiratory exacerbations, assisted treatments are usually preferable.
Positive expiratory pressure ("PEP") masks: (Astra Meditec Ltd, PO Box 13, Stroud, Gloucester, GL5 3DL). These masks are used to open up and recruit obstructed lung, allowing air to move behind secretions and assist in mobilising them. Breathing out against a slight resistance (10 to 20 cms of water) prevents the smaller bronchial tubes from collapsing down and thus permits the continuing upward movement of any secretions (Tyrell et al, 1986; Falk & Anderson, 1991). The technique also allows the patient more independence. In assessing patients suitable for a PEP mask, one of the 8 available resistors (diameters from 1.5 to 5mm) is selected to produce an expiratory pressure of 10 to 20 cms of water during the middle phase of expiration. The resistor is checked regularly to ensure the desired pressure is maintained. The treatment can be performed in the sitting or postural drainage position. The technique involves ten breaths, with slightly active expiration, through the mask followed by a period of huffing, coughing and breathing control. This cycle is repeated at least 3 times in each postural drainage (PD) position or until maximum clearance of secretions is achieved. If performed in the sitting position, the cycle is repeated for approximately 10 minutes. Most benefit is obtained by patients who produce sputum and have obstructed airways, where premature airway closure during expiration contributes to retained secretions. PEP permits greater patient independence and is useful when conventional techniques may be difficult, e.g., after operation for insertion of a Port-A-Cath device. The technique is usually contraindicated in patients with a previous pneumothorax or bullae evident on the chest X-ray.
PEP with aerosol delivery: With the application of PEP airways are kept open during exhalation promoting airflow to previously obstructed airways and a resultant improvement of mucus clearance from these areas. If used in conjunction with bronchodilators, the PEP will enhance the deposition of the aerosol.
The flutter device: (Scandipharm International, Powys, UK). The Flutter VRPI is a small plastic device which contains a large ball bearing which repeatedly interrupts the outward flow of air. This generates a controlled oscillating positive pressure which mobilises respiratory secretions. A Flutter session consists of about 10-15 breaths followed by huffing and breathing control. This is repeated for 15 or 20 minutes depending on the individual need. Studies have been conducted to assess the effect of the Flutter VRP1 with both positive (Homnick et al, 1998; Konstan et al, 1994) and negative (Pryor et al, 1994) results. The Flutter can be used as an adjunct to other forms of physiotherapy or as a treatment in its own right (McIlwaine et al, 1997). Gondor found that the Flutter resulted in greater improvement in pulmonary function after one week in hospitalised patients when compared to conventional chest physiotherapy, although there was no difference detectable after two weeks treatment (Gondor et al, 1999).
Autogenic drainage: This term describes a series of breathing exercises devised by the Belgian physiotherapist Jean Chevaillier. The aim is to dislodge and collect mucus from the lungs and then clear these secretions by breathing at various lung volumes (Chevaillier, 1984; Schoni, 1989).There are three phases - the Unstick, Collect and Evacuate when breathing at low, mid and high lung volumes to mobilise, collect and expectorate secretions respectively. It should be taught by a person skilled in its use and trained in the procedure. A modification of the original technique is now used in Germany (David, 1991) and a recent trial has provided evidence that the technique is of some benefit (Miller et al, 1995). We have found this a very difficult technique to use in young children, but for the young adolescent who can co-operate fully and demonstrate effective breathing control this technique has been found to combat some associated problems of wheeze and airway collapsibility and has been well received.
Physical activity: Regular vigorous exercise is beneficial for the CF patient and should be encouraged. Swimming, cycling, skipping, tennis, squash and jogging are all excellent forms of exercise. It has been suggested that chest physiotherapy can be replaced by aerobic exercise in selected patients (Andreasson et al, 1987) though there are few data showing the beneficial effect of exercise on sputum clearance. A study from Manchester showed that exercise provided definite cardiovascular and respiratory benefits and might aid sputum expectoration but the ACBTs was more effective in this respect than exercise (Salh et al, 1989). Zach reported an increase in sputum clearance when twice weekly swimming was added to the normal chest physiotherapy regimen (Zach et al, 1981). Thus, most studies show exercise cannot replace physiotherapy but should be considered as complementary to it (Webb et al, 1995).
Equipment: Effective postural drainage equipment is provided by the CF unit for home use. Foam wedges or vinyl sag bags are used between the age of 18 months and 5 years. As the child gets older, many are supplied with a specially designed foldable bed frame, e.g., the Chesham frame (Chesham Engineering Co Ltd, 217 Berkhampton Rd, Chesham, Bucks HP5 3AP) and the Becky bed. Such apparatus is either provided by health authorities or from the Regional CF Unit's funds from voluntary contributions - for which we are most grateful.
Paediatrics: (special points)It is important that all patients are seen by an experienced physiotherapist with specialist knowledge in the treatment of cystic fibrosis as soon as the diagnosis is confirmed. If routine care is carried out at a local hospital it is advisable to have additional advice from the specialised team at a Regional Cystic Fibrosis Unit. Initially the infant has a passive role in his/her chest physiotherapy, which consists of postural drainage, chest percussion and vibratory shakings (Prasad, 1993). All postural drainage positions are normally taught within the first 6 months following diagnosis.Active participation is encouraged and initiated at appropriate ages.Blowing is the first active participation in treatment and is introduced between eighteen months and 3 years. The treatment cycle would consist of short bursts of percussion approximately 30 seconds to a minute in length and blowing with vibrations or shakes on expiration repeated approximately 4 times. This cycle is repeated 3 or 4 times in each position. The forced expiratory technique (FET) and coughing are introduced between the ages of 3 and 5 years. The cycle of treatment includes percussion, vibration and breathing control alternating with one or two huffs (FET). Between 5 and 7 years of age, thoracic expansion exercises are incorporated to replace the blowing, 4 to 6 thoracic expansion exercises replace 4 blows. This is a representative and ideal treatment programme which is regularly re-assessed and modified to suit the individual patient. A development from this programme is towards self treatment. The implementation of this will vary, with some children being proficient in self treatments from the age of 11 years where others prefer assisted treatments for longer. Adults (special points) It is important that adults with CF are educated and become responsible for their own chest care. They must recognise the signs of increased infection and contact the hospital at these times for assessment and treatment with intravenous antibiotics if necessary. Those who have not attended a specialised CF unit previously often require considerable education, explanation of the importance of doing their chest physiotherapy treatment at home, and encouragement with this programme on their own now that they may no longer have parental supervision. These patients require regular input, support and monitoring from a physiotherapist experienced in dealing with and treating adults who have cystic fibrosis. Most CF adults produce sputum, and a daily session of postural drainage combined with the active cycle of breathing techniques (Webber, 1990), modified if required to suit each patient's needs, is encouraged. Treatment is increased to twice daily and more for patients producing excess sputum and during exacerbations of their chest infection. Exercise is actively encouraged for all patients, both for outpatients and whilst in hospital, adjusting the exercise programme to each individual's requirement.
Conclusions: With modern treatment many young CF patients have no regular cough and produce no sputum for many years. However, there is evidence that even in patients with mild chest involvement, regular daily physiotherapy maintains the chest in better condition than when this treatment is omitted (Reisman et al, 1988). For patients with few or no chest symptoms Leeds recommends one daily session of FET or postural drainage. If the patient has a productive cough, physiotherapy should be performed at least twice daily and more frequently with colds. Regular monitoring of the patient’s and family’s technique by a physiotherapist who is expert in the various techniques used in the treatment of cystic fibrosis is essential (Morton et al, 1988; Worthington & Kelman, 1996).
References
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