Physiotherapy

PHYSIOTHERAPY

The Association of Chartered Physiotherapists in Cystic Fibrosis will publish their "Standards of Care and Good Clinical Practice for Physiotherapy Management of Children and Adults with Cystic Fibrosis" edited by Penney Argent, Lisa Morrison and Ammani Prasad in 2011; it is an excellent review of the subject.

1959 Doyle B. Physical therapy in treatment of cystic fibrosis. Phys Therapy Rev 1959; 39:24 - 27. (Prepared under the direction of Harry Shwachman). [PubMed]
An early report of physical therapy for the chest in cystic fibrosis. Shwachman mentions that active physical therapy was instituted in his clinic in 1957 and considers it to be one of the more important introductions into the treatment regimen. Barbara Doyle (figure 24) was a physical therapist from Eire who received her physical therapy preparation at Dublin School of Physiotherapy and was a member of the Chartered Physiotherapy Society of Great Britain. From 1956-1958 she was an exchange visitor in the United States and worked at the Children’s Medical Center, Boston. She used the so-called “English” methods (Reid JMW in Physiotherapy for Chest Diseases. In Marshall G, Perry KMA. (Eds.) Diseases of the Chest. St Louis: Butterworth & Company. 1952; 2:395-413). This report describes the current physiotherapy treatment for people with CF as done in Shwachman’s clinic in Boston and based on the experience with various chest conditions at the Hospital for Sick Children, Great Ormond Street, London.

Figure 24: Barbara Doyle. From the paper.

Lannefors L et al. (J R Soc Med 2004; 97 (Suppl 44):8-25) in an excellent review of CF physiotherapy note the first reference to the use of postural drainage was by Ewart in 1901 (Ewart W. Lancet 1901; ii: 70) who referred to it as “empty bronchus treatment by posture in bronchiectasis of children”. Although few children with CF would have survived infancy at that time, bronchiectasis as an after effect of pneumococcal pneumonia, whooping cough or measles was relatively common in the pre-antibiotic era. Ewart advocated continuous drainage for hours at a time with the patient sleeping in the various positions. Later the detailed anatomy of the various lobes was described (Nelson HP. BMJ 1934; 11:251-255) and in 1949 the nomenclature of the anatomy was agreed (Anonymous. Nomenclature of broncho-pulmonary anatomy; an international nomenclature accepted by the Thoracic Society. Thorax 1950; 5: 222-228).

1962 Denton R. Bronchial secretions in cystic fibrosis. The effects of treatment with mechanical percussion vibration. Am Rev Respir Dis 1962; 86:41-6. [PubMed]
One of many publications by Dr Robert Denton from Philadelphia. As the effect of chest clapping and vibrations was considered to be helpful in moving bronchial secretions, the effect of rapid repetitive percussion with a mechanical device was examined in 23 patients with CF who had moderate and marked pulmonary involvement.
The study with a mechanical percussor (figure 10) is complex and impressive, even involving a “control percussor” that did not vibrate. Over a 12 minute period, there was a significant increase in secretions produced with the percussor + vibrations compared to percussor with simulated vibrations and unassisted postural drainage; this was significant in 19 of the 23 patients although the vital capacity did not change significantly.

Figure 10: Percussor in use. From the paper with permission.

This was the first of a number of papers on mechanical percussion as an aid to chest physiotherapy. Some years later in the UK the “Salford percussor” (Maxwell & Redmond, 1979 below; Flowers et al, 1979 below) received a cool reception from the UK physiotherapists. However, the delivery of vibrations by a jacket or vest eventually became part of standard approved therapy in the USA thanks to the work of Warren Warwick (Warwick & Hansen, 1991 below). Again this more recent mechanical aid, “the Vest”, was slow to find favour with physiotherapists in the UK!

1979 Pryor JA, Webber BA, Hodson ME, Batten JC. Evaluation of the use of forced expiration technique as an adjunct to postural drainage in treatment of cystic fibrosis. BMJ 1979; 2:417-418. [PubMed]
The classic paper on forced expiratory technique (FET) from the Brompton Hospital, London comparing conventional physiotherapy with FET without the involvement of an assistant. The latter was both more efficient (producing 45.6 g vs. 63.1 g sputum per day) and quicker (358.5 vs. 652.1 mg sputum per minute) than conventional physiotherapy and was not improved by involving an assistant. The authors concluded - “Patients with cystic fibrosis who had to rely on the help of others for their home treatment may now perform more effective treatment without help”.

The value of chest physiotherapy in clearing bronchial secretions in conjunction with postural drainage in patients producing more than 30ml of sputum per day was established first by Cochrane GM et al, (BMJ 1977; 2:1181-1183) who studied the specific airways conductance (SGAW) of 23 patients with copious sputum production and airflow obstruction before and after physiotherapy to determine the effect of bronchial secretions on pulmonary function. Chest physiotherapy to remove these secretions had the effect of reducing airflow obstruction, as measured by SGAW. These findings were taken to suggest that sputum has a detrimental effect on pulmonary function and that physiotherapy can reduce airways obstruction. Physiotherapy for children with CF had been introduced into Shwachman’s clinic in 1957 by Barbara Doyle (Doyle et al, 1959 above). This forced expiratory technique represented a significant advance allowing the patient more independence and less reliance on others.

1979 Flower KA, Eden RI, Lomax L, Mann NM, Burgess J. New mechanical aid to physiotherapy in cystic fibrosis. BMJ 1979; 2:630-631. [PubMed]
The authors analysed the effects of the percussion provided by professional physiotherapists for the first time and reproduced in the small portable “Salford Percussor”. Although initially popular with a few parents there was no subsequent work on the device and it was not accepted by UK physiotherapists who, as a group generally, have not been enthusiastic about mechanical aids to physiotherapy – as evidenced by their more recent lack of enthusiasm for the mechanical vest!!! (also Denton, 1962 above; Maxwell & Redmond, 1979 below)

1979 Maxwell M, Redmond A. Comparative trial of manual and mechanical percussion technique with gravity-assisted bronchial drainage in patients with cystic fibrosis. Arch Dis Child 1979; 54:542-544. [PubMed]
Here 14 patients with CF were studied by measuring sputum volumes and respiratory function. The results with the mechanical percussor (figure 22) were comparable with those with manual percussion. All the patients and parents preferred the mechanical percussor.
However, there is no further mention of the mechanical percussor by either the Manchester group (Flower et al, 1979 above) or Belfast group (Maxwell & Redmond) and they did not gain the support of he majority of UK physiotherapists who seem, as a group, to be unimpressed by mechanical aids for physiotherapy. Mechanical methods had been reported previously in an excellent trial by Robert Denton (Am Rev Respir Dis 1962; 86:41-46 above).

Aileen Redmond (figure 23) was paediatrician at the Belfast paediatric CF unit and had worked with Harry Shwachman in Boston – one of the first centres to use physiotherapy. She published on a wide variety of subjects including CF and was one of the few UK experts in CF at the time and one of the first to introduce neonatal IRT CF screening in Northern Ireland in the early Eighties. Her CF unit at the Belfast Children’s Hospital was highly regarded and she eventually achieved a purpose built CF unit – Cherry Tree House in Belfast.

1983 Desmond KJ, Schwenk WF, Thomas E, Beaudry PH, Coates AL. Immediate and long term effects of chest physiotherapy in patients with cystic fibrosis. J Pediatr 1983; 103:538-542. [PubMed]
Spirometric and plethysmographic evaluations were performed before chest physiotherapy (CPT) and at five and 30 minutes post-CPT. The pre-CPT measurements after a three-week period with no CPT were compared with the values while receiving CPT on a regular twice daily basis. Their findings showed that although there may be little immediate functional improvement when CPT is received on a regular basis, a three-week period without CPT resulted in a worsening of respiratory function which was reversed with renewal of regular chest physiotherapy.

This is one of the few studies evaluating the efficacy of chest physiotherapy. Previously Pryor et al, (1979 above) had evaluated the forced expiratory technique. Subsequently Reisman et al, (1988 below) performed a trial of physiotherapy in Toronto which also showed the benefit of routine physiotherapy. Considering the huge time commitment of the patients and their families to physiotherapy there were surprisingly few publications evaluating the efficacy of physiotherapy treatment.

1984 Falk M, Kelstrup M, Anderson JB, Kinoshita T, Falk P, Stovring S, Gothgen I. Improving the ketchup bottle method with positive expiratory pressure, PEP, in cystic fibrosis. Eur J Resp Dis 1984; 65:423-432. [PubMed]
The first report of the Positive Expiratory Pressure (PEP mask) for physiotherapy from Copenhagen that proved to be a popular and effective method of treatment. The mechanics of this method had been evaluated by one of the authors using parts of excised human lungs (Andersen J B et al. Scand J Respir Dis 1979; 60:260-266. [PubMed]. Falk et al studied the acute effects of four different chest physical therapy regimens using a randomised cross-over design in 14 patients with cystic fibrosis. PEP was well accepted by the patients, who preferred treatment with PEP, and the authors suggested PEP be incorporated in chest physical therapy regimens. (Tyrell et al, 1986 below for the first UK study of PEP from Nottingham; also Murray JF. The ketchup-bottle method. [Editorial] N Eng J Med 1979; 300:1155-1157). A trail of PEP from George Davidson's unit in Vancouver had a significant influence on physiotherapy practice in N. America (McIlwaine PM et al. J Pediatr 1997; 131:506-508.below).

1986 Tyrrell JC, Hiller EJ, Martin J. Face mask physiotherapy in cystic fibrosis. Arch Dis Child 1986; 61:598-611. [PubMed]
This paper was from Dr Joan Hiller’s Paediaitric CF Unit at Nottingham City Hospital. Dr Joan Hiller (figure 39) founded the Nottingham CF Unit having previously worked in the USA with Dr Nancy Huang in Philadelphia.
This study was coordinated by Dr Jenny Tyrell, then the CF Research Fellow in Nottingham, and evaluated the use of the 'PEP' mask with forced expiratory coughing which was compared with conventional physiotherapy over a one month period. No difference was shown in symptom scores, sputum production, or simple lung function tests. The mask was well accepted and allowed independent treatment by older patients.
The PEP mask became a popular method physiotherapy giving some degree of independence to the patient (also Falk et al, 1984 above and many subsequent papers supporting the use of the PEP).

1988 Reisman JJ, Rivington-Law B, Corey M, Marcotte J, Wannamaker E, Levison H J. Role of conventional physiotherapy in cystic fibrosis. Pediatr 1988; 113:632-636. [PubMed]
A frequently quoted paper from Toronto of a 3-year prospective study to compare the long-term effects of postural drainage accompanied by percussion and the forced expiratory technique with the effects of the forced expiratory technique alone. Patients who performed the forced expiratory technique alone had mean annual rates of decline that were significantly different from zero for forced expiratory volume in 1 second (p <0.001), forced expiratory flow between 25% and 75% of vital capacity (p <0.001), and Shwachman clinical score (p< 0.004). In the group performing conventional physiotherapy with percussion and postural drainage, only the mean annual rate of decline for forced expiratory flow between 25% and 75% of vital capacity was significantly different from zero (p<0.03), and it was significantly different from the mean rate of decline associated with the forced expiratory technique alone (p<0.04). The authors concluded that conventional chest physiotherapy should remain a standard component of therapy in cystic fibrosis
Physiotherapy is a major and time-consuming component of therapy and the worst part of treatment for many patients and parents. This frequently quoted study was one of the few to demonstrate that the time was well spent and so the paper was useful and was quoted widely for this reason (also Pryor et al, 1979 above; also Desmond et al, 1983; Tyrell et al, 1986 below).

Figure 39: Dr Joan Hiller. Author's photo

1991 Warwick WJ, Hansen LG. The long-term effect of high frequency chest compression therapy on pulmonary complications of cystic fibrosis. Pediatr Pulmonol 1991; 11:265-271. [PubMed]
A high-frequency chest compression (HFCC) device for clearance of mucous secretions from airways was tested in 16 patients with CF with significant improvement in pulmonary function for the HFCC period, which averaged 22 months per patient. The device consists of a variable air pulse delivery system and a non-stretch inflatable vest worn by the patient to cover the entire torso. Ninety-four percent of patients' regression line slopes for percent predicted forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) became more positive during self-administered HFCC therapy as compared to slopes before HFCC therapy, when manual chest physical therapy was used.
Although this device (figure 20) has never found favour amongst physical therapist in the UK, over the next 15 years “the vest” became popular and widely used in North America by some 60% of people with CF and by many others with chronic respiratory disorders. The device has been pioneered by veteran CF centre director Warren Warwick (figure 21) from Minnesota and has been the subject of many subsequent publications showing comparative efficiency with other forms of physiotherapy and improvements with alterations in the wave form created. The first reports on high frequency chest wall compression were from King M et al.(Am Rev Respir Dis 1983; 128:511-515.[PubMed]; Am Rev Respir Dis 1984; 130:703-706.[PubMed]).

1994 Arens R, Gozal D, Omlin KJ, Vega J, Boyd KP, Keens TG, Woo MS. Comparison of high frequency chest compression and conventional physiotherapy in hospitalized patients with cystic fibrosis. Am J Res Crit Care 1994; 150:1154-1157. [PubMed]
A study of 50 people with CF admitted for acute pulmonary exacerbations that were randomly allocated to receive either high frequency chest compression (HFCC) or conventional physiotherapy (CPT) three times a day. After seven and 14 days of treatment, improvements were similar in the two study groups, leading to patient discharge after similar periods of hospitalization. The authors concluded that HFCC and CPT are equally safe and effective when used during acute pulmonary exacerbations in CF patients. They suggested that HFCC may provide an adequate alternative in management of CF patients in a hospital setting. (Also Warwick WJ, Hansen LG, 1991 above)

1997 Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky A. Postural drainage and gastro-oesophageal reflux in infants with cystic fibrosis. Arch Dis Child 1997; 76:148-150. [PubMed]
First of series of papers from Brenda Button (figure 43), a physiotherapist from Melbourne, Australia, noting the possible dangers of inhalation when infants with CF were in the head down position during postural drainage; infants with CF were known to have an increased incidence of gastro-oesophageal (GO) reflux. Physiotherapy with and without head down tilt were compared using 24 hour pH oesophageal monitoring. Standard physiotherapy with head down tilt was associated with a significant increase in GO reflux in the infants with CF.
This was an important study and, with Brenda Button’s subsequent publications, certainly had a major influence on the techniques of physiotherapy recommended for CF infants (see also Malfroot & Dab, 1991 above for earlier studies on reflux in CF infants; Button et al, 2004 also confirmed GO reflux as common and important in adults with CF)

1997 McIlwaine PM, Wong LT, Peacock D, Davidson AG. Long-term comparative trial of conventional postural drainage and percussion versus positive expiratory pressure physiotherapy in the treatment of cystic fibrosis. J Pediatr 1997; 131:506-508. [PubMed]
Forty patients were randomised to receive either postural drainage and percussion or PEP mask physiotherapy over one year. Those using the PEP mask had significantly better respiratory function after 1 year and the authors concluded that this was the more effective method of physiotherapy.
This study from Professor George Davidson’s CF centre in Vancouver, Canada had a significant influence on physiotherapy practice in North America. A subsequent study from this unit, comparing PEP and Flutter methods, showed PEP to be superior in maintaining pulmonary function and reducing the need for hospital admissions (McIlwaine et al. J Pediatr 2001; 138:845-850).
The study was influential in changing the routine physical therapy practice in North America.

2000 Schneiderman-Walker J, Pollock SL, Corey M, Wilkes DD, Canny GJ, Pedder L, Reisman JJ. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J Pediatr 2000; 136:304-310. [PubMed]).
To evaluate the effects of a 3-year home exercise program on pulmonary function and exercise tolerance in mildly to moderately impaired patients with CF and to assess whether regular aerobic exercise is a realistic treatment option. An improved sense of well-being was reported with exercise and pulmonary function declined more slowly in the exercise group than in the control group, suggesting a benefit for patients with CF participating in regular aerobic exercise (percentage predicted decline in FVC 2.42 vs. 0.25 and FEV1 3.47 vs. 1.46).

This is one of a number of studies that confirmed exercise was good for people with CF. It was always apparent following patients in the clinic that those people with CF who were very active seemed to remain in better condition which is really not surprising.

2003 Button BM. Heine RG. Catto-Smith AG. Olinsky A. Phelan PD. Ditchfield MR. Story I. Chest physiotherapy in infants with cystic fibrosis: to tip or not? A five-year study. Pediatr Pulmonol 2003; 35:208-213. [PubMed]
There is controversy about the need for postural drainage physiotherapy in asymptomatic infants with cystic fibrosis (CF). In this study Brenda Button and her colleagues compared the effectiveness of standard postural drainage chest physiotherapy (SPT) with a modified physiotherapy regimen without head-down tilt (MPT) in 20 young infants with CF. Patients receiving SPT had more days with upper respiratory tract symptoms than those on MPT (70 +/- 32.8 vs. 37 +/- 24.9 days; P = 0.04) and required longer courses of antibiotics (23 +/- 28.5 vs. 14 +/- 11.2 days; P = 0.05). Chest x-ray scores were similar at diagnosis but were worse at 2(1/2) years for those receiving SPT (P = 0.03). Forced vital capacity and forced expired volume in 1 sec (FEV(1)) at 5-6 years was lower for SPT than for MPT (P < 0.05). In conclusion, MPT was associated with fewer respiratory complications than SPT in infants with CF.

Brenda Button's work has been largely influential in most physiotherapists omitting the head down position in the physiotherapy recommendations fro infants with cystic fibrosis.

2004 Warwick WJ. Wielinski CL. Hansen LG. Comparison of expectorated sputum after manual chest physical therapy and high-frequency chest compression. Biomed Instru Tech 2004; 38:470-475. [PubMed]
These results show that sputum production by subjects with CF who receive CPT by certified respiratory therapists can be as great as the sputum produced by the same subjects who receive HFCC. The results also suggest that unknown factors attributed to the therapists may produce different levels of effort from time to time that may decrease the respiratory therapists' effectiveness, whereas the HFCC therapy may be more consistently effective because it is entirely machine based.

A helpful practical paper from Warren Warwick on the use of the vest compared with manual physiotherapy.

2004 Phillips GE. Pike SE. Jaffé A. Bush A. Comparison of active cycle of breathing and high-frequency oscillation jacket in children with cystic fibrosis. Pediatr Pulmonol 2004; 37:71-75.[PubMed]
Comparison of the active cycle of breathing techniques (ACBT) with the Hayek Oscillator Cuirass, performing HFCC on secretion clearance in children with CF during an exacerbation. Ten children (7 males; median age, 14 years; range, 9-16) received either two supervised sessions using HFCC or two self-treatment ACBT sessions in random order on successive days. Baseline pulmonary function was similar prior to treatments. Sputum weight increased significantly with ACBT compared with HFCC during treatment (5.2 g vs. 1.1 g, P < 0.005, morning; 4.1 g vs. 0.7 g, P < 0.01, afternoon). Pulmonary function improved significantly after morning ACBT (forced vital capacity (FVC): 2.67 l to 2.76 l, P < 0.03; forced expiratory volume in 1 sec (FEV1): 1.59 l to 1.62 l, P < 0.03). Following afternoon ACBT, there was a significant increase in FVC (2.64 to 2.79, P < 0.02), but no significant change in FEV1. Pulmonary function did not change at any time following HFCC. Compared with ACBT, HFCC by Hayek Cuirass is not an effective airway clearance treatment modality for children with CF during an infective exacerbation.

These are small numbers and small volumes of sputum. The findings reinforce the lack of enthusiasm from UK physiotherapists for the "vest" which is used by many people with CF in the USA apparently with satisfactory results.

2005 Button BM, Roberts S, Kotsimbos TC, Levvey BJ, Williams TJ, Bailey M, Snell GI, Wilson JW. Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation. J Heart Lung Transpl 2005; 24:1522-9.
Gastroesophageal reflux (GER) may contribute to declining lung function both before and after lung transplantation. Twenty-four patients were studied – GER, both symptomatic and silent, is a frequent and significant problem in people with CF after lung transplantation. The advice was that this condition should be aggressively treated and if necessary by surgery if GER persists.

2005 Barak A, Wexler ID, Efrati O, Bentur L, Augarten A, Mussaffi H, Avital A, Rivlin J, Aviram M, Yahav Y, Kerem E. Trampoline use as physiotherapy for cystic fibrosis patients. Pediatr Pulmonol 2005; 39:70-73.
Physicians and physiotherapists who care for CF patients have recommended the use of trampolines as a methods of improving cardiopulmonary performance, encouraging sputum production, and improving general well-being. Despite some therapeutic and recreational benefits associated with trampoline use, papers in the paediatric literature mostly document an increased incidence of injuries, ranging from minor trauma to spinal cord injuries and even death! This review examined the accumulated published data regarding the use of trampolines, to assess their potential contributions and disadvantages for CF patients, and to define whether trampoline use should be recommended. There were approximately 60 articles that primarily dealt with trampolines, out of which only two dealt with CF. Unfortunately most of these articles are reports pertaining to injuries related to the use of trampolines, with only a few describing the medical, physiologic, and/or psychological benefits of trampolines.
Based on the accumulated data, the presumed benefits of trampoline use for CF patients are not proven. Furthermore, the suggested benefits could be acquired using other types of exercise. Weighing the known definite risks of trampolines against the potential benefits that are not unique to this modality suggests that the use of trampolines for CF should not be recommended. In fact the main outcome of trampoline use appeared to be injury!!

2005 Dodd ME. Prasad SA. Physiotherapy management of cystic fibrosis. Chron Respir Dis 2005; 2:139-149.
This is a review by recognised UK experts on the subject. They note that “for many years physiotherapy for cystic fibrosis (CF) was synonymous with a daily routine of postural drainage and percussion. Improved longevity and a desire for independence, together with a clearer understanding of the pathophysiology of the disease, has led to the development of many airway clearance techniques. The contribution of 'chest' physiotherapy remained unchallenged until recent times when lack of robust evidence and the presentation of asymptomatic infants and adults led some physiotherapists to question this traditional approach. As survival increases into the fourth and fifth decade unique complications have evolved and physiotherapy management is challenged with non-respiratory aspects of treatment. This review considers the evidence for, and current practice of airway clearance, the value of exercise and the emerging problems of musculoskeletal complications and urinary incontinence”.
The first author Mary Dodd, (figure 31) is a consultant physiotherapist. She was the co-founder along with Prof. Kevin Webb of the Bradbury adult CF Centre in Manchester. Mary has been closely involved in CF clinical care and research for many years since starting working with CF with Dr Holzel in Manchester. She has published on most aspects of physiotherapy for people with CF and is a leading authority on the subject both in the UK and internationally

2006 Balfour-Lynn IM, Carr SB, Madge SL. A comparison of prevalence of urinary incontinence in girls. Pediatr Pulmonol 2006; 41:1065-1068.
Another study on urinary incontinence - this time on younger patients. The physiotherapists have taken an increasing interest in bladder dysfunction in cystic fibrosis. Girls with CF aged 11 to 17 years were studied and urinary incontinence was present in 17 (33%) of 51 girls, compared with only 4 (16%) of 25 of those with asthma and 2 (7%) of 27 healthy controls. The problem was associated with increasing severity of lung disease (also in adults with CF Cornacchia et al, 2001 above; Orr A et al. BMJ; 322:1521).

2007 Kempainen RR, Williams CB, Hazelwood A, Rubin BK, Milla CE. Comparison of high-frequency chest wall oscillation with differing waveforms for airway clearance in cystic fibrosis. Chest 2007; 132:1227-1232.
Using the vest device single-session sputum production is comparable with sine and triangular waveform high-frequency chest wall oscillation devices.
There is a steady output of work on the Vest physiotherapy device and although some seems to be related to companies competing for sales, it does seem to be a major advance particularly in convenience for all concerned - particularly the patients. The use of the Vest is one of a number of major differences between treatment in the UK and USA where over 50% of people with CF use the Vest but virtually no patients in the UK use it. Two obvious reasons are the cost of the equipment (£10K in the UK) and the undoubted lack of enthusiasm of UK CF physiotherapists for the technique!

2004 Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky. Chest physiotherapy, gastro-oesophageal reflux and arousal in infants with cystic fibrosis. Arch Dis Child 2004; 89:435-439. [PubMed]
This is similar to Brenda Button’s 1997 study (above) comparing standard physiotherapy (SPT) and modified physiotherapy without the head down position (MPT). The Melbourne team monitored the cardio-respiratory state during the procedures. It was confirmed that more episodes of gastro-oesophageal reflux occurred when the head down position was used; the left lateral position was associated with fewer episodes than the others. There were more episodes in supine and prone in the SPT head down and the infants were more likely to cry. Oxygen saturation was lower in SPT but improved with non-nutritive sucking (i.e. a dummy). The authors concluded that the SPT is associated with gastroesophageal reflux, distressed behaviour and lower oxygen saturation.

These studies certainly influenced the physiotherapy recommendations for CF infants particularly as screened infants often have very little in the way of respiratory secretions to remove, although their respiratory function tests are usually significantly below normal (Ranganathan et al, 2001 above). Not all physiotherapists and paediatricians initially accepted that reflux was increased in the head down position. For example the Brompton CF team in London refuted the need to change physiotherapy routines in infants with CF (Phillips GE et al. Holding the baby: head downwards positioning for physiotherapy does not cause gastro-oesophageal reflux. Eur Resp J 1998; 12:954-957. 9817174).

2005 Button BM, Roberts S, Kotsimbos TC, Levvey BJ, Williams TJ, Bailey M, Snell GI, Wilson JW. Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation. J Heart Lung Transpl 2005; 24:1522-9. [PubMed]
Gastroesophageal reflux (GER) may contribute to declining lung function before and after lung transplantation. Twenty-four patients were studied. GER, both symptomatic and silent, is a frequent and significant problem in people with CF after lung transplantation. The advice was that this condition should be aggressively treated and if necessary by surgery if GER persists.

2005 Dodd ME. Prasad SA. Physiotherapy management of cystic fibrosis. Chron Respir Dis 2005; 2:139-149. [PubMed]
This is a review by Mary Dodd one of the recognised experts on the subject. She notes that “for many years physiotherapy for cystic fibrosis (CF) was synonymous with a daily routine of postural drainage and percussion. Improved longevity and a desire for independence, together with a clearer understanding of the pathophysiology of the disease, has led to the development of many airway clearance techniques. The contribution of 'chest' physiotherapy remained unchallenged until recent times when lack of robust evidence and the presentation of asymptomatic infants and adults led some physiotherapists to question this traditional approach. As survival increases into the fourth and fifth decade unique complications have evolved and physiotherapy management is challenged with non-respiratory aspects of treatment. This review considers the evidence for, and current practice of airway clearance, the value of exercise and the emerging problems of musculoskeletal complications and urinary incontinence.


Fig. 38: Dr Mary Dodd CBE

This is a detailed review by Mary Dodd (figure 38), consultant physiotherapist in the Manchester Adult CF centre. She was the co-founder along with Prof. Kevin Webb of the Bradbury Adult CF Centre in Manchester. Mary has been closely involved in CF clinical care and research for many years since starting working with CF with the late Dr Holzel in Manchester. She has published on most aspects of physiotherapy for people with CF and is a leading authority on the subject both in the UK and internationally.

2005 Barak A, Wexler ID, Efrati O, Bentur L, Augarten A, Mussaffi H, Avital A, Rivlin J, Aviram M, Yahav Y, Kerem E. Trampoline use as physiotherapy for cystic fibrosis patients. Pediatr Pulmonol 2005; 39:70-73. [PubMed]
Physicians and physiotherapists who care for CF patients have recommended the use of trampolines as a methods of improving cardiopulmonary performance, encouraging sputum production, and improving general well-being. Despite some therapeutic and recreational benefits associated with trampoline use, papers in the paediatric literature mostly document an increased incidence of injuries, ranging from minor trauma to spinal cord injuries and even death!
This review examined the accumulated published data regarding the use of trampolines, to assess their potential contributions and disadvantages for CF patients, and to define whether trampoline use should be recommended. There were approximately 60 articles that primarily dealt with trampolines, out of which only two dealt with CF. Unfortunately most of these articles are reports pertaining to injuries related to the use of trampolines, with only a few describing the medical, physiologic, and/or psychological benefits of trampolines.

Based on the accumulated data, the presumed benefits of trampoline use for CF patients are not proven. Furthermore, the suggested benefits could be acquired using other types of exercise. Weighing the known definite risks of trampolines against the potential benefits that are not unique to this modality suggests that the use of trampolines for CF should not be recommended. In fact the main outcome of trampoline use appeared to be injury!! Subsequently support came from Ireland where daily trampoline use in half the patients had resulted in no injuries requiring medical treatment (Currant & Mahony, 2008). [PubMed]

2006 Prasad SA, Balfour-Lynn IM, Carr SB, Madge SL. A comparison of prevalence of urinary incontinence in girls with cystic fibrosis, asthma and healthy controls. Pediatr Pulmonol 2006; 41:1065-1068. [PubMed]
Another study on urinary incontinence - this time on younger patients. In recent years the physiotherapists have taken an increasing interest in bladder dysfunction in CF. Girls with CF aged 11 to 17 years were studied and urinary incontinence was reported by 17/51 (33%) girls, compared with only 4/25 (16%) of those with asthma and 2/27 (7%) healthy controls. The problem was associated with increasing severity of lung disease. (also described in adults with CF by Cornacchia et al, 2001 above; Orr A et al. BMJ; 322:1521).


Fig. 39: Armmani Prasad


Fig. 40: Dr Peter Bye


Fig. 41: Mark Elkins

Armmani Prasad (figure 39) is the senior Physiotherapist at the CF Unit Great Ormond Street Hospital for Children, London and one the leading CF physiotherapists in the UK and internationally. She has written extensively on CF and also been an invited speaker at many conferences both in the UK and abroad and regularly advises the CF Trust on matters relating to CF care.

There is a steady output of work on the Vest physiotherapy device and although some seems to be related to companies competing for sales, it does seem to be a major advance particularly in convenience for all concerned. The use of the Vest is one of a number of major differences between treatment in the UK and USA where over 50% of people with CF use the Vest in contrast to the very few in the UK. Two obvious reasons are the cost of the equipment (£10K in the UK) and the undoubted lack of enthusiasm of UK CF physiotherapists for the technique!

2008 Prasad SA, Main E, Dodd ME; Association of Chartered Physiotherapists. Finding consensus on the physiotherapy management of asymptomatic infants with cystic fibrosis. Pediatr Pulmonol 2008; 43:236-244. [PubMed]
This study aimed to provide expert consensus regarding the physiotherapy management of asymptomatic infants with CF using a Delphi consensus method. Twenty-five senior paediatric physiotherapists from Specialist CF Centres throughout the UK participated in the study. Consensus was high but consensus could not be achieved on whether routine daily chest physiotherapy is necessary in 'asymptomatic' babies. An agreed amendment to the original statement allows professionals to modify or change traditional practice with the sanction of their senior colleagues. There had been a considerable amount of discussion as to the practice of tipping infants which Brenda Button and colleagues from Melbourne found caused oesophageal reflux in a significant proportion.

2009 Flume PA, Robinson KA, O'Sullivan B, Finder JD, Vender RL, Willey-Courand DB, White TB, Marshall BC. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Clinical Practice Guidelines for Pulmonary Therapies Committee. Respiratory Care 2009; 54:522-537.[PubMed]
A CF Foundation committee found no evidence that one method of airway clearance was superior to the others and although the evidence for benefit was not strong, recommended daily airway clearance be performed and regular exercise taken by all patients.

A number of studies have failed to show one method of airway clearance better than the others although physiotherapy is better than no physiotherapy.

2009 Heijerman H. Westerman E. Conway S. Touw D. Döring G. consensus working group. Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: A European consensus. J Cyst Fibros 2009; 8:295-315. [PubMed]
In this European consensus document we review the current status of inhaled medication in CF, including the mechanisms of action of the various drugs, their modes of administration and indications, their effects on lung function, exacerbation rates, survival and quality of life, as well as side effects.

A detailed consensus document with no less than 249 references.

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