The History of Cystic Fibrosis by Dr James Littlewood OBE

Edited and produced by Daniel Peckham

 

In 2011 the UK Cystic Fibrosis Trust published the Association of Chartered Physiotherapists in Cystic Fibrosis (ACPCF) "Standards of Care and Good Clinical Practice for Physiotherapy Management Cystic Fibrosis" edited by Penney Argent, Lisa Morrison and Ammani Prasad; it is an excellent review of the subject and available on the CF Trust website (www.cysticfibrosis.org.uk) or the ACPCF website (www.csp.org.uk/professional-networks/acpcf).

 

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 ) 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 : 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 ) 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 : 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 main text) 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) 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).

 

 

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 main text) 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 main text) 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 main text), 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).

 

 

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 many physiotherapists omitting the head down position in the physiotherapy recommendations for 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.

 

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 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]

 

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 (figure) and Armmani Prasad (figure) recognised experts on the subject. They 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. Dr Mary Dodd CBE

Mary Dodd (figure) is 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.

 

 

 

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).

Armmani Prasad (figure 2006 main text) 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. She is now also manager of the Great Ormond Street CF centre.

 

 

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. [PubMed]
Using the vest device, single-session sputum production is comparable with the sine and the triangular waveform high-frequency chest wall oscillation devices.

There is a steady output of work on the various physiotherapy vest devices 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 (approximately £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 the authors 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.

 

2010 Mislaying M, Wong LT, Chillers M, Davidson GF. Long-term comparative trial of two different physiotherapy techniques; postural drainage with percussion and autogenic drainage, in the treatment of cystic fibrosis. Pediatr Pulmonol 2010; 45:1064-1069. [PubMed]
In N. America, over the past decade, various airway clearance techniques (ACT) have been introduced for the treatment of cystic fibrosis (CF). These Canadian authors hypothesized that autogenic drainage (AD), an ACT developed in Belgium, would be as effective as postural drainage with percussion (PD) in treating patients with CF. They were similar similar but the patients preferred the autogenic drainage as 10 of 17 receiving autogenic drainage refused to change to postural drainage after a year.

 

2010 Osman LP, Roughton M, Hodson ME, Pryor JA. Short-term comparative study of high frequency chest wall oscillation and European airway clearance techniques in patients with cystic fibrosis. Thorax 2010; 65:196-200. [PubMed]
High frequency chest wall oscillation (HFCWO) is standard treatment for airway clearance in the USA and has recently been introduced in the UK and Europe. There is little published research comparing HFCWO with airway clearance techniques (ACTs) frequently used in the UK and Europe.

The aim of this study was to compare the short-term effects of HFCWO with usual ACTs in patients with cystic fibrosis hospitalised with an infective pulmonary exacerbation. A 4-day randomised crossover design was used. Patients received either HFCWO on days 1 and 3 and usual ACTs on days 2 and 4 or vice versa. Wet weight of sputum, spirometry and oxygen saturation were measured. Perceived efficacy, comfort, incidence of urinary leakage and preference were assessed. Data were analysed by mixed model analysis.

RESULTS: 29 patients (72% male) of mean (SD) age 29. 4 (8. 4) years and mean (SD) forced expiratory volume in 1 s (FEV(1)) percentage predicted (FEV(1)%) 38 (16. 7) completed the study. Significantly more sputum was expectorated during a single treatment session and over a 24 h period (mean difference 4. 4 g and 6. 9 g, respectively) with usual ACTs than with HFCWO (p<0. 001). No statistically significant change in FEV(1)% or oxygen saturation was observed after either HFCWO or usual ACTs compared with baseline. 17 patients (55%) expressed a preference for their usual ACT. The authors concluded during both a finite treatment period and over 24 h, less sputum was cleared using HFCWO than usual ACT. HFCWO does not appear to cause any adverse physiological effects and may influence adherence.

This study from the Royal Brompton exemplifies the lack of enthusiasm for the HFCWO method shown by UK physiotherapists!!! The fact that that the majority of US patients use HFCWO and virtually no UK patients do is interesting. Undoubtedly the cost of the apparatus (c. £10,000) is a major deterrent - presumably a cost shouldered by the medical insurance in the USA; also the lack of enthusiasm of the UK physiotherapists is another significant factor. Certainly individual UK patients who have used "the vest" at home have been quite enthusiastic.

 

2010 Denyer J, Prince I, Dixon E, Agent P, Pryor J, Hodson M. Evaluation of the Target Inhalation Mode (TIM) breathing maneuver in simulated nebulizer therapy in patients with cystic fibrosis. J Aero Med Pulmon Drug Deliver 2010; 23 Suppl 1:S29-36. [PubMed]
Adaptive Aerosol Delivery (AAD) systems provide efficient drug delivery and improved lung deposition over conventional nebulizers by combining real-time analyses of patient breathing patterns and precisely timed aerosol delivery. Delivery and deposition are further enhanced by breathing techniques involving slow, deep inhalations.

This exploratory study assessed the acceptability of slow, deep inhalations in 20 patients with cystic fibrosis (CF) during up to eight simulated nebulizer treatments with the I-neb AAD System. The breathing maneuver, Target Inhalation Mode (TIM) breathing, involved the lengthening of the patient's inhalation time over successive breaths with guidance from auditory and tactile (vibratory) feedback from the device.

At the end of the first treatment, most patients felt that the instructions were easy to understand (90%) and that the vibratory feedback was pleasant (65%). Half of the patients found the procedure to be comfortable. At the end of the final treatment, most patients felt that the breathing maneuver was easy to understand (90%) and use (80%), but that the duration of the breath was too long (100%). Logged data revealed that 90% of patients were able to comply with the breathing maneuver. The two patients unable to comply had a forced vital capacity of <1.75 L. The average treatment time decreased from 288.4 to 141.6 sec during the first and final treatments, respectively.

The authors conclude that their study provides preliminary evidence of the acceptability of the TIM breathing maneuver in patients with CF and their ability to perform repeated TIM breathing during simulated nebulizer therapy with the I-neb AAD System.

The Adaptive Aerosol is another advance in making routine inhalation therapy more acceptable to patients by increasing efficiency and reducing time spent.

 

 

2011 Association of Chartered Physiotherapists in Cystic Fibrosis (ACPCF) "Standards of Care and Good Clinical Practice for Physiotherapy Management Cystic Fibrosis" edited by Penney Argent, Lisa Morrison and Ammani Prasad

This is an excellent review of the subject and available on the CF Trust website (www.cysticfibrosis.org.uk) or the ACPCF website (www.csp.org

.uk/professional-networks/acpcf).

 

Fig. Lisa Morrison
 
Fig. Ammani Prasad
 
Fig. Penney Argent
 
Professor Allan Coates

Penney Argent (figure) is deputy Director of Rehabilitation and Therapies at the Royal Brompton and Harefield Hospitals, London and a professional lead for physiotherapy.Her directorate has about 100 staff members and includes an adult cystic fibrosis team.

 

Lisa Morrison (figure) has worked in cystic fibrosis for the past 19 years, first at the Regional CF Centre in Leeds with the last 12 as Clinical Specialist Physiotherapist in the adult CF centre at the Gartnavel Hospital Glasgow. She is particularly interested in exercise management and has presented locally and nationally on this topic. Lisa is involved both nationally and internationally with various aspects of CF physiotherapy.

 

Ammani Prasad . Following qualification Ammani specialised in paediatric respiratory care, working extensively in the fields of paediatric intensive care and paediatric respiratory medicine, particularly cystic fibrosis.

More recently Ammani has been involved in coordinating the work within the Cystic Fibrosis Unit at GOSH. She is extensively involved in joint work with the Cystic Fibrosis Trust and Department of Health regarding clinical pathways, commissioning of cystic fibrosis care and Peer Review. She now manages the Cystic Fibrosis Unit at GOSH.

 

 

2011 McCormack P, McNamara PS, Southern KW. A randomised controlled trial of breathing modes for adaptive aerosol delivery in children with cystic fibrosis. J Cyst Fibros 2011;. 10:343-349. [PubMed]
Modern nebulisers have reduced treatment times by utilising mesh technology for aerosol production. A further modification (target inhalation mode (TIM)) that may reduce treatment delivery times further. Following a baseline period on tidal breathing mode (TBM), children with CF on long-term aerosol therapy were randomly allocated to either TIM, which optimises patient inhalations through a direct feedback mechanism, or to continue TBM. The primary outcome was nebuliser treatment times with secondary outcomes being adherence and patient preference.

The ten children allocated TIM reduced their mean (SD) treatment times from 6.9(2.9) to 3.7(2.3) minutes (p<0.001). In contrast, treatment times were unchanged in the ten children allocated TBM. Mean adherence was maintained in the TIM group but declined in patients allocated TBM by >5%. All children preferred TIM to TBM.

The authors concluded that TIM reduces nebuliser treatment times and may positively impact on adherence, although longer duration studies are required to examine this.

This seems to be a definite advance in improving the efficiency and acceptability of inhalation therapy which is central to modern treatment of CF. Previous evaluation in adults with CF also provided evidence of acceptability and efficacy (Denyer J et al. J Aerosol Med Pulm Drug Deliv 201023 Suppl 1:S29-S36). [PubMed]

 

2011 Coates AL, Green M, Leung K, Chan J, Ribeiro N, Ratjen F, Charron M. A comparison of amount and speed of deposition between the PARI LC STAR nebulizer and an investigational eFlow nebulizer. J aeros med pulmon drug del 2001; 24:157- 163. [PubMed]
The potency and physical properties of many of the drugs used in the treatment of cystic fibrosis

necessitates the use of nebulization, a relatively time-consuming pulmonary delivery method. Newer, faster and more efficient delivery systems are being proposed.

The purposes of this study was to compare the length of time it took to deliver the equivalent of normal saline nebulized for 10 min in a PARI LC STAR nebulizer to that of an investigational PARI eFlow.

The investigational eFlow delivered 8.6 +/- 1.0 mg, approximately 90% of the lung dose compared to the LC STAR , 9.6 +/- 1.0 mg, but did in less than half the time (p < 0.02 for both). There were no differences in central versus peripheral distribution for either device. The authors concluded the investigational eFlow was both faster and more efficient than the LC STAR nebuliser.

One of many studies from Professor Coates (figure) and his colleagues at Toronto aimed at improving the efficiency of inhalation therapy in people with CF confirming the superiority of the eFlow nebuliser.

 

2012 O'Clock GD. Lee YW. Lee J. Warwick WJ. High-frequency and low-frequency chest compression: effects on lung water secretion, mucus transport, heart rate, and blood pressure using a trapezoidal source pressure waveform. IEEE Transactions on Biomedical Engineering 2012; 59:106-114. [PubMed]
High-frequency chest compression (HFCC), using an appropriate source (pump) waveform for frequencies at or above 3 Hz, can enhance pulmonary clearance for patients with cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD). Using a trapezoidal HFCC source pressure waveform, secretion of water from epithelial tissue and transport of mucus through lung airways can be enhanced for patients with CF and COPD. At frequencies below 3 Hz, low-frequency chest compression (LFCC) appears to have a significant impact on the cardiovascular system. For a trapezoidal source pressure waveform at frequencies close to 1 Hz, LFCC produces amplitude or intensity variations in various components of the electrocardiogram time-domain waveform, produces changes at very low frequencies associated with the electrocardiogram frequency spectra (indicating enhanced parasympathetic nervous system activity), and promotes a form of heart rate synchronization. It appears that LFCC can also provide additional cardiovascular benefits by reducing peak and average systolic and diastolic blood pressure for patients with hypertension

The latest publication from Warren Warwick and colleagues. High frequency chest compression which Warren described in the earl Nineties has been validated in a number of studies and is now widely used particularly in the USA. Now here is evidence of additional cardiovacular effects.

 

2013  Bradley JM, Blume SW, Balp MM, Honeybourne D, Elborn JS.  Quality of life and healthcare utilisation in cystic fibrosis: a multicentre study. Eur Respir J 2013; 41:571-7. [PubMed]

The aim of this study was to discover the health status and healthcare utilisation associated with pulmonary exacerbations in cystic fibrosis (CF) and chronic Pseudomonas aeruginosa infection.

Patients with CF from five UK CF centres attended two visits, 8-12 weeks apart. They were classified at visit 1 as being in one of the three health states: no current pulmonary exacerbation; "mild" (no hospitalisation) pulmonary exacerbation; and "severe" (hospitalisation) pulmonary exacerbation. All patients completed the Cystic Fibrosis Questionnaire-Revised (CFQ-R) and EuroQol (EQ-5D) and a clinical form, and forced expiratory volume in 1 s (FEV1) was measured at visits 1 and 2. Annual healthcare utilisation data were collected. 94 patients of mean±sd age 28.5±8.2 yrs and FEV1 58.7±26.8% were recruited.

60 patients had no pulmonary exacerbation, 15 had a mild and 19 had a severe pulmonary exacerbation at visit 1. EQ-5D and CFQ-R data showed that the worse the exacerbation, the poorer the health-related quality of life (HRQoL). There were strong relationships between the CFQ-R and EQ-5D domain scores. The mean rate of pulmonary exacerbations per patient per year was 3.6 (1.5 in hospital and 2.2 at home). The mean length of stay per hospital pulmonary exacerbation was 9 days. As exacerbation status worsens, patients experience worse HRQoL.

The authors confirmed that there is a significant healthcare burden associated with treatment of pulmonary exacerbation and long-term prophylaxis.

 

Judy Bradley

Judy Bradley (figure) is Professor of Physiotherapy at the University of Ulster. She has had a distinguished career in respiratory diseases particularly cystic fibrosis both nationally and internationally. She was presented with the Award of Distinguished Research Fellowship by the University in 2004 in recognition of her contribution to research in the area of respiratory physiotherapy. She was subsequently promoted to a Reader Joint Appointment at the University of Ulster/Belfast Health and Social Care Trust in 2005 and Professor in 2013.

 

2013 Kriemler S. Kieser S. Junge S. Ballmann M. Hebestreit A. Schindler C. Stussi C. Hebestreit H. Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibros 2013; 12:714-720. [PubMed]                                                                                            A study to determine the positive effects of long-term exercise training on FEV1. 39 Swiss patients with CF were randomly divided into strength training (ST, n=12), endurance training (AT, n=17) and controls (CON(CH), n=10), and also compared with age-matched Swiss (n=35) and German (n=701) CF registry data. A partially supervised training of 3x30 min/week for 6 months took place with measurements at baseline and after 3, 6, 12 and 24 months. Primary outcome was FEV1 at 6 months.

The FEV1 increased significantly in both training groups compared with the controls (AT:+5.8+0.95, ST:+7.4+2.5, CON(CH):-11.5+2.7% predicted, p<0.001) and both registry groups at 6 months. At 24 months, changes in favour of the training groups persisted marginally compared with controls, but not compared with registry data.

The authors concluded partially supervised training over 6 months improved FEV1 but effects were basically gone 18 months off training. They suggest regular long-term training should be promoted as essential part of treatment in CF.

 

2013 Ledger SJ. Owen E. Prasad SA. Goldman A. Willams J. Aurora P. A pilot outreach physiotherapy and dietetic quality improvement initiative reduces IV antibiotic requirements in children with moderate-severe cystic fibrosis. J Cyst Fibros 2013; 12:766-772.[PubMed]        The current model of care for children with moderate-severe CF is focused on intensive inpatient intervention, regular outpatient clinic review and specialist outreach care as required. An alternative model providing more regular physiotherapy and dietetic outreach support, in addition to these specialist services, may be more effective. Introduction of such a policy resulted in a 23% reduction in inpatient IV antibiotic requirement and 20% reduction in home IV antibiotic requirement during the intervention year. The authors concluded that this pilot programme demonstrated a reduction in IV and admission requirements with a cost benefit in a small group of children with moderate-severe CF.

The confirmation of benefit from this more intensive approach is to be expected and welcome. It is doubtful if such a finding would warrant a full scale trial however.

 

2013 McIlwaine MP. Alarie N. Davidson GF. Lands LC. Ratjen F. Milner R. Owen B. Agnew JL. Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis. Thorax 2013; 68:746-751. [PubMed]

Positive expiratory pressure (PEP) is the most commonly used method of airway clearance (AC) in Canada for patients with cystic fibrosis (CF) whereas, in some countries, high frequency chest wall oscillation (HFCWO) is the preferred form of airway clearance. There have been no long-term studies comparing the efficacy of HFCWO and PEP in the CF population. This study was to determine the long-term efficacy of HFCWO compared with PEP mask therapy in the treatment of CF as measured by the number of pulmonary exacerbations (PEs). A randomised controlled study was performed in 12 CF centres in Canada. After a 2-month washout period, subjects were randomised to perform either HFCWO or PEP mask therapy for 1 year.

RESULTS: 107 subjects were enrolled in the study; 51 were randomised to PEP and 56 to HFCWO. There were 19 dropouts within the study period, of which 16 occurred prior to or at the time of randomisation. There were significant differences between the groups in the mean number of PEs (1.14 for PEP vs 2.0 for HFCWO) and time to first PE (220 days for PEP vs 115 days for HFCWO, p=0.02). There was no significant difference in lung function, health-related quality of life scores or patient satisfaction scores between the two groups. PEP mask therapy required a shorter treatment time.

The results of this study favour PEP and do not support the use of HFCWO as the primary form of AC in patients with CF. Apparently the previous study on PEP from Vancouver by Maggie McIlwaine and her colleagues had a major influence on the introduction of the PEP mask into N. America (McIlwaine PM et al. J Pediatr 2001; 138:845-850. [PubMed]).

 

 

2014 Schneiderman JE, Wilkes DL, Atenafu EG, Nguyen T, Wells GD, Alarie N, Tullis E, Lands LC, Coates AL, Corey M, Ratjen F. Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis. Eur Respir J. 2014 Mar;43(3):817-23. doi: 10.1183/09031936.00055513. Epub 2013 Oct 31. [PubMed]
Free full text

Exercise is beneficial for patients with cystic fibrosis (CF) but long-term effects of physical activity on lung function evolution are unknown. We evaluated the longitudinal relationship between changes in habitual physical activity (HPA) and rate of decline in lung function in patients with CF. We tracked HPA using the Habitual Activity Estimation Scale, forced expiratory volume in 1 s (FEV₁) and Stage I exercise tests in 212 patients with CF over a 9-year period. Adjusting for sex, baseline age and FEV₁, mucoid Pseudomonas aeruginosa and CF-related diabetes, mean ± sd FEV₁ % predicted decreased by 1.63 ± 0.08% per year (p<0.0001) while mean ± sd HPA increased by 0.28 ± 0.03 h·day(-1) per year (p<0.0001) over the study period. A greater increase in HPA was associated with a slower rate of decline in FEV₁ (r=0.19, p<0.0069). Dividing subjects into "high" and "low" activity (above or below the mean rate of change of activity, respectively), a steeper rate of FEV₁ decline was observed for low (-1.90% per year) compared to high (-1.39% per year) (p=0.002). Increases in HPA are feasible despite progression of lung disease and are associated with a slower rate of decline in FEV₁, highlighting the benefit of regular physical activity, and its positive impact on lung function in patients with CF.

 


2015 Doumit M; Jaffe A.  Use of the Lung Flute for sputum induction in children with cystic fibrosis: a pilot study. Pediatr Pulmonol 2015; 50(4):340-3.      [PubMed]
This study aimed to assess the effectiveness of the Lung Flute in obtaining a sputum sample from children with cystic fibrosis (CF) that were not productive of sputum with coughing alone. Children attending an outpatient CF clinic who were not able to provide a sample with coughing alone were eligible. Each child used the Lung Flute on two occasions at least one month apart. The primary outcome was expectoration of a sputum sample. The authors found the lung flute to be clinically useful and an easy device for sputum induction in children with CF. They suggested further research comparing its effectiveness to other sputum induction methods is  warranted.

 
- The lung flute is a medical device used to clear mucus from congested lungs with low-frequency sound waves. The device consists of a mouthpiece and a plastic reed which vibrates within a chamber to create sound waves in the chest cavity. These sound waves vibrate and break up mucus deposits in the lungs, allowing cilia to more easily move these deposits from the lungs to the throat.  The lung flute was granted approval by the FDA in 2010 for use in patients with chronic obstructive pulmonary disease

 

Lung flute

 

 
2015 Freitas DA; Dias FA; Chaves GS; Ferreira GM; Ribeiro CT; Guerra RO; Mendonca KM.  Standard (head-down tilt) versus modified (without head-down tilt) postural drainage in infants and young children with cystic fibrosis.  Cochrane Database of Systematic Reviews. 3:CD010297, 2015. [PubMed]
Postural drainage is used primarily in infants with cystic fibrosis from diagnosis up to the moment when they are mature enough to actively participate in self-administered treatments. However, there is a reported risk of gastroesophageal reflux associated with this technique. The authors eventually concluded that the use of a postural regimen with a 30 degree head-up tilt is associated with a lower number of gastroesophageal reflux episodes and fewer respiratory complications in the longterm. The 20 head-down postural drainage position was not found to be significantly different from the 20 head-up tilt modified position. Nevertheless, the fact that the majority of reflux episodes reached the upper oesophagus should make physiotherapists carefully consider their treatment strategy.
 

-  There have been a number of publications on the most suitable form of physiotherapy for infants and young children since Brenda Button’s first important report of the head down position causing more frequent episodes of oesophageal reflux (Button BM et al, 1997.[PubMed]). In 2008, 25 senior physiotherapists from the Association of Chartered Physiotherapists in the UK were unable to reach a consensus on the need for physiotherapy in asymptomatic CF infants (Prasad SA et al, 2008). Recent Standards from the UK CF Trust advise, “If gastro-oesophageal reflux has been identified consider using modified postural drainage avoiding a head down tilt” (Agent P, Morrison L, Prasad A, last updated 2013). (See also Topics -> Physiotherapy).  In practice experienced physiotherapists use their clinical judgment in each individual infant conscious of the fact that GO reflux is a a possibility.

 

2015 Sawicki GS, Chou W, Raimundo K, Trzaskoma B, Konstan MW. Randomized trial of efficacy and safety of dornase alfa delivered by eRapid nebulizer in cystic fibrosis patients.  J Cyst Fibros. 2015 Nov;14(6):777-83. doi: 10.1016/j.jcf.2015.04.003. Epub 2015 Apr 25. [PubMed]
Efficacy and safety of dornase alfa via an electronic nebulizer with vibrating membrane technology have not been formally assessed in randomized clinical trials.  87 CF patients (≥6years) were randomized in a crossover study to receive dornase alfa 2.5mg/d in 2-week periods with the Pari eRapid and Pari LC Plus jet nebulizers. The primary end point was comparison of forced expiratory volume in the first second. Safety, quality of life, and treatment satisfaction/preference were also compared between devices.
Lung function was equivalent between nebulizers. Most domain scores from the Cystic Fibrosis Questionnaire-Revised and Treatment Satisfaction Questionnaire for Medication instruments were similar but patients strongly preferred the eRapid. Mean patient-reported administration times were shorter with the eRapid vs the LC Plus (2.7 vs 10.2min). Adverse events were similar between devices.
 
Administration of dornase alfa via the eRapid nebulizer resulted in comparable efficacy and safety, shorter nebulization times, and higher patient preference.