Penny Agent – Deputy Director of Rehabilitation & Therapies, Royal Brompton & Harefield NHS Foundation Trust, London, UK.
Physiotherapy is one of the mainstays of daily CF management. This summary of recent research within CF covers the following aspects: a long-term randomised controlled trial of two airway clearance techniques, two systematic reviews evaluating the timing of dornase alpha (DNase) and also the use of non-invasive ventilation in CF, the effects of a year long supervised outpatient exercise and physiotherapy programme for children with CF, and finally the impact of pain on quality of life and treatment. It is encouraging to see the use of well designed long-term randomised controlled controls within physiotherapy as too often studies are short-term or single treatment sessions which makes results interpretation difficult when outcome measures may not be sensitive enough to represent change in this chronic long-term disease.
Long-term multi-centre randomised controlled study of high frequency chest wall oscillation (HFCWO) versus positive expiratory pressure (PEP) mask in CF
McIllwaine M, Alarie N, Davidson GF et al. Thorax 2013;68:746-51
PEP masks are the most commonly used method of airway clearance in Canada for patients with CF, whereas in America (just over the border!) the HFCWO (vibrating Vest) is the preferred form of airway clearance. There are no previous long-term studies comparing these two airway clearance techniques and therefore a national (Canada) multi-centre (12 centres) randomised controlled trial was carried out to determine the long-term efficacy of HFCWO compared with PEP mask therapy as measured by the number if pulmonary exacerbations. 107 subjects were enrolled across the 12 centres, with 51 subjects randomised to PEP and 56 to HFCWO.
Results showed that the number of pulmonary exacerbations was significantly higher in patients performing HFCWO compared with those performing PEP (on average 1.14 for PEP vs 2.0 for HFCWO). Patients using HFCWO experienced their next pulmonary exacerbation after starting the treatment earlier than those using PEP (220 days for PEP vs 115 days for HFCWO which was statistically meaningful). There were no significant differences in lung function, health related quality of life or patient satisfaction scores between the two groups. PEP mask therapy also 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 airway clearance in patients with CF. Whilst the overall number of pulmonary exacerbations requiring intravenous antibiotics was low, the number of pulmonary exacerbations requiring intravenous antibiotics in the HFCWO group was more than three times the number in the PEP group.
It is well known that the inhalation of the enzyme dornase alpha (pulmozyme/DNase) reduces the thickness and stickiness of sputum and improves clinical outcomes of people with CF. A systematic review was conducted investigating the timing of dornase alpha inhalation on measures of clinical efficacy, in relation to airway clearance therapy or time of day.
Five studies (122 participants) met the inclusion criteria with study intervention periods ranging from 2 to 8 weeks. Conclusions stated that inhalation of dornase alpha after airway clearance therapy is not more or less effective that the traditional recommendation to inhale nebuliser dornase alpha 30mins prior to airway clearance therapy. There is no strong evidence to indicate one timing regimen is better than another, and individuals should discuss their optimal regime with the specialist CF physiotherapist.
NIV may be a means to temporarily reverse or slow the progression of respiratory failure in CF. It is widely used clinically in CF both for the management of respiratory failure but also to assist with airway clearance.
Fifteen trials (106 participants) were identified which compared a form of NIV in people with CF with acute or chronic respiratory failure. NIV may be a useful addition to other airway clearance techniques particularly if people have difficulty expectorating sputum. When NIV is used in addition to oxygen it may improve the amount of oxygen absorbed and the amount of carbon dioxide cleared from the body during sleep to a greater extent than oxygen therapy alone in moderate to severe disease. These benefits have largely been demonstrated in single treatment sessions with small numbers of participants. The impact of the use of NIV on pulmonary exacerbations and disease progression remains unclear. There is a need for long term randomised controlled trials to determine the clinical effects of NIV in CF airway clearance and exercise.
The effect of a year long supervised outpatient exercise and physiotherapy programme in children with CF was investigated. Subjects with CF aged greater than 10 years had received more than 4 intravenous antibiotic courses in a year were enrolled and seen fortnightly for supervised exercise three times weekly.If they were,they completed additional physiotherapy sessions extra to the usual chest physiotherapy. Outcome measures included the modified shuttle test (a type of walking test where participants increase their rate of walking between two points), quality of life at baseline and at 1 year, and number of intravenous antibiotic days. Twelve subjects were enrolled. Results showed a the supervised programme results in a significant reduction in number of days that intravenous antibiotics were needed from 60 (56-64) in 2009 to 50(44-56) in 2010, along with an improved modified shuttle test distance 735 (603-867) verses 943 (725-1161)..
Overall this study showed that supervised, outpatient exercise and physiotherapy are associated with improvements in quality of life and exercise tolerance, a reduction in intravenous antibiotic days and a trend towards reducing lung function decline in children with cystic fibrosis. A significant reduction in cost of intravenous antibiotics (by £66,384) was also shown during the one year study period. Such cost-benefit may have implications for workforce planning and service provision.
Pain has been reported in CF but its clinical significance and the physical and psychosocial impact in adults who are clinically stable and acutely unwell have not been described. The aim of this observational study was to describe the intensity and location of pain and its relationship with the health related quality of life and pain catastrophizing in adults with CF. Patients completed three questionnaires when they were clinically stable and during an acute exacerbation. 73 participants were included during a period of clinical stability with 33 repeating the measurements during an acute exacerbation. The mean age was 29 (9SD) years and FEV1 60.5 (24.9)%predicted. 89% of stable participants reported mild pain and 79% of those with exacerbations. Severity of lung disease did not affect prevalence or intensity of pain. Pain interfered with airway clearance therapy during exacerbations and was related to a poorer physical function, regardless of clinical status.
Although pain intensity is generally mild, those with a negative emotional response to pain have significant impaired health related quality of life.