Is there a difference in airway clearance practices between children with non cystic fibrosis bronchiectasis and cystic fibrosis?
Abstract: Studies have investigated airway clearance techniques in cystic fibrosis (CF) but few exist for non-CF bronchiectasis particularly in children. The aim of this study is to compare airway clearance practices in New Zealand children with CF and non-CF bronchiectasis. The study was conducted in the context of a clinical quality assurance audit of a hospital clinic service. Children and their families who live in the Greater Auckland area and who attend tertiary respiratory clinics were interviewed by a physiotherapist, who completed a questionnaire on frequency of cough, amount and type of airway clearance performed. Demographic data, age at diagnosis, duration of disease and the New Zealand Deprivation Index were noted. Children were grouped according to age-appropriate airway clearance techniques. One hundred and six questionnaires were completed (81 bronchiectasis; 25 CF). Significant differences were demonstrated for ethnicity, deprivation score and duration of disease between the groups. In the 9-17 year olds 75% used a positive expiratory pressure device (23/35 bronchiectasis; 13/13 CF). In the 0-4 year olds 60% performed modified postural drainage (13/25 bronchiectasis; 5/5 CF). When well, children with CF did more regular airway clearance than those with bronchiectasis (p=0.0004). Both groups increased the amount of airway clearance when unwell (p=>0.05). Similar numbers (<8%) reported doing no airway clearance techniques. The implications of the findings for paediatric physiotherapy practice in New Zealand are discussed.

Key Words: Bronchiectasis, Airway clearance techniques, Cystic fibrosis, Adherence, Physiotherapy
Article Type: Report
Subject: Cystic fibrosis (Care and treatment)
Cystic fibrosis (Demographic aspects)
Cystic fibrosis (Research)
Respiratory therapy (Health aspects)
Respiratory therapy (Research)
Bronchiectasis (Care and treatment)
Bronchiectasis (Demographic aspects)
Bronchiectasis (Research)
Physical therapy (Health aspects)
Physical therapy (Research)
Therapeutics, Physiological (Health aspects)
Therapeutics, Physiological (Research)
Authors: Butler, Sarah G.
Hill, Louisa J.
Harrison, Julie
Reed, Peter
Nikora, Gwen
Takai, Coral
Byrnes, Catherine A.
Edwards, Elizabeth A.
Pub Date: 11/01/2008
Publication: Name: New Zealand Journal of Physiotherapy Publisher: New Zealand Society of Physiotherapists Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2008 New Zealand Society of Physiotherapists ISSN: 0303-7193
Issue: Date: Nov, 2008 Source Volume: 36 Source Issue: 3
Topic: Event Code: 310 Science & research
Product: Product Code: 8000436 Respiratory Therapy NAICS Code: 621 Ambulatory Health Care Services
Geographic: Geographic Scope: New Zealand Geographic Code: 8NEWZ New Zealand
Accession Number: 207945636
Full Text: INTRODUCTION

While debate continues regarding the lack of an evidence base for airway clearance techniques (ACTs) in Cystic Fibrosis (CF) and non-CF bronchiectasis (BX) management (Jones and Rowe 2007, Wallis and Prasad 1999), clinical guidelines continue to recommend these as best practice (Chang et al 2002, Edwards et al 2004b, Cystic Fibrosis Trust 2002, Lannefors et al 2004). The rationale is to reduce mucus retention--preventing or reducing the number of respiratory infections and therefore progression of lung disease ultimately improving prognosis (Lannefors et al 2004, Wallis and Prasad 1999). ACTs have been reported to reduce cough, reduce shortness of breath, improve exercise capacity and quality of life in CF (Chang et al 2002, Edwards et al 2004b, Cystic Fibrosis Trust 2002, Lannefors et al 2004, Lavery et al 2007). There is limited evidence to show that one technique is superior (Jones and Rowe 2007). Optimal amounts and type of ACT are also unknown. This has led authors to conclude that no single ACT is best for all patients and recommended treatment regimes should be individualized and undertaken regularly (Lannefors et al 2004). However, these require lifestyle modifications for the child and family which many find difficult (Abbott et al 1994, Abbott et al 1996, Conway et al 1996, Fielding and Duff 1999, Kyngas et al 2000, Lavery et al 2007, O'Neill et al 2002, White et al 2007).

Reported adherence to treatments in CF disease has varied depending on the specific treatment studied. For example, in children there is 16-20% adherence recorded for dietary supplementation but up to 67-84% for dornase alpha (Modi et al 2006). Chest physiotherapy is at the lower end of adherence (29-55%) when measured in studies of CF adults (Abbott et al 1994, Abbott et al 1996, Conway et al 1996, Kettler et al 2002, White et al 2007) with only one study recording adherence as high as 70% (White et al 2007) and reports of between 40-47% in children (Modi et al 2006). In addition, the adherence recorded varies depending on whether it is ascertained by parental report, child report, daily diary, pharmacy refills or electronic monitoring (usually decreasing across these parameters) (Modi et al 2006). Although there is much written about ACTs in the paediatric CF population there is very little on paediatric non-CF BX (Jones and Rowe 2007, O'Neill et al 2002) and results cannot necessarily be extrapolated between these groups.

There are a number of reasons why regular and effective airway clearance practices may be more important for New Zealand children with non-CF BX than other populations:

* The prevalence and incidence of non-CF BX has been reported to be very high (Twiss et al 2005);

* The disease seen is severe and widespread based on lung function and number of lobes affected (Edwards et al 2003, Edwards et al 2004a);

* A comparative study of exercise in children with CF and non-CF BX in the UK has shown significant exercise limitation in children with non-CF BX (Edwards et al 2003, Edwards et al 2004a).

In addition a previous unpublished clinical audit of 20 children with non-CF BX during establishment of the BX clinic at Starship Children's Hospital in Auckland in 1999 (conducted by GN, CT) showed 84% used head down postural drainage and active cycle of breathing technique with 21% intermittently compliant and 48% non-compliant. Thirty five percent felt this was of little or no use, with 75% either decreasing or not altering treatment frequency when the child was unwell. Thirty-five percent of the children reported being either 'not usually happy' or 'never happy' with their ACT, alarmingly often giving 'pain' or 'discomfort' as reasons for this. In response, the service developed educational resources (diagram, pictures and video) including a written action plan for use during stability and exacerbations. Each family was linked with an experienced respiratory physiotherapist for visits in clinic and at home. The CF service already had this educational and physiotherapy support, and families appeared to have a better understanding of the disease.

The aims of this current study were to use a questionnaire to compare airway clearance practices in children attending the BX and CF clinics at Starship Children's Hospital, a tertiary paediatric hospital, to examine how demographic data related to patterns of ACT use and to review whether the educational input had altered levels of adherence in our clinic.

METHODS

Only families living in the Greater Auckland Region and who attended either the Starship CF or non-CF BX clinics were invited to participate over a 6 month period from May 2006. During the time the audit was undertaken 25 of the potential 50 children who attended the Starship CF clinic, and 106 of the potential 150 children who attended the Starship Bx clinic were approached. None of the families declined to join the study. Following verbal consent, a questionnaire was completed with each family by one of three respiratory physiotherapists (SB, LH, JH) in the outpatient clinic, on the ward or in the child's home. All participants spoke English although an interpreter was available if necessary. Respondents (usually the mother, and the child if old enough) were all asked the same questions relating to the amount of daily coughing (as a crude measure of symptom severity), number of sessions and type of ACTs performed when well and unwell. School aged children were also asked the number of formal exercise sessions per week of more than 30 minutes duration. Demographic data, age at diagnosis, duration of disease and the New Zealand Deprivation 2001 Index (NZDep2001) (Salmond and Crampton 2002) were recorded. The index ranges from score 1 (10% least deprived nationally) to score 10 (10% most deprived).

The questionnaire results were interpreted in three age brackets (0-4, 5-8 and 9-18 years) corresponding to age-appropriate recommendations on ACTs and increasing self-responsibility. Statistical advice and analyses were undertaken by a statistician (PR) with the Stats Direct (www. statsdirect.com) MS Excel add-in software. The children's characteristics were expressed as means with standard deviations or medians with ranges depending upon whether they were normally distributed as ascertained by the Kolmogorov-Smirnov test. Fishers Exact statistic was used to test for differences in the distribution of responses between the Bx and CF cohorts, and between the age categories. A p value less than 0.05 was taken to be statistically significant. Verbal consent was obtained from the parent and child if old enough. The survey was designed as a clinical quality assurance audit and although discussed with the local ethics committee formal approval was not required.

RESULTS

One hundred and six questionnaires were completed over the 6 month study period--81/150 non-CF BX (54% of the clinic population) and 25/50 CF children (50% of the clinic population) with no significant differences seen between age or gender (see Table 1). There was a significant difference in ethnicity with 85% of children with BX from Maori or Pacific Island peoples and 72% of children with CF from European descent (p<0.0001). Significant differences were seen in the deprivation scores resulting in 75% of children with BX compared to 12% of children with CF living in the 30% most deprived areas (decile 8 and above) of New Zealand. The children with CF were diagnosed significantly younger and had a longer duration of lung disease (see Table 1).

There was no statistical difference between the groups with amounts of cough when well. In the BX cohort: 20 (25%) reported no cough, 24 (30%) reported coughing with exercise only and 24 (30%) coughed both day and night. Of the CF cohort: 11 (44%) reported no coughing, 7 (28%) reported coughing during exercise, and 5 (20%) coughed both day and night. These differences were not statistically significant. There was a statistical difference in reported cough between the groups when unwell (p=0.01) with all the BX group reporting significant cough, 5 (6%) during the day and 76 (94%) both day and night, compared to the CF group with 2 (8%) denying coughing even when unwell, 4 (16%) reporting coughing during the day or with exercise and 19 (76%) coughing day and night.

When well, 30 (37%) of children with BX did their ACT at least daily compared to 20 (80%) of children with CF (p=0.0004). When unwell, the difference was less significant with 63 (78%) of BX and 22 (88%) of CF patients doing their ACT at least once a day (p = ns). There was no significant difference between the groups in those doing no ACT (6 (7%) with BX, 1 (4%) with CF, p= >0.05). Reasons given why regular ACT were not undertaken included nearly half saying it was 'hard for them to do' (46% with BX, 44% with CF), they 'couldn't be bothered' (38% with BX, 64% with CF), it 'hurt' (14% with BX, 27% with CF), or because of a lack of time (43% with BX, 9% with CF).

In the 9-18 year age group 28 (38%) of those with BX and 14 (54%) of those with CF reported having problems with ACTs. Despite these difficulties 65 (80%) and 11 (44%) of children with BX or CF respectively felt that doing ACTs made them feel better, which was significantly different between the groups (p=0.0008). In the 9-18 year olds 75% used a Positive Expiratory Pressure device (PEP) (23/35 bronchiectasis; 13/13 CF) (see Table 2). In the 0-4 year olds 60% performed modified postural drainage (13/25 bronchiectasis; 5/5 CF) (see Table 2). One hundred percent of the CF and 81% of the BX children did their ACT at home (p>0.05), 14% had treatment elsewhere such as at relatives' homes and 5% were treated at school.

In the 5-8 year old children, 71% of the BX and 72% of the CF played sport at least once a week (p=>0.05) and 19% and 43% respectively played organised sport at least every day/week (p=0.03). In the older children and young people (9-18 years), 72% of the BX and 100% of the CF teenagers played sport at least once a week (p=0.03) and 28% and 62% respectively played organised sport at least every day per week (p=0.03).

DISCUSSION

Our practice (Edwards et al 2004b), in keeping with others (Chang et al 2002, Fibrosis 2002, Lannefors et al 2004, Wallis and Prasad 1999), is to recommend regular ACT and exercise programmes for all children with CF and non-CF BX, despite minimal supporting research evidence (Jones and Rowe 2007). As far as we are aware this is the first report to compare self-reported adherence to ACTs and exercise in these groups of children.

Three decades ago, a review of 537 studies showed the mean adherence to long term therapies was 54% (range 41-61%) (Sackett and Snow 1979). In our study, the adherence to recommendations on ACT in Bx was well below this level when well (37%) but above average when unwell (78%). However, the adherence in our study for CF was far above average when well or unwell. The earlier diagnosis and longevity of disease in CF may have had a beneficial effect on adherence. Regular chest physiotherapy, including airway clearance training from an early age may facilitate acceptance and enable caregivers to develop these skills, while introducing new treatments to older children can be fraught with difficulties (Patterson et al 2005, White et al 2007). This is different from the finding that adherence decreases as duration and complexity of treatment increases (Kettler et al 2002). Resourcing for clinics are also different with the CF society providing the clinic with a full time social worker and 16 hours of physiotherapist time in the community, while there is no BX family support group, a part time physiotherapist, no dedicated social worker and no current cultural support worker. A study of adults with CF conducted through the UK CF Trust showed consultation with a physiotherapist was positively associated with increased adherence (Myers and Horn 2006).

A similar, small percentage of both groups (<8%) undertook no ACTs. Although reasons given for not doing regular treatments varied, in the main they were 'could not be bothered' and 'lack of time'. These are consistent with other studies reporting interview data in teenage and adult CF groups with 61%-80% giving these same two reasons (Abbott et al 1996, Conway et al 1996, Fielding and Duff 1999, Myers and Horn 2006, White et al 2007). Other responses from the studies included 26-45% felt they were well without performing ACT (Abbott et al 1996, Conway et al 1996, White et al 2007), 13-37% felt their disease was less severe than others (Abbott et al 1996, White et al 2007), 10%-53% were too tired (Modi et al 2006, White et al 2007), 35% said it interrupted their social life (Abbott et al 1996) and 22-35% simply forgot (Abbott et al 1996, White et al 2007). In one study respondents gave twice as many reasons for omitting ACT than for any other treatment studied (Conway et al 1996). The lack of time reported in the BX cohort may be due to the number of families attending BX clinic with higher numbers of children in the family.

Studies assessing adherence in CF show that perceived severity or increased 'worry' about their disease was associated with improved adherence (Abbott et al 1996, Gudas et al 1991). Patients who report an immediate improvement in symptoms after ACT have also been shown to have improved compliance (Lavery et al 2007). This may account for the increase in compliance seen in the BX group when they became unwell. However, while more of the BX cohort compared to the CF reported feeling better after performing their ACT, the BX group did less regular treatment than the CF group.

Overall, the BX group continued to have lower adherence rates than their CF counterparts despite being managed by the same medical team. The Chronic Care Model endorsed by the World Health Organisation suggests that patient outcomes can be improved by proactive healthcare providers informing and activating patients (Lavery et al 2007). In a study of adult BX patients perceived obstacles to self-management included lack of information and confidence (Lavery et al 2007). The significant cultural differences between the families attending the BX or CF clinics (Twiss et al 2005) are likely to impact on understanding the disease and treatment. In our clinic in 1999, 15/20 (75%) of BX families either only maintained or actually decreased ACT during an acute exacerbation. In this study 63/81 (78%) did at least daily sessions when unwell following extra educational input. While this may be seen as an improvement it is still suboptimal.

Exercise also improves both physical and mental well-being and has been shown to improve aerobic capacity, endurance exercise capacity and inspiratory muscle strength in both BX and CF (Barak et al 2005, Lannefors et al 2004) with 17-38% admitting a preference for exercise than doing ACT (Abbott et al 1994, Conway et al 1996, Myers and Horn 2006, White et al 2007). In our survey, children with BX undertook less sport than children with CF, perhaps because of the socioeconomic differences seen and/or the severity of lung disease with increased cough reported in the BX group. Formal exercise testing has yet to be reported for New Zealand children with BX, but the literature suggests significant exercise limitation in comparison with normal children and with CF (Edwards et al 2004a).

The type of ACTs best suited to individual respiratory patients continues to be debated, with the choice decided by culture, tradition, economics and geographical influences (O'Neill et al 2002),(Barak et al 2005, Homnick 2007, Lavery et al 2007, Patterson et al 2005, Thompson et al 2002). A 2002 survey of UK BX clinics (predominantly adult) showed the majority used active cycle of breathing technique (91.5%) and/or postural drainage (75.6%) (O'Neill et al 2002). In contrast, in our study 75% of the patients over the age of 9 years used PEP. They found it more discrete, more enjoyable and more flexible (e.g. able to do in car, at the marae, etc). Caregivers also reported finding PEP easier to monitor and they did not have to be actively involved in the treatment. This confirms previous findings that the least difficult programme and those requiring the least alteration to lifestyle will tend to be favoured (Barak et al 2005, Gudas et al 1991, Homnick 2007, Thompson et al 2002). We have also found PEP easier to teach in school settings, which are the only treatment session administered to some of the patients. While other devices such as Flutter and Acapella are currently used by some of the children in both clinics at the time the study was undertaken none of the patients used either of these devices. In the 0-4 year age group, 60% were treated without a head down tip subsequent to demonstration that the previous head down recommendation caused increased gastro-oesophageal reflux in children with CF (Button et al 2003, Button et al 2004, Lannefors et al 2004).

This survey has a number of weaknesses and the findings cannot be extrapolated to other populations or groups. In this report of a quality assurance audit, only approximately 50% of the clinic populations were able to be surveyed within the study period but none approached declined. There were fewer children with CF within the catchment area which led to unequal numbers between the groups. The questions asked were based on standard questions asked at clinic visits by our physiotherapy team; however no measures of reliability and validity were undertaken. Three interviewers (all physiotherapists) were involved rather than one or repeat interviews by 2 people.

This survey only assessed adherence to the recommended physiotherapy programme by direct interview; this may well have led to over-reporting of adherence by the families. One study has showed significant differences in adherence when reported by parent, child, daily diary record or measured by electronic devices (Modi et al 2006). Specifically, ACT frequency adherence decreased from 74% by parent report to 67% by child report to 51% by diary record (Modi et al 2006). Other studies used a scale or a score for interview data to assess adherence (Abbott et al 1994, Conway et al 1996).

While these risks of bias created could have been addressed by the questionnaire being postal or completed independently, this may have reduced the response rate. Finally cough was the only parameter used as a crude measure of severity of disease rather than radiological, lung function or formal exercise testing, and severity of disease may affect adherence as mentioned above (Abbott et al 1996, Passero et al 1981). The primary study aim was not to assess severity of disease but to investigate airway clearance practices. Despite these weaknesses the study has a number of important implications for paediatric respiratory and physiotherapy practice in New Zealand.

CONCLUSIONS

In this survey, undertaken as part of a quality assurance audit, the Bx group showed less adherence to the treatment recommendations than their CF counterparts. The significant differences documented regarding ethnicity, age of diagnosis, disease duration and socioeconomic status may provide some explanation. We acknowledge that, as this survey was undertaken in the clinics of only one hospital, the findings may not be generalisible to other centres. If we could improve some of the socioeconomic hurdles and provide equal resourcing for the two clinics, plus improve access to culturally appropriate education around the disease and treatment, the differences in adherence between these groups might not be so great.

Key Points

* Current best practice is to recommend airway clearance techniques which are individualized and undertaken regularly for all children with cystic fibrosis and bronchiectasis.

* This is the first report to compare self-reported adherence to prescribed airway clearance techniques in children with cystic fibrosis and non-CF bronchiectasis.

* New Zealand children with non-CF bronchiectasis showed less adherence to treatment recommendations than their CF counterparts.

* In New Zealand improving some of the socioeconomic hurdles and providing equal resourcing may improve adherence in these children.

ACKNOWLEDGEMENTS

Nil. Funding: nil

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Sarah G Butler, Grad Dip Phys

Louisa J Hill, BSc (Hons) Physiotherapy

Julie Harrison, BHSc

Peter Reed, DPhil

Gwen Nikora, MBChB

Coral Takai, MBChB

Catherine A Byrnes, FRACP, MBChB, GCCE

Elizabeth A Edwards, PhD, FRACP, MBChB

Department of Paediatrics, University of Auckland & Department of Respiratory Medicine, Starship Children's Hospital, Auckland, New Zealand

ADDRESS FOR CORRESPONDENCE

Dr Elizabeth Edwards, Department of Respiratory Medicine, Starship Children's Hospital, Private Bag 92024, Auckland, New Zealand. Phone: 64 9 307 4949 ext 7960. Fax: 64 9 307 8977. E-mail: lize@adhb.govt.nz
Table 1. Demographics for the children with cystic
fibrosis and non-CF bronchiectasis.

Demographics            Bronchiectasis    Cystic fibrosis    P values

Number                        81                 25             --
Sex (boys)              43 boys (53%)      15 boys (60%)        ns
Ethnicity
  European %              11 (13.6%)         18 (72.0%)      <0.0001
  Maori %                 11 (13.6%)          6 (24.0%)      <0.0001
  Pacific Island %        58 (71.6%)          1 (4.0%)       <0.0001
  Other %                  1 (1.2%)           0 (0%)            ns
Current age
  Median (range)       7.7 (0.05-17.79)   9.0 (0.97-17.52)      ns
Age at diagnosis
  Median (range)        4.8 (0-16.89)       0.1 (0-9.83)     <0.0001
Longevity of disease
  Median (range)       2.8 (0.17-10.25)   7.3 (1.17-17.83)   <0.0001
NZDepScore
  Median (range)           9 (1-10)           4 (1-10)       <0.0001

ns = not significant

Table 2. Airway clearance techniques used in children with CF
and non-CF bronchiectasis.

Groups                      BX            CF           Total

Age                       0 to 4        0 to 4        0 to 4
years
Number of children          25             5            30
ACT
  None                    1 (4%)          --          1 (3%)
  Modified PD & P        13 (52%)      5 (100%)      18 (60%)
  PD & P                 11 (44%)         --         11 (37%)
  Modified PD & ACBT        --            --            --
  PD & P & ACBT             --            --            --
  PEP                       --            --            --
  PEP & Bubble PEP          --            --            --

Groups                      BX            CF           Total

Age                       5 to 8        5 to 8        5 to 8
years
Number of children          21             7            28
ACT
  None                    1 (5%)          --          1 (3%)
  Modified PD & P           --            --            --
  PD & P                    --            --            --
  Modified PD & ACBT      2 (10%)       1 (14%)       3 (11%)
  PD & P & ACBT          12 (57%)       2 (29%)      14 (50%)
  PEP                       --            --            --
  PEP & Bubble PEP        6 (29%)       4 (57%)      10 (36%)

Groups                      BX            CF           Total

Age                       9 to 18       9 to 18       9 to 18
years
Number of children          35            13            48
ACT
  None                    1 (4%)          --          1 (2%)
  Modified PD & P           --            --            --
  PD & P                    --            --            --
  Modified PD & ACBT      4 (11%)         --          4 (8%)
  PD & P & ACBT           7 (19%)         --          7 (15%)
  PEP                    23 (67%)      13 (100%)     36 (75%)
  PEP & Bubble PEP          --            --            --

BX = bronchiectasis; CF = cystic fibrosis; ACT = airway clearance
technique; Mod = modified; PD = postural drainage; P = percussion;
PEP = positive expiratory pressure; ACBT = active cycle of breathing
technique
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