Adherence to Aerosol Therapy in Young People With Cystic Fibrosis: Patient and Parent Perspectives Following Electronic Data Capture.

The benefits of improved treatments for cystic fibrosis (CF) depend on optimal adherence, which remains problematic, particularly to aerosol therapy. In this study, we explored the process of adhering to aerosol therapy from the perspective of both adolescents with CF and their parents. Interviews were conducted individually with six adolescents and six parents, informed by accurate adherence data from an electronically chipped, aerosol device. Interview transcripts from audio-recordings were analyzed using grounded theory method (GTM). Major themes revealed differences in perspective between parent and adolescent, with this relationship mediating the cognitive and emotional processes that play a significant role in adherence behavior. These processes are further influenced by interactions with the aerosol therapy treatment regimen, device characteristics, and the context in which adherence is taking place. Parents and adolescents have different views of treatment and how to manage it. Both need to be addressed if optimal adherence is to be achieved.

A formative study exploring perceptions of physical activity and physical activity monitoring among children and young people with cystic fibrosis and health care professionals.

BACKGROUND: Physical activity (PA) is associated with reduced hospitalisations and maintenance of lung function in patients with Cystic Fibrosis (CF). PA is therefore recommended as part of standard care. Despite this, there is no consensus for monitoring of PA and little is known about perceptions of PA monitoring among children and young people with CF. Therefore, the research aimed to explore patients' perceptions of PA and the acceptability of using PA monitoring devices with children and young people with CF. METHODS: An action research approach was utilised, whereby findings from earlier research phases informed subsequent phases. Four phases were utilised, including patient interviews, PA monitoring, follow-up patient interviews and health care professional (HCP) interviews. Subsequently, an expert panel discussed the study to develop recommendations for practice and future research. RESULTS: Findings suggest that experiences of PA in children and young people with CF are largely comparable to their non-CF peers, with individuals engaging in a variety of activities. CF was not perceived as a barrier per se, although participants acknowledged that they could be limited by their symptoms. Maintenance of health emerged as a key facilitator, in some cases PA offered patients the opportunity to 'normalise' their condition. Participants reported enjoying wearing the monitoring devices and had good compliance. Wrist-worn devices and devices providing feedback were preferred. HCPs recognised the potential benefits of the devices in clinical practice. Recommendations based on these findings are that interventions to promote PA in children and young people with CF should be individualised and involve families to promote PA as part of an active lifestyle. Patients should receive support alongside the PA data obtained from monitoring devices. CONCLUSIONS: PA monitoring devices appear to be an acceptable method for objective assessment of PA among children and young people with CF and their clinicians. Wrist-worn devices, which are unobtrusive and can display feedback, were perceived as most acceptable. By understanding the factors impacting PA, CF health professionals will be better placed to support patients and improve health outcomes.

Autogenic drainage for airway clearance in cystic fibrosis.

BACKGROUND: Autogenic drainage is an airway clearance technique that was developed by Jean Chevaillier in 1967. The technique is characterised by breathing control using expiratory airflow to mobilise secretions from smaller to larger airways. Secretions are cleared independently by adjusting the depth and speed of respiration in a sequence of controlled breathing techniques during exhalation. The technique requires training, concentration and effort from the individual. It is important to systematically review the evidence demonstrating that autogenic drainage is an effective intervention for people with cystic fibrosis. OBJECTIVES: To compare the clinical effectiveness of autogenic drainage in people with cystic fibrosis with other physiotherapy airway clearance techniques. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews, as well as two trials registers (31 August 2017).Dtae of most recent search of the Cochrane Cystic Fibrosis Trials Register: 25 September 2017. SELECTION CRITERIA: We identified randomised and quasi-randomised controlled studies comparing autogenic drainage to another airway clearance technique or no therapy in people with cystic fibrosis for at least two treatment sessions. DATA COLLECTION AND ANALYSIS: Data extraction and assessments of risk of bias were independently performed by two authors. The authors assessed the quality of the evidence using the GRADE system. The authors contacted two investigators for further information pertinent to their published studies. MAIN RESULTS: Searches retrieved 35 references to 21 individual studies, of which seven (n = 208) were eligible for inclusion. One study was of parallel design with the remaining six being cross-over in design; participant numbers ranged from 17 to 75. The total study duration varied between four days and two years. The age of participants ranged between seven and 63 years with a wide range of disease severity reported. Six studies enrolled participants who were clinically stable, whilst participants in one study had been hospitalised with an infective exacerbation. All studies compared autogenic drainage to one (or more) other recognised airway clearance technique. Exercise is commonly used as an alternative therapy by people with cystic fibrosis; however, there were no studies identified comparing exercise with autogenic drainage.The quality of the evidence was generally low or very low. The main reasons for downgrading the level of evidence were the frequent use of a cross-over design, outcome reporting bias and the inability to blind participants.The review's primary outcome, forced expiratory volume in one second, was the most common outcome measured and was reported by all seven studies; only three studies reported on quality of life (also a primary outcome of the review). One study reported on adverse events and described a decrease in oxygen saturation levels whilst performing active cycle of breathing techniques, but not with autogenic drainage. Six of the seven included studies measured forced vital capacity and three of the studies used mid peak expiratory flow (per cent predicted) as an outcome. Six studies reported sputum weight. Less commonly used outcomes included oxygen saturation levels, personal preference, hospital admissions or intravenous antibiotics. There were no statistically significant differences found between any of the techniques used with respect to the outcomes measured except when autogenic drainage was described as being the preferred technique of the participants in one study over postural drainage and percussion. AUTHORS' CONCLUSIONS: Autogenic drainage is a challenging technique that requires commitment from the individual. As such, this intervention merits systematic review to ensure its effectiveness for people with cystic fibrosis. From the studies assessed, autogenic drainage was not found to be superior to any other form of airway clearance technique. Larger studies are required to better evaluate autogenic drainage in comparison to other airway clearance techniques in view of the relatively small number of participants in this review and the complex study designs. The studies recruited a range of participants and were not powered to assess non-inferiority. The varied length and design of the studies made the analysis of pooled data challenging.

Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time.

OBJECTIVES: Treatment regimen for families of children with cystic fibrosis (CF) is considerable, particularly when nebulised therapies for chronic Pseudomonas aeruginosa airway infection are prescribed. Adherence to these regimens is variable, particularly in adolescence. Previously, we reported children to be more adherent in evenings compared to mornings, suggesting an association with time-pressure. The aim of this study was to determine whether adherence would be better in adolescent patients at weekends and during school holidays when time-pressures may be less. STUDY DESIGN: 24 patients (14 male, median [range] age 13.9 [11.1-16.8] years) were enrolled from two regional paediatric CF centres in the United Kingdom. Data for a full scholastic year, were downloaded openly from a breath-activated data logging nebuliser (I-neb™). Adherence (% of doses taken÷expected number) was calculated during term-times, holidays, weekends and weekdays, for each patient. RESULTS: Large variations in adherence were seen between patients. However, adherence during term-time was significantly better than holidays (p<0.001). Weekday adherence was better than weekend adherence in term-time but not holidays. Interestingly, patients prescribed three daily treatments took on average 1.4 treatments/day, a similar number to those prescribed two daily treatments. CONCLUSION: Overall adherence to inhaled therapies was reasonable, but significantly reduced during holiday periods. This suggests a need for families to have not only time, but also structure in their daily routine to maintain optimal adherence to long-term therapies. It is important for CF teams to appreciate these factors when supporting families.

New nebulizer technology to monitor adherence and nebulizer performance in cystic fibrosis.

Topical delivery of aerosolized therapies is an established treatment for chronic airway infection and inflammation in cystic fibrosis (CF). Recent developments in nebuliser technology have enabled Adaptive Aerosol Delivery (AAD) of mesh generated aerosol particles resulting in more efficient airway deposition than existing jet nebulizers. An additional feature of these new devices is the ability to record and examine the performance of the device by downloading stored data (electronic data capture). In a series of studies we have used this downloading facility to monitor treatment times and examine adherence to nebulizer therapy in our pediatric patients. We found routine adherence monitoring is possible in busy CF clinic. We have shown that good adherence to treatment can be maintained in both patients chronically infected with Pseudomonas aeruginosa on long-term therapy, and in patients with first/new growths of Pseudomonas on short-term eradication therapy. When adherence was examined from an individual perspective, we demonstrated a wide variation both between and within individual patients. A further modification of AAD technology, Target Inhalation Mode (TIM) optimises patient inhalations through a direct feedback mechanism. This new breathing mode has also been evaluated in our pediatric CF clinic in a recent randomized controlled trial (RCT) and we have shown that children maintain adherence to treatment through the TIM mouthpiece and average treatment times reduced from 6.9 to 3.7 min when using TIM. This is a new era of aerosol delivery and novel advances in medical devices need to be monitored and assessed rigorously, particularly as new and potentially expensive therapies emerge from translational studies. Electronic data capture enables CF teams to work in an open partnership with patients to achieve the common goals of improving drug delivery and reducing patient burden.

A randomised controlled trial of breathing modes for adaptive aerosol delivery in children with cystic fibrosis.

BACKGROUND: Aerosol delivery is a cornerstone of CF airways disease management. New nebulisers have reduced treatment times by utilising mesh technology for aerosol production. We have evaluated a further modification (target inhalation mode (TIM)) that may reduce treatment delivery times further. METHODS: 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. RESULTS: 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. CONCLUSION: TIM reduces nebuliser treatment times and may positively impact on adherence, although longer duration studies are required to examine this. (ISRCTN65617839).

Open adherence monitoring using routine data download from an adaptive aerosol delivery nebuliser in children with cystic fibrosis.

BACKGROUND: Adaptive aerosol delivery (AAD) nebuliser devices can reduce treatment times whilst enabling adherence to be monitored using inbuilt data logs. Using one such device, we have monitored nebulised antibiotic adherence in children with Cystic Fibrosis (CF). METHODS: In CF children infected with Pseudomonas aeruginosa, downloaded data from an AAD device was used to calculate morning, evening and overall monthly adherence to antibiotic therapy over a year. RESULTS: Overall monthly adherence to nebulised antibiotic therapy in 28 children was maintained between 60 and 70% over the year. Considerable variation in adherence, both between and within patients, was evident (Mean [SD] coefficient of variation, 37[44]%). Evening adherence (75[37]%) was better than morning adherence (58[34]%: p=0.012). Treatment regimens were changed in 8/28 patients based on adherence data. CONCLUSIONS: Routine adherence monitoring can be implemented in an outpatient setting. Using this type of information it is possible to identify which aspects of treatment can be improved and to work together with families to individualize treatments.