Nebuliser cleaning and disinfection practice in the home among patients with cystic fibrosis

Abstract

Background:

Nebulised delivery of different classes of drugs is of fundamental importance in therapeutic regimens relating to both the management of disease progression in cystic fibrosis disease and its associated complications. The aim of this study was to determine if current nebuliser hygiene practices in the home environment by paediatric and adult cystic fibrosis populations are appropriate to ensure appropriate infection control and prevention measures have been addressed.

Methods:

An Audit Questionnaire Study was completed with adult cystic fibrosis patients (n=20) or with parents of cystic fibrosis children (n=24), through a healthcare professional interview on a one-to-one basis, during either a home visit or during patient/parent attendance at cystic fibrosis clinic.

Results:

Hygienic practices relating to nebuliser care varied, with paediatric carers more likely to clean and disinfect their devices. This study suggests there is much variation and confusion with regard to how to clean and disinfect nebulisers, as well as who is responsible for delivering this advice.

Conclusion:

The adult cystic fibrosis community in particular needs to be educated on practicalities associated with nebuliser hygiene and the reasons why this is important. Furthermore, to date there is a lack of a universally recommended guideline suitable for all types of cystic fibrosis nebulisers that all relevant pharmaceutical manufacturers advocate.

Background

Cystic fibrosis (CF) is an autosomal recessive and progressive disease affecting predominately Caucasian populations of European ancestry, with an incidence of one in 2500 (Ponzano et al, 2018). Symptoms of CF are related to the wide tissue distribution of a defective protein, the CF transmembrane conductance regulator (CFTR), encoded by a gene on chromosome 7 (Castellani and Assael, 2017). CFTR functions as a cAMP-dependent anion channel that transports chloride and bicarbonate across epithelial surfaces and disruption of these ion transport processes plays a central role in the pathogenesis of CF (Gentzsch and Mall, 2018). Failure or altered expression of this protein results in abnormal viscous mucus secretions that occlude the airway and ductal lumens, leading to recurrent pulmonary infections, pancreatic insufficiency with malabsorption, intestinal obstruction syndromes, CF-related diabetes, male infertility and in some cases, liver disease (Martiniano et al, 2016).

Bronchiectasis develops as a result of CF and increases the risk of chronic respiratory infection. This then leads to a high incidence of morbidity and mortality. A therapeutic approach has been inhaled therapies and in particular nebulised drugs, including bronchodilators, antibiotics, mucolytics and osmotics, in addition to oral, inhaled and intravenous medication (Supplementary Figure 1). Nebulised delivery of drugs offers the advantages of the distribution of high drug concentrations in the airways and a reduction in systemic toxicity (Agent and Parrott, 2015). The daily therapeutic schedule for the CF patient is therefore both complex and demanding, particularly in relation to time, with nebulised therapies claiming a major proportion of this regime at an average 108 minutes per day (Sawicki et al, 2009).

It is vital that CF patients understand the importance of nebuliser hygiene, both in terms of preventing infection associated with contaminated nebulisers, as well as optimum therapeutic drug delivery. Given the high costs, both in burden of care and the financial cost of medication, it is important that nebuliser efficacy is optimised through appropriate care and hygiene. Such optimal parameters have been addressed by nebuliser manufacturers through design, therefore this places great emphasis on nebuliser education with the CF multidisciplinary team (CF-MDT), as well as the patient, in performing routine cleaning, disinfection and maintenance.

Several studies have highlighted contaminated nebulisers as potential reservoirs of bacteria, including Burkholderia cenocepacia, Stenotrophomonas maltophilia and Pseudomonas aeruginosa, as well as fungi and yeast organisms, which may be delivered to the CF lung via a device (Hutchinson et al, 1996; Blau et al, 2007; Peckham et al, 2016). Such an extraneous introduction of many of these micro-organisms to the upper and lower airways of the CF patient could be clinically detrimental to the health of the patient. During the clinical timeline of CF disease progression, patients may become colonised from other CF patients, from the immediate healthcare estate or from the environment. Certain organisms, especially Pseudomonas aeruginosa, Staphylococcus aureus and Burkholderia cenocepacia, have been shown to be important respiratory pathogens in CF patients, therefore it is best practice to attempt to eradicate these organisms when they are first detected microbiologically (Castellani et al, 2018). Treatment for eradication generally involves nebulised antibiotics, including tobramycin and colomycin. Inappropriate cleaning and disinfection of the patients’ nebulisers in this scenario could lead to the re-introduction of bacterial pathogens to the patients’ respiratory tracts and subsequently the establishment of a vicious cycle of nebuliser contamination and patient re-infection. The continuance of nebuliser device contamination and patient re-infection may eventually lead to the patient becoming chronically colonised, which has been shown to lead to decreasing lung function, poorer nutrition, more pulmonary exacerbations and higher mortality (Emerson et al, 2002). Infection prevention and control advice aims to break this cycle of re-infection. Patient adherence to the appropriate maintenance, cleaning and disinfection of their nebulisers is essential in supporting this principle.

Although there have been several reports in the literature on adherence to nebulised therapies in CF, there is a relative paucity of reports detailing CF patient adherence to nebuliser cleaning and disinfection (Hogan et al, 2015; Jones et al, 2015). The aim of this study was therefore to investigate the cleaning and disinfection practices relating to nebuliser care and maintenance of two CF groups, namely individuals who attended (i) a regional paediatric CF centre and (ii) a regional adult CF centre.

Methods

An Audit Questionnaire Study was designed to evaluate cleaning and disinfection practices relating to nebuliser care and maintenance within the CF community at two regional CF centres, namely the Northern Ireland Paediatric CF Centre, Royal Belfast Hospital for Sick Children and the Northern Ireland Regional Adult Cystic Fibrosis Centre, Belfast City Hospital. Participation in this survey was voluntary and completed questionnaires were anonymised. To maintain anonymity, age, sex and any other identifiers of persons participating in the survey were not recorded. Questionnaires were completed by a personal interview between a senior member of the CF-MDT. Parents of paediatric patients attending the CF clinic or during home visits by the paediatric CF nurse completed the survey of 10 questions in one sitting, lasting approximately 10–15 minutes. Adult CF patients completed the survey in one sitting alongside the CF physiotherapist during their in-patient stay or during their attendance at CF clinic. Both paediatric and adult surveys were completed during the same week. Verbal informed consent was obtained from study participants, who also received a survey information leaflet. This audit did not require formal ethical approval because it was an audit of existing patient practices to drive quality improvement. Questions examined the types of compressors and drug chambers used, the washing and disinfection practices and influences that motivated or prevented nebuliser hygiene maintenance. Practices were audited against manufacturers’ instructions (Tables 1 and 2) and such guidance was subsequently reinforced by the healthcare professional with the CF patient/parent at the conclusion of the interview.

Table 1.

Manufacturers’ instructions on cleaning SideStream nebulisers.

Cleaning	Disinfection
SideStream reusable (Philips Respironics) PT1200; 2013 Frequency After each use Method • Take apart SideStream top, medicine cup, baffle and mouthpiece and wash in warm soapy water • Rinse all parts in clean cold water • Shake out excess water and allow to air dry • Reassemble making sure the baffle is in the correct position	Frequency Weekly Method • Take apart SideStream and boil all parts (not tubing or face mask) in water with 2–3 drops of dish-washing liquid for 10 minutes • Rinse, dry and reassemble as directed
SideStream Plus (Philips Respironics) Frequency After each use Method • Disconnect the SideStream Plus from the tubing. Unscrew the top from the medicine cup, rinse out the nebuliser and refill with clean water. Reassemble, reconnect the tubing and run SideStream Plus again for a few seconds to flush out any drugs trapped in the nebuliser jets • Take apart the nebuliser again • Wash all items (except tubing) in hot soapy water • Rinse all parts in clean water • Shake off excess water and allow to air dry • Reassemble and store in a clean place Porta-neb Manual Control unit: Clean the inside and outside of the case with a damp cloth	Frequency Once a week Method • Disassemble the nebuliser • Boil the SideStream Plus parts (except tubing) in water with 2–3 drops of dish-washing liquid for 10 minutes • Rinse all parts in clean water for at least 2 minutes • Shake out excess water and allow to air dry • Reassemble and store in a clean place. • The SideStream Plus may also be washed once a week in the top rack of a dishwasher Caution: To reduce the risk of infection, clean and disinfect by boiling in hot water the nebuliser between treatments

Table 2.

Manufacturer’s instructions on how to wash and disinfect eFlow®rapid nebuliser system Type 178G1005.

Cleaning	Disinfection
Frequency The nebuliser handset and the aerosol head immediately after each use	Frequency The nebuliser handset parts and the aerosol head at least once a day
Method • Disassemble the nebuliser system • Place all nebuliser parts and aerosol head in warm tap water with a little washing-up liquid for approx. 5 minutes • If the parts are heavily soiled, move the nebuliser parts gently back and forth in the washing water • Rinse all nebuliser parts and the front and back of the aerosol head thoroughly under running tap water • Shake all parts to remove excess water • Place the individual parts on a dry, clean surface and allow to dry completely Control unit Clean the housing surface of the control unit and the cable as necessary with a clean, damp cloth	Method Commercially available disinfector • To ensure effective disinfection, use a thermal disinfector with an operating time of at least 6 minutes • Follow manufacturer’s instructions • Take the parts out of the disinfector immediately after completion of disinfection • Place them on a dry, clean surface and allow them to dry completely Boiling in distilled water • Boil in saucepan for at least 5 minutes • Remove parts and shake all parts to remove excess water • Place the individual parts on a dry, clean surface and allow to dry completely Chemical disinfection Bomix®plus (Bode) 2% solution for 5 minutes

(https://www.pari.com/fileadmin/user_upload/PARI.comINT/Documents/IFU/178D1007-Instructions-for-use-eFlow-rapid.pdf).

Results

In total, 24 parents of children with CF and 20 adults with CF completed this survey, the results of which are shown in Tables 3, 4 and 5 and Figure 1. Two nebulisers, namely the Porta-neb and eFlow, and associated drug chambers were used by both cohorts. The paediatric cohort primarily used the Porta-neb (62.5%), whereas the adults primarily used the eFlow (65.0%) (Table 3).

Table 3.

Nebulisers and drug chambers used by patients in this study.

	Paediatric (n = 24)	Adult (n = 20)
Type of compressor Porta-neb eFlow Both	62.5% (15) 12.5% (3) 25.0% (6)	5.0% (3) 65.0% (13) 20.0% (4)
Drug chambers used SideStream SideStream plus Ventstream eFlow Altera	62.5% (14) 16.7% (4) 8.3% (2) 33.3% (8) -	30.0% (6) 5.0% (1) 5.0% (1) 85.0% (17) 25.0% (5)

Table 4.

Details of at-home methods used for washing nebulisers.

	Paediatric (n = 24)	Adult (n = 20)
Frequency of washing: Each use Every two uses Daily Two or more times per week Weekly	95.8% (23) - - 4.2% (1) -	60.0% (12) 5.0% (1) 10.0% (2) 15.0% (3) 10.0% (2)
Water used: Boiling water Warm/hot water Luke-warm water Cold water Dishwasher Not specified	37.5% (9) 54.2% (13) 4.2% (1) 4.2% (1) - -	30.0% (6) 50.0% (10) 5.0% (1) 5.0% (1) 5.0% (1) 5.0% (1)
Detergent: Dishwashing Hand soap None	79.2% (19) 4.2% (1) 16.6% (4)	65.0% (13) - 35.0% (7)
Rinse: Yes Occasionally No	79.2% (21) 4.2% (1) 16.6% (2)	50.0% (10) - 50.0% (10)
Drying: Air dried Kitchen roll Tea towel Hairdryer Shake None, directly into steam disinfector Chemical disinfectant solution Not answered	41.7% (10) 25.0% (6) 4.2% (1) - 4.2% (1) 16.7% (4) 4.2% (1) 4.2% (1)	65.0% (13) 20.0% (4) - 15.0% (3) - - - -
Cleaning of compressor: Wipes Damp cloth/kitchen roll Bleach Never cleaned Not specified	45.8% (11) 8.3% (2) 16.7% (4) 20.8% (5) 8.3% (2)	30.0% (6) 10.0% (2) 5.0% (1) 25.0% (5) 30.0% (6)

Table 5.

Results from study detailing methods used for disinfecting nebulisers.

	Paediatric (n = 24)	Adult (n = 20)
Frequency of disinfection: At least once daily Two or three times/week Weekly Every 2 months Never Not sure	41.7% (10) 20.8% (5) 16.6% (4) - 20.8 % (5) -	- 5.0% (1) 30.0% (6) 5.0% (1) 50.0% (10) 10.0% (2)
Method of disinfection steam Boiling water Chemical disinfection Microwave (Sterisac) Unsure Never done	37.5% (9) 29.2% (7) 8.3% (2) 4.2% (1) - 20.8% (5)	- 35.0% (7) 5.0% (1) - 10.0% (2) 50.0% (10)
Rinse after disinfection: Yes No N/A	52.6% (10/19) 47.4% (9/19) -	25.0% (2/8) 62.5% (5/8) 12.5% (1/8)
Drying method post disinfection: air dried Kitchen roll Tea towel Dried but method not given Not dried, left in disinfector Hairdryer	47.4% (9/19) 21.1% (4/19) 5.3% (1/19) 5.3% (1/19) 15.8 (3/19) 5.3% (1/19)	75.0% (6/8) 12.5% (1/8) - - - 12.5% (1/8)
Storage after cleaning: plastic box Kitchen roll/tea towel/table Compressor Steam steriliser Soapy water eFlow bag Cupboard Milton disinfecting solution Not specified	58.3% (14) 12.5% (3) 4.2% (1) 12.5% (3) - - 4.2% (1) 4.2% (1) 4.2% (1)	15.0% (3) 25.0% (5) 15.0% (3) - 5.0% (1) 5.0% (1) - - 35.0% (7)

Figure 1.

Factors that promote cleaning of nebuliser devices.

Paediatric nebulisers were washed more frequently than adult nebulisers. Approximately one-third of both cohorts washed with boiling water and the majority used hot tap water (Table 4). Dishwashing detergent was commonly used in both paediatric and adult cohorts followed by rinsing then the preferred method of air drying (Table 4). In the paediatric cohort, immediately following washing 20.8% of individuals disinfected their nebulisers either by steam or chemical disinfection (Table 4). Antibacterial wipes were commonly used to clean the compressor itself on an ad hoc basis; however, 20–25% of individuals reported it was never cleaned.

The frequency of disinfection varied. Adults were more likely to report never decontaminating their nebulisers (50%) than parents (21%). In addition, none of the adults disinfected their nebuliser daily (Table 5). The preferred methods of disinfection are shown in Table 2. Both cohorts, paediatric (70.8%) and adult (100%), believed that their nebuliser hygiene practices were effective with infection prevention and cleanliness being the primary reasons given for cleaning the nebulisers (Figure 1). Adult patients indicated they would clean their nebulisers more frequently if the device became blocked due to drug residue, e.g., with salt crystals when nebulising hypertonic saline or when nebulising Bramitob, which was reported as being “sticky”.

In the paediatric cohort, the majority of parents (19/24, 79.2%) stated that nothing would prevent them from cleaning their child’s nebuliser with the remaining indicating holidays (n=2), sleepovers (n=1) and time (n=2) as preventative reasons. In the adult cohort 14/20 (70%) stated that nothing would prevent them from cleaning their nebulisers with the remaining indicating time (n=2), illness (n=2) and laziness (n=2) as preventative reasons. The majority of the paediatric cohort, 23/24 (95.8%), reported being given instructions on cleaning when their nebuliser was issued; however, this was the case in only 10.5% of the adult cohort, with 42.1% (8/19) stating they had never received instructions.

Discussion

This survey originated from a need to establish the current behaviour in relation to cleaning and disinfecting nebulisers in a paediatric and adult CF population. In this study, when patient practices were compared against the broad key principles of manufacturers’ guidelines, namely (i) frequency of washing, (ii), frequency/method of disinfection, (iii) whether a rinse step is involved in washing and disinfection and (iv) nebuliser drying method, several important deviations from manufacturers’ guidelines were noted. All manufacturers advise that nebulisers are washed after each use. In general, there was good compliance of patient practice against this particular guideline with the paediatric cohort (96%). In contrast, only 60% of adults met this standard. Likewise, as the majority of adult patients use an eFlow nebuliser, which the manufacturer recommends is disinfected daily, none of the adults reported performing disinfection on a daily basis. Furthermore, it is worrying to note that 50% of adults never disinfect their nebuliser, irrespective of nebuliser type and manufacturers’ guidance. Regarding rinsing after washing, although the universal manufacturers’ guidance is to rinse after washing, there was good compliance with the paediatric cohort (79%), but again poor compliance with the adult cohort (50%). Regarding rinsing after disinfection, of paediatric and adult respondents who reported active disinfection, 53% and 25% respectively reported rinsing their nebuliser. Regarding the drying of nebulisers, all manufacturers advocate air drying nebulisers after washing. Our survey reported that 42% of paediatric respondents did air dry their device, whereas 65% of adults reported air drying their device. However, not everyone used passive air drying as their method, but attempted to proactively dry the device in some manner, i.e., active drying with a kitchen roll/tea towel or hair dryer. Of the paediatric cohort (n=5) who did not dry their device, the device was immediately disinfected, thus negating the need for drying after washing.

This baseline measure of current practice revealed that a high proportion of adult CF patients (50%) and 20% of paediatric carers never disinfect their nebuliser, with none of the adults disinfecting it daily and only 42% of parents of children with CF doing so. These findings concur with a national study in Belgium, which reported that although the majority of patients cleaned their nebulisers on a daily basis, only 57% performed disinfection daily (Reychler et al, 2009).

Additionally, the current finding that many individuals rinsed their nebulisers following disinfection is of concern as some patients indicated the use of tap water as opposed to boiled or sterile water, thereby potentially introducing contaminating pathogenic micro-organisms to their washed and disinfected nebulisers. The expectation of disinfection would be to produce a device that is free from pathogenic micro-organisms, therefore it is important to protect the integrity of this disinfection stage by not employing a practice that would lead to any recontamination. Interestingly, there is confusion with patients as to what type of water manufacturers recommend is used after washing and disinfection. Also, the availability of certain water types, i.e. “sterile” and “distilled” water leads to issues of availability and access. Some manufacturers recommend “running” water, indicating tap water, whereas others recommend “clean” water, which could be interpreted as tap water. Tap water should not be advocated for the final rinse as it can be contaminated with Pseudomonas aeruginosa and as such CF patients may re-contaminate their nebuliser after having successfully washed and disinfected it (Caskey et al, 2018). Furthermore, active drying following disinfection is not advocated as this may also be a source of recontamination (Hohenwarter et al, 2016) and, in the case of mesh nebulisers, filaments from drying cloths or tissue may lodge in the mesh pores.

Mesh nebulisers, e.g., eFlow®rapid (PARI), which work on the principle of delivering nebulised drugs with vibratory movements through a mesh, have become popular with the therapeutic delivery of CF drugs in the home, resulting in reduced nebulisation time, higher deposition in the lung and ease of use, contributing to patient preference and improved adherence (Rottier et al, 2009; Daniels et al, 2013; Moore and Mastoridis, 2017). It has been reported that cleaning and maintenance have a substantial preventative effect on mesh pollution, i.e., clogging of orifices that may otherwise result in increased nebulisation times, e.g., 6.7 min to 9.8 min over a 6-month period (Rottier et al, 2009). Once the mesh has been polluted, commencement of cleaning is ineffective and as such regular cleaning and disinfection should be encouraged to ensure the initiation of such pollution is minimised. Pollution has also been attributed to the practice of nebulising a variety of drugs in the same nebuliser. In an attempt to address this issue PARI has developed an “easycare” device, which is a hinged cleaning aid with a built-in funnel opening and receptacle in which the pores in the membrane of the aerosol head are mechanically rinsed free by backwashing in the opposite direction with a solution of isotonic sodium chloride solution (0.9% w/v), distilled water and deionized purified water (PARI Medical Holding GmbH (DE), 2016). This procedure must be performed after routine cleaning of nebuliser parts and the aerosol head; once completed, all nebuliser parts including the aerosol head must be disinfected. The inclusion of this extra cleaning procedure further contributes to the burden of treatment. Interestingly, many patients in a French study were unaware of availability of this procedure (Baravalle-Einaudi et al, 2017).

Many CF organisations worldwide including the CF Foundation (Saiman et al, 2014) and manufacturers offer varied and, at times, conflicting advice regarding nebuliser care and hygiene, which may result in confusion for the CF patient. According to the 2001 European Respiratory Society Guidelines on the use of nebulisers, it was suggested that “manufacturers should undertake appropriate tests and trials to permit the production of evidence-based instructions” (Boe et al, 2001), which to date have not been published. It is interesting to note that in accordance with our findings, it has been reported that not all patients undertake correct nebuliser maintenance in accordance with manufacturers’ instructions, particularly in relation to the vibrating mesh nebuliser procedure (Baravalle-Einaudi et al, 2017). From this study, it is evident that patients have adopted differing hygienic practices regarding their nebulisers, which were primarily influenced by instruction at the time of issue, with only a single adult patient out of both cohorts referring to the manufacturers’ instructions. Of the 11 adult patients who stated that they had previously received instructions regarding nebuliser cleaning and disinfection, six recalled that this guidance was given while they were under paediatric care. In most cases, these adult patients referred to guidance given historically. Therefore, one learning point from the current study is that nebuliser cleaning and disinfection guidance needs to be reinforced at time of transition between paediatric and adult care. In addition, there may be occasions when such instruction was given to parents of very young patients and not to the patient because of their young age. On two occasions, young patients stated that their nebuliser care was undertaken by their mother and they were unsure of how to perform such procedures themselves. This adds further emphasis on ensuring the transition to adult care allows for the young transitioning patient to be fully informed to ensure they take optimal ownership of their nebuliser care and maintenance.

Robust, practical and universal cleaning and disinfection instructions are required. Notably, recent studies have concluded that steam disinfection with the aid of “baby bottle steam sterilisers” is such an approach as it is a simple, effective and not time consuming (Towle et al, 2013, 2014; Hohenwarter et al, 2016). It must be noted that such “steam sterilisers” are not sterilisers but disinfectors and, as such, do not kill spore-forming organisms (Towle et al, 2016). Of further note is that disinfection protocols should be used subsequently to the cleaning of nebuliser parts, which is required to remove drug and sputa residue. Additionally, maintenance of mesh nebulisers should detail not only optimum cleaning and disinfection procedures but also ensure the methods advocated are permissible in terms of ensuring the mesh does not get damaged due to the use of corrosive chemical disinfection, e.g., hypochlorite or by inappropriate microwave steam sterilisation procedures (Baravalle-Einaudi et al, 2017).

The limitations of many studies published to date should be noted, namely that the effectiveness of disinfection methods has been concluded from laboratory-based studies, where nebuliser parts have been artificially contaminated with known numbers of micro-organisms. This is in contrast to real-life scenarios, where patients’ nebulisers may potentially be contaminated with micro-organisms existing in biofilms. Studies employing artificially contaminated devices may not be as comparable to naturally contaminated devices, thus evidence originating in such studies needs to be interpreted with care. Ultimately, studies yielding evidence that is incorporated into evidence-based guidelines should emulate the real-world scenario as much as possible.

The majority of paediatric carers and adult patients believed their nebuliser hygiene practices to be effective with no suggestion or awareness of the possible inadequacy. As such, these findings demonstrate a notable gap between current practice and best practice in relation to nebuliser hygiene, which may in turn adversely affect the health of CF patients. Patients need to be able to make informed decisions on their healthcare, therefore clear guidance on cleaning and disinfecting their nebulisers is of utmost importance. It must be acknowledged, however, that there are difficulties in adhering to a hygiene regime when both patients and the CF-MDT are subjected to varied and conflicting advice. The availability of standardised evidence-based manufacturers’ instructions should therefore be promoted, which could encourage patients to engage in cleaning and disinfecting practices in relation to their nebulisers. Adherence is difficult to measure when we are unclear of what the expected behaviour is that we should be observing. As a result of our study findings, additional patient-healthcare professional co-production initiatives are being developed. This involves the development of evidence-based best practice advice to be disseminated by leaflets and videos amongst the patient CF community both at charity events and during attendance at CF clinics and home visits.

Overall, this study is important as it highlights that patients may not be adhering to the limited manufacturers’ guidance that is currently available. However, it should be noted that there are certain limitations relating to this study, namely this was a two-centred study, involving a relatively small population size. Larger studies involving many CF centres would have allowed comparison between centres in geographically different regions and studies involving direct observation of patients during washing/disinfection practices would have removed interview bias.

In conclusion, CF is a lifelong condition and as such the practice of key basic skills such as cleaning and disinfecting nebulisers should not be assumed but actively reviewed periodically by the CF-MDT to ensure adequate knowledge and understanding. It is vital the CF community is updated on recent findings in relation to these issues at key times such as transition and at annual review. Consistent with the philosophy of this research is the idea of getting the basics right. Thus, by empowering people to engage in nebuliser hygiene, we are attempting to delay or prevent further complications that increase the treatment burden to people with CF.