Abstract
BACKGROUND: Nebulized therapies form an important component of treatment in people with cystic fibrosis (CF). It is important for people with CF to continue to take their nebulized medications when traveling.
METHODS: A self-completing anonymous questionnaire was developed, as part of a quality improvement project, to support people with CF educational needs when traveling. The questionnaire was prepared to gain an insight into (1) adherence to nebulized therapies when traveling and (2) nebulizer cleaning and disinfection practices while traveling. Polar questions (yes/no response) were mainly employed as well as free text and closed questions.
RESULTS: There were 68 respondents to the survey, including 31 males, 33 females, and 4 respondents who did not enter their sex. Respondents who declared their age (n = 63) ranged from 17–71 y (median = 30 y; with 94% of respondents in age range 20–39 y). When traveling, 38% (25/66) of respondents indicated that nebulized therapy was not performed during travel. The most common method of nebulizer maintenance while traveling was washing with soap and water (43%), followed by boiling water (18%), as well as the employment of 5 other methods of nebulizer maintenance. Some respondents (2%) indicated that they did not perform any method of nebulizer maintenance while traveling until they returned home.
CONCLUSIONS: This study identified that nebulizer care and hygiene are less than optimal when traveling as well as identifying a worrying trend of taking a “nebulizer vacation.” People with CF need to be aware of risks to their health in being nonadherent with their nebulized medication(s) while traveling as well as risks of acquiring a new pathogen through suboptimal cleaning/disinfection/drying management of their nebulizer. CF multidisciplinary teams should emphasize the importance of sustaining nebulized treatments when traveling and practicing effective nebulizer washing, disinfection, and drying procedures.
Introduction
Cystic fibrosis (CF) is an autosomal recessive disease of mainly white populations of European ancestry, which is exacerbated by a continuous cycle of respiratory inflammation and lung infection, which may become chronic, leading to increasing disease severity.1 For a seminal review of the pathophysiology of the disease, please see Shteinberg and colleagues.1 The production of thick, viscous sputum as a result in the physiological problems of transporting chloride ions allows for the entrapment of environmental bacteria and fungal organisms, which may eventually lead to chronic colonization and infection. Therefore, people with CF are vulnerable to acquiring new environmental organisms in the lower respiratory tract. While at home in familiar surroundings, people with CF can manage such risks better than when faced with new environments with geoclimatic variation favoring a range of new pathogens, eg, Burkholderia pseudomallei in Southeast Asia/Northern Australia, uncertainty of the quality of local potable water supplies, and lack of domestic facilities to support proper hygiene of nebulizers and other medical devices.2,3
New treatments, particularly the CF transmembrane conductance regulator (CFTR) modulators, have led to a dramatic and rapid improvement in CF-related symptoms in patients taking this oral medication, which have positively resulted in increased life expectancy, improved quality of life, and more stable CF disease.4 With increased demand for travel within the CF community, CF teams are revisiting previously published evidence-led travel advice to help guide their patients safely. Recommendations relating to travel have been previously outlined, with the responsibility of individual health care teams to offer advice to people with CF prior to travel outside of their home country.5
Health risks associated with traveling and travel destination activities, current severity of health, travel insurance requirements, and transport of medical devices and medications are important considerations for people with CF contemplating travel.4 A travel survey from 2017 among people with CF reported on several aspects of travel, namely the geographical extent of travel, where 96% of respondents (n = 100) had traveled abroad, with 14% now limiting their travel based on medical advice.4 The survey reported that undertaking adventurous activities while traveling was common and that lack of adequate insurance coverage while traveling was a concern, with 18% traveling with no insurance and 23% traveling with insurance that did not cover CF. The survey probed the types of illness people with CF experienced when traveling abroad, which included 10% had a CF-related illness (7 chest infections, 2 dehydration, one pancreatitis) and 12% experienced a non-CF–related illness (4 sunburn, 3 gastroenteritis, 3 ear infections, one fall, one gastro-oesophageal reflux).4 The survey listed 10 important issues for people for CF undertaking travel abroad, in cluding (1) stage, severity, and stability of lung disease; (2) optimization of health pretravel; (3) provision of detailed medical report and certificates; (4) travel insurance for health care abroad; (5) destination factors (temperature, humidity, altitude); (6) availability of specialist CF care at destination; (7) medications, treatment, and equipment to be taken abroad; (8) recreational activities planned; (9) salt supplements and avoidance of dehydration; and (10) specific individual advice for destination and activities planned.
Given that this survey originated in an adult CF center in the North of England, similar to that in Belfast, we wished to probe other areas relating to nebulizer hygiene not covered in the previous survey, to be complementary to the existing data, as such data would be relevant and of interest to CF centers globally.
Nebulizer cleaning, disinfection, and drying are extremely important in CF in order to maintain an optimally functioning device that is safe and does not pose an infection risk. Maintenance of the CF nebulizer involves the separate processes of cleaning, disinfecting, and drying after each use and thus adds a considerable treatment burden to an already heavy treatment burden, including airway clearance.2 There have been several reports that CF nebulizers can become contaminated with a diverse variety of microorganisms, some of which are environmental organisms, but, more importantly, several that are of clinical importance, including Achromobacter xylosoxidans, Burkholderia cepacia complex, Pseudomonas aeruginosa, Stenotrophomonas maltophilia, and Staphylococcus aureus.2,6-8 In addition, nebulizers have been shown to be contaminated with fungi.9 As a result, several recommendations are available to help guide in the hygienic maintenance of these devices in terms of cleaning, disinfection, and drying procedures.2,10,11
In our CF center, we advocate that people with CF maintain their nebulizer in line with the care and hygiene recommendation as detailed by the manufacturer of the device as well as following the publication of recent recommendations for nebulizer cleaning and disinfection.2
To date, there have been no reports on the adherence of people with CF relating to their nebulized therapies when on vacation or how they manage and maintain their nebulizer while away from home in terms of preventing acquisition of new respiratory infections due to nebulizer hygiene practices. The aims of our project were, therefore, to (1) examine people with CF adherence to nebulized therapies, (2) examine nebulizer hygiene practices among people with CF when traveling, and (3) to develop an educational animated video to support the educational needs of people with CF when traveling.
QUICK LOOK
Current Knowledge
Travel away from home presents challenges for people with cystic fibrosis (CF) in terms of adherence to nebulized treatments and with cleaning/disinfection of the nebulizer when traveling. The introduction of CF transmembrane conductance regulator modulators has positively transformed the well-being of people with CF, resulting in many wishing to travel. Nebulized care is an important component of daily CF therapy, with cleaning, disinfecting, and drying of the nebulizer remaining just as essential when traveling as at home.
What This Paper Contributes to Our Knowledge
Respondents identified that nebulizer care and hygiene were not optimal when traveling as well as people with CF taking a “nebulizer vacation” during travel. Respiratory health care professionals should emphasize the importance of continuation of therapy when traveling as well as maintaining the nebulizers in a safe manner to prevent acquisition of new respiratory pathogens.
Methods
Questionnaire
A self-completing anonymous questionnaire was developed, as part of a quality improvement project, to support people with CF educational needs when traveling (see related supplementary materials at http://www.rcjournal.com). The questionnaire was prepared to gain an insight into (1) adherence of people with CF to nebulized therapies when traveling; and (2) nebulizer cleaning, disinfecting, and drying practices of people with CF while on vacation. The questionnaire consisted of 8 questions, including 2 demographic questions, one question exploring nebulized treatment adherence, 3 questions relating to nebulizer hygiene practices, and 2 general questions. Polar questions (yes/no response) were mainly employed as well as free text and closed questions. These question types were employed to collect respondent demographics and responses to the questions asked. Results were reported as a frequency and percentage. Formal ethical approval was not required from a UK National Health Service (NHS) research ethics committee as the questionnaire and survey were not considered research as defined by the United Kingdom Policy Framework for Health and Social Care Research. However, the survey was conducted using the guiding code of ethics and practices established by the American Association for Public Opinion Research (AAPOR), which addresses ethical considerations for survey researchers. The questionnaire was purposely designed to avoid any potential for identification of individual respondents and so excluded name, date of birth, address, and any other potential identifiers.
Survey
In accordance with the AAPOR code of ethics and practices, respondents were made aware that the survey was entirely voluntary, non-incentivizing, and anonymous. None of the questions were mandatory, and respondents could voluntarily choose not to answer any questions and could withdraw from the survey at any time without consequence. Hard copies of the questionnaire were distributed to people with CF attending CF out-patient clinics at the Northern Ireland Regional Adult Cystic Fibrosis Center at Belfast City Hospital during the summer months (July–August) of 2019. The need for educational messaging/resources via a suitable communications modality was examined and developed to address the findings from the survey.
Results
Respondent Demographics
Response rate of the survey was 91.9% of adult CF clinic attendees (68/74). Six attendees did not wish to undertake the survey either (1) because they were not interested in traveling or (2) they were simply not interested in completing the survey. In total, there were 68 respondents to the survey, including 31 males, 33 females, and 4 respondents who did not enter their sex. Respondents who declared their age (n = 63) ranged from 17–71 y (median = 30 y; with 94% of respondents in age range 20–39 y).
Adherence to Nebulized Medication While Traveling
While traveling, 38% (25/66) of respondents indicated that nebulized therapy was not performed during travel. Of those respondents who continued their nebulized medication while traveling, all of those who responded brought their portable nebulizer with them while traveling. Related comments from our survey respondents articulated that nebulized therapies are not performed while traveling (1) unless the person is ill; (2) the person disliked carrying their nebulizer through airports; (3) age related; stopping prescribed nebulizer therapies while traveling was more likely when the person was younger; and (4) geography related; nonadherence if international travel was involved. One respondent indicated that they do not travel abroad, but it was unclear if travel was curtailed due to the heavy treatment burden or not.
Methods of Nebulizer Washing, Disinfecting, and Drying When Traveling
The most common method of nebulizer maintenance when traveling was washing with soap and water (43%), followed by boiling water (18%), as well as the employment of 5 other methods of nebulizer maintenance (Fig. 1). Methods of nebulizer drying included air drying (84%) and drying with a towel (8%). Other less frequently employed methods of drying (8%) included drying with a hair dryer, shaking the nebulizer, storing in a bag, and drying with a paper towel.
Nebulizer cleaning methods employed when traveling.
A small proportion (9%, 6/68) of respondents was unaware that an electrical outlet adapter was required when traveling to countries with different electrical plug/socket configurations.
A small proportion of study respondents (2%) proactively did not perform any hygienic maintenance on their nebulizer when traveling for fear of acquiring a pathogen during the cleaning and disinfection process but rather elected to clean and disinfect their device on return home.
Development of Travel-Related Educational Video Animation
Analyses of the study survey findings highlighted deficits in practices relating to adherence to nebulized medications as well as to nebulizer hygiene, which we wished to address by the production of video animation for people with CF. Therefore, a short 1 min 46 s animation was produced in draft form, which was circulated to health care staff and to a people with CF focus group within our CF center. Respondents were asked to qualitatively comment on the video in terms of its suitability and health messaging. A finalized video animation was produced entitled All Aboard – Travel Recommendations With Cystic Fibrosis and is hosted on YouTube (https://youtu.be/-HZ0OdlQyws Accessed May 10, 2022). This animation includes reference to nebulizer care and hygiene as well as covering general points about CF-related travel.
Discussion
The recent arrival of licensed and reimbursed CFTR modulators in the United Kingdom for the treatment of CF disease, namely ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor, has made a profound impact on the manifestations of CF disease, with major improvements in the clinical status of the disease as well as improvements in quality of life. A recent report of patient perspectives from 101 people with CF following initiation of elexacaftor/tezacaftor/ivacaftor described a rapid positive impact on respiratory symptoms, sleep quality, general well-being and physical self-esteem, and a reduction in overall treatment burden.12 People with CF have experienced renewed and unexpected physical strength leading to greater self-confidence, autonomy, and long-term planning after treatment initiation, including the formulation of new life goals.12 One of these new goals described was travel. Therefore, we anticipate a significant rise in travel internationally in those young adults who have stable CF and where travel would not pose as significant a risk to their health as compared to times pre-CFTR modulator therapy.
One issue that CF health care professionals are already concerned with patients feeling the benefit with the new CFTR modulators is adherence to medication and treatments, especially those treatments that carry a heavy burden, such as nebulized therapies. Our survey showed that 38% of adults already indicated that nebulized therapy was not performed during travel. This may be due to the psychological wish to forget CF and their disease for the short period of time that they are traveling and rather focus on the positive excitement that vacations generate. Whereas our survey did not further probe the rationale for discontinuing nebulized therapies while away from home, we anticipate these reasons may include (1) the amount of quality vacation time required to be dedicated to these therapies, (2) the inconvenience of carrying a bulky nebulizer and compressor or controller through airports and on flights with ever-diminishing baggage allowances, and (3) the self-perceived marginal benefit that nebulizer therapies can add to the already stable patient. People with CF need to be aware of the importance of continuing their prescribed nebulized therapies, while at home or during travel, even if they are stable and feeling well due to improved CFTR therapies, until further clinical evidence and research shows discontinuation of this critical aspect of their care is safe. We hypothesize for those individuals currently taking prescribed nebulized anti-pseudomonal antibiotics that continuation of this therapy while away may additionally and inadvertently act as a process of prophylaxis to prevent the acquisition of new local environmental strains of P. aeruginosa and other environmental pathogens from water and water-related activities.
Nebulized therapies also suffer disproportionally from a high treatment burden given (1) the time involved for nebulization of medicines; (2) the inconvenience of carrying the nebulizer and compressor/controller units; and (3) the need to regularly clean, disinfect, dry, and maintain such equipment while at home and away. When asked about nebulizer maintenance when abroad, most respondents employed soap and water to wash the nebulizer when away from home. Whereas it is encouraging that patients attempted some form of nebulizer cleaning while away, they need to be counseled regarding the microbiological risks of using local water of uncertain potability for the purposes of nebulizer washing and rinsing, which may be contaminated with pathogens, including P. aeruginosa. Furthermore, it is important to emphasize to those people with CF who only wash their nebulizer the importance of performing a disinfection and drying process after washing to help minimize microbiological risks from using water. Water may be an important source of this Gram-negative pathogen.13 Likewise, people with CF should avoid exposure to other high-risk water-related environments, including Jacuzzi, spas, and hot tubs, while on vacation, where there may be an enhanced opportunity to visit such recreational facilities.14 While abroad, many local water treatment specifications may differ from those defined at home; so people with CF need to be aware of this and try to incorporate disinfection with steam, as detailed in recent CF nebulizer hygiene recommendations2 or with boiling water, where access to steam disinfection is not available. Other disinfection options approved by the CF Foundation, including 70% isopropyl alcohol and hydrogen peroxide soaks followed by sterile water rinse, have been published.10,11 Such alternatives as these may be considered to allow families to choose whatever disinfection option works best for their individual situation while traveling. However, where possible, we always advocate the use of heat in the form of steam or boiling water as the optimum method to disinfect nebulizer equipment.2 The development of alternative drug formulations and delivery systems, such as dry powder inhalers for the delivery of antibiotics to the lung, offers alternatives to nebulized delivery that are convenient and easy to clean that may be more pragmatic when traveling.
When compared to how people with CF maintain their nebulizer at home when not traveling, a recent study at the same adult CF center showed that 65% of adults with CF washed their nebulizer with dish soap, followed by boiling water (30%) compared to 43% and 18%, respectively, while traveling.15
The main method of drying the nebulizer after washing and disinfection was to allow it to dry passively in air. While traveling to unfamiliar destinations, care needs to be given to placing the cleaned nebulizer in a location that avoids sinks and bathrooms where it may become recontaminated from splashes and surfaces contamination. There is also the potential for people with CF to place the nebulizer outside in warm destination climates to speed up the drying process, but recent evidence from South America has shown that drying outdoors increased the chance of contamination (odds ratio 4.87 [95% CI 1.10–21.61], P = .038) when compared with drying with a cloth, paper towel, fan/dryer, or the compressor/compressed air.16 Furthermore, it is important to educate the traveler on the importance of effective drying of the nebulizer after washing and disinfection. A recent study demonstrated that nebulizer drying, if applied properly, had the ability to reduce counts of P. aeruginosa to nondetectable levels. Equally, this study showed that if the device was not dried thoroughly and moisture remained then the device was able to support the survival of P. aeruginosa at high numbers, which would constitute an infection risk to the traveler with CF.17 Additionally, certain bacterial CF pathogens, including S. aureus and Mycobacterium abscessus, have been shown to survive drying; therefore, it is important to ensure the effectiveness of the primary disinfection process.18,19
Those people with CF traveling to countries where mains voltage is not 220–240 V AC, particularly to North America and the Caribbean, where mains voltage is 110 V AC, should be aware that this change of voltage can have an important negative effect on nebulizer delivery performance20 as well as with the thermal performance of baby bottle disinfectors employed in nebulizer disinfection.21 Therefore, the use of an electrical voltage outlet adapter can be a critical piece of equipment to include when traveling.
Following completion of the questionnaire and survey, we produced a short educational video animation about travel with CF, which was critically appraised by a focus group of adult people with CF. This animation was designed to holistically cover all aspects of CF travel and included guidance for people with CF on nebulizer care and hygiene when traveling. Links to additional educational materials for nebulizer care can be found in the supplementary material (see related supplementary materials at http://www.rcjournal.com).
Study Limitations
This study was from a single adult CF center and performed during peak travel season. It would be useful to repeat the study in several CF centers from more than one country as well as comparing the responses from adult persons with CF with those from parents of children with CF. Additionally, the current study was performed prior to the onset of COVID-19; and therefore, it would be valuable to repeat the study now, approximately 2 years into the COVID-19 pandemic, in order to estimate the influence of this pandemic on nebulizer adherence and hygiene practices.
No formal survey validation analyses were performed due to (1) a relatively small survey population size and (2) difficulty in reassessment due to the anonymous nature of the survey and questionnaire. The questionnaire did not investigate types of inhaled medications, nebulizer/compressor type, or airway clearance therapy, which may be useful to compare in future studies. Adherence to therapy was only for travel and may not reflect an individual’s overall adherence to therapy. Adherence to nebulized therapies in CF continues to be a major challenge. Therefore, there is a need to pursue research of more efficient means of nebulization/inhaled medications to encourage adherence on a daily basis but also while traveling.
Presently, there are no licensed vaccines available for the major etiological agents of bacterial and fungal infections in people with CF, including P. aeruginosa, S. aureus, B. cepacia complex, the nontuberculous mycobacteria, as well as Aspergillus. This, therefore, places greater emphasis on the importance of continuation of prescribed nebulized therapies in CF while traveling abroad. People with CF should be vigilant against new potential sources of infection when traveling, particularly in environments that may harbor CF respiratory pathogens and that may contaminate their nebulizer. Nebulizers should be rigorously cleaned, disinfected, and then dried thoroughly according to approved methods. People with CF should seek the clinical advice and guidance from their CF multidisciplinary health care team about their travel plans so that appropriate, proportionate, and evidence-based guidance is given to help mitigate the risk of acquiring a respiratory pathogen directly from the environment to keep themselves and their nebulizers safe when traveling.
Conclusions
This study identified that nebulizer care and hygiene are less than optimal while traveling as well as a worrying trend of people with CF taking a nebulizer vacation. Specifically, the study highlighted the wide variation in nebulizer washing, disinfecting, and drying procedures practiced by survey respondents while traveling. A significant number of respondents admitted not performing nebulizer therapies while traveling. The main method of nebulizer drying while traveling was by passive air.
People with CF need to be aware of risks to their health in being nonadherent with their nebulized medication(s) while traveling as well as risks of acquiring a new pathogen through suboptimal cleaning/disinfecting/drying management of their nebulizer. CF multidisciplinary teams should emphasize the importance of sustaining nebulized treatments when traveling and practicing effective nebulizer washing, disinfecting, and drying procedures.
Acknowledgments
We wish to acknowledge with thanks all those persons with CF who kindly took the time to complete the questionnaire.
Footnotes
- Correspondence: Beverley C Millar PhD, Northern Ireland Public Health Laboratory, Belfast City Hospital, Lisburn Road, Belfast, Northern Ireland, BT9 7AD United Kingdom. E-mail: bcmillar{at}niphl.dnet.co.uk
The authors have disclosed no conflicts of interests.
These data were presented by Professor Beverley Cherie Millar at the 44th European Cystic Fibrosis Society Conference, held virtually June 9–12, 2021.
This project was a product of the Cystic Fibrosis Study Buddies Program and supported by charitable grants from Vertex Pharmaceuticals (CG-2017-106614 and CG-2015-104576). Vertex Pharmaceuticals did not play any role in project conceptualization, design, execution, analysis, nor any editorial role in manuscript writing or approval.
This study was performed at Belfast City Hospital, Belfast, Northern Ireland, United Kingdom.
Supplementary material related to this paper is available at http://www.rcjournal.com.
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