CONCERNS FOR PEOPLE WITH CYSTIC FIBROSIS (PWCF) WHEN TRAVELLING PRE COVID-19.

Editor,

There are currently over 300 adult and 200 paediatric persons with cystic fibrosis (PwCF) in Northern Ireland, who attend the regional CF centres at the Belfast City Hospital and the Royal Belfast Hospital for Sick Children, respectively. A combination of a high burden of daily treatment combined with moderate to high disease severity has made travelling difficult or impractical for many PwCF, although travelling is becoming more popular for young adults with CF, who have relatively stable disease. For some PwCF, the prospect of travelling to unknown destinations may generate fear of acquiring a new respiratory infection and other concerns, as depicted in the artwork of the Front Cover of this issue of the journal, however advances in therapies including transmembrane conductance regulator (CFTR) modulators, have enabled many PwCF to consider and embark upon travel to various global destinations.1

The front cover of this issue of the Journal depicts “Travelling with cystic fibrosis”, A person with cystic fibrosis’ perspective of travelling with CF - Caroline Anne Moreland 2019 (with permission). The picture shows a collection of cats, which represent PwCF. The artist describes them “as odd and outside of normal society, hence the moustaches and eye-patches. Like most people, they have a desire to travel and explore the world but are limited by the issues which are represented by the thorns of the rose bush. The roses themselves are a gesture to “65 roses”, the phrase used to help children pronounce “cystic fibrosis”. So rather than travel, they stay at home because of restrictions, represented by the zipper.”

Through a service improvement project, we examined travel-related difficulties and concerns experienced by our local CF adult population, so that we are able to further support PwCF health literacy needs, when preparing, during and post travel. To establish this baseline, we designed a self-completing, voluntary, anonymous questionnaire amongst adult PwCF attending outpatient CF clinics during the summer of 2019 (pre-COVID), to gain an insight into (i) specific concerns when travelling and (ii) the availability of online travel advice/resource from UK CF Centres and other sources. Free text questions were used to collect respondent demographics and responses to the questions asked. The survey was conducted using the guiding code of ethics and practices established by the American Association of Public Opinion Research (AAPOR) (www.aapor.org). In addition, a Virtual Focus Group was held to explore the PwCF experience when travelling. The availability of published online travel guidance from CF Centres (n=25) and CF charities were also examined.

There were 68 respondents to the survey, including 31 males, 33 females and 4 respondents who did not enter their gender. Respondents who declared their age (n=63) ranged from 17 to 71 years (median = 30 years; 94% of respondents in age range 20-39 years). PwCF cited nine concerns when travelling, as listed in Table 1. The most frequently cited concern was access to affordable travel insurance and confusion with providers of such cover. Previously, it has been reported that 18% of PwCF have travelled with no insurance and 23% have travelled with insurance which did not cover CF.2 Another common concern was the need to maintain an effective cold chain for temperature-sensitive medications, particularly DNAse, as a mucolytic agent to aid airway clearance. One PwCF was concerned about their PEG when swimming and attempted to mitigate any potential contamination from water, by applying a waterproof dressing to the PEG. Another PwCF reported damage of liquid nutrition packs during transit, negatively impacting on their holiday experience and as such has resulted in avoidance of future air travel.

Table 1

Concerns raised by people with cystic fibrosis (PwCF) realting to travel

Analysis of online travel resources for PwCF showed that 9/25 CF centres in the UK offered varied freely available online travel advice, although there was no detailed advice on the cleaning of the nebuliser when travelling. The UK CF Trust offers valuable travel advice3 but refers PwCF back to their NHS CF healthcare team, for specific individual clinical guidance. Further valuable resources are available to address the needs of PwCF when travelling, including the British Thoracic Society’s (BTS) guidelines on air travel in people with respiratory disease4,5 and the European Cystic Fibrosis Society (ECFS) recommendations on travelling with CF.6

The current policy in most CF centres is that PwCF are encouraged to discuss with their CF healthcare team at clinic, their potential travel plans well in advance of the actual proposed date of travel. Such travel plans may include requirements/aspirations, for work/leisure/family and further details are sought, including intended destination, the number of hours flying required, the anticipated time away from home, facilities at their intended destination, etc. These are reconciled in conjunction with their clinical status and where appropriate, discussed with the CF multidisciplinary team, so that the PwCF is well informed and prepared for travel, when/where travel is a safe option.

In contrast, it is interesting to note differences in the perceptions of PwCF and those of healthcare professionals, in relation to travel. Previously, Hirche and colleagues5 listed recommendations from the healthcare professional’s viewpoint in an evidence-based manner, as detailed in Table 2. When the concerns of the PwCF are compared to the recommendations from the healthcare professional, complaints about in-flight coughing, acquiring an infection on the flight, another unknown PwCF on the flight and issues at security, were unique to the PwCF. Healthcare professionals listed an additional 14 CF travel-related considerations. This comparison highlights an important chasm between PwCF and the healthcare team and emphasises the need for good communication, to improve health literacy amongst PwCF and improve patient safety when travelling.

Table 2

Checklist for the CF-healthcare team relating to considerations for people with cystic fibrosis when travelling (adapted from5)

Travel recommendations to PwCF should be a syntheses of the concerns articulated by the PwCF, as well as recommendations from the CF healthcare team. In order to support these, we have prepared a new and novel short animation entitled “All aboard – Travel Recommendations with Cystic Fibrosis”7 to help guide PwCF considering travelling.

The arrival of SARS CoV-2 in early 2020 and post-BREXIT arrangements have further transformed and confounded the travel landscape for PwCF. CF multidisciplinary teams should be aware of these patient-articulated factors that may still limit travel opportunities for those patients who are clinically fit-to-travel and should attempt to engage with the relevant stakeholders through enhanced communication to help facilitate travel arrangements for PwCF.