The COVID-19 pandemic has created a global public health emergency, and public health restrictions have been in place in most countries [16]. This study was designed to assess the impact of COVID-19 and associated restrictions on PWCF. Our study showed that a large proportion of PWCF deferred their hospital visits. The major reason for deferral was fear of contracting COVID-19, and in a small number of cases, the hospital CF unit was closed. This deferral rate among those aged < 35 years was almost twice than in those ≥ 35 years. However, no such pattern was highlighted during this study with respect to gender and location of the PWCF. Deferrals also impacted rehabilitation therapies, medical care at the hospital, and appointments with GP. A non-significantly greater proportion of respondents aged ≥ 35 years had to put off their diagnostic tests as compared to those aged < 35 years. Deferral of a hospital visit is of great concern as a routine examination is key to early intervention in infective respiratory exacerbations and other CF-related diseases. Online consultation method has been well-received by PWCF who have found it to be a convenient alternative for aspects of multidisciplinary care [17]. Many PWCF found online consultation new and were satisfied with this method. Prescription through email was also popular among study participants. Most countries introduced similar measures to reduce the transmission of COVID-19. Havermans et al. reported cancellation of hospital appointments by patients in Belgium due to the fear of infection from hospitalised COVID-19 patients [18]. The CF team in Milan cancelled all routine clinic face-to-face appointments to avoid unnecessary hospital visits and virus spread [19]. A Swiss study conducted during the pandemic on 327 subjects found that half of the outpatient clinic appointments of PWCF were either cancelled/postponed due to lockdown [20]. Another Italian study, conducted by Nobili et al., found that patients who were followed up at their clinics tried to avoid unnecessary hospital access and stayed at home as recommended, delaying their scheduled hospital visits [21]. The Eurordis Rare Disease COVID-19 survey report, which included 6945 respondents from 36 European countries and covered 1250 rare disease types, including CF, found that almost half of the respondents did not visit the hospital due to fear of contracting COVID-19; 34% reported that they were not allowed to visit the hospital if their health problem was not related to COVID-19; and 25% reported that their hospital or rare disease care provider unit was closed [13].

Online medical consultations were introduced and 87.8% of PWCF found them useful. Other countries followed suit; a study from Western Australia showed similar findings, with more than 90% of participants with CF strongly agreed/agreed that teleconsultation could be an excellent way of managing CF care [22]. A further study of telehealth performed at Virginia Commonwealth University Adult CF Center showed that 100% of the patients considered access to telehealth for improved care during the pandemic, and 80% supported telehealth implementation in the future in VCU CF Center [23]. A study in Milan documented the cancellation of routine appointments by the CF team who monitored the clinical conditions of patients via phone calls or emails [19]. According to the Eurordis Rare Disease COVID-19 survey report, 50% of the participants engaged in online consultations. Among those who did, 90% found online consultation/telemedicine services, and online education tools designed to assist in managing their rare disease themselves beneficial [13].

The Medicinal Products (Prescription & Control of Supply) (Amendment) Regulations 2020 and the Misuse of Drugs (Amendment) Regulations 2020 were signed into law in March 2020 as part of health-related initiatives enforced in Ireland to curb the viral spread [24]. They introduced the “national electronic prescription transfer system” which allows a prescription to be electronically transferred from the health care professional directly to the pharmacist [25, 26]. This was welcomed by the CF community, with 88% in our study in favour. Repeat prescriptions emailed directly to pharmacy will be continued.

COVID-19 significantly increased the unemployment rate, and decreased hours of work and labour force participation during that period [27]. In our study, it was observed that a greater proportion of older adults stopped working while they were cocooning. Also, over one-third of employers were perceived as unsympathetic towards PWCF who were not working as cocooning. Those who were employed were more energetic and happier as compared to those who were not employed. No significant difference by gender was observed.

The key mental health issues faced by the majority of PWCF during the pandemic were increased levels of nervousness and tiredness. PWCF aged < 35 years experienced more mental distress during the pandemic as compared to the older group, particularly in terms of nervousness and feeling downhearted. Cocooning was recommended at the start of the pandemic by the Irish Government for older people and those considered highly vulnerable to COVID-19, including those living with CF [4]. Studies from several countries including Turkey, the United Kingdom (UK), Belgium, and Italy showed similar findings with high levels of anxiety and depression among PWCF and their families [19, 28, 29]. From a general population perspective, an Irish study of 1000 people in March and April 2020 (during the initial restrictions) found that 41% of respondents felt lonely, 23% recorded depression, 20% reported anxiety, and 18% reported clinically relevant post-traumatic stress [30]. Another survey conducted by the Central Statistics Office in Ireland, titled “Social Impact of COVID-19 Survey April 2020”, reported that 35.5% of respondents from general Irish households reported feeling very nervous, 32.4% felt downhearted and depressed, and 26.6% felt lonely “at least some of the time” in April 2020 [14]. Those with CF were deemed to be in the ‘highly vulnerable’ group, which confers an additional psychological burden.

A limitation of this study is that this was a self-reported study of PWCF recruited through social media and the CF Ireland website. Findings of our study however concur with other authors internationally. This study was conducted at the end of the second wave of COVID-19 in Ireland, a time when there was talk of vaccines, but all were in the trial stages with no definite hope yet. Future work should look at how the rollout of vaccination may change the psychological wellbeing of adults with CF.