Adult Cystic Fibrosis Centre Research Articles

P101 Provision of home spirometry in a physiologist-led service: experiences from a large adult cystic fibrosis centre

Objectives: Spirometry is an important marker of lung health in people with cystic fibrosis (pwCF). During the SARS-CoV-2 pandemic, the aerosol-generating nature of spirometry coupled with service user shielding disrupted its routine monitoring. To maintain continuity, we implemented a home spirometry service to complement our telemedicine clinic. Methods: We provided NuvoAir Air Next© home spirometers in clinic or by courier to those of our adult CF cohort with good service engagement and spirometry technique. Training and instruction was given by ARTP-registered physiologists, who are also available at telemedicine clinics to grade spirometry and offer support to service users. Results are uploaded by pwCF via a connected phone. We report our experiences, and the quality of independently-performed home spirometry as graded against ATS/ERS guidelines. Results: Over 4 months, we provided 302 of the 354 pwCF attending our centre with spirometers ([mean ± SD] age 32 ± 11 years (range 17–76), BMI 23.4 ± 4 kgm–2, % predicted FEV1 76.5 ± 23.6. Of the 300, 2 did not tolerate home spirometry, one due to an incompatible phone, and one due to poor compliance. 20 (6.8%) pwCF have yet to use their spirometer. 278 (93%) have performed a total of 5,344 manoeuvres across 1,636 sessions (mean sessions 5.8, mean interval 14.9 days, see Table). We report no adverse events. Quality of spirometry below grade D was often repeated after physiologist feedback. Grade E spirometry was often recorded when only a single acceptable spirometry was performed (20%) or when end-of-test criteria had not been met (46.4%). [Table Presented] Conclusion: Home spirometry can be implemented in a large cohort in a short time frame. Engagement and quality of independently performed spirometry in this group of expert performers was high, but falls below that of hospital quality spirometry. The availability of physiologists to support this service in real-time is an important factor in this and in the reinforcement of reproducible spirometry.

EPS1.06 A retrospective audit of home-based spirometry quality in a large UK adult cystic fibrosis centre

Objectives: Spirometry measurement is the gold standard for assessing disease severity in cystic fibrosis (CF). Poor-quality spirometry tests can result in inaccurate measurement of FEV1 and FVC. Manchester Adult CF Centre has transformed from hospital to home-based spirometry testing during COVID-19. Unlike hospital-based spirometry, home-based spirometry relies entirely on the subject to obtain good quality spirometry. Therefore, we sought to ascertain the quality of home-based spirometry and the test errors in our patients. Methods: A convenience sample of adults with CF attending Manchester Adult CF Centre were provided with a NuvoAir home-based spirometer to perform routine lung function between March–October 2020. NuvoAir respiratory platform consists of a mobile phone application, Bluetooth spirometer and an online results portal. The spirometer also provides feedback to patients’ spirometry quality. Initial patient set-up was performed in-hospital or virtually with a member of the CF clinical team. All patient sessions were included irrespective of quality of spirometry test session. Acceptability and repeatability criteria were applied as per NuvoAir software in line with ATS/ERS guidelines, along with assigning a quality grading A–Faccording to ATS/ERS standardised pulmonary function report criteria at time of testing. Results: 66 CF patients (32 female) mean age 31.3 (18–55) performed 343 spirometry sessions totaling 1,041 individual spirometry tests with a NuvoAir device were graded as follows: Grade A = 30.3%, Grade B = 36.2%, Grade C = 3.5%, Grade D = 2.6%, Grade E = 16.6%, Grade F = 10.8%. Further analysis of all 1,041 tests for common errors indicated BX – Back extrapolation 2%, TP- Time to Peak (slow start) 14.6%, CO – Cough 1.4%, ET – Early termination 0.5%, CE – Cessation or glottic closure 12.9%. Overall, general tests A–C considered usable was 70%. Conclusion: The results show good-quality standards can be achieved through home-based spirometry.

P194 Implementation of a cystic fibrosis-specific virtual pulmonary rehab program

Objectives: Pulmonary rehabilitation (PR) is a key management strategy for people with chronic respiratory disease (BTS Guidelines, 2013). However, in cystic fibrosis (CF), despite the benefits of regular exercise being well documented (Radtke et al, 2017), there is limited uptake. Concerns regarding infection control, lack of CF education, and participating with an older age group may be contributing factors. A pilot study demonstrated good clinical benefits to participants in a CF-specific PR program (Bowen et al, 2019) and current CF Foundation guidelines advise that patients with advanced lung disease should be offered such programs (Kapnadak et al, 2020). We aimed to establish a CF-specific PR program at Manchester Adult CF Centre, as part of the CF Trust Physiotherapy Fellowship service development project. Method: Patients completed a bespoke 8-week program, comprised of biweekly 1:1 virtual exercise sessions. Each session included aerobic and strength training linked to patient goals and was progressed according to reported Borg and muscle fatigue scores. A 1-minute sit to stand test (STS) was performed pre-program and repeated at the end. Enrolled patients had access to an online educational website, which covered a range of CF-specific topics. Patient feedback was collected at the end of the program. Results: [Table Preseteed] Patient feedback indicated that 100% of participants felt their goals had been met and that they would recommend the program to other people with CF (PWCF). Conclusion: Some of the program design was in accordance with what was achievable during the COVID-19 pandemic. Alteration of outcome measures may be required as clinical services normalise. Although a small sample, initial feedback has been extremely positive, indicating that a virtual PR approach may be suitable in engaging PWCF in physical activity. Further data is needed to demonstrate if the program is of clinical benefit and to identify the optimum time-frame of the intervention.

Telomere Length in Cystic Fibrosis Patients – Are Patients with CF Ageing Too Quickly?

Life expectancy for patients living with Cystic Fibrosis (CF) is increasing year on year and there is growing interest in the ageing process in CF. Telomeres are repetitive sequences of DNA that cap the ends of eukaryotic chromosomes and shorten with ongoing cell division, thus providing a marker of replicative history and biological ageing. We aimed to investigate whether telomere length as a function of age differs between patients with CF and healthy individuals and whether telomere length is associated with severity of the patient’s CF condition. Peripheral blood samples and demographic data were collected from 47 consenting patients (age 1 to 57 years) with CF attending their routine annual review appointment at the All Wales Adult CF Centre and Noah’s Ark Children’s’ Hospital in Cardiff, UK. Telomere length profiles were assessed from peripheral blood samples, using the high resolution single telomere length analysis technique (STELA) and compared to healthy control telomere length data. Patients with CF had significantly shorter telomere lengths than healthy individuals, when adjusting for age (p<0.001). Telomere length is decreasing 70% more quickly in the CF cohort than healthy controls. Telomere length does not appear to correlate with markers of disease severity. Telomere lengths are significantly shorter in individuals with CF than in the age-adjusted healthy population. This is suggestive of premature biological ageing of peripheral blood leukocytes in CF patients.

WS14.6 Working together, apart: developing a new model for a cystic fibrosis psychology-led virtual support group

Objectives: While challenging, the COVID-19 pandemic has increased opportunities for virtual working in cystic fibrosis (CF) services, including patient groups. It is an established necessity that psychological support groups are jointly facilitated, which poses a challenge for lone Psychologists. In the context of COVID-19 and pressures on services, MDT members are less available to collaborate. Psychology referrals have increased and people with CF (PWCF) are at increased risk of social isolation. A virtual group was developed incorporating two CF services;addressing both the psychotherapeutic needs of PWCF and the need for collaboration of the lone Psychologists. Method: Two Psychologists from Glasgow and London adult CF centres connected online in June 2020. Similarities in therapeutic approach, experience of lone working, and need to increase outpatient contact were identified. Key themes to structure a series of groups were identified from Psychology referrals. The logistics of cross-NHS Trust working, GDPR, and IT issues were discussed and navigated. In January 2021 approval was given by stakeholders, including Information Governance. The group terms of reference and a pathway for patient recruitment and screening were developed. Results: Working as lone CF Psychologists can be isolating, with less opportunity for creativity. Collaborating has reduced isolation and made it possible to develop a virtual patient group. Regular meetings helped us stay connected and committed in a time of uncertainty and change. Our experiences mirror the anticipated benefits of the support group for PWCF. We hope to model this as facilitators. Conclusion: We present a way to overcome the barriers of isolation and lone-working to creatively collaborate on running virtual patient groups. This new service model presents opportunities for diversity and new ways of thinking. We hope that the group will help to reduce social isolation for PWCF in the same way it has done for two lone CF Psychologists.

Emerging Alternatives to Conventional Clinic Visits in the Era of COVID-19: Adoption of Telehealth at VCU Adult Cystic Fibrosis Center.

Cystic fibrosis (CF) is a genetic disease in which consistent follow-up care is required to avoid a decline in pulmonary and nutritional health. It is believed that if a CF patient ceases treatment for 2 days, this can result in an exacerbation. One week of missed treatments can result in a hospitalization and 1 month of missed treatments can result in an earlier demise. With a global pandemic that has affected more than 9 million people, many CF clinics were required to take steps to avoid transmission of this dangerous virus. This may result in delays in delivery of timely CF care due to closure of clinics and pulmonary function testing (PFT) laboratories and limited staff allowed on site for conducting in-person visits. These measures, along with suggestions from the Cystic Fibrosis Foundation (CFF) to extend the social distancing longer than traditional CDC recommendations for the CF community, create an urgent need to explore novel ways to deliver safer care via new standards in chronic health conditions like CF. Especially, as these preventive strategies may be necessary for long-term maintenance, few objective alternatives exist to guide clinicians and allied health professionals in CF centers how to proceed in this new era. This also presents an opportunity for novel approaches that could improve delivery of CF care with remote monitoring and real-time delivery of care in patients’ home environments. Such emerging approaches could benefit patient care, leading to reduced costs and readmissions and improved access to care, medication adherence, and patient communication. We summarize our own experience and discuss the emerging delivery of CF care which can be generalizable to other pulmonary illnesses.

Editorial introductions

Editorial introductions

Sergančiųjų cistine fibroze 2014–2017 metų klinikinių duomenų analizė. Suaugusiųjų cistinės fibrozės centro patirtis

Tyrimo tikslas. Ištirti Lietuvos sveikatosmokslų universiteto ligoninėsKauno klinikų Suaugusiųjų cistinės fibrozės (CF) centre 2014–2017 m. gydytų pacientų klinikinius duomenis. Tyrimo metodai. Atlikta retrospektyvioji nuo 2014 m. sausio mėn. iki 2017 m. gruodžio mėn. Suaugusiųjų CF centre gydytų pacientų klinikinių duomenų analizė remiantis Europos CF draugijos pacientų registro kriterijais ir reikalavimais. Tyrimo rezultatai. 2014–2017 m. laikotarpiu centre iš viso gydyta 16 sergančiųjų CF (6 moterys ir 10 vyrų): 2014 m. gydyta 12 pacientų, vėliau – po 14.Minėtu laikotarpiu mirė du pacientai bei centras pasipildė 2 naujais sergančiaisiais. 81,2 proc. pacientų (n=13) CF diagnozuota iki 18metų, suaugusiojo amžiuje – 18,8 proc. (n=3). Visiems sergantiesiems diagnozė patvirtinta genetiškai, o devyniems nustatytas ir padidėjęs chloro jonų kiekis prakaite. Bent viena ∆F508 mutacija nustatyta visiems pacientams, abi ∆F508 mutacijos – 5 (31,3 proc.) asmenims. Nagrinėjamu laikotarpiu užfiksuotas sergančiųjų CF amžiaus vidurkio augimas nuo 24,6metų (2014 m.) iki 27,8metų (2017 m.). Plaučių funkcija, vertinta pagal vidutinį forsuoto iškvėpimo tūrį per pirmą sekundę (FEV1), išliko panaši – 59,7 proc. būtinojo dydžio (b. d.) (2014 m.) ir 60,5 proc. b. d. (2017 m.). Vidutinis kūnomasės indeksas (KMI) taip pat nekito – po 19,6 kg/m2 2014 m. ir 2017 m. Remiantis skreplių mikrobiologinio tyrimo rezultatais, dažniausiai buvo nustatoma lėtinė Staphyloccocus aureus infekcija – 75,0 proc. sergančiųjų 2014 m.; 57,1 proc. – 2015 m., 58,3 proc. – 2016 m., 64,3 proc. – 2017 m. Tuo tarpu lėtinė Pseudomonas aeruginosa infekcija diagnozuota rečiau – 18,3 proc. 2014 m., 14,3 proc. – 2015 m., 16,7 proc. – 2016 m. ir 14,3 proc. – 2017 m. Intermituojantis gydymas įkvepiamuoju antipseudomoniniu antibiotiku tobramicinu buvo skiriamas dviem pacientams, iš kurių vienam dėl išsivysčiusio atsparumo pakeistas į įkvepiamąjį kolistiną. Simptominio gydymo pagrindą sudarė įkvepiamasismukolitikas dornazė alfa, kasos fermentai ir bronchus plečiamieji vaistai. Vienam pacientui diagnozuotas su CF susijęs cukrinis diabetas, dviem pacientams nustatytas lėtinis kvėpavimo nepakankamumas bei taikyta nuolatinė deguonies terapija. Trys pacientai buvo įtraukti į plaučių transplantacijos laukiančiųjų sąrašą, du iš jų mirė. Išvados. 2014–2017 m. Suaugusiųjų CF centre gydytų pacientų amžius didėjo neblogėjant plaučių funkcijai ir nemažėjant KMI bei santykinai retai diagnozuojant lėtinę P. aeruginosa infekciją, o tai rodo CF centre teikiamos medicininės pagalbos veiksmingumą.

Noninvasive Ventilation in Cystic Fibrosis: Clinical Indications and Outcomes in a Large UK Adult Cystic Fibrosis Center.

Noninvasive ventilation (NIV) is routinely used to treat patients with cystic fibrosis and respiratory failure. However, evidence on its use is limited, with no data on its role in disease progression and outcomes. The aim of this study was to assess the indications of NIV use and to describe the outcomes associated with NIV in adults with cystic fibrosis in a large adult tertiary center. A retrospective analysis of data captured prospectively on the unit electronic patient records was performed. All patients with cystic fibrosis who received NIV over a 10-y period were included in the study. A priori, 2 groups were identified based on length of follow-up, with 2 subgroups identified based on duration of NIV treatment. NIV was initiated on 64 occasions. The duration of follow-up was categorized as > 6 months or < 6 months in 31 (48.4%) and 33 (51.6%) occasions, respectively. The most common indications for starting NIV were chronic (48.5%) and acute (32.8%) hypercapnic respiratory failure. Among those with a follow-up > 6 months, subjects who stopped using NIV early showed a steady median (interquartile range) decline in FEV1 (pre-NIV: -0.04 [-0.35 to 0.03] L/y vs post-NIV: -0.07 [-0.35 to 0.01] L/y, P = .51), while among those who continued using it had an improvement in the rate of decline (pre-NIV: -0.25 [-0.52 to -0.02] L/y vs post-NIV: -0.07 [-0.13 to 0.16] L/y, P = .006). No differences in intravenous antibiotic requirement or pulmonary exacerbations were noted with the use of NIV. Pneumothorax and massive hemoptysis occurred independently in 4 cases. NIV is being used in cystic fibrosis as adjunct therapy for the management of advanced lung disease in a similar fashion to other chronic respiratory conditions. Adherence to NIV treatment can stabilize lung function but does not reduce pulmonary exacerbations or intravenous antibiotic requirement.

Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis

Individuals with cystic fibrosis (CF) have an increased risk for gallbladder abnormalities and biliary tract disease, but the reported incidence of these manifestations of CF varies widely in the literature. With the approval of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA), increasing numbers of CF patients have been initiated on highly effective cystic fibrosis transmembrane regulator (CFTR) modulator therapy. While elevations in hepatic panel are known potential side effects of CFTR modulators, there have been no published cases of biliary disease or acute cholecystitis attributed to these medications. In this case series, we describe seven patients at two adult CF centers with biliary colic shortly after initiation with ELX/TEZ/IVA, six of whom required cholecystectomy.

Influenza B outbreak at an adult cystic fibrosis centre - Clinical impact and factors influencing spread

IntroductionAn outbreak of Influenza B occurred at a large United Kingdom (UK) regional adult cystic fibrosis (CF) centre in May 2016. This was late in the UK 2015–2016 influenza season and occurred on a specialist ward with strict infection control procedures. This study investigates the spread of influenza, clinical consequences and potential contributing factors. MethodsPatient records, clinical status and pulmonary function data were reviewed for all inpatients during this period. Respiratory viral PCR results, influenza vaccination status of patients and staff, and environmental factors were also recorded. Affected patients were prospectively monitored for the following three months. Results10 of 21 inpatients developed influenza B between 5th and 12th May 2016, an attack rate of 48%. All those characterised were confirmed as the same strain of influenza B/Victoria-lineage. Influenza infection resulted in a mean FEV1 reduction of 10.5% (SD 11.3, p = 0.012), which persisted at 3 months post infection (p = 0.003). Nine of the affected cases rooms were in close proximity on the ward while patients in the two isolation rooms with enhanced ventilation did not become infected. Ventilation measurements in affected rooms ranged from 1.75 to 2.10 air changes/hour, below national recommendations. Seventy percent of affected inpatients had received the 2015/16 trivalent seasonal influenza vaccine, which did not contain a B/Victoria-lineage influenza B virus. ConclusionThis influenza B outbreak in CF adults had a high attack rate and a significant clinical impact. Room ventilation and a limited protection from the seasonal influenza vaccine were possible contributory factors.

P336 “Nobody knows what the patient is actually taking!” Understanding nebuliser regimens in an adult cystic fibrosis centre: a systems optimisation project

P336 “Nobody knows what the patient is actually taking!” Understanding nebuliser regimens in an adult cystic fibrosis centre: a systems optimisation project

P314 Introduction of nebulised levofloxacin in an adult cystic fibrosis centre

P314 Introduction of nebulised levofloxacin in an adult cystic fibrosis centre

P067 Using a national cystic fibrosis Registry to explore longitudinal outcome measures at an adult cystic fibrosis centre in comparison with other centres

P067 Using a national cystic fibrosis Registry to explore longitudinal outcome measures at an adult cystic fibrosis centre in comparison with other centres

EPS2.06 Early experiences of a bowel cancer screening programme in an adult cystic fibrosis centre

ePS2.06 Early experiences of a bowel cancer screening programme in an adult cystic fibrosis centre

P219 Symkevi: real-world experience in an ill cohort at an adult cystic fibrosis centre

P219 Symkevi: real-world experience in an ill cohort at an adult cystic fibrosis centre

P309 The use of Dry Powder Mannitol (DPM) – experience at a large adult cystic fibrosis centre

P309 The use of Dry Powder Mannitol (DPM) – experience at a large adult cystic fibrosis centre

P317 Patient experience and satisfaction with 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre and potential barriers to use

P317 Patient experience and satisfaction with 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre and potential barriers to use

EPS2.07 Are women with cystic fibrosis at the All Wales Adult Cystic Fibrosis Centre attending for cervical screening?

ePS2.07 Are women with cystic fibrosis at the All Wales Adult Cystic Fibrosis Centre attending for cervical screening?

P329 To evaluate adherence to NIV for airways clearance at the All Wales Adult Cystic Fibrosis Centre (AWACFC)

P329 To evaluate adherence to NIV for airways clearance at the All Wales Adult Cystic Fibrosis Centre (AWACFC)