Telemedicine and cystic fibrosis: Do we still need face-to-face clinics?

There has been growing interest in telemedicine for cystic fibrosis over recent years based largely on convenience for patients and/or increasing the frequency of surveillance and early detection which, it is assumed, could improve treatment outcomes. During 2020, the covid-19 pandemic catalysed the pace of development of this field, as CF patients were presumed to be at high risk of infection. Most clinics adapted to digital platforms with provision of lung function monitoring and sample collection systems. Here, we present the views of multidisciplinary team members at a large paediatric CF centre on what has worked well and what requires further optimisation in the future. In response to the question posed, 'Do we still need face to face clinics?' our answer is 'Yes, but not every time, and not for everyone'.

Monitoring lung function of young people with CF at home: Is it reliable? Results from the climb-CF study

Introduction: Conventionally, young people with CF are reviewed in clinic in person every 2-3 months;home monitoring is not standard. At each visit, depending on age, they perform spirometry supervised by a CF team member to guide clinical decisions. Previous studies have suggested that short-term variation of 6.3% FEV1% predicted (FEV1%pred) is “normal” and a change >13% is likely to represent disease progression (Taylor-Robinson, et al. Thorax. 2012). Handheld turbine spirometers are readily available, and the COVID-19 pandemic has catalysed major changes in practice, with centres reducing clinic visits in favour of home monitoring. The recent completion of our home monitoring feasibility study (CLIMBCF) allowed us to test the hypothesis that home and clinic spirometers give equivalent results. Methods: We designed a study-specific mobile phone app incorporating integrated Bluetooth no-touch data capture from a Vitalograph BT spirometer. We recruited participants aged 2 to 17 years from 8 sites in 2 countries. Suitably aged children were provided with a spirometer, trained in its use and asked to record values at least twice weekly. Clinic visits continued every 8-12 weeks for the 6 months of the study. Here, we compare FEV1 from clinic visits with those obtained unsupervised at home on the same (±1) day. Raw data were converted to GLI FEV1%pred. Where multiple episodes were available for an individual, the first was included in this analysis. We also calculated coefficient of variation (CoV) on all home measurements obtained during diary-defined periods of clinical stability. Results: Paired readings were available from 67 participants (37 female). Median (IQR) age was 10 (7;14) years. Median (IQR) FEV1% values were significantly different between clinic (90.4 [80.4;100.6]%) and home (85.4 [70.7;93.8]%) (p<0.0001). Values from the 2 devices correlated (r2=0.85;p <0.001) but there was substantial bias with higher clinic values from 51 (77.6%) participants. Mean±SD difference between clinic and home FEV1 was 6.5% ± 8.2% with limits of agreement (LoA) of -9.6% to + 22.7%. This bias remained but was less marked in the subgroup ≥12 years (3.8% ± 8.9%;LoA -13.6% to +21%). Median (IQR) CoV in stable children (n=74;age 7 [7;12] years;20 [8;35] measurements per participant) was 9.9% (5.8;14.2%). Discussion: Home spirometers tend to under-read compared to supervised measurements in the clinic. This is similar to previous comparisons of clinic spirometry with handheld devices carried out simultaneously, both supervised by a physiologist (Avdimiretz, et al. Pediatr Pulmonol. 2020). The greater bias in our study shows this may be even more marked when the manoeuvre is carried out unsupervised. While the bias is lower in older participants the limits of agreement are still wide. CoV of repeated measures is higher than reported from clinics, making the home technique less sensitive to deterioration. Unsupervised remote and supervised clinic spirometry are not equivalent. Uncritically giving patients spirometers to use at home may mislead and better strategies are needed.

What is feasible when it comes to monitoring young people with cystic fibrosis at home? The results of the climb CF study

Introduction: There are >350,000 health apps available on app stores across the world however CF is usually assessed at hospital visits. It is unclear whether monitoring children with CF at home is feasible or useful. With the advent of COVID-19 home monitoring has become more necessary. Here we report the results of the CLIMB-CF study which explored the feasibility of home monitoring and potential obstacles. Methods: We designed a study-specific application with input from the CF Trust Youth Advisor Group, and enrolled participants aged 2 to 17 years of age across mulitple sites in the UK and Canada for 6 months. They were asked to complete measures either daily (wellness, cough severity, appetite, sputum volume, breathlessness and tiredness scores, heart rate, oxygen saturations, temperature, respiratory rate, activity and sleep disturbances) or twice a week (weight, and lung function if >5 years of age). After 2 months participants were also given the option to collect samples at home (urine, nasal secretions, sputum, throat swabs and dried blood spots [DBS]). At the end of the study parents and participants over 12 completed feasibility questionnaires. Results: Of 148 (aim was 160), 2 withdrew prior to starting due to social issues, 2 failed to achieve clinical stability and 8 withdrew during the study. Median age of the participants was 7.9 years and 82 (57%) were female. Over the 6 months the median completion of all measures was 40.1% (13.6-69.9%). 2 to 5 year olds completed 54.4% (20.5-77%), 6 to 11 year olds 45.8% (27.0-71.6%) and young people over 12 completed only 15.6% (9.8-30.0%). 77.5% of all participants contacted the study team for technical help at least once. One participant withdrew due to frustration with a piece of equipment. Parents who felt home monitoring did not, or only slightly interfered with their usual activities completed 52% (28.3- 77.6%) of measures compared with those perceiving a heavier burden, 29.9% (9.2-74.5%). Participants over 12 who felt home monitoring did not interfere with their usual activities completed 37.8% (14.5-73.2%) of measures compared to only 9.6% (5.6-22.3%) of measures if they felt it moderately interfered. 73.5% of parents found the ability to monitor their child's health very helpful or helpful. Teenagers felt that sputum collection, nasal secretion collection and DBS collection and spirometry were least acceptable. Discussion: The majority of participants and parents felt home monitoring did not negatively impact their lives. When designing future home monitoring applications those participants who felt home monitoring did not impact their daily lives completed more measures. Teenagers entered less data than younger children;even if they perceived home monitoring was not having a significant impact on their daily lives, completion rates were poor. Interestingly, spirometry was one of the measures teenagers found least acceptable. A significant proportion of our participants required technical help during the study. Our findings should impact on future home monitoring strategies.

Home sputum-induction testing in children with cystic fibrosis

Introduction: Sampling for airway infection is challenging in children with CF who may not be able to spontaneously expectorate sputum even during exacerbation. In the CF Sputum-induction Trial (CF-SpIT) (Ronchetti, et al. Lancet Respir Med. 2018;6(6):461-71), we compared induced-sputum (IS) to cough swab (CS) and 6-lobe bronchoalveolar lavage (BAL) in children with CF. IS isolated 3 times as many pathogens as CS and performed as well as the gold standard 2-lobe BAL in a sensitivity analysis against all pathogens identified. IS is now part of routine clinic care in many CF centres, but physiotherapist resources often limit its availability. Some of our patients already perform IS at home. Face-to-face clinics are currently on hold during the COVID-19 pandemic. We took this time to systematically evaluate home sputum-induction testing (HomeSpIT) in our paediatric CF clinic. Methods: We approached all children aged >5 years who were already using hypertonic saline (HS) in their home physiotherapy routine. Details of how to perform home sputum-induction with an instructional video were provided at www.uhwchildren.com/respiratory/home-sputum-induction. We focussed the HomeSpIT procedure around the simple sequence COUGH, HUFF, COUGH, CLEAR THROAT, SPIT, to be performed during and after HS physiotherapy. Patients were posted instructions and a sputum pot, and received a follow-up call. Same day routine CS was performed at the hospital during drive-by delivery of the home sputum sample. Primary outcome was pathogen detection rate for IS and CS. Results: We identified 42 eligible children. 4 had no transport and another 4 were unkeen to visit the hospital during COVID-19 lockdown. 34 patients therefore attempted HomeSpIT. Median age was 11.5 years (IQR 9.1-14.0). 32/34 (96%) were well and had no cough. 27/34 (79%) had previously performed at least one clinic IS procedure with a physiotherapist. 32/34 (96%) patients managed to produce a sample. 100% IS samples were reported as mucoid, mucopurulent or purulent by the microbiology lab. 12/32 (38%) IS samples were pathogen-positive compared to 6/32 (19%) CS samples (McNemar test p=0.04). 5/12 (42%) IS samples identified more than one pathogen. In total, 19 bacterial pathogens were isolated: P. aeruginosa (n=6), S. aureus (n=6), S. maltophilia (n=3), B. cepacia complex (n=1), A. xylosoxidans (n=1), S. marcescens (n=1) and Acinetobacter (n=1). 19/19 (100%) pathogens were isolated on IS compared with 6/19 (32%) on CS. (McNemar test p<0.001). Families reported HomeSpIT to be easy to perform (median Likert score 8/10 [IQR 6-10]), and were willing to repeat the procedure when needed (median Likert score 10/10 [IQR 8.75-10]). Discussion: Home sputum-induction testing (HomeSpIT) is acceptable and successful in children >5 years. HomeSpIT isolated 3 times as many pathogens as cough swab, similar to that found with clinic sputum-induction procedures in the CF-SpIT study. Establishing HomeSpIT in children with CF age >5 years may initially require some practise for patients, but it will reduce aerosol-producing procedures in clinic, and allow valuable physiotherapist time to be committed to younger children, who require more expertise during sputum-induction procedures.