D. Lau

202 Bone mineral density and fractures at the All Wales Adult CF Centre (AWACFC)

CF related low bone mineral density (CFRLBMD) occurs in 34% CF patients (Haworth 2001). 14% of CF patients have evidence of vertebral fractures and 20% nonvertebral fractures (Paccou 2010). Aim To determine the prevalence of CFLBMD and fractures in patients attending the AWACFC. Method The following information taken from the annual reviews of patients attending the AWACFC: age, sex, history and nature of fractures, FEV 1 %, BMI, Z score Lumbar spine (LS) and hip and whether the patient was maintained on calcium/vitamin D and bisphosphonate, duration of bisphosphonate use and change in Z score over this period. Patients completed a fracture history questionnaire. Results 254 (144 male) patients were included with a mean (SD) age, FEV 1 % and BMI of 28.8 (9.4) years, 65.7% (25.6) and 22.8 kg/m 2 (4.65) respectively. Their median (range) Z score at lumbar spine (LS) and hip were –0.87 (–4.2 to 2.4) and –0.64 (–3.7 to 2.6) respectively. 57 (32 male) of 254 (22%) patients had CFRLBMD. Of these 57, 55 (96%) were on calcium/vit D and 44 (77%) on bisphosphonate therapy. Patients with CFRLBMD had lower FEV 1 % [47.5 vs 70.7 (p 2 (p Conclusion The prevalence of CFRLBMD at the AWACFC is slightly lower than Haworth study which may reflect a trend to a more aggressive approach to CF therapies over the past decade. The prevalence of fractures in our cohort is similar to other studies.

135 Transformational care at the All Wales Adult CF Centre (AWACFC) – the impact of ivacaftor (Kalydeco®) one year on

Background 11 patients attending the AWACFC have completed one year of treatment on ivacaftor. In demonstrating cost effectiveness of therapies it is important to assess patients holistically including quality of life (QOL) data. We therefore aimed to establish the effectiveness, including QOL data, of ivacaftor use in our local patient cohort one year after initiation of treatment. Method The following data was collected from the 11 patients attending the AWACFC at initiation of and one year after starting ivacaftor: FEV 1 %, BMI, sweat chloride, CF respiratory quality of life scores (CFQresp) and IV antibiotic days per year. Results All 11 patients (3 male) are delF508/G551D with a mean (SD) age of 28 (13) years. Pre ivacaftor Post ivacaftor Change p Sweat chloride (mmol/L) 120 (21) 57 (16) —63 FEV1% 63.5 (26.2) 74.5 (27) 11.0 BMI (kg/m 2 ) 20.9 (2.9) 22.6 (3.1) 1.7 0.009 CFQ resp 63.1 (26.7) 89.4 (11.0) 26.3 0.004 IV antibiotic days per year 37.0 (22.7) 4.5 (7.4) —32.5 Values are mean (SD). Conclusion In our adult cohort it appears ivacaftor has been clinically effective for patients with delF508/G551D with significant improvements in sweat chloride, FEV 1 %, BMI, CFQresp and IV antibiotic days per year. The magnitude of this improvement is similar to previous published studies of larger cohorts (Ramsey et al 2011, McKone 2014). These positive results have been an important source of evidence in funding decisions for ivacaftor therapy in Wales.

WS10.6 Are cystic fibrosis patients frail? Is 50 the new 80?

Background: Socio-economic (SE) deprivation has been linked with poorer outcomes in chronic illness. CF, prevalent across all SE groups involves a high burden of care. Our large regional adult centre (n = 201) covers a wide SE catchment area and we wished to assess the impact of this deprivation on the health of our current adult CF patients. Methods: Patients were assigned an index of multiple deprivation (IMD) score [1] based on postcode and subdivided into quintiles (Quintile 1: least deprived, Quintile 5: most deprived). The IMD score accounts for seven domains (including income, education, housing and healthrelated outcomes). For each quintile – BMI, lung function, IV antibiotic courses (n) and failure to attend clinic (DNA %) were determined over a four-year period. Bivariate correlations were carried out and Fisher’s exact test (two-tailed) was used to compare quintiles. The results are expressed as Mean±SD. Results: Mean age 30.1±9.1 yrs. We found no correlation between IMD scores and no significant differences between quintiles for FEV1, FVC and BMI. There was a higher DNA % rate (r = 0.68; p < 0.0001) and greater use of IV antibiotics (r = 0.19, p = 0.01) in patients from more deprived areas. Conclusions: Those living in more SE deprived areas do not appear to have significant differences in their health status over a four-year period. However, this is at the expense of more IV antibiotics, a surrogate for more intensive treatment and perhaps indicates in part, IV antibiotic use as a ‘rescue treatment’ in some patients to maintain their overall health.

212 Does our current diagnosis of cystic fibrosis related liver disease meet current diagnostic criteria

Background Although cystic fibrosis related liver disease (CFRLD) has emerged as the third most common cause of mortality in cystic fibrosis patients, it is currently poorly recognised. Aims To confirm the diagnosis of CFRLD in patients referred to the All Wales Adult Cystic Fibrosis Centre (AWACFC) and to ensure that no patients are being treated unnecessarily. Methods All patients holding a diagnosis of CFRLD attending the AWACFC were identified and their diagnosis of CFRLD was reviewed according to recognised diagnostic criteria (Debray et al 2011) alongside any prescription of ursodeoxycholic acid (UDCA) for them. Patients’ records were reviewed to ascertain whether patients had been investigated for other potential causes of liver disease. Results It was originally believed that 31% (78/249) of the CF patients at the AWACFC had a diagnosis of CFRLD. 45% (35/78) of these diagnoses were confirmed according to the recognised criteria. The true prevalence of CFRLD in our population is 14%. 55% of patients do not meet current criteria for CFRLD and 39% had been receiving UDCA unnecessarily. Conclusion Diagnoses of CFRLD should be regularly reviewed and confirmed using the approved diagnostic criteria and other causes of liver disease should be excluded. All deranged liver biochemistry may not be CFRLD and there is a degree of subjectivity to the interpretation of ultrasonography reports which could introduce a degree of inaccuracy with regard to the diagnosis of CFRLD. Despite recommendations stating that patients with suspected CFRLD should be investigated for other potential causes of liver disease less than a third of our cohort underwent these investigations.

265 Drug allergy documentation in the All Wales Adult CF Centre (AWACFC)

Aims i. To examine the validity of drug allergy documentation in the AWACFC. ii. To explore how effective communication is between AWACFC and patients’ GPs regarding their drug allergies and whether patient knowledge of their own allergies matches AWACFC and GP records. Method Allergy records from the patients’ latest annual reviews were reviewed retrospectively. Allergy records were requested from the patients’ GP and a questionnaire regarding drug allergies was given out to patients. The data was also used to identify if allergies were documented correctly. Allergy was defined as a usually harmless substance resulting in immunological activation (Robinson et al. 2002). Results Of the 240 patients attending the AWACFC 106 (44%) patients had a complete dataset of allergy records from the GP, AWACFC annual review and patient questionnaire. Only 40 of the 106 patients (38%) had allergy documentation from AWACFC, GP and patient questionnaire that matched. A total of 164 drug allergies were documented with 67 patients (63%) having at least one drug allergy documented. 34 of these 67 patients (51%) had at least one drug allergy documented where the description did not meet the definition of a true allergy. Conclusion Communication regarding drug allergies between the patient, GP and AWACFC was poor. It was difficult to characterise true allergies as many of the documented allergies lacked descriptions. Over half the patients had at least one drug allergy documented that was not an allergy. Misdiagnosing an adverse event as an allergy can result in patients needlessly missing out on optimal treatment.

195 The effect of aminoglycoside antibiotics therapy on audiovestibular function in cystic fibrosis patients at the All Wales Adult Cystic Fibrosis Centre (AWACFC)

Aims 1.To determine the prevalence and severity of audiovestibular (AV) disease in adult CF patients attending the AWACFC. 2.To examine any potential link between AV disease and number of days of IV aminoglycosides (AG) received and duration of nebulised AG. Method The annual reviews of patients attending the AWACFC between 2009 and 2014 were reviewed retrospectively. Patients who had reported AV symptoms and received an assessment at an AV clinic were included and their cumulative number of days of IV AG and the length of time receiving nebulised AG was collected. Results 30 (12 male) of the 254 (12%), with a mean (SD) age of 34.2 (8.9) years, had described AV symptoms and been seen in AV clinic. 19 of the 30 patients were found to have some sensorineural (SN) hearing loss. Patients with SN hearing loss had a greater mean number of days on IV AG (340 days) than those with measured normal hearing (247 days) but the least days nebulised AG. 7 patients had vestibular dysfunction (VD). Patients with VD had a greater mean number of days on IV AG than those with normal vestibular function (386 vs 152 days). Conclusion The prevalence of SN hearing loss in AWACFC is less than previous reports (CF Trust 2009) perhaps due to underreporting of symptoms. SN hearing loss and VD were associated with greater number of days on IV AG which concurs with previous studies (Mulheran 2001). The greater the number of days on nebulised AG conferred less hearing damage. This may be explained by nebulised AG being withheld during course of IV AG or by greater long term nebulised AG usage resulting in reduced need for more toxic IV courses of AG. However, patient adherence was not assessed.

WS9.6 The All Wales Adult Cystic Fibrosis Centre (AWACFC) Virtual Instruction of Exercise With Technology to Enhance Care – VIEWTEC Programme

WS9.5 The role of daily physical activity on exercise performance in adults with cystic fibrosis D. Savi1, M. Di Paolo2, N.J. Simmonds3, T. Perelli1, M. Varchetta1, S. Bertasi1, G. Cimino1, P. Troiani1, V. D’Alu1, S. Quattrucci1, S. Cucchiara1, P. Palange2. 1Sapienza University of Rome, Department of Pediatrics and Pediatric Neurology, Cystic Fibrosis Center, Rome, Italy; 2Sapienza University of Rome, Department of Public Health and Infectious Diseases, Rome, Italy; 3Royal Brompton Hospital and Imperial College, Department of Cystic Fibrosis, London, United Kingdom

235 The CF couch potato! Habitual physical activity in an adult CF population

Background: Physical activity has been shown as beneficial to CF. Patients health and quality of life are positively affected by physical training (Wilkes 2009). Increased habitual physical activity (HPA) has been shown to reduce the speed of lung function decline. Physical inactivity significantly contributes to exercise intolerance and skeletal muscle weakness in CF (Troosters 2009). Objectives: To compare the HPA of stable adult outpatient CF patients with recognised ranges in other chronic diseases. Method: 10 patients wore the Sensewear® electronic activity monitors. Demographic and anthropometric data were collected. Results: 10 patients (8 male) participated with mean (SD) age, FEV1%, BMI and FFMI of 28.3 years (8.1), 54.2% (19.8), 22.1 kg/m2 (2.0) and 17.9 kg/m2 (1.8) respectively. Patients wore the Sensewear® for a mean (SD) 6.5 days (4.4) and the mean (SD) percentage time on body was 91.6% (8.9). Their mean (SD) shuttle-walk test was 491.4m (202.4). Their mean (SD) Metabolic Equivalents (METS) activity during the monitored period was 1.7 (0.18) with patients spending a mean (SD) time of 200 minutes (71) engaged in activity greater than 3 METS. The mean (SD) number of steps taken per day was 4552 (2475). Conclusion: The mean METS per day equates to very low activity levels such as knitting with patients only spending around 3 hours doing activities equating to light activity such as playing pool which is considered inadequate for cardiovascular conditioning (Jette 1990). The average steps per day are below those considered normal for people living with chronic disability/disease (range 7000–9000) (TudorLocke 2011).

217 A novel use of BrEaD (breathlessness, eating and drinking)

Background: Patients with cystic fibrosis (CF) are at risk of reduced Fat Free Mass (FFM) which is a marker of skeletal muscle mass. Associations between lung function and body composition using either DEXA or bioelectrical impedance (BIA) have previously been reported in CF patients however hand-grip strength (HGS) assessment is not routinely utilised. Aim: To identify the potential use of HGS as a tool to help identify altered body composition in CF patients. Method: Body composition (FFM calculated as fat free mass index (FFMI)) using BIA and HGS were assessed at annual review in 118 adult patients attending AWACFC. Gender, age, pancreatic status, body mass index (BMI) and FEV1% data were collected. Results: 118 patients (73 male) were assessed with mean (SD) age 28.7 years (10.0), FEV1% of 66.6% (24.9), BMI 23.6 kg/m2 (5.9), FFMI 17.8 kg/m2 (2.4) and HGS of 33.9 kg (10.3). 104 (88%) were pancreatic insufficient. HGS strongly correlated with FFM (r = 0.77) and FFMI (r = 0.64) (both p 0.05). However, analysing the females alone FEV1% did correlate with HGS (r = 0.44, p = 0.002). Conclusion: HGS is a cheap, portable, quick and reliable tool which appears to correlates well with FFM and FFMI as measured by BIA. Its strong correlations with FFM may suggest it has potential as a surrogate marker of skeletal muscle mass and it may be a useful adjunct in identifying patients where there is possible loss of FFM. Further longitudinal studies are required.

216 Hand strength, is it time to get a grip?

Background: Patients with cystic fibrosis (CF) are at risk of reduced Fat Free Mass (FFM) which is a marker of skeletal muscle mass. Associations between lung function and body composition using either DEXA or bioelectrical impedance (BIA) have previously been reported in CF patients however hand-grip strength (HGS) assessment is not routinely utilised. Aim: To identify the potential use of HGS as a tool to help identify altered body composition in CF patients. Method: Body composition (FFM calculated as fat free mass index (FFMI)) using BIA and HGS were assessed at annual review in 118 adult patients attending AWACFC. Gender, age, pancreatic status, body mass index (BMI) and FEV1% data were collected. Results: 118 patients (73 male) were assessed with mean (SD) age 28.7 years (10.0), FEV1% of 66.6% (24.9), BMI 23.6 kg/m2 (5.9), FFMI 17.8 kg/m2 (2.4) and HGS of 33.9 kg (10.3). 104 (88%) were pancreatic insufficient. HGS strongly correlated with FFM (r = 0.77) and FFMI (r = 0.64) (both p 0.05). However, analysing the females alone FEV1% did correlate with HGS (r = 0.44, p = 0.002). Conclusion: HGS is a cheap, portable, quick and reliable tool which appears to correlates well with FFM and FFMI as measured by BIA. Its strong correlations with FFM may suggest it has potential as a surrogate marker of skeletal muscle mass and it may be a useful adjunct in identifying patients where there is possible loss of FFM. Further longitudinal studies are required.