Adult Cystic Fibrosis Centre Research Articles

P099 Nontuberculous mycobacteria (NTM) infection in adults with cystic fibrosis: a surveillance from Slovenian adult cystic fibrosis centre

P099 Nontuberculous mycobacteria (NTM) infection in adults with cystic fibrosis: a surveillance from Slovenian adult cystic fibrosis centre

WS10.2 Normal weight obesity in cystic fibrosis is associated with glucose tolerance irregularities in cystic fibrosis at a UK adult cystic fibrosis centre

WS10.2 Normal weight obesity in cystic fibrosis is associated with glucose tolerance irregularities in cystic fibrosis at a UK adult cystic fibrosis centre

WS02.4 Developing an enhanced transition pathway for young people with additional needs at the Manchester Adult Cystic Fibrosis Centre (MACFC)

WS02.4 Developing an enhanced transition pathway for young people with additional needs at the Manchester Adult Cystic Fibrosis Centre (MACFC)

P245 Screening and treatment of Cystic Fibrosis-Related Diabetes at a UK adult cystic fibrosis centre

P245 Screening and treatment of Cystic Fibrosis-Related Diabetes at a UK adult cystic fibrosis centre

P312 Patient reported use, effects and tolerance of 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre

P312 Patient reported use, effects and tolerance of 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre

Shifting Paradigms Regarding the Role of Nontuberculous Mycobacteria in Patients with Cystic Fibrosis Advanced Lung Disease

Nontuberculous mycobacteria (NTM) are challenging pathogens in cystic fibrosis (CF). The best approach is unknown, in particular, in patients in need of a lung transplant (LTX). NTM sputum-positive patients were identified from all CF patients treated at the University Hospital of Zurich Adult CF Center. Course of disease and resistance to macrolide therapy was recorded. Of 6 patients who received a LTX, NTM patients were compared with patients without NTM for survival and development of chronic lung allograft dysfunction (CLAD). Of 111 CF patients, 43 (38.7%) patients grew at least one NTM culture of which 28 (25.2%) reached the stringent criteria for NTM positivity defined by three NTM positive cultures. With 32 (74.4%) isolates, M. abcessus spp. (MABSC) were the most frequent and acquired at an earlier age as compared to other NTM species (20 y [17-8] vs. 27 y [24-33]). NTM patients tended to be male and homozygous for PheF508del mutation, in particular, patients with MABSC, and more likely to require a LTX than patients without NTM (14.3% vs. 2.4%, p = 0.035). Of 28 MABSC patients with susceptibility testing, only three out of 16 (18.8%.) patients receiving treatment achieved long-term culture negativity. Treated patients were more likely to develop clarithromycin resistance than patients without treatment (68.8% vs. 16.7%, p = 0.019) with no difference in developing advanced lung disease (ALD), receiving a LTPL or death. 2 of the 6 patients undergoing LTX suffered from infection with MABSC, both of them being NTM positive before LTX. No difference in long-term survival nor in developing CLAD was observed. Two patients developed pulmonary and one extra-pulmonary infection. NTM patients are at greater risk of ALD and requiring LTX, with MABSC infection acquired at an earlier age and being more common in patients with PheF508del homozygous genotype. It is unclear whether this reflects a more rapid underlying course of CF. In MABSC patients, response to chemotherapy was poor and development of resistance common. NTM infection after LTX did not result in increased mortality or development of CLAD and BOS, but was associated with infection. Our experience supports that NTM infection should not generally be regarded as an absolute contraindication for LTX, but NTM eradication prior to LTX should be attempted.

Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators – an international survey

BackgroundAs their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown. MethodsA survey was sent to lead clinicians of adult CF centres in Europe, the United Kingdom (UK), United States of America (USA), Australia and Israel requesting anonymised data on pregnancy outcomes in women using CFTR modulators before and during pregnancy and lactation. ResultsWe identified 64 pregnancies in 61 women taking IVA (n = 31), LUM/IVA (n = 26) or TEZ/IVA (n = 7), resulting in 60 live births. In 44 pregnancies, CFTR modulators were either continued throughout pregnancy or temporarily stopped and then restarted. Two maternal complications were deemed related to CFTR modulator therapy; cessation of modulator therapy resulted in clinical decline in 9 women prompting resumption of therapy during pregnancy. No modulator-related complications were reported in infants exposed in utero and/or during breastfeeding. ConclusionsCFTR modulators were reported to be generally well tolerated in pregnancy and breastfeeding, with only 2 maternal complications that were deemed related to CFTR modulator therapy. Women stopping CFTR modulators in pregnancy may experience a decline in clinical status and in the cases identified in this survey, restarting therapy led to a clinical improvement. Current experience remains limited and longer-term prospective follow-up is required to exclude delayed adverse effects.

Retinopathy and microalbuminuria are common microvascular complications in cystic fibrosis-related diabetes.

Aims:To study the prevalence of microvascular complications and renal changes associated with cystic fibrosis-related diabetes (CFRD).Methods:This retrospective cohort study was conducted at the West Midlands Adult Cystic Fibrosis centre, United Kingdom. Data regarding age, sex, microalbuminuria, retinopathy neuropathy, and biochemical results were collected for all people with CFRD who had an annual review from 1 January 2018 to 31 December 2018 at the centre. Descriptive statistics were analysed using STATAv15.1.Results:A total of 189 patients were included, of which 56.6% were male and median age (interquartile range) was 33 (27–39) years; 79.4% (150/189) had their annual review in 2018. Those with a biochemically impaired renal function numbered 7.2% (13/180) and 22.7% (32/141) had microalbuminuria; 17.2% (10/58) had diabetes related retinopathy. No one in our cohort had diabetic ulcers; however, 10.3% (13/126) had absent foot pulses.Conclusion:We found a higher prevalence of microalbuminuria compared with retinopathy in a large cohort of cystic fibrosis adults. This study demonstrates the need for regular specialist follow-up to facilitate early identification of such complications and a long-term prospective cohort to understand underlying mechanisms.

Improvement in contraceptive coverage and gynecological care of adult women with cystic fibrosis following the implementation of an on-site gynecological consultation

ObjectiveOur study aimed to evaluate the impact of the introduction of a new gynecologic referral service in our adult Cystic Fibrosis (CF) center on contraceptive coverage, gynecological follow-up regularity, and cervical cancer screening coverage. Study designWe implemented an on-site gynecological consultation in our adult CF center in 2015. We compared the results of two surveys conducted successively in 2014 and in 2017 in a cohort of women with CF attending the Lyon CF center. Women completed the same self-report written questionnaire as in 2014. Main outcome measures were the comparisons of contraceptive coverage, gynecological follow-up regularity, and cervical cancer screening coverage between 2014 and 2017. ResultsAll the 136 women (100%) who attended the clinic in 2017 participated. Contraceptive prevalence rate increased from 69%(CI95%:60.3–78.1) to 86%(CI95%:79.6–92.9) between 2014 and 2017 (p = 0.005). Among transplanted patients, the contraceptive prevalence rate was 92.3%(CI95%:82.0–100) in 2017. Long acting reversible contraceptive use markedly increased from 10% to 21.6% (p = 0.005). The proportion of women that reported an access to gynecological care increased between 2014 and 2017 (74%(CI95%:66.3–82.0) vs 91%(CI95%:86.9–95.4), p < 0.005) and reached 100% among transplanted patients. Cervical cancer screening improved (55%(CI95%:51.2–68.8) vs 85%(CI95%:78.6–90.6) women ever screened) (p < 0.0005) and reached 100% among transplanted patients. ConclusionsWe observed an improvement in contraceptive coverage and gynecological care of adult women with CF following the implementation of a dedicated gynecological consultation in the CF center. ImplicationsService linkages and formal links between CF centers and gynecologists can facilitate access to disease-specific contraceptive counseling, adequate gynecological management and cervical cancer screening.

Burkholderia cenocepacia ET12 transmission in adults with cystic fibrosis

This report describes transmission of a Burkholderia cenocepacia ET12 strain (ET12-Bc) at the Toronto Adult Cystic Fibrosis (CF) Centre occurring from 2008 to 2017. Epidemiological and genomic data from 11...

Exploring the implications of different approaches to estimate centre-level adherence using objective adherence data in an adult cystic fibrosis centre – a retrospective observational study

BackgroundAccurate centre-level medication adherence measurement allows identification of highly performing CF centres, drives shared learning and informs quality improvement. Self-reported adherence is unreliable but data-logging nebulisers can capture objective data. However, adherence levels in current literature are limited by the use of agreed prescriptions and convenience sampling. In this single-centre retrospective study, we quantified the differences in centre-level adherence with different methods of calculating adherence (unadjusted vs normative adherence) and different data sampling frames (convenience sampling vs including difficult to obtain data). MethodsAdherence data were objectively captured using I-neb® from 2013-2016 in Sheffield Adult CF Centre. Adults on non data-logging devices, on ivacaftor or with previous lung transplantation were excluded. Adherence was calculated based on agreed regimen (‘unadjusted adherence’) or minimum required regimen (‘normative adherence’). I-nebs® not brought to clinic were downloaded during home visits. Adults not on any inhaled therapy but with chronic Pseudomonas aeruginosa infection were included by counting their adherence as “0”. ResultsOf the 131 included adults, 126 provided I-neb® data. Calculating unadjusted adherence from I-nebs® brought to clinics resulted in the highest centre-level adherence (median 41.8% in 2013). Median adherence reduced after sequentially accounting for minimum required regimen (40.0% in 2013), I-nebs® not brought to clinics (32.9% in 2013) and adults not on any inhaled therapy (31.0% in 2013). ConclusionsDifferent approaches of calculating adherence produced different adherence levels. Adherence levels based only on agreed regimen among adults who readily brought their nebulisers to clinics can over-estimate the effective adherence of CF centres.

Omalizumab for asthma and allergic bronchopulmonary aspergillosis in adults with cystic fibrosis

BackgroundIn cystic fibrosis (CF), omalizumab has been used for difficult-to-treat asthma and allergic bronchopulmonary aspergillosis (ABPA) but safety and efficacy data are limited for this population. MethodsWe assessed patients receiving omalizumab for asthma or ABPA in the Toronto adult CF center between 2005 and 2017. We evaluated treatment safety and efficacy by analyzing changes in FEV1% predicted (FEV1pp) max value, slope and variability captured by the area under the curve (AUC), the cumulative dose of systemic corticosteroids (SCS), use of intravenous (IV) antibiotics and hospitalization days before omalizumab and up to 1 year after treatment initiation. Linear mixed effects model was used for FEV1pp slope and the trapezoidal rule for FEV1pp AUC. ResultsTwenty-seven CF patients received omalizumab, 16 (59.3%) for asthma and 11 (40.7%) for ABPA. No significant omalizumab-related adverse effects were observed. In the asthmatic group, the max value of FEV1pp improved on omalizumab and the cumulative dose of SCS decreased. In the ABPA group, the rate of FEV1pp decline (slope) and the variability of FEV1pp (AUC) improved on omalizumab. In ABPA patients, the cumulative SCS dose was not significantly different but 4 (36%) patients decreased their SCS dose by >50% compared to baseline. Days on IV antibiotics and hospital days did not differ significantly before and while on omalizumab therapy. ConclusionsIn adult CF patients with difficult-to-treat asthma or ABPA, omalizumab should be considered. Larger studies are needed to identify patient characteristics that may predict response to omalizumab.

Changes in Clinical Markers During A Short-Term Transfer Program of Adult Cystic Fibrosis Patients from Pediatric to Adult Care.

Background:Transition from child-oriented to adult-oriented health care in Cystic Fibrosis (CF) has become more important over recent decades as the survival of people with this disease has increased. The transition process usually begins in adolescence, with full transfer completed in early adulthood.Objective:This study investigated the impact of a short-term transfer program on clinical markers in an adult CF cohort still being managed by pediatricians.Methods:Clinically relevant data from the year before (T-1), the time of Transfer (T) and the year after the transfer (T+1) were analysed retrospectively.Results:39 patients (median age 29.0 years; 64% male) were transferred between February and December 2016. Lung function had declined significantly in the year before transfer (in % predicted: Forced Expiratory Volume in 1 second (FEV), 62.8 vs. 57.7, p <0.05; Forced Vital Capacity (FVC), 79.9 vs. 71.1, p<0.05), but remained stable in the year after transfer (in % predicted: FEV: 56.3; FVC 68.2). BMI was stable over the whole observational period. There was no relevant change in chronic lung infection with P. aeruginosa, Methicillin-Resistant Staphylococcus aureus (MRSA) and Burkholderia sp. during the observation period. The number of patient contacts increased significantly in the year after versus the year before transfer (inpatient: 1.51 vs. 2.51, p<0.05; outpatient: 2.67 vs. 3.41, p<0.05).Conclusions:Our data show that, within the framework of a structured transfer process, it is possible to transfer a large number of adult CF patients, outside a classic transition program, from a pediatric to an adult CF center in a short period of time, without any relevant changes in clinical markers and, stability.

Intravenous antibiotic use and exacerbation events in an adult cystic fibrosis centre: A prospective observational study.

In CF, people with higher FEV1 are less aggressively treated with intravenous (IV) antibiotics, with resultant negative impact on their health outcomes. This could be entirely clinician-driven, but patient choice may also influence IV use. In this prospective observational study, we explored IV recommendations by clinicians and IV acceptance by adults with CF to understand how clinical presentations consistent with exacerbations resulted in IV use. Clinical presentations consistent with exacerbations, IV recommendation by clinicians and IV acceptance by patients were prospectively identified for every adult with CF in Sheffield throughout 2016, excluding those who had lung transplantation (n = 7) or on ivacaftor (n = 13). Relevant demographic data, e.g. %FEV1, were extracted from medical records. Multi-level mixed-effects logistic regression models were used to compare IV recommendations vs non-recommendations for all clinical encounters, and IV acceptance vs non-acceptance for all IV recommendations. Among 186 adults (median age 27 years, median FEV1 78.5%), there were 434 exacerbation events and 318 IV use episodes following 1010 clinical encounters. Only 254 (58.5%) of exacerbations were IV treated. A diagnosis of exacerbation, higher number of symptoms and lower %FEV1 were independent predictors for IV recommendation by clinicians. Higher number of symptoms and lower %FEV1 were also independent predictors for IV acceptance by adults with CF. Lower IV use among adults with higher %FEV1 was influenced by both clinicians' and patients' decisions. Using IV antibiotics as an exacerbation surrogate could under-estimate exacerbation rates and conceal differential treatment decisions according to varying clinical characteristics.

P267 Two years' experience of lumacaftor/ivacaftor treatment at an adult cystic fibrosis centre in Athens, Greece

P267 Two years' experience of lumacaftor/ivacaftor treatment at an adult cystic fibrosis centre in Athens, Greece

P127 Long-term outcomes of cohort segregation on the prevalence of a transmissible strain of Pseudomonas aeruginosa at an adult cystic fibrosis centre

P127 Long-term outcomes of cohort segregation on the prevalence of a transmissible strain of Pseudomonas aeruginosa at an adult cystic fibrosis centre

P146 Detection of the Rec A gene of the Burkholderia cepacia complex from sputum samples of an adult cystic fibrosis centre in Argentina

P146 Detection of the Rec A gene of the Burkholderia cepacia complex from sputum samples of an adult cystic fibrosis centre in Argentina

P150 Achromobacter prevalence, species distribution and infection status results from a large UK adult cystic fibrosis centre

P150 Achromobacter prevalence, species distribution and infection status results from a large UK adult cystic fibrosis centre

P122 Eradication of Pseudomonas with inhaled tobramycin in a large adult cystic fibrosis centre

P122 Eradication of Pseudomonas with inhaled tobramycin in a large adult cystic fibrosis centre

P250 Pattern of use of NIV: 10-year experience of a large UK adult cystic fibrosis centre

P250 Pattern of use of NIV: 10-year experience of a large UK adult cystic fibrosis centre