Workshop 9. Diversity in CF care
WS09.1 Should we advocate a structured venous access policy in adults with CF? K. Colpaert1 , C. Ruelens1 , N. Lorent1 , P. Van Bleyenbergh1 , L.J. Dupont1 . 1 University Hospital Leuven, CF Unit, Department of Respiratory Diseases, Leuven, Belgium Background: Peripherally Inserted Central Catheter (PICC) & Totally Implanted Access Device (TIVAD) are widely used to provide venous access for CF patients. Increased concerns have been raised on the long-term risks. Method: Increase in observed complications at our center has prompted us to survey problems with PICC & TIVAD from 2006 to 2013 in order to establish a structured venous access policy. Results: Despite overall satisfaction with PICC we registered an adverse event rate of 20% (3% insertion complication, 6% insertion site problems, 2% thrombosis and 9% other PICC dysfunction) which resulted in removal of PICC in 1/3 of events. The number of TIVAD insertions declined slightly during this period. Retaining TIVAD after lung transplantation was associated with an increased risk of bloodstream infections (41% of pts with TIVAD, 75% asymptomatic) and 5 patients with TIVAD developed severe mitral valve insufﬁciency resulting in valve replacement surgery in 3 patients. Four incidents of problematic insertion of ECMO or hemodialysis catheters were registered in the 2 years. Discussion: Based on these data and recent literature the following venous access policy was adopted. – screening for hypercoagulability – use of peripherally inserted catheters in both arms – insertion of PICC restricted to non-dominant arm – LMWheparin SC during PICC in pts with previous thrombosis or hypercoagulability – promoting TIVAD in pts with >3 IV / year – insertion TIVAD restricted to non-dominant arm – yearly Doppler-US in pts with PICC or TIVAD – immediate removal of TIVAD after transplantation
WS09.3 Professional quality of life among CF healthcare providers L. Goldbeck1 , A. Fidika1 , H.-E. Heuer2 , S. Junge3 , C. Smaczny4 , A. Reimann5 . 1 University of Ulm Medical Centre, Child and Adolescent Psychiatry/Psychotherapy, Ulm, Germany; 2 CF Centre Hamburg-Altona, Hamburg, Germany; 3 Hannover Medical School, Pediatric Pulmonology and Neonatology, Hannover, Germany; 4 University Hospital of Frankfurt, CF Centre, Frankfurt, Germany; 5 Mukoviszidose Institute gGmbH, Bonn, Germany Objectives: Professional quality of life (ProQOL) is a validated concept used across various helping professions. It appears relevant considering the level of functioning of healthcare professionals (HCP). We aimed to describe ProQOL among HCP serving patients with cystic ﬁbrosis (CF) and to explore the association with demographic and work-related variables. Methods: HCP from 15 German CF-centers were asked to ﬁll in a questionnaire regarding demographic and work-related information and the Professional Quality of Life Scale measuring Compassion Satisfaction (CS), Burnout (BO), and Secondary Traumatic Stress (STS). Results: Preliminary results based on N = 57 responding HCP (M = 45.5 years; 77% female; 23% physicians, 32% nurses, 45% allied healthcare professionals) revealed that CS (M = 37.9±4.7) was reported more frequently compared to BO (M = 21.3±3.7) and STS (M = 19.6±3.9). No differences were found for gender or age, profession, and full-time versus part-time work, neither for duration of work experience nor for general or CF-speciﬁc case load. Years of serving patients with CF was signiﬁcantly correlated with STS (r = 0.34; p = 0.011). Conclusion: The study group is characterized by a high level of professional satisfaction and relatively low levels of burnout and secondary traumatic stress. However, cumulative secondary traumatic stress may occur over the years when serving patients with CF. Selection effects will be discussed, as well as the possible beneﬁts to screen HCP for ProQOL.
WS09.2 Radiation exposure in adults with CF attending Bristol Adult CF Centre
WS09.4 The role of resilience in quality of life of adult patients with cystic ﬁbrosis (CF)
C. Addy1 , M. Symes1 , N. Bell1 , K. Bateman1 , D. Nazareth1 . 1 University Hospital Bristol, Bristol Adult CF Centre, Bristol, United Kingdom
B. Rabanser1 , H. Mitmansgruber2 , U. Smrekar2 , T. Beck2 , K. Niedermayr1 , J. Eder1 , H. Ellemunter1 . 1 Medical University of Innsbruck, Cystic Fibrosis Centre, Innsbruck, Austria; 2 Medical University of Innsbruck, Department of Medical Psychology, Innsbruck, Austria
Objectives: As survival in CF adults increases their exposure to diagnostic ionising radiation is rising. Exposure to 10 milliSievert (mSv) per annum is sufﬁcient to increase malignancy risk (O’Connell et al. 2012). We wished to estimate radiation exposure in our adult population and identify clinical risk factors for high exposure. Methods: Total diagnostic radiation exposure (expressed as mean±SD mSv) over a twelve month period was calculated (using standard reference doses) for 196 patients attending our centre. Clinical parameters for those with high (>10 mSv) and low (<10 mSv) radiation exposure were compared. Results: 591 investigations were performed. The average radiation dose was 3.7±8.8 mSv. Thoracic imaging accounted for 68% (91±84 mSv) and abdominal imaging 7% (6.3±0.04 mSv) of studies. 11% had high exposure (10 mSv − mean 21.3, maximum 88.9 mSv). CFRD, DIOS and infection with Pseudomonas aeruginosa (PsA) and Mycobacterium abscessus (Mabs) was more common in those with greater levels of radiation exposure, reﬂecting more signiﬁcant disease in these individuals. Conclusion: Care should be exercised when ordering ionising radiation investigations, particularly in those with higher burden of CF disease. Table: Demographics of high and low exposure groups Factor
Low exposure, <10 mSv (n = 172)
High exposure, 10 mSv (n = 22)
Age (years) Gender (M%/F%) FEV1 (%) % with CFRD % with DIOS % Colonised with PsA % Colonised with Mabs Days of IV antibiotics
30±9 57%/43% 77±24% 36% 19% 61% 6% 21.3±30.1
28±9 64%/36% 70±25% 64% 55% 77% 32% 66.2±63.6
Objectives: Although several psychological studies on coping and quality of life suggested high levels of resilience in CF patients, empirical data on resilience in CF are barely existent. Thus, the purpose of the study was to investigate resilience in adult CF patients and its impact on quality of life. Methods: 57 adult CF patients (18−59 years) participated in the study at their routine medical check-up. In addition to their regular psychological assessment including the Cystic Fibrosis Questionnaire − Revised (CFQ-R), the Resilience Scale (RS) was administered. The constructs were also correlated with physical parameters (age, gender and lung function). Results: Patients reported high levels of resilience and overall good quality of life in all domains. Patient’s resilience was signiﬁcantly higher compared to the large healthy reference group (t = 4.7510, p = 0.0001) and was independent of gender (t = 0.826, p = 0.412), age (r = 0.076, p = 0.573) and lung function (FEV1%, r = 0.165, p = 0.220). Signiﬁcant associations between resilience and CFQ-R domains could be conﬁrmed. Conclusion: Our study is one of the ﬁrst attempts to explicitely test resilience in CF patients and could demonstrate high levels of resilience in these patient group and positive correlations with quality of life. These results suggest that resilience could be seen as an important factor on quality of life and coping with CF. Therefore fostering resilience early in patient’s life should be encouraged.