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194 Sensorineural hearing loss in patients with cystic fibrosis: A cross-sectional study
Posters
8. Complications of CF
S107
191 Lung function and health care resource utilization in patients with cystic fibrosis
193 Isolated lung transplantation (LT) for cystic fibrosis patients with portal hypertension (PHT)
J.L. Rubin-Cahill1 , S. Thayer2 , A. Watkins2 , J. Wagener1 , P. Hodgkins1 , M.S. Schechter3 . 1 Vertex Pharmaceuticals Incorporated, Boston, United States; 2 Optum, Eden Prairie, United States; 3 Virginia Commonwealth University, Richmond, United States
E. Cuquemelle1 , S. Hillaire2 , D. Grenet3 , S. De Miranda3 , F. Parquin1 . 1 Hˆopital Foch, Department of Thoracic Surgery, Suresnes, France; 2 Hˆopital Foch, Department of Medicine, Cystic Fibrosis Center, Suresnes, France; 3 Hˆopital Foch, Department of Pneumology, Cystic Fibrosis Center, Suresnes, France
Objectives: To investigate the association between FEV1 and annual health care resource utilization (HCRU) in patients diagnosed with cystic fibrosis (CF). Methods: Chart data were linked to claims from patients with CF aged 6 years enrolled for 18 months in a US commercial health plan, identified between 7/08 and 5/13. Patient HCRU was calculated for the most recent 12 months (followup) and was considered CF-related if ICD-9-CM diagnosis 277.0x appeared in any position on the claim. The best percent predicted FEV1 from the chart in the 6 months prior to follow-up (baseline) was used to categorize lung dysfunction as severe (<40), moderate (40−<70), or mild (70). Results: In total, 241 patients were included in unadjusted analyses (mean age 23.9 y; 71.8% mild, 23.2% moderate, 5.0% severe). During the follow-up period, 99% of patients had 1 outpatient (OP) office visit with mean 6.5 visits/year; 42.3% had 1 inpatient (IP) stay (mean 1.61) with mean length 13.8 days. A significantly (P = 0.01) higher percentage of severe patients (83.3%) had 1 IP stay vs moderate (44.6%) and mild (38.7%). Severe patients also had significantly (P < 0.05) higher utilization of antidepressants, fluoroquinolone antibiotics, azithromycin, and oxygen. No significant differences by lung dysfunction were observed on OP office visits, utilization of inhaled tobramycin or other inhaled antibiotics, dornase alfa, or pancreatic enzymes. Conclusion: With declining lung function, patients received more IP treatment, medication for depression and oxygen. No differences were observed in OP HCRU and most chronic therapies by lung dysfunction; all categories require intensive management.
Objectives: PHT is the main manifestation of liver disease in adults CF patients. The risk of hepatic decompensation after isolated LT is unknown. The model for end-stage liver disease (MELD) score is not relevant for the indication to combined lungs-liver transplantation. We presented the postoperative outcome of isolated lung transplantation in CF patients with asymptomatic PHT. Methods: From 2008 to 2014, 14 isolated LT (over 299) were proceeded over CF patients with PHT. Results: All Patients had asymptomatic liver disease before LT but all had PHT on the CT scan: splenomegaly (14−19 cm) in 10 patients, portosystemic collaterals in 8, all had portal vein dilatation >14 (diameter 17±2.3 mm). The liver stiffness measurement (LMS) was elevated 18±8 kPa when in our adult CF center median LSM was 5.9 kPa in 241 CF patients. Thirteen patients had normal liver and renal function. Gastroesophageal varices were absents in 10 screened patients. After LT, 4 patients required ECMO, 6 developed easy to treat ascites associated with transient renal insufficiency (4) or intestinal complications (2). One developed transient ascitis 7 months after LT associated with sepsis and renal dysfunction. Hepatocellular function remain normal in all patients. Every patient is still alive (mean time 42±22 months). Conclusion: Carefully selected CF patients with end-stage lung disease and portal hypertension can tolerate isolated lung transplantation. The non-invasive evaluation of the severity of PHT has to be evaluated. Prospective multicentric study is need to clearly define the indications of combined lung–liver transplantation.
192 ABPAs in Pseudomonas aeruginosa colonized CF patients
194 Sensorineural hearing loss in patients with cystic fibrosis: A cross-sectional study
F. De Baets1 , L. De Keyzer1 , P. Schelstraete1 , S. Van Daele1 , S. Wanyama2 . 1 Ghent University Hospital, Paediatric Pulmonology, Ghent, Belgium; 2 Scientifique Institute of Public Health, Brussels, Belgium Objectives: Allergic Bronchopulmonary Aspergillosis syndrome (ABPAs) occurs in 10 to 15% of the CF patients. We intend to study facilitators and consequences of ABPAs in P. aeruginosa (Ps) colonized CF patients. Methods: Sixty four ABPAs and Ps colonized patients matched for age and gender were compared to 128 controls. FEV1, FVC, infectious exacerbations and BMI were studied 10 years before until 10 years after the index year. Results: On the index year no differences were found for BMI, FVC and FEV1. Patients with ABPAs have a higher prevalence of CFRD and significantly more were on treatment with inhaled corticosteroids, RhDnase, azithromycin and inhaled tobramycin. Cumulative values (FEV1, FVC, days of IV antibiotics) over the period before the index year were similar, over the 10 years period after the index year significantly lower values for FEV1 (p < 0.03) were found in the ABPAs group. Before the index year no difference in IV antibiotic treatment days was recorded, after the index year patients with ABPAs showed significantly more treatment days (p = 0.01). In ABPAs patients more IV antibiotic days were found after the index year compared to before (p < 0.001) which was not the case in the control group (p = 0.56). Significantly more patients with ABPAs had at least one positive culture for A. fumigatus (p < 0.009). Conclusion: In chronic Ps colonized CF patients lower lung function values are not a facilitating factor for ABPAs. Inhaled corticosteroids, RhDnase, azithromycin and inhaled tobramycin were significantly more prevalent in the ABPA group. ABPAs patients needed significantly more antibiotics, in contrast with controls, after than before the index year.
K. Van Hoorenbeeck1,2 , N. De Bruyne2 , M. Lequesne1 , P. Van de Heyning2,3 , S. Verhulst1,2 , V. Van Rompaey2,3 . 1 Antwerp University Hospital, Pediatrics, Edegem, Belgium; 2 University of Antwerp, Faculty of Medicine and Health Sciences, Antwerp, Belgium; 3 Antwerp University Hospital, Otorhinolaryngology and Head & Neck Surgery, Edegem, Belgium Objectives: In cystic fibrosis (CF), airway infections caused by Gram-negative bacteria are often observed and commonly treated with aminoglycosides (AG). These antibiotics are known for their ototoxicity, making CF patients prone to the development of sensorineural hearing loss (SNHL) and vestibular hypofunction. The aim of this study was to determine the prevalence of SNHL in CF patients and its relationship to AG use. Methods: We performed a cross-sectional study on audiometry data obtained in a pediatric CF clinic. Medical records between 2012 and 2014 were reviewed. All patients had audiometry data available. Data were collected on age, gender, audiological measures and the use of AG. Results: 44 patients were included consecutively. Mean age was 13±7 years. Male:female ratio was 1:1. AG were administered at least once in 43%, 14% received more than 5 AG courses. Hearing loss was reported by pure-tone average of 0.5, 1 and 2 kHz over 25 dB (0% SNHL), >2 frequencies with >25 dB (14% SNHL), 1 frequency >25 dB (25% SNHL), >2 adjacent frequencies >15 dB (39% SNHL) and >25 dB at 8 kHz (17% SNHL). A statistically significant effect of the frequent use of AG (>5 courses) was only observed in bilateral SNHL at 8 kHz (p = 0.03). Conclusion: SNHL was reported in an important proportion of CF patients. We could only detect an effect of AG use when looking at bilateral SNHL at 8 kHz. The effects of AG on SNHL are smaller than previously described. This might be related to the lack of selection bias in this study.
8. Complications of CF
S107
191 Lung function and health care resource utilization in patients with cystic fibrosis
193 Isolated lung transplantation (LT) for cystic fibrosis patients with portal hypertension (PHT)
J.L. Rubin-Cahill1 , S. Thayer2 , A. Watkins2 , J. Wagener1 , P. Hodgkins1 , M.S. Schechter3 . 1 Vertex Pharmaceuticals Incorporated, Boston, United States; 2 Optum, Eden Prairie, United States; 3 Virginia Commonwealth University, Richmond, United States
E. Cuquemelle1 , S. Hillaire2 , D. Grenet3 , S. De Miranda3 , F. Parquin1 . 1 Hˆopital Foch, Department of Thoracic Surgery, Suresnes, France; 2 Hˆopital Foch, Department of Medicine, Cystic Fibrosis Center, Suresnes, France; 3 Hˆopital Foch, Department of Pneumology, Cystic Fibrosis Center, Suresnes, France
Objectives: To investigate the association between FEV1 and annual health care resource utilization (HCRU) in patients diagnosed with cystic fibrosis (CF). Methods: Chart data were linked to claims from patients with CF aged 6 years enrolled for 18 months in a US commercial health plan, identified between 7/08 and 5/13. Patient HCRU was calculated for the most recent 12 months (followup) and was considered CF-related if ICD-9-CM diagnosis 277.0x appeared in any position on the claim. The best percent predicted FEV1 from the chart in the 6 months prior to follow-up (baseline) was used to categorize lung dysfunction as severe (<40), moderate (40−<70), or mild (70). Results: In total, 241 patients were included in unadjusted analyses (mean age 23.9 y; 71.8% mild, 23.2% moderate, 5.0% severe). During the follow-up period, 99% of patients had 1 outpatient (OP) office visit with mean 6.5 visits/year; 42.3% had 1 inpatient (IP) stay (mean 1.61) with mean length 13.8 days. A significantly (P = 0.01) higher percentage of severe patients (83.3%) had 1 IP stay vs moderate (44.6%) and mild (38.7%). Severe patients also had significantly (P < 0.05) higher utilization of antidepressants, fluoroquinolone antibiotics, azithromycin, and oxygen. No significant differences by lung dysfunction were observed on OP office visits, utilization of inhaled tobramycin or other inhaled antibiotics, dornase alfa, or pancreatic enzymes. Conclusion: With declining lung function, patients received more IP treatment, medication for depression and oxygen. No differences were observed in OP HCRU and most chronic therapies by lung dysfunction; all categories require intensive management.
Objectives: PHT is the main manifestation of liver disease in adults CF patients. The risk of hepatic decompensation after isolated LT is unknown. The model for end-stage liver disease (MELD) score is not relevant for the indication to combined lungs-liver transplantation. We presented the postoperative outcome of isolated lung transplantation in CF patients with asymptomatic PHT. Methods: From 2008 to 2014, 14 isolated LT (over 299) were proceeded over CF patients with PHT. Results: All Patients had asymptomatic liver disease before LT but all had PHT on the CT scan: splenomegaly (14−19 cm) in 10 patients, portosystemic collaterals in 8, all had portal vein dilatation >14 (diameter 17±2.3 mm). The liver stiffness measurement (LMS) was elevated 18±8 kPa when in our adult CF center median LSM was 5.9 kPa in 241 CF patients. Thirteen patients had normal liver and renal function. Gastroesophageal varices were absents in 10 screened patients. After LT, 4 patients required ECMO, 6 developed easy to treat ascites associated with transient renal insufficiency (4) or intestinal complications (2). One developed transient ascitis 7 months after LT associated with sepsis and renal dysfunction. Hepatocellular function remain normal in all patients. Every patient is still alive (mean time 42±22 months). Conclusion: Carefully selected CF patients with end-stage lung disease and portal hypertension can tolerate isolated lung transplantation. The non-invasive evaluation of the severity of PHT has to be evaluated. Prospective multicentric study is need to clearly define the indications of combined lung–liver transplantation.
192 ABPAs in Pseudomonas aeruginosa colonized CF patients
194 Sensorineural hearing loss in patients with cystic fibrosis: A cross-sectional study
F. De Baets1 , L. De Keyzer1 , P. Schelstraete1 , S. Van Daele1 , S. Wanyama2 . 1 Ghent University Hospital, Paediatric Pulmonology, Ghent, Belgium; 2 Scientifique Institute of Public Health, Brussels, Belgium Objectives: Allergic Bronchopulmonary Aspergillosis syndrome (ABPAs) occurs in 10 to 15% of the CF patients. We intend to study facilitators and consequences of ABPAs in P. aeruginosa (Ps) colonized CF patients. Methods: Sixty four ABPAs and Ps colonized patients matched for age and gender were compared to 128 controls. FEV1, FVC, infectious exacerbations and BMI were studied 10 years before until 10 years after the index year. Results: On the index year no differences were found for BMI, FVC and FEV1. Patients with ABPAs have a higher prevalence of CFRD and significantly more were on treatment with inhaled corticosteroids, RhDnase, azithromycin and inhaled tobramycin. Cumulative values (FEV1, FVC, days of IV antibiotics) over the period before the index year were similar, over the 10 years period after the index year significantly lower values for FEV1 (p < 0.03) were found in the ABPAs group. Before the index year no difference in IV antibiotic treatment days was recorded, after the index year patients with ABPAs showed significantly more treatment days (p = 0.01). In ABPAs patients more IV antibiotic days were found after the index year compared to before (p < 0.001) which was not the case in the control group (p = 0.56). Significantly more patients with ABPAs had at least one positive culture for A. fumigatus (p < 0.009). Conclusion: In chronic Ps colonized CF patients lower lung function values are not a facilitating factor for ABPAs. Inhaled corticosteroids, RhDnase, azithromycin and inhaled tobramycin were significantly more prevalent in the ABPA group. ABPAs patients needed significantly more antibiotics, in contrast with controls, after than before the index year.
K. Van Hoorenbeeck1,2 , N. De Bruyne2 , M. Lequesne1 , P. Van de Heyning2,3 , S. Verhulst1,2 , V. Van Rompaey2,3 . 1 Antwerp University Hospital, Pediatrics, Edegem, Belgium; 2 University of Antwerp, Faculty of Medicine and Health Sciences, Antwerp, Belgium; 3 Antwerp University Hospital, Otorhinolaryngology and Head & Neck Surgery, Edegem, Belgium Objectives: In cystic fibrosis (CF), airway infections caused by Gram-negative bacteria are often observed and commonly treated with aminoglycosides (AG). These antibiotics are known for their ototoxicity, making CF patients prone to the development of sensorineural hearing loss (SNHL) and vestibular hypofunction. The aim of this study was to determine the prevalence of SNHL in CF patients and its relationship to AG use. Methods: We performed a cross-sectional study on audiometry data obtained in a pediatric CF clinic. Medical records between 2012 and 2014 were reviewed. All patients had audiometry data available. Data were collected on age, gender, audiological measures and the use of AG. Results: 44 patients were included consecutively. Mean age was 13±7 years. Male:female ratio was 1:1. AG were administered at least once in 43%, 14% received more than 5 AG courses. Hearing loss was reported by pure-tone average of 0.5, 1 and 2 kHz over 25 dB (0% SNHL), >2 frequencies with >25 dB (14% SNHL), 1 frequency >25 dB (25% SNHL), >2 adjacent frequencies >15 dB (39% SNHL) and >25 dB at 8 kHz (17% SNHL). A statistically significant effect of the frequent use of AG (>5 courses) was only observed in bilateral SNHL at 8 kHz (p = 0.03). Conclusion: SNHL was reported in an important proportion of CF patients. We could only detect an effect of AG use when looking at bilateral SNHL at 8 kHz. The effects of AG on SNHL are smaller than previously described. This might be related to the lack of selection bias in this study.