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WS08.3 Reliability of the Alfred Wellness Score (AweScore) for use in adults with CF
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Workshops / Journal of Cystic Fibrosis 16S1 (2017) S1–S62
CF: the lung, the heart, the gut, the mind: all intertwined WS08.1 How do we navigate the transition from cystic fibrosis to lung transplant? N. West1, A. Thaxton1, E. Tallarico1, E. Bush2, P. Shah1, C. Merlo1. 1Johns Hopkins University, Division of Pulmonary and Critical Care Medicine, Baltimore, United States; 2Johns Hopkins University, Surgery, Baltimore, United States Objectives: Therapies have increased median survival in cystic fibrosis (CF) to 40 years, yet many individuals with CF develop progressive disease. Lung transplantation (LT) remains an effective treatment for end-stage lung disease, as average survival after LT is 7 years. Transition programs from pediatric to adult care for CF have improved outcomes, yet little is known about the transition from CF to LT care. Methods: This is a cross-sectional study surveying CF providers in accredited CF centers in the US. The survey explores CF center experience with LT, transition practices for patients needing LT, and communication between CF team, LT team, and patients. Results: 243 of 632 (38.5%) surveys were completed. 92 (75.4%) of the 122 CF centers, and 50 (74.6%) of the 67 LT centers responded. Half of respondents have over 10 years of experience in CF and 36.4% of respondents have a LT program associated with their CF center. Only 69.4% of CF teams meet formally to discuss patients who may require LT. 34.9% meet weekly, yet 40.4% meet only on an “as needed” basis. The majority of CF teams provide education and assess patient understanding of LT, but only 46.1% have a visit focused on LT, and only 20.9% have a member of the LT team come to that visit. Most centers (77.3%) reported a dedicated LT team meeting, but only 20.3% have a CF caregiver present at this meeting. Respondents reported concern that patients were discussed too late (21.8%), were referred too late (19.3%), became too sick to refer (29.7%), and were impacted negatively due to timing (36.6%). Lastly, 91.1% reported interest in implementing a protocol for LT transition. Conclusion: There is currently a lack of a standardized process for coordinating care from CF to LT; responses from CF caregivers demonstrate a wide variation in practice. Further research is needed to standardize a program that will streamline the transition process and help to improve outcomes for individuals with CF who undergo LT. WS08.2 Exploring the potential impact of gastro-oesophageal reflux on CF lung disease R.W. Lord1,2, J.S. Pearson3, P.J. Barry1, P.J. Whorwell3, R.B. Jones3, P.S. McNamara4,5, R. Beynon4, J.A. Smith2,6, A.M. Jones1. 1Manchester Adult CF Centre, Manchester, United Kingdom; 2University of Manchester, Manchester, United Kingdom; 3University Hospital South Manchester NHS Trust, Department of Gastroenterology, Manchester, United Kingdom; 4 University of Liverpool, Liverpool, United Kingdom; 5Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom; 6University Hospital South Manchester NHS Trust, Respiratory Department, Manchester, United Kingdom Background: Studies have shown large amounts of gastro-oesophageal reflux (GOR) in CF patients, which frequently has characteristics which may favour aspiration – asymptomatic, proximal oesophageal extent and supine. Exposure to refluxate may worsen CF lung disease, as it contains acid, digestive enzymes and microbes, which can include respiratory pathogens. Objectives: To establish if there is a relationship between GOR and CF lung disease. Methods: We are conducting a prospective observational study in stable CF patients, measuring oesophageal reflux with combined pH and impedance ( pH-MII) and reflux symptoms using the RESQ-7 questionnaire. In a preliminary analysis we have collected retrospective data from the medical records for lung function and number of courses of intravenous antibiotics. Results: 21 of 30 recruited completed the study (mean age 28 years, mean FEV1 50% predicted, 19 males). Total number of reflux episodes was
increased in 72% (median 89, IQR 68–132, normal range <75 episodes). High risk reflux (increased proximal and/or supine reflux) was noted in 62%. Non-acid events (median 54, IQR 21–78) were more frequent than acid events (median 36.5, IQR 13–66), but 14/21 were on acid suppression medications. 5/21 had increased total reflux parameters (median 89, IQR 68–98), but on RESQ-7 had no symptoms. Interestingly, number of intravenous antibiotic treatments positively correlated with total reflux events (r = 0.45, p = 0.004) and suggested potential trends with high risk parameters (e.g. proximal reflux r = 0.37, p = 0.1). However there were no correlation between reflux parameters and spirometry. Conclusion: CF patients have large amounts of “high-risk” reflux, and our data suggests a potential relationship between reflux severity and the number of pulmonary exacerbations, represented by the requirement for intravenous antibiotics. To explore this further a reliable biomarker of aspiration, such as sputum pepsin, is required. WS08.3 Reliability of the Alfred Wellness Score (AweScore) for use in adults with CF B.M. Button1,2,3, L.M. Wilson1,2,3, L. Kimmell1,2, A.E. Holland4, F. Finlayson3, E. Williams3, A. Talbot3, D. Keating1,3, J. Wilson1,3. 1Monash University, Department of Medicine, Nursing and Health Sciences, Melbourne, Australia; 2 Alfred Health, Physiotherapy Department, Melbourne, Australia; 3Alfred Health, Cystic Fibrosis Unit, Melbourne, Australia; 4Alfred Health and La Trobe University, Physiotherapy Department, Melbourne, Australia Background: The need for a reliable domain-based user-friendly (short, quick and easy) wellness score for use in the clinical and research settings for adults with CF was identified. Objective: To test the reliability of the recently developed AweScore in adults with CF in the inpatient and outpatient settings during stable periods and exacerbations. Methods: The AweScore was developed with multi-disciplinary team (MDT) input including medical, nursing, nutrition, psychosocial and physiotherapy clinicians in response to the Physiotherapy Department requiring an outcome measure to evaluate CF patient outcomes. The 5 domains have 2 questions each: day and nocturnal cough, sputum volume, energy, appetite, targeted weight, mood, anxiety, sleep amount and quality and general health. Each question is scored by circling a number on a visual analogue scale with anchors from 0 (least state of wellness) to 10 (maximum wellness) with a total score possible 100%. The reliability was tested in the inpatient and outpatient settings with each patient completing 2 questionnaires in a 24 hour period. Results: Forty adults (19 male) with CF mean age(SD): 32.8[9.6] (range 19–56) years; FEV1% 54.2[19.2] (28–105)%; FVC 71.5[16.1] (41–104)% BMI 23.08[6.5] (17.5–26.8) participated. The reliability of the Total Score intraclass correlation was 0.989 (95% confidence interval (CI) 0.979–0.994) with a mean difference between scores of −0.200 (CI −0.971 to 0.571) and limits of agreement −5.019 to 4.619. The ICC domain scores ranged from 0.972 to 0.874 for all the individual domains. The lower bound of the 95% confidence interval exceeded 0.8 for all the domains except mood which was 0.775 to 0.931. The AweScore took an average of one minute to complete and patients reported that it was appealing to undertake and they valued the MDT focus on wellness. Conclusion: The AweScore is a reliable tool for measurement of multidimensional wellness in adults with CF that is appealing to patients. WS08.4 Is there cardiac autonomic dysfunction among patients with cystic fibrosis? E. Hatziagorou1, S. Giannakoulakos2, E. Kouidi2, A. Kampouras1, M. Anifanti2, A. Deligiannis2, J. Tsanakas1. 1School of Medicine University of Thessaloniki, Cystic Fibrosis Unit, Hippokration Hospital, Thessaloniki, Greece; 2 Aristotle University of Thessaloniki, Laboratory of Sports Medicine, Thessaloniki, Greece Objective: To assess heart rate variability (HRV) indices, which are sensitive markers of cardiac autonomic neuropathy, among patients with CF and to correlate them with disease severity.
Workshops / Journal of Cystic Fibrosis 16S1 (2017) S1–S62
CF: the lung, the heart, the gut, the mind: all intertwined WS08.1 How do we navigate the transition from cystic fibrosis to lung transplant? N. West1, A. Thaxton1, E. Tallarico1, E. Bush2, P. Shah1, C. Merlo1. 1Johns Hopkins University, Division of Pulmonary and Critical Care Medicine, Baltimore, United States; 2Johns Hopkins University, Surgery, Baltimore, United States Objectives: Therapies have increased median survival in cystic fibrosis (CF) to 40 years, yet many individuals with CF develop progressive disease. Lung transplantation (LT) remains an effective treatment for end-stage lung disease, as average survival after LT is 7 years. Transition programs from pediatric to adult care for CF have improved outcomes, yet little is known about the transition from CF to LT care. Methods: This is a cross-sectional study surveying CF providers in accredited CF centers in the US. The survey explores CF center experience with LT, transition practices for patients needing LT, and communication between CF team, LT team, and patients. Results: 243 of 632 (38.5%) surveys were completed. 92 (75.4%) of the 122 CF centers, and 50 (74.6%) of the 67 LT centers responded. Half of respondents have over 10 years of experience in CF and 36.4% of respondents have a LT program associated with their CF center. Only 69.4% of CF teams meet formally to discuss patients who may require LT. 34.9% meet weekly, yet 40.4% meet only on an “as needed” basis. The majority of CF teams provide education and assess patient understanding of LT, but only 46.1% have a visit focused on LT, and only 20.9% have a member of the LT team come to that visit. Most centers (77.3%) reported a dedicated LT team meeting, but only 20.3% have a CF caregiver present at this meeting. Respondents reported concern that patients were discussed too late (21.8%), were referred too late (19.3%), became too sick to refer (29.7%), and were impacted negatively due to timing (36.6%). Lastly, 91.1% reported interest in implementing a protocol for LT transition. Conclusion: There is currently a lack of a standardized process for coordinating care from CF to LT; responses from CF caregivers demonstrate a wide variation in practice. Further research is needed to standardize a program that will streamline the transition process and help to improve outcomes for individuals with CF who undergo LT. WS08.2 Exploring the potential impact of gastro-oesophageal reflux on CF lung disease R.W. Lord1,2, J.S. Pearson3, P.J. Barry1, P.J. Whorwell3, R.B. Jones3, P.S. McNamara4,5, R. Beynon4, J.A. Smith2,6, A.M. Jones1. 1Manchester Adult CF Centre, Manchester, United Kingdom; 2University of Manchester, Manchester, United Kingdom; 3University Hospital South Manchester NHS Trust, Department of Gastroenterology, Manchester, United Kingdom; 4 University of Liverpool, Liverpool, United Kingdom; 5Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom; 6University Hospital South Manchester NHS Trust, Respiratory Department, Manchester, United Kingdom Background: Studies have shown large amounts of gastro-oesophageal reflux (GOR) in CF patients, which frequently has characteristics which may favour aspiration – asymptomatic, proximal oesophageal extent and supine. Exposure to refluxate may worsen CF lung disease, as it contains acid, digestive enzymes and microbes, which can include respiratory pathogens. Objectives: To establish if there is a relationship between GOR and CF lung disease. Methods: We are conducting a prospective observational study in stable CF patients, measuring oesophageal reflux with combined pH and impedance ( pH-MII) and reflux symptoms using the RESQ-7 questionnaire. In a preliminary analysis we have collected retrospective data from the medical records for lung function and number of courses of intravenous antibiotics. Results: 21 of 30 recruited completed the study (mean age 28 years, mean FEV1 50% predicted, 19 males). Total number of reflux episodes was
increased in 72% (median 89, IQR 68–132, normal range <75 episodes). High risk reflux (increased proximal and/or supine reflux) was noted in 62%. Non-acid events (median 54, IQR 21–78) were more frequent than acid events (median 36.5, IQR 13–66), but 14/21 were on acid suppression medications. 5/21 had increased total reflux parameters (median 89, IQR 68–98), but on RESQ-7 had no symptoms. Interestingly, number of intravenous antibiotic treatments positively correlated with total reflux events (r = 0.45, p = 0.004) and suggested potential trends with high risk parameters (e.g. proximal reflux r = 0.37, p = 0.1). However there were no correlation between reflux parameters and spirometry. Conclusion: CF patients have large amounts of “high-risk” reflux, and our data suggests a potential relationship between reflux severity and the number of pulmonary exacerbations, represented by the requirement for intravenous antibiotics. To explore this further a reliable biomarker of aspiration, such as sputum pepsin, is required. WS08.3 Reliability of the Alfred Wellness Score (AweScore) for use in adults with CF B.M. Button1,2,3, L.M. Wilson1,2,3, L. Kimmell1,2, A.E. Holland4, F. Finlayson3, E. Williams3, A. Talbot3, D. Keating1,3, J. Wilson1,3. 1Monash University, Department of Medicine, Nursing and Health Sciences, Melbourne, Australia; 2 Alfred Health, Physiotherapy Department, Melbourne, Australia; 3Alfred Health, Cystic Fibrosis Unit, Melbourne, Australia; 4Alfred Health and La Trobe University, Physiotherapy Department, Melbourne, Australia Background: The need for a reliable domain-based user-friendly (short, quick and easy) wellness score for use in the clinical and research settings for adults with CF was identified. Objective: To test the reliability of the recently developed AweScore in adults with CF in the inpatient and outpatient settings during stable periods and exacerbations. Methods: The AweScore was developed with multi-disciplinary team (MDT) input including medical, nursing, nutrition, psychosocial and physiotherapy clinicians in response to the Physiotherapy Department requiring an outcome measure to evaluate CF patient outcomes. The 5 domains have 2 questions each: day and nocturnal cough, sputum volume, energy, appetite, targeted weight, mood, anxiety, sleep amount and quality and general health. Each question is scored by circling a number on a visual analogue scale with anchors from 0 (least state of wellness) to 10 (maximum wellness) with a total score possible 100%. The reliability was tested in the inpatient and outpatient settings with each patient completing 2 questionnaires in a 24 hour period. Results: Forty adults (19 male) with CF mean age(SD): 32.8[9.6] (range 19–56) years; FEV1% 54.2[19.2] (28–105)%; FVC 71.5[16.1] (41–104)% BMI 23.08[6.5] (17.5–26.8) participated. The reliability of the Total Score intraclass correlation was 0.989 (95% confidence interval (CI) 0.979–0.994) with a mean difference between scores of −0.200 (CI −0.971 to 0.571) and limits of agreement −5.019 to 4.619. The ICC domain scores ranged from 0.972 to 0.874 for all the individual domains. The lower bound of the 95% confidence interval exceeded 0.8 for all the domains except mood which was 0.775 to 0.931. The AweScore took an average of one minute to complete and patients reported that it was appealing to undertake and they valued the MDT focus on wellness. Conclusion: The AweScore is a reliable tool for measurement of multidimensional wellness in adults with CF that is appealing to patients. WS08.4 Is there cardiac autonomic dysfunction among patients with cystic fibrosis? E. Hatziagorou1, S. Giannakoulakos2, E. Kouidi2, A. Kampouras1, M. Anifanti2, A. Deligiannis2, J. Tsanakas1. 1School of Medicine University of Thessaloniki, Cystic Fibrosis Unit, Hippokration Hospital, Thessaloniki, Greece; 2 Aristotle University of Thessaloniki, Laboratory of Sports Medicine, Thessaloniki, Greece Objective: To assess heart rate variability (HRV) indices, which are sensitive markers of cardiac autonomic neuropathy, among patients with CF and to correlate them with disease severity.