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2 9 T H A N N UA L N O R T H A M E R I C A N FINAL PROGRAM OCTOBER 8-10, 2015 PHOENIX CONVENTION CENTER PHOENIX, AZ PROGRAM COMMITTEE CONFERENCE CHAIRS Amanda R. Leonard, M.P.H., R.D., C.D.E. Wayne J. Morgan, M.D. MEMBERS Frank J. Accurso, M.D. Wayne J. Morgan, M.D. Cynthia L. Adams A.P. Naren, Ph.D. Robert J. Beall, Ph.D. Michael R. Narkewicz, M.D. Debbie Benitez, R.N., M.S.N., ACNP Kecia L. Nelson, M.S.W., LCSW Scott M. Blackman, M.D., Ph.D. Matthew Nippins, P.T., D.P.T., C.C.S. Michael P. Boyle, M.D., FCCP Christopher M. Penland, Ph.D. Preston W. Campbell, III, M.D. Raymond J. Pickles, Ph.D. André M. Cantín, M.D. Alexandra L. Quittner, Ph.D. J.P. Clancy, M.D. Felix Ratjen, M.D., Ph.D., FRCPC Mitchell L. Drumm, Ph.D. Clement L. Ren, M.D. Jordan M. Dunitz, M.D. Kim Reno, M.S.W., LISW Mary B. Dwight Kristin A. Riekert, Ph.D. Raymond A. Frizzell, Ph.D. Steven M. Rowe, M.D., MSPH Antoinette Gardner, B.S.N., M.Ed., A.E.C., CCRC Ronald C. Rubenstein, M.D., Ph.D. Cynthia A. George, M.S.N., F.N.P., B.C. Kathryn A. Sabadosa, M.P.H. John W. Hanrahan, Ph.D. Lisa Saiman, M.D., M.P.H. Heather Y. Hathorne, M.A.E., R.R.T., CCRC Sarah Jane Schwarzenberg, M.D. Manu Jain, M.D., M.Sc. Richard H. Simon, M.D. Elizabeth Joseloff, Ph.D. Pradeep K. Singh, M.D. Michael W. Konstan, M.D. William R. Skach, M.D. Amanda R. Leonard, M.P.H., R.D., C.D.E. Eric J. Sorscher, M.D. Mary K. Lester, R.R.T., R.C.P. Anne Stephenson, M.D., Ph.D. Andrew J. Lipton, M.D., M.P.H., T.M. David A. Stoltz, M.D., Ph.D. John J. LiPuma, M.D. Philip Thomas, Ph.D. Paula Lomas, M.A.S., R.N., CCRP Katherine L. Tuggle, Ph.D. Bruce C. Marshall, M.D. Jeffrey J. Wine, Ph.D. Martin Mense, Ph.D. David C. Young, Pharm.D. 1 | 2015 North American CF Conference THANK YOU TO OUR EXHIBITORS & SUPPORTERS Platinum Supporters Silver Supporters CF Services, A Walgreens Alliance Pharmacy............................ Booth #600 Alcresta Pharmaceuticals, Inc. ................................... Booth #915 CVS Health.............................................................................. Booth # 910 Conference Bags Pens in Conference Bag Relaxation Station – Booth #604 Showcase Theater – October 8, 12:10 p.m. – 12:30 p.m. Gilead Sciences, Inc............................ Booth #401, 511 & 316 Hotel Key Cards Exhibit Hall Hospitality Junior Investigators Mobile App Workshops #15, 20, 22 & 26 Symposium #5, 12 & 18 Hydration Station Showcase Theater – October 8, 12:40 p.m. – 1:00 p.m. Genentech ................................................................................. Booth #411 Novartis Pharmaceuticals .............................................. Booth #901 Pfizer, Inc. ........................................................................................ No Booth TLCRx Pharmacy ................................................................... Booth #317 Corporate Supporters Vertex Pharmaceuticals ................ Booth #611, 519 & 518 Live Streaming Diamond Supporters AbbVie.................................................. Booth #301 & 311 Speakers & Fellows Reception Gold Supporters Actavis Pharma, Inc. ...................................... Booth #1019 & 918 Power Bar Ambry Genetics...................................................................... Booth #501 Anthera Pharmaceuticals................................................... No Booth Chiesi USA, Inc. .................................................................... Booth #810 Electromed, Inc..................................................................... Booth #610 Hill-Rom........................................................................................ Booth #801 International Biophysics Corporation.................. Booth #711 IV Solutions............................................................................... Booth #816 Nivalis Therapeutics, Inc................................................ Booth #811 PARI Pharma & PARI Respiratory Equipment. Booth #310 Pharmaceutical Specialties, Inc.................................. Booth #817 PTC Therapeutics.................................................................. Booth #515 Medical Bag Raptor Pharmaceuticals........................................................ No Booth Exhibitors & Supporters as of September 16, 2015 Callion Pharma........................................................................... Booth #815 Nationwide Children’s Hospital.......................................... No Booth Cystic Fibrosis & Freedom Pharmacies, Inc. ....... Booth #302 nnd Medical Technologies................................................. Booth #718 Dignity Health............................................................................ Booth #618 Philips Respironics................................................................... Booth #900 DKBmed ........................................................................................ Booth #419 PolyChrome Medical............................................................. Booth #914 Diplomat Pharmacy, Inc. .................................................... Booth #902 PulmoTrace, Inc. ................................................................... Booth #1011 Dynamiks Health Care, LLC.............................................. Booth #719 Rare Patient Voice, LLC....................................................... Booth #717 ELITechGroup, Inc. ................................................................. Booth #715 RespInnovation....................................................................... Booth #1013 Elsevier, Office of CME........................................................ Booth #912 RespirTech..................................................................................... Booth #300 Foundation Care Pharmacy.............................................. Booth #619 Rho Research.............................................................................. Booth #813 Hayek Medical Devices........................................................ Booth #318 Valley Children’s Healthcare.......................................... Booth #1010 Illumina, Inc. ............................................................................... Booth #904 Vitalograph, Inc. ...................................................................... Booth #616 Med Systems, Inc. .................................................................. Booth #505 Westmed, Inc. ........................................................................... Booth #911 Monaghan Medical Corp................................................... Booth #614 Wiley Publishing....................................................................... Booth #314 MVW Nutritionals, Inc.......................................................... Booth #304 2 | 2015 North American CF Conference SPECIAL APPRECIATION TO OUR PLATINUM LEVEL SUPPORTERS 3 | 2015 North American CF Conference TAB SHEET 1 CONCIERGE TAB SHEET CONCIERGE Bag Check UPS Store – PCC West Building, Level 100 Bag check will be available at the UPS Store in the Phoenix Convention Center, West Building, 100 Level. Hours of Operation Wednesday, October 7 7:00 a.m. - 6:00 p.m. Thursday, October 8 7:00 a.m. - 6:00 p.m. Friday, October 9 7:00 a.m. - 6:00 p.m. Saturday, October 10 7:00 a.m. - 4:00 p.m. Bag check service only, available for $5 per bag checked. Official Conference Hotels Sheraton Downtown Phoenix 602-262-2500 Hyatt Regency Phoenix 602-525-1234 Renaissance Phoenix Downtown 602-333-0000 Hotel Palomar Phoenix 602-253-6633 The Westin Phoenix Downtown 602-429-3500 Hotel San Carlos 602-253-4121 The Springhill Suites Phoenix Downtown 602-307-9929 Holiday Inn Express & Suites Phoenix Downtown-Ballpark 602-452-2020 Phoenix Hotel and Suites 602-595-4444 Wyndham Garden Phoenix Midtown 602-604-4900 Hampton Inn Phoenix Midtown 602-200-0990 Hilton Garden Inn Phoenix Midtown 602-279-9811 Fairfield Inn & Suites Midtown Phoenix 602-716-9900 Hilton Phoenix Suites 602-222-1111 *Hilton Phoenix Airport 480-894-1600 *Crowne Plaza Phoenix Airport 602-273-7778 *Courtesy Hotels Security Security for all NACFC attendees is a priority and attendees must do their part to help facilitate personal safety and the safety of others. Attendees must wear their registration badge at all times when at the Phoenix Convention Center. We encourage attendees to remove their badges when not in official conference facilities. Session Overflow Rooms Rooms 125 AB & 128 AB Limited seating, PowerPoint slides and audio for open sessions exceeding seating capacity will be offered in Rooms 125 AB & 128 AB at the Phoenix Convention Center, North Building. Sessions selected for overflow presentations will be based on first and greatest need. All open session seating is limited to capacity and is available on a first-come, first-served basis. 5 | 2015 North American CF Conference CONCIERGE Shuttle Services Transportation Management Services is the official NACFC transportation provider. Complimentary shuttle service will be provided from Wednesday, October 7 – Saturday, October 10 from the PCC and Sheraton to the select conference hotels listed below. This service does not extend to include ancillary meetings that are not a part of the official NACFC program. Select Conference Hotels Hilton Garden Inn Phoenix Midtown Wyndham Garden Phoenix Midtown Phoenix Hotel and Suites Hampton Inn Phoenix Midtown Fairfield Inn & Suites Midtown Phoenix Hilton Phoenix Suites Shuttle service will run approximately every 15 minutes between the PCC, Sheraton and select conference hotels according to the schedule below. A shuttle representative will be onsite at the Phoenix Convention Center for any questions or concerns. ADA-accessible vehicles are available upon request; individuals will need to see a representative for further information. Date Times Pick-up and Drop-off Locations Wednesday, October 7 6:30 a.m. – 10:30 a.m. 5:00 p.m. – 8:00 p.m. 8:00 p.m. – 10:00 p.m. 6:30 a.m. – 10:30 a.m. 4:30 p.m. – 8:30 p.m. 8:00 p.m. – 10:00 p.m. 6:30 a.m. – 10:30 a.m. 4:00 p.m. – 8:00 p.m. 8:00 p.m. – 10:00 p.m. 6:30 a.m. – 10:30 a.m. 4:00 p.m. – 8:00 p.m. 8:00 p.m. – 1:00 a.m. PCC & select Conference Hotels PCC & select Conference Hotels Sheraton Hotel & select Conference Hotels PCC & select Conference Hotels PCC & select Conference Hotels Sheraton Hotel & select Conference Hotels PCC & select Conference Hotels PCC & select Conference Hotels Sheraton Hotel & select Conference Hotels PCC & select Conference Hotels PCC & select Conference Hotels All Official Conference Hotels Thursday, October 8 Friday, October 9 Saturday, October 10 Phoenix Light Rail The Phoenix Light Rail provides service between most of the Official Conference Hotels, as well as the Courtesy Hotels listed below, and the Phoenix Convention Center for a fee of $4.00 all day. Hours of operation: Sunday-Thursday: 5:00 a.m. – 11:00 p.m. and Friday-Saturday: 5:00 a.m. – 2:00 a.m. Courtesy Hotels Shuttle service will not be available to the Hilton Phoenix Airport Hotel or the Crowne Plaza Phoenix Airport Hotel. These hotels are located directly on the Phoenix Metro Light Rail Line. Courtesy Hotels Hilton Phoenix Airport Crowne Plaza Phoenix Airport 6 | 2015 North American CF Conference CONCIERGE Phoenix Pedicabs Pedicabs are readily available in Downtown Phoenix and provide a great method of getting between the hotels and the convention center, or just around the downtown area for a nominal cost. Phoenix Ambassador Program The members of the Phoenix Ambassador Program have proudly served Downtown Phoenix for more than 20 years. Clad in orange shirts, these roving concierges are on duty seven days a week, 365 days a year to help you — whether that means making a lunch recommendation, walking you to your hotel or simply offering helpful directions to get you where you need to go. Ambassadors are on duty Monday-Friday from 6:30 a.m. – 11:00 p.m. and Saturday-Sunday from 8:00 a.m. – 11:00 p.m. Speaker Ready Room Room 228 AB The Speaker Ready room is located at the Phoenix Convention Center, North Building, in Room 228 AB. Speakers with PowerPoint presentations MUST report to the Speaker Ready Room as soon after their arrival as possible, and no later than FOUR hours before their session begins. This is critical to provide ample time to review and confirm final presentations, troubleshoot any embedded videos, links, sound needs or other components, in an effort to help ensure successful sessions. Session leaders who want to learn how to operate the speaker timer should also visit the Speaker Ready Room. Hours of Operation Phoenix Convention Center for All Presenters Tuesday, October 6 3:30 p.m. - 7:30 p.m. Wednesday, October 7 7:00 a.m. - 6:00 p.m. Thursday, October 8 7:00 a.m. - 6:00 p.m. Friday, October 9 7:00 a.m. - 6:00 p.m. Saturday, October 10 7:00 a.m. - 4:30 p.m. 7 | 2015 North American CF Conference TAB SHEET 2 POLICIES TAB SHEET POLICIES CF Foundation Infection Prevention & Control Policy Important Note to NACFC Participants: Infection Prevention and Control The health and well-being of people with CF is the primary concern of the Cystic Fibrosis Foundation — it is at the heart of all we do. To help protect the health of people with CF, the Foundation has an Infection Prevention and Control policy in place for all of its events and meetings. The policy permits the participation of one person with CF at indoor events. This will reduce the serious risk of cross-infection between people with the disease. Attending NACFC 2015 In keeping with this policy, the NACFC Program Planning Committee may invite one person with CF to speak in venue and time specific sessions at the 2015 conference. These invitations will be for event specific sessions only. We strongly recommend that invited speakers stay only for your session. We invite all who are unable to attend the conference in person to view select live-streamed sessions. For more information about which sessions will be live-streamed and how you can register to join online, please visit the NACFC website www.nacfconference.org. Note: To reduce the risk of people with CF spreading or acquiring dangerous germs, we strongly recommend people with CF not attend this meeting. Please be aware that others with CF might choose to attend NACFC and NACFC-related activities without notifying event organizers. We strongly discourage this; however, we cannot guarantee others with CF will not be present. Important Reminder for All Participants As a reminder, here are some simple yet effective steps you can take to help minimize the spread of germs: 1. Clean your hands frequently with soap and water or alcohol-based hand gel. 2. Use a tissue when coughing or sneezing, throw it away, then clean your hands. 3. Avoid touching your eyes, nose or mouth. 4. Stay away from others and seek medical attention if you are ill. 5. Maintain a distance of at least six feet from those with a respiratory infection. 6. Use an elbow bump instead of a handshake as an alternative greeting. Thank you for your cooperation in helping to safeguard the health of those who attend our conference. For more information, please contact the CF Foundation at (800) FIGHT CF or visit www.cff.org. You can also view a webcast about the Foundation’s Infection Control Policy. 9 | 2015 North American CF Conference POLICIES Conference Proceedings Publication The conference proceedings were published as a supplement to Pediatric Pulmonology. The publication is available on the internet on the Wiley Online Library (journal subscription not required) and a hard copy and a USB drive version were mailed to those who registered before September 11 and requested it during the registration process. A limited number of hard copies may be available to those who did not request it previously or who wish to purchase an additional copy (one copy per person is included in the registration fee, except for One-day and Exhibit Only registrants). Please visit the Pediatric Pulmonology counter in the North 300 Lobby at the Phoenix Convention Center to obtain a book. If available, the cost for an additional book is $150. Electronic Devices PLEASE NOTE THAT ATTENDEES ARE STRICTLY PROHIBITED FROM TAKING PHOTOS OF SLIDES AND/OR POSTERS. As a courtesy to others please silence cell phones and tablets during sessions. Photography Disclaimer The CF Foundation will take photographs at the 2015 NACFC to reproduce and to distribute in NACFC educational, news or promotional materials and other CF Foundation publications, whether in print, electronic or other media, including the conference website. By participating in the NACFC, you permit the CF Foundation to use, reproduce and distribute photographs taken of you for such purposes and waive your legal rights to pursue any cause of action against the CF Foundation related to such photographs. If you have any concerns regarding the use of photographs taken of you during the NACFC, please contact (301) 907-2513 or [email protected]. Special Interest & Related Study Groups The Special Interest and Related Study Group meetings will be held at the Sheraton Phoenix Downtown Hotel and other official conference hotels. See the fold-out insert in the back pocket of this program for more information and exact locations. Ticketed Events Attendees who pre-registered for limited attendance sessions (Special Classes, Short Courses, Brown Bags, Roundtables, Consultation Clinics, Junior Investigators, Closing Event, Speakers/Fellows Reception), received printed tickets with their badge during the self check-in process. Attendees must bring tickets to ticketed sessions. Attendees will not be admitted to the session without a ticket. Attendees who are not registered for one of these sessions/events and wish to attend, should check availability at the customer service desk in the Registration area in the North 300 Lobby at the Phoenix Convention Center. Attendees who pre-registered for a session or the Closing Event and cannot attend, should return their ticket(s) to the customer service desk so that the seat can be used by another attendee. Additionally, attendees wishing to attend a full session may arrive at the session and wait 'stand-by'. Once the session is underway, attendees may be admitted if space permits on a first-come, first-served basis. 10 | 2015 North American CF Conference TAB SHEET 3 PCC MAP TAB SHEET DOWNTOWN PHOENIX NACFC OFFICIAL CONFERENCE HOTELS HOTELS NOT SHOWN ON MAP* · Phoenix Hotel and Suites · Wyndham Garden Phoenix Midtown · Hampton Inn Phoenix Midtown · Hilton Garden Inn Phoenix Midtown · Fairfield Inn & Suites Midtown Phoenix · Hilton Phoenix Suites · Hilton Phoenix Airport · Crowne Plaza Phoenix Airport *3 miles from Phoenix Convention Center 11 | 2015 North American CF Conference PHOENIX CONVENTION CENTER FLOOR PLANS West Building North Building Plenary Sessions Hall AB Attendee Resource Center Speaker & Fellows Reception Hall C Meeting Rooms 301 A, BC, D Registration Exhibition Hall Junior Investigators Rooms 212-213 300 Level Press Room Room 205 West Building North Building Meeting Rooms 301 A,BC,D Registration Plenary Sessions Exhibit Hall & Posters Attendee Resource Center Speaker Ready Room 228 AB Mee 200 Level ting West Building Roo 21-2 ms 2 Executive Conference Center Closing Event Ballroom 120 ABCD 32 Short Courses Brown Bag Lunches Consultations Clinics Rooms 101-106 Exhibits & Posters E-Connect Power Bar Wi-Fi Lounge Showcase Theater Conference Cafe Hall E Ballroom 100 Level ting Mee Bag Check UPS Store Entrance WEST s 121 West Building North Building Short Courses Brown Bag Lunches Consultations Clinics Rooms 101-106 Meeting Rooms 121-132 Closing Event 120 ABCD -132 Shuttle Buses 3rd Street NORTH m Roo Meeting Rooms Entrance NORTH North Building Executive Speaker Ready Room Conference Center 228 AB Press Room Meeting Rooms 221-232 Junior Investigators Rooms 201-213 12 | 2015 North American CF Conference EVERYTHING THEY NEED WHEN THEY NEED IT™ Every journey is different— every moment is important In 1988, the Cystic Fibrosis (CF) Foundation established CF Services to offer access to CF medications and pharmacy services. Now, as a Walgreens Alliance Pharmacy, we are building on that tradition through the continued commitment to the CF community. For comprehensive care through all of life’s moments, entrust your patients to CF Services. Visit booth 600 to learn more. Proud supporter of the 29th Annual NACFC. ©2015 Walgreen Co. All rights reserved. 66323-373-0815 Pfizer's Rare Disease Research Unit is Proud to sponsor the 2015 NACFC in support of individuals and families living with CF. Working Together in Rare Diseases TAB SHEET 4 PROGRAM TAB SHEET GENERAL INFORMATION Discipline / Track Key To help you decide & prioritize which sessions to attend, each session title, where applicable, is noted with the following information: Discipline Track The name of each primary discipline track as represented by the respective NACFC Program Planning Committee subcommittee is listed at the beginning of each session title, where applicable, in this program. Primary Discipline Key APP&D = Airways Physiology, Pathophysiology & Defense CFTR = CFTR CLIN = Clinical GC = Genetic Counselor GMS = Genetics & Model Systems INF/MIC = Infection/Microbiology NRS = Nursing NTR = Nutrition NT = New Therapies PHARM = Pharmacist PT & RT = Physical & Respiratory Therapy PSY = Psychologist/ Psychiatrist RC = Research Coordinator SW/PSYCH = Social Work/Psychosocial Session Category The session category listed at the end of each session title in this program section is based upon the following definitions: Care: Any discipline (e.g., nursing, respiratory therapy, psychology) that is based in the clinical setting (e.g., clinic, hospital, home care) whose purpose is to share knowledge & processes designed to improve the care of people with CF. Research: Any discipline that is based in the laboratory or clinical research setting whose purpose is to expand knowledge & improve the understanding of CF. Combination: This topic combines care & research elements. The 2015 NACF conference proceedings publication will be available on a USB drive, in addition to the printed form. Attendees will also be able to view a copy of each accepted abstract at the NACFC Attendee Resource Center. Icon Key Denotes sessions recorded for online viewing. Denotes live streaming session. 15 | 2015 North American CF Conference PROGRAM AT-A-GLANCE TUESDAY, OCTOBER 6 THURSDAY, OCTOBER 8 3:30 p.m. - 7:30 p.m. General & Exhibitor Registration (PCC) 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (PCC) 3:30 p.m. - 7:30 p.m. General & Exhibitor Registration at The Sheraton Phoenix Downtown (Sheraton) 7:00 a.m. - 7:30 a.m. Continental Breakfast for Caregiver & PGR Attendees Large Group Exhibitor Registration (By Appointment Only) 7:30 a.m. - 9:30 a.m. Physicians Grand Rounds WEDNESDAY, OCTOBER 7 7:30 a.m. - 9:30 a.m. Caregivers Sessions by Discipline: Genetic Counselors/ Nrs-AP/Nrs/Ntr/Pharm/PT & RT/RC/SW-PSYCH 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (PCC) 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (Sheraton) 8:00 a.m. - 5:00 p.m. Special Classes 1) NRS: Nuts & Bolts for New CF Caregivers (E. Bryson, L. Feenan) 2) PT & RT: Minimizing Postural Deficits: Physical Therapy Musculoskeletal & Neuromotor Control Interventions for Children & Adults With CF ((M. Massery) 8:00 a.m. - 10:00 p.m. Special Interest & Related Study Groups 11:00 a.m. - 3:00 p.m. Psychosocial Working Group 12:30 p.m. - 2:30 p.m. CF Registry Meeting 9:00 a.m. - 10:00 a.m. Poster Set-up 9:30 a.m. - 10:00 a.m. Coffee Break 10:00 a.m. - 11:45 a.m. Workshop Session I 1) CFTR: Insights Into CFTR Biology (Z. Bozoky, P. Thibodeau) 2) GMS-APP&D: New Advances in Cell & Animal Models (M. Bagnat, L. Clarke) 3) NT: Novel Targets & Outcome Measures (J. Chmiel, T. Laguna) 4) INF/MIC: Epidemiology & Management of Infection (T. Kidd, S. Heltshe) 5) CLIN: Bone & Endocrine/Non-diabetes (M. Putman, V. Tangpricha) 3:30 p.m. - 7:30 p.m. Short Courses 6) CLIN: Epidemiology of CF (C. Goss, S. Stanojevic) 1) CLIN: Eating Behaviors in CF (S. Powers, E. Reid) 2) CLIN: Nuts & Bolts for the Research Coordinator (E. Hartigan, S. Hurban) 3) CLIN: Advanced Drug Therapy for CF (K. Olivier, D. Young) 4) SW/PSYCH: The Essentials of CF Psychopharmacology (A. Georgiopoulos, B. Smith) 5) SW/PSYCH: Advanced Cognitive Behavioral Strategies for Anxiety & Depression (C. Duncan, D. Friedman) 6) NTR: CF Nutrition 101: Medical Nutrition Therapy Optimizes CF Outcomes Across the Lifespan, Infants to Adults (K. McDonald, S. Michel) 7) PT & RT: Beginning Airway Clearance for the New CF Team Member (B. Button, M. McIlwaine) 3:30 p.m. -7:30 p.m. Special Session by Invitation Only: Introduction to CF Quality Improvement: The Virtual Improvement Program-Fundamentals ‘VIP-F’ Launch (R. Messier, B. Oliver, K. Petren) 4:00 p.m. - 6:00 p.m. Poster Set-up 8:00 a.m. - 10:00 p.m. Special Interest & Related Study Groups 7) CLIN: Creating a Culture of Research (R. Fowler, H. Hathorne) 8) NRS: Implementing Infection Control Guidelines: Successes & Challenges (K. Barnico, E. Bryson) 9) SW/PSYCH: Implementation of Mental Health Screening: Strategies & Successes (K. Reno; M. Schechter) 10) NTR: Nutrition Research (J. Fulton, T. Schindler) 11:50 a.m. - 1:50 p.m. Poster Session I (All numbers) 11:50 a.m. - 1:50 p.m. Lunch Break/Visit Exhibits/View Posters 11:50 a.m. - 4:15 p.m. Exhibit Hall Open 2:00 p.m. - 3:50 p.m. Symposium Session I 1) CFTR: CFTR Properties & Organ-specific Disease (E. Cormet-Boyaka, J. Hanrahan) 2) APP&D: The Failure to Clear CF Mucus (S. Donaldson, D. Stoltz) 3) INF/MIC: Challenging Topics in Infectious Disease (C. Haworth, V. Waters) 4) CLIN: CFTR & Pancreatitis (S. Freedman, M. DiMagno) 5) CLIN: Toward Rational Management of Pulmonary Exacerbations: Part III (P. Flume, C. Goss, D. VanDevanter) 6) NRS: Partnering With Patients & Families to Promote Healthy Lifestyles (B. Harkness, C. Richless) 7) PT & RT: Non-pharmacological Treatment of Pain in CF: A Musculoskeletal Approach (K. Afshar, K. Moffett) 3:50 p.m. - 4:20 p.m. Coffee Break 4:20 p.m. - 6:00 p.m. ALL: Plenary Session I: Personalized Medicine (J. Clancy) 6:30 p.m. - 8:00 p.m. ALL: Welcome Reception 16 | 2015 North American CF Conference PROGRAM AT-A-GLANCE FRIDAY, OCTOBER 9 FRIDAY, OCTOBER 9 7:00 a.m. - 6:00 p.m. General Registration (PCC) 12:30 p.m. - 1:50 p.m. Junior Investigators Best Abstract Sessions 7:30 a.m. - 8:45 a.m. Poster Session II (Even #s)/Continental Breakfast/View Exhibits 11) CFTR: Novel Insights Into CFTR 2015 (C. Bear, J. Kappes) 7:30 a.m. - 6:00 p.m. Exhibit Hall Open 8:00 a.m. - 10:00 p.m. Special Interest & Related Study Groups 9:00 a.m. - 10:00 a.m. ALL: Plenary Session II: Opening Doors to CF Clinical Research (G. Retsch-Bogart) 10:00 a.m. - 10:30 a.m. Coffee Break 2:00 p.m. - 3:45 p.m. Workshop Session II 12) APP&D: Mobilizing CF Mucus (C. Ehre, P. Quinton) 13) GMS: Genetics, Genomics & Big Data Sets (R. Darrah, J. Taylor-Cousar) 14) NT: Early Studies of Novel Small Molecule Therapies (P. Haggie, D. Sheppard) 15) INF/MIC: Microbiome & Emerging Pathogens (D.. Conrad, J. Madan) 16) CLIN-INF/MIC-NTR: CF Newborn Screening (P. Farrell, A. Savant) 10:30 a.m. - 12:20 p.m. Symposium Session II 8) CFTR: It’s In Your Genes: Understanding CFTR Gene Regulation to Improve CF Treatment (A. Harris, M. Walsh) 9) CFTR-NT: Developing Co-therapies to Modulate CFTR (M. Amaral, J. Pilewski) 10) NT: Assessing Presymptomatic Children (T. Rosenow, E. Zemanick) 17) CLIN: Gastroenterology/Liver/Pancreas (J. Abraham, M. Solomon) 18) NRS: Innovative Strategies in Inpatient & Outpatient Care Management (A. Dressman, J. Yeley) 19) SW/PSYCH-NRS: Working Together: Pediatric & Adult Centers Collaborating (S. Self, J. Weiland) 11) INF/MIC: MRSA Update (D. Gilpin, C. Ren) 20) PT & RT: Aerosol Therapy: How Are We Delivering Inhaled Medications in 2015: Past, Present & Future (K. Richards, G. Sawicki) 12) CLIN: CF With Fresh Eyes: A Partnership to Create the Future? (P. Batalden, C. George, M. L. Phillips) 3:45 p.m. - 4:45 p.m. Coffee Break /Informal Poster Viewing 13) NRS: When Addiction Takes Over: Managing Dependence Issues in the Patient With CF (N. Felicetti, C. Muirhead) 4:00 p.m. - 6:00 p.m. Fellows Session (Adult) 14) NTR: Achieving Optimal Nutrition in CF (A. Maqbool, M. Marcus ) 12:30 p.m. - 1:50 p.m. Lunch Break/View Posters/Visit Exhibits 12:30 p.m. - 1:50 p.m. U.S. Pediatric/Adult/Affiliate Program Directors Meetings 4:00 p.m. - 6:00 p.m. Fellows Session (Pediatric) 4:00 p.m. - 6:00 p.m. Caregivers Sessions by Discipline: Epi-Stat/Nrs-AP/Nrs/ Ntr/Pharm/PT & RT/RC-Updates/RC/SW 6:00 p.m. - 7:30 p.m. Speakers & Fellows Reception & Private Poster Viewing Session (By Invitation Only) 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons 1) CLIN: Laboratory Processing: Not Just the Basics (J. Baker) 2) NRS: The Special Transition Needs of Adolescents With Autism & Other Cognitive Delays (C. Tomczyk) 3) SW/PSYCH: Communication Strategies to Create High Functioning Teams (K. Nelson, J. Wooldridge) 4) NTR: Nutrition in the NICU (S. Gemma, L. Grande) 5) PT & RT: Exercise Testing Interpretation & Implementing an Exercise Prescription (M. Bowman, R. Harris) 17 | 2015 North American CF Conference PROGRAM AT-A-GLANCE SATURDAY, OCTOBER 10 SATURDAY, OCTOBER 10 7:00 a.m. - 3:00 p.m. General Registration (PCC) 12:30 p.m. - 1:50 p.m. Luncheon Roundtables 7:30 a.m. - 8:45 a.m. Poster Session III (Odd numbers) 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons 7:30 a.m. - 8:45 a.m. Continental Breakfast/Informal Poster Viewing 6) GMS: Genome Editing (M. Drumm, P. Harrison) 7:30 a.m. - 2:20 p.m. Exhibit Hall Open 7) INF/MIC: Integrating the NTM Guidelines Into Clinical Care (L. Saiman) 7:30 a.m. - 8:45 a.m. Consultation Clinics 8) SW/PSYCH: Managing the Maze of Medication & Access Programs (E. Langfelder-Schwind, A. Plachta) 1) NRS: When Communications Go Bad: Sharing Difficult News/Dealing With Conflict (N. Argel, A. Gardner) 9) SW/PSYCH: Psychosocial Needs for the Newly Diagnosed Patient: Pediatric & Adult (C. Greene, A. Moog) 2) SW/PSYCH: Chronic Sorrow & Spiritually (N. Concotelli-Fisk, D. Grossoehme) 10) NTR: Adult Nutrition: Emerging Issues (G. Garlow, L. Mannik) 3) NTR: GI/Nutrition Consultation Clinic (M. Kowalczyk, J. Matel) 12:30 p.m. - 2:20 p.m. Lunch Break/View Posters/Visit Exhibits 4) PT & RT: PT & RT Case Presentations: Adherence Methods That Work for the PT & RT (K. Riekert, K. von Berg) 12:30 p.m. - 2:20 p.m. U.S. Center & Program Directors Meeting 9:00 a.m. - 10:00 a.m. ALL: Plenary Session III: There Is No Health Without Mental Health (A. Quittner, S. Elborn, B. Smith) 10:00 a.m. - 10:30 a.m. Coffee Break 2:20 p.m. Exhibit Hall Closed 2:30 p.m. - 4:20 p.m. Symposium Session III 15) APP&D-INF/MIC: Anti-inflammatories for CF: Pathways to Therapeutics (A. Cantin, J. Chmiel) 10:30 a.m. - 12:15 p.m. Workshop Session III 16) GMS: How Do CF Genetics & Clinical Practice Interface? (S. Blackman, H. Corvol) 21) CFTR: Understanding & Fixing Mutant CFTR (M. Gray, A. Swiatecka-Urban) 17) NT: Strategies for Rare CFTR Mutations (R. Rubenstein, N. Sharma) 22) APP&D: CF Airway Inflammation, Biomarkers & Therapeutic Targets (G. McElvaney, J. Cousar) 19) CLIN: Update on Lung Transplantation in CF (R. Aris, D. Hadjiliadis) 23) APP&D: Non-CFTR Ion Transport Targets for CF (H. de Jonge, N. Keiser) 24) SPECIAL FORMAT: NT: New Therapy & Outcomes Poster Discussion (A. Gaggar, P. Zeitlin) 25) INF/MIC: Pathogenesis of Infection (H. Johansen, C. Manoil) 26) CLIN: CF Pulmonary Disease: Diagnosis, Evaluation & Treatment (N. Chaudary, L. Vanscoy) 27) CLIN: Cystic Fibrosis Related Diabetes (S. Blackman, M. Rayas) 28) NRS-SW/PSYCH: Managing the Complex Needs of the Adult CF Patient (M. Markovitz, M. Nelsen) 18) INF/MIC: Microbial Interactions in the CF Airways (A. Ashare, S. Lory) 20) CLIN: Caroline McPherson Symposium: Pro/Con Debates in Early CF Lung Disease (<6 Years of Age) (C. Daines, S. Sagel) 21) SW/PSYCH: The Impact of CF on Relationships Throughout the Life Span (J. Nash, K. Riekert) 4:30 p.m. - 6:30 p.m. Caregivers Sessions by Discipline: Nrs/Ntr/Pharm/PT/ RT/SW 7:30 p.m. Closing Event CONFERENCE ADJOURNS 29) SW/PSYCH: Inpatient Management of Behaviors: Adult & Pediatric (J. Cary; S. Filigno) 30) PT & RT: Update on Exercise (F. Cerny, H. Hebestreit) 18 | 2015 North American CF Conference TAB SHEET 5 WEDNESDAY TAB SHEET 5 WEDNESDAY FINAL PROGRAM WEDNESDAY, OCTOBER 7 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (PCC) North 300 Level Lobby 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (Sheraton) Encanto Foyer 8:00 a.m. - 5:00 p.m. Special Classes LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. MPD01 PT & RT: Minimizing Postural Deficits: Physical Therapy Musculoskeletal & Neuromotor Control Interventions for Children & Adults With CF (Combination) 126 ABC Instructor Mary Massery, P.T., D.P.T. Description This course will demonstrate the simultaneous role of trunk muscles for regulation of postural control & ventilator support & the consequences of this duality. Instruction & hands-on practice for PTs will be provided. Assessment & treatment of musculoskeletal & neuromuscular impairments will be discussed. An in-depth case study will be used to demonstrate screening & intervention strategies. Objectives 1. Recall musculoskeletal abnormalities, inadequate core stabilization strategies & breathing patterns that may contribute to the abnormal development of the rib cage, shoulder &/or spine secondary to CF. 2. Demonstrate musculoskeletal intervention strategies to correct or minimize deformities. 3. Demonstrate neuromuscular intervention strategies to minimize secondary functional impairments. NB01 NRS: Nuts & Bolts of CF (Care) 127 ABC Instructors Elizabeth A. Bryson, R.N., M.S.N., C.N.P., C.S. Lynn M. Feenan, R.N., M.S.N. Associate Instructors Alix Ashare, M.D., Ph.D. Susan C. Casey, R.D., C.D. Dabney M. Eidson, R.R.T. Starla G. Martinez, M.D. Matthew Nippins, P.T., D.P.T., C.C.S. Kim Reno, M.S.W., LISW Valerie J. Waters, M.D., M.Sc. Description This day long class is designed for new CF healthcare providers of any discipline. It provides a broad overview of many aspects of CF including: pulmonary, airway clearance, nutrition, microbiology, adult care & psychosocial aspects of CF life. The day ends with a panel of CF family members talking about life with CF. The class serves as a basic foundation for the new CF clinician. Objectives 1. Describe the basic pathophysiology of CF. 2. List at least three types of airway clearance. 3. Name three standard nutritional therapies for CF. 19 | 2015 North American CF Conference FINAL PROGRAM WEDNESDAY, OCTOBER 7 11:00 a.m. - 3:00 p.m. Psychosocial Working Group Sheraton-Maryvale A Chair Martha S. Markovitz, M.S.W., LCSW Description This meeting is open to anyone interested in the clinical or research aspects of the psychosocial issues surrounding CF. 12:30 p.m. – 2:30 p.m. Registry Coordinator’s Luncheon Sheraton-Valley of the Sun C-E 3:30 p.m. - 7:30 p.m. Short Courses LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. SC01 CLIN-NTR: Eating Behaviors in CF (Care) 106 ABC Instructors Scott W. Powers, Ph.D., ABPP Elizabeth Reid, M.S., R.D. Description Nutrition, weight & BMI are a focus at every CF clinic visit for children, families & the CF care team. Meal time can be stressful for many patients & their families if they are worried about weight & growth. This course will discuss building & maintaining a positive feeding relationship, from infancy through childhood, using child development, nutrition recommendations & the latest research. Objectives 1. Identify & discuss behaviors that can promote positive attachment between caregivers & infants to promote a positive feeding environment. 2. Discuss areas for anticipatory guidance & support of caregivers to meet the special nutrition needs of children with CF. 3. Discuss ways to help families with calorie intake goal setting & tracking of progress. SC02 CLIN: Nuts & Bolts for the Research Coordinator (Research) 104 AB Instructors Elizabeth R. Hartigan, M.P.H., R.N., C.R.M. Sandra K. Hurban, R.N., B.S.N., CCRC Associate Instructors Mahrya Johnson, B.A., CCRP Kim Simmons, B.S.N. Jonathan Spahr, M.D. Description This short course will describe the core knowledge & skills research coordinators need to manage the daily activities of a research study while ensuring research subject safety . Organizational strategies will be discussed. Clinical trial outcome measures specific to cystic fibrosis will be discussed & a patient's perspective on clinical trial participation will be presented. Objectives 1. Identify common comorbidities seen in medical histories of patients with CF & medications used to treat them. 2. Describe clinical trial outcome measures specific to CF. 3. Employ knowledge gained & identify tools to organize study documents, manage study visits & documentation of data to ensure compliance with GCP. 20 | 2015 North American CF Conference FINAL PROGRAM WEDNESDAY, OCTOBER 7 3:30 p.m. - 7:30 p.m. Short Courses LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. SC03 CLIN: Advanced Drug Therapy for CF (Care) 105 AB Instructors Kenneth N. Olivier, M.D., M.P.H. David C. Young, Pharm.D. Associate Instructors Scott H. Donaldson, M.D. Jeffery T. Zobell, Pharm.D. Description The overall goal of this short course to provide CF care providers with the skills & knowledge necessary to provide advanced therapy via an interactive discussion & case-based format. This short course will address the following therapeutic areas: 1. CFTR Modulators & Novel Therapies 2. Advanced Drug Delivery for an Acute Pulmonary Exacerbation 3. Nontuberculous Mycobacteria 4. ABPA Objectives 1. Describe & evaluate CFTR modulators & new/novel therapies for CF. 2. Discuss & design an evidence-supported continuous/extended infusion beta-lactams & inhaled antibiotics regimen for treating an acute pulmonary exacerbation. 3. Create & assess therapeutic options to treat NTM & ABPA infections in CF. SC04 SW/PSYCH: Psychopharmacology for CF 101 (Care) 103 AB Instructors Anna M. Georgiopoulos, M.D. Beth A. Smith, M.D. Associate Instructors Stephanie S. Filigno, Ph.D. Amy E. Mueller, LCSW Deborah F. Queen, M.D. Ahmet Z. Uluer, D.O., M.S. Description New guidelines from the CF Foundation & European CF Society recommend screening & treatment for depression & anxiety. This course will teach practitioners what they need to know about using psychotropic medication in individuals with CF. Objectives 1. Assess anxiety & depression symptoms in individuals with CF before prescribing medication. 2. Review how to use medications recommended by the CFF/ECFS to treat anxiety & depression in individuals with CF. 3. Identify CF-specific factors that may influence the use of psychotropic medication. 21 | 2015 North American CF Conference FINAL PROGRAM WEDNESDAY, OCTOBER 7 3:30 p.m. - 7:30 p.m. Short Courses LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. SC05 SW/PSYCH: Advanced Cognitive Behavioral Strategies for Depression & Anxiety (Care) 102 AB Instructors Christina L. Duncan, Ph.D. Deborah Friedman, Ph.D. Associate Instructors Amanda Montgomery, M.S.W., LCSW Description This short course is intended for clinicians who have had some exposure to CBT for anxiety and/or depression interested in building on their comfort level & skill to integrate CBT into their practices. The focus will be on CBT interventions that can be introduced in brief sessions with patients & in busy clinic settings. There will be opportunities for hands-on & interactive learning. Objectives 1. Practice assessment & case conceptualization using a CBT model. 2. Practice several essential CBT techniques for the treatment of anxiety & depression. 3. Identify potential barriers & solutions to the integration of CBT into clinical practice. SC06 NTR: CF Nutrition 101 (Care) 101 ABC Instructors Catherine M. McDonald, Ph.D., R.D., C.S.P. Suzanne H. Michel, M.P.H., R.D., L.D.N. Associate Instructors Amanda R. Leonard, M.P.H., R.D., C.D.E. Karen M. Maguiness, M.S., R.D., C.S.P. Ronalee Robert, R.D., C.D.E. Jillian Sullivan, M.D, MSCS Description This course will the following specific topics: age-appropriate recommendations, pediatric feeding behavior, pancreatic enzyme replacement, GI challenges, vitamin/ mineral supplementation, pregnancy, CF-related diabetes, & end-of-life nutrition. Available resources will be reviewed as well as current evidence-based literature. Objectives 1. Describe the relationship of weight, growth, body mass & optimal nutritional status to CF health outcomes. 2. Summarize GI challenges, recommended vitamin & mineral supplementation & enzyme management throughout the life cycle. 3. Summarize nutrition challenges & management techniques of adults who have CF including CF-related diabetes & pregnancy. 22 | 2015 North American CF Conference FINAL PROGRAM WEDNESDAY, OCTOBER 7 3:30 p.m. - 7:30 p.m. Short Courses LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. SC07 PT & RT: Beginning Airway Clearance for the New CF Team Member (Care) 105 C Instructors Brenda M. Button, Ph.D. Maggie McIlwaine, Ph.D., MCSP Description In this course different types of current evidence-based modern ACTs will be presented, including the Active Cycle of Breathing Technique; Autogenic Drainage; Positive Expiratory Pressure (PEP); Oscillating PEP & external oscillating devices. Also the rationale for techniques, how they affect the pathophysiology of CF together with indications & contraindications & precautions will be discussed. Objectives 1. Discuss the different types of evidence-based Modern Airway Clearance Techniques currently available. 2. Describe selection of individiualized airway clearance techniques & situations when they are contraindicated. 3. Discuss the importance of exercise & adherence to therapy across the lifespan in CF. 3:30 p.m. - 7:30 p.m. Special Session (By invitation only) LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. SPQI Special Session by Invitation Only: Introduction to CF Quality Improvement: The Virtual Improvement Program-Fundamentals ‘VIP-F’ Launch (Care) 102 C Instructors Randall Messier, M.T., M.S.A., PCMH, C.C.E. Brant Oliver, Ph.D., M.S., M.P.H., APRN-BC Kristofer Petren Description This comprehensive short course will provide didactic & applied education on practical improvement measurement skills for rapid cycle systems performance improvement in CF care, including (1) defining measures; (2) linking measures to aims, improvement (change) ideas & the data collection plan; (3) creating run charts, SPC charts; & performance dashboards; & (4) utilizing the CF registry & cascading measures to inform & empower improvement work. Objectives 1. Demonstrate how to link operationally defined measures to improvement aims & change ideas. 2. Demonstrate how to create a basic data collection plan. 3. Apply measurement skills to create & interpret run charts, XmR charts, p charts & performance dashboards. 23 | 2015 North American CF Conference TAB SHEET 5 THURSDAY FINAL PROGRAM THURSDAY, OCTOBER 8 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (PCC) North 300 Level Lobby 7:00 a.m. - 7:30 a.m. Continental Breakfast for Caregivers & Physicians Grand Rounds Attendees 120 Foyer 200 Level Corridors 7:30 a.m. - 9:30 a.m. Caregivers Sessions by Discipline CG01 CLIN: Genetic Counselors: CF in the Genomic Era: Alterations in CFTR Genetic Counselors & Alterations in Care (Care) 226 ABC Moderators Rebecca Marsick Darrah, Ph.D., M.S. Sumedha Ghate, M.S., C.G.C. Speakers Mei Baker, M.D., FACMG Richard B. Parad, M.D., M.P.H. Karen Raraigh, M.G.C., C.G.C. Description This session will highlight a new clinical classification termed cystic fibrosis screen positive, inconclusive diagnosis (CFSPID), & management recommendations. We will also review the mutation vetting process utilized by the CFTR2 team, & provide CFTR2 updates. Finally we will review data & utility of the WI expanded CFTR 250 mutation panel research study. Objectives 1. Describe the new designation (CFSPID) & management for infants with an inconclusive diagnosis following newborn screening. 2. Describe the characterization criteria of CFTR variants as CF-causing, non-CF causing, or resulting in varying clinical consequences. 3. Evaluate the feasibility & utility of next generation sequencing expanded CFTR mutation panel for CF newborn screening. CG02 NRS: Advanced Practice: The CF Huddle (Care) 301 D Moderators Lynne M. Fries, P.A.-C., MPAS, D.P.T. Barbara T. Jansma, CRNP Speakers Marianne Ferrin, M.S.N., ACNP-BC Lisa J. Lunsford, D.N.P., CPNP Theresa Flint Rodgers, R.N., D.N.P., CRNP-AC/PC Alicia Turner, R.N., M.S.N., F.N.P.-C. Jana B. Yeley, R.N., M.S.N., N.P.-B.C. Description Nurse Practitioners & Physician Assistants have become an important component of the CF team & a factor in future CF care. This session is focused on the needs & interests of mid level providers who are involved in Cystic Fibrosis care. Information on CF Foundation resources will be reviewed. Focused discussion groups will be conducted regarding topics pertinent to NPs & PAs. Objectives 1. Identify resources to promote personal & professional growth in CF practice. 2. Identify practice challenges & trends. 3. Formulate & expand problem-solving options. 25 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 7:30 a.m. - 9:30 a.m. Caregivers Sessions by Discipline CG03 NRS: Nursing Education Caregivers (Care) 301 BC Moderators Connie L. Fenton, R.N., B.S.N. Diane Schachner, R.N., PCNS, B.S.N., M.S.N. Speakers Traci D. Bell, B.S.N. Connie L. Fenton, R.N., B.S.N. Mary Helmers, R.N., B.S.N. Kathryn Killeen, M.S.N., ACNP Description This session is designed to explain & demonstrate practical approaches to improving the clinic experience for both patient/families & professional caregivers. Practical advice & demonstration will provide caregivers with ideas to improve the CF clinic appointment for patients & providers. Ideas for improving assessment & education during clinic appointments will be discussed. Objectives 1. Identify ways to focus clinic appointments to what the patient wants to learn & identify ways to look at alternative methods to obtain & deliver standard CF treatments. 2. Discuss a tool to help facilitate explanation of & maneuvering through testing during a clinic visit. 3. Discuss ways to improve the nursing assessment & education pertaining to endof-life choices & pathways. CG04 NTR: Update on Nutrition Guidelines (Combination) 224 AB Moderators Karen M. Maguiness, M.S., R.D., C.S.P. Amy E. Schrader, R.D., L.D. Speakers Hui Chuan J. Lai, Ph.D., R.D. Amanda R. Leonard, M.P.H., R.D., C.D.E. Karen M. Maguiness, M.S., R.D., C.S.P. Amy E. Schrader, R.D., L.D. Description This session will provide updates on recent nutrition guidelines for CF including the use & interpretation of WHO vs. CDC growth charts for children with CF less than two years of age; the newly established enteral/tube feeding guidelines; a detailed explanation of the preschool nutrition guidelines algorithm; & clinical criteria for malnutrition in the adult patient with cystic fibrosis. Objectives 1. Differentiate between WHO & CDC growth charts to accurately establish goals for patients with CF less than two years. 2. Summarize findings from the CFF Enteral Guidelines Committee recommendations. 3. Define flow process for efficient nutritional treatment in the preschooler & current characteristics of adult malnutrition & explain the importance of early recognition & treatment. 26 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 7:30 a.m. - 9:30 a.m. Caregivers Sessions by Discipline CG05 CLIN: PHARM: Collaborative CF Pharmacist Research (Research) 221 ABC Moderators Paul M. Beringer, Pharm.D. Daniel Cortes, B.Sc.Pharm. Speakers Eva Cho, B.Sc., B.Sc.Pharm., ACPR Catherine E. O'Brien, Pharm.D. Victoria Su, B.Sc.Pharm., ACPR, Pharm.D. Breanna Wyman, Pharm.D.(c) Description This session will present various pharmacist-led collaborative research projects: 1) survey of ABPA treatment at US CF centers; 2) update of "Multi-center Evaluation to Establish Patterns of Treatment of MRSA in CF patients"; 3) survey of CF pharmacy practice & IV antipseudomonals in CAN CF centres; & 4) evaluation of medication adherence in British Columbia paediatric & adult CF centres. Objectives 1. Describe current ABPA treatment in CF patients across the U.S. 2. Discuss preliminary data of the MRSA Collaborative Research group. 3. Describe factors that have been associated with poor adherence in the CF population. CG06 PT & RT: Stretching & Stabilization to Promote Airflow Mobility (Care) 229 AB Moderators Denise M. Coleman, P.T., D.P.T., Pilates Certified Instructor Sharon A. Ross, P.T., R.Y.T.-200 Speakers Wayne Barfield, R.R.T. Denise M. Coleman, P.T., D.P.T., Pilates Certified Instructor Mary Lester, R.R.T. Marcin Madej, P.T., D.P.T. Mary Massery, P.T. Description This session will use a "round robin" format where participants rotate through 3 stations: 1) Practicing Pilates-based exercises to facilitate improved postural strength & breathing patterns. 2) Learning soft tissue & joint mobilization techniques geared toward improving rib cage mobility for ease of breathing. 3) Hands-on practice of combining thoracic stretching, clapping with ACBT. Objectives 1. Demonstrate core stabilization exercises to improve ease of breathing. 2. Practice thoracic/trunk mobilization exercises to improve work of breathing & postural alignment. 3. Demonstrate thoracic stretching, clapping & ACBT combined & streamlined as an active AWC method for the CF patient. 27 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 7:30 a.m. - 9:30 a.m. Caregivers Sessions by Discipline CG07 CLIN: RC: Managing Patient Expectations: What Happens When Patients Are Not Eligible? (Research) 222 ABC Moderator Ashley Keller, M.P.H. Speakers Tina Hicks, R.N. Ashley Keller, M.P.H. Lisa A. Monchil, R.R.T.-N.P.S., CCRC Description This session will explore various situations of patient ineligibility for trials. What happens when patients are not eligible for a clinical trial, but want to participate? What happens if a study enrolls quickly & you are unable to get a patient into a trial? Discussion will include suggestions in dealing with various scenarios & ways to keep ineligible patients engaged in clinical research. Objectives 1. Discuss the challenges/barriers that research coordinators face when a patient is ineligible for a clinical research study. 2. Identify ways to overcome the challenges/barriers associated with patient ineligibility for trials. 3. Identify ways to keep patients that are not actively participating in trials engaged in research. CG08 SW/PSYCH: Maintaining Ethics & Boundaries in Clinical Care (Care) 227 ABC Moderators Carol S. Chace, M.S.W., LICSW Al Lew, LICSW Speakers David Beyda, M.D. Carol S. Chace, M.S.W., LICSW Al Lew, LICSW Deanna C. Reese, LCSW, MSSW, BSSW Lori Yoder, M.S.W. Description Attendees will learn about using social media with patients through a review of current literature, how to avoid pitfalls of boundary violations, & review of medical ethics pertaining to the use of social media & boundary violations. The session goals will be met through case presentations & discussion led by a medical ethicist & session moderators. Objectives 1. Describe current research on social media & the potential for boundary violations. 2. Discuss our own social media presence & how it may inadvertently produce ethical dilemmas. 3. Explain the breadth of social media sites colleagues use personally & professionally. 28 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 7:30 a.m. - 9:30 a.m. Physicians Grand Rounds (Care) 120 BC Chairs Manu Jain, M.D., M.Sc. Clement L. Ren, M.D. Presenters & Acute Neurologic Deficits in a Young Man With Cystic Fibrosis Discussants Kimberly A. McBennett, M.D., Ph.D. Discussant: Kelly Milton, M.D. Sudden Death of a 17-day-old Infant With Cystic Fibrosis Kathryn S. Moffett, M.D., FAAP Discussant: Erin Rowell, M.D. DRESS Syndrome in a Patient With Cystic Fibrosis Robert A. Kaslovsky, M.D. Discussant: Vanessa Gildenstern, M.D. A Case of Worsening Mycobacterial Infection & Chronic Low Level Pancreatitis Treated in a New Way Denis Hadjiliadis, M.D., M.H.S., FRCP(C) Discussant: Steven M. Rowe, M.D., MSPH Description In this session, four interesting clinical cases will be presented. Each case will highlight a relatively unusual but instructive aspect of managing CF patients. Objectives 1. Relate complications of CF & its therapies to unusual somatic manifestations. 2. Identify subtle life-threatening abdominal complications of CF. 3. Illustrate alternative clinical responses to CFTR modulation. 8:00 a.m. - 10:00 a.m. Special Interest & Related Study Groups See insert for location 9:00 a.m. - 10:00 a.m. Poster Set-Up Exhibit Hall E 9:30 a.m. - 10:00 a.m. Coffee Break 29 | 2015 North American CF Conference 120 Foyer 100 Level Corridors 301 Foyer FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W01 CFTR: Insights Into CFTR Biology (Research) 301 BC Chairs Zoltan Bozoky, Ph.D. Patrick H. Thibodeau, Ph.D. 10:00 a.m. Introduction Zoltan Bozoky, Ph.D. 10:10 a.m. Abstract Interdomain Interactions & Stabilization Of CFTR Folding Mutations Patrick H. Thibodeau, Ph.D. 1 10:25 a.m. Abstract Functional Characterization Of Stabilized CFTR Protein Constructs Andrei A. Aleksandrov, Ph.D. 2 10:40 a.m. Abstract Analysis Of The Effects Of Rare Cystic Fibrosis Mutations On CFTR Expression, Stability & Function 3 David N. Sheppard, Ph.D. 10:55 a.m. Abstract Pharmacological Myth Of CFTR: One Plus One Is Larger Than Two Wen-Ying Lin, Ph.D. 4 11:10 a.m. Abstract Correction Of CFTR Variants By Modulation Of The Protein Translation Machinery Darren M. Hutt, Ph.D. 5 11:25 a.m. Abstract Pharmacological Rescue Of Mutant CFTR: Exploring Mutant Specific Therapies Deborah M. Cholon, Ph.D. 6 Description This workshop is designed to address the cutting-edge research questions to get insights into CFTR biology. It will focus on mutation-specific protein expression, folding, stability & function especially, but not limited for researchers or anyone who is interested in expanding their knowledge on CFTR structure. Objectives 1. Evaluate the current state of mutation-specific therapies & correction of CFTR variants. 2. Describe the role of interdomain interactions on expression, folding, stability & function of CFTR mutations. 3. Identify key modulators that correct trafficking & functional defects. 30 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W02 APP&D-GMS: New Advances in CF Animal Models (Research) 129 AB Chairs Michel Bagnat, Ph.D. Lane L. Clarke, D.V.M., Ph.D. 10:00 a.m. Introduction Michel Bagnat, Ph.D. 10:10 a.m. Abstract The CFTR -/- Rat Is Susceptible to Pseudomonas Aeruginosa Infection Susan E. Birket, Pharm.D., Ph.D. 139 10:25 a.m. Abstract Generation of BAC Transgenic Mice Expressing Human CFTR Lane L. Clarke, D.V.M., Ph.D. 86 10:40 a.m. Abstract CFTR Knockout Mice Exhibit Abnormal Constitutive & Bile Acid-Stimulated Bile Flow & Biliary ATP Concentration 87 Qin Li, Ph.D. 10:55 a.m. Abstract CFTR Regulates the Phagocytic Function & Inflammatory Phenotype of Alveolar Macrophages From Birth 88 Nicholas W. Keiser, Ph.D. 11:10 a.m. Abstract Development of Bronchiectatic Airways in the Absence of Major Lung Infections in CF Ferrets 89 Bradley Rosen, D.O. 11:25 a.m. Abstract Discovering a Molecular Basis for Airway Smooth Muscle Dysfunction Though Genome-Wide Transcriptional Analysis in the CF Pig 90 Daniel Cook Description This workshop will introduce two rodent models of CF (ΔF508 CFTR rat & a CF mouse expressing human CFTR) & provide new insights into biliary & respiratory CF disease from experiments performed using CF mouse, ferret or pig models. Particular emphasis will be given to evidence demonstrating infection- & inflammation-independent defects in CF airway function. Objectives 1. Summarize the advantages/disadvantages of new CF rodent models (the ΔF508 CFTR rat & human CFTR expressing mouse). 2. Explain implications of defective biliary ATP release in CF mice & age-dependent defects in CF ferret alveolar macrophages. 3. Summarize implications of sterile bronchiectasis in CF ferret lungs & inflammation-independent smooth muscle dysfunction in CF pig airways. 31 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W03 NT: Novel Targets & Outcome Measures (Combination) 124 AB Chairs James F. Chmiel, M.D., M.P.H. Terri A. Laguna, M.D., MSCS 10:00 a.m. Introduction Terri A. Laguna, M.D., MSCS 10:10 a.m. Abstract Effect of Ivacaftor in R117H Patients Following FDA Approval: Early Results of the G551D Observational-Expanded & Extended (GOAL E-2) Study 190 Scott D. Sagel, M.D., Ph.D. 10:25 a.m. Abstract Progression & Variability of LCI in Preschool Children With Cystic Fibrosis Renee Jensen, R.R.T. 191 10:40 a.m. Abstract A Randomized, Double-blind, Placebo-controlled Trial of Repeated Nebulization of Non-viral CFTR Gene Therapy in Patients With Cystic Fibrosis 192 Eric Alton, M.A., M.D., FRCP, F. Med. Sci. 10:55 a.m. Abstract Mesenchymal Stem Cell LL-37 in Cystic Fibrosis Tracey L. Bonfield, Ph.D. D (ABMLI) 193 11:10 a.m. Abstract Hormone Fluctuations Correlate With Respiratory Symptoms in Patients With Cystic Fibrosis 195 Raksha Jain, M.D. 11:25 a.m. Abstract Nano-based Autophagy Targeting to Control CF Pathogenesis Neeraj Vij, Ph.D. 196 Description In this session, new therapeutic advances in both the clinical & basic science world will be presented. Clinicians & basic scientists alike will find value in the discussion about novel approaches to treat CF lung disease in addition to the presentation of outcome measures appropriate for young children. Objectives 1. Identify the latest advances in treatment focused on inflammation, gene therapy & mesenchymal stem cells currently under study for CF. 2. Describe the applicability of lung clearance index as an outcome measure for lung disease progression in young children. 3. Assess the impact ivacaftor & hormone fluctuations have on the trajectory of disease progression. 32 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W04 INF/MIC: Epidemiology & Management of Infection (Research) 301 D Chairs Timothy Kidd, Ph.D. Sonya L. Heltshe, Ph.D. 10:00 a.m. Introduction Sonya L. Heltshe, Ph.D. 10:05 a.m. Abstract Evaluation of Burkholderia spp. Eradication in CF Patients Victoria Ch Su, B.Sc. Pharm, ACPR, Pharm.D. 291 10:20 a.m. Abstract Selective Drug Interaction Between Azithromycin & Inhaled Tobramycin: Replication Dataset & Additional In Vitro Work 292 Dave Nichols, M.D. 10:35 a.m. Abstract Interim Data From a Prospective 5-year Registry Study to Monitor Aztreonam Susceptibility of P. aeruginosa Isolates From Patients With CF in the US 293 Emily DiMango, M.D. 10:50 a.m. Abstract Patterns of P. aeruginosa Infection in a Contemporary CF Birth Cohort: Emergence & Transition Between Stages of Infection 294 Umer Khan, M.S. 11:05 a.m. Abstract The Effect of Ivacaftor Treatment on Airway Microbial Community Dynamics in Patients With G551D (the "Celtic" Mutation) 295 Gisli G. Einarsson, Ph.D. 11:20 a.m. Abstract Airborne Non-tuberculosis Mycobacteria in the Home & CF Clinic: Potential of Fomites as a Mechanism of Transmission 296 Jerry A. Nick, M.D. 11:35 a.m. Abstract Aztreonam for Inhalation Solution & Tobramycin Inhalation Solution Continuous Alternating Therapy for CF Patients With Chronic P. Aeruginosa Infection 428 Patrick A. Flume, M.D. Description Observations in respiratory microbiology are presented, including factors associated with Burkholderia spp. eradication & NTM acquisition. Emphasis on P. aeruginosa (Pa) will include trends in aztreonam susceptibility, response to combined tobramycin & azithromycin & analyses describing stages of infection with mucoid Pa. Ivacaftor's effect on the microbiome in 'Celtic' G551D is presented. Objectives 1. Describe current trends in P. aeruginosa infection in CF & variable response to common therapeutics including antibiotics & CFTR modulators. 2. Explain environmental risk factors for non-tuberculosis mycobacteria & potentially successful eradication regimens for Burkholderia. 3. Use observations of the complex respiratory microbiome to improve CF care. 33 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W05 CLIN: Bone & Endocrine/Non-diabetes (Combination) 120 A Chairs Melissa S. Putman, M.D. Vin Tangpricha, M.D., Ph.D. 10:00 a.m. Introduction Melissa S. Putman, M.D. Vin Tangpricha, M.D., Ph.D. 10:10 a.m. Abstract Short Stature in Children with Cystic Fibrosis Victor Harrison, M.D. 588 10:25 a.m. Abstract Exploring the Prevalence & Treatment of Bone Disease in CF: Comparative Assessment of Two Adult International CF Centres Matched for Latitute 589 Ranjani Somayaji, M.D. 10:40 a.m. Abstract Characterization of the Bone Health Status by DEXA Scanning of Adult CF Patients Attending a Regional UK Center 590 Maeve O'Driscoll 10:55 a.m. Abstract Bone Disease in Cystic Fibrosis: Expression of Osteocalcin & RANK-L/OPG Ratio in Human Osteoblasts With the F508-DEL Mutation in CFTR 591 Jacky Jacquot, Ph.D. 11:10 a.m. Abstract The Absence of the CFTR Leads to Alterations in the Osteoblast & Bone Marrow Phenotypes 592 Valerie Orlando 11:25 a.m. Abstract Bone Development in the Cystic Fibrosis Ferret Model Michael S. Stalvey, M.D. 593 Description This workshop will be focused on selected abstracts on clinical endocrinology topics important to patients with CF. Topics for this session include bone & growth disorders. Objectives 1. Describe the pathophysiology of CF bone disease. 2. Discuss the role of growth hormone in children with CF. 3. Illustrate the importance of animal models to the understanding of CF bone disease. 34 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W06 CLIN: Epidemiology of CF (Combination) 121 ABC Chairs Christopher H. Goss, M.D., M.Sc. Sanja Stanojevic, Ph.D. 10:00 a.m. Introduction Christopher H. Goss, M.D., M.Sc. 10:10 a.m. Abstract Continuity of Care During Transfer From Pediatric to Adult Cystic Fibrosis Care Centers in the United States 486 Gregory Sawicki, M.D., M.P.H. 10:25 a.m. Abstract How Much FEV1 Variability Can Be Explained By Pulmonary Exacerbations? Wayne J. Morgan, M.D. 488 10:40 a.m. Abstract Risk Factors Associated With Rapid FEV1 Decline in Adults With Early-stage CF Lung Disease: Cohort Study of the US CF Foundation Patient Registry 489 Elliott C. Dasenbrook, M.D., M.H.S. 10:55 a.m. Abstract Association of Number of IV-treated Exacerbations in the Prior Year & the Time to Pulmonary Exacerbation 423 Donald R. VanDevanter, Ph.D. 11:10 a.m. Abstract Poor Recovery From Pulmonary Exacerbations Does Not Lead to Accelerated Decline in FEV1 490 Don B. Sanders, M.D., M.S. 11:25 a.m. Abstract Comparison of Survival in the CF Population Between Canada & the US Using a Unified Approach 493 Anne Stephenson, M.D., Ph.D. Description During this workshop, a number of key aspects of clinical epidemiology in CF will be explored. These topics will address the use of observational data to study survival differences between countries (example US & Canada), aspects of transition of care, pulmonary exacerbation & lung function decline. Objectives 1. Understand factors associated with lung function decline & acute pulmonary exacerbation, including lung function variability. 2. Explain potential reasons for differing survival in CF between countries. 3. Understand how transition of care from pediatric to adult centers occurs in the US. 35 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W07 CLIN: Creating a Culture of Research (Research) 120 BC Chairs Robert E. Fowler Heather Y. Hathorne, M.A.E., R.R.T., CCRC 10:00 a.m. Introduction Robert E. Fowler Heather Y. Hathorne, M.A.E., R.R.T., CCRC 10:10 a.m. Invited Speaker Clinical Research Awareness Among Cystic Fibrosis Patients & Families Attending the University of Oklahoma Cystic Fibrosis Center Deanna Thomas , B.A., CCRP 10:30 a.m. Invited Speaker Subversive Techniques to Promote Research Amongst Clinical Staff Cynthia Leyva 10:50 a.m. Invited Speaker Improving Nutritional Status Among Adults With Cystic Fibrosis by Assessing Knowledge & Attitudes Toward Nutrition & Weight Kathryn Poch 11:10 a.m. Invited Speaker Increasing the Culture of Research at UT Southwestern Medical Center Using EQUIP-CR & Feedback From Patients & Families Presenter Ashley Keller , M.P.H. 11:30 a.m. Discussion Description This workshop will focus on recruiting, team building, integrating within into the clinical team. This session will focus on innovative strategies sites have used to create or improve the culture of research at their center. Objectives 1. Identify useful techniques to integrate research & clinical teams. 2. Define strategies to create & strengthen the culture of research at your center. 3. Apply lessons learned from eQUIP coaches with successful integrations experiences. 36 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W08 NRS: Implementing Infection Prevention & Control Guidelines: Successes & Challenges (Care) 301 A Chairs Katherine Barnico, R.N., B.S.N. Elizabeth A. Bryson, M.S.N., PPCNP-BC, C.S. 10:00 a.m. Introduction Katherine Barnico, R.N., B.S.N. 10:05 a.m. Invited Speaker Update of 2013 IPC Guidelines Lisa Saiman, M.D., M.P.H. 10:20 a.m. Invited Speaker Practical Implementation of IPC Guidelines: Success, Challenges & Psychosocial Implications: Inpatient Setting Ahmet Z. Uluer, D.O., M.S. 10:35 a.m. Invited Speaker Practical Implementation of IPC Guidelines: Success, Challenges & Psychosocial Implications: Outpatient Setting Michelle A. Powers, R.N., B.S.N. 10:50 a.m. Invited Speaker Practical Implementation of IPC Guidelines: Success, Challenges & Psychosocial Implications: Respiratory Equipment Catherine O'Malley, R.R.T., N.P.S. 11:05 a.m. Invited Speaker Practical Implementation of IPC Guidelines: Success, Challenges & Psychosocial Implications: Community Carol S. Chace, M.S.W., LICSW 11:20 a.m. Panel Q&A Betsy Bryson, R.N., M.S.N., PPCNP-BC, C.S. 11:40 a.m. Conclusion & CFF Resources Description This workshop is for the CF care team in expanding their knowledge & application of the CF Infection Prevention & Control Guidelines. Topics covered include updates in the CFF guidelines & implementation of the guidelines in the hospital & community setting. We will discuss barriers to implantation of the guidelines as well as provide educational tools, case studies & practical skills. Objectives 1. Identify current infection prevention & control guidelines on the care of CF patients & apply them to the inpatient & ambulatory hospital settings. 2. Explain the implementation & barriers of CF infection prevention & control practice in the community, school & work settings. 3. Discuss the psychosocial impact of infection control guidelines on individuals with CF & their families. 37 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W09 SW/PSYCH: Implementation of Mental Health Screening: Strategies & Successes (Combination) 122 ABC Chairs Kim Reno, M.S.W., LISW Michael S. Schechter, M.D., M.P.H. 10:00 a.m. Introduction Kim Reno, M.S.W., LISW Michael S. Schechter, M.D., M.P.H. 10:10 a.m. Abstract Force Field Analysis of Mental Health Screening & Treatment in Six Accredited CF Centers 604 Kim Reno, M.S.W., LISW 10:25 a.m. Abstract Self-Reported Quality of Life, Anxiety & Depression Symptoms in Children With Cystic Fibrosis 607 Sonia Graziano, Psy.D. 10:40 a.m. Abstract Cystic Fibrosis & Mindfulness-Based Stress Reduction Programs: Impact on Depression & Quality of Life 609 Paul K. Mohabir, M.D. 10:55 a.m. Abstract Routine Depression Screening: Feasibility & Patient Outcomes in CF. The Buffalo Experience 605 Danielle M. Goetz, M.D. Christine M. Roach, R.N., B.S.N. Beth A. Smith, M.D. 11:25 a.m. Abstract Recommendations for CFF Support of Prevention, Screening & Treatment of Anxiety & Depression in Individuals With Cystic Fibrosis & Caregivers Michael S. Schechter, M.D., M.P.H. 11:40 a.m. Discussion Description Studies over the last few years have made it increasingly clear that depression & anxiety are highly prevalent in individuals with CF as well as their parent caregivers. Clinical care guidelines for preventing, screening & treating anxiety & depression have been developed & submitted for publication. This session will provide an opportunity to hear strategies of successful guideline implementation. Objectives 1. Define prevalence of anxiety & depression in the CF population. 2. Describe successful methods for implementing anxiety & depression screening & treatment in CF care centers. 3. Assess patient outcomes in anxiety & depression screening. 38 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 10:00 a.m. - 11:45 a.m. Workshop Session I W10 NTR: Nutrition Research (Research) 120 D Chairs Judy A. Fulton, M.P.H., R.D., L.D.N. Terri Schindler, M.S., R.D. 10:00 a.m. Introduction Terri Schindler, M.S., R.D. 10:10 a.m. Abstract Ivacaftor Improves Linear Growth in G551D Cystic Fibrosis Children: Results of a Multi-center, Placebo-controlled Study 554 Michael S. Stalvey, M.D. 10:25 a.m. Abstract Safety & Efficacy of a Novel Microbial Lipase (NM-BL) in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis 555 Gavin R. Graff, M.D. 10:40 a.m. Abstract Early Supplementation Normalized Serum Vitamin A & E But Not Vitamin D in Infants With CF Diagnosed Through Newborn Screening 556 Lyanne Chin 10:55 a.m. Abstract Novel Immobilized Lipase Device Enhances Nutritional Benefits of Enteral Feeding & Provides Flexibility for Shorter Feedings & Reduced Volumes 557 Greta L. Loring, Ph.D. 11:10 a.m. Abstract Utilization of Total Parenteral Nutrition & Intralipids in Pediatric Cystic Fibrosis Patients 558 Zena Ghazala, M.D. 11:25 a.m. Abstract Implementation of a Billing Process for Medical Nutrition Therapy by a Registered Dietitian at a Large Cystic Fibrosis Center 559 Kimberly G. Stephenson, M.S., R.D., C.S.P. Description Presenters will provide results of research projects & advancements that impact the nutritional outcomes in patients with CF. Objectives 1. Describe the outcomes of early vitamin supplementation in CF infants & identify successful billing processes for medical nutrition therapy in a large CF center. 2. Discuss the effects of ivacaftor on linear growth in G551D CF children. 3. Identify new nutrition practices. 39 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 11:50 a.m. - 1:50 p.m. Poster Session I (All Numbers) Discipline/Topic # Discipline/ Topic # Discipline/Topic # CFTR 1-84 Pulmonary 399-422 424-456 GI/Nutrition 554-587 Airways, Physiology, Pathophysiology & Defense 85-150 Physical & Respiratory Therapy 457-476 Endocrine 588-603 Genetics 151-172 Transplantation 477-485 Psychosocial/ Behavioral 604-661 Model Systems 173-189 Epidemiology & Newborn Screening 486-514 423 Nursing Issues 662-676 New Therapies, Biomarkers & Outcome Measures 190-290 Quality Improvement 515-553 Education 677-683 Infection/ Microbiology 291-398 11:50 a.m. - 1:50 p.m. Lunch Break/Visit Exhibits/View Posters 11:50 a.m. - 4:15 p.m. Exhibit Hall Open 40 | 2015 North American CF Conference Exhibit Hall E Exhibit Hall E FINAL PROGRAM THURSDAY, OCTOBER 8 2:00 p.m. - 3:50 p.m. Symposium Session I S01 CFTR: CFTR Properties & Organ-specific Disease (Research) 120 D Chairs Estelle A. Cormet-Boyaka, Ph.D. John W. Hanrahan, Ph.D. 2:00 p.m. Introduction John W. Hanrahan, Ph.D. 2:10 p.m. S1.1 Role of CFTR in Insulin Secretion by Pancreatic Beta Cells Hsiao Chang Chan, Ph.D. 2:35 p.m. S1.2 CFTR & Airway Host Defense Mohmoud Abou Alaiwa, M.D. 3:00 p.m. S1.3 Bioassays of CFTR Function in Sweat Glands Jeffrey Wine, Ph.D. 3:25 p.m. S1.4 The CFTR Defect in Hematopoietic Cells Gerry N. McElvaney, M.B., FRCPI, FRCPC Description The overall purpose of this symposium is to highlight recent findings on CFTR in different organ systems. Objectives 1. Identify the roles of CFTR in four different cell types: pancreatic B cells, airway epithelial cells, sweat glands & neutrophils. 2. Explain the mechanisms by which a loss of CFTR function may contribute to CF symptoms. 3. Discuss the expected impact of CFTR correctors & potentiators in different tissues. S02 APP&D: The Failure to Clear CF Mucus (Research) 132 ABC Chairs Scott H. Donaldson, M.D. David A. Stoltz, Ph.D. 2:00 p.m. Introduction Scott H. Donaldson, M.D. 2:10 p.m. S2.1 The Link Between Airway Mucus Concentration & CF Pathogenesis Brian Button Ph.D. 2:35 p.m. S2.2 CF Mucus Properties & Attachment in the Intestinal & Respiratory Tracts Gunnar C. Hansson, M.D., Ph.D. 3:00 p.m. S2.3 Using Newborn CF & Non-CF Pigs to Understand Mucus Lynda S. Ostedgaard, Ph.D. 3:25 p.m. S2.4 The Effect of Correctors & Potentiators on CF Mucus & Its Transportability Steven M. Rowe, M.D., MSPH Description This symposium will focus on our current understanding of how loss of CFTR function affects the biophysical properties of mucus & mucociliary transport. Objectives 1. Review the properties of mucus in humans with CF & CF animal models. 2. Describe the mucociliary transport defects in the CF airway. 3. Identify the effects of CFTR restoration on CF mucus. 41 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 2:00 p.m. - 3:50 p.m. Symposium Session I S03 INF/MIC: Challenging Topics in Infectious Disease (Combination) 120 A Chairs Charles S. Haworth, M.D., FRCP Valerie J. Waters, M.D., M.Sc. 2:00 p.m. Introduction Valerie J. Waters, M.D., M.Sc. 2:10 p.m. S3.1 Clostridium Difficile & Other Gut Infections in Patients With Pulmonary Disease Including CF Barry Plant, M.D. 2:35 p.m. S3.2 Eradication Therapy for Pseudomonas Aeruginosa; Beyond the Basics Felix Ratjen, M.D., Ph.D., FRCPC 3:00 p.m. S3.3 Nontuberculous Mycobacterial Infections in Individuals with CF Andres Floto, M.D., Ph.D., FRCP 3:25 p.m. S3.4 The Role of Viruses & the Impact of Newer Diagnostic Testing in CF Pedro A. Piedra, M.D. Description This session will focus on challenging topics in infectious diseases management of CF including the role of viruses & the impact of newer diagnostic testing, Clostridium difficile infections & how they impact CF patients, nontuberculous mycobacterial infections in CF & the eradication of Pseudomonas aeruginosa both in younger children as well as in adults. Objectives 1. Describe the role of viruses & newer diagnostic testing in patients with CF. 2. Discuss the impact of atypical infections such as Clostridium difficile & nontuberculous mycobacterial infections in CF. 3. Examine the different eradication strategies for Pseudomonas aeruginosa infection in children & adults with CF. S04 CLIN: CFTR & Pancreatitis (Combination) 120 BC Chairs Matthew J. DiMagno, M.D. Steven D. Freedman, M.D., Ph.D. 2:00 p.m. Introduction Steven D. Freedman, M.D., Ph.D. 2:10 p.m. S4.1 Role of CFTR in Normal Exocrine Pancreatic Function Steven D. Freedman, M.D., Ph.D. 2:35 p.m. S4.2 Defining Therapeutic Targets Based on the Pathogenesis of Pancreatitis Related to CFTR Matthew J. DiMagno, M.D. 3:00 p.m. S4.3 Clinical Manifestations of Pancreatitis in Classic & Atypical CF Keith C.Y. Ooi, MBBS, DipPaed, FRACP, Ph.D. 42 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 2:00 p.m. - 3:50 p.m. Symposium Session I 3:25 p.m. S4.4 Impact of CFTR on Beta Cell Function in the Pancreas, Clinical & Therapeutic Implications Antoinette Moran, M.D. Description This session will focus on the role of CFTR in normal exocrine pancreatic function & how defects predispose to pancreatitis. The link between specific CFTR mutations & risk for pancreatitis & exocrine pancreatic insufficiency will be discussed. Lastly, the impact of CFTR on beta cell function & the factors that predispose to CF-related diabetes mellitus will be reviewed. Objectives 1. Define the role of CFTR in normal exocrine pancreatic function & how defects lead to pancreatitis. 2. Identify how specific CFTR mutations predict the risk of pancreatitis & the implications for specific therapies. 3. Describe the impact of loss of normal CFTR function on beta cell function & its clinical implications. S05 CLIN: Toward Rational Management of Pulmonary Exacerbations: Part III (Combination) 301 BC Supported by an educational grant from Gilead Sciences, Inc. Chairs Patrick A. Flume, M.D. Christopher H. Goss, M.D., M.Sc. Donald R. VanDevanter, Ph.D. 2:00 p.m. Introduction Patrick A. Flume, M.D. 2:10 p.m. S5.1 The Current State of Exacerbation Management Don B. Sanders, M.D., M.S. 2:35 p.m. S5.2 The STOP Trial: An Observational Pilot & Feasibility Study of Exacerbation Management Natalie E. West, M.D., M.H.S. 3:00 p.m. S5.3 The Challenge of Exacerbation Trial Design: What Is the Endpoint? Donald R. VanDevanter, Ph.D. 3:25 p.m. S5.4 The Next Chapter for the Treatment of Acute Pulmonary Exacerbation in CF: What Is the Next Study? Christopher H. Goss, M.D., M.Sc. Description Pulmonary exacerbations are common events in patients with CF but there is no standard approach to treatment. We will review what we have learned from registry data, clinical studies, & an observational study of exacerbations, & how this will be used in the design of interventional studies of the treatment of exacerbations. Objectives 1. Review the epidemiology of CF pulmonary exacerbations & their management. 2. Summarize the topline results from an observational study of pulmonary exacerbations. 3. Define the relevant clinical endpoints for the design of interventional studies of pulmonary exacerbations. 43 | 2015 North American CF Conference FINAL PROGRAM THURSDAY, OCTOBER 8 2:00 p.m. - 3:50 p.m. Symposium Session I S06 NRS: Partnering With Patients & Families to Promote Healthy Lifestyles (Care) 301 A Chairs Beth Harkness, R.N., B.S.N. Connie I. Richless, R.N., M.N. 2:00 p.m. Introduction Connie I. Richless, R.N., M.N. 2:10 p.m. S6.1 Mindful Strategies to Deal With Stress, Anxiety & Conflict Associated With Chronic Illness Daniel S. Lewin, Ph.D. 2:35 p.m. S6.2 Wellness Through Diet: A New Spin on Healthy Eating for the Whole Family of a CF Household Laura Grande, M.S., R.D., C.S.P., L.D. 3:00 p.m. S6.3 Is There a Role for Alternative Therapies & Evidence-based Natural Healing Techniques in CF Care? Sonja R. Ams, L.Mt., R. EEGT, N.L.P. 3:25 p.m. S6.4 Exercise & Fitness in CF: What Workouts Work? David M. Orenstein, M.D., M.A. Description This symposium focuses on a holistic approach to wellness strategies for patients & families through dietary, physical, emotional & lifestyle interventions. The presentations will encompass coping strategies & stress reduction, a new spin on healthy dietary considerations, evidence-based complementary healing modalities & practical exercises & their impact on patients' health. Objectives 1. Describe potential coping strategies & alternative therapies that promote health & wellness. 2. Discuss three dietary considerations to promote a healthy lifestyle for individuals with CF & their families. 3. List recommended exercises for individuals with CF to promote their physical well-being. S07 PT & RT: Non-pharmacological Treatment of Pain in CF: A Musculoskeletal Approach (Care) Chairs Kamyar Afshar, D.O. Kathryn S. Moffett, M.D., FAAP 2:00 p.m. Introduction Kamyar Afshar, D.O. 2:10 p.m. S7.1 Living With Pain Kamyar Afshar, D.O. 2:35 p.m. S7.2 Virtual Reality & the Management of Pain Jeffrey I. Gold, Ph.D. 44 | 2015 North American CF Conference 301 D FINAL PROGRAM THURSDAY, OCTOBER 8 2:00 p.m. - 3:50 p.m. Symposium Session I 3:00 p.m. S7.3 Hypnotic Approaches for Discomfort: Freeing the Mind & Body to Become More Physically Active Ran Anbar, M.D. 3:25 p.m. S7.4 Changing CF Postural Outcomes With Physical Therapy Mary Massery, P.T., D.P.T. Description Chronic pain is a common issue in individuals with CF. When identified, health care providers routinely prescribe pharmacological agents to reduce the burden of pain. This session will discuss the current state of pain issues & delve into nonpharmacological interventions, such as physical medicine & behavioral modification, useful in pain management strategies for individuals with CF. Objectives 1. Review the qualitative descriptors of pain in CF. 2. Recall the complementary & non-pharmacologic modalities for pain. 3. Describe practical techniques in behavioral & physical therapy, such as hypnosis, behavior modification & thoracic release & identify how these learned techniques can be implemented with minimal effort or resources. 3:50 p.m. - 4:20 p.m. Coffee Break North 300 Level Lobby 4:20 p.m. - 6:00 p.m. Plenary Session I ALL: Personalized Medicine (Combination) John Paul Clancy, M.D. Halls AB Description This session will discuss the concept of personalized medicine in CF, including how therapies are currently personalized & how new medications & tools will allow us to personalize therapy in a precise way. Objectives 1. Define the role of CFTR-targeted therapies in personalization of CF care. 2. Describe how patient-derived model systems may facilitate the evaluation of new therapies on a patient-specific basis. 3. Evaluation the role of emerging monitoring tools to track new CF patient interventions. 6:30 p.m. - 8:00 p.m. ALL: Welcome Reception 45 | 2015 North American CF Conference Heritage Park TAB SHEET 5 FRIDAY FINAL PROGRAM FRIDAY, OCTOBER 9 7:00 a.m. - 6:00 p.m. General & Exhibitor Registration (PCC) North 300 Level Lobby 7:30 a.m. - 8:45 a.m. Poster Session II (Even Numbers) Exhibit Hall E 7:30 a.m. - 8:45 a.m. Continental Breakfast/Visit Exhibits Exhibit Hall E 7:30 a.m. - 6:00 p.m. Exhibit Hall Open Exhibit Hall E 8:00 a.m. - 10:00 p.m. Special Interest & Related Study Groups See Insert for location 9:00 a.m. - 10:00 a.m. Plenary Session II ALL: Opening Doors to CF Clinical Research (Research) George Z. Retsch-Bogart, M.D. Halls AB This session will review highlights of clinical research from the past year & look forward to the large number of new studies ahead. The increased number of Description studies & potential new therapies will allow even more patients & centers to get engaged in clinical research. New approaches & tools will be needed to accomplish this work. This session will address the role centers will play going forward. 1. Describe & summarize key results for recent trials in CF & explain their significance within the therapeutic class. 2. Review the upcoming trials across each therapeutic area & illustrate the increased need for patient & center participation to meet the goals of better Objectives therapies. 3. Demonstrate the importance of research in improving outcomes & explain how to access research information. 10:00 a.m. - 10:30 a.m. Coffee Break 47 | 2015 North American CF Conference 120 Foyer 100 Level Corridors 301 Foyer FINAL PROGRAM FRIDAY, OCTOBER 9 10:30 a.m. - 12:20 p.m. Symposium Session II S08 CFTR: It’s in Your Genes: Understanding CFTR Gene Regulation to Improve CF Treatment (Research) 120 BC Chairs Ann Harris, Ph.D. Martin J. Walsh, Ph.D. 10:30 a.m. Introduction Ann Harris, Ph.D. Martin J. Walsh, Ph.D. 10:40 a.m. S8.1 Modulation of Gene Expression Using Epigenome Editing Eric Mendenhall, Ph.D. 11:05 a.m. S8.2 Higher Order Chromatin Structure & Expression of the CFTR Locus Ann Harris, Ph.D. 11:30 a.m. S8.3 Non-coding RNAs & Topological Chromatin Domains Predict the Fate of CFTR Transcription by RNA Polymerase II Martin J. Walsh, Ph.D. 11:55 a.m. S8.4 Identification of Cis- & Trans-Regulatory Factors That Govern the Specific Expression of CFTR Gene: New Targets for CF Magali Taulan-Cadars, Ph.D. Description Mechanisms of regulation of expression of the CFTR gene are complex & involve aspects of chromatin structure, transcriptional & post-transcriptional control. Substantial recent progress in investigating these mechanisms opens the possibility of manipulating gene expression for therapeutic benefit. Specifically increasing CFTR mRNA abundance could provide more substrate for corrector drugs. Objectives 1. Apply a general understanding of gene expression mechanisms & discuss how this knowledge may be applied to alter gene expression levels for therapeutic benefit. 2. Discuss how the CFTR gene is regulated in differentiated cell types. 3. Explain novel methods to find regulatory elements & understand how they function. S09 CFTR-NT: Developing Co-therapies to Modulate CFTR (Combination) Chairs Margarida D. Amaral, Ph.D. Joseph M. Pilewski, M.D. 10:30 a.m. Introduction Joseph M. Pilewski, M.D. 10:40 a.m. S9.1 Long-term Clinical Effects of CFTR Co-therapy With Lumacaftor/Ivacaftor Christiane De Boeck, M.D., Ph.D. 11:05 a.m. S9.2 How to Target Different Combinations of CFTR Mutations Margarida D. Amaral, Ph.D. 48 | 2015 North American CF Conference 120 D FINAL PROGRAM FRIDAY, OCTOBER 9 10:30 a.m. - 12:20 p.m. Symposium Session II 11:30 a.m. S9.3 New Strategies to Identify Correctors Puay-Wah Phuan, Ph.D. 11:55 a.m. S9.4 Regulatory Considerations Applicable to the Development for CFTR Modulator Therapies Anthony G. Durmowicz, M.D. Description This session will highlight the issues around co-therapies from the clinical to the development. Objectives 1. Discuss the need for co-therapies in CF for different combinations of CFTR mutations & the benefits & disadvantages of co-therapies. 2. Analyze how to screen for additive/synergistic drugs. 3. Demonstrate how to assess benefit for CF co-therapies. S10 NT: Assessing Presymptomatic Children (Combination) 120 A Chairs Timothy Rosenow, B.Sc. Edith T. Zemanick, M.D. 10:30 a.m. Introduction Edith T. Zemanick, M.D. 10:40 a.m. S10.1 How Can Biomarkers Help in Children With CF? Scott D. Sagel, M.D., Ph.D. 11:05 a.m. S10.2 Imaging the CF Lung in Young Children: Developments in CT & MRI Timothy Rosenow, B.Sc. 11:30 a.m. S10.3 Lung Clearance Index to Monitor Lung Function in Young Children Felix Ratjen, M.D., Ph.D., FRCPC 11:55 a.m. S10.4 Of Mucins, Microbiome & Metabolome: Assessing the Pathobiology of Early CF Airways Disease Charles R. Esther, Jr., M.D., Ph.D. Description CF lung disease often begins before the onset of overt clinical symptoms. Objective monitoring of disease progression is challenging, particularly in young children. This symposium will highlight advances in lung function, imaging, metabolomics & biomarkers aimed at detecting early changes in the CF lung. Objectives 1. Review the need for improved, sensitive measures of lung disease in presymptomatic children with CF. 2. Assess the role of chest imaging & lung clearance index in detection of lung disease in presymptomatic children. 3. Evaluate the utility of biomarkers in monitoring disease progression in presymptomatic children. 49 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 10:30 a.m. - 12:20 p.m. Symposium Session II S11 INF/MIC: MRSA Update (Combination) 301 BC Chairs Deirdre F. Gilpin, Ph.D. Clement L. Ren, M.D. 10:30 a.m. Introduction Clement L. Ren, M.D. 10:40 a.m. S11.1 Epidemiology & Outcomes of Staphylococcus Aureus Infection in CF Lucas R. Hoffman, M.D., Ph.D. 11:05 a.m. S11.2 Methicillin Resistance in Staphylococcus Aureus (MRSA) Genetic Types in MRSA & Their Relevance to CF Marianne S. Muhlebach, M.D. 11:30 a.m. S11.3 STAR-Too Clinical Trial Christopher Goss, M.D., M.Sc. 11:55 a.m. S11.4 Emerging Therapies in CF: AeroVanc for the Treatment of Chronic MRSA Elliott C. Dasenbrook, M.D., M.H.S. Description This session will provide an update on MRSA infection in CF patients. Objectives 1. Review the most recent findings regarding the epidemiology & outcomes associated with MRSA infection in CF patients. 2. Describe current studies designed to eradicate MRSA infection in CF patients. 3. Summarize the current & emerging therapies for MRSA infection in CF patients. S12 CLIN: CF With Fresh Eyes: A Partnership to Create the Future? (Care) 131 ABC Supported by an educational grant from Gilead Sciences, Inc. Chairs Paul Batalden, M.D. Cynthia A. George, M.S.N., F.N.P. Mary-Leigh Phillips (Remote) 10:30 a.m. Introduction Cynthia A. George, M.S.N., F.N.P. 10:40 a.m. S12.1 Windows on CF Today Remote Panelist: Mary-Leigh Phillips 10:55 a.m. S12.2 Introduction on Co-production of a Service Paul Batalden, M.D. 11:10 a.m. S12.3 Panelists Share Reactions to Co-production of Service Live Panelists: Debbie Benitez, R.N., M.S.N., ACNP, Gregory Sawicki, M.D., M.P.H., Kathryn A. Sabadosa, M.P.H. Remote Panelist: Daniel Schulke, J.D. 11:40 a.m. S12.4 Lessons Learned From Other Disease Communities Peter Margolis, M.D., Ph.D. 50 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 10:30 a.m. - 12:20 p.m. Symposium Session II 12:10 p.m. S12.5 Importance of Taking Co-production Into Consideration Bruce C. Marshall, M.D. Description This symposium will explore some of the implications of co-production in the world of CF. Patients, family & professionals will work together to offer an introduction to the idea & to begin the process of seeing its relevance in the daily work life of all involved in designing the future of CF healthcare services. Objectives 1. Demonstrate that knowledgeable, skillful patients & professionals working together are essential for better outcomes in CF. 2. Recall that fear of new ways to collaborate must be replaced with exploration & discovery of new ways to work together. 3. Discuss the appreciation of the role that language plays in framing our personal understanding & action-taking. S13 NRS: When Addiction Takes Over: Managing Dependence Issues in the 121 ABC Patient With CF (Care) Chairs Nicola J. Felicetti, R.N., B.S. Corinne Muirhead, Pharm.D. 10:30 a.m. Introduction Nicola J. Felicetti, R.N., B.S. 10:40 a.m. S13.1 Two Case Studies: Supporting One Another When Outcomes Are Not Favorable Nicola J. Felicetti, R.N., B.S. 10:55 a.m. S13.2 Marijuana & CF: Controversies Associated With Patient Use Corinne Muirhead, Pharm.D. 11:05 a.m. S13.3 The Rational Use of Opioids in CF Rob Horowitz, M.D. 11:15 a.m. S13.4 Addressing Issues Surrounding Pain Management & Drug-Seeking Behaviors in CF Katie Whitley, R.N., B.S.N. Description This symposium will provide insight into the multidisciplinary & multifaceted levels of care that are necessary to provide health support for the patient with CF while concurrently managing the challenges of a substance use disorder. Objectives 1. List generally accepted, inappropriate & controversial indications for opioid prescription for painful conditions in CF. 2. Distinguish between opioid misuse, abuse, addiction & pseduoaddiction in patients with CF. 3. Discuss the complexity of addictive medication management in patients with CF, including skills to address existing concerns & prevent substance use problems. 51 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 10:30 a.m. - 12:20 p.m. Symposium Session II S14 NTR: Achieving Optimal Nutrition (Combination) 301 A Supported by Gilead Sciences, Inc. Chairs Asim Maqbool, M.D. Mary Marcus, M.S., R.D., C.S.P. 10:30 a.m. Introduction Asim Maqbool, M.D. 10:40 a.m. S14.1 CF Infant Growth & Pulmonary Status in the First Year of Life: The BONUS Study Daniel Leung, M.D. 11:05 a.m. S14.2 Making the Case for DHA in CF Steven Freedman, M.D., Ph.D. 11:30 a.m. S14.3 Making the Case for Linoleic Acid in CF Birgitta Strandvik, M.D., Ph.D. 11:55 a.m. S14.4 Nutritional Status of Infants with CF Diagnosed After Nationwide Newborn Screening in the US: Preliminary Findings from the FIRST Study Cohort HuiChuan Lai, Ph.D., R.D. Description This session will review current knowledge related to DHA, linoleic acid & fatty acid metabolism in CF. We will also discuss emerging data on early nutrition & growth in infants with CF. Objectives 1. Describe & discuss the concepts & controversies of LA supplementation in CF. 2. Describe & discuss the concepts & controversies of DHA supplementation in CF. 3. Compare & contrast preliminary results & findings in optimizing infant growth & nutrition from an ongoing study & a recently concluded study of infants with CF. 12:30 p.m. - 1:50 p.m. U.S. Pediatric Program Directors Meeting* Sheraton - Encanto *Invitations were coordinated independently from the NACFC registration process. Contact [email protected] with questions. (LIMITED SESSION: Attendance is limited to Directors of CFF-accredited Pediatric CF Programs. Pre-registration is required. ADMISSION BY TICKET ONLY.) 12:30 p.m. - 1:50 p.m. U.S. Adult Program Directors Meeting* Sheraton - Maryvale *Invitations were coordinated independently from the NACFC registration process. Contact [email protected] with questions. (LIMITED SESSION. Attendance is limited to Directors of CFF-accredited Adult CF Programs. Pre-registration is required. ADMISSION BY TICKET ONLY.) 12:30 p.m. - 1:50 p.m. U.S. Affiliate Program Directors Meeting* Sheraton - Ahwatukee *Invitations were coordinated independently from the NACFC registration process. Contact [email protected] with questions. (LIMITED SESSION. Attendance is limited to Directors of CFF-accredited Affiliate CF Programs. Pre-registration is required. ADMISSION BY TICKET ONLY.) 52 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. BB01 CLIN: Laboratory Processing: Not Just the Basics (Research) 105 ABC Moderator Jessica A. Baker, B.S. Speakers Jessica A. Baker, B.S. Jane Vroom, B.S. Description Quality control is key when processing specimens for a research study. Laboratory experience can range from former lab technicians to those who have never held a pipette. We will look at techniques from the obvious to the obscure to ensure proper specimen processing, as well as ways to troubleshoot should things go awry in the lab. Objectives 1. Review laboratory basics including aseptic technique, sample handling, processing & shipping. 2. Identify other laboratory practices & procedures that can make a significant difference in terms of specimen quality. 3. Discuss personal laboratory experiences & what can be learned from them. BB02 NRS: The Special Transition Needs of Adolescents With Autism & Other Cognitive Delays (Care) 106 AB Moderator Carlye T. Tomczyk. M.S.N. Speakers Christopher Boys, Ph.D., L.P. Alyssa Falos, LCSW Description This session will review the strategies used at a CF center in an effort to share the processes that have worked well & facilitate discussion with other CF teams. Discussion will include input from CF providers, social work & psychology. Objectives 1. Identify challenges & psychosocial stressors for adolescents with autism & developmental delays & their parents/caregivers. 2. Explain strategies to assess family support & community resources & facilitate thorough communication with adult CF team. 3. Identify strategies to equip parents & caregivers with the advocacy skills to ensure ongoing therapeutic alliances with adult CF team. BB03 SW/PSYCH: Communication Strategies to Create High Functioning Teams (Care) 102 AB Moderators Kecia L. Nelson, M.S.W., LCSW Jamie L. Wooldridge, M.D. Speaker Jamie L. Wooldridge, M.D. Description Communication basics will be reviewed & practiced at this interactive session. Objectives 1. Identify effective communication strategies for the healthcare setting. 2. Evaluate current communications practices on your team. 3. Practice communications strategies. 53 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. BB04 NTR: Nutrition in the NICU (Care) 102 C Moderators Susan L. Gemma, M.S., R.D., L.D. Laura Grande, M.S., R.D., C.S.P., L.D. Speakers Vanessa Carr, M.S., R.D.N., L.D.N. Catherine Fox, M.S., R.D., L.D. Elizabeth Reid, M.S., R.D.N. Description This session will focus on the infant with CF & meconium ileus & its associated nutrition complications. The speakers will present an overview of the nutritional management of these infants. The session will include a panel of dietitians with experience in caring for these nutritionally challenged infants. Objectives 1. Review the anatomy & key functions of the gastrointestinal tract. 2. Describe the complexity of nutritional interventions for an infant with CF born with meconium ileus. 3. Identify strategies for managing infants post meconium ileus. BB05 PT & RT: Exercise Testing Interpretation & Implementing an Exercise Prescription (Combination) 101 BC Moderators Matthew L. Bowman, P.T., D.P.T. Ryan Harris, Ph.D. Description Exercise capacity has been shown to predict patient outcomes, independent of lung function. Routine exercise testing is recommended, not only to evaluate exercise tolerance but to obtain the most effective exercise prescription for each individual patient. This session will expose you to the ins & outs of conducting an exercise test along with what & how to use the data. Objectives 1. Recall the essential monitoring requirements that are needed to conduct maximal exercise testing in patients with CF. 2. Identify further insight into the comprehensive information that can be obtained from a maximal exercise test in patients with CF. 3. Describe how maximal exercise testing can be used in a clinical setting to prescribe exercise to patients with CF. 12:30 p.m. - 1:50 p.m. Junior Investigators – Supported by Gilead Sciences, Inc. LIMITED SESSION. Pre-registration is required. ADMISSION BY TICKET ONLY. Basic Science Chairs Raymond J. Pickles, M.D. Assem Ziady, Ph.D. Jennifer Bomberger, Ph.D. Panelists Jonathan Cohn, M.D. Katherine Tuggle, Ph.D. Kerstin Pohl, B.Sc., Ph.D. 54 | 2015 North American CF Conference West Building 213 AB FINAL PROGRAM FRIDAY, OCTOBER 9 12:30 p.m. - 1:50 p.m. Junior Investigators – Supported by Gilead Sciences, Inc. LIMITED SESSION. Pre-registration is required. ADMISSION BY TICKET ONLY. Clinical Research West Building 212 ABC Chairs Alexandra L. Quittner, Ph.D. Anna M. Georgiopoulos, M.D. Panelists Frank J. Accurso, M.D. Kathryn Ramsey, Ph.D. 2:00 p.m. - 3:45 p.m. Workshop Session II W11 CFTR: Novel Insights Into CFTR 2015 (Research) 120 A Chairs Christine E. Bear, Ph.D. John C. Kappes, Ph.D. 2:00 p.m. Introduction Christine E. Bear, Ph.D. 2:10 p.m. Abstract Interactions & Cooperativity Between P-Glycoprotein Structural Domains Determined By Thermal Unfolding 7 Zhengrong Yang 2:25 p.m. Abstract The Role Of Regulatory Region On Calcium-Induced CFTR Activation Zoltan Bozoky, Ph.D. 8 2:40 p.m. Abstract Cotranslational Disruption of NBD1 Folding By CFTR2 Processing Mutations Hideki Shishido, Ph.D. 9 2:55 p.m. Abstract Inhibitors Of CFTR Ubiquitination Act Synergistically With Small Molecule Correctors to Enhance F508Del-CFTR Maturation & Activity 10 Jennifer Goeckeler-Fried, B.S. 3:10 p.m. Abstract Monitoring CFTR Thermal Unfolding By Cysteine Labeling & Control of CFTR Function By Cysteine Cross-Linking 11 Lihua He, Ph.D. 3:25 p.m. Abstract Predicting Readthrough Therapy Efficiency by Measuring Recoded Channel Activity 12 Alexandre Hinzpeter, Ph.D. Description This session deals with fundamental questions central to our understanding of how the CFTR protein is structured & functions as an anion channel. There are also presentations related to the molecular basis for disease cause by mutations in CFTR & therapeutic strategies to repair such defects. Objectives 1. Discuss the current understanding regarding structure function relationships in CFTR. 2. Explain molecular defects caused by mutation. 3. Describe the mechanism of action of emerging interventions targeting defects caused by CFTR mutations. 55 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W12 APP&D: Mobilizing CF Mucus (Research) 121 ABC Chairs Camille Ehre, Ph.D. Paul M. Quinton, Ph.D. 2:00 p.m. Introduction Paul M. Quinton, Ph.D. 2:10 p.m. Abstract An Acidic PH Increases the Viscosity of Cystic Fibrosis Airway Surface Liquid Xiao Xiao Tang, Ph.D. 91 2:25 p.m. Abstract Altered ASL PH & Viscosity in Cultured Small Airway Cells From Cystic Fibrosis Pigs 92 Xiaopeng Li, Ph.D. 2:40 p.m. Abstract Delayed Goblet Cell Exocytosis in CFTR KO Intestinal Organoids Lane L. Clarke, D.V.M., Ph.D. 93 2:55 p.m. Abstract CFTR Rescue Affects Secreted Mucins & Mucus Martina Gentzsch, Ph.D. 94 3:10 p.m. Abstract Gel-forming Mucus in Response to Infection & Inflammation in Normal & Cystic Fibrosis Airways 95 Mehmet Kesimer, Ph.D. 3:25 p.m. Abstract Pharmacological Approaches to Clear Mucus From the Lungs Camille Ehre, Ph.D. 96 Description This session will provide a state-of-the art assessment of factors affecting mucus viscoelasticity & mucus clearance in in vitro & in vivo models of CF. These presentations will focus on the relationship between defective ion transport, fluid secretion & mucus biophysical properties, as well as methods for evaluating mucus transport & potentially normalizing mucus in a CF environment. Objectives 1. Explain effects of ion composition on biophysical properties of mucus. 2. Describe CF & normal mucus secretion & transport. 3. Identify pharmacological strategies to promote mucus clearance. 56 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W13 GMS: Genetics, Genomics & Big Data Sets (Research) 124 AB Chairs Rebecca Marsick Darrah, Ph.D., M.S. Jennifer L. Taylor-Cousar, M.D. 2:00 p.m. Introduction Jennifer L. Taylor-Cousar, M.D. 2:10 p.m. Abstract Common & Rare Variation Associated With the F508DEL Mutation Identified by Deep Re-sequencing of CFTR in 602 Cystic Fibrosis Patients 151 Briana Vecchio-Pagan 2:25 p.m. Abstract Variations in MRNA Stability & Protein Processing Associated With Splice-site & Nonsense Mutations in CFTR 152 Neeraj Sharma, D.V.M., Ph.D. 2:40 p.m. Abstract Read-through Level in the Most Frequent CFTR Mutations Linked to Premature Termination Condons 153 Isabelle Sermet-Gaudelus, M.D. 2:55 p.m. Abstract Loss of Angiotensin II Type 2 Receptor Restores CF Pulmonary Phenotype Rebecca Marsick Darrah, Ph.D., M.S. 154 3:10 p.m. Abstract Reduced SLC26A9 Function Increases Intestinal Obstruction in Cystic Fibrosis Mice 155 Megan S. Vitko 3:25 p.m. Abstract Variation in the Epithelial Sodium Channel Gene SCNN1B is Strongly Associated With Decline in Lung Function in the Epic Cystic Fibrosis Cohort 156 Mary Emond, Ph.D. Description During this session, we will explore the role of DNA & mRNA variation in CFTR mutations as well as the role of non-CFTR genetic modifiers in the phenotypic expression of disease. Objectives 1. Describe the contributions of CFTR variation in phenotypic manifestations of disease. 2. Analyze how differential expression of CFTR mutations affects the potential for therapeutic targets. 3. Discuss the potential role of non-CFTR genetic modifiers in the phenotypic expression of disease. 57 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W14 NT: Early Studies of Novel Small Molecule Therapies (Research) 132 ABC Chairs Peter M. Haggie, Ph.D. David N. Sheppard, Ph.D. 2:00 p.m. Introduction Peter M. Haggie, Ph.D. 2:10 p.m. Abstract Correction of F508DEL CFTR Using Nanoparticles Delivering Triplex-forming Peptide Nucleic Acid Molecules 197 Marie E. Egan, M.D. 2:25 p.m. Abstract Novel Therapeutic Approach for Cystic Fibrosis Caused by the W1282X Premature Termination Codon 198 Peter M. Haggie, Ph.D. 2:40 p.m. Abstract Mechanistic Analysis of Rare CFTR2 Alleles Eric J. Sorscher, M.D. 199 2:55 p.m. Abstract Amplifiers Are a New Class of CFTR Modulators That Increase the Abundance of CFTR Proteins & Combined With Potentiators & Correctors Enhance CFTR Chloride 200 John Miller 3:10 p.m. Abstract The ENAC Inhibitor P-1037 Is a CFTR-independent Therapeutic Agent That Promotes Sustained Airways Hydration & Mucociliary Transport 201 Bill Thelin, Ph.D. 3:25 p.m. Abstract SPLUNC1 Peptide-derivatives With Increased Efficacy & Decreased Renal Side Effects In Vitro & In Vivo 202 Robert Fellner, Ph.D. Description This session will consider novel small molecule therapeutic approaches directed at CF relevant drug targets, including delF508-CFTR, epithelial sodium channels & less frequent CFTR mutations. The session provides perspectives from academic & industrial laboratories. Objectives 1. Discuss innovative approaches to restore the expression, stability & function of delF508-CFTR. 2. Review the challenges of developing rational new therapies for rare CF mutations. 3. Explain the therapeutic potential for CF lung disease of small molecules that modify ENaC function. 58 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W15 INF/MIC: Microbiome & Emerging Pathogens (Research) 301 BC Supported by an educational grant from Gilead Sciences, Inc. Chairs Douglas J. Conrad, M.D. Juliette Madan, M.D., M.S. 2:00 p.m. Introduction Douglas J. Conrad, M.D. 2:10 p.m. Abstract Prospective Algorithm for Treatment of Nontuberculous Mycobacteria in Cystic Fibrosis (PATIENCE) Trial Stacey L. Martiniano, M.D. 2:25 p.m. Abstract Inhibition of Pseudomonas Aeruginosa by Staphylococcus Aureus-Escherichia Coli Co-culture 299 Vanessa Phelan 2:40 p.m. Abstract Early Evolution of Pseudomonas Aeruginosa During CF Infection Peter Jorth, Ph.D. 300 2:55 p.m. Abstract Culture Enriched Metagenomic Sequencing of the CF Lung Microbiota Provides a More Thorough Understanding of Microbial Processes During Exacerbation 302 Fiona Jane Whelan 3:10 p.m. Abstract Microbial Community Composition in Explante CF Lungs Michael M. Tunney, Ph.D. 304 3:25 p.m. Abstract Taxonomic & Functional Metagenomic Analysis of Sputum Samples From Stable CF Patients With Lower & Higher Pulmonary Function Decline 306 Annamaria Bevivino, B.S. Description The dynamic interactions of the microbial populations in the airway of CF patients can drive short & long term health. Newer technologies & bioinformatic approaches provide a comprehensive characterization & demonstrate interactions in unprecedented detail. This workshop highlights the CF microbiome, emerging pathogens & their impact on the health of CF patients. Objectives 1. Describe new technologies & bioinformatic approaches that allow the comprehensive analysis of the CF microbiome. 2. Identify known & emerging pathogens in CF patients & how they might interact with other microbial pathogens to drive CF patient health. 3. Discuss genomic & phenotypic adaptations of the microbial communities to survive in the CF airway. 59 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W16 CLIN-INF/MIC-NTR: CF Newborn Screening (Combination) 120 BC Chairs Philip M. Farrell, M.D., Ph.D. Adrienne Savant, M.D., M.S. 2:00 p.m. Introduction Philip M. Farrell, M.D., Ph.D. Adrienne Savant, M.D., M.S. 2:10 p.m. Abstract Differences in Outcomes Between Early & Late Diagnosis of Cystic Fibrosis in the NBS Era 494 Keith C.Y. Ooi , MBBS, DipPaed, FRACP, Ph.D. 2:25 p.m. Abstract Feeding, Growth & Essential Fatty Acid Status in Infants With CF Diagnosed Through Newborn Screening in 2012-13 495 HuiChuan J. Lai, Ph.D., R.D. 2:40 p.m. Abstract Pancreatic Enzyme Use in Infants With CF in the First Year of Life Drucy Borowitz, M.D. 496 2:55 p.m. Abstract Clinical Blood Hematology & Chemistry in CF Infants Sonya L. Heltshe, Ph.D. 497 3:10 p.m. Abstract Clinical Outcomes in Pediatric Patients With CFTR-related Metabolic Syndrome (CRMS) 498 Renee Temme, M.S., C.G.C. 3:25 p.m. Abstract Phenotypes Associated With (IVS8)-(TG)M-5T Increasing (TG) Repeat Length in Children With a Positive California Cystic Fibrosis Newborn Screen 499 Danieli B. Salinas, M.D. Description Early studies helped us to understand the benefit of early diagnosis through newborn screening for CF. This session will help further our knowledge & understand what happens to these patients after the newborn screen is positive. Objectives 1. Compare differences in outcomes for patients diagnosed early versus late in the era of newborn screening. 2. Identify the characteristics in early life for infants, such as nutritional status, including pancreatic supplementation practices & hematologic & chemical laboratory outcomes. 3. Describe the outcomes for patients with specific special outcomes, such as CRMS & polyT/TG repeats. 60 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W17 CLIN: Gastroenterology/Liver/Pancreas (Combination) 120 D Chairs James M. Abraham, M.D. Missale Solomon, M.D. 2:00 p.m. Introduction James M. Abraham, M.D. Missale Solomon, M.D. 2:10 p.m. Abstract Cystic Fibrosis Colorectal Cancer Screening: A Single Center Experience David Niccum, M.D. 560 2:25 p.m. Abstract Ultrasound Screening for Liver Disease in Pediatric Cystic Fibrosis: Reducing the Cost, Reducing the Burden 561 Latifa Patel, MBChB(Hons), M.Phil(Child Health) 2:40 p.m. Abstract Improving Nutrition in Pediatric Cystic Fibrosis Patients Joseph N. Walter, M.D. 562 2:55 p.m. Abstract In-line Digestive Enzyme Cartridge (EFICTM), as a Part of Enteral Feeding, Facilitates Effective Fat Absorption & Increases Omega 3 Plasma Levels in Pig 563 Model of Exocrine Pancreatic Insufficiency (EPI) Danica Grujic, Ph.D. 3:10 p.m. Abstract TMEM16A, an Apical Membrane Cl- Channel, Is Present & Functional in CF Cholangiocytes & Represents a Therapeutic Target to Improve Bile Flow in CF 565 Liver Disease Amal K. Dutta, M.D., Ph.D. 3:25 p.m. Abstract Fecal Human Beta-defensin 2 Levels in Children With Cystic Fibrosis 566 Chee Ooi, FRACP Description A focused discussion of emerging evidence for improving care & clinical outcomes in adult & pediatric patients with gastrointestinal/hepatic/pancreatic (GI) sequelae of Cystic Fibrosis (CF), particularly regarding concerted treatment & screening initiatives that will come into play for more CF patients with progressively rising life expectancy outcomes. Objectives 1. Evaluate available screening strategies for evolving CF GI diseases. 2. Discuss how structured Quality Improvement processes can strengthen the patient/provider partnership in CF Nutrition. 3. Examine strategies to optimize pancreatic enzyme supplementation & efficacy in CF patients. Consider potential biomarkers & therapeutic pathways for future treatment of CF GI diseases. 61 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W18 NRS: Innovative Strategies in Inpatient & Outpatient Care Management (Care) 131 ABC Chairs Amanda Dressman, R.N., M.S.N., C.N.P. Jana B. Yeley, R.N., M.S.N., N.P.-B.C. 2:00 p.m. Introduction Jana B. Yeley, R.N., M.S.N., N.P.-B.C. 2:10 p.m. Abstract Socialization Among Inpatients With Cystic Fibrosis Via Technology & An Infection Control Approved Novel Playroom 662 Danielle Corrigan, M.D. 2:25 p.m. Abstract Investigating the Use of Social Media in Cystic Fibrosis Education Anju Anand, M.D. 663 2:40 p.m. Abstract Feasibility of Home Spirometry Measurement in Children With Cystic Fibrosis Aarti Shakkottai, M.D. 664 2:55 p.m. Abstract Standardized Nursing Tool for Telephone Assessment of Pulmonary Exacerbation: A Quality Improvement Project 665 Janine Cassidy, M.S.N., R.N., C.P.N. 3:10 p.m. Abstract Advanced Care Planning in Pediatric CF Patients: A Quality Improvement Project Traci M. Kazmerski, M.D. 666 3:25 p.m. Abstract Improving Clinical Care of the CF Patient in a New Impatient Setting Kathleen L. Peeke, M.S., R.N., A.P.N. 667 Description A review of various "hot topics" in the CF centers across the nation. Improving care in both settings with quality improvement, education & technology. Includes projects with patients, families & staff of both the inpatient & outpatient setting. Objectives 1. Discuss some options for using social media & technology. 2. Review tools for evaluating pulmonary exacerbations. 3. Describe & apply education for CF providers. 62 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W19 SW/PSYCH-NRS: Working Together: Pediatric & Adult Centers Collaborating (Care) 122 ABC Chairs Staci T. Self, M.S.W. Jeanne Weiland, R.N., M.S.N., C.N.P. 2:00 p.m. Introduction Staci T. Self, M.S.W. Jeanne Weiland, R.N., M.S.N., C.N.P. 2:10 p.m. Abstract Combined Adult & Pediatric Quarterly Team Meetings Provide Value to the Team Lisa A. Burns, M.D. 610 2:25 p.m. Abstract Journey to Independence: A Qualitative Needs Assessment for Transition in Cystic Fibrosis 611 Robert Lucia, M.A., LCPC, NCMHC Heather McLauchlan, M.D. 2:40 p.m. Abstract Development & Implementation of a Comprehensive Transition Program Facilitated by a Transition Checklist 612 Jennifer Ruddy, M.D. 2:55 p.m. Abstract The Dallas Dashboard: Quality Improvement in Transition of CF Care Jamie Becker, Ph.D. 613 3:10 p.m. Abstract Transition Program at a Large CF Center: Readiness, Handoff & Transfer – How Are We Doing? 614 Suzanne Cumming, R.N., B.S.N. 3:25 p.m. Abstract "Ready, Set, Go" Video: A Journey to Transition Barbara R. Baum, LMSW 615 Monica R. Lopez, M.S.W. Description Adult & pediatric CF teams across the country work hard to ensure that young adults are prepared for a transition from pediatric to adult care. Presenters will share strategies & tools for improving communication between pediatric & adult teams & will demonstrate how collaboration between teams, patients & families leads to successful entrance into adult CF care. Objectives 1. Describe the value of collaboration between pediatric & adult CF care teams. 2. Identify tools that facilitate communication & partnership between pediatric & adult providers. 3. Review strategies to prepare young adults to successfully transition from pediatric to adult CF care. 63 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 2:00 p.m. - 3:45 p.m. Workshop Session II W20 PT & RT: Aerosol Therapy: How Are We Delivering Inhaled Medications in 2015: Past, Present & Future (Combination) 301 A Supported by an educational grant from Gilead Sciences, Inc. Chairs Kathleen Richards, R.R.T. Gregory Sawicki, M.D., M.P.H. 2:00 p.m. Introduction Gregory Sawicki, M.D., M.P.H. 2:10 p.m. Abstract Choosing a Nebulizer That Satisfies Cystic Fibrosis Patients' Need & Hospital Budget 457 Shine-Ann Pai, B.S., R.R.T. 2:25 p.m. Abstract The Advantages of the Protective Role of Hyaluronic Acid & Mannitol to Hypertonic Saline Inhalation Treatment 458 Ugo Pradal, M.D. 2:40 p.m. Abstract Long-term Safety & Efficacy of Tobramycin Inhalation Powder Hard Capsules (TIPTM) in Patients With CF: An Extension to the ETOILES Study 459 Isabel L. Virella-Lowell, M.D. 2:55 p.m. Abstract Medication Adherence Comparing Tobramycin Inhalation Powder & Solution in Patients With CF Using the Dutch Pharmo Database Network 460 Paul Mastoridis 3:10 p.m. Abstract Frequency of Self-reported Barriers in Relation to Medication Adherence in Adolescents With Cystic Fibrosis 461 Christina Nicolais 3:25 p.m. Abstract A Retrospective Analysis of Patient Chest Physiotherapy Practices & Adherence to Inhaled Therapies Before & After CFTR Modulation With Ivacaftor 462 Claire Hickey Description Physicians must choose from an ever-expanding arsenal of therapies intended to maintain lung health for patients with CF. There has been extensive research not only in the development of these medications, but also in their improved delivery. This workshop will focus on the safety & efficacy, as well as patient adherence in the evolving era of aerosolized medications. Objectives 1. Describe strategies for choosing nebulizers for the inpatient setting & minimizing immediate side effects of aerosolized therapies. 2. Discuss the long-term safety & efficacy of Tobramycin Inhalation Powder (TIP™) & adherence to TIP™ versus Tobramycin Inhalation Solution. 3. Identify barriers to medication adherence & discuss the changes to adherence after using ivacaftor. 3:45 p.m. - 4:45 p.m. Coffee Break 64 | 2015 North American CF Conference Exhibit Hall E FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Caregivers Sessions by Discipline CG09 CLIN: Epi/Stat: Data, Data Everywhere (Combination) 226 ABC Moderators Theodore G. Liou, M.D. Rhonda Szczesniak, Ph.D. Speakers David J. Pasta, M.S. Annalisa Piccorelli, Ph.D. Brandie D. Wagner, Ph.D. Description This session will summarize the latest design & analysis applications for CF studies while simultaneously providing a platform for biostatisticians & epidemiologists to discuss, compare & contrast novel approaches. Objectives 1. Apply techniques for separating age effects from cohort (calendar year) effects in patient registries. 2. Discuss left truncation, in the form of delayed entry, in joint models for survival & lung function outcomes. 3. Assess the utility of novel statistical methods for CF studies. CG10 NRS: Holding On To Hope – Advanced Practice Skills to Diagnose & Treat Anxiety & Depression (Care) 232 ABC Moderators LaCrecia J. Thomas, R.N., M.S.N., CPNP-AC/PC Elika Rad, M.S.N., R.N., N.P.-C. Speakers Lynne M. Fries, MPAS, D.P.T., P.A.-C. Brant Oliver, Ph.D., M.S., M.P.H., APRN-BC Yelizaveta Sher, M.D. Description Implementing guidelines & diagnostic tools for depression & anxiety with a survey of treatment options using interactive case studies. Objectives 1. Create a plan for implementation of the CFF anxiety & depression screening guidelines. 2. Identify tools for diagnosis of anxiety & depression & techniques for communicating the diagnosis with patients & families. 3. Compare management strategies & treatment plans for depression & anxiety. 65 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Caregivers Sessions by Discipline CG11 NRS: Nursing Caregivers: The "Other" Disciplines (Care) 231 ABC Moderators Lisa J. Lunsford, D.N.P., CPNP Darci Pfeil, R.N., M.S., APNP Speakers Julie L. Aulner, P.T. Kay L. Batzer, R.R.T., CPFT Catherine A. Decker, Pharm.D., A.E.-C. Erin Seffrood, M.S., R.D., C.S.P., C.D. Description CF nurses are placed in the unique position of managing multiple issues for their pediatric & adult patients. This session will feature an opportunity to hear from the other disciplines: dietary, pharmacy, respiratory therapy & physical therapy & will include an interactive discussion on the nuances of nutritional supplements as well as practical tips on adherence to ACT & other therapies. Objectives 1. Discuss the intricacies of enzyme programs as well as dosing of enzymes & the various avenues to maintaining the BMI. 2. Describe the dynamic interplay of chest physiotherapy & exercise including huff coughing in maintaining lung function. 3. Describe common interactions, adherence strategies & timing of medications commonly used in CF including antibiotics. CG12 NTR: CF Gut Lumen Abnormalities & the Gut Microbiome (Care) 224 AB Moderators Eileen Potter, M.S., R.D. Kimberly G. Stephenson, M.S., R.D., C.S.P. Speakers Amy Garcia, M.D. Daina B. Kalnins, R.D., M.Sc. Missale Solomon, M.D. Kimberly G. Stephenson, M.S., R.D., C.S.P. Jackie Taylor, R.D., C.S.P. Description This session is designed for dietitian caregivers to learn more about abnormalities of the CF intestine & pancreas & their effect on digestion & absorption. Topics included in this session will be: the CF gut lumen, the gut microbiome, pancreatic status, probiotics, & practical applications to medical nutrition therapy. Objectives 1. Identify CF-specific changes in the gut lumen. 2. Formulate nutrition care plans that treat identifiable GI symptoms in CF. 3. Describe changes in pancreatic status related to CF & their effect on absorption. 66 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Caregivers Sessions by Discipline CG13 CLIN: PHARM: Clinical Controversies in CF Care (Care) 221 ABC Moderators Kevin L. Epps, Pharm.D., BCPS Corinne Muirhead, Pharm.D. Speakers Lori Chen, Pharm.D. Hanna Phan, Pharm.D., BCPS Lindsey D. Pinckney, Pharm.D. Danielle Tice, Pharm.D. Description This caregiver's session will focus on elements of CF care management in which clinical controversies may exist. Discussions will include antifungal management of ABPA, use of non-commercially available nebulized antimicrobials, issues with anticoagulation agents in CF & challenges & optimization efforts of medication adherence. Objectives 1. Assess antifungal therapies for treatment of ABPA. 2. Review use of IV antimicrobial agents used for nebulization. 3. Evaluate treatment modalities for anticoagulation in a CF patient. CG14 PT & RT: Updates on Airway Clearance (Combination) 229 AB Moderators Jennifer L. Agnew, B.Sc., P.T., B.H.K. Ed M. Conway, B.B.A., R.R.T., FAARC Speakers Ginger Browning, B.S., R.R.T. Brenda M. Button, Ph.D. Ruth A. McCartney, MBChB Lisa Morrison, B.Sc., MCSP Jeanette Seal, A.A.S. Description This session will provide evidenced based decision making when choosing the correct airway clearance device for your patient. The session explores the use of PEP & oscillating PEP & the benefits they provide patients. The session will explore the use of self-management for airway clearance with patients admitted to the hospital. Objectives 1. Identify the right airway clearance device for your patient utilizing the evidence. 2. Create a self-management plan for patients admitted to the hospital. 3. Discuss the benefits of using PEP & oscillating PEP from the evidence perspective. 67 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Caregivers Sessions by Discipline CG15 CLIN: RC: Updates (Combination) 222 ABC Moderators Patricia A. Burks, R.N., M.A., CCRC Heather Y. Hathorne, Ph.D., M.A.E., R.R.T., CCRC Amy Hoffman, M.P.H. Speakers Patricia A. Burks, R.N., M.A., CCRC Trenton Gordon, B.A. Heather Y. Hathorne, Ph.D., M.A.E., R.R.T., CCRC Amy Hoffman, M.P.H. Kelsie Pearson, B.A. Christina M. Roman, M.P.H. Description This session will include updates for the research coordinators from the Research Coordinator Representatives & Advisory Committee for NACFC, the Cystic Fibrosis Foundation & the TDN. These updates are useful in assisting the research coordinators to coordinate caring for research subjects & organizing research at their center with the most up-to-date information. Objectives 1. Identify new information for research coordinators from the Research Coordinator Representatives & Advisory Committee. 2. Identify new information for research coordinators from the Cystic Fibrosis Foundation. 3. Identify new information for research coordinators from the Therapeutics Development Network. CG16 CLIN: RC: Special Topics (Research) Moderator Heather Y. Hathorne, Ph.D., M.A.E., R.R.T., CCRC Speaker Heather Y. Hathorne, Ph.D., M.A.E., R.R.T., CCRC Description This is a networking session for research coordinators to share & learn best practices. Objectives 1. Express new ideas for research coordinators. 2. Compare best practices for research coordinators. 3. Create new strategies for research coordinators. 68 | 2015 North American CF Conference 222 ABC FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Caregivers Sessions by Discipline CG17 SW/PSYCH: Developing & Strengthening Community Relationships (Care) 227 ABC Moderators Anne M. Daggett, M.S.W., LCSW Chelsea E. Toth, M.S.W. Speakers Carol S. Chace, M.S.W., LICSW Carla A. Hart, B.S., M.S.Ed., M.P.H., M.Ed., Ed.D. Tina M. LaTurner, R.N., B.S.N., C.P.N. Kat Quinn, B.S.W., M.S. Description Cystic fibrosis affects many aspects of life: school, work, interpersonal relationships, other social environments, as well as access to medical insurance. Participants will further their understanding of developing & strengthening community relationships. This session will highlight the benefits of extending CF care outside of the medical setting. Objectives 1. Describe the benefits & challenges in the development of community programs & relationships. 2. Identify the processes that have been utilized in the development of specific community relationships by various individuals in the CF community. 3. Discuss barriers & challenges to creating & maintaining community relationships, as they pertain to pediatric & adult CF environments. 69 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Pediatric Fellows Session (Care) 121 ABC Chairs Marie E. Egan, M.D. Felix Ratjen, M.D., Ph.D., FRCPC Moderator Traci M. Kazmerski, M.D. Presenters The Answer Is Under the House-A Case of Hypersensitivity Pneumonitis in a Child With CF Christina Papantonakis, M.D. CF Liver Disease Treated With Combined Liver-Pancreas Transplant Daniela Castano, M.D. A Rare Case of Viral Induced Pericardial Effusion in a Young Male With CF Angela O. Delecaris, M.D. Lightening Can Strike Twice: Autoimmunity in CF Meghan E. McGarry, M.D., M.S. The Role of TPAIT in the Setting of Chronic Pancreatitis Due to CFTR Mutations Elissa M. Downs, M.D., M.P.H. Development of a Pleural Effusion After a Lobar Pneumonia in a Teenage Patient With CF Elizabeth L. Kramer, M.D., Ph.D. DRESS Syndrome in Cystic Fibrosis Benjamin D. Goldstein, M.D. Description This session is a series of selected 10 minute summaries of a patient case, clinical course & resolution followed by a five minute interactive, moderated discussion of the lessons learned for pediatric CF care & providers. Objectives 1. Identify the key concepts and discuss teaching points for each case presentation. 2. Review these concepts and key points and evaluate their importance to your CF Center and your clinical practice. 3. Translate these concepts and new knowledge into action items to improve clinical practice and patient outcomes. 70 | 2015 North American CF Conference FINAL PROGRAM FRIDAY, OCTOBER 9 4:00 p.m. - 6:00 p.m. Adult Fellows Session (Care) 122 ABC Chairs Joseph M. Pilewski, M.D. James R. Yankaskas, M.D. Moderator Michael J. Noto, M.D., Ph.D. Presenters Monozygotic Twins: Not So Identical After All! Michael Waller, MBBS, MRCP Turn Your Head & Cough: An Unusual Case of Cough in an Adult With CF Jacqueline Kruser, M.D. Portopulmonary Hypertension Due to CF Liver Disease: Long Lasting Efficacy of Tadalafil as a Single Oral Agent Rohit Gupta, MBBS Chylothorax in a CF Patient Nikunj A. Bhatt, M.D., M.S. Lung Transplantation in a Patient With Nontuberculosis Mycobacterium Infection Brian Shigenobu Furukawa, M.D. Invasive Colorectal Cancer Discovered After Bilateral Single Lung Transplantation for Severe CF Pulmonary Disease David Niccum, M.D. Decompensation of Lung Function in Three Patients With CF & G551D Mutation Following Withdrawal of Ivacaftor Aaron Trimble, M.D. Description This session is a series of selected 10 minute summaries of a patient case, clinical course & resolution followed by a five minute interactive, moderated discussion of the lessons learned for adult CF care & providers. Objectives 1. Discuss the key features & lessons learned for each case presentation. 2. Assess the relevance of those features to your practice, to your adult CF patients & to your program. 3. Apply these lessons & the knowledge gained to your Adult CF Program & to your care team’s & patients’ educational goals. 6:00 p.m. - 7:30 p.m. Speakers & Fellows Reception (By invitation only) Supported in part by AbbVie 71 | 2015 North American CF Conference Hall C TAB SHEET 5 SATURDAY FINAL PROGRAM SATURDAY, OCTOBER 10 North 300 Level Lobby 7:00 a.m. - 3:00 p.m. General Registration (PCC) 7:30 a.m. - 8:45 a.m. Poster Session III (Odd Numbers) Exhibit Hall E 7:30 a.m. - 8:45 a.m. Continental Breakfast Exhibit Hall E 7:30 a.m. - 2:20 p.m. Exhibit Hall Open Exhibit Hall E 7:30 a.m. - 8:45 a.m. Consultation Clinics LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. CC01 NRS: When Communications Go Bad: Sharing Difficult News/Dealing With Conflict (Care) 101 BC Moderators Natalia Argel, B.S.N, R.N.-B.C. Antoinette Gardner, B.S.N., M.Ed., A.E.-C., CCRC Case Titles Complex Challenges of a Missed Diagnosis in an Infant With Cystic Fibrosis & Speakers Kara G. Young, M.S., C.G.C. Future Planning & Motivation for a Patient Experiencing Addiction, Abuse & Non-Adherence Lindsey Currie, R.N. Leukemia Melenie A. Meyers, R.N. Transition Derailment: Good Intentions Gone Bad Cathy Mims, R.N., B.S.N., C.P.N. Description Delivering & receiving difficult news is never easy. This session will explore a variety of situations where difficult news was delivered. Communication barriers, including perception, will be explored. This will provide providers, families & peers an open discussion of where communication was "lost" & what techniques could have been used to improve the communication outcome. Objectives 1. Discuss alternative ways to engage & support patients/families & amongst team members when delivering bad news. 2. Identify the importance of collaboration with families, interdisciplinary teams & inpatient care teams. 3. Identify the importance of communication & goal setting with patients & families. 73 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 7:30 a.m. - 8:45 a.m. Consultation Clinics LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. CC02 SW/PSYCH: Chronic Sorrow & Spirituality (Care) 106 AB Moderators Nanette M. Concotelli-Fisk, LISW Daniel Grossoehme, D.Min. Case Titles Sadness: It's Part of the Journey With CF & Speakers Blaithin O'Dea, D.Clin.Psy. Against All Odds: Finding the Strength to Persevere in Raising Three Children With CF Kimberly Morse, LCSW The Influence of Chronic Sorrow in a Woman's Decision About Lung Transplant Kenya Wilder, LCSW Honoring D's Story of Illness & Transplant Journey: A CF Patient Care Matter of Spiritual Faith That Is In Synergy & Synchronicity With Resilience Jean L. Vinette, M.S.W., R.S.W. Description This session will offer group consultation on four cases of providing care for persons with CF which include a spiritual dimension. Objectives 1. Illustrate nature of chronic sorrow in providing psychosocial-spiritual care for persons dealing with CF. 2. Illustrate nature of spirituality in providing psychosocial-spiritual care for persons dealing with CF. 3. Apply group discussion information to clinical care of persons dealing with CF. CC03 NTR: GI/Nutrition (Care) 105 ABC Moderators Monika Kowalczyk, M.D. Julie L. Matel, M.S., R.D., C.D.E. Case Titles From Birth to a Year: A Nutritional Journey & Speakers Suzie Nolan, M.Sc. Achieving Nutritional Goals in the Face of Addiction & Psychotropic Therapy Suzanne Hollander, M.S., R.D., C.S.P. Nutrition Management of the Obese Pancreatic Insufficient Adult Patient With CF Michele Nicolo, R.D., C.D.E. CF Gut? Trials of the Probiotic Symprove & the Low FODMAP Diet Veronica Lynch, B.Sc. Hons Description This session will present a variety of complex gastrointestinal & nutrition cases, which will facilitate discussion about the challenges of managing the GI manifestations of CF. Objectives 1. Discuss GI complications in pancreatic insufficient infants with post-surgical gut. 2. Define the challenges of managing gastrointestinal symptoms of CF & comorbid psychiatric illness & obesity. 3. Examine the role of irritable bowel syndrome, probiotics & FODMOP diet in gastrointestinal symptoms of CF. 74 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 7:30 a.m. - 8:45 a.m. Consultation Clinics LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. CC04 PT & RT: Adherence Methods That Work for the PT & RT (Care) 102 AB Moderators Kristin A. Riekert, Ph.D. Karen M. von Berg, P.T., D.P.T. Case Titles A Complex Family With Multiple Barriers Have Three Children With CF & Speakers Crystal Garman, M.S.W., LCSW My OT Is Helping Me Learn to Take Care of Myself . . . Something I've Never Understood Jamie Wilcox, O.T.D., OTR/L The Oscillatory CoughAssist E70 Improved Adherence With Airway Clearance During Twin Pregnancy Lisa Morrison, B.Sc.(Hons), GradDipPhys Description Therapies only work if people do them; however we know that adherence to airway clearance, medications & exercise are poor among people with CF. Through the use of case presentations, this course will review strategies that PTs & RTs have successfully used to support their patients' adherence, as well as identify challenging cases & opportunities to improve patient-centered care. Objectives 1. Demonstrate enhanced communication skills with patients to discuss treatment adherence. 2. Identify & conceptualize barriers to treatment adherence. 3. Apply a personalized & patient-centered strategy to promote improved adherence. 9:00 a.m. - 10:00 a.m. Plenary Session III ALL: There Is No Health Without Mental Health Alexandra Quittner Ph.D. J. Stuart Elborn M.D., FRCP Beth Smith M.D. Halls AB Description This session focuses on the prevalence of depression & anxiety in both individuals with CF & caregivers. The session focuses on the published results from the TIDES study, the International Guidelines on Mental Health in CF & on implementation & dissemination of these guidelines. Objectives 1. Summarize the prevalence of depressive & anxious symptoms among individuals with CF & parent caregivers. 2. Identify how psychological symptoms negatively affect disease management, including adherence, clinic attendance, health outcomes & quality of life. 3. Identify evidence-based intervention strategies that address symptoms of depression & anxiety. 75 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W21 CFTR: Understanding & Fixing Mutant CFTR (Research) 132 ABC Chairs Michael A. Gray, Ph.D. Agnieszka Swiatecka-Urban, M.D., FASN 10:30 a.m. Introduction Agnieszka Swiatecka-Urban, M.D., FASN 10:40 a.m. Abstract 13 Increasing the Pool of CFTR Available for Correction: Discovery of New CF Experimental Therapeutics That Inhibit Ubiquitin Ligase Wook Joon Chung, Ph.D. 10:53 a.m. Abstract 14 Targeting the Ribosome as a Novel Approach to Correcting CFTR Class II Trafficking Defects Kathryn E. Oliver, M.S., Ph.D. Candidate 11:06 a.m. Abstract 15 Potentiators of Defective ΔF508-CFTR Channel Gating That Do Not Interfere With Corrector Action Puay-Wah Phuan, Ph.D. 11:19 a.m. Abstract 16 Sustained Increases in Cytosolic Calcium Induce CFTR Internalization Michael A. Gray, Ph.D. 11:32 a.m. Abstract 17 The Pseudomonas Aeruginosa Toxin Pyocyanin Decreases Expression of CFTR & Impairs Rescue of ΔF508-CFTR Via Activation of the MEK/ERK MAPK Pathway Estelle Cormet-Boyaka, Ph.D. 11:45 a.m. Abstract 18 Studying Rare Mutations & Putative CFTR Rescue by VX770 & VX809 Using FIS Assay in CF Colon Organoids Anabela Ramalho, Ph.D. 11:58 a.m. Abstract 19 Personalized Medicine: Elucidating Mechanistic Basis Toward Potential Treatment for a Novel CF-Mutation Kavisha Arora, Ph.D. Description This workshop is designed to review novel mechanisms regulating the cell surface density of CFTR channels, describe therapeutic advances to correct the mutant CFTR function & discuss personalized approach for CF treatment. Objectives 1. Review novel mechanisms regulating the cell surface density of CFTR channels. 2. Describe therapeutic advances to correct the mutant CFTR function. 3. Discuss personalized approach for CF treatment. 76 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W22 APP&D: CF Airway Inflammation, Biomarkers & Therapeutic Targets (Research) 126 ABC Supported by an educational grant from Gilead Sciences, Inc. Chairs Gerry N. McElvaney, M.B., FRCPI, FRCPC Jennifer L. Taylor-Cousar, M.D. 10:30 a.m. Introduction Jennifer L. Taylor-Cousar, M.D. 10:40 a.m. Abstract 97 Improving Host Defense Defects by Inhibiting ASL Acidification in Cystic Fibrosis Viral Shah, B.S. 10:55 a.m. Abstract 98 Calprotectin Expression Is Constitutively Altered in the CF Pig at Birth Prior to the Onset of Neutrophil Mediated Inflammation Robert Gray, MBChB, Ph.D. 11:10 a.m. Abstract 99 Analyzing the Immune Repertoire in CF & Non-CF Lungs William Harris, M.D. 11:25 a.m. Abstract 100 Ivacaftor Decreases Sputum Pseudomonas Density & Inflammation in CF Patients With G551D Mutations Katherine Hisert, M.D., Ph.D. 11:40 a.m. Abstract 101 The Basophil Surface Marker CD203C Identifies Aspergillus Fumigatus Sensitisation in Cystic Fibrosis Gillian Lavelle, B.Sc. 11:55 a.m. Abstract 103 Identification of Small Metabolites Associated With Metastable & Acute Cystic Fibrosis Airway Disease by High-resolution Metabolomics Rabindra Tirouvanziam, Ph.D. Description In this session, we will discuss the role of various pathobiologic contributors to inflammation in the CF airway, identify potential biomarkers for assessing this inflammation & review the role of therapeutic agents in combating inflammation in CF. Objectives 1. Identify various pathobiologic contributors to airway inflammation in CF. 2. Analyze appropriate biomarkers of inflammation in CF. 3. Evaluate the anti-inflammatory efficacy of a CFTR modulator. 77 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W23 APP&D: Non-CFTR Ion Transport Targets for CF (Research) 124 AB Chairs Hugo R. de Jonge, Ph.D. Nicholas W. Keiser, Ph.D. 10:30 a.m. Introduction Nicholas W. Keiser, Ph.D. 10:40 a.m. Abstract 104 Short palate lung & nasal epithelial clone 1 (SPLUNC1)dissociates & internalizes the epithelial sodium channel (ENAC) Christine Kim 10:55 a.m. Abstract 105 A Novel Inhibitor Of Channel Activating Proteases: Putting The Cap On ENAC James Reihill, Ph.D., B.Sc. 11:10 a.m. Abstract 106 Limited Pulmonary Exposure to TGF-BETA Inhibits CFTR & ANO-1 (TMEM16A) Expression & Enhances Mucus Production in CFTR+ & CFTR-/- Mice John Paul Clancy, M.D. 11:25 a.m. Abstract 107 Co-expression of SLC26A9 Isoforms Leads to Chloride-bicarbonate Exchange Carol A. Bertrand, Ph.D. 11:40 a.m. Abstract 108 SLC6A14 Modifies the Composition of Airway Surface Fluid in Non-CF & CF Primary Airway Cultures Michelle Di Paola 11:55 a.m. Abstract 109 Human Native Small Airways Secrete Bicarbonate Through CAMP- & CA++Mediated Pathway A.K.M. Shamsuddin, Ph.D. Description CF airways are dehydrated due to lack of chloride & bicarbonate secretion into the lumen; modulation of CFTR activity can alter ion concentration on the airway surface. In addition to CFTR, there are other potential channel targets for modulation of ion secretion by the epithelium. This session will cover several of these other ion channels & how their activity may be modulated. Objectives 1. Explain why non-CFTR ion channels represent a potential target for CF therapy. 2. Describe how inhibition of ENaC can be achieved at the molecular level. 3. Compare the molecular composition & functions of different non-CFTR epithelial anion channels. 78 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W24 SPECIAL FORMAT: NT: New Therapies Poster Discussion Session/ Workshop (Combination) 301 BC Chairs Amit Gaggar, M.D. Pamela Zeitlin, M.D., Ph.D. Abstracts MIR-9 & ANO1: Therapeutics Targets In Cystic Fibrosis? Florence Sonneville 203 Abstracts Utilisation Of A Novel Proteasetag™ Activity Immunoassay for Measurement of Neutrophil Elastase In BAL From Children With Cystic Fibrosis 204 Kelly Moffitt, Ph.D. Abstracts The Impact Of CFTR Modulation With Ivacaftor On Circulating Inflammatory Mediators In Patients With The G551D Mutation 205 Nicola Ronan Abstracts Phase II Study Of Digitoxin For CF Pamela Zeitlin, M.D., Ph.D. 207 Abstracts Urinary Biomarkers In Patients With Cystic Fibrosis Correlate With FEV1 & Lung Microbiome Diversity 208 Emilie Vallieres, M.D. Abstracts MBW & MRI Differentiate Disease Severity In Stable CF Lung Disease & Detect Response to Therapy in Children & Adolescents 209 Mirjam Stahl Abstracts Personalized Treatment For CF-Patients With Extremely Rare Mutations Based On The Intestinal Organoid Model 210 Gitte Berkers, M.D. Abstracts Safety & Efficacy of Treatment With Lumacaftor in Combination With Ivacaftor In Patients With CF Homozygous For F508del-CFTR 211 Michael Konstan, M.D. Abstracts Correlation Of Sweat Chloride & Percent Predicted FEV1 In Cystic Fibrosis Patients Treated With Ivacaftor 213 Fred Van Goor, Ph.D. Abstracts Qr-010 Treatment For Cystic Fibrosis: Assessing The Airway-Mucus Barrier In Delivery 214 Vera Brinks Abstracts Correlation Between Sweat Chloride Concentration & Lung Function In CF Patients Patrick Sosnay, M.D. 215 Abstracts End-Expiratory Lung Volumes On CT Are Not Elevated in The Presence Of "Gas Trapping" in Young Children With CF 216 Tim Rosenow, B.Sc. Abstracts Safety & Disposition Of The Novel ENAC Blocker P-1037 In CF Patients, Compared to Healthy Subjects 217 Karl Donn, Ph.D. 79 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III Abstracts A Phase 2, 16-Week, Randomized, Double-Blind, Placebo-Controlled Study to Assess Kb001-A 218 James Chmiel, M.D., M.P.H. Abstracts Novel Potentiators Augment Efficacy of Translational Readthrough In CFTR Nonsense Mutations 219 Venkateshwar Mutyam Abstracts Improved Rate Of Decline In FEV1 Is Not Associated With Acute Improvement In FEV1 In Patients With CF Treated With Ivacaftor 221 Edward McKone, M.D. Abstracts Corrective Effects On Airway Epithelial Function By Human Amniotic Epithelial Cells (HAEC) When Treating Cystic Fibrosis 222 Luke Garratt, B.Sc. Abstracts Biomarkers of Early Lung Disease In Infants With Cystic Fibrosis Theresa Laguna 223 Abstracts Consistency Of Multiple Readers For Nasal Potential Difference (NPD) Parameters 224 George Solomon, M.D. Abstracts Small-Molecule Pendrin Inhibitors as a Potential Therapeutic Approach For Cystic Fibrosis 225 Peter Haggie, Ph.D. Abstracts Ivacaftor Decreases Mortality In G551D Patients With Severe Lung Disease Peter Barry, M.D. 226 Abstracts Proteomics Platform Reveals Novel Biomarkers Of Bronchiectasis In Cystic Fibrosis Emily Deboer 227 Abstracts Linking Metabolomics & Transcriptomics to Identify Biomarkers Of P. Aeruginosa Mucoidy 228 Heather Bean, Ph.D. Abstracts In Vivo Human Nasal Lavage Metabolomics Suggest Biomarkers Of Lung Disease Severity In Cystic Fibrosis 229 Deepika Polineni, M.D., M.P.H. Description This session will address new disease-related outcomes & novel therapeutic approaches in the treatment of patients with CF. Objectives 1. Describe new disease-related outcome measures & potential application in patients with CF. 2. Assess compounds currently in early phase studies as new potential therapeutics for CF. 3. Evaluate potential opportunities & barriers to the advent of new outcome measures & therapeutics in the care of CF patients. 80 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W25 INF/MIC: Pathogenesis of Infection (Research) Chairs 129 AB Helle Krogh Johansen, M.D., D.M.S. Colin Manoil, Ph.D. Introduction Colin Manoil, Ph.D. 10:30 a.m. 10:40 a.m. Abstract 307 Regional Variation of Microbiota, Inflammation & Parenchymal Lung Damage in Subjects With Cystic Fibrosis Alix Ashare, M.D., Ph.D. 10:55 a.m. Abstract 308 Metabolomics of Sputum Supports a Polymicrobial & Ceramide-mediated Hyperinflammation Model of CF Disease Robert A. Quinn, Ph.D. 11:10 a.m. Abstract 309 What Makes Pseudomonas aeruginosa Persist in the Lungs of CF Patients? Helle Krogh Johansen, M.D., D.M.S. 11:25 a.m. Abstract 310 Host & Microbial Polymers Passively Aggregate CF Pathogens Producing Resistance to Killing & Promoting Interspecies Interactions Patrick R. Secor, Ph.D. 11:40 a.m. Abstract 311 Analysis of Pyocyanin-resistant Staphylococcus aureus Identifies Mechanisms of Pyocyanin Toxicity & Genetic Determinants of Pyocyanin Resistance Michael J. Noto, M.D., Ph.D. 11:55 a.m. Abstract 312 The Burkholderia cepacia complex Fix System Senses Oxygen & Mediates Virulence Matthew Schaefers Description This session will summarize fundamental advances in understanding how dominant species of bacteria infecting CF airways evolve, compete with each other & affect host tissues. Objectives 1. Demonstrate how dominant CF pathogens interact with each other & with the host & how it contributes to pathogenesis. 2. Describe how Pseudomonas aeruginosa persist & evolve in the airways of CF patients. 3. Illustrate bacterial interaction mechanisms & CF microbial diversity in CF airways. 81 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III CLIN: CF Pulmonary Disease: Diagnosis, Evaluation & Treatment W26 (Combination) 122 ABC Supported by an educational grant from Gilead Sciences, Inc. Chairs Nauman Chaudary, M.D. Lori L. Vanscoy, M.D. Introduction Nauman Chaudary, M.D. Lori L. Vanscoy, M.D. 10:30 a.m. 10:40 a.m. Abstract 399 Frequency & Duration of Rhinovirus Infections in Children With Cystic Fibrosis & Healthy Controls: A Longitudinal Cohort Study J. S. Dijkema 10:55 a.m. Abstract 400 Hypoxia in Sleep Despite Supplemental Oxygen Anne May, M.D. 11:10 a.m. Abstract 401 Improved Lung Function in Preschool Children With CF Over the Last Decade Julie Duncan Abstract 402 Assessment of the Effectiveness of Home Monitoring Trial in CF to Identify & Treat Acute Pulmonary Exacerbation: The EICE Study Results Christopher H. Goss, M.D., M.S. 11:40 a.m. Abstract 403 Emerging Regional Structure-Function Relationship in CF Lungs in Adolescents Using CT, UTE MRI & Hyperpolarized Xenon MRI Robert Thomen, M.S. 11:55 a.m. Abstract 404 Long-term Tiotropium Therapy in CF: A Retrospective Cohort Study Carsten Schwarz, M.D. 11:25 a.m. Description This session will discuss controversies in CF monitoring & therapy. Objectives 1. Compare HRV infection in children with CF to healthy controls. 2. Identify PFT abnormalities in CF preschool children in the era of NBS. 3. Discuss effectiveness of home spirometry & symptom assessment in CF. 82 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W27 CLIN: Cystic Fibrosis-Related Diabetes (Combination) Chairs 301 A Scott M. Blackman, M.D., Ph.D. Maria S. Rayas, M.D. 10:30 a.m. Abstract 594 Altered Insulin & Calcium Signaling in Pancreatic Beta Cells Lacking Functional CFTR Channels Fiona Manderson Koivula 10:40 a.m. Abstract 595 A Transient Metabolic Recovery From Early Life Glucose Intolerance in Cystic Fibrosis Ferrets John F. Engelhardt, Ph.D. 10:55 a.m. Invited Speaker Abnormal Glucose Tolerance in Infants & Young Children with Cystic Fibrosis Katie Larson Ode, M.D. 11:10 a.m. Abstract 596 Glucose Dysregulation: The Prevalence of Hypoglycemia in Adults With Cystic Fibrosis During an Oral Glucose Tolerance Test Lisa Mannik, M.Sc., R.D. 11:25 a.m. Abstract 598 Insulin Secretion in Post-Pubertal Adolescents & Adults With Cystic Fibrosis & Normal Glucose Tolerance Saba Sheikh, M.D. 11:40 a.m. Abstract 598 Continuous Glucose Monitoring Reveals Glucose Abnormalities in CF Youth Despite Normal Oral Glucose Tolerance Testing Christine Chan, M.D. 11:55 a.m. Abstract 599 Comparison of Oral Glucose Tolerance Test With Modified Mixed Meal Tolerance Test in Patients With Cystic Fibrosis Kelly Sakellaris, M.S. Description This session will provide an overview of research submitted as abstracts in the field of CFRD. Presentations will address the role of CFTR in the pancreatic beta cells & describe early glucose derangements appreciated in CF animal models & young children with CF. The presentations also will explore alternative methods to screen for CFRD. Objectives 1. Discuss the role of CFTR in insulin secretion & calcium regulation. 2. Discuss early glucose abnormalities appreciated in CF-ferrets & young children with CF. 3. Identify drawbacks to the OGTT & discuss potential alternative methods to screen for CFRD. 83 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W28 Chairs NRS-SW/PSYCH: Managing the Complex Needs of the Adult CF Patient (Care) 121 ABC Martha S. Markovitz, M.S.W., LCSW Mary E. Nelsen, B.S.N., J.D. Introduction Martha S. Markovitz, M.S.W., LCSW 10:30 a.m. 10:40 a.m. Invited Speaker Advance Care Planning in the Adult CF Population Traci M. Kazmerski, M.D. 10:55 a.m. Abstract 616 Association of Opioids on Frequency & Duration of Hospitalizations for Acute Pulmonary Exacerbation Corinne Muirhead, Pharm.D. 11:10 a.m. Abstract 619 The Investigation of Quality of Life & Adherence in Patients With the G551D Mutation Receiving Ivacaftor Therapy Heather Y. Hathorne, Ph.D., M.A.E., R.R.T., CCRC 11:25 a.m. Abstract 617 FEV1 Preservation Through Social Security Benefits Beth Sufian, J.D. 11:40 a.m. Invited Speaker Innovative Strategies Which Impact Depression & Quality of Life: The Experiences of the Partners in Living Initiative Siri Vaeth, M.S.W. 11:55 a.m. Abstract 620 The Use of Home Telehealth as an Education & Communication Tool for Adults With Cystic Fibrosis Mary K. Lester, R.R.T. Description This session will present a diversity of approaches in managing complex needs of adults with CF. Topics include identification of current challenges in chronic pain management; impact of new therapies on quality of life; accessing resources to enable utilization of legal/social resources & provide interventions/approaches which empower adults with tools for improved care. Objectives 1. Describe challenges in adult care from a multidisciplinary perspective. 2. Increase awareness of complex issues in addressing care. 3. Propose interventions & strategies which promote health & well-being of adult patients. 84 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W29 Chairs SW/PSYCH: Inpatient Management of Behaviors: Adult & Pediatric (Care) 131 ABC Jamie L. Cary, B.A., CCLS Stephanie Spear Filigno, Ph.D. Introduction Stephanie Spear Filigno, Ph.D. 10:30 a.m. 10:40 a.m. Abstract 624 The W's of Inpatient Behavioral Consultation in Pediatric CF: Who, When, Why & (So) What Kristin Kullgren 10:55 a.m. Abstract 622 Process Evaluation of Including an Occupational Therapist Within a Multidisciplinary CF Care Team Joanne Park, O.T.D., OTR/L 11:10 a.m. Abstract 623 Promoting Healthy Lifestyles Through Improving Disease Self-management Jamie Wilcox, O.T.D., OTR/L 11:25 a.m. Invited Speaker The Development of an Inpatient Sleep Hygiene Guideline Nicole Orkoskey, M.A., CCLS 11:40 a.m. Abstract 624 Self-Administration of In-patient Medications: A Pilot Study in Children With Cystic Fibrosis Khola Khan, M.Pharm. 11:55 a.m. Abstract 625 An Incentive Program Improved Adherence to Inpatient Respiratory Treatments & Participation in Physician Rounds & Overall Wellbeing in CF Patients Cynthia Leyva Joanna Fredlund , M.S.W. Description This workshop will highlight services & programs led by various disciplines to target improvement in emotional & behavioral health for both pediatric & adult populations during inpatient admissions. Topics will include how these services aim to promote independence, treatment adherence & partnership in the inpatient environment to support generalization of skills to the home environment. Objectives 1. Identify the various disciplines that can provide emotional & behavioral support during pediatric & adult inpatient populations. 2. Discuss how goal setting & behavioral intervention support can increase patient engagement & partnership in their care during admissions. 3. Identify how inpatient behavioral support can translate to improved care in the home setting. 85 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 10:30 a.m. - 12:15 p.m. Workshop Session III W30 PT & RT: Update on Exercise (Combination) Chairs 127 ABC Frank J. Cerny, Ph.D., M.Div. Helge Hebestreit, M.D., Ph.D. Introduction Frank J. Cerny, Ph.D., M.Div. 10:30 a.m. 10:40 a.m. Abstract 463 Cardiopulmonary Exercise Testing: A Tool to Monitor Cystic Fibrosis Lung Disease Elpis Hatziagorou, M.D., Ph.D. 10:55 a.m. Abstract 465 A Single Maximal Exercise Test Improves Lung Function in Patients With Cystic Fibrosis Reva Crandall, R.R.T., CPFT 11:10 a.m. Abstract 466 Effects of a Partially Supervised Combined Exercise Program on Glycemic Control in Cystic Fibrosis: Pilot Study Nadia Beaudoin, R.R.T., B.Sc. 11:25 a.m. Abstract 467 The Physical Activity Questionnaire as a Tool to Target Exercise Intervention in Children & Adolescents With Cystic Fibrosis Nicola L. Collins, B.Sc.(Hons) 11:40 a.m. Abstract 468 Exercise Prescribing Patterns Among CF Providers: Are they FITT? Mana Rao, M.D. 11:55 a.m. Invited Speaker An Update on the Exercise Testing Consensus Helge Hebestreit, M.D., Ph.D. Description Regular activity is an important part of clinical care for CF patients. This session will present recent research regarding the relationship between exercise testing & disease status, the effects of exercise on pulmonary function & glycemic control & patterns of patient activity & exercise prescription. Current consensus of exercise testing & emerging research will be presented. Objectives 1. Describe the relationship between exercise test response & lung disease of CF & demonstrate standard testing practices. 2. Discuss the effects of exercise on lung function & glycemic control in patients. 3. Evaluate activity patterns of patients & prescription patterns of clinicians. 86 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons LIMITED SESSION. Pre-registration is required. ADMISSION BY TICKET ONLY. PLEASE NOTE: Luncheon Roundtables are held concurrently with Brown Bag Luncheons in the 12:30 p.m. time slot. Please refer to the Roundtable listing starting on page 89. BB06 GMS: Genome Editing (Research) 102 C Moderators Mitchell L. Drumm, Ph.D. Patrick Harrison, Ph.D. Speakers Mitchell L. Drumm, Ph.D. Patrick Harrison, Ph.D. Description This session will be structured into three "courses" of 25 minutes each. Each course has two 4-5 minute short talks, each followed by 7-8 minutes of discussion. The session will focus on an overview & background of genome editing, as well as basic & advanced techniques & applications to CF. Objectives 1. Explain & discuss what gene editing is, how it is performed & how it can be used both as a research tool & potential therapeutic agent. 2. Express the ease with which gene editing can now be performed by any researchers with basic molecular biology skills. 3. Discuss the advantages & disadvantages, limitations & challenges of the technique. BB07 INF/MIC: Integrating the NTM Guidelines Into Clinical Care (Care) 106 AB Moderator Lisa Saiman, M.D., M.P.H. Speakers Andres Floto, M.D., Ph.D., FRCP Charles S. Haworth, M.D., FRCP Jerry A. Nick, M.D. Kenneth N. Olivier, M.D., M.P.H. Description In this interactive session which will include audience response options, case vignettes of CF patients with positive cultures for non-tuberculous mycobacteria will be presented. Both routine management & issues that involve consulting an expert will be discussed by the panel & audience. Objectives 1. Discuss management strategies for people with CF with positive cultures for M. abscessus complex. 2. Describe different treatment options for people with CF with positive cultures for M. abscessus complex. 3. Identify utility of susceptibility testing & therapeutic drug monitoring & learn strategies for monitoring & managing drug-related toxicities. 87 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons LIMITED SESSION. Pre-registration is required. ADMISSION BY TICKET ONLY. PLEASE NOTE: Luncheon Roundtables are held concurrently with Brown Bag Luncheons in the 12:30 p.m. time slot. Please refer to the Roundtable listing starting on page 89. BB08 SW/PSYCH: Managing the Maze of Medication & Access Programs (Care) 101 BC Moderators Elinor Langfelder-Schwind, M.S. Amy Plachta, LMSW Speakers Elinor Langfelder-Schwind, M.S. Amy Plachta, LMSW Beth Sufian, J.D. Description Access to proper care & medications specific to CF can be a challenge. Medications are expensive & insurers are becoming resistant to pay for specialty drugs. Patients & providers in CF centers require help to navigate the maze. This session will focus on the qualifications of different medication access programs. Resources & services for genetic counseling & testing will also be discussed. Objectives 1. Describe the various access programs & the various methods available to centers to advocate for unmet patient needs. 2. Discuss how to assess each individual patient to find out which programs they might appropriate. 3. Review how a patient can utilize genetic counseling services. BB09 SW/PSYCH: Psychosocial Needs for the Newly Diagnosed Patient: Pediatric & Adult (Care) 102 AB Moderators Cindy D. Greene, L.S.W. Abbigail L. Moog, B.S.W., M.S.W. Speakers Mary Jo Chambers, M.S.W., LCSW Sheila J. Durand, C.S.W., R.D.H. Martha S. Markovitz, M.S.W., LCSW Erin Millard, Psy.D. Bill Taub, M.S.W., LCSW Description In this session we will look at the psychosocial needs of the newly diagnosed CF patient for both the pediatric & adult population. Objectives 1. Discuss the psychosocial needs for newly diagnosed pediatric & adult patients. 2. Identify interventions used to assist patients & families coping with a new CF diagnosis. 3. Summarize resources available to the newly diagnosed CF patient. 88 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 12:30 p.m. - 1:50 p.m. Brown Bag Luncheons LIMITED SESSION. Pre-registration is required. ADMISSION BY TICKET ONLY. PLEASE NOTE: Luncheon Roundtables are held concurrently with Brown Bag Luncheons in the 12:30 p.m. time slot. Please refer to the Roundtable listing starting on page 89. BB10 NTR: Adult Nutrition: Emerging Issues (Care) 105 ABC Moderators Gretchen M. Garlow, M.S., R.D., L.D.N., CNSC Lisa Mannik, M.Sc., R.D. Speakers Deborah Friedman, Ph.D. Gretchen M. Garlow, M.S., R.D., L.D.N., CNSC Lisa Mannik, M.Sc., R.D. Description As individuals with CF age well into adulthood, unique nutrition issues have emerged including obesity, dyslipidemia & diabetes. Management of these issues may be overwhelming for adults with CF, especially while managing family & work responsibilities. This session will explore the unique nutritional concerns of the aging CF cohort & review psychosocial considerations to aid adherence. Objectives 1. List the emerging nutrition-related complications associated with the aging CF cohort. 2. Describe novel approaches to the management of nutrition-related issues for adults with CF. 3. Discuss psychosocial issues that may present as barriers to managing the nutritional care of adults with CF. Luncheon Roundtables 12:30 p.m. - 1:50 p.m.. LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. APP&D 1 APP&D: Advances in MRI Chest Imaging Scott Nagle, M.D., Ph.D. CFTR 2 CFTR: Optimizing Mutant CFTR Function in CF Patients TBD CFTR 3 CFTR: New Understanding of Structure/Function Relationships in CFTR Patrick H. Thibodeau, Ph.D. CFTR 4 CFTR: Understanding the Mechanisms of Action of CFTR Modulators Christine E. Bear, Ph.D. CFTR 5 CFTR: CFTR & It's Interactome William E. Balch, Ph.D. CFTR 6 CFTR: Novel CFTR Gene Therapy Strategies Assem G. Ziady, Ph.D. CFTR 7 CFTR: Novel Platforms to Identify & Test CFTR Active Drugs Martin Mense, Ph.D. 89 | 2015 North American CF Conference Sheraton Phoenix Ballroom FINAL PROGRAM SATURDAY, OCTOBER 10 Luncheon Roundtables 12:30 p.m. - 1:50 p.m.. LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. Sheraton Phoenix Ballroom CLIN 8 CLIN: Opioids in CF Rob Horowitz, M.D. CLIN 9 CLIN: Palliative Care Issues in CF Jessica L. Goggin, M.A.S., R.N. CLIN 10 CLIN: Statistics 101: Number-crunching for the Non-statistician Jenna Sykes CLIN 11 CLIN: Antibiotic Desensitization: How to & What to Watch Out For Daniel Cortes, B.Sc.Pharm. CLIN 12 CLIN: Newborn Screening Quality Improvement Susanna A. McColley, M.D. CLIN 15 CLIN: Infection Prevention & Control Implementation Ginny Drapeau, R.N., B.S.N., CCRP CLIN 16 CLIN: GI Issues: Colon Cancer Screening David Niccum, M.D. CLIN 17 CLIN: How to Conduct Data Mining of Registry Data Theodore G. Liou, M.D. CLIN 18 CLIN: Pediatric to Adult Transition & Creating Tools for Transition Alyssa A. Falos, LCSW CLIN 19 CLIN: Renal Disease 101 Amy Sussman, M.D. CLIN 20 CLIN: Designing Surveys Karen Homa, Ph.D. CLIN 21 CLIN: Management of Malabsorption When Pancreatic Enzyme Dosage Is Optimized Steven D. Freedman, M.D., Ph.D. CLIN 22 CLIN: DIOS & Constipation Management Michael Wilschanski, MBBS CLIN 23 CLIN: Management of Impaired Glucose Tolerance Katie Larson Ode, M.D. CLIN 24 CLIN: Upper GI Tract Disease Management: GERD David N. Assis, M.D. CLIN 25 CLIN: Lung Clearance Index Jessica E. Pittman, M.D., M.P.H. INF/MIC 26 INF/MIC: Toward Standardized Treatment of Exacerbations Don B. Sanders, M.D., M.S. INF/MIC 27 INF/MIC: Treatment of Non-tuberculous Mycobacteria Stacey L. Martiniano, M.D. 90 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 Luncheon Roundtables 12:30 p.m. - 1:50 p.m.. LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. Sheraton Phoenix Ballroom INF/MIC 28 INF/MIC: Evolution of Pseudomonas During Infection Helle Krogh Johansen, M.D., D.M.S. INF/MIC 29 INF/MIC: Activity of Airway Bacterial Communities Robert A. Quinn, Ph.D. INF/MIC 30 INF/MIC: CF Gut Microbiome TBD NT 31 NT: Evaluating & Theratyping Rare CFTR Mutant Alleles David N. Sheppard, Ph.D. NT 32 NT: Strategies for Addressing CFTR Premature Truncation & Splicing Defects David M. Bedwell, Ph.D. NT 33 NT: Recent Advances in Gene Therapy Relevant to Treatment of CF Lung Disease TBD NT 34 NT: Gene Editing Technologies for CF Therapeutic Development & Clinical Intervention Brian R. Davis, Ph.D. NT 35 NT: Biomarkers to Evaluate CF Clinical Interventions Terri A. Laguna, M.D., MSCS NT 36 NT: Monitoring CF Lung Disease With MRI Harm A.W.M. Tiddens, M.D., Ph.D. NT 37 NT: LCI to Assess CF Lung Disease & Experimental Therapeutics Sanja Stanojevic, Ph.D. NRS 38 NRS: Developing a Pediatric Gastrostomy Protocol Jillian Brenton, R.D., L.D. NRS 39 NRS: THE Talk: Sex, Drugs & Alcohol Carolyn Heyman, R.N. NRS 40 NRS: CRMS Guidelines Revisited: Five to 10 Years Post Diagnosis Jeannine Cheatham, M.S., A.P.N., PCNS-BC NRS 41 NRS: CF Buddy: Partnering Students in Chronic Care Jane Young, R.N., A.S.N., A.E.-C. NRS 42 NRS: Transition Clinic Palma Schmidt, M.S.N., A.P.N.-B.C. NRS 43 NRS: Increasing Compliance of Oral Glucose Tolerance Testing in Your Clinic April D. Smith, B.S.N., R.N. NRS 44 NRS: Management Strategies for Pediatric CFRD Care Theresa Flint Rodgers, D.N.P., CRNP NRS 45 NRS: Sexual Health Education in CF Teens Rebecca Pettit, Pharm.D., M.B.A. 91 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 Luncheon Roundtables 12:30 p.m. - 1:50 p.m.. LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. Sheraton Phoenix Ballroom NRS 46 NRS: Managing CFRD: A Team Approach to Care Mary Sachs, APRN, C.N.P., M.S.N. NRS 47 NRS: Strategies for Transitioning Infants From Newborn CF Clinics Collette M. Davis, M.S.N., CPNP NRS 48 NRS: Development of a Nutrition App to Improve Nutrition in CF Judy A. Pitts, R.N., M.S., CPNP NRS 49 NRS: Factors Influencing Referral of Pediatric CF Patients for Lung Transplant Evaluation Erin Wells, R.N., B.S.N., C.P.N. NRS 50 NRS: CF Registry: Making Data Entry a Priority Kendra Darnell, R.N. NRS 51 NRS: Outpatient Exercise Program for CF Janine Cassidy, R.N., M.S.N., C.P.N. NTR 52 NTR: Ivacaftor & Nutrition Ronalee Robert, R.D., C.D.E. NTR 53 NTR: Nutrition Assessment (Beyond BMI) Brandi Pigg, M.S., R.D., L.D.N., C.L.C. NTR 54 NTR: Enzymes & Tube Feedings Kathy Santoro, M.Ed., R.D., L.D., C.S.P. NTR 55 NTR: CF Nutrition 101 Jean Tomezsko, Ph.D., R.D., L.D.N. NTR 56 NTR: Nutrition Charting Templates/Smartphrases Susan Gemma, M.S., R.D., L.D. NTR 57 NTR: Drug & Nutrient Interactions Sally Phillips, M.S., R.D./L.D., C.D.E. NTR 58 NTR: Infant Nutrition Susan C. Casey, R.D., C.D. NTR 59 NTR: Nutrition & Teen Pregnancy Vanessa Carr, M.S., R.D.N., L.D.N. NTR 60 NTR: Billing for Nutrition Visits Courtney V. Busby, R.D. NTR 61 NTR: Blenderized Tube Feedings Aimee Goyette, R.D. NTR 62 NTR: Managing Vitamin Levels Kathy Stansfield, R.D. PT & RT 63 PT & RT: Balancing Exercise & Airway Clearance in the Hospitalized Patient With CF Sarah Wright, P.T. 92 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 Luncheon Roundtables 12:30 p.m. - 1:50 p.m.. LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. Sheraton Phoenix Ballroom PT & RT 64 PT & RT: Motivational Interviewing to Improve Physical Activity John D. Lowman, Ph.D., P.T., C.C.S. PT & RT 65 PT & RT: Preparing Patients for Lung Transplant Anne Gould, P.T. PT & RT 66 PT & RT: PT in End-of-Life: When Not to Give Up Scott P. Russell, P.T., D.P.T., C.C.S., G.C.S. PT & RT 67 PT & RT: Exercise Testing in Patients With CFRD Ryan Harris, Ph.D., C.E.S., FACSM PT & RT 68 PT & RT: Airway Clearance in the Home Courtney Edman, P.T. PT & RT 69 PT & RT: Ideas for Managing Chronic Cough & Associated Fatigue: Impact on QOL Eena Kapoor, P.T., D.P.T. PT & RT 70 PT & RT: Getting PT Involved With CF Care Matthew Nippins, P.T., D.P.T., C.C.S. PT & RT 71 PT & RT: Action Plan for CF RT Inpatient Care Christine Presta, R.R.T., N.P.S. PT & RT 72 PT & RT: Infection Control in the CF Center: What's Working & Looking Toward the Future William "Bryan" Carbaugh, II, R.R.T. PT & RT 73 PT & RT: Making the Connection: Bridging the Gap Between Outpatient & Inpatient Care: A Pediatric Model Christiana P. Guthrie, R.R.T. PT & RT 74 PT & RT: Wearing Two Hats: Pediatric & Adult CF RT Wayne Barfield, R.R.T. PT & RT 75 PT & RT: Out of the Box: Non-traditional Aggressive Airway Clearance Michelle W. Tackett, R.R.T. PT & RT 76 PT & RT: New to CF RT: Mentoring as a Bridge to the Big Picture Dabney M. Eidson, B.S., R.R.T.-N.P.S. RC 77 RC: Dividing Up the Work Theresa Kump, B.A. RC 78 RC: Differences in Recruiting: Adult vs. Pediatric Challenges Dawn M. Kruse, CCRC RC 79 RC: Increasing Institution Visibility Rebekah Hibbard, B.S., CCRC RC 80 RC: Building Your Clinical Trial Budget Colleen E. Dunn, B.A., CCRC, R.R.T. 93 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 Luncheon Roundtables 12:30 p.m. - 1:50 p.m.. LIMITED SESSION: Pre-registration is required & tuition fee applies. ADMISSION BY TICKET ONLY. Sheraton Phoenix Ballroom SW/ PSYCH 81 SW/PSYCH: Pediatric CF SW 101 Dena Wentz, M.S.W., L.S.W. SW/ PSYCH 82 SW/PSYCH: Adult CF SW 101 Katie Hall, LMSW SW/ PSYCH 83 SW/PSYCH: Teaming Up With Child Life Jamie L. Cary, B.A., CCLS SW/ PSYCH 84 SW/PSYCH: Mindful Meditation in CF Monica Eisenhardt, M.S.W., LCSW SW/ PSYCH 85 SW/PSYCH: Networking & Supervision in CF Social Work Elissa Cohen Engel, M.S.W., L.S.W. SW/ PSYCH 86 SW/PSYCH: Permanency Planning for Adults With CF Yoni Stevens, M.S.W. SW/ PSYCH 87 SW/PSYCH: The Interactive Clinic Visit-Helping to Maximize Families Clinic Visit Winsome Sewell, LCSW SW/ PSYCH 88 SW/PSYCH: Cyber CF Nanette Concotelli-Fisk, LISW SW/ PSYCH 89 SW/PSYCH: The Impact of Social Isolation: How Can We Help? Chelsea E. Toth, M.S.W. PHARM 90 PHARM: Navigating the Specialty Pharmacy Waters Lynda Roe, C.Ph.T. PHARM 91 PHARM: Treatment of MRSA Sis Ueng, Pharm.D. PHARM 92 PHARM: Optimizing Pancreatic Enzyme Therapy Connie Takawira, Pharm.D. PHARM 93 PHARM: Maximizing Therapies for NTM Zubin Bhakta, Pharm.D. PHARM 94 PHARM: ABPA Treatment & Monitoring Kimberly Ehlert, Pharm.D. PHARM 95 PHARM: Practical Management of CFRD Colleen Sakon, Pharm.D. 94 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 12:30 p.m. - 2:20 p.m. U.S. Center & Program Directors Meeting* Sheraton Valley of the Sun C-E *Invitations were coordinated independently from the NACFC registration process. Contact [email protected] with questions. (For directors of all U.S. CF centers & CFF-accredited Adult & Affiliate Programs. One representative per program should attend.) ADMISSION BY TICKET ONLY. 2:30 p.m. - 4:20 p.m. Symposium Session III S15 APP&D: Anti-inflammatories for CF: Pathways to Therapeutics (Combination) 121 ABC Chairs Andre M. Cantin, M.D. James F. Chmiel, M.D., M.P.H. 2:30 p.m. Introduction James F. Chmiel, M.D., M.P.H. 2:40 p.m. S15.1 Promising Inflammatory Pathways to Target Janet Allen 3:05 p.m. S15.2 Animal Models for Pre-Clinical Exploration of Anti-inflammatory Therapeutics Tracey L. Bonfield, Ph.D., D(ABMLI) 3:30 p.m. S15.3 Lessons Learned From Anti-inflammatory Trials in CF Michael W. Konstan, M.D. 3:55 p.m. S15.4 Design of Anti-inflammatory Clinical Trials in CF Felix Ratjen, M.D., Ph.D., FRCPC Description Anti-inflammatory therapy is a mainstay of CF treatment. However, new & more effective anti-inflammatories are needed. In 2014 CFF sponsored a series of workshops to identify & address critical questions related to the development of new anti-inflammatory agents. In this symposium, the recommendations from the workshop will be reviewed & the impact they may have on future drug development. Objectives 1. Identify the most promising targets for anti-inflammatory therapy. 2. Discuss the importance & limitations of pre-clinical animal models in antiinflammatory drug development. 3. Explain the lessons learned from previous anti-inflammatory clinical trials. 95 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 2:30 p.m. - 4:20 p.m. Symposium Session III S16 GMS: How Do CF Genetics & Clinical Practice Interface? (Combination) 132 ABC Chairs Scott M. Blackman, M.D., Ph.D. Harriet Corvol, M.D., Ph.D. 2:30 p.m. Introduction Scott M. Blackman, M.D., Ph.D. Harriet Corvol, M.D., Ph.D. 2:40 p.m. S16.1 CFTR Genetic Diversity: Ongoing Efforts to Annotate CF-Causing Variants: Implications of CFTR2 on Therapy Patrick R. Sosnay, M.D. 3:05 p.m. S16.2 New Findings From the International CF Gene Modifier Consortium Michael R. Knowles, M.D. 3:30 p.m. S16.3 Incorporating Gene Mutation Data Into CF Newborn Screening & Pediatric CF Care Clement L. Ren, M.D. 3:55 p.m. S16.4 Putting It All Together: CFTR & Modifier Genetics in the Clinic Edward F. McKone, M.D. Description Speakers in this session will describe the characterization of genetic variants at the CFTR locus & discuss the impact of genetic variants in other genes (genetic modifiers) on CF. We will discuss ways in which CFTR & genetic modifier information can be incorporated into clinical management, at the time of CF diagnosis & through childhood & adulthood. Objectives 1. Describe how to best annotate whether a mutation in CFTR causes CF or is a neutral variant. 2. Relate how & which genes other than CFTR can affect CF & its complications. 3. Discuss what genetic information about CFTR & other genes can add to the clinical care for people with CF, at the time of CF diagnosis & throughout childhood & adulthood. S17 NT: Strategies for Rare CFTR Mutations (Research) 124 AB Chairs Ronald C. Rubenstein, M.D., Ph.D. Neeraj Sharma, D.V.M., Ph.D. 2:30 p.m. Introduction Neeraj Sharma, D.V.M., Ph.D. 2:40 p.m. S17.1 Merging Molecular Pathologies With Mechanisms of Drug Action Philip Thomas, Ph.D. 3:05 p.m. S17.2 Illuminating Rare CFTR Luminal Mutations Laurence C. Suaud, Ph.D. 3:30 p.m. S17.3 Therapies Targeting Rare CFTR Mutations Require Development of New Patient Specific Tools Christine E. Bear, Ph.D. 96 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 2:30 p.m. - 4:20 p.m. Symposium Session III 3:55 p.m. S17.4 Targeted Correction & Restored Function of the CFTR Gene in CF-induced Pluripotent Stem Cells Brian R. Davis, Ph.D. Description Among 80,000 CF patients worldwide, almost 2,000 CFTR variants have been reported. However, the molecular mechanisms that underlie the physiologic consequences for the majority of these rare variants are unknown. This session will detail our current understandings & approaches to restoring the function of these rare, non-F508del CFTR variants. Objectives 1. Describe how rare mutations cause CFTR dysfunction using genetic & cell biological approaches. 2. Describe the approach to developing novel, mechanism-based small molecule therapeutic options to treat patients with rare mutations in a personalized fashion. 3. Describe the uses of induced pluripotent stem cells & gene editing strategies in targeted correction & restoration of CFTR function. S18 INF/MIC: Microbial Interactions in the CF Airways (Research) 129 AB Supported by an educational grant from Gilead Sciences, Inc. Chairs Alix Ashare, M.D., Ph.D. Stephen Lory, Ph.D. 2:30 p.m. Introduction Alix Ashare, M.D., Ph.D. Stephen Lory, Ph.D. 2:40 p.m. S18.1 Inactivation of Quorum Sensing Redirects Bacterial-Host Interactions Dao Nguyen, M.D. 3:05 p.m. S18.2 Polymorphonuclear Leukocytes Regulate Growth of Pseudomonas Aeruginosa in the Lungs of CF Patients Thomas Bjarnsholt, Ph.D. 3:30 p.m. S18.3 Regional Isolation Drives Diversification within CF Lungs Peter Jorth, Ph.D. 3:55 p.m. S18.4 Viral-Bacterial Interactions in CF Jennifer M. Bomberger, Ph.D. Description This session will focus on describing the complex interactions between host immune cells & microbial pathogens in the lungs of patients with CF. Objectives 1. Summarize data regarding the complexity of microbial pathogens in the CF lung. 2. Discuss host factors that impact P. aeruginosa infection in CF. 3. Summarize recent findings on the role of respiratory viral infections on disease progression in CF. 97 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 2:30 p.m. - 4:20 p.m. Symposium Session III S19 CLIN: Update on Lung Transplantation in CF (Combination) 131 ABC Chairs Robert Aris, M.D. Denis Hadjiliadis, M.D., M.H.S., FRCP(C) 2:30 p.m. Introduction Robert Aris, M.D. 2:40 p.m. S19.1 Referral for Lung Transplantation: How Does the Lung Allocation Score (LAS) Work & What Are the Issues Getting on the List Denis Hadjiliadis, M.D., M.H.S., FRCP(C) 3:05 p.m. S19.2 Managing the Myriad of Extrapulmonary CF Complications After Lung Transplant: A Primer From the Perspective of a CF Center Jordan A. Dunitz, M.D. 3:30 p.m. S19.3 Infectious Considerations for Patients with CF Undergoing Lung Transplantation Leonard Jason Lobo, M.D. 3:55 p.m. S19.4 Breathing Easy for Eight Years Allison Hall Description This session will address lung transplant access for patients with Medicaid, a contrast between US & Canadian policies, common complications after transplant, issues of survival prediction in patients with end-stage CF & effect of infection on transplantation outcomes. A lung transplant recipient will also share perspectives about quality of life & what to expect with transplantation. Objectives 1. Describe current lung allocation in the United States & issues with access to the waiting list. 2. List common complications in CF patients after lung transplantation. 3. Discuss issues with difficult to manage infections in patients with CF undergoing lung transplantation. 98 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 2:30 p.m. - 4:20 p.m. Symposium Session III S20 CLIN: Caroline McPherson Symposium: Pro/Con Debates in Early CF Lung Disease (<6 Years of Age) (Care) 301 BC Chairs Cori L. Daines, M.D. Scott D. Sagel, M.D., Ph.D. 2:30 p.m. Introduction Scott D. Sagel, M.D., Ph.D. 2:32 p.m. S20.1 Lung Function Testing Should Be Routinely Performed to Assess & Monitor Early CF Lung Disease-Pro Stephanie D. Davis, M.D. 2:50 p.m. S20.2 Lung Function Testing Should Be Routinely Performed to Assess & Monitor Early CF Lung Disease-Con Margaret Rosenfeld, M.D., M.P.H. 3:08 p.m. S20.3 Surveillance Bronchoscopy Should Be Routinely Performed to Assess Early CF Lung Disease-Pro Stephen M. Stick, Ph.D., M.B., B.Chir., MRCP, FRACP 3:26 p.m. S20.4 Surveillance Bronchoscopy Should Not Be Routinely Performed to Assess Early CF Lung Disease-Con Claire E. Wainwright, M.D., MBBS 3:44 p.m. S20.5 Staphylococcus Aureus Should Be Treated Aggressively in Infants & Young Children With CF-Pro Alan R. Smyth, M.D., MBBS, MRCP, FRCPCH 4:02 p.m. S20.6 Staphylococcus Aureus Should Be Not Treated Aggressively in Infants & Young Children With CF-Con Lucas R. Hoffman, M.D., Ph.D. Description Monitoring & aggressive treatment of lung disease during early childhood are critical to improving outcomes in CF. Yet, clear & convincing evidence-based data to guide optimal clinical care in young children with CF are lacking. This symposium will utilize a pro-con style debate to address three controversial topics in early CF lung disease (<6 years of age). Objectives 1. Debate the evidence for & against the use of surveillance lung function testing to assess & monitor early CF lung disease. 2. Debate the risks & benefits of surveillance bronchoscopy to assess early CF lung disease. 3. Discuss the risks, benefits & evidence of early aggressive Staphylococcus aureus prevention & treatment in infants & young children with CF. 99 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 2:30 p.m. - 4:20 p.m. Symposium Session III S21 SW/PSYCH: The Impact of CF on Relationships Throughout the Lifespan (Combination) 122 ABC Chairs John P. Nash, LMSW Kristin A. Riekert, Ph.D. 2:30 p.m. Introduction John P. Nash, LMSW 2:40 p.m. S21.1 The Impact of CF on Sibling Relationships Trudy Havermans, Ph.D. 3:05 p.m. S21.2 Friendship Quality & Health-related Outcomes Among Adolescents with CF Sarah Helms, Ph.D. 3:30 p.m. S21.3 Impact of Disclosure to Colleagues, Friends & Romantic Partners on Older Adolescent & Adult Psychosocial Outcomes Adrienne Borschuk, M.S. 3:55 p.m. S21.4 Interdisciplinary Cystic Fibrosis Team Perspectives on the Correlation Between Sexuality/Intimacy & Adherence in Adolescent & Adult Relationships Elissa Cohen Engel, M.S.W., L.S.W. Description How CF affects the development & quality of non-parental relationships & how these relationships subsequently affect the well-being of people with CF is not often considered by healthcare providers. This session will provide data on the impact of relationships to illustrate their importance & identify ways to support these critical relationships. Objectives 1. Compare & contrast the manner in which a CF diagnosis can affect relationships, including familial, peer, collegial & romantic relationships. 2. Describe the influence of personal relationships on the physical & psychosocial well-being of people with CF. 3. Evaluate strategies the CF Care Team can use to support the attainment of health & rewarding relationships. 4:00 p.m. - 4:30 p.m. Coffee Break 100 | 2015 North American CF Conference 200 Level Corridors FINAL PROGRAM SATURDAY, OCTOBER 10 4:30 p.m. - 6:30 p.m. Caregivers Clinics CG18 NRS: Organizing Your Clinic (Care) 222 ABC Moderator Kathryn Kinyon Munch, D.N.P., CPNP, A.N.H.-B.C. Speaker Catherine S. Keezel, B.S.N., M.S.N., CPNP Description The major focus of this session will be to share resources used to run an efficient adult/ pediatric CF clinic through the use of quality improvement tools which will include: meeting skills, the 5 P's, development of a fish diagram & value stream mapping. Objectives 1. Apply meeting skills to be used in an effective quality improvement project. 2. Describe a quality improvement project that they would like to implement in their CF center. 3. Design a quality improvement project which they will present to their CF team. CG19 NTR: CFRD (1st Hour) & Nutrition, Exercise in CF (2nd Hour) (Care) 224 AB Moderators Kimberly Altman, M.S., R.D., C.S.P., C.D.N. Erin Seffrood, M.S., R.D., C.S.P., C.D. Speakers Marcin Madej, P.T., D.P.T. Elizabeth Mannon, R.D. Darci Pfeil, R.N., M.S., CPNP David Ruderman, PT, D.P.T. Beth Vandenlangenberg, N.P., C.D.E. Description The first half of this session will explore innovations in the management of IGT & CFRD. We will discuss the creation of a multidisciplinary clinic for patients with CF & glucose abnormalities, the use of insulin in IGT, & continuous glucose monitoring in IGT & CFRD. The second half of this session will focus on the role of exercise in CF, & how to optimize nutrition with exercise. Objectives 1. Describe benefits of a multidisciplinary CF & diabetes clinic. 2. Identify ways to optimize blood glucose control in CF. 3. Describe the benefits of exercise in CF. 101 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 4:30 p.m. - 6:30 p.m. Caregivers Clinics CG20 CLIN: PHARM: CF Pharmacist Practice-based Research Network: Updates & Future Planning (Research) 221 ABC Moderators Michelle Condren, Pharm.D., A.E.-C., C.D.E. Hanna Phan, Pharm.D., BCPS Speakers Lisa Hong, Pharm.D. Jessica Louie, Pharm.D. Description The investigators of several collaborative trials will discuss the design of current studies while focusing on obstacles & successes. A description of the pharmacy practice-based research network, based on a disseminated survey, will be presented. Pharmacists will brainstorm research questions & discuss preliminary methodology. Objectives 1. Describe methods to overcome barriers during collaborative research projects. 2. Identify resources needed for successful project management in a practicebased research network. (PBRN). 3. Develop future research questions for the CF pharmacist PBRN, including possible study design. CG21 PT & RT: PT: Resistance Training Over the Lifespan of Patients With CF (Care) 229 AB Moderators Valerie Matthews, P.T., D.P.T. Scott P. Russell, P.T., D.P.T., C.C.S., G.C.S. Speakers Anne Gould, P.T. Holly L. Loosen, P.T. Valerie Matthews, P.T., D.P.T. Matthew Nippins, P.T., D.P.T., C.C.S. Scott P. Russell, P.T., D.P.T., C.C.S., G.C.S. Jane E. Schneiderman, Ph.D., M.Sc. Description This session is designed for PTs to improve their understanding of the benefits of resistance training within the CF population across the lifespan. Basic exercise physiology will be reviewed, including how CFTR defects in skeletal muscle impact functional muscle performance. Simple assessment measures of strength & power will be identified & exercise prescription parameters will be discussed. Objectives 1. Identify the benefits of resistance training for pediatric & adult CF patient populations. 2. Discuss training parameters for key muscle groups that are important for the CF patient population. 3. Discuss special considerations for resistance training for patients both pre- & post-lung transplantation. 102 | 2015 North American CF Conference FINAL PROGRAM SATURDAY, OCTOBER 10 4:30 p.m. - 6:30 p.m. Caregivers Clinics CG22 RT: Infection Control: Stories From the Trenches (Care) 226 ABC Moderators Cathy O'Malley, R.R.T., N.P.S. Paulette M. Ratkiewicz, R.R.T., CPFT Speakers Mary Lester, R.R.T. Erin Moore Joanne Salazar, R.R.T., CPFT, N.P.S. Adrienne Savant, M.D., M.S. Lauren Willis, B.S., R.R.T.-N.P.S. Description This session will present a variety of stories about infection prevention & control in CF. The stories will be presented by individuals caring for people with CF & will highlight the challenges & the importance of IC in CF, particularly as it relates to the respiratory care practitioner. In addition, this session will review the recommendations for respiratory care equipment from the CFF. Objectives 1. Describe the current IC recommendations for cleaning & disinfecting the nebulizer & explore how best to care for the other respiratory care devices used in CF care. 2. Discuss the challenges clinicians & families face in striving to implement the IC recommendations for patients with CF. 3. Analyze & formulate strategies to provide infection prevention & control measures. CG23 SW/PSYCH: Bridging the Gap: How to Become the Social Worker Your Patient Thinks You Should Be (Care) 102 AB Moderators Susan C. Horky, LCSW Ronnie Sharpe Speakers Angela Miney, B.A. Description This session will use survey results, presentations, film & a panel of family members/ people with CF to provide participants with effective strategies for engaging people with CF & their families. Objectives 1. Apply strategies for engaging people with CF & their families. 2. Identify three methods for bridging the gap between social work & family views. 3. Describe three ways to learn more about families, (e.g. enhanced listening skills, use of videos). 7:30 p.m. Closing Event Limited Session. Pre-registration is required & an extra fee applies. 103 | 2015 North American CF Conference 120 ABCD NOTES 104 | 2015 North American CF Conference One Community. Many Benefits.* Find out what’s new at Booth #918 *Program restrictions apply. Offer not valid for patients enrolled in Medicare, Medicaid, or other federal or state healthcare programs. Program expires June 2016. Actavis reserves the right to rescind, revoke, or amend this program without notice. By registering for the program, the patient understands and agrees to comply with the full Terms and Conditions of this offer. Please see full program Terms and Conditions at www.Live2Thrive.org, or speak to a representative. Actavis® and its design are trademarks of Actavis, Inc. or its affiliates. Live2Thrive® and its design are trademarks of Actavis, Inc. or its affiliates. ©Actavis 2015. All rights reserved. UNB36049 ANNOUNCING FUTURE NORTH AMERICAN CF CONFERENCES SPONSORED BY THE CYSTIC FIBROSIS FOUNDATION October 27-29, 2016 Orange County Convention Center; Orlando, Florida November 2-4, 2017 Indiana Convention Center; Indianapolis, Indiana October 18-20, 2018 Colorado Convention Center; Denver, Colorado 08/15
