29th annual north american - North American Cystic Fibrosis

Transcription

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.
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.
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
SPECIAL APPRECIATION TO OUR
PLATINUM LEVEL SUPPORTERS
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.
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
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
Sheraton Hotel & select Conference Hotels
All Official Conference Hotels
Thursday, October 8
Friday, October 9
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
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.
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.
7:00 a.m. - 4:30 p.m.
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.
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 nacfc@cff.org.
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.
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
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
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
Rooms 201-213
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.
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 (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
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
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)
9:00 a.m. - 10:00 a.m. ALL: Plenary Session II: Opening Doors to CF Clinical
Research (G. Retsch-Bogart)
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)
PROGRAM AT-A-GLANCE
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)
7:30 a.m. - 8:45 a.m. Continental Breakfast/Informal Poster Viewing
6) GMS: Genome Editing (M. Drumm, P. Harrison)
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)
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)
TAB SHEET 5
WEDNESDAY
TAB SHEET 5
WEDNESDAY
FINAL PROGRAM
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.
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.
FINAL PROGRAM
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
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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)
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.
TAB SHEET 5
THURSDAY
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
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.
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
120 Foyer
100 Level Corridors
301 Foyer
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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?
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
W07
CLIN: Creating a Culture of Research (Research)
120 BC
Chairs Robert E. Fowler
10:00 a.m.
Introduction
Robert E. Fowler
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
Implications: Outpatient Setting
Michelle A. Powers, R.N., B.S.N.
10:50 a.m.
Invited
Speaker
Implications: Respiratory Equipment
Catherine O'Malley, R.R.T., N.P.S.
11:05 a.m.
Invited
Speaker
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
10:10 a.m. Abstract Force Field Analysis of Mental Health Screening & Treatment in Six Accredited CF
Centers
604
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
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
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
Exhibit Hall E
Exhibit Hall E
FINAL PROGRAM
THURSDAY, OCTOBER 8
S01 CFTR: CFTR Properties & Organ-specific Disease (Research)
120 D
Chairs Estelle A. Cormet-Boyaka, Ph.D.
2:00 p.m.
Introduction
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
S03 INF/MIC: Challenging Topics in Infectious Disease (Combination)
120 A
Chairs Charles S. Haworth, M.D., FRCP
2:00 p.m.
Introduction
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
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
2:10 p.m.
S4.1 Role of CFTR in Normal Exocrine Pancreatic Function
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
2:00 p.m.
Introduction
Patrick A. Flume, M.D.
2:10 p.m.
S5.1 The Current State of Exacerbation Management
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?
3:25 p.m.
S5.4 The Next Chapter for the Treatment of Acute Pulmonary Exacerbation in CF:
What Is the Next Study?
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.
FINAL PROGRAM
THURSDAY, OCTOBER 8
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
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.
301 D
FINAL PROGRAM
THURSDAY, OCTOBER 8
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.
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
Heritage
Park
TAB SHEET 5
FRIDAY
FINAL PROGRAM
FRIDAY, OCTOBER 9
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
Exhibit Hall E
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.
120 Foyer
100 Level Corridors
301 Foyer
FINAL PROGRAM
FRIDAY, OCTOBER 9
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.
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
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.
120 D
FINAL PROGRAM
FRIDAY, OCTOBER 9
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?
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
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
S11 INF/MIC: MRSA Update (Combination)
301 BC
Chairs Deirdre F. Gilpin, Ph.D.
10:30 a.m.
Introduction
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
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
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
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
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 cffcenterassist@cff.org 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
(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
(LIMITED SESSION. Attendance is limited to Directors of CFF-accredited Affiliate
CF Programs. Pre-registration is required. ADMISSION BY TICKET ONLY.)
FINAL PROGRAM
FRIDAY, OCTOBER 9
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
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.
West Building 213 AB
FINAL PROGRAM
FRIDAY, OCTOBER 9
12:30 p.m. - 1:50 p.m. Junior Investigators – Supported by Gilead Sciences, Inc.
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.
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
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.
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
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.
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.
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.
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
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.
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.
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
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.
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.
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.
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.
2:00 p.m.
Introduction
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.
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.
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
Chairs Kathleen Richards, R.R.T.
2:00 p.m.
Introduction
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
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
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.
Speakers Amy Garcia, M.D.
Daina B. Kalnins, R.D., M.Sc.
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
CG13 CLIN: PHARM: Clinical Controversies in CF Care (Care)
221 ABC
Moderators Kevin L. Epps, Pharm.D., BCPS
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.
FINAL PROGRAM
FRIDAY, OCTOBER 9
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.
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.
222 ABC
FINAL PROGRAM
FRIDAY, OCTOBER 9
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.
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.
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.
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
Hall C
TAB SHEET 5
SATURDAY
FINAL PROGRAM
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
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
W22 APP&D: CF Airway Inflammation, Biomarkers & Therapeutic Targets
(Research)
126 ABC
Chairs Gerry N. McElvaney, M.B., FRCPI, FRCPC Jennifer L. Taylor-Cousar, M.D.
10:30 a.m.
Introduction
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.
FINAL PROGRAM
W23 APP&D: Non-CFTR Ion Transport Targets for CF (Research)
124 AB
Chairs Hugo R. de Jonge, Ph.D.
10:30 a.m.
Introduction
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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?
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.
FINAL PROGRAM
CLIN: CF Pulmonary Disease: Diagnosis, Evaluation & Treatment
W26 (Combination) 122 ABC
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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
11:10 a.m.
Abstract
619
The Investigation of Quality of Life & Adherence in Patients With the G551D
Mutation Receiving Ivacaftor Therapy
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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.
FINAL PROGRAM
BB10 NTR: Adult Nutrition: Emerging Issues (Care)
105 ABC
Moderators Gretchen M. Garlow, M.S., R.D., L.D.N., CNSC
Speakers Deborah Friedman, Ph.D.
Gretchen M. Garlow, M.S., R.D., L.D.N., CNSC
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.
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
CFTR
4
CFTR: Understanding the Mechanisms of Action of CFTR Modulators
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.
Sheraton
Phoenix
Ballroom
FINAL PROGRAM
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
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
INF/MIC
27
INF/MIC: Treatment of Non-tuberculous Mycobacteria
Stacey L. Martiniano, M.D.
FINAL PROGRAM
Sheraton
Phoenix
Ballroom
INF/MIC
28
INF/MIC: Evolution of Pseudomonas During Infection
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
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
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.
FINAL PROGRAM
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.
FINAL PROGRAM
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
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.
FINAL PROGRAM
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.
FINAL PROGRAM
12:30 p.m. - 2:20 p.m. U.S. Center & Program Directors Meeting*
Sheraton Valley of the Sun C-E
(For directors of all U.S. CF centers & CFF-accredited Adult & Affiliate Programs.
One representative per program should attend.)
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
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.
FINAL PROGRAM
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
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.
2:30 p.m.
Introduction
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
FINAL PROGRAM
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
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.
FINAL PROGRAM
S19 CLIN: Update on Lung Transplantation in CF (Combination)
131 ABC
Chairs Robert Aris, M.D.
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
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.
FINAL PROGRAM
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.
2:30 p.m.
Introduction
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.
FINAL PROGRAM
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.
200 Level Corridors
FINAL PROGRAM
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.
FINAL PROGRAM
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.
Speakers Anne Gould, P.T.
Holly L. Loosen, P.T.
Valerie Matthews, P.T., D.P.T.
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.
FINAL PROGRAM
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.
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
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.
120
ABCD
NOTES
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

29th annual north american - North American Cystic Fibrosis

Transcription

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