Funding for this research was provided by:
Cystic Fibrosis Foundation Therapeutics (LIOU13A0, LIOU14Y4)
National Heart, Lung, and Blood Institute (HL125520)
Ben B. and Iris M. Margolis Foundation
Claudia Ruth Goodrich Stevens Family Endowment Fund (None)
Received: 18 September 2018
Accepted: 11 March 2019
First Online: 26 April 2019
: DRC is an Honorary Fellow at Nuffield College. His pioneering work in Statistics includes introduction of the current methods of logistic regression [CitationRef removed] and proportional hazards models [CitationRef removed]. He was awarded the Guy Medal in both Silver and Gold from the Royal Statistical Society, the Kettering Prize and Gold Medal for Cancer Research, the Copley Medal from the Royal Society, and he recently won the first International Prize in Statistics awarded jointly by the American Statistical Association, the International Biometric Society, the Institute of Mathematical Statistics, the International Statistical Institute, and the Royal Statistical Society. His lifelong experience improving the design of experiments [CitationRef removed, CitationRef removed] was instrumental in the design of the current study.RK received her DPhil under the supervision of DRC from the University of Oxford. She is now Associate Professor in the Department of Medical Statistics at the London School of Hygiene and Tropical Medicine, is funded by a Medical Research Council Methodology Fellowship, and teaches Master’s level courses in medical statistics. She helped the study greatly by participating in the teaching video (Additional files 1, 2 and 3) with DRC and with our initial study design.Our non-MWCFC collaborators brought skills and experience not found in the MWCFC. NL is Director of the Adult CF Program and Associate Professor in the Department of Medicine, Division of Pulmonary and Critical Care Medicine at the Johns Hopkins University. He has published in the area of assessment of pain in patients with CF [CitationRef removed] and brings that expertise to our study. ALQ is a behavioral scientist and clinical psychologist at the Miami Children’s Research Institute. She was awarded the inaugural Mattingly Leadership in Mental Health Care Award in 2016 by the US CF Foundation, and she contributed her expertise on quantitative measurements of depression, anxiety and quality of life [CitationRef removed] to the study.The study benefited greatly from guidance by the external advisory committee. JPC is Professor and Research Director for the Division of Pulmonary Medicine, Department of Pediatrics, at the U of Cincinnati and has been a member or leader of multiple research organizations within the CF Foundation, the European CF Society and the National Institutes of Health in the US (NIH). KNO is Senior Clinician and Chief of the Pulmonary Branch of the National Heart, Lung and Blood Institute (NHLBI) of the NIH and leader of the Chronic Airway Infection Laboratory at the NHLBI. He has led multiple studies of bronchiectasis and infections, especially non-tuberculous <i>Mycobacteria</i>. JSE is Faculty Pro-Vice Chancellor of the School of Medicine, Dentistry and Biomedical Sciences and Faculty in the Institute for Health Sciences and the Centre for Experimental Medicine, Queen's University Belfast, UK. He was President of the European CF Society for 8 years and Trustee, Chair or Member of the Medical Advisory Committee of the CF Trust in the UK since 2002. He received knighthood in 2012 for his work in health care in Northern Ireland. He has been author and driving force for many studies that have improved knowledge and care of patients with CF.The authors from the Mountain West CF Consortium have been meeting and working collaboratively for over 20 years including a recent publication [CitationRef removed]. The long-standing close relationships between these individuals and teams enhanced the ability to design and perform this complex multicenter study.
: Our project was reviewed and approved by the Investigational Review Board (IRB) of the University of Utah (IRB_00011571), the St. Luke’s Health System IRB (IRB Protocol No.: 13–0547), the IRB of Billings (Study 14.07), the Western IRB (for the Las Vegas CF Center) (STUDY NUM 1146159, WIRB PRO NUM: 20140721, INVEST NUM: 112264, WO NUM: 1–837046-1, Protocol Number 20130530), the National Jewish Health IRB (Study Number: HS-2797), Colorado Multiple IRB (for Children’s Hospital Colorado) (COMIRB Protocol 13–3172), the Phoenix Children’s Hospital IRB (PCH IRB #13–087), the University of Arizona IRB (Protocol Number: 1407394040), and the Human Research Review Committee in the Human Research Protections Office at the University of New Mexico (Study ID 14–085). No patients were enrolled prior to IRB approval. All patients younger than 18 provided assent with informed written consent provided by parents or other legal guardians. Each adult patient gave informed written consent on his or her own behalf.
: Not applicable.
: TGL, JAF, JLJ, YL, KAP and JBV received other support from the CFF (CC132-16 AD, LIOU14Y0, LIOU14P0) and the National Heart Lung and Blood Institute (NHLBI) of the National Institutes of Health (NIH) (R01 HL125520) and received support during the current study for performing clinical trials from Abbvie, Gilead, Nivalis, Novartis, Proteostasis, Inc., Savara and Vertex. FRA received additional other support from the NHLBI/NIH (R01 HL125520), the National Science Foundation (EMSW21-RTG) and the Margolis Foundation of Utah. PSB received other support from the CFF (Center and TDC grants) and the NHLBI/NIH (U01 HL114623) and received support for a clinical trial from Alcresta. BAC received other support from the CFF (C112–12, C112-TDC09Y, 10063SUB, 41,339,154.s132P010379SUB) and received support for clinical trials from Genentech, Novartis and Vertex. CLD received other support from the CFF (C004–11, C004-TDC09Y, DAINES11Y3) and from the Health Resources and Services Administration (T72MC00012). TH received other support from the CFF (PACE, Center Grant) and received support for clinical trials from Celtaxsys and Vertex. JRH received other support from the NHLBI/NIH (HHSN268200900018C) and the Veterans Administration Healthcare System (I01 BX001533). JL received other support from the CFF (C017-11AF). CN received other support from the CFF (C138–12). PR received other support from the CFF (C003–12, C003-TDC09Y). SDS received other support from the CFF (AQUADEK12K1, SAGEL11CS0, GOAL13K2, NICK13A0, SAGEL14K1, NICK15R0) and the NHLBI/NIH (U54 HL096458) and the NCATS/NIH (Colorado CTSA Grant Number UL1 TR002535). JLT-C received other support from the CFF (TDC) and the NHLBI/NIH (HL103801) and received support for clinical trials from Vertex. KNO is funded by the intramural research program of the NHLBI, NIH.
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