Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias. Although rare enough to be considered an orphan disease, the annual incidence of IPF is 94 cases per 100,000 person-years.1 IPF accounts for 15,000 to 40,000 deaths per year in the United States—and the incidence and mortality of IPF are increasing.1-3 The disease is characterized by an insidious onset and progressive decline in lung function because of fibrosis of the lung parenchyma. Despite the recent approvals of two new drugs, each shown to slow the decline in lung function, there is no therapeutic cure for IPF.4 The only treatment shown to improve survival is lung transplantation.5 Indeed, the prognosis of IPF is poor, with median survival estimates of 3.8 years or less.1
An international coalition of societies, including the American Thoracic Society, published comprehensive guidelines for the diagnosis and management of pulmonary fibrosis in 2011 and subsequently revised eight treatment recommendations in 2015.4,5 Despite the availability of these evidence-based guidelines, significant gaps in the care of patients with IPF remain. These gaps include delayed or inaccurate diagnosis, delayed referral to specialty care, limited use of recommended multidisciplinary (interprofessional) care, variable approaches to management, and poor patient-provider communication.6
The Pulmonary Fibrosis Foundation (PFF), founded in 2000, established the Care Center Network (CCN) as its central effort to improve and standardize medical care in pulmonary fibrosis. As of December 2017, the PFF CCN includes 45 centers in 27 states. The PFF CCN has the potential to improve patient outcomes by sharing local expertise and clinical excellence in IPF diagnosis and disease management with other CCN sites and community clinicians. In practice, the CCN sites face diverse challenges, and recent discussion of these issues by stakeholders demonstrated the clear need for a consistent “top-down” strategy that incorporates current standards of practice and can be extended to all centers.7 Thus, one of the PFF’s CCN goals is to bring together the collective resources of centers and facilitate intercenter communication to establish current standards of practice in the management of interstitial lung disease (ILD).7
1. Raghu G, Chen S-Y, Yeh W-S, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: Incidence, prevalence, and survival, 2001–11. Lancet Respir Med. 2014;2(7):566-572. doi:10.1016/ S2213-2600(14)70101-8.
2. Hutchinson JP, McKeever TM, Fogarty AW, Navaratnam V, Hubbard RB. Increasing global mortality from idiopathic pulmonary fibrosis in the twenty-first century. Ann Am Thorac Soc. 2014;11(8):1176-1185. doi:10.1513/ AnnalsATS.201404-145OC.
3. Nalysnyk L, Cid-Ruzafa J, Rotella P, Esser D. Incidence and prevalence of idiopathic pulmonary fibrosis: Review of the literature. Eur Respir Rev. 2012;21(126):355-361. doi:10.1183/09059180.00002512.
4. Raghu G, Rochwerg B, Zhang Y, et al. An Official ATS/ERS/JRS/ALAT Clinical practice guideline: Treatment of idiopathic pulmonary fibrosis. an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3-e19. doi:10.1164/ rccm.201506-1063ST.
5. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. doi:10.1164/rccm.2009-040GL.
6. Spagnolo P, Tonelli R, Cocconcelli E, Stefani A, Richeldi L. Idiopathic pulmonary fibrosis: Diagnostic pitfalls and therapeutic challenges. Multidiscip Respir Med. 2012;7(1):42. doi:10.1186/2049-6958-7-42.
7. Cottin V, Roman J, Eu J, Furlong P, Sherry D, Morthanos J. Leveraging the PFF Care Center Network. In: PFF Summit, from Bench to Bedside 2015. Washington, DC; 2015. http://www.pffsummit.org/.
8. Heath C, Heath D. Switch: How to Change Things When Change Is Hard. New York, NY: Penguin Random House; 2010.
9. Margolis PA, Peterson LE, Seid M. Collaborative Chronic Care Networks (C3Ns) to transform chronic illness care. Pediatrics. 2013;131(Supplement 4):S219-23. doi:10.1542/peds.2012-3786J.
10. Pulmonary Fibrosis Foundation. The Facts: PFF Care Center Network and PFF Patient Registry. http://www.pulmonaryfibrosis.org/docs/default-source/news-documents/pulmonaryfibrosisfoundation_pffregistryfactsheet.pdf?sfvrsn=0. Accessed October 20, 2017.
11. National Committee for Quality Assurance. NCQA’s Patient-Centered Medical Home (PCMH) 2011. https://www.ncqa.org/.
12. Hunt VL, Chaudhry R, Stroebel RJ, North F. Does pre-ordering tests enhance the value of the periodic examination? Study design-process implementation with retrospective chart review. BMC Health Serv Res. 2011;11:216. doi:10.1186/1472-6963-11-216.
13. Rivo J, Page TF, Arrieta A, et al. The impact of comprehensive pre-visit preparation on patient engagement and quality of care in a population of underserved patients with diabetes: Evidence from the care management medical home center model. Popul Health Manag. 2016;19(3):171-177. doi:10.1089/pop.2015.0063.
14. Casalino LP, Nicholson S, Gans DN, et al. What does it cost physician practices to interact with health insurance plans? Health Aff (Millwood). 2009;28(4):w533-43. doi:10.1377/hlthaff.28.4.w533.
15. Russell A-M, Ripamonti E, Vancheri C. Qualitative European survey of patients with idiopathic pulmonary fibrosis: Patients’ perspectives of the disease and treatment. BMC Pulm Med. 2016;16:10. doi:10.1186/ s12890-016-0171-y.
16. Collard HR, Tino G, Noble PW, et al. Patient experiences with pulmonary fibrosis. Respir Med. 2007;101(6):1350-1354. doi:10.1016/j. rmed.2006.10.002.
17. Schoenheit G, Becattelli I, Cohen AH. Living with idiopathic pulmonary fibrosis. Chron Respir Dis. 2011;8(4):225-231. doi:10.1177/1479972311416382.
18. Wuyts WA, Peccatori FA, Russell A-M. Patient-centred management in idiopathic pulmonary fibrosis: Similar themes in three communication models. Eur Respir Rev. 2014;23(132):231-238. doi:10.1183/09059180.00001614.
19. Smith CW. The patient, the provider, and participatory medicine—are we a house divided? J Particip Med. 2014;6:e3. http://www.jopm.org/opinion/editorials/2014/02/06/the-patient-the-provider-and-participa- tory-medicine-are-we-a-house-divided/. Accessed December 4, 2017.
20. Institute of Medicine. Partnering with patients to drive shared decisions, better value, and care improvement. In: Roundtable on Value and Science-Driven Health Care. Washington, DC: National Academies Press (US); 2014.
21. Bonvicini KA, Perlin MJ, Bylund CL, Carroll G, Rouse RA, Goldstein MG. Impact of communication training on physician expression of empathy in patient encounters. Patient Educ Couns. 2009;75(1):3-10. doi:10.1016/j. pec.2008.09.007.
22. Weng C, Li Y, Berhe S, et al. An Integrated Model for Patient Care and Clinical Trials (IMPACT) to support clinical research visit scheduling workflow for future learning health systems. J Biomed Inform. 2013;46(4):642- 652. doi:10.1016/j.jbi.2013.05.001.
23. Institute of Medicine. The Learning Healthcare System: Workshop Summary. Washington, DC: The National Academies Press; 2007. doi:10.17226/11903.
24. Laursen DH, Christensen KB, Christensen U, Frølich A. Assessment of short and long-term outcomes of diabetes patient education using the health education impact questionnaire (HeiQ). BMC Res Notes. 2017;10(1):213. doi:10.1186/s13104-017-2536-6.
25. Politi MC, Dizon DS, Frosch DL, Kuzemchak MD, Stiggelbout AM. Importance of clarifying patients’ desired role in shared decision making. BMJ. 2013;347:7066. http://www.bmj.com/bmj/section-pdf/750201?path=/bmj/347/7936/Analysis.full.pdf. Accessed December 4, 2017.
26. Elwyn G, Frosch D, Thomson R, et al. Shared decision making: a model for clinical practice. J Gen Intern Med. 2012;27(10):1361-1367. doi:10.1007/s11606-012-2077-6.
27. Haskard KB, Williams SL, DiMatteo MR, Rosenthal R, White MK, Goldstein MG. Physician and patient communication training in primary care: Effects on participation and satisfaction. Heal Psychol. 2008;27(5):513- 522. doi:10.1037/0278-6126.96.36.1993.
28. Kreuter M, Bendstrup E, Russell A-M, et al. Palliative care in interstitial lung disease: Living well. Lancet Respir Med. 2017;5(12):968-980. doi:10.1016/ S2213-2600(17)30383-1.
29. Bajwah S, Ross JR, Wells AU, et al. Palliative care for patients with advanced fibrotic lung disease: A randomised controlled phase II and feasibility trial of a community case conference intervention. Thorax. 2015;70(9):830-839. doi:10.1136/thoraxjnl-2014-206583.
30. Higginson IJ, Bausewein C, Reilly CC, et al. An integrated palliative and respiratory care service for patients with advanced disease and refractory breathlessness: A randomised controlled trial. Lancet Respir Med. 2014;2(12):979-987. doi:10.1016/ S2213-2600(14)70226-7.
This continuing education supplement was developed from interviews with interstitial lung disease (ILD) centers, both in the Pulmonary Fibrosis Foundation Care Center Network and those outside of it. It is part of a three-phase curriculum, IPF Education for ILD Centers and Their Communities: Applying Behavior Change Theory to Facilitate and Measure Adoption of Current Standards of Practice Across Individuals and Systems in IPF. The supplement content brings together data from a patient survey and a previous CME/CE activity in this curriculum, “Practice Self-Assessment for Diagnosis and Management of Idiopathic Pulmonary Fibrosis (IPF),” found at: https://tinyurl.com/IPFAssess17, both of which were used to develop the interview questions for the interstitial lung disease centers.
This supplement was written by a team at Global Academy for Medical Education, LLC, Pro-Change Behavior Systems, and Stephanie Breslan, MS, medical writer.
Neither the editors of CHEST Physician nor the Editorial Advisory Board nor the reporting staff contributed to its content. The ideas and opinions expressed are those of the faculty and do not necessarily reflect the views of the supporters, Global Academy for Medical Education, LLC, Postgraduate Institute for Medicine, or the Publisher.
Copyright © 2017 by Global Academy for Medical Education, LLC, Frontline Medical Communications Inc., and its Licensors. All rights reserved. No part of this publication may be reproduced or transmitted in any form, by any means, without prior written permission of the Publisher. Global Academy for Medical Education, LLC, will not assume responsibility for damages, loss, or claims of any kind arising from or related to the information contained in this publication, including any claims related to the products, drugs, or services mentioned herein.