Despite newer treatment options and increasing awareness of pulmonary fibrosis (PF) in recent years, diagnostic delays and misdiagnosis remain common occurrences in this patient population.
The findings from a study published in 2019, for example, revealed a median diagnostic delay of 2.1 years in patients with idiopathic PF (IPF), and 41% of these patients reported misdiagnosis of respiratory symptoms prior to receiving an accurate diagnosis.1 The authors concluded that further efforts are needed to increase awareness among patients and physicians to reduce such delays.
As a central goal of PF Awareness Month and their ongoing work, the Pulmonary Fibrosis Foundation (PFF) aims to meet this challenge along with many other issues affecting PF communities.2 In addition, the PFF Care Center Network (CCN) and PFF Patient Registry represent invaluable assets in the quest to improve PF research and patient outcomes.3
We interviewed the following experts to learn more about these endeavors: Sonye Danoff, MD, PhD, senior medical advisor for the PFF CCN and Patient Registry; and Kevin R. Flaherty, MD, MS, steering committee chair for the PFF CCN and Patient Registry.
What prompted the creation of the PFF Care Center Network, and why was there a need for such a system?
Dr Flaherty: PF is a complex disease, and diagnosis and treatment can be challenging as the symptoms — eg, shortness of breath, fatigue, and dry, persistent cough — can be common in other illnesses. Therefore, patients often experience a misdiagnosis in the early stages, leading to a significant delay in an accurate diagnosis. Following a diagnosis of PF, patients typically have very little understanding of the disease.
We created the CCN so that people living with PF can find experienced medical professionals who understand their disease and can provide comprehensive, high-quality care. From clinical staff to pathologists and radiologists, each site provides access to a multidisciplinary team of experts who are experienced in diagnosing and treating PF.
Dr Danoff: To add to that, the CCN provides an infrastructure that facilitates research on PF and accelerates the development of clinical trials. An important part of that research is the PFF Patient Registry, a database of patients with PF who have agreed to share detailed medical information for use in this research.
Our care centers also allow us to educate communities at the local level about the disease, including medical providers, PF patients and their caregivers, and the general public. Our ultimate goals are to promote earlier diagnosis of PF and ensure that patients receive the highest standard of care.
What is the process for a site to become a PFF Care Center, including some of the main requirements?
Dr Flaherty: When selecting sites to add to the PFF CCN, we consider a center’s specific programs and its geographic location in order to best serve the needs of the broader PF community.
Below are some requirements we look out for when selecting a site.4
- Above all, care centers should provide excellent patient care. They should have an established Interstitial Lung Disease (ILD) program with access to all the care needed to support patients living with fibrotic lung disease, including special consult services, social work, diagnostic testing, palliative care, sleep studies, pulmonary rehabilitation, and lung transplant. We want to ensure that these centers have the infrastructure, person power, and services to provide excellent patient care. For instance, each center is required to have a care center director, nurse or equivalent clinical staff, radiologist, and pathologist on site.
- We also want CCN sites — from students and residents to fellows and healthcare professionals — to be involved in training and educating those who are studying and caring for patients with ILD so that we can continue to move the field forward.
- The centers must be actively engaged in PF research, whether participating in surveys or enrolling patients in clinical trials.
- They should be a part of or affiliated with a PF support group.
- They should have willingness to participate in PFF programs and with the other care centers to change the landscape of PF research and care.
The PFF is undergoing a program evaluation of the CCN, specifically in regard to the application and acceptance process of nationwide medical institutions. Our assessment of the CCN will guide future expansion to best serve the community to enhance access, awareness, and care.
What are the most notable recent advances in PF research and treatment —both in general and related to the PFF CCN?
Dr Danoff: One of the first real breakthroughs in the growing field of PF was in October of 2014, when the US Food and Drug Administration (FDA) approved the first 2 antifibrotic drugs for the treatment of IPF, offering new hope to patients. This included the approvals of pirfenidone and nintedanib. Before this, there were no approved therapies in the United States specifically for IPF, and patients had limited options for symptom management. Since that time, the FDA has also approved the use of nintedanib for patients who have other forms of ILD that progresses despite the use of other appropriate therapies.
Also, just last year the PFF announced the launch of PRECISIONS, a collaborative study funded by the National Institutes of Health and Three Lakes Foundation, that aims to transform the diagnosis and treatment of IPF — as well as other forms of PF — by moving it into the era of precision medicine.5 PRECISIONS will further define the genetic risk factors influencing the development and potential progression of PF, and it will hopefully allow for the identification of important biomarkers to assist in the diagnosis and care of patients with PF. The PFF is contributing to this study through the PFF Registry and Biorepository. This study would not have been feasible if not for the efforts of the PFF CCN.
Since the CCN launched, what have you observed or heard from patients, families, and others regarding the unique benefits of this model?
Dr Flaherty: Since the launch, the feedback has been very positive from both the medical and patient communities. By engaging in the CCN, we’ve created a collaborative environment and an ongoing dialogue with physicians and researchers, which has helped us to curate a resource library with up-to-date information for healthcare professionals and allowed us to bring clinicians and patients together. Clinicians appreciate the resources that we provide, and patients and caregivers are happy to have a dedicated focus on them, as well as the support of other patients in a similar situation. Many feel helpless when diagnosed with the disease, so the ability to provide support to others and take part in important research is valued.
What are remaining needs and future directions in this area?
Dr Danoff: Currently, we have 68 medical centers in 33 states, and we are looking to increase coverage in more states nationwide so that patients can more easily access a care center site in their local area.3 We also plan to expand our Registry, incorporating a virtual component, so that we can reach not only more patients who cannot travel to a local CCN site, but also caregivers and family members to help generate a pathway to future research. We need much more concentrated research on PF to improve quality of life for patients and to identify additional safe and effective therapies for PF, and eventually our ultimate goal is to identify a cure.
Since PF encompasses many types of ILD, we want to use this expansion to help better educate the community and advance research in all forms of PF. This requires the cooperation of healthcare professionals, scientists, and patients from across the country to bring together large enough groups to understand some of the rarer forms of PF. The PFF CCN along with the Registry has provided the infrastructure to begin this effort. As the CCN expands to include other regions, we hope to provide the same opportunities to even more patients nationwide.
Dr Flaherty: We could not build this momentum without the generous support of our partners, from corporations and foundations to individuals who are dedicated to improving the lives of those affected by PF. Importantly, advances in the field of PF are directly tied to the patients and their participation in the PFF Registry, clinical trials, and advocacy initiatives. The pathway toward a cure is only identified with the direct involvement of patients, caregivers, and families collaborating with researchers, clinicians, and regulatory agencies.
What are the PFF’s goals for PF Awareness Month this year?
Dr Danoff: Our goal this year for PF Awareness Month is to further educate the public about this life-threatening disease. Spotlighting our patient community, the PF Heroes who are providing their PF stories, is a powerful way to spread awareness and offer hope. Earlier this year, the PFF conducted an awareness survey of more than 2000 US adults, and the survey results show a clear need to increase understanding of PF.6 For example, almost 9 in 10 Americans do not know the symptoms associated with the disease.
Our mission is to mobilize the entire PF community — from physicians to patients and caregivers — in providing disease education so that patients are better able to recognize the early signs and start conversations with their physicians.
You can follow along on the PFF’s social channels (@pfforg) with the hashtags #PFMonth and #BlueUp4PF, and to learn more, visit the Pulmonary Fibrosis Foundation Awareness Month website.
1. Hoyer N, Prior TS, Bendstrup E, Wilcke T, Shaker SB. Risk factors for diagnostic delay in idiopathic pulmonary fibrosis. Respir Res. 2019;20(1):103. doi:10.1186/s12931-019-1076-0
2. Pulmonary Fibrosis Foundation. PF Awareness Month. https://www.pulmonaryfibrosis.org/get-involved/pf-awareness Accessed on September 20, 2020.
3. Pulmonary Fibrosis Foundation. PFF Care Center Network. https://www.pulmonaryfibrosis.org/medical-community/pff-care-center-network Accessed on September 20, 2020.
4. Pulmonary Fibrosis Foundation. PFF Care Center Criteria. https://www.pulmonaryfibrosis.org/docs/default-source/medical-community-documents/pff-care-center-criteria-2019-v-03-updated_03-01-2019.pdf Accessed on September 20, 2020
5. Pulmonary Fibrosis Foundation. NIH awards major grant in pulmonary fibrosis research. November 4, 2019.
6. Pulmonary Fibrosis Foundation. National Awareness Survey 2020. https://www.pulmonaryfibrosis.org/docs/default-source/marketing-brochures/pff-national-consumer-survey-report—for-review-updated-2-19-2020.pdf Accessed on September 20, 2020