A Conversation With Amy Hajari Case, M.D., Chief Medical Officer of the Pulmonary Fibrosis Foundation

Amy Hajari Case, M.D.

Amy Hajari Case, M.D., was named the new chief medical officer for the Pulmonary Fibrosis Foundation (PFF) in October 2024.

Case is the director of the Interstitial Lung Disease Program and Pulmonary and Critical Care Research Department at Piedmont Healthcare in Atlanta. She joined the PFF as the senior medical adviser for education and awareness in 2020.

As the chief medical officer of the PFF, which recent trends in the understanding and treatment of pulmonary fibrosis have you observed, and how do these trends impact patient care?

It’s been a little over 10 years since the antifibrotics pirfenidone [Esbriet] and nintedanib [Ofev] were approved by the FDA. I think providers have become more comfortable over time with the use of those medications for patients with idiopathic pulmonary fibrosis and for patients with different types of progressive pulmonary fibrosis. These antifibrotics have become the standard of care therapy for pulmonary fibrosis.

As we’re looking forward to new therapies, with the pipeline being as robust as it is, we’ll need to understand how to deploy the different medications that become available to us against the background of these existing therapies. We’re exploring multiple different pathways now to try to enhance and augment the disease-directed therapy.

We’ve made significant strides in the comprehensive management of pulmonary fibrosis, particularly through nonpharmacologic therapies like supplemental oxygen and pulmonary rehabilitation. Our approach to managing comorbidities, such as pulmonary hypertension related to interstitial lung disease, has also become more advanced and nuanced. Together, these improvements lead to better care for patients.

How is PFF addressing the disparities in care and outcomes for patients with pulmonary fibrosis across different demographics and regions?

There are more than 250,000 people living with pulmonary fibrosis and interstitial lung disease in the U.S., and we recognize that most patients will not be seen at one of our 88 care center sites across the country. However, our care center and clinical associate sites do an outstanding job of education and connecting with other healthcare groups to enhance care in their regions.

The PFF actively collaborates with our academic colleagues, industry partners and those in community practices on research and quality interstitial lung disease care. We have several working groups that are looking at ways to bring the standard of care to patients in underserved areas. For example, recent studies show disparities in rural U.S. patients, including increased risk for idiopathic pulmonary fibrosis, decreased antifibrotic utilization and worse disease severity at presentation, explained by indicators of worse neighborhood-level socioeconomic status. Our Rural Health Outreach Committee developed a position statement for both patients and providers. The statement identifies strategies that impact patient care, research and advocacy.

We also want to make research opportunities as accessible as possible. In 2022, we launched the PFF Community Registry, a decentralized platform that allows us to connect with people wherever they are in the U.S. The community registry collects data from patients, caregivers, biological family members, lung transplant recipients and those who have lost a loved one to pulmonary fibrosis, interstitial or lung disease. Currently, more than 2,400 participants are enrolled in the community registry.

Can you highlight any recent research breakthroughs or clinical trials that are particularly exciting or promising in the field of pulmonary fibrosis?

The therapeutic pipeline is stronger than ever, with numerous developments in progress. We’re optimistic that in the next few years we’ll see the approval of some of these promising new therapies.

PRECISIONS, the first pharmacogenomic study in idiopathic pulmonary fibrosis, is a compelling study to follow. It was pragmatically designed and is looking at a simple antioxidant medication, N-acetylcysteine, for people with a very specific TOLLIP genotype. In addition to the primary endpoint of the N-acetylcysteine impact, researchers are analyzing a wide range of data to explore broader implications.

The positive topline data on nerandomilast from the FIBRONEER studies is also exciting, and we look forward to seeing the additional results presented and published later this year. It’s possible that, within the next year, we’ll have another valuable tool for treating idiopathic pulmonary fibrosis.

There are several late-phase 2 and phase 3 studies underway that are worth watching, with many opportunities for patient participation. We are grateful to all the patients participating in this research, as their involvement is essential to advancing treatment options and improving care for all. Patients can learn more about current studies by using the PFF’s Clinical Trial Finder.

What are some of the key challenges currently facing patients with pulmonary fibrosis, particularly regarding diagnosis, access to treatments, and support resources?

Delays and missed diagnoses are all too common for patients with pulmonary fibrosis, often due to similarities in presentation to more prevalent pulmonary and cardiac conditions. Also, awareness of pulmonary fibrosis as a potential underlying cause remains limited among both healthcare professions and the general public. By increasing awareness, we can improve diagnostic accuracy. After all, if a rare disease isn’t considered, it’s unlikely to be diagnosed.

One of the ways we’re working to expand access to treatments is through our Care Center Network’s seven clinical associate sites. Each of these community-based programs is strategically paired with one of our Care Center Network sites to engage directly with local practices and extend their reach into the surrounding areas. By connecting with these communities, we aim to raise awareness and ensure that individuals have access to providers who can make accurate diagnoses and guide them toward the appropriate therapies and support they need.

A critical challenge for our community is access to supplemental oxygen. Patients are dealing with disease progression and will need supplemental oxygen at some point to get through their day. The PFF is working with 30 leading health organizations that are calling on Congress to support the Supplemental Oxygen Access Reform (SOAR) Act. This legislation aims to ensure that people enrolled in Medicare who need supplemental oxygen can access the correct type and levels of oxygen needed for them to live full, active lives.

What role do you see patient advocacy and education playing in the management of pulmonary fibrosis, and how is the foundation working to promote awareness of this condition?

Patient advocacy and education play a vital role in the management of pulmonary fibrosis. We want to empower patients with knowledge and help them to live their best lives. We just recently unveiled an education program, “PF Basics: Info for Newly Diagnosed Patients,” featuring six online modules that guide and support people who are newly diagnosed with pulmonary fibrosis or interstitial lung disease. This curated program answers patients’ most pressing questions about these diseases and reminds them that they are not alone. The PFF is here for them every step of the way.

Advocacy is essential to communicating the importance of federal funding for pulmonary fibrosis research and supplemental oxygen reform. Because of the PF community’s participation in and commitment to advocacy, public funding for PF research has steadily increased in recent years.

The patient is at the center of everything we do. Each of our working groups includes patient-level involvement, and we actively engage patients, families, and caregivers in the development of position statements and best practices and in shaping our research, strategic planning and educational resources. Their needs and perspectives guide our efforts.