Respiratory Care Week: Pulmonary Fibrosis by the Numbers

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Pulmonary fibrosis (PF) is a life-threatening disease that remains largely unknown. Respiratory Care Week (Oct. 25–31) is the perfect time to educate the public about the disease and what’s being done to fight it — and to thank respiratory therapists (RTs) nationwide, who take the lead in helping patients cope with the disease. To start, it’s important to discuss PF by the numbers — from disease awareness to advocacy and research.

Over 200,000 Americans are living with PF in the U.S.
Tens of thousands of Americans are living with PF, a progressive, debilitating lung disease with many causes but no known cure. Pulmonary fibrosis means scarring in the lungs that, over time, can destroy the normal lung and make it hard for oxygen to get into the blood.

86% of Americans don’t know the symptoms of PF
PF’s most common symptoms are a dry, persistent cough, fatigue and shortness of breath. As PF can share the same symptoms of many respiratory infections, including COVID-19 and the common cold, it is essential to note that when such symptoms become chronic, it can be an early warning sign of PF. Because symptoms are common to other illnesses, it can lead to later stage diagnoses.

More than 50,000 individuals are diagnosed with PF every year
However, the disease disproportionately affects the elderly — in fact, one in 200 over the age of 70 have idiopathic pulmonary fibrosis, the most common form of PF. Other at-risk populations include those with a history of smoking and a family history of interstitial lung disease, as well as those occupationally and environmentally exposed to mold and inorganic dust.

3,600 patient-years of data power the PFF Registry
The Pulmonary Fibrosis Foundation (PFF) is proud to spearhead the PFF Registry, the first nationwide study and biorepository supporting researchers in diagnosing, treating and potentially curing PF. Since its inception, the Registry has collected data from over 2,000 patients, amounting to an expansive 3,600 patient-years of data. Observational research like the PFF Registry complements basic, translational and clinical research to create a wholistic approach to disease management.

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12 biomarkers point to early detection and treatment of PF
To support the development of PF medication, the PFF has collaborated with Bristol Myers Squibb Company to launch the PROLIFIC Prognostic Lung Fibrosis Consortium. This program will allow pharmaceutical companies to utilize Registry data and biosamples to validate laboratory tests. Focusing on 12 specific biomarkers, PROLIFIC members will compare results across different clinical trials to identify those that predict disease progression and facilitate more rapid regulatory approval.

Further utilizing biomarkers for drug development is the PRECISIONS study (Prospective tReatment EffiCacy in IPF uSing genOtype for Nac Selection trial and Molecular Endophenotyping in Idiopathic Pulmonary Fibrosis and Interstitial Lung Diseases), the first-ever clinical trial to apply the principles of precision medicine to the treatment of idiopathic pulmonary fibrosis and diagnosis of interstitial lung diseases and pulmonary fibrosis.

32 PF ambassadors empower the PF community — and beyond
The PFF Ambassador program enlists PF patients, caregivers, lung transplant recipients and healthcare professionals as spokespersons to share their PF journeys. Sharing their stories is crucial in informing and enhancing disease awareness while further supporting critical research endeavors.

No cure yet, but many available treatments and support
While there is currently no cure for PF, clinicians have a number of ways to treat the disease, including the use of medications, oxygen therapy, non-medical treatments (such as exercise) and even lung transplantation.

In observance of Respiratory Care Week, I would also like to thank those in the respiratory therapist profession, as RTs play an essential role in empowering PF patients with the knowledge and skills to best manage the disease, improve quality of life and delay disease progression.

For more information about PF, please visit www.AboutPF.org. To download the full PFF 2020 National Awareness Survey report, please click here.

Written by

Dr. Gregory P. Cosgrove is Chief Medical Officer of the Pulmonary Fibrosis Foundation.

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