One in 20 people will live with a rare disease at some point in their life*. Rare Disease Day, celebrated annually on February 28, provides an opportunity to raise awareness for diseases that are not well known, such as pulmonary fibrosis (PF). The Pulmonary Fibrosis Foundation (PFF) is working to increase understanding of PF to drive home the importance of early diagnosis to help improve the quality of life for those living with this disease.
Pulmonary Fibrosis: A Rare Lung Disease
PF is a progressive, debilitating lung disease that causes scarring of the lung tissue and affects more than 200,000 Americans. PF is more likely to occur in those who are 60 years and older and in individuals with a history of smoking. While over 50,000 new cases are diagnosed annually, the disease remains largely unknown. In fact, nearly 9 in 10 Americans do not know the symptoms, which include shortness of breath, fatigue and a dry, chronic cough, according to the PFF**. These symptoms are common, are often attributed to other causes and can appear mild in the early stages – all of which can lead to late-stage diagnoses.
Early Diagnosis is Key
Since PF is a debilitating, life-threatening disease, a precise and early diagnosis is crucial. However, for PF patients, the road to diagnosis can be long and difficult. Too often, the disease goes misdiagnosed or takes months to years to identify. And, many patients have never heard of the disease until they get the diagnosis. Pinpointing symptoms and taking action early by starting conversations with your doctor are key to maximizing treatment options and improving patient outcomes.
To help improve patient diagnosis and care, the PFF launched the public awareness campaign “Pinpoint PF” to help patients pinpoint risk factors and symptoms early. I encourage at-risk patients and caregivers to visit AboutPF.org to learn more about the disease. There, patients will find a PF Risk List of symptoms and risk factors to take to their next doctor’s appointment. To help facilitate a clear diagnosis, it’s important to ask your doctor to listen for crackles in your lungs, which can be indicative of PF, and to ask about pulmonary function and/or high-resolution computed tomography (HRCT) tests.
AboutPF.org also offers a way to find a local pulmonologist through the PFF Care Center Network, which includes medical centers with experience in the diagnosis and comprehensive treatment of PF. A knowledgeable team of PF experts can help make sure you receive an accurate diagnosis and the most up-to-date treatments and symptom management recommendations, which may include oxygen therapy, pulmonary rehabilitation, the use of medications and lung transplantation.
PF Advocacy Continues to Drive Education and Awareness
On March 10, PFF advocates will meet with legislators and congressional staff in a virtual version of Hill Day to raise awareness amongst decision-makers about PF, its impact on patients' lives and the need for expanded federally-funded research. Hill Day is an opportunity for patients and their caregivers to advocate for legislation that will improve the lives of people living with PF. It is important we continue to advocate for legislation to lead research for a cure so people with PF will live longer, healthier lives. The PF community is invited to apply to participate in Hill Day 2021, here.
You can become a PFF advocate too! Watch below to learn how:
Spread the Word
The PFF is striving to provide patients, caregivers, family members and healthcare providers with the resources necessary to more fully understand PF. You can access these resources here.
By sharing useful information and providing helpful resources, the visibility of PF will grow and drive home the importance of early diagnosis, which will help to improve the quality of life for patients. Help spread the word about PF to your friends and loved ones by visiting https://aboutpf.org/#share.
At the PFF, we will continue our efforts to combat this deadly disease, support individuals who are living with PF and eventually find a cure. Together, we will make a difference by letting the world know about pulmonary fibrosis.
**PFF National Consumer Survey Report: https://www.pulmonaryfibrosis.org/docs/default-source/marketing-brochures/pff-national-consumer-survey-report---for-review-updated-2-19-2020.pdf?sfvrsn=c479c8d_0
About the Creator
Sr. Medical Advisor for Research/Health Care Quality for the Pulmonary Fibrosis Foundation; Assoc. Professor of Medicine in the Division of Pulmonary Sciences & Critical Care and Dir. of the Interstitial Lung Disease Program at CU Anschutz.