I am an ambassador for the Pulmonary Fibrosis Foundation (PFF) and a patient advocate for pulmonary fibrosis.
As someone living with pulmonary fibrosis (PF), it can be frustrating to see how unknown and misunderstood the disease really is. This lack of awareness caused me to go undiagnosed for years. I’m hopeful that sharing my story will raise awareness about symptoms, treatments, and more.
An Unexpected Diagnosis
My story begins with a fun deep-water snorkeling outing that turned scary when I suddenly couldn’t get air into my lungs. As an experienced swimmer, it was very strange when I felt so winded that I couldn’t even call for help. Fortunately, the captain noticed my distress and got people to help me back into the boat.
My doctors initially ascribed the breathlessness to a heart condition, which allowed my PF to go undiagnosed for three years. In 2017, I was finally diagnosed with idiopathic pulmonary fibrosis (IPF), a type of PF without a known cause. Today, I am committed to increasing awareness of the disease and its early symptoms so that others will be diagnosed earlier.
What Exactly Is Pulmonary Fibrosis?
PF is a progressive and debilitating disease that, over time, leads to scarring of the lungs. The disease has no known cure.
While more than 200,000 Americans are living with PF, awareness remains alarmingly low. In fact, a vast majority (86%) of Americans do not know the symptoms of PF, according to a recent survey from the Pulmonary Fibrosis Foundation (PFF).
Symptoms and Risk Factors
The three most common symptoms are:
- shortness of breath
- dry, persistent cough
Symptoms can appear mild or absent early in the disease stage, and they mimic the symptoms of many other illnesses, such as the common cold. This means that it often takes a long time to diagnose the disease.
Because it is rare, many physicians may not see a case in a year or even longer. Even when noticeable on a chest X-ray, it may go undetected because the physician is looking for something else. In my case, nearly three years passed from the onset of early symptoms until an actual diagnosis.
Unfortunately, I chose to ignore the extreme shortness of breath, a dangerous thing to do. All of us must be proactive with our doctors.
Common risk factors include those over the age of 60, current or past smokers, and those with a family history of interstitial lung disease.
As always, when researching a medical condition, take what you read with a grain of salt. When doing my research online, I was told life expectancy was two to five years. No one can predict exactly how long a person with PF will live. There are people with PF who live much longer than five years, and, unfortunately, those who don’t live long after a late-stage diagnosis. Everyone is different, and there are some strategies that can make your life better.
While it is true that there is no known cure for PF, there are a number of helpful treatments.
- Supportive care treatments such as supplemental oxygen, medication, and pulmonary rehabilitation can lessen symptoms of cough, shortness of breath, or fatigue.
- For some types of PF, there are medications called "antifibrotics" that can slow down disease progression by about 50% on average.
- Lung transplantation is also a treatment option for some people with advanced PF and is considered on a case-by-case basis.
While learning to live with IPF is challenging, joining a support group helps provide emotional relief and valuable information from others also on this journey.
How to Advocate for Yourself and Others
Take notes about your symptoms, prepare for your appointments, and ask questions. AboutPF.org features helpful resources to assist with finding a specialist and identifying symptoms and risk factors, including the PFF’s Care Center Network and a downloadable checklist of symptoms and risk factors patients can share with their doctor.
Every September is Pulmonary Fibrosis Awareness Month (PFAM) to promote awareness of PF through events and education. Increasing understanding of this unsuspected disease will help drive earlier diagnoses, advocate for needed research, and ultimately find a cure. It is important to drive conversations around symptoms, risk factors, treatments, and more, so patients can start conversations with their doctors.
This content is accurate and true to the best of the author’s knowledge and does not substitute for diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed health professional. Drugs, supplements, and natural remedies may have dangerous side effects. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.
Cynthia Zirkwitz from Vancouver Island, Canada on September 14, 2020:
Very good information and a well-written article.