Pulmonary fibrosis (PF) is one of a family of related diseases called interstitial lung diseases that can result in lung scarring. As the lung tissue becomes scarred, it interferes with a person’s ability to breathe. In some cases, the cause of pulmonary fibrosis can be found. But most cases of pulmonary fibrosis have no known cause. These cases are called idiopathic pulmonary fibrosis (IPF).
Pulmonary fibrosis can develop slowly or quickly. In some people, the disease stays the same for years. Usually, a person’s breathing symptoms become worse over time. A person with pulmonary fibrosis eventually may be short of breath even at rest. The anti-arrhythmic drug amiodarone (Brand names Pacerone and Cordarone) has been linked to Pulmonary fibrosis along with a number of other dangerous side effects.
What is pulmonary fibrosis?
Pulmonary fibrosis (PF) is a disease marked by scarring of the tissue inside and between the air sacs in the lungs. When the scar forms, the tissue becomes stiff and thicker. This makes it harder for oxygen to pass through the walls of the air sac into the bloodstream. Once the lung tissue becomes scarred, the damage cannot be reversed. In most cases, there is no known cause for the disease. In these cases, it is called idiopathic pulmonary fibrosis or IPF.
Am I at risk for pulmonary fibrosis?
The list of substances and conditions that can lead to pulmonary fibrosis is long, and includes age, environmental and occupational exposures, including tobacco smoke, autoimmune disorders, and certain medications. Idiopathic pulmonary fibrosis sometimes runs in families, so a genetic component is suspected. Talk to your doctor about your work and health history, and be sure to mention if you have family members with IPF.
What are the symptoms of pulmonary fibrosis?
The symptoms of pulmonary fibrosis can be similar to the symptoms of other lung diseases and can include shortness of breath, cough, shallow breathing, weight loss, fatigue and aching. Some people with PF develop widening and rounding of the tips of the fingers or toes known as clubbing. If you have any of these symptoms, talk to your doctor who will conduct tests to determine if you have pulmonary fibrosis.
How can I have a good quality of life with pulmonary fibrosis?
Ask your doctor if you are eligible for pulmonary rehabilitation. Pulmonary rehabilitation is a program of education and exercise classes that teach you about your lungs and your disease, and how to exercise and be more active with less shortness of breath. The classes take place in a group setting, giving you the chance to meet others with your condition, and both give and receive support. Practicing breathing exercises may also help. Work with your doctor to brainstorm other ways you can maintain your quality of life.
Am I eligible for any clinical trials?
Clinical trials are being conducted to better understand how pulmonary fibrosis develops and to advance the treatments available. Clinical trials may give you access to new types of treatment being studied. Your doctor can help determine if enrolling in a clinical trial is the right choice for you.
What to Expect
Every person who is diagnosed with pulmonary fibrosis has a unique experience with the disease. Some people stay in stable condition for years, while others may experience rapidly-worsening symptoms and overall health. For many, it is somewhere in between. Although no one can predict your prognosis for certain, you can help yourself by maintaining a healthy lifestyle and working closely with your care team.
Managing the Disease
If you have pulmonary fibrosis there are steps you can take to stay healthy.
The Lung Association recommends patients join their Living with Pulmonary Fibrosis and caregivers join our Caring for Pulmonary Fibrosis online support communities to connect with others facing this disease. You can also call the Lung Association’s Lung Helpline at 1-800-LUNG-USA to talk to a trained respiratory professional who can answer your questions and connect you with support.