What is pulmonary fibrosis?

Pulmonary fibrosis simply means scarring in the lungs. The scarring in the lungs interferes with the lungs’ ability to exchange oxygen and carbon dioxide. This causes shortness of breath and coughing in most people. Pulmonary fibrosis can cause low oxygen in the bloodstream that can affect vital body organs and function. Once the lung has been damaged by scarring, there is no natural way for the body to repair it.

What causes pulmonary fibrosis?

You may never have heard of pulmonary fibrosis (PF) or interstitial lung disease (ILD) before now.  Interstitial lung diseases are relatively rare compared to chronic pulmonary disease (COPD), a much more common breathing-related ailment. 

Pulmonary fibrosis (PF) is a condition that isn’t just caused by just one disease. More than 200 different lung diseases, types of Interstitial Lung Disease (ILD), all can cause inflammation or scarring of the lungs. The scarring makes it harder for the lungs to extract oxygen and transfer it to the other organs in your body. Some pulmonary fibrosis gets worse with time, called progressive pulmonary fibrosis.

Pulmonary fibrosis may be caused by exposure to harmful chemicals or environmental irritants or by other diseases such as rheumatoid arthritis. For some patients, the cause cannot be determined. In this case, the diagnosis is called idiopathic.

There may be a genetic or hereditary component that increases the likelihood of some types of pulmonary fibrosis among close relatives. The research in this are is ongoing and the medical field has much to learn about this.

Available Therapies Can Help

While there is no cure available for PF or ILD currently, medications and treatment are available that can significantly slow the progression of lung fibrosis. The US Food and Drug Administration has approved two drugs to treat pulmonary fibrosis. And other therapies such as pulmonary rehabilitation and other medicines to manage symptoms can slow the disease progression, maintain your overall health, and improve your quality of life.

While you may have heard or read that the average life span for patients is 2 to 3 years after diagnosis, that prediction is based on old data and is not true for all of the fibrotic lung diseases. Many patients live much longer and not all diseases progress. Patients’ disease may progress rapidly or slowly. Medical research and clinical trials may offer more effective treatments soon. The availability of anti-fibrotic drugs has significantly changed the prognosis of people with pulmonary fibrosis. Two drugs are currently approved by the US FDA and the healthcare community advises patients to begin taking them early to slow the progression of pulmonary fibrosis.

A lung transplant is an option for some pulmonary fibrosis patients who qualify. A lung transplant is a major operation with risks and must be followed by long-term medications. And, there are not enough donor lungs for all patients. For that reason, hospitals establish criteria for selecting patients who will qualify to receive donated lungs. For those who are able to have it, a lung transplant is a life-changing option.

There is a lot of information on the web about pulmonary fibrosis, possible treatments and life expectancy. Be aware that some of the information you can find is outdated, inaccurate, and does not apply to all people with pulmonary fibrosis. The condition is complex and patients experience very different journeys. When you are looking for answers, be careful to rely on trusted, credible resources. We have selected the most trustworthy resources to share on our website.