There is no cure for pulmonary fibrosis, and in most cases the root cause remains unidentified. But following your healthcare team's guidelines will help you breathe easier and manage the condition.

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Of the numerous diseases that can affect your lungs, pulmonary fibrosis could be one of the most perplexing. Although physicians can sometimes pinpoint a cause of the disease, in most cases no underlying culprit is ever identified. And, since the disease manifests differently from person to person, it can be challenging to predict each patient’s life expectancy.

Still, one thing that is clear about pulmonary fibrosis—and just about any lung disease, for that matter—is that adhering to a healthy lifestyle and following the treatment regimen your healthcare team outlines for you may help slow the disease’s progression. It also will allow you to maximize your breathing and your quality of life.

What Is Pulmonary Fibrosis?

Pulmonary fibrosis occurs when the tissue inside and between the air sacs (alveoli) in the lungs becomes scarred. This scarring makes the tissue thick and stiff, inhibiting a person’s breathing ability. The disease affects about 140,000 Americans, and is most common among those ages 50 to 75, according to the American Lung Association.

Pulmonary fibrosis symptoms include shortness of breath (especially during physical exertion), a dry cough, unintentional weight loss, achiness in your muscles and joints, fatigue, and a widening/rounding of the fingertips and toes (clubbing).

The pulmonary fibrosis stages range from 1 to 4. Stage 1 pulmonary fibrosis includes the early symptoms of dry cough and shortness of breath. Stage 2 pulmonary fibrosis may require oxygen with activity. Stage 3 pulmonary fibrosis may require oxygen 24 hours a day. Stage 4 pulmonary fibrosis requires advanced, high flow oxygen at all times.

In most cases, the exact cause of the lung scarring is unknown—experts refer to this condition as idiopathic pulmonary fibrosis (IPF). However, several factors may contribute to pulmonary fibrosis: genetics; cigarette smoking; exposure to silica, coal dust (black lung), and other environmental pollutants; use of certain medications, including some chemotherapy drugs; radiation therapy to the chest; and some viral infections.

Additionally, scientists are studying a link between gastroesophageal reflux disease (GERD, or acid reflux) and IPF. About nine out of 10 people with IPF have GERD, according to the National Institutes of Health, and experts theorize that stomach acid regurgitated into the esophagus might be inhaled and, consequently, damage lung tissue.

Pulmonary Fibrosis Complications

The prognosis for patients with pulmonary fibrosis varies from person to person: Some may remain stable for several years, while others experience rapid development and worsening of the disease. Over time, breathing difficulties may intensify, to the extent that a patient will have trouble breathing even at rest.

As the disease advances, one or more complications may develop, including lung collapse, lung infections, blood clots in the lung, respiratory failure, and lung cancer. Moreover, the small arteries in your lungs can become compressed by growing scar tissue, causing pressure in the pulmonary arteries to increase and resulting in pulmonary hypertension. This disorder forces your heart’s right ventricle to work harder to push blood through the pulmonary arteries. As a result, the ventricle may weaken and lead to right-sided heart failure.

Pulmonary Fibrosis Treatment Options

Pulmonary fibrosis has no cure, but treatments are available to help manage the disease and ease symptoms. For patients with mild, moderate, or severe IPF, the U.S. Food and Drug Administration has approved two medications—nintedanib (Ofev) and pirfenidone (Esbriet, Pirfenex, Pirespa)—both of which may help slow the progression of the disease. For some forms of the disease, doctors may prescribe prednisone or other corticosteroids to quell inflammation, as well as the immune-suppressing drug azathioprine (Azasan®, Imuran®).

You also may require supplemental oxygen therapy, which can help you breathe easier and allow you to stay active. Some people use the oxygen occasionally, while others need it all the time and carry a portable oxygen canister.

Also, ask your doctor about taking part in pulmonary rehabilitation, a program that can help you learn more about pulmonary fibrosis, teach you how to stay active despite the disease, educate you on your oxygen needs at rest and during physical activity, and provide you with support and counseling to cope better with the disease.

Finally, if you’re diagnosed with pulmonary fibrosis, undergo an evaluation for a lung transplant, which can improve your quality of life and possibly prolong your life.

Do Your Part After a Pulmonary Fibrosis Prognosis

To stay as healthy as possible despite pulmonary fibrosis, it’s important to work with your healthcare team and take all your medications exactly as your doctor prescribes them. Notify a medical professional promptly if you experience symptoms or any unusual changes in the way you feel.

Also, follow these tips from the American Lung Association:

• If you smoke, talk to your healthcare team about ways to quit. Avoid exposure to secondhand smoke.
• Work with your healthcare providers to develop an exercise program to help you preserve your strength and lung function.
• Get a flu shot every year, and ask your physician if you should be vaccinated against pneumonia.
• Eat a healthy diet, and optimize your body weight.
• Consume smaller, more frequent meals to avoid stomach fullness, which can make it more difficult to breathe.
• Join a support group, such as the American Lung Association’s Better Breathers Clubs and Living With Pulmonary Fibrosis online support community. And, tell your doctor about any feelings of anxiety or depression you might have.