What does "Pulmonary Fibrosis" mean?
The word “pulmonary” means lung and the word “fibrosis” means scar tissue— similar to scars that form on the skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs.
Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, people can become progressively more short of breath.
The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can't pinpoint what's causing the problem. When a cause can't be found, the condition is termed idiopathic pulmonary fibrosis.
The lung damage caused by pulmonary fibrosis can't be repaired, but medications and therapies can help ease symptoms and improve quality of life. For some patients, a lung transplant might be appropriate.
Symptoms of Pulmonary Fibrosis:
The most common symptoms of pulmonary fibrosis are dry, persistent cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.
Due to a lack of oxygen in the blood, some people with pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to pulmonary fibrosis or idiopathic pulmonary fibrosis and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth.
How Do Doctors Recognize and Diagnose Pulmonary Fibrosis?
There are three consequences of pulmonary fibrosis. Doctors use these consequences to recognize that someone has PF:
1. Stiff Lungs. Scar tissue and inflammation make your lungs stiff. Stiff lungs are hard to stretch, so your breathing muscles have to work extra hard just to pull air in with each breath. Your brain senses this extra work, and it lets you know there’s a problem by triggering a feeling of breathlessness (or “shortness” of breath) while exerting yourself.
Also, stiff lungs hold less air (they shrink a bit). Doctors take advantage of this “shrinking” to diagnose and track the disease using breathing tests (called Pulmonary Function Tests) that measure how much air your lungs can hold. The more scar tissue your lungs have, the less air they will hold.
2. Low blood oxygen. Scar tissue blocks the movement of oxygen from the inside of your air sacs into your bloodstream. For many people living with pulmonary fibrosis, oxygen levels are only reduced a little bit while resting, but their oxygen levels drop quite a bit during activity. The brain can sense these low oxygen levels, triggering breathlessness.
Doctors will check your oxygen levels to see if they drop after walking, which could be a clue that PF might be present. Doctors also often prescribe oxygen to be used through a nasal cannula or a facemask during activity and sleep for those with PF. As pulmonary fibrosis progresses, oxygen may be needed 24 hours a day and flow rates may increase.
3. “Crackles" lung sounds. Your doctor may have told you that “crackles” were heard in your lungs. Crackles (also called “rales”) sound like Velcro being pulled apart.
They are heard in many lung diseases because any type of problem affecting the air sacs (such as PF, pneumonia, or a buildup of fluid in the lungs from heart failure) can cause crackles. Some people with pulmonary fibrosis don’t have crackles, but most do.
Can pulmonary fibrosis be reversed?
Unfortunately, lung damage due to pulmonary fibrosis is permanent (not reversible). Getting diagnosed and starting treatment as early as possible may help your lungs work better, longer.
How is pulmonary fibrosis treated?
Most pulmonary fibrosis treatments focus on easing symptoms and improving your quality of life.
Your provider may recommend one or more treatments:
• Medication: Two medications — pirfenidone (Esbriet®) and nintedanib (OFEV®) —may slow down lung scarring. These medications can help preserve lung function.
• Oxygen therapy: Giving your body extra oxygen helps you breathe more easily. It may also increase your energy and strength.
• Pulmonary rehabilitation: Staying active in this special exercise program may improve how much (or how easily) you can do everyday tasks or activities.
• Lung transplant: A lung transplant replaces one or both diseased lungs with a healthy lung (or lungs) from a donor. It offers the potential to improve your health and quality of life. A lung transplant is major surgery, and not everyone is a candidate. Ask your provider if you may be eligible for a lung transplant.
Can pulmonary fibrosis be cured?
No cure for pulmonary fibrosis exists today. But researchers around the world are working to change that.