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Pulmonary Fibrosis: What You Need to Know

Pulmonary Fibrosis: What You Need to Know

Apria Editorial |

If you have been diagnosed with pulmonary fibrosis, you may have many questions about this condition and how it affects your health. Pulmonary fibrosis is a lung disease that causes scarring and thickening of the lung tissue, making it harder for oxygen to pass into your bloodstream. This can lead to shortness of breath, fatigue, coughing, and other symptoms that can interfere with your daily activities and quality of life.

In this article, we will explain what causes pulmonary fibrosis, how it is diagnosed and treated, and what you can do to cope with this chronic condition.

What causes pulmonary fibrosis?

Pulmonary fibrosis can have many different causes, but in many cases, the exact cause is unknown. This is called idiopathic pulmonary fibrosis (IPF). Some of the possible factors that can damage the lung tissue and lead to scarring include:

  • Long-term exposure to harmful substances, such as silica dust, asbestos fibers, coal dust, grain dust, bird, and animal droppings, etc.
  • Certain medications, such as chemotherapy drugs, heart medications, anti-inflammatory drugs, etc.
  • Radiation therapy for lung or breast cancer
  • Genetic factors that make you more susceptible to lung damage
  • Medical conditions that affect the immune system or connective tissue, such as dermatomyositis, sarcoidosis, scleroderma, etc.

The scarring process in pulmonary fibrosis is irreversible and progressive, meaning that it worsens over time. However, the rate of progression can vary from person to person. Some people may experience rapid worsening of their symptoms (acute exacerbation), while others may have a slower decline in lung function.

How is pulmonary fibrosis diagnosed?

To diagnose pulmonary fibrosis, your doctor will review your medical and family history, ask you about your symptoms and exposure to potential lung irritants, and perform a physical exam. Your doctor will also order some tests to confirm the diagnosis and assess the severity of your condition. These tests may include:

  1. Chest X-ray: This test shows images of your chest and can reveal the scar tissue typical of pulmonary fibrosis. It can also be used to monitor the course of the disease and treatment.
  2. CT scan: This test uses a computer to combine X-ray images taken from different angles to produce detailed cross-sectional images of your lungs. It can help determine the extent and pattern of lung damage caused by pulmonary fibrosis.
  3. Echocardiogram: This test uses sound waves to create images of your heart. It can measure the pressure on the right side of your heart, which can increase due to pulmonary fibrosis.
  4. Lung function tests: These tests measure how much air your lungs can hold and how quickly you can move air in and out of your lungs. They can also measure the oxygen level in your blood. Some common lung function tests are spirometry, pulse oximetry, exercise stress test, and arterial blood gas test.
  5. Lung biopsy: This test involves removing a small sample of lung tissue for microscopic examination. It can help confirm the diagnosis of pulmonary fibrosis or rule out other conditions. The tissue sample can be obtained by bronchoscopy (a flexible tube inserted through your nose or mouth into your lungs) or by surgery.

How is pulmonary fibrosis treated?

There is no cure for pulmonary fibrosis, but there are treatments that can help ease your symptoms, improve your quality of life, and slow down the progression of the disease. The treatment options depend on the cause and severity of your condition, as well as your overall health and preferences. Some of the common treatments are:

Medications: There are two drugs approved by the FDA for treating IPF: nintedanib and pirfenidone. These drugs work by slowing down the scarring process in the lungs. They may have some side effects, such as nausea, diarrhea, liver problems, etc., so you need to take them under close supervision by your doctor. Other medications that may be prescribed for pulmonary fibrosis include corticosteroids, antibiotics, antacids and anti-inflammatory drugs.

Oxygen therapy involves using a device that delivers oxygen through a nasal cannula or a mask. It can help improve your breathing and maintain adequate oxygen levels in your blood. You may need oxygen therapy at home or when you exercise or travel.

Learn more about oxygen therapy.

Pulmonary rehabilitation: This is a program that includes exercise training, education, counseling, and support for people with chronic lung diseases. It can help you improve your physical fitness, manage your symptoms, cope with stress and anxiety, and enhance your quality of life.

Learn more about pulmonary rehab

Lung transplant: This is a surgery that involves replacing one or both of your diseased lungs with healthy ones from a donor. It may be an option for people with severe pulmonary fibrosis who do not respond to other treatments. However, it has many risks and complications, such as infection, rejection, bleeding, etc., and requires lifelong medication and follow-up care.

How can you cope with pulmonary fibrosis?

Living with pulmonary fibrosis can be challenging, but there are some steps you can take to cope better with your condition and improve your well-being. These include:

  1. Quitting smoking and avoiding exposure to secondhand smoke and other lung irritants
  2. Getting vaccinated for flu and pneumonia to prevent respiratory infections
  3. Eating a balanced diet that provides enough calories, protein, vitamins and minerals
  4. Drinking plenty of fluids to keep your mucus thin and easy to cough up
  5. Practicing breathing techniques that can help you breathe more efficiently and relax
  6. Joining a support group or online community where you can share your experiences and feelings with others who have pulmonary fibrosis
  7. Seeking professional help if you have signs of depression or anxiety, such as sadness, hopelessness, loss of interest, irritability, etc.

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Pulmonary fibrosis is a serious lung disease that can affect your ability to breathe and enjoy life. However, with proper diagnosis, treatment, and self-care, you can manage your symptoms, slow down the disease progression, and live a fulfilling life.


What is the life expectancy of a person with pulmonary fibrosis?
The life expectancy for someone with pulmonary fibrosis can vary widely, and it is challenging to predict for any individual due to factors such as age, overall health, and how advanced the disease is at diagnosis. Generally, the median survival for someone with idiopathic pulmonary fibrosis (IPF), a common form of the disease, is estimated to be between 3 to 5 years after diagnosis. However, it's important to note that some people live much longer, especially with early diagnosis and proper management of the condition.

What is the main cause of pulmonary fibrosis?
Pulmonary fibrosis can be caused by a variety of factors, and often the exact cause is unknown, which is referred to as idiopathic pulmonary fibrosis (IPF). Known causes include long-term exposure to harmful substances like asbestos, silica dust, and coal dust, certain medications (including some chemotherapy drugs and heart medications), radiation therapy for cancers, genetic predisposition, and medical conditions affecting the immune system or connective tissues like sarcoidosis or scleroderma. However, in many cases, the cause remains unidentified.

Can you recover from pulmonary fibrosis?

Currently, there is no cure for pulmonary fibrosis, and the scarring of lung tissue caused by the disease is irreversible. Treatment focuses on managing symptoms, slowing the progression of the disease, and improving quality of life. Medications, oxygen therapy, pulmonary rehabilitation, and in some cases, a lung transplant, are among the available treatment options. While these treatments can help manage the condition, they do not reverse the damage already done to the lungs.

What are the four stages of pulmonary fibrosis?

Pulmonary fibrosis can be classified into four stages based on the severity of the disease, although this staging is not always used in clinical practice:

  1. Mild Stage: At this stage, symptoms may be barely noticeable or not present at all. Lung function tests might show minimal impairment.
  2. Moderate Stage: Symptoms such as shortness of breath and fatigue become more evident, especially during physical activity. Lung function tests may show a noticeable decline in capacity.
  3. Severe Stage: Symptoms are more pronounced even during rest, and daily activities are increasingly difficult. Lung function tests indicate significant impairment.
  4. Very Severe Stage: At this advanced stage, symptoms are constant and debilitating. Oxygen levels may be critically low even at rest, and there is a significant impact on quality of life. Advanced medical interventions might be necessary.

It's important to note that the progression of pulmonary fibrosis can vary greatly from person to person.

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