What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a serious life-long chronic lung disease that causes lung scarring, making it difficult to breathe. As the lung tissue becomes scarred and thickened, it becomes harder for the lungs to take in oxygen. Symptoms may develop rapidly or may take years to develop. Symptoms usually include difficulty breathing and a cough. There is no cure for pulmonary fibrosis, and it is considered a terminal illness. Medications help manage symptoms and slow the progress of the disease.

Causes

Most cases of pulmonary fibrosis are due to an unknown cause, which is called idiopathic pulmonary fibrosis.

Other cases may be caused by factors including:

• Smoking
• Genetics (runs in your family)
• Environmental toxins, like asbestos or other hazardous or harmful chemicals or pollutants
• Autoimmune diseases
• Certain medications
• Radiation therapy

Being an older male, over the age of 50, may also put you at a greater risk of developing pulmonary fibrosis.

Symptoms

Symptoms of pulmonary fibrosis depend on their severity and how quickly the disease progresses.

Symptoms may include:

• Difficulties breathing
• Shortness of breath (often with exertion)
• Dry cough
• Fatigue
• Aching muscles and joints
• Unintended weight loss
• Clubbing of the fingers and toes (widening and rounding of the tips)

In idiopathic pulmonary fibrosis, if shortness of breath suddenly becomes worse over a few days or weeks, it can become life-threatening. This is called an acute exacerbation, and it can be caused by another underlying condition or illness. Often, the cause is unknown.

What Are the Stages of Pulmonary Fibrosis?

Pulmonary fibrosis progresses at varying rates between individuals. It is a chronic and progressive, life-long terminal disease. For some people, it may take weeks to months for symptoms to become severe, while for others, it may take years to worsen and become severe.

People with idiopathic pulmonary fibrosis sometimes experience what is called an acute exacerbation. This is when shortness of breath suddenly becomes worse over a few days or weeks, and it can be life-threatening.

As a progressive disease, each time there is a flare-up, it will make it harder to breathe, and lung functioning will not return to what it was prior to the flare-up. This means each time there is a flare-up, a new baseline of lung functioning is created, and each flare-up will continue to degrade a person’s ability to breathe.

Treatments, including certain medications, only help to manage symptoms and slow the progression of the disease.

Diagnosing

Pulmonary fibrosis is typically diagnosed using several diagnostic methods, in part because symptoms of PF are similar to other conditions. Your doctor will conduct a physical examination, take a thorough medical history, and ask about your symptoms and the environments where you spend most of your time.

During the physical examination, your doctor will likely use a stethoscope to listen to your lungs, checking for any crackling sounds at the base of your lungs. If pulmonary fibrosis is suspected, your doctor will most likely recommend the following tests:

• Blood tests. These tests are ordered to rule out other medical conditions or provide doctors with insight into the effects of the disease progression.
• Imaging tests. A chest X-ray or CT scan may be able to rule out other lung conditions or confirm pulmonary fibrosis by seeing the lung scarring.
• Breathing tests. Pulmonary functioning tests measure how well your lungs work and lung capacity.
• Oxygen desaturation tests. This test measures the oxygen levels in your blood by attaching a probe to your finger or forehead while you walk for 6 minutes.
• Biopsy. Sometimes a lung biopsy is performed to confirm a diagnosis of pulmonary fibrosis. A lung biopsy is performed by making a small incision in the ribs and collecting a sample of lung tissue.

Treatment

Treatment for pulmonary fibrosis centers around symptom management and slowing disease progression. Unfortunately, there is no cure for pulmonary fibrosis. The lung damage caused by the disease is not reversible. Treatment will depend on disease progression and symptom severity.

Common treatments include:

• Medication. Pirfenidone (Esbriet®) and nintedanib (OFEV®) are two specific medications that may help to slow down lung scarring and preserve lung function.
• Oxygen therapy. Supplying your body with more oxygen may help to improve breathing and increase energy and strength.
• Pulmonary rehabilitation. This program helps to improve how much or how easily one can perform everyday tasks or activities.
• Lung transplant. A lung transplant is a major surgery and not every person is a good candidate for it. During this procedure, one or both diseased lungs are replaced by a healthy lung or lungs. If the transplant is successful, it has the potential to significantly improve the quality of life.

Living with Pulmonary Fibrosis

Pulmonary fibrosis is a chronic, life-long terminal disease. Although there is no cure, there are treatments that may help slow disease progression and manage symptoms. Early diagnosis and disease management are key to helping maintain a quality of life and slow the disease progression.

If you are experiencing symptoms of pulmonary fibrosis, please contact your healthcare provider. Find a Baptist Health doctor today!