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What is Pulmonary Fibrosis?

Pulmonary fibrosis (PF) is a family of more than 200 interstitial lung disease (ILDs) that cause inflammation and/or scarring of the lungs.

About 30,000 Canadians live with PF. Approximately half of this group of people have Idiopathic Pulmonary Fibrosis (IPF), meaning the cause is uncertain or unknown.

Understanding Pulmonary Fibrosis

The interstitium is the part of the lung that holds all of the structures together like scaffolding on a building.
If we zoom in, we can see the airways. At the end of these airways are air sacks called alveoli.
In healthy lungs, the oxygen that you breathe in goes into the air sacks, crosses the interstitium and diffuses into your blood vessels to deliver oxygen to your body.
Carbon dioxide – our waste gas – diffuses out of the blood into the alveoli, and we breath it out.
Oxygen needs to cross the interstitium to get into the blood stream.
When you have pulmonary fibrosis there is usually inflammation and scarring of this interstitium. It becomes thickened and impairs the flow of oxygen into the body.

What causes pulmonary fibrosis?

Pinpointing the cause an individual case of PF can be challenging. Known causes include: occupational exposures to asbestos and inorganic dusts, organic compounds (mold, bacteria…) some medications, autoimmune diseases such as scleroderma or rheumatoid arthritis, chemo or radiation therapy. Smoking is not a cause, but it is a known risk factor. Often, there is no identifiable cause.

What are the symptoms of PF?

Generally, a feeling of shortness of breath or breathlessness upon exertion is an early sign of pulmonary fibrosis, sometimes accompanied by a persistent cough. Because symptoms can develop very gradually, it can take years before other common respiratory problems can be ruled out.

What to expect as PF progresses

Pulmonary fibrosis is a chronic disease. It behaves differently for each individual. However, one can typically expect that once the fibrosis scars the lung, that piece of the lung is permanently damaged. Ultimately many people with the disease become candidates for a lung transplant. Medication, good nutrition and exercise and emotional support can go a long way towards improving the quality of life of people living with the disease.

Healthy Lung VS Lung with Pulmonary Fibrosis

More on What is Pulmonary Fibrosis

CPFF Presents Documentary

CPFF Documentary – Breathless for Change

This documentary explores the courage and resilience of people living with PF and uncovers the barriers to accessing equitable healthcare for treatment and disease management.

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