What is Pulmonary Fibrosis (PF)?

A short explanation of what Pulmonary Fibrosis is?

Lung disease is any problem in the lungs that prevents the lungs from working properly. There are three main types of lung disease:
1. Airway diseases – These diseases affect the tubes (airways) that carry oxygen and other gases into and out of the lungs. They usually cause a narrowing or blockage of the airways. Airway diseases include asthma, COPD and bronchiectasis. People with airway diseases often say they feel as if they’re “trying to breathe out through a straw.”
2. Lung tissue diseases – These diseases affect the structure of the lung tissue. Scarring or inflammation of the tissue makes the lungs unable to expand fully (restrictive lung disease). This makes it hard for the lungs to take in oxygen and release carbon dioxide. People with this type of lung disorder often say they feel as if they are “wearing a too-tight sweater or vest.” As a result, they can’t breathe deeply. Pulmonary fibrosis and sarcoidosis are examples of lung tissue disease.
3. Lung circulation diseases – These diseases affect the blood vessels in the lungs. They are caused by clotting, scarring, or inflammation of the blood vessels. They affect the ability of the lungs to take up oxygen and release carbon dioxide. These diseases may also affect heart function. An example of a lung circulation disease is pulmonary hypertension.

Approximate Number of Deaths per Year in Canada by Disease
Heart Disease 49,000
Prostate Cancer 29,480
Lung Cancer 20,500
From Complications of Parkinson’s 8,760
Pulmonary Fibrosis 5,000
Breast Cancer 5,000
From Complications of ALS 600
Directly attributed to MS 145
Cystic Fibrosis 60

Restrictive Lung Disease
Restrictive lung disease, also called Interstitial Lung Disease (ILD), may affect lung tissue by causing scarring, inflammation (swelling) or thickening of lung tissue. This makes the lungs unable to expand fully. It becomes hard for the lungs to take in oxygen and release carbon monoxide. Oxygen and carbon dioxide molecules have a hard time passing through the lung tissue to enter or exit the blood stream.

Symptoms of restrictive lung disease include shortness of breath, fatigue especially with activity, chest tightening and increased mucus.

Interstitial Lung Disease:
Interstitial lung disease is a general category that includes many different lung conditions. All interstitial lung diseases affect the interstitium, a part of the lungs’ anatomic structure.

The interstitium is a lace-like network of tissue that extends throughout both lungs. The interstitium provides support to the lungs’ microscopic air sacs (alveoli). Tiny blood vessels travel through the interstitium, allowing gas exchange between blood and the air in the lungs. Normally, the interstitium is so thin it can’t be seen on chest X-rays or CT scans.

All forms of interstitial lung disease cause thickening of the interstitium. The thickening can be due to inflammation, scarring, or extra fluid (edema). Some forms of interstitial lung disease are short-lived; others are chronic and irreversible.

Pulmonary Fibrosis
Pulmonary (Lungs) Fibrosis (Scarring) is part of an overlaying lung disease called “Interstitial Lung Disease” which has been divided into 5 main groupings: Drug-Induced Diseases; Exposures; Genetic / Inherited; Systemic Diseases; Idiopathic.

Pulmonary Fibrosis (PF) is a restrictive lung disease that allows oxygen to enter the lungs but not enter the bloodstream due to the lung tissues becoming scarred and over time thickens and becomes more widespread, resulting in shortness of breath and vital organs being deprived of the necessary oxygen to survive. PF affects an estimated 30,000 Canadians with about 6,000 new cases each year in Canada. Idiopathic (we don’t know) Pulmonary Fibrosis (IPF) is the most common form of PF lung disease and as many as 5,000 people pass away each year.

There is no known cure for PF and is often fatal within 3-5 years of diagnosis, and is more common in men than women.

What are the symptoms of Pulmonary Fibrosis?
Symptoms of PF usually have a gradual onset and may include:
• Shortness of breath, particularly during or after physical activity
• Spasmodic, dry cough
• Gradual, unintended weight loss or weight gain
• Fatigue and weakness
• Chest discomfort
• Clubbing, or enlargement, of the ends of the fingers due to a buildup of tissue.

What causes Pulmonary Fibrosis?
PF can be caused by many things, some of the known causes of PF include:
• Job-related and environmental factors
Being exposed to toxins and pollutants over the long term. These toxins and pollutants may include silica dust, asbestos fibers, grain dust, and some bird and animal droppings.
• Radiation treatments
For lung or breast cancer may also damage the lungs
• Smoking
• Gastroesophageal Reflux Disease (GERD)
• Certain medicines
Medicines to treat cancer, heart medicines and antibiotics can cause lung damage
• Certain medical conditions
Tuberculosis, pneumonia, lupus, rheumatoid arthritis, sarcoidosis, and scleroderma
• Genetics

Once these have been ruled out there is still a very large population of patients who will never find the “causation” of their disease, hence the large category (200+) of Idiopathic Pulmonary Fibrosis (IPF).

Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, the scarring gets worst and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen.

IPF is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels.

Treatments
Unfortunately there is no cure for PF and IPF.

Lung Transplant
Currently a lung transplant is the only effective treatment for PF and IPF.
 

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