Pulmonary Fibrosis Awareness

This is some information regarding Pulmonary Fibrosis and what it means.

Pulmonary Fibrosis Awareness

Lung Diseases

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 asthmaCOPDand 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 sarcoidosisare 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

The most common lung diseases include:

Obstructive Lung Disease

Obstructive lung disease, also called COPD or chronic obstructive pulmonary disease, affects the airways and air sacs (alveoli) of the lungs. The airways narrow or become blocked, decreasing the amount of air exhaled out of the lungs. People with obstructive lung disease may feel like they are trying to breathe out through a straw. Over time, the lungs may get bigger because the air gets trapped.

Symptoms of COPD include shortness of breath, chest tightness, and increased mucus, wheezing and coughing.

Treatments may include medicines, inhalers, oxygen use, breathing retraining, exercise, and surgery or lung transplant

COPD is a lung disease that includes chronic bronchitis and emphysema.

  • In chronic bronchitis, your airways become swollen and can be filled with mucus, which can make it hard for you to breathe.
  • In emphysema, the air sacs (alveoli) in your lungs are damaged which can make it hard for you to breathe.

In 80-90% of cases, it is caused by smoking. Other causes of COPD can include:

  • genetic reasons (alpha-1 antitrypsin deficiency)
  • occupational dusts and chemicals
  • second hand smoke
  • frequent lung infections as a child
  • wood smoke and other biomass (animal dung, crop residues) fuel used for cooking.

Obstructive lung diseases include:

Alpha-1 antitrypsin deficiency – an air sac disease passed down through families that may cause lung and liver disease. People with this disease can develop emphysema.

Asthma – lung irritants and allergens cause the airways to swell, narrow and tighten.

Bronchiectasis – damage, scarring and widening of the large airways caused by recurring swelling or infection of the airways. People with this disease are at risk for frequent lung infections.

Bronchiolitis obliterans syndrome – damaged and inflamed airways from chemical particles, lung infections or inflammation in lung transplant patients. This leads to scarring that blocks the airways in the lungs.

Chronic bronchitis – frequent infections that cause inflamed airways, increased mucus, shortness of breath, wheezing and chest tightness. Treatment may include antibiotics, steroids and oxygen use. Chronic bronchitis means that you have had these episodes a few times a year for 2 years or more. The main cause of chronic bronchitis is smoking.

Cystic fibrosis – a disease passed down through families that causes thick, sticky mucus to build up in the lungs, digestive tract and other areas of the body.

Emphysema – the air sacs lose their elasticity and become overinflated. This causes air trapping, shortness of breath and a decrease in gas exchange. The main cause of emphysema is smoking.

Restrictive Lung Disease

Restrictive lung disease, also called interstitial lung disease, 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.

Other conditions, such as obesity and scoliosis or side curve to the spine, may also prevent the lungs from expanding fully and be considered a restrictive lung disease.

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

Treatments may include medicines to decrease swelling or the progression of the disease, breathing retraining, exercise, oxygen use, and surgery or lung transplant.

Restrictive lung diseases include:

Autoimmune connective tissue disorders may affect the connective tissue in the body and the lungs, causing inflammation, swelling, hardening and scarring.

Rheumatoid arthritis – a disorder that causes inflammation of the body’s joints because of increased immune cell production. About 1 in 10 people with rheumatoid arthritis develop restrictive lung disease. Scarring of the lungs occurs from the body’s overactive immune system attacking the lungs.

Scleroderma – immune cells produce more collagen, causing the body’s skin to harden or scar. One type of scleroderma, called systemic sclerosis, can cause hardening or scarring in many parts of the body, including the lungs.

SjÖgren’s syndrome – autoimmune disease of unknown cause that causes dryness of the eyes, mouth and other body parts. Pulmonary symptoms act like interstitial lung disease, causing swelling and inflammation.

Bronchiolitis obliterans with organizing pneumonia (BOOP) / Cryptogenic organizing pneumonia (COP) – a rare condition where the small airways (bronchioles) and air sacs (alveoli) become inflamed and blocked with connective tissue.

Hypersensitivity pneumonitis – a disease that causes inflammation of the alveoli in the lungs due to an allergic reaction to dust, fungus, molds or chemicals. Exposure comes most often from the person’s occupation or hobbies. The disease causes symptoms that are similar to the flu.

  • Bird fancier’s lung / pigeon breeder’s disease – from inhaling bird feathers or droppings.
  • Farmer’s lung – from inhaling mold that grows on hay, straw or grain.

Pneumoconiosis – a disease caused by inhaling workplace dust. The disease causes coughing and shortness of breath. It may lead to pulmonary fibrosis.

  • Asbestosis – from inhaling asbestos fibers.
  • Black lung disease – from inhaling coal dust (coal miners).
  • Siderosis – from inhaling iron from mines or welding fumes.
  • Silicosis – from inhaling silica dust.

Pulmonary fibrosis – lung tissue becomes scarred overtime, making it hard to breathe. Scarring may occur from the environment, chemotherapy, radiation, certain medicines, autoimmune disease or unknown cause.

Sarcoidosis – disease of unknown cause where abnormal growths, called granulomas, grow in the tissue of the lungs, skin or lymph nodes, causing inflammation. The disease may progress into pulmonary fibrosis or bronchiectasis.

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.

 Interstitial Lung Disease:

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

Some of the types of interstitial lung disease include:

Interstitial pneumonia: Bacteria, viruses, or fungi may infect the interstitium of the lung. A bacterium called Mycoplasma pneumonia is the most common cause.

Idiopathic pulmonary fibrosis: A chronic, progressive form of fibrosis (scarring) of the interstitium. Its cause is unknown.

Nonspecific interstitial pneumonitis: Interstitial lung disease that’s often present with autoimmune conditions (such as rheumatoid arthritis or scleroderma).

What is 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. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis, and these are treated differently. It is important to work with your doctor to determine if you have IPF or another form of interstitial lung disease.

What is 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.  Unlike COPD which is an obstructive lung disease that doesn’t allow oxygen to enter the lungs because of blockage therefore oxygen can’t enter the bloodstream.  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 PF?

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 PF?

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 can also damage the lung tissue

  • 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 of IPF.

Diagnosis

As patients with PF experience symptoms and also have an underlying pattern of scarring similar to other lung disorders, PF can be very difficult to diagnose.  To diagnose PF, a doctor will take a complete medical history and then order one or more of the following diagnostic procedures.

  • Chest Imaging (X-Ray)
  • CT Scan
  • Pulmonary Function Test
  • Exercise Test
  • Six Minute Walk Test
  • Bronchoscopy
  • Lung Biopsy

Treatments

Unfortunately there is no cure for PF and currently there are only 2 drugs available to slow the progression for early stages of PF.

Pulmonary Rehabilitation

The focus on physical exercise and breathing techniques.

Lung Transplant

Currently a lung transplant is the only effective treatment for PF.

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