Pulmonary Fibrosis

What You Should Know About Pulmonary Fibrosis

Respiratory ailments can diminish our quality of life, and among the many diseases that can make our breathing difficult is pulmonary fibrosis. This rare disorder (fewer than 200,000 cases a year) can make breathing difficult due to lung scarring over time. We can give you all the facts about pulmonary fibrosis, its symptoms, and potential treatments for living with this disorder. 

What is pulmonary fibrosis? 

You may have heard the term pulmonary fibrosis, but do you know what it means? Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes scarred. This damaged tissue thickens, making breathing difficult since the lungs don't work correctly. As this condition worsens, you can become progressively shorter of breath. Unfortunately, the damage caused by pulmonary fibrosis is unrepairable, which can lead to the need for a lung transplant. Still, medications and therapies have been developed to ease some symptoms and improve quality of life.

Pulmonary fibrosis symptoms

The Mayo Clinic identifies symptoms of pulmonary fibrosis as: 

• Shortness of breath 
• Dry cough 
• Fatigue 
• Clubbing of the fingers or toes (widening and rounding of the tips) 
• Muscle and joint aches 
• Unexplained weight loss 

As with many other ailments, the symptoms and severity can vary from person to person. Some affected by pulmonary fibrosis can get ill quickly, while others experience moderate symptoms that worsen slowly over months or years. 

What causes pulmonary fibrosis

It would be convenient if there were a single cause of pulmonary fibrosis, but unfortunately, there isn't. There may even be cases that don't have a definitive reason. This is called idiopathic pulmonary fibrosis. The damage to the tissue around the air sacs (alveoli) makes passing oxygen into your bloodstream more difficult, and many factors can cause this. Some potential causes of pulmonary fibrosis can include: 

• Occupational/environmental factors 
• Radiation treatments 
• Medications 
• Medical conditions 

Long-term exposure to environmental factors such as silica dust, asbestos fibers, hard metal dust, coal, grain dust, inhalation of bird/animal droppings, and cigarette smoking could all be causes of pulmonary fibrosis. 

These can be just some of the causes of pulmonary fibrosis, and since there is no cure, complications from this disorder can be severe. 

Complications

• Pulmonary Hypertension: High blood pressure in your lungs due to small arteries and capillaries compressed by scar tissue. 
• Lung Cancer: Pulmonary fibrosis increases your risk of contracting lung cancer. 
• Respiratory Failure: Often, this is the last stage of chronic lung disease due to dangerously low oxygen levels in the blood. 
• Right-Sided Heart Failure: This serious condition happens when the heart's lower right ventricle pumps harder due to partially blocked arteries. 
• Lung Complications: Pulmonary fibrosis, as it progresses, can lead to blood clots in the lung, a collapsed lung, or even lung infections. 

With each individual experiencing pulmonary fibrosis differently, there can be risk factors that can increase your chances of developing this respiratory disorder. Some factors contributing to developing pulmonary fibrosis include age, certain occupations, excessive smoking, and even cancer treatments, which can increase your risk of developing pulmonary fibrosis. Even certain genetic factors and gender can increase your risk.

Diagnosis

Your doctor may opt for specific tests to detect if you may indeed be suffering from pulmonary fibrosis, and some include: 

• Chest X-Rays   
• Computerized Tomography (CT scan) 
• Echocardiogram  
• Pulse Oximetry 
• Pulmonary Function Testing   
• Exercise Stress Test  
• Arterial Blood Gas Test  
• Bronchoscopy 
• Surgical Biopsy 
• Blood Tests 

Idiopathic Pulmonary Fibrosis Treatments 

Unfortunately, the scarring from pulmonary fibrosis is irreversible, and nothing has been proven effective at stopping the progression of this disease. However, some treatments may slow the disease's progression and improve some symptoms temporarily. These may improve a patient's quality of life, and your doctor will evaluate your condition to determine the appropriate treatment. Some treatments may include: 

1. Medications: Newer medications, such as pirfenidone and nintedanib, can help slow the progression of idiopathic pulmonary fibrosis and have been approved by the FDA. 

2. Oxygen Therapy: This therapy can't stop lung damage, but it can make breathing and exercise easier, improve complications from low blood oxygen levels, lower blood pressure on the right side of your heart, and improve sleep and well-being. 

3. Pulmonary Rehab: This helps manage symptoms and improves daily function. These rehab activities include physical exercise, breathing techniques, nutritional counseling, and education. 

4. Lung Transplant: Your doctor may decide you are a candidate for a lung transplant, but it comes with complications such as rejection or infection. A lung transplant can improve your quality of life and help you live longer.