What is Pulmonary Fibrosis?

Pulmonary fibrosis is a serious lung disease that affects the respiratory system. The disease impacts the connecting tissue in the lung and the alveoli (air sacs inside the lungs). The thin walls of the alveoli get thick and scarred, also called becoming fibrotic. The architecture of the lung makes it stiffer and is less efficient at delivering oxygen into the bloodstream. As lung damage progresses, the rigid lung tissues demand additional energy, causing breathing difficulties. The symptoms of pulmonary fibrosis can vary depending on the individual and the severity of the disease. Some common symptoms include shortness of breath, a persistent cough, fatigue, weight loss, and chest pain.

Causes

When a person is diagnosed with pulmonary fibrosis, sometimes there is a clear association with another illness, medication, treatment, or exposure to certain elements. Some causes of pulmonary fibrosis include:

• Exposure to toxins such as asbestos, coal dust, or silica
• Certain medications
• Chronic
• Long term exposure to high levels of organic dusts, fungus, or molds.
• Autoimmune conditions such as systemic lupus, scleroderma, rheumatoid arthritis and Sjogren’s syndrome
• Hereditary

Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis (IPF). IPF is staged with terms such as mild, severe, early and advanced based on pulmonary function tests.

Risk Factors

Certain risk factors increase the likelihood of being diagnosed with pulmonary fibrosis. Having one or more risk factors does not mean you are guaranteed to get the disease one day, but it can place you at higher risk. Risk factors of pulmonary fibrosis include:

• Older age, between ages 50 and 70
• Male biological sex
• Smoking
• Working around dust or fumes
• Other medical conditions and medications
• Radiation exposure

Treatments

Current pulmonary fibrosis treatments are focused on the symptoms as there is no cure for the disease. Preventing more lung scarring and helping patients stay active and healthy are priorities in addition to medication, oxygen therapy, pulmonary rehabilitation, lung transplant and lifestyle changes. The progression of pulmonary fibrosis is highly variable. Treatments, disease management and prognosis depend on the specific type of pulmonary fibrosis diagnosed. It is crucial to share new or worsening symptoms with your doctor in order to work together on the most effective treatment plan.

Life Expectancy

Due to the variation of the disease and its progression, the life expectancy of those living with pulmonary fibrosis is difficult to pinpoint. In severe cases of idiopathic pulmonary fibrosis, the disease may take the lives of patients within three to five years from diagnosis. Even among patients with similar diagnoses, some may experience rapid progression while others can remain at the same level of functioning for a longer time. Some may experience complications through sudden declines in lung function and oxygen levels resulting in other problems such as heart failure and high blood pressure in the lungs.

Pulmonary fibrosis is a formidable lung disease that can have a significant impact on an individual’s life. While living with pulmonary fibrosis can be challenging, there are support resources available to help individuals and their families cope with the disease. The Comprehensive Sleep and Breathing Center is a group of highly experienced professionals who can help to enhance your quality of life while living with pulmonary fibrosis.