Idiopathic Pulmonary Fibrosis FAQs

It is hard to predict life expectancy with IPF because the course of the disease can be very different from one person to another. For some, the course of the disease can be relatively stable. For others, the disease may progress more quickly. Flare-ups make it worse.

Pulmonary fibrosis can have more possible causes. Here are a few:

• Occupations such as those that involve working with organic substances, asbestos, silica dust, ground stone or metal dust
• Certain chronic conditions (e.g. lupus, rheumatoid arthritis, scleroderma)
• Infections (e.g. tuberculosis, pneumonia)
• Certain strong medications (e.g. antibiotics, chemotherapy, heart medications)
• People who smoke or former smokers may be more likely to develop pulmonary fibrosis than people who have never smoked

When there is a known cause of pulmonary fibrosis, removal of the person from the environment or occupation causing the disease is very important.

However, in the majority of cases, the cause of pulmonary fibrosis is not known and not completely understood. This is known as “Idiopathic Pulmonary Fibrosis” or IPF. Idiopathic means “of unknown origin”. Not knowing what caused it makes the treatment more challenging.

Oxygen is not addicting. If it has been prescribed, you need to use it. Avoiding it because you believe you will become addicted or for any other reason will not help you. It is important to know, however, that oxygen is considered to be a drug. Do not increase the level unless a health-care provider has advised you to.

A lung transplant has the potential to increase the survival rate. However, there are also many risks involved with it. Some of those risks are related to the medication used to prevent the body from rejecting the new lungs. Other risks such as infection are related to the operation itself.

Before deciding if someone with IPF is a good candidate for a lung transplant, the medical team looks at many factors, including the overall health and lifestyle of the person. Talk to the health-care provider or a specialist to see if a lung transplant would be a possibility.