Understanding Idiopathic Pulmonary Fibrosis (IPF)

Welcome to “Understanding Idiopathic Pulmonary Fibrosis”. This presentation will describe idiopathic pulmonary fibrosis and how it is treated. It is intended to supplement discussions with your healthcare professional. What is idiopathic pulmonary fibrosis? Let’s start by looking at the name of this disease. The word “idiopathic” means that the cause of the disease is not known. “Pulmonary” means it affects the lungs. “Fibrosis” means scarring. The words together mean “an unknown factor is causing the lungs to become scarred.” Idiopathic pulmonary fibrosis, or IPF, is a serious, “progressive” disease. This means it gets worse over time. How quickly IPF worsens is different for each person. For some, it can stay stable for years, while for others it can worsen quickly. But it always progresses. Idiopathic pulmonary fibrosis is more common than we once thought. About 132,000 people are affected in the United States, with 50,000 new cases every year. It is more common in men than women and mostly affects people over the age of 50. So, how does IPF affect the lungs? First, let’s review how healthy lungs work. Normally, the lungs expand and contract fully as you breathe. When you breathe in, air passes through your lungs until it reaches the tiny air sacs called alveoli. The air sacs have walls that are built to allow air to pass freely through to the small blood vessels that surround them. When you breathe in, oxygen from the air passes through into the bloodstream. When you exhale, carbon dioxide passes from the bloodstream into your lungs and out of your body. When you have IPF, the lungs can’t expand fully. This is called “restrictive” breathing; it becomes harder for people to breathe in and causes them to become short of breath. This happens because IPF causes the body to produce too much scar tissue in and around the air sacs inside the lungs. As this scar tissue builds up, it causes the lungs to become stiff and blocks the oxygen and carbon dioxide from getting through. As this scar tissue builds up, it causes the lungs to become stiff and blocks the oxygen and carbon dioxide from getting through. The scar tissue often looks like honeycombs when the lungs are viewed on a high resolution computed tomography scan, also called an HRCT scan. Doctors look for these patterns when they are trying to diagnose IPF. With IPF, it is important to have realistic expectations. While each person’s situation is different, these are some things you should understand: • Because IPF is a “progressive” disease, you can expect that it will continue to get worse over time. As of now, there is no known cure • Lung function declines at different rates in different people. Some may progress rapidly while others may remain the same for long periods of time • At some point, you may need to be prescribed oxygen to supplement your breathing • As time goes by, your difficulty breathing will cause you to be less active Although there is no cure yet, there are ways to help treat IPF. Your doctor may suggest a variety of options. Oxygen can help you breathe easier, function better, and stay more active. It is considered a medicine, so your doctor will need to prescribe it. Pulmonary therapy, also called pulmonary rehabilitation, or PR, can improve your energy level and your ability to function, and reduce your shortness of breath. Combined with PR, exercise can help you maintain physical strength and also contribute to your general wellbeing. The Food and Drug Administration (FDA) has recently approved medicines that may help treat IPF. Ask your doctor if you would be a good candidate for this kind of treatment. A lung transplant may be an option, but not everyone is eligible for it. Discuss this with your doctor. It can be difficult to deal with a serious disease like IPF. Remember, you’re not alone. You have help from many sources: • Family and friends can listen, help, and support your health-related decisions • Your healthcare team can provide ongoing, consistent medical care • IPF support groups can help you cope with the challenges of IPF • Community organizations can provide people with home health aides, hospice care, and many other ways to assist you You also have help from a Boehringer Ingelheim Clinical Educator. This healthcare professional can provide personal education, and has been trained to help you understand IPF so you can make informed decisions about your healthcare. Speak with your doctor about setting up an appointment with a Clinical Educator or call 1-866-673-6366. Thank you for your attention to this program, “Understanding Idiopathic Pulmonary Fibrosis,” produced by Boehringer Ingelheim. For more information visit lungsandyou.com.