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- What is IPF?
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease that causes permanent damage to the lungs. The tissue damage that occurs with IPF may be the result of incomplete or inappropriate healing in response to tiny injuries in the deepest tissues within the lungs. In response to these injuries, tiny air sacs in the lung called alveoli thicken and stiffen, causing a type of scarring (fibrosis). This makes it difficult for the oxygen you breathe in to enter the bloodstream.
- IPF is an interstitial lung disease. What does this mean?
The “interstitial” in interstitial lung disease (ILD) refers to the tissue between the alveoli and blood vessels in the lungs.
- What are the causes of IPF?
Unlike other forms of interstitial lung disease, a diagnosis of IPF means that the cause of the lung scarring can’t be identified.
The medical community has not yet determined why some people develop IPF, while other people with similar risk factors do not. While the genetic factors in the disease are not well understood, scientists believe that, in some cases, IPF may be the result of an interaction between the environment and a person’s genetic (inherited) potential for the disease.
- Why can it take so long to diagnose IPF?
IPF is difficult to diagnose because the signs and symptoms often mimic other conditions, such as heart or lung disease. For example, patients with chronic obstructive pulmonary disease (COPD) and asthma also have shortness of breath. If a physician is not specifically looking for signs of IPF with specific tests, such as an HRCT, it is possible mild fibrosis will not be detected until symptoms develop.
- What is the purpose of pulmonary function tests?
Pulmonary function tests help your doctor assess how fibrosis is affecting your lungs. Regular testing creates an important record of the health of your lungs.
Through monitoring, you and your doctor can determine how well you are responding to therapies, how rapidly your condition is progressing, and potential next steps. Regular interactions with your doctor will also help ensure that you receive the most current and best possible treatments.
- What happens as IPF progresses?
Patients with severe IPF symptoms experience increasing breathlessness. These patients may require supplemental oxygen therapy and additional medications to manage increased coughing and breathlessness.
- What is the life expectancy for people with IPF?
The average life expectancy after a diagnosis of IPF is between 2 and 5 years, but the course of each patient’s disease will vary. It’s important to remember that this is an average calculated across a large population. This may not reflect your experience or lifespan expectation. Also, these numbers may change when new studies are published.
- What medications should I be taking to treat symptoms of IPF?
Your doctor may provide prescription drugs, supplements, or over-the-counter medications to treat and help manage the symptoms of IPF. Patients with IPF may have additional health problems, such as gastroesophageal reflux disease (GERD), pulmonary hypertension, or sleeping disorders. These medical conditions may require additional medications or treatments.
- Should I change my diet?
While there are no diets that have been proven to specifically help patients with IPF, a balanced, nutritious diet is essential for staying as healthy as possible. Your doctor or dietician can give you further guidelines for healthy eating.
- Should I exercise?
People with chronic respiratory problems sometimes limit their physical activities in an attempt to avoid shortness of breath. Regular exercise strengthens your muscles and makes them more resistant to fatigue. With practice and training, you can learn ways to help maintain activities and perform regular tasks. Your doctor can help you develop an exercise program that is right for you.
- What are the benefits of pulmonary rehabilitation?
The goal of pulmonary rehabilitation is to help you learn ways to better perform day-to-day activities and manage feelings of breathlessness. Studies have shown that pulmonary rehabilitation can help patients with lung disorders by improving their ability to exercise and maintaining the ability to do daily activities.
Commonly consisting of a series of sessions over several weeks, pulmonary rehabilitation teaches you ways to better manage life with IPF by providing you with monitored exercise, breathing techniques, nutritional counseling, and emotional support.
- What is supplemental oxygen therapy?
Supplemental oxygen therapy can help deliver additional oxygen to the blood and help your ability to exercise. Your doctor will test the saturation level of oxygen in your blood to determine whether you need supplemental oxygen. Some patients may need supplemental oxygen all the time. Others may only need supplemental oxygen during sleep and exercise. Only a doctor can determine if you can benefit from supplemental oxygen. Talk to your doctor to see if supplemental oxygen therapy is right for you.
- Where do I get supplemental oxygen?
If your doctor has prescribed supplemental oxygen, remember to use it as prescribed. The correct amount of oxygen in the bloodstream is necessary to maintain normal body function. Low blood oxygen levels can lead to additional health problems.
- What should I expect if I enroll in a clinical trial?
Remember that clinical trials are not intended to provide individual benefits for you. Instead, they are intended to provide information about a therapy or outcome that may benefit all IPF patients. If you want to participate in a clinical trial, you should have a full understanding of what will be required. Different clinical trials have different requirements, and you must meet certain requirements to participate in a clinical trial.
- Am I a candidate for a lung transplant?
If you are in good physical condition, other than the lung fibrosis, you may be eligible for lung transplantation. You will need to discuss this with your doctor.
- What are the benefits and risks of a lung transplant?
A lung transplant has the potential to extend lifespan. In recent years, survival rates after single and double lung transplantation have improved. However, there are risks involved with this procedure, including serious complications from infections and rejection of the lung . Talk to your doctor to see if you’re a candidate for lung transplantation.
Learn more about IPF terms and what they mean.
Alveoli (al-VEE-oh-lie): Tiny air sacs in the lungs where carbon dioxide leaves the bloodstream and oxygen enters the bloodstream
Diffusing capacity tests: Measure how well gases are exchanged between the lungs and blood
DLCO (carbon monoxide diffusing capacity) test: Measures how carbon monoxide is exchanged by the lungs
Fibrosis (fie-BRO-sis): Formation of fibrous scar tissue
Gastroesophageal reflux disease or GERD: A regurgitation of stomach acids into the esophagus and throat, causing heartburn, acid indigestion, and possibly injury to the lining of the esophagus—also called acid reflux disease. Not all patients with GERD can feel the effects of acid reflux, even if it is occurring
HRCT (high-resolution computed tomography) or CT: A type of X-ray that produces detailed images of the body, including the lungs
Hypoxemia: Having too little oxygen in the blood. Hypoxemia is measured by a test called pulse oximetry
Idiopathic (id-ee-oh-PATH-ik): Of unknown cause
Interstitial lung disease or ILD: A large group of lung disorders that cause inflammation and/or scarring (fibrosis) of interstitial lung tissue
Interstitium: The tissue between the pulmonary alveoli and blood vessels in the lungs
IPF: Idiopathic pulmonary fibrosis
Pulmonary: Relating to the lungs
Pulmonologist: A physician specializing in the lungs
Pulse oximetry: A non-invasive method for monitoring a patient's blood oxygen saturation
Radiologist: A physician specializing in using radiology tests (eg, X-rays) to diagnose illness
Respiratory therapist: A healthcare professional specializing in pulmonary treatment
Spirometry (spuh-ROM-uh-tree): A type of test that evaluates how air moves in and out of your lungsBack to top