Pulmonary fibrosis is a disease in which the tissues in your lungs get scarred, and become thick and stiff over time. As the lungs stiffen, it becomes difficult for oxygen to efficiently be pumped to the brain and other essential organs. Sometimes doctors can find the cause of pulmonary fibrosis, but when they can’t (which is often) the condition is called idiopathic pulmonary fibrosis (IPF). Idiopathic pulmonary fibrosis can be a confusing and overwhelming diagnosis, so we’ve put together some key information to help you understand the frequently asked question: “What is idiopathic pulmonary fibrosis?”
Causes of Idiopathic Pulmonary Fibrosis:
By definition, if the cause of IPF was known it wouldn’t be diagnosed as IPF. That said, there are some theories on causes including that IPF is a kind of autoimmune disorder, and there may be genetic and/or environmental factors. The common theory currently is inflammation (in the lungs) happens as a response to a microscopic injury, which leads to scarring.
IPF most commonly affects older adults (50 – 75 years old).
Idiopathic Pulmonary Fibrosis Symptoms:
Unfortunately, IPF symptoms may not appear until the disease has already made some progression. The most common symptom is shortness of breath, also called dyspnea (some people say “breathlessness”), but many people dismiss this as a result of being out of shape or just part of the aging process. As the disease progresses breathing difficulties can make activities such as dressing and showering difficult. Additional IPF symptoms include rapid weight loss, hacking cough, loss of appetite, fatigue, and chest discomfort.
Idiopathic Pulmonary Fibrosis Treatments:
Currently there is no known cure for IPF. There are currently no FDA-approved therapies to treat IPF, though patients can opt to participate in clinical trials. For some patients, medications can help reduce the inflammation in the lungs. Oxygen therapy, or supplemental oxygen, is often prescribed for people with IPF, and respiratory therapy can be helpful.
Many people live three to five years after being diagnosed with idiopathic pulmonary fibrosis, and the most common cause of death is respiratory failure. A lung transplant is considered the best option to extend the lives of people with IPF, so it is important to discuss that with your doctor as soon as possible (ideally when the diagnosis is made).
There are also a number of things the individual has control of that can also help those with IPF remain healthier including eating healthy, getting plenty of rest, learning (and practicing!) relaxation techniques, staying active, and connecting with other IPF sufferers. For more information on ways to stay active with IPF, read our blog Staying Active with COPD. Each of these IPF topics can be covered in depth, but my goal here was help you better understand idiopathic pulmonary fibrosis.