Idiopathic pulmonary fibrosis, or IPF, is considered a rare disease but is more common than we once thought, with up to 207,000 people affected in the United States and about 58,000 new cases diagnosed each year. This diagnosis is truly heartbreaking because though there are some treatments that may extend a patient’s lifespan, there is no cure for IPF. Dr. Albert Rizzo, American Lung Association’s Chief Medical Officer answered our top questions about this serious lung disease.

1. What is IPF? 

IPF is a specific lung disease in a larger category of lung conditions known as Interstitial Lung Diseases. As in other forms of interstitial lung disease, IPF affects the tissue surrounding the air sacs, or alveoli, in your lungs. As IPF develops, the normally thin, lacy alveoli walls become thick, stiff and scarred. When healthcare providers are unable to identify what caused this change, it is called idiopathic.

Though the exact cause is not known, certain people are more likely to develop IPF. For example, IPF is more common in men than women and mostly affects people over 50 years of age. Other factors that may contribute to developing IPF include smoking cigarettes.

2. What are common symptoms and complications of IPF? 

Because it is often slow in progression and symptoms are similar to other chronic lung diseases, the diagnosis of IPF can be difficult. “High resolution CT scans of the chest and at times biopsies are helpful in confirming the diagnosis,” says Dr. Rizzo. The most common symptoms are a dry, hacking cough, shortness of breath and chest discomfort. Your doctor may hear crackles or “dry rales” when listening to your chest.  As the disease progresses, people with IPF may also begin to experience extreme fatigue, muscle aches, weight loss and clubbing of the extremities. For many people in later stages of IPF, breathing can be difficult even at rest.

People living with IPF will experience exacerbations, or bouts of worsening symptoms. That can further cause low levels of oxygen in your blood (hypoxemia) and respiratory failure. There are also comorbid conditions, like gastroesophageal reflux disease (GERD), sleep apnea, and pulmonary hypertension (PH) that can co-exist in people who are diagnosed with IPF. 

3. How is IPF treated? 

Idiopathic pulmonary fibrosis is a progressive disease, so as the fibrosis builds up over time, patients will gradually become more breathless and will have an increased need for supplemental oxygen. However, this progression and timeline differs from person to person. This is why it is so important for people diagnosed with IPF to work closely with their healthcare provider to monitor their symptoms and create a treatment plan.

Treatment plans may include medication, pulmonary rehabilitation, oxygen therapy or even lung transplant surgery. “Up until about 5-6 years ago, there were no specific therapies for IPF, but now anti-fibrotics can be used in many patients to slow the progression of the disease,” said Dr. Rizzo. In addition, you may need to make lifestyle changes to improve your overall health. Eating nutritious food, staying active, reducing stress and protecting your lungs by quitting smoking if you currently use tobacco products are a few things your healthcare provider may suggest. 

4. Are there clinical trial options for IPF? 

When discussing treatment options with your physician, you may want to ask about clinical trials. Visit clinicaltrials.gov to learn more about new drugs that are being tested or current drugs are being evaluated in different ways. If you live near a major academic center, it is worth reviewing their website for IPF clinical trials for which you might be a candidate.

5. What resources are out there for IPF patients and caregivers?

Being diagnosed with IPF yourself, or having a loved one who is, can be overwhelming, which is why resources like the American Lung Association’s Patient & Caregiver Network, the Living with Pulmonary Fibrosis and the Caring for Pulmonary Fibrosis online support community can help answer your biggest questions. You can also call the Lung Association's Lung HelpLine at 1-800-LUNGUSA to talk to a trained respiratory professional or registered nurse who can answer your questions and connect you with support.

Support for this educational content is provided by Horizon Therapeutics.

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