If you’ve been diagnosed with idiopathic pulmonary fibrosis (IPF), you may be full of questions about what comes next.

A pulmonologist can help you figure out the best treatment plan. They can also advise you on lifestyle changes you can make to reduce your symptoms and achieve a better quality of life.

Here are 10 questions you can bring to your pulmonologist appointment to help you better understand and manage your life with IPF.

You may be more familiar with the term “pulmonary fibrosis.” It means scarring of the lungs. The word “idiopathic” describes a type of pulmonary fibrosis where doctors can’t identify the cause.

IPF involves a scarring pattern called usual interstitial pneumonia. It’s a type of interstitial lung disease. These conditions scar lung tissue found between your airways and bloodstream.

Even though there isn’t a definite cause of IPF, there are some suspected risk factors for the condition. One of these risk factors is genetics. Researchers have identified that a variation of the MUC5B gene gives you a 30 percent risk of developing the condition.

Other risk factors for IPF include:

  • your age, since IPF generally occurs in people older than 50
  • your sex, as men are more likely to develop IPF
  • smoking
  • comorbid conditions, such as autoimmune conditions
  • environmental factors

IPF affects about 100,000 Americans, and is therefore considered a rare disease. Each year, doctors diagnose 15,000 people in the United States with the condition.

Worldwide, about 13 to 20 in every 100,000 people have the condition.

Every person who receives an IPF diagnosis will have a different level of breathing difficulty at first. You may be diagnosed in the early stages of IPF when you just have mild labored breathing during aerobic exercise. Or, you may have pronounced shortness of breath from daily activities like walking or showering.

As IPF progresses, you may experience more difficulty breathing. Your lungs may get thicker from more scarring. This makes it difficult to create oxygen and move it into your bloodstream. As the condition gets worse, you’ll notice that you breathe harder even when you’re at rest.

The outlook for your IPF is unique to you, but there isn’t a cure right now. Many people live about 3 to 5 years after being diagnosed with IPF. Some people live longer or shorter amounts of time, depending on how quickly the disease progresses. The symptoms you may experience over the course of your condition vary.

There are other symptoms of IPF. These include:

  • a nonproductive cough
  • fatigue
  • weight loss
  • pain and discomfort in your chest, abdomen, and joints
  • clubbed fingers and toes

Talk to your doctor if new symptoms arise or if they get worse. There may be treatments that can help ease your symptoms.

You may be at risk of having or developing other lung conditions when you have IPF. These include:

  • blood clots
  • collapsed lungs
  • chronic obstructive pulmonary disease
  • pneumonia
  • pulmonary hypertension
  • obstructive sleep apnea
  • lung cancer

You may also be at risk of having or developing other conditions such as gastroesophageal reflux disease and heart disease. Gastroesophageal reflux disease affects 9 out of 10 people with IPF.

IPF isn’t curable, so treatment goals will focus on keeping your symptoms under control. Your doctors will try to keep your oxygen level stable so you can complete daily activities and exercise.

Treatment for IPF will focus on managing your symptoms. Treatments for IPF include:


The U.S. Food and Drug Administration approved two new medications in 2014: nintedanib (Ofev) and pirfenidone (Esbriet). These medications can’t reverse damage to your lungs, but they can slow the scarring of lung tissue and the progression of IPF.

Pulmonary rehabilitation

Pulmonary rehabilitation can help you manage your breathing. Several specialists will teach you how to manage IPF.

Pulmonary rehabilitation can help you:

  • learn more about your condition
  • exercise without aggravating your breathing
  • eat healthier and balanced meals
  • breathe with greater ease
  • save your energy
  • navigate the emotional aspects of your condition

Oxygen therapy

With oxygen therapy, you’ll receive a direct supply of oxygen through your nose with a mask or nasal prongs. This can help ease your breathing. Depending on the severity of your IPF, your doctor may recommend you wear it at certain times or all of the time.

Lung transplant

In some cases of IPF, you may be a candidate to receive a lung transplant to prolong your life. This procedure is generally only performed in people under 65 without other serious medical conditions.

The process of receiving a lung transplant can take months or longer. If you do receive a transplant, you’ll have to take medications to prevent your body from rejecting the new organ.

To prevent your symptoms from getting worse, you should practice good health habits. This includes:

  • stopping smoking immediately
  • washing your hands regularly
  • avoiding contact with people who are sick
  • getting vaccinations for flu and pneumonia
  • taking medications for other conditions
  • staying out of low-oxygen areas, like planes and places with high elevations

Lifestyle adjustments can ease your symptoms and improve your quality of life.

Find ways to stay active with IPF. Your pulmonary rehabilitation team may recommend certain exercises. You may also find that walking or using exercise equipment at a gym relieves stress and makes you feel stronger. Another option is to get out regularly to engage in hobbies or community groups.

Eating healthy foods may also give you more energy to keep your body strong. Avoid processed foods high in fat, salt, and sugar. Try to eat healthy foods like fruits, vegetables, whole grains, and lean proteins.

IPF can affect your emotional well-being, too. Try meditating or another form of relaxation to calm your body. Getting enough sleep and rest may also help your mental health. If you feel depressed or anxious, speak to your doctor or a professional counselor.

Finding a support network is important when you’ve been diagnosed with IPF. You can ask your doctors for recommendations, or you can find one online. Reach out to family and friends as well and let them know how they can help you.

Support groups enable you to interact with a community of people who are experiencing some of the same challenges as you. You can share your experiences with IPF and learn about ways to manage it in a compassionate, understanding environment.

Living with IPF can be challenging, both physically and mentally. That’s why it’s so important to actively see your pulmonologist and ask them about the best ways to manage your condition.

While there isn’t a cure, there are several steps you can take to slow progression of IPF and achieve a higher quality of life.