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Idiopathic pulmonary fibrosis (IPF) is a rare lung disease, with only about 10 to 60 cases per 100,000 people in the United States. So you’ll likely find that many people have never heard of IPF.

The rarity of this disease may lead to misunderstanding. If you or someone close to you has been diagnosed with IPF, well-meaning friends and family members may have asked questions about your condition. Here’s a guide to help you answer the questions people close to you might have about IPF.

You’ll likely have to start your conversation by explaining what IPF is.

In short, it’s a disease that causes scar tissue to form deep inside your lungs. This scarring — called fibrosis — stiffens your lung’s air sacs so they can’t deliver enough oxygen into your bloodstream and out to the rest of your body.

You can explain that this chronic lack of oxygen is why you feel tired and get short of breath when you walk or exercise. You may also have a cough that is caused by irritation associated with this scarring and a loss of mucus.

With many lung diseases, people have a natural tendency to wonder if smoking is to blame. If you smoked, you can respond that the habit might have increased your risk of getting the disease.

However, cigarette smoking doesn’t necessarily cause IPF, but it can make fibrosis worse.

Other factors, including pollution, exposure to certain types of dust like silica and asbestos, certain medications, and viral infections, may have increased your risk.

Gene mutations may also have played a role. But in most cases, IPF isn’t due to smoking or any one lifestyle factor. In fact, the word “idiopathic” means that doctors do not know exactly what caused this lung disease.

Anyone who is close to you might have already witnessed the symptoms of IPF. Let them know that because IPF prevents enough oxygen from getting out to your body, it’s harder for you to breathe.

That means you might have trouble doing physical activities — like taking a shower or walking up and down the stairs. For some people, even talking on the phone or eating may become difficult as the condition worsens. Clubbed fingers can also be a symptom of IPF.

You can explain the symptoms you experience and that you might have to miss some social events when you don’t feel well.

Let the person know that although there is no cure for IPF, treatments such as medication and oxygen therapy can help manage symptoms like shortness of breath and coughing.

If the person asks why you can’t get a lung transplant, you can tell them this treatment isn’t available to everyone with IPF. You have to be a good candidate and be healthy enough to undergo surgery. And if you meet these criteria, you’ll have to get on an organ transplant waiting list, which means waiting until a donor lung becomes available.

This can be one of the hardest questions to answer, especially if a child is asking it. The possibility of death is as hard on your friends and family as it likely is on you.

Older research published in 2010 shares that the median IPF survival rate is 2 to 3 years. While these numbers sound scary, you can explain that they can be misleading.

Though IPF is a serious disease, everyone who develops the condition experiences it differently. Some people live for many years without having any well-defined health problems. Factors like age, general health, and how severe the IPF is when diagnosed can affect your outlook. Starting anti-fibrotic medication may help improve life expectancy.

Treatments — especially a lung transplant — could improve your outlook dramatically. Reassure the person that you’re doing what you can to stay healthy.

If your doctor’s office gives out pamphlets on IPF, have some on hand to provide.

You can also point people to web resources like the:

These organizations offer educational resources and videos that describe IPF and its symptoms and treatment.

Encourage the person to attend a support group meeting with you to learn more about what it’s like to live with IPF daily.

If you’re close to them, you might encourage them to also join you at a doctor’s visit. Then they can ask your doctor any other questions they might have about your condition.