Idiopathic pulmonary fibrosis (IPF) affects anywhere from 2 to 29 people out of 100,000. Because IPF isn’t a common disease, there are plenty of misconceptions about it.

Clearing up those misunderstandings is made harder because many of its symptoms, such as shortness of breath, fatigue, sore muscles and joints, shallow breathing, and gradual weight loss, aren’t obvious to others. They may be greatly affecting your quality of life, however.

As you learn to cope with IPF and try to sort out fact from fiction, keep these myths and truths in mind.

1. Myth: You obviously got IPF because you smoked cigarettes

To begin with, there is nothing obvious about the cause of IPF. “Idiopathic” means the cause of the disease is unknown.

Cigarette smoking certainly increases your risk for developing IPF and other lung diseases. Some viral infections and exposure to airborne pollutants can also raise your odds of developing IPF. Even some medications, such as the chemotherapy drug methotrexate, may cause pulmonary fibrosis. A family history of IPF may also increase your chances of having the disease.

If you’ve received an IPF diagnosis, as opposed to another form of pulmonary fibrosis, it’s because there is no obvious cause.

2. Myth: You should only be out of breath when you’re active

This may be the case early on in your disease, but if it reaches a severe stage, you may have trouble breathing even at rest. Don’t let anyone tell you how you should be feeling or what your symptoms should be. Also, keep in mind that you can learn breathing techniques that can help you breathe a little easier.

3. Myth: You’ll have to be on oxygen 24 hours a day

While many people with IPF are on oxygen therapy, there is no guarantee you will need it all day and night. Or ever. Some people with IPF need oxygen only when sleeping. Others need it only during physical activity.

Oxygen therapy involves a portable tank that pumps oxygen through a tube connected to a mask you wear over your nose and mouth or to two smaller tubes in your nostrils. Oxygen therapy can help you breathe a little easier, and it’s sometimes essential to make sure your lungs properly supply your blood with oxygen to nourish the rest of your body.

If you ever do need oxygen therapy, whether it’s for an hour at a time or 24 hours a day, know that it’s generally safe and effective. The usual side effects are a temporary morning headache for those who receive oxygen all night, and dryness in the nostrils.

4. Myth: You only have a few months to live

The average life expectancy after diagnosis is three to five years, but many people live longer than that. Symptoms can remain stable for many years. It all comes down to how severe your case is and how fast it’s progressing.

It can be difficult to predict the progression of your IPF at first. If you follow your doctor’s advice about treatment and lifestyle changes, you may be able to slow the advancement of IPF and preserve some quality of life for years to come.

5. Myth: Stem cells can cure IPF

Stem cell therapy holds promise for the treatment of IPF, but we’re years away from stem cells being about to cure or reverse IPF. The goal of stem cell therapy is to use stem cells, which can grow into many different types of cells, to help create healthy tissue that replaces tissue scarred by IPF. Clinical trials are ongoing. Ask your doctor for more information.

6. Myth: IPF is the same as cystic fibrosis

While cystic fibrosis and IPF are both chronic lung diseases that can make breathing difficult, they’re very different conditions.

The word “fibrosis” simply means the scarring and thickening of connective tissue. It can occur in your lungs, but also in your heart, brain, liver, and other organs.

Cystic fibrosis is a genetic condition that leads to frequent lung infections and an abnormal buildup of mucus in your system. It also affects your pancreas and other organs.

IPF doesn’t lead to infections. Instead, symptoms arise from the scarring and thickening of tissue deep in your lungs. Other organs are affected only when they start to experience a drop in their oxygen supply.

7. Myth: IPF is predictable, and it usually affects people the same way

Even members of the same family who have IPF each experience the disease differently. Symptoms differ from one person to the next. The speed with which IPF progresses is also unique to each person with the disease. You may experience rapid changes in your symptoms, or you may have long stretches during which there is little change.

The takeaway

If you read or hear anything about IPF that doesn’t sound right or that seems scary or unbelievable, check with your doctor. The internet has a lot of good information as well as misleading information. Even well-meaning friends or relatives can add to the stress and confusion you’re already battling. Be careful. Join a support group to hear from others with firsthand experience with the disease. They may be some of your best sources of the truth about IPF.