Why You Should Ask Your Doctor About Idiopathic Pulmonary Fibrosis

Medically reviewed by Elaine K. Luo, MD on February 23, 2017Written by Colleen Story

idiopathic pulmonary fibrosis

If you’re a smoker, it makes sense to ask your doctor about idiopathic pulmonary fibrosis (IPF), a rare and incurable lung disease. Even if you don’t smoke, there are other reasons why you may want to check on the health of your lungs.

Gastroesophageal reflux disease (GERD), for example, increases your risk for IPF. If you’ve gone through radiation treatments for cancer, or are regularly exposed to dust and environmental pollutants at your job, you also need to ask about this disease.

If you have none of these risk factors, but you have a family history of lung disease, it’s best to open a dialogue with your primary care physician right away. When IPF is detected early, treatments can help slow its progression and improve quality of life.

Here are seven other reasons why you may want to ask your doctor about IPF at your next appointment.

1. IPF is on the rise

IPF is generally a rare lung disease, but recent studies suggest that the number of cases may be increasing. A 2013 study published in the reported that the disease is “underappreciated” in terms of how many people it affects. The authors added that incidence is on the rise and is expected to increase with the aging population.

The agrees, stating that between 2004 and 2012, the number of people with the disease increased from 37 for every 100,000 to 50 for every 100,000. A 2014 study in the reported that death from IPF is also increasing steadily worldwide.

These and other statistics suggest that more people may be dealing with this disease in the coming years. Check with your doctor to make sure your lungs are healthy. If you’re developing the disease, the earlier you can find out, the better.

2. IPF makes it harder to breathe

Your lungs are typically very flexible. They expand and contract to take in and expel air. IPF comes along and makes them stiff and inflexible. For some unknown reason, scar tissue forms inside the lungs, making it harder to breathe.

You can imagine how stiff and hard a scar is on your skin. When scars form in your lungs, they become thick and hardened, too. Over a period of years, the scar tissue spreads. As it does, your lungs are less able to do their job.

How fast the disease progresses depends on the person. In some people, it will develop quickly. Breathing will become more difficult within a few years. In other cases, the disease progresses more slowly.

3. The symptoms of IPF can go undetected for years

Usually if something is wrong, you can tell: When you’re getting the flu, you may have body aches or a fever. If you break a bone, the pain tells you that something isn’t right.

But with IPF, the symptoms can be subtle, and even confusing. In the early stages of the disease, you may experience no symptoms at all. The first noticeable symptom is usually shortness of breath, but this can be easily dismissed. You may feel like you’re just out of shape, or that you overexerted yourself. If the symptom initially shows up during exercise, it’s easy to think that maybe you’ve just gotten a little older.

Another early symptom may include a mild, dry cough. Again, this can be attributed to many things. You may think you have a cold, that the air is too dry, or that you’re suffering from allergies. Asking your doctor early on about even mild symptoms can help you better manage any potential problems.

4. Early diagnosis is best

Even if you have clear symptoms of IPF, like chest pain, coughing fits, fatigue, muscle aches, joint pain, and shallow breathing, diagnosis can be difficult. Pneumonia and heart disease can also cause these types of symptoms, so doctors have to do a little detective work.

That means it can take awhile to determine if you have IPF. Doctors may need to conduct several tests, and observe your progress for a few weeks. They will likely ask about your family history, as IPF is known to have a strong genetic component. The earlier you can get started on the process, the better.

Several studies have suggested that early diagnosis is key for extending survival. In 2013, found that early diagnosis helped customize treatment for each person. A showed that early diagnosis was critical to proper care, and provided the widest range of options to manage the disease.

5. Early treatments may slow the progression of IPF

The FDA recently approved new medications to treat IPF. These have shown promise in helping to slow down the progression of the disease, but again, it’s important to discover the disease as soon as possible.

According to a 2014 study in the , an earlier diagnosis is actually a prerequisite for earlier treatment, and may improve long-term outcomes.

Researchers noted that there may actually exist a “window of opportunity” when the drugs work best. This window usually occurs early on.

A delayed diagnosis, on the other hand, can result in faster disease progression. It may also result in lower survival rates.

6. Early treatments improve quality of life

The sooner you know you have IPF, the sooner you can adopt lifestyle habits that help support the health of your lungs.

You know that exercise is important for overall health. Staying in shape can also help you better manage IPF. According to a 2016 study in , people who had a higher fat-free mass index were more likely to survive longer with the disease. Fat-free mass index is a measure of your body’s muscle mass. Knowing this early on can inspire you to continue to work out to keep your muscles strong.

Yet exercise can become difficult when your lungs don’t work quite right. Pulmonary rehabilitation can help you learn new breathing techniques that will help you to stay active even as the disease progresses. If you have GERD, which is strongly correlated with IPF, treating that more consistently can also help you enjoy fewer symptoms.

These and other steps can help you become more prepared for how the disease may affect you in the future. They can also help you adopt a holistic approach to treating the disease, so that you can do everything possible to slow it down and increase your quality of life.

7. Early diagnosis may help someone in your family

Research has found that genetics play a large role in IPF. It’s unclear at this point exactly which genes may be involved. Studies have shown several that are associated with lung function. Researchers continue to look into it, to see if they can discover new ways to treat the disease.

Meanwhile, it’s clear that IPF runs in families. That means if you’re diagnosed, you can share that information with others who are related to you. Your information may help someone else to get an early diagnosis, which could end up extending their life.

For your overall health and longevity, and for your family’s safety, ask your doctor about your lung health today.

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