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What is Idiopathic Pulmonary Fibrosis? Understanding Your Disease and Options

What is Idiopathic Pulmonary Fibrosis? Understanding Your Disease and Options

Pulmonary fibrosis is a disease that causes lung tissue to become thick and stiff over time, making it harder for lungs to transfer oxygen into your bloodstream. The word fibrosis means scarring, referring to the thickened lung tissue. In some cases, doctors understand why the lung tissue has become scarred. It can be linked to cigarette smoking, viral infections and gastroesophageal reflux disease (GERD). However, in most cases, the cause is unknown. These cases are called idiopathic pulmonary fibrosis (IPF).

While we don’t know what causes IPF, we do know that genetics may play a role. Studies suggest that, in familial cases of IPF, family members often have a genetic disposition for the disease. Studying the disease on a cellular level allows researchers to better understand the genetic mutations that may cause the condition, resulting in knowledge that may lead us to answers.Currently, there is no cure for IPF.

What Does Idiopathic Pulmonary Fibrosis Feel Like?

Your lungs function to take in oxygen and send it into your body through your bloodstream. Each lung contains around 300 million tiny air sacs that fill up with air each time you inhale. This is relatively easy for people with healthy lungs, whose air sacs are thin, allowing oxygen and carbon dioxide to pass through very easily.

In people with IPF, however, the scar tissue on the air sacs’ walls makes them thick and hard. This makes it very difficult for oxygen and carbon dioxide to flow through the air sacs, causing that person to feel out of breath.

Many people with IPF often experience rapid, shallow breathing and sometimes have a hacking cough that doesn’t go away. When a doctor listens to an IPF patient’s lungs with a stethoscope, he or she might hear a crackling noise. Some people with IPF experience “clubbing” of their extremities, meaning a widening and rounding of their fingers and toes. Other symptoms include feeling unusually tired,achy and unintentional weight loss.

Who is affected by Idiopathic Pulmonary Fibrosis?

IPF is a condition that generally affects middle-aged and older adults, and the severity of the disease varies from person to person. In some cases, the disease progresses rapidly, whereas in other people the disease might stay the same for years. About 48,000 people are diagnosed with IPF each year in the United States.

Many people who have been diagnosed with IPF only live about three to five years after diagnosis. The most common cause of death in those with IPF is respiratory failure, followed by pulmonary hypertension, heart failure, pulmonary embolism, pneumonia and lung cancer.

Research has helped doctors be able to learn more about IPF, resulting in the ability to diagnose the condition more quickly. Additionally, researchers are currently studying several medicines that may help slow the progression of the disease, improving quality of life and potentially increasing the lifespan of IPF patients. Cellular research has also shown great promise in potentially slowing the progression and improving quality of life for IPF patients as well.

If you or a loved one suffers from idiopathic pulmonary fibrosis, cellular therapy might be an option. Contact us today to learn more about cellular therapy for IPF.


  1. Phoebe

    12 months ago

    Hi Carol,

    There are some autoimmune conditions that may cause certain chronic lung diseases to develop. However, autoimmune conditions, like rheumatoid arthritis (RA), affect everyone differently. If you’re concerned about your RA or have questions about if your RA may be causing your chronic, productive cough, we recommend talking with your doctor. Because your doctor knows you and your health situation well, he or she will be able to discuss your concerns, answer your questions and best guide you.

    Kind Regards,

    The Lung Institute

  2. Carol Anne Ranney

    12 months ago

    I have very early RA. In addition, since the beginning of this year, I have had a chronic productive cough which just will not go away. It gets better, then worse again. No fever or other symptoms. Could this be related to RA even though only one hand is mildly affected?

  3. Phoebe

    1 year ago

    Hi Fred,

    First and foremost, we’re sorry to hear about the challenges you’ve been facing with IPF. IPF and other chronic lung diseases are progressive, meaning they will worsen over time. Like you, many IPF patients take medications, such as Ofev. Doctors prescribe these types of medications to help manage symptoms and to potentially slow the progression of the disease. We recommend you talk with your doctor about the effectiveness of Ofev and to discuss your questions. Because your doctor knows you and your health situation well, he or she will be able to best guide you.

    Kind Regards,

    The Lung Institute

  4. Fred freels

    1 year ago

    I was diagnosed with IPF about 9 yes ago and had not progressed much. Then in august 2015 i had a bout with PNeumonia then my Dr prescribed ofev which i have been TAKing since. I have shortness of breath when I do strenuous exercise, but recover Prettywell within ABout 5 mins. Since there us no cure I’m assuming the ofev Is Keeping the progression at BEy. The only side affect from the ofev is some Diarrhea and gas. Is there any knowntime when the Ofev will be ineffective?

  5. Phoebe

    1 year ago

    Dear Annette,

    It’s important to remember that pulmonary fibrosis affects people differently and at varying rates of progression. For some, pulmonary fibrosis may progress rapidly, and for others, it may progress slowly. We recommend talking with your doctor about your questions and concerns. Your doctor knows you and your health situation well, so he or she will be able to best guide you and best answer your questions.

    Kind Regards,

    The Lung Institute

  6. Annette Cea

    1 year ago

    I am trying to be certain whether or not pulmonary fibrosis is much the same: 3 -5 yrs. Life span after diagnosis confirmation?

* Every patient is given a Patient Satisfaction Survey shortly after treatment. Responses to the 11-question survey are aggregated to determine patient satisfaction with the delivery of treatment.

^ Quality of Life Survey data measured the patient’s self-assessed quality of life and measurable quality of improvement at three months.

All claims made regarding the efficacy of Lung Institute's treatments as they pertain to pulmonary conditions are based solely on anecdotal support collected by Lung Institute. Individual conditions, treatment and outcomes may vary and are not necessarily indicative of future results. Testimonial participation is voluntary. Lung Institute does not pay for or script patient testimonials.

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