Facebook Pixel

Pulmonary Fibrosis: The Facts

By HERWriter
Rate This

The lungs are primarily responsible for ensuring that our bodies -- organs, tissues, muscles, tendons, etc. -- receive the oxygen they need to function. The oxygen is delivered to the rest of our body by the blood. The lungs are also responsible for releasing carbon dioxide (the byproduct of our body using oxygen to function) from our bodies and back into the air around us. As the carbon dioxide is expelled, it is replaced by oxygen and the whole process continues.

If anything impedes or affects the lungs' capacity to eliminate carbon dioxide, take in oxygen and oxygenate the blood that will travel through our body, every aspect of our physical being suffers. Our muscles won't work like they should. Our organs won't work like they should. Lung impairment can affect the quality of sleep we get at night which in turn will affect our ability to focus and concentrate on the things we need to do throughout the day.

One such condition that can affect this optimum balance, is pulmonary fibrosis.

What is Pulmonary Fibrosis?

Pulmonary fibrosis is a chronic, progressive disease where alveoli and interstitial tissues (tissues in between the cells) of the lungs become scarred. This scarred tissue compromises the lungs' ability to complete the carbon dioxide/oxygen swap.

It is believed that this scar tissue develops after repeated episodes of injury to the lungs through infections and exposure to harmful environmental sources. There is no known specific disease or condition that can be pinpointed as the main source because there are so many conditions that can affect how lungs function.

Those people who develop pulmonary fibrosis without an identifiable cause are said to have idiopathic pulmonary fibrosis. Unfortunately for these patient, this type of fibrosis does not respond to medical therapy.

It is also know by other names: chronic interstitial pneumonitis, Hamman-Rich Syndrome, and diffuse fibrosing alveolitis.

According to The Pulmonary Fibrosis Foundation, 5 million people worldwide suffer from this disease, with over 500,000 of those within the continental United States. It is believed that this number is actually higher because many more cases go undiagnosed or misdiagnosed. While the typical patient is in their forties or fifties, cases have been seen in children as young as seven and in people as old as eighty.


The most common symptoms of pulmonary fibrosis include:

- shortness of breath (dyspnea), particularly with activity;
- continual dry, hacking cough;
- fatigue and weakness;
- chest discomfort; and
- loss of appetite and weight loss without trying.

As you can see this list has components similar to many other lung diseases and ailments. The difference between idiopathic and nonidiopathics variants of the disease remains illusive and doctors are still trying to determine the best way of naming and classifying cases.

In addition to the medical history, physical exam, and chest X-ray - which will work to eliminate any other possible lung conditions, but will not be enough to provide a definitive diagnosis of pulmonary fibrosis - patients may also be referred for:

- High-resolution computertized tomography (HRCT) scan, which provides a sharper and more detailed pictures of the lungs than CT scans or conventional chest X-rays;

- Pulmonary function tests which will evaluate how well your lungs function, specifically how much oxygen your lungs can hold, and how long it takes for your lungs to expel all the air;

- Oximetry, which is a measurement of the level of oxygen in your blood stream through the use of a small clamp device that is placed on one of your fingers;

- Exercise stress test, which is carried out on a treadmill or stationary bike to evaluate how your lungs function when you're active; and

- Biopsy of lung tissue.

Treatments for Pulmonary Fibrosis

There is no known cure for pulmonary fibrosis and as we read above idiopathic pulmonary fibrosis does not respond to treatment. For those cases of nonidiopathic pulmonary fibrosis, which do respond to treatment, such efforts are aimed at reducing symptoms and improving quality of life.

Testing is currently underway on medications that will treat the scarring, and other medications may be used to help reduce the inflammation that causes the scarring, but these have met with limited success in reducing the overall progress of the disease.

Since there is no way currently to repair or reverse the lung tissue or to stop the scar tissue from developing altogether, the condition will continue to worsen.

In addition to medications, younger sufferers who have not responded to other treatment options, may be eligible for a lung transplant. Oxygen therapy and pulmonary rehabilitation to help patients cope with the disease day-by-day are non-medication based methods that help to improve the quality of life for patients.

For more information on clinical trials on drugs for treating pulmonary fibrosis, please visit: www.clinicaltrials.gov or contact the Pulmonary Fibrosis Foundation through the information on their website.

Sources: www.pulmonaryfibrosis.org; www.lung.ca (Canadian Lung Association); www.mayoclinic.com; www.medicineNet.com

Add a Comment6 Comments

i llllllll ................ The website has not been the greatest...
I'm told, after 7 years with pulmonary fibrosis and hypertension, that I'm at end stage. I have 30% lung function. I've been on oxygen for about six years. As long as I'm sitting, I feel fine. I am out of breath if I walk more than 10-15 feet. Because of other conditions (diabetes; I get transfusions for low red blood cell and HGB counts; kidneys are beginning to fail), I am not a transplant candidate. At age 64, the Doctor has basically said "good bye and good luck - I probably won't see you again". I feel like it's not the END... but what is the "end" going to be like. What should I expect and what should I prepare my family for? I'm serious -- what can I physically expect the END to be like? Why can't I get a straight answer?

April 26, 2011 - 5:35pm
HERWriter (reply to Carol Day)

Sounds like you need to talk to palliative care people...those health professionals who look after those people who are living out their last days. I don't think they can tell you precisely what to expect because everyone is different. If you don't feel like it's the end then it probably isn't. From a medical perspective, doctors may feel that there is nothing else they can do for you except make you comfortable.

My article was intended to educate people, patients and families on what do expect with these conditions and symptoms, not deal with such a prognosis. I don't know that anything can really prepare you for that (although I have a more "religious" perspective on that that I'll share through the messaging board if you like). Perhaps you would like to contribute to Empowher through a HerShare article, or series of. Kind of chronicle what happens from here. It would certainly help those patients and families who are in the same situation, wondering the same questions. It would be a great legacy to pass along.

April 26, 2011 - 6:13pm
EmpowHER Guest

Thank you for your advocacy efforts to let others know about this disease!

Jennifer Bulandr
Director of Community Relations
Pulmonary Fibrosis Foundation
www.pulmonaryfibrosis.org - Join us in finding a cure!

January 29, 2010 - 3:24pm
HERWriter (reply to Anonymous)

You're welcome, Jennifer.

January 29, 2010 - 5:47pm
EmpowHER Guest

Anyone interested in more information, patient support, drug trial contacts, the congressional bill for PF research funding and more, please contact the Coalition for Pulmonary Fibrosis at 888-222-8541, or go to our website at www.coalitionforpf.org.

January 13, 2010 - 12:33pm
Expert HERWriter Guide Blogger (reply to Anonymous)

Anon - Thank you for posting this information. Can you tell us more about the coalition, and your personal involvement? We'd love to hear from you!

January 13, 2010 - 6:49pm
Enter the characters shown in the image.
By submitting this form, you agree to EmpowHER's terms of service and privacy policy
Add a Comment

We value and respect our HERWriters' experiences, but everyone is different. Many of our writers are speaking from personal experience, and what's worked for them may not work for you. Their articles are not a substitute for medical advice, although we hope you can gain knowledge from their insight.

Idiopathic Pulmonary Fibrosis

Get Email Updates

Idiopathic Pulmonary Fibrosis Guide

Have a question? We're here to help. Ask the Community.


Health Newsletter

Receive the latest and greatest in women's health and wellness from EmpowHER - for free!