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Idiopathic Pulmonary Fibrosis also known as IPF
Student Health Information Page compiled by: Cathy Kalnicky

Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a disease in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis (1). As your lung tissue thickens, your lungs can't properly move oxygen into your bloodstream. As a result, your brain and other organs don't get enough oxygen they need. Idiopathic (id-ee-o-PATH-ick) means your physician does not know what caused you to have this lung disease (1). Genetics may possibly play a role in causing your IPF. If more than one member of your family has been diagnosed with IPF, the disease is then called familial IPF.

How common is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis has an estimated prevalence of 13 to 20 per 100,000 people worldwide. About 100,000 people are affected in the United States, and 30,000 to 40,000 new cases are diagnosed each year. (2)  Up to fifty percent of the cases of IPF are not diagnosed correctly. 

Symptoms:

You may notice a cough and shortness of breath with activity. The cough can be a constant dry hacking cough. You may notice you tire out quickly and feel weak. You may have some pain or discomfort to your chest. The shortness of breath and cough may prevent you from taking part in your usual daily activities

The doctor will listen to your lungs with a stethoscope and he will hear a crackling noise. This is described as a Velcro like tearing sound.

As your lungs develop more scar tissue, your symptoms may worsen. As the disease progresses you may notice you are becoming more out of breath while taking part in everyday activities like showering, getting dressed, speaking on the phone, or even eating.

Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to IPF and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth. (3)

Diagnosis

Sometimes to get the proper diagnosis you may need to see a Pulmonologist. This is a doctor who specializes in diseases of the lungs. IPF is a serious disease that usually affects middle-aged and older adults. It is more common in men than woman. The severity of IPF may vary from person to person and physicians do not know why. The disease may stay the same for years and you may not notice much of a change in your breathing. In other patients with IPF the disease may progress quickly and you may require oxygen therapy at all times in order to treat your shortness of breath.

We do know there are some possible environmental exposures that you may have been in contact with may have resulted in you being diagnosed with IPF. Some of the suspected causes of IPF are:

  • Metal or wood dusts
  • Asbestos
  • Farming
  • Raising birds
  • Molds
  • Certain drugs (drugs to help treat cancers called chemotherapies)
  • Cigarette smoking
  • Possible diagnosis of Gastroesophageal Reflux disease or GERD

It may be necessary for you to have the following diagnostic testing performed to confirm the diagnosis:

  • Radiology testing known as a high resolution CAT scan of the chest (this helps identify the pattern of scarring in your lungs)
  • Pulmonary function testing (this determines the stiffness of your lungs)
  • Exercise testing / 6 minute walk test

If a clear diagnosis cannot be made from reviewing the above tests, you may need to have a surgical procedure that will take a small sample of the affected area of your lung. This is called a surgical lung biopsy.

What to expect at your doctor visit:

  • Your life will be reviewed.
  • The medical staff will be collecting information to determine if you have had exposure to any of the compounds that are suspected of causing the disease.
  • Your complete life history will be reviewed.
  • A very through physical will be performed.
  • Your radiology tests will be reviewed.
  • Sometimes your CAT scan may show a honeycombing effect and this can be used to determine your survival.
  • Your blood will be tested to look for certain autoimmune diseases. An autoimmune disease is an illness that occurs when the body tissues are attacked by its own immune system. (4)

Living with IPF:

Receiving a diagnosis of IPF may be a shock to you and your family. Hearing there is no cure for IPF may upset you and your family. You may see a gradual worsening of your pulmonary function tests over time. Many people may survive about 3 to 5 years after a diagnosis of IPF is made. Researchers are studying new medications in hope that one of these medications may be able to stop the scarring of your lungs. You can look for clinical trials by using a website called ‘clinicaltrials.gov’. This website provides you with an overview of the research being done for IPF. It is recommended that patients with IPF take part in pulmonary rehab. This is physical therapy or a monitored exercise program that teaches the patients how to control their breathing with activity. You may need to wear oxygen at all times or during activity. A prescription from your doctor is needed. If your condition worsens you may need a surgical procedure to remove your lungs and replace them with someone else’s. This is a lung transplant. Either one or both of your lungs could be removed and transplanted with someone else’s lungs.

Once you are diagnosed with IPF it is important to stay in shape and remain active. If you have not stopped smoking, you will be advised to quit. Eating a healthy diet, maintain a proper weight and get enough rest.

There are support groups for patients and families living with a diagnosis of IPF. These groups can provide you and your family with current information about IPF. These organizations can assist you in dealing with a diagnosis of IPF. It is very important that you talk to your loved ones about what your wishes are when your disease get worse.

Resources and Support

To learn more about your disease and other support services in your community contact:

American Lung Association
1301 Pennsylvania Ave. NW, Suite 800
Washington, DC 20004
T: 202.785.3355
www.lung.org

Pulmonary Fibrosis Association
230 East Ohio Street, Suite 304
Chicago, Illinois 60611
T: 888.733.6741
www.pulmonaryfibrosis.org

Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd #343
Culver City, CA 90232
(888) 222-8541
www.coalitionforpf.org

The Pulmonary Paper
www.pulmonarypaper.org

References

  1. Merriam-Webster, http://www.merriam-webster.com/
  2. Prendergast TJ, Ruoss SJ, Seeley EJ. Chapter 9. In: McPhee SJ, Hammer GD, eds. Pathophysiology of Disease. 6th ed. New York: McGraw-Hill; 2010. Pulmonary Disease
  3. King, TE. Chapter 261. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 18th ed. New York: McGraw-Hill; 2012. Interstitial Lung Diseases
  4. American Lung Association, http://www.lung.org/lung-disease/pulmonary-fibrosis/
  5. Ganesh Raghu, Harold R. Collard, Jim J. Egan, Fernando J. Martinez, Juergen Behr, Kevin K. Brown, Thomas V. Colby, Jean-Francxois Cordier, Kevin R. Flaherty, Joseph A. Lasky, David A. Lynch, Jay H. Ryu, Jeffrey J. Swigris, Athol U. Wells, Julio Ancochea, Demosthenes Bouros, Carlos Carvalho, Ulrich Costabel, Masahito Ebina, David M. Hansell, Takeshi Johkoh, Dong Soon Kim, Talmadge E. King, Jr., Yasuhiro Kondoh, Jeffrey Myers, Nestor L. Mu¨ller, Andrew G. Nicholson, Luca Richeldi, Moise´s Selman, Rosalind F. Dudden, Barbara S. Griss, Shandra L. Protzko, and Holger J. Schu¨nemann, on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary FibrosisAmerican Thoracic Society Documents. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management
  6. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). American Journal Critical Care Medicine 2000, 161: 646-664.