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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Idiopathic pulmonary fibrosis


Other Names for this Disease

  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis
  • Fibrosing alveolitis, cryptogenic
  • Hamman-Rich disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My wife was recently diagnosed with idiopathic pulmonary fibrosis. She is being treated with N-Acetylcysteine, an anti-oxidant supplement.  Is there a newer therapy or treatment for this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis is a condition in which tissue deep in the lungs becomes thick and stiff, or scarred, over time. As a result the lungs lose their ability to move oxygen to the brain and throughout the body.  Common symptoms include shortness of breath, and dry, hacking cough. “Idiopathic” refers to cases that’s cause is unknown.[1]
Last updated: 12/9/2008

How is idiopathic pulmonary fibrosis treated?

The goals of treating idiopathic pulmonary fibrosis (IPF) are to prevent more lung scarring, relieve symptoms, maintain the ability to be active, keep the individual healthy, and improve the quality of life.[1] Treatment is usually based on age, medical history, co-existing medical problems, and severity of the fibrosis.[1] In general, treatment may include medicines to reduce inflammation in the lungs and to prevent more scarring, oxygen therapy, pulmonary rehabilitation, and lung transplantation.[1]
Last updated: 12/9/2008

Are there any research studies investigating new therapies for the treatment of idiopathic pulmonary fibrosis?

Yes. The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently, there are a number of clinical trials enrolling individuals with idiopathic pulmonary fibrosis. Many of these trials are investigating new therapies. To find these trials, click here. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if your wife is eligible for any clinical trials.  

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov
Web site: http://clinicalcenter.nih.gov/  

If your wife is interested in enrolling in a clinical trial, she can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand  

A tutorial about clinical trials that can also help answer her questions can be found at the following link from the National Library of Medicine:
http://www.nlm.nih.gov/medlineplus/tutorials/cancerclinicaltrials/htm/lesson.htm  

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases (ORD), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8  
Last updated: 12/9/2008

References
Other Names for this Disease
  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis
  • Fibrosing alveolitis, cryptogenic
  • Hamman-Rich disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.