Pulmonary Fibrosis

Pulmonary fibrosis (PF) results from scarring of the lung, a process which gradually replaces the air sacs of the lungs with dense fibrotic tissue. This scarring thickens lung tissue and causes an irreversible loss of the lung’s ability to transfer oxygen into the bloodstream.

  • The symptoms of pulmonary fibrosis include:

    • Shortness of breath, particularly with exertion
    • Chronic dry, hacking cough
    • Fatigue and weakness
    • Discomfort in the chest
    • Loss of appetite
    • Rapid weight loss

There is a growing body of evidence which points to a genetic predisposition for pulmonary fibrosis. Because the origin and development of the disease is not completely understood, however, misdiagnosis is common. Available scientific evidence indicates that the scarring process is a reaction to microscopic injury of the lung.

  • The onset of PF has been associated with:

    • Inhaled environmental or occupational pollutants
    • Diseases such as scleroderma, rheumatoid arthritis, lupus and sarcoidosis
    • Certain medications
    • Therapeutic radiation

There are currently no effective treatments or a cure for pulmonary fibrosis. Supplemental oxygen improves patients’ quality of life and exercise capacity. Single lung transplant may be considered for some patients. The pharmacological agents designed to treat lung scarring are still early in their development, while treatments intended to suppress inflammation have shown only limited success in reducing the fibrotic progress.

Mortality rates range from 50 to 70%, and the average duration from diagnosis to death is about 2 to 4 years. Spontaneous remissions do not occur.  Better survival has been associated with younger age, female gender, earlier stage of disease and a beneficial response to corticosteroids.  Lower survival has been associated with male gender, advanced disease (worse pulmonary function tests and X-rays) and certain cellular defects.

Human and Social Costs
Pulmonary fibrosis affects 200,000 people in the United States and five million people worldwide. Over 40,000 deaths result from this disease, a toll on par with that of breast cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven in to the eighties. Current research indicates that many infants are afflicted by the related pediatric interstitial lung disease.

The Potential for Stem Cell Cures and Therapies
Stem cell research currently offers tremendous promise for an effective cure or treatment for pulmonary fibrosis. Recent evidence indicates that abnormal stem cells may be to blame for the overproduction of the fibrous tissue that causes PF, raising the possibility that the disease is treatable by replacing these cells with healthy new ones.

Dr. Anne Bishop of the Imperial College Tissue Engineering and Regenerative Medicine Centre at Chelsea and Westminster Hospital, comments: "This research will make it possible eventually to repair lungs that have been damaged by disease, by implanting fully functioning lung cells to repopulate damaged areas. Also, unlike transplantation from a donor, the cells can be developed in such a way that the body will not reject them."

That’s why many people suffering from pulmonary fibrosis and other respiratory ailments, their families and groups like Cure Pulmonary Fibrosis & COPD and Cystic Fibrosis Research, Inc., strongly support stem cell research – and have endorsed the California Stem Cell Research and Cures Initiative.

Partial list of sources:

The Pulmonary Fibrosis Foundation
- http://www.pulmonaryfibrosis.org/ipf.htm

Duke University
- https://www.fpf.duke.edu/disease.html

Imperial College London, Tissue Engineering & Regenerative Medicine Centre
- http://www.med.imperial.ac.uk/Divisions/8/index.html

The University of Vermont, Vermont Lung Center

Ali NN, Edgar AJ, Samadikuchaksaraei A, Timson CM, Romanska HM, Polak JM, Bishop AE. Derivation of type II alveolar epithelial cells from murine embryonic stem cells. Tissue Engineering 2002; 8: 541-550 [Abstract]

BT Suratt, CD Cool, AE Serls, M Varella-Garcia, EJ Shpall, KK Brown, GS Worthen, (April 30, 2003), Human pulmonary chimerism following hematopoietic stem cell transplantation. Am. J. Respir. Crit. Care Med. 2003, doi:101164.200301-1450C. [Abstract]

Health News, The First Evidence Of 'Chimerism' In The Human Lung, July 30, 2003

Australian Broadcasting Corporation, August 24, 2003, “Stem Cell Research Brings Lung Disease Cure Closer

Paid for by YES on 71: Coalition for Stem Cell Research and Cures, #1260661
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