Pulmonary fibrosis
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Pulmonary fibrosis is generally considered a fatal disease. Over time, the lungs become scarred and reduce the capacity for breath and oxygen consumption. Pulmonary fibrosis is considered the end stage of interstitial lung disease, which simply refers to a variety of lung diseases that lead to scarring of the lung tissue. Most physicians believe that pulmonary fibrosis is a progressive lung disease that begins with recurrent damage to the small air sacs in the lungs known as alveoli. Scarring, which is known as fibrosis, ultimately prevents the lungs from expanding and being able to take deep, full breaths, leading to inept oxygen saturation.


The symptoms of pulmonary fibrosis are likely to go unnoticed until the disease has advanced well beyond the stage of reversible lungs damage. In most cases, patients believe that their shortness of breath and their dry cough are the results of a lingering cold or getting older. Most patients do not realize the extent of their situation until after they are unable to breathe through the most mundane chores like eating and walking from one room to another.

At this point, additional symptoms are likely to emerge, such as chronic fatigue, significant and unexplained weight loss, and muscles and joints that ache or hurt. Other symptoms might include chest pressure or an inability to catch one’s breath.


The human lung is quite intricate and understanding the causes of pulmonary fibrosis require an intricate understanding of the lungs. The air that travels into the lungs eventually ends up in the tiny air sacks called alveoli, where gases are exchanged in the process of human breathing. However, when damaging particles or other elements are able to mar the thin interstitium tissue that is responsible for the lining and separating of the small air sacs, the sacs may not heal. Even microscopic damage can accumulate over time and lead to significant scarring of these air sacs. This, in the end, results in pulmonary fibrosis.


There are various risk factors associated with pulmonary fibrosis including, age, gender, occupational hazards and other environmental concerns, previous radiation and chemotherapy, smoking, certain viruses, and genetic factors. While there have been small children and infants who have developed the disease, most victims range in the middle to later adult years. Men are more susceptible to the disease than women, and people who have worked in environments laden with potentially harmful airborne debris run a very high risk of developing the disease.
pulmonary fibrosis
Image: Pulmonary Fibrosis


Pulmonary fibrosis can resemble many various diseases, including heart disease. A physician will systematically rule out other possibilities before identifying the problem as pulmonary fibrosis. The only definitive test available for definite determination remains a biopsy of the tissue of the air sacs. A bronchoscopy is usually one of the preferred methods of extracting tissue for a biopsy. This procedure entails the use of a very small and flexible tube which removes tissue samples about the size of a pin head. A surgical biopsy may be done when this option is not practical or unavailable.


Complications related to pulmonary fibrosis can be severe and life threatening. Heart failure involving the right side, pulmonary hypertension (the increase of blood pressure inside the lungs) low blood oxygen levels, and respiratory failure. Any one of these complications has potential life ending possibilities.


Treatment options are not as progressive as most patients would hope. Many patients are initially treated with corticosteroids in order to help suppress the immune system. Thus far, no treatment options have shown any real signs of slowing the progression of the disease nor helped to alleviate the symptoms.

For young and otherwise healthy patients, a lung transplant offers the only hope of real improvement. Of course, with transplant lists being bogged with recipients and sparse with donors, finding a good match can be very difficult.

Some patients who combine treatment with prednisone and add 600 milligram doses three times daily of the enzyme N-acetylcysteine have experienced a greater relief of symptoms for a longer period of time. Not all patients respond to this sort of treatment, but those who do have a better quality of life for longer. Oxygen therapy and pulmonary rehabilitation can also help improve the patient’s quality of life.

Clinical trials can often open up new windows for patients, and there are usually several that are in need of willing participants. Patients should consider their participation in a clinical trial carefully. Those who participate offer a greater chance not only to themselves, but to others as well.
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