“Tony award winning singer and longtime Las Vegan, Robert Goulet, is in critical condition at Cedar-Sinai Medical Center in Los Angeles. Goulet, who is 74, was rushed to a Las Vegas hospital in September, where he was diagnosed with interstitial pulmonary fibrosis, a rare but sometimes fatal condition. Goulet has been on a ventilator for about a month and is in need of a lung transplant.”
What is interstitial pulmonary fibrosis? Here is a synopsis:
Interstitial Lung Disease (ILD) is a general term that includes a variety of chronic lung disorders. When a person has ILD, the lung is affected in three ways. First, the lung tissue is damaged in some known or unknown way. Second, the walls of the air sacs in the lung become inflamed. Finally, scarring (or fibrosis) begins in the interstitium (or tissue between the air sacs), and the lung becomes stiff.
Shortness of breath is the main symptom of idiopathic pulmonary fibrosis. Since this is a symptom of many types of lung disease, making the correct diagnosis may be difficult. The shortness of breath may first appear during exercise. The condition then may progress to the point where any exertion is impossible. If the disease progresses, the person with IPF eventually may be short of breath even at rest. Other symptoms may include a dry cough (without sputum). When the disease is severe and prolonged, heart failure with swelling of the legs may occur.
People with different types of ILD may have the same kind of symptoms but their symptoms may vary in severity. Their chest X-rays may look alike. Further testing is usually recommended to identify the specific type of ILD a person has. Some ILDs have known causes and some (idiopathic) have unknown causes.
The tissue between the air sacs of the lungs is called the interstitium. Interstitial lung disease is named after this tissue because this is the tissue affected by fibrosis (scarring). Interstitial lung disease is sometimes also known as “interstitial pulmonary fibrosis.” The terms interstitial lung disease, pulmonary fibrosis and interstitial pulmonary fibrosis are often used to describe the same condition.
The course of these diseases is unpredictable. If they progress, the lung tissue thickens and becomes stiff. The work of breathing then becomes more difficult and demanding. Some of the diseases improve with medication if treated when inflammation occurs. Some people may need oxygen therapy as part of their treatment.
The diseases may run a gradual course or a rapid course. People with ILD may notice variations in symptoms — from very mild to moderate to very severe. Their condition may remain the same for long periods of time or it may change quickly.
Several causes of pulmonary fibrosis are known. They include:
1. Occupational and environmental exposures. Many jobs – particularly those that involve mining or that expose workers to asbestos or metal dusts — can cause pulmonary fibrosis. Workers doing these kinds of jobs may inhale small particles (like silica dusts or asbestos fibers) that can damage the lungs, especially the small airways and air sacs, and cause scarring (fibrosis).Agricultural workers also can be affected. Some organic substances, such as moldy hay, cause an allergic reaction in the lung. This reaction is called Farmer’s Lung and can cause pulmonary fibrosis. Other fumes found on farms are directly toxic to the lungs.
2. Sarcoidosis. A disease characterized by the formation of granulomas (areas of inflammatory cells), which can attack any area of the body but most frequently affects the lungs.
3. Drugs. Certain medicines may have the undersirable side effect of causing pulmonary fibrosis. Check with your doctor about the medicines you are taking and ask about any possible side effects.
4. Radiation. (treatment for breast cancer)
4. Connective tissue or collagen diseases such as rheumatoid arthritis and systemic sclerosis.
5. Genetic/familial. This is not as common as the other causes listed.
When all known causes of interstitial lung disease have been ruled out, the condition is called “idiopathic” (of unknown origin) pulmonary fibrosis (IPF).
Source:
Robert Goulet Dies: http://tinyurl.com/292xcg
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