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Re: A message from Tracie re: Early Pulmonary Fibrosis Symptoms Are Easy to Ignore

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Great info. Thanks for sharing!Blessings,Beckytiodaat@... wrote: An article from MCW Healthlink was sent to you by Tracie with the following note: From . ==================================== TITLE: Early Pulmonary Fibrosis Symptoms Are Easy to Ignore ORIGINAL URL: http://healthlink.mcw.edu/article/1031002711.html ABSTRACT: Pulmonary fibrosis, a hardening of the

lungs caused by scarring, progressively impairs one's ability to breathe. Patients typically have symptoms for five years before they seek a doctor's advice, says Dr. Rose Franco. ARTICLE: Pulmonary fibrosis, a hardening of the lungs caused by fiber-like scarring, is a condition that progressively impairs one's ability to breathe. It is fortunately very rare, occurring in less than 1% of adults, but accounts for about 40,000 deaths a year in the US. With pulmonary fibrosis, "The lungs lose their sponginess and become very stiff, impairing their ability to deliver oxygen to the bloodstream," explains Rose Franco, MD, Assistant Professor of Pulmonary/Critical Care Medicine and Associate Director of the Sleep Disorders Program at the Medical College of Wisconsin. She is a pulmonary specialist who often cares for patients with this condition. Dr. Braza practices at the Froedtert & The Medical College of Wisconsin Pulmonary Medicine

Clinic. Unfortunately, patients typically have symptoms for five years before they seek a doctor's advice, says Dr. Franco. "Our bodies are so well constructed that you can lose more than half of your lung capacity before you notice any problem," she notes. "In the vast majority of cases, the doctor will have to carefully review the patient's work history, medical history and medication history, then follow up with a variety of tests to determine the cause of the fibrosis and what course to follow," she says. Inflammation Leads to Scarring Pulmonary fibrosis occurs when lung cells or the spaces between them fill up with either proteins or inflammatory cells, blocking the flow of oxygen through the blood vessels. Fibrosis first shows up in the very narrow "interstitial" walls between lung cells, and eventually involves the alveoli (air sacs), the bronchioles (small, branching tubes that conduct oxygen to the air sacs), and the capillaries (the

tiny blood vessels that carry oxygen) of the lungs. "Over time, the inflammatory cells will cause the destruction of air sacs and blood vessels," says Dr. Franco. "Then you have more trouble getting oxygen into the system and getting rid of carbon dioxide. The air sacs and blood vessels have become scarred and non-functional." The primary symptoms of pulmonary fibrosis are quite general, and could be explained by a number of health problems. Symptoms include: -Fatigue and weakness -Discomfort in the chest -Loss of appetite -Rapid weight loss But while some of these symptoms may appear minor, it is critical to be alert to early signs of the disease. Seeing a doctor is very important if the symptoms persist, stresses Dr. Franco. "Be especially aware of a persistent cough that lasts for more than three months, or shortness of breath that is out of proportion to that of your peers with the same level of exertion."

Varieties of Pulmonary Fibrosis Pulmonary fibrosis is a large category, covering a variety of severe lung conditions that lead to inflammation and possibly scarring, says Dr. Franco. Among them: -Pneumoconiosis: Some occupational dusts cause intense inflammation of the lungs. The most common lung diseases are associated with regular contact with organic dusts such as coal (producing "brown lung"), asbestos, or silica. -Systemic Collagen Vascular Diseases: Fibrosis can also be the outcome of such systemic diseases as lupus or rheumatoid arthritis (collagen is a tough, glue-like protein; malfunctioning can cause a hardening of lung tissue). These diseases can result in progressive scarring of the lung and loss of function. -Wegener's Granulomatosis: This rare condition results in destructive inflammation of the blood vessels (vasculitis), which damages the lungs and kidneys by impairing blood flow and destroying normal tissue. -Hypersensitivity

Pneumonitis: This is a condition that usually occurs among farmers working with moldy, wet hay. It occurs much more frequently in whites than in African-Americans, where the incidence is very rare. People with this condition may develop hypersensititivity to the bacterium or mold; recurrent episodes of pneumonitis eventually progress to scarring (fibrosis) if not recognized early and therapy instituted. -Sarcoidosis: This condition produces inflammatory clusters of cells called granulomas, which often occur in the lung tissue. Scarring of the lungs will develop in only about a third of people with sarcoidosis. Sarcoidosis is found in disproportionately high numbers among African-Americans in the Midwest and it is thought that some cases may be due to a genetic predisposition. -Idiopathic Pulmonary Fibrosis: This category covers those cases where no clear cause can be determined. Most pulmonary fibrosis cases fall into this category. Testing "If

pulmonary fibrosis is suspected, we will do CAT scans to determine where the lungs may be inflamed and where they are scarred. We will also perform breathing tests, both at rest and with activity," Dr. Franco notes. This testing allows the doctor to measure how the lungs are functioning and can also be used to monitor the progress of the disease. In fibrosis the lungs will shrink as they harden, leading to decreased total lung capacity and decreased diffusion capacity for gases. Frequently, in the early stages of disease, testing the patient during activity is especially revealing. "A test involving walking and simultaneously measuring the oxygen level through the skin (pulse oximetry) may be the only place where the scarring shows up," says Dr. Franco. "We will also do blood tests for collagen vascular disease. It turns out that arthritic conditions such as scleroderma, lupus, and rheumatoid arthritis not only affect the joints, but also the lungs," she

states. "We often also do an echocardiogram to look at the heart structure and make sure that the heart isn't the source of the breathing problem." In many cases, making the diagnosis requires a lung biopsy. There are two varieties. A bronchoscopy is used when sarcoidosis is suspected. In this procedure a lighted scope will be inserted through the throat into the air sacs to retrieve a tiny sample of tissue about the size of the tip of a pen. The other type of biopsy is surgical, and is conducted by a cardiothoracic surgeon. A small incision is made in the chest and a tiny scope is slid between the ribs to take tissues from two or three spots. "This yields much better results and gives us a better set of samples," says Dr. Franco. "But this option is used when we know it isn't sarcoidosis and the patient is not too ill to tolerate general anesthesia and a lung surgery." Prevention A substantial number of pulmonary fibrosis cases cannot be

explained by hereditary factors. "In most cases, its sheer bad luck," says Dr. Franco. However, there are definite preventive steps that can be taken: -Be proactive about the possible threats to your lung health from your workplace and hobbies. Learn about the possible health risks of the tools and materials you use. At work, these can include lubricants used in the auto industry, asbestos, silica, and coal dust from metal or wood shavings. Hobbies that involve the use of propellants for spray painting or coating projects can also pose a threat. -One of the most important steps in preventing lung disease is using a respirator to protect your lungs from substances that could cause scarring. In some cases, patients might need to make the move to a less risky occupation. -Be alert to any cough that lasts more than three months, or unusual shortness of breath after activity. "Surprisingly, the condition is not associated with smoking by itself," says Dr.

Franco, while stressing that smoking strongly aggravates pulmonary fibrosis and is to be strictly avoided. "In the majority of cases we see, something else is contributing." Lung Transplants For some pulmonary fibrosis patients, a lung transplant can be a viable solution. But the wait for a lung transplant can be long, and Dr. Franco notes that there is "a very limited group of donors" whose lungs will produce a sufficient match. Despite the twin difficulties of a short supply of donor lungs and finding the right match, the process of transplantation has become more successful over the past two decades. "Lung transplants have been done since the 1980s, and long-term success for these transplant recipients has improved greatly as we have become smarter about controlling the complications of transplantation," observes Dr. Franco. To increase the availability of transplanted lungs to the living, Dr. Franco urges everyone to sign the

donor statement on the back of their drivers' licenses. Courses of Treatment "We haven't found anything that will cure pulmonary fibrosis," says Dr. Franco. "That is especially true for idiopathic cases because we simply have no proven treatment. Most patients have progressive decline in their lung capacity despite treatment and many patients succumb to the disease within two years because of pneumonia or cardiac problems. In every case, one of our goals is to improve the patient's quality of life." "Oxygen treatment is good for relieving breathlessness and reduces the strain on the heart. Typically, oxygen helps patients to stay active so that they don't become deconditioned" (so unaccustomed to movement that the muscles weaken, lose tone, and atrophy). For patients with less acute, non-specific inflammation, the steroid prednisone can be effective. "Prednisone is able to slow down the inflammation, and in some cases puts the scarring

process into remission," she says. "If we have any hope of improving the chances of saving lung function for people suffering from pulmonary fibrosis, it's before their lungs are scarred and still in an inflammatory stage," Dr. Franco says. "We can't resurrect scar tissue into normal tissue, so we have to fight the battle to keep the scarring from progressing." There are numerous current national trials being undertaken to study new therapies for pulmonary fibrosis; most of them require that patients are enrolled when their condition is not far advanced. "The benefit of these drugs may only be seen when patients are diagnosed and treated early in the disease," says Dr. Franco. "Early diagnosis so very important and patient participation in national trials is our only hope for finding an effective cure for this disease." =========================== The MCW Heathlink Website and FREE email newsletter are provided by

the Medical College of Wisconsin as part of their outreach and community service efforts. To subscribe to the email newsletter, please go to http://healthlink.mcw.edu/content/subscribe.cgi PLEASE NOTE: MCW HealthLink is provided for educational purposes. The information is not intended as a substitute for medical care, or for the advice of a physician. Please consult your doctor to determine how the information here pertains to you. --------------------------- Copyright 2001 Medical College of Wisconsin

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