pulmonary fibrosis?

Answer 1

These are all good answers, but JeanO is off on some of her information. This disease kills 50,000 people every year in the United States. At least that many people are diagnosed and many, many more are misdiagnosed. The only definitive diagnosis comes from a lung biopsy. The disease kills twice as many African Americans as any other race. It is believed that this disease may have environmental triggers, but is probably genetic in nature. One of the best places to find information out about pulmonary fibrosis is at:

www.pulmonaryfibrosis.org

It has been 4 years since my initial diagnosis, but investigation into old chest x-rays has shown I have had active disease since at least 1997. The average life span from initial diagnosis is 3 to 5 years and there is no cure. Research has shown the disease to respond to different medications, but none has reduced the mortality rate. Hope this helps a little.

Answer 2

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.

Answer 3

In the United States there are about 30 000 new cases each year with about 6 cases per 100, 000 in the UK. To quote my GP "We are talking hens' teeth here"

The progression of the disease is very variable with some people rapidly going down hill but others surviving for fifteen years or more. I was told the average was five years from diagnosis.

There are no racial preferences for the diease but there may be genetic links. It can strike at any age and I have heard of cases of babies developing it. It affects more non smokers than smokers and more females than males. In the UK it is often called Cryptogenic Fibrosing Alveolitus (CFA). Drugs may slow down the disease but the only real cure so far is a transplant.

Diagnosis is only certain after a sample has been taken, this can be from a wash to surgery. The indicators on a CT scan are areas that look like ground glass but they may be symptomatic of other dideases like NSIP - which I had.

I did an internet search when I was diagnosed as possible CFA and took a file into my GP.

Answer 4

Idiopathic pulmonary fibrosis, the most common form of IIP, causes progressive pulmonary fibrosis predominantly in male smokers. Symptoms and signs develop over months to years and include exertional dyspnea, cough, and fine (Velcro) crackles. Diagnosis is based on history, physical examination, chest x-ray, and pulmonary function tests and is confirmed with HRCT, lung biopsy, or both if necessary. No specific treatment has proven effective, but corticosteroids, cyclophosphamide, azathioprine, or a combination are often given. Most patients deteriorate even with treatment; median survival is < 3 yr from diagnosis.