What Is Pulmonary Fibrosis?

By John Lindell

Pulmonary fibrosis is the final stage of a lung disease that creates scarring in the lungs. Many disorders cause pulmonary fibrosis, which injure the small air sacs that line the lungs, the alveoli, the main factor in the disease. The scarring, or fibrosis, of the lungs makes it hard to breathe and can lead to death in severe cases. There is no cure for pulmonary fibrosis; a lung transplant is a person's only option.


When you inhale, tiny particles end up in your lungs. These are usually expelled, but some still linger in the lungs. Long-term exposure to substances such as asbestos and silica dust, chemical fumes and dust can result in damage to the lungs. The lungs compensate by producing immune system organisms, but the particles are too resistant and result in scarring. Over time, the lungs lose elasticity and the ability to transfer oxygen to the bloodstream.


Some of the symptoms of pulmonary fibrosis include a pronounced shortness of breath, making physical activity difficult. Coughing, tiredness, weight loss, loss of appetite, painful muscles and joints and chest pain are all signs of this disease. Many people attribute some of these symptoms to being out of shape physically and the disease goes undiagnosed. The symptoms will get worse as time passes, with the speed of the disease's progress varying from person to person.


It can often be tough for doctors to diagnose pulmonary fibrosis. It mimics other lung ailments and many times the cause cannot be detected. Physical exams and X-rays may not be enough to identify it, leading to such tests as CT scans, MRIs and biopsies where pieces of lung tissue are removed to be looked at closely. Knowing your history of previous exposure to substances that precipitate pulmonary fibrosis such as asbestos can give the doctor added clues.


Pulmonary fibrosis has many complications, all of them quite serious. It will reduce the amount of oxygen you can take in, leading to low oxygen levels in the blood, leaving you short of breath and fatigued. Raised blood pressure in your lungs can turn deadly in addition to right-sided heart failure, both caused by scar tissue in the lungs from fibrosis. Respiratory failure is often the last stage of the condition, with blood oxygen levels precariously low and carbon dioxide build-up in the bloodstream; this usually is fatal.


Scarring of lungs cannot be reversed by medical science and nothing can stop the scarring involved with pulmonary fibrosis from continuing. Steroids can be given, but they have serious side effects in most people. The attempt will be made to improve the quality of the patient's life, with oxygen therapy helping the person to breathe easier. Pulmonary rehabilitation can help the person to breathe easier. Younger patients may be eligible for lung transplants.

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