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Know all about pulmonary fibrosis

Pulmonary Fibrosis is a chronic malady whose exact cause is unknown and therefore, it is termed idiopathic. In this progressively worsening condition, the lungs get scarred, and the healing process for unknown reasons repairs the damage with scar tissue, an inelastic fibrous connective tissue. These scar tissues cannot be replaced. Pulmonary fibrosis is not very uncommon. The individual types are rare and difficult to identify.

It is believed that various factors interact in the development of this disease, with heredity having an important role. Exposure to certain toxic chemicals, certain medications, and medical conditions, coal and silica dusts, asbestos fiber, heavy metal dust, radiation therapy, grain dust, and animal and bird dropping are all the major contributors. Long term smoking also seems to be a factor. With loss of elasticity the lungs cannot expand and contract restricting breathing. Loss of normal tissue affects the gas transfer resulting in shortage of oxygen in blood. As the disease progresses the lung becomes more and more scar tissue and restricts the blood flow leading to pulmonary hypertension which drags the heart into complications.

The disease normally starts with shortness of breath, inability to breathe deep, and reached a stage when breathing itself becomes painful. Constant tiredness, a dry hacking cough, weight loss which has no discernable cause, ache in muscles and joints and clubbing of fingers and toes are other symptoms of pulmonary fibrosis. The intensity of symptoms and the course of the disease vary widely among patients.

The progress of the disease can, in some cases, be slowed down by some modern drugs like pirfenidone and nintedanib the former produces rashes, nausea and diarrhea while the latter produces diarrhea and nausea. There are newer drugs for pulmonary fibrosis treatment which may have greater effects but have not got FDA approval yet. Some of the complications are pulmonary hypertension with later right ventricular failure –cor pulmonale and finally respiratory failure. Lung transplant in certain case can be an effective pulmonary fibrosis treatment .

It is fairly clear this is a disease with inferior prognosis and lots of suffering. Many studies are being conducted to find a cure to this illness and only time can tell how successful these would be.

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