Pulmonary fibrosis is a serious, life-threatening complicate of sickle cell disease. Pulmonary fibrosis is a combination of several lung disorders that affects the respiratory system, thus it is a lifelong lung disease, it causes lung scarring (this is the process that causes tissues to scar and thicken over time), the lung damage worsens gradually over time, and hard stiffened lung tissues do not expand as well as they should, making it much difficult to breathe.
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Pulmonary fibrosis affects the connecting tissue in the lung and the alveoli (the air sacs present in the lungs). Alveoli are tiny, very delicate air sacs in the lungs that help oxygen get into the bloodstream when one inhales air.
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Symptoms may come on at an early stage or it may take years to develop. No known standard cure yet, and so it eventually leads to death. Pulmonary fibrosis has been classified as interstitial lung disease. The interstitial tissues are cells that make up the space between blood vessels and other structures inside the lungs.
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Pulmonary fibrosis is capable of causing damage to these cells, it is a rare disease. Medications have been used to slow down scarring and prevent further complications and help preserve lung function.
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Idiopathic pulmonary fibrosis is the most common type of interstitial lung disease or pulmonary fibrosis. The known symptoms of pulmonary fibrosis are; breathing in short, shallow spurts, dry cough that does not go away, fatigue (extreme and excessive tiredness, no matter the amount of sleep one gets), shortness of breath, and weight loss.
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Pulmonary fibrosis is diagnosed by blood tests which provide details of any progressive disease, imaging tests which could be a chest X-ray or a computed tomography scan (CT scan) that may rule out other lung-related diseases, breathing tests called pulmonary function tests that show how well the lungs function, oxygen desaturation study that measures blood oxygen levels, and biopsy.
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